<*-££. Class rNV . 3A \ Book , Y f ffi GpightN CQFVRIGIIT DEPOSIT. PLATE I Opaque Optic-Nerve Fibres. — (After Beard.) — This is aeon- genital condition of the fundus oculi, in which the medullary substance of the optic-nerve fibres continues out over the retina instead of stopping at the papilla as it normally should do. In this condition and location we obtain the most direct and complete observation of the intact nervous tissue during life that it is possible to have. A TREATISE ON DISEASES OF THE NERVOUS SYSTEM BY L. HARRISON METTLER, A.M., M.D. Associate Professor of Neurology, College of Medicine of the University of Illinois; Professor of Mental and Nervous Diseases in the Chicago Clinical School; Con- sulting Neurologist to the Norwegian Deaconess' Home and Hospital, Chicago. Complete in One Volume grofusetu ^Illustrated CLEVELAND PRESS CHICAGO 1905 LIBRARY Of CONGRESS Two Copies Received' DEC 22 1904 ^Copyrutm entry CUSS JO XXc, Noj copy b. Copyright 1904 BY THE CLEVELAND PRESS CHICAGO PREFACE The Neurone Doctrine is now an accepted fact. Its teachings have done more to illuminate the dark places of neurology than has any single scientific generalization heretofore promulgated. In spite of the fact that in regard to many of its details much has yet to be learned, the main principles which it lays down are universally acknowledged to be scientifically accurate and practically useful. The present treatise has been written with the view of presenting the subject of neurology in consonance with this doctrine. The diseases are classified, so far as possible, upon that basis. The neuronic structure of the nervous sys- tem is given special emphasis. And the role of the neurone in the matter of the pathology and symptomatology of these diseases is kept well in view. The author feels that the time has arrived for the frank * recognition of this great doctrine, not merely in histology but also in the greater field of neurology. He is convinced that one cannot acquire a proper conception of modern neurology without an adequate knowl- edge of the neuronic structure of the nervous system. Mistakes in the diagnosis and blunders in the treatment of diseases of the nervous sys- tem can be minimized only by keeping well in mind the facts taught in the neurone theory. If the present volume will enable the student and the practitioner to behold the entire field of neurology — modern neu- rology — under the brilliant illumination cast upon it by this scientific generalization, the highest wish of the author will have been attained. The unity of the nervous apparatus and its functions, the author believes, is not to-day taught as emphatically as it should be. In some quarters the tendency is marked to discuss the nervous system and its diseases in such minute detail that the universal working of the nervous system as a single, uniform, albeit compound organ, seems to have been lost sight of. This is shown particularly in connection with the subject of the cerebral localizations. Psychology is a science as well as neurology ; and as neurosis underlies psychosis, it is incumbent that a proper conception of modern psychology should be possessed to fully comprehend the nature of the cerebral localizations, the psychic symptoms of nearly all the nervous affections and the entire psychic side of neurology. The author has endeavored to make plain from the O PREFACE standpoint of modern psychology many of the psycho-physical manifes- tations of the nervous organ. In the presentation of the individual diseases, their diagnosis and pathology have been especially dwelt upon, the writer being of the firm opinion that treatment and general therapeutics are absolutely valueless — sometimes positively dangerous — in the absence of a cor- rect or approximately correct diagnosis. Neurology's hardest prob- lems are in the diagnostic sphere. Given a correct diagnosis, the treat- ment is generally a very simple affair. Diagnosis and pathology rest upon anatomy and physiology, hence much space has been given to normal as well as abnormal neurology in the following work. The proper classification of some nervous affections is still a mat- ter of uncertainty. One has but to recall such troubles as the myo- pathies, the periodical paralyses, Landry's paralysis, certain types of polyneuritis, exophthalmic goitre, progressive facial hemiatrophy, my- asthenia gravis. Bell's mania, disseminated sclerosis, hypertrophic pachymeningitis and many others. The author has classified them in accordance with his own view and with what he believes to be the general view among the best authorities. For valuable assistance and helpful suggestions the author feels his indebtedness to many friends and desires to avail himself of this opportunity to thank especially Dr. Walter M. Fitch for some exquisite photographic work ; Dr. Charles H. Beard, Dr. Oscar Dodd and Dr. Willis O. Nance for their ophthalmological assistance ; Dr. G. B. Hassin and others. Grateful acknowledgment is also extended to the various publish- ing firms who have courteously permitted the use of illustrations from works issued by them. L. H. M. TABLE OF CONTENTS SECTION A. INTRODUCTION. Peculiarities of the Nervous System and its diseases ly Classification of nervous diseases 22 Etiology of the diseases of the nervous system 25 Degeneracy 29 Pathology of the diseases of the nervous system 35 Symptomatology and Diagnosis of the diseases of the nervous sys- tem 41 Examination of the patient 42 Electro-diagnosis 64 Treatment of the diseases of the nervous system 75 SECTION B. THE NEURONIC DISEASES. The Neurone and the Neuronic structure of the nervous system . . 89 The Neurone Doctrine 89 The Neuronic Architecture of the nervous system 96 The Neuroses 101 a. The cerebrospinal neuroses 102 Hysteria 102 Psychoneuroses related to Sleep and Hysteria 138 Dreams 141 Pavor nocturnus 141 Nightmare 141 Automatism 142 Somnambulism 142 Hypnotism and hypnosis 143 Catalepsy 146 TABLE OF CONTEXTS Lethargy 146 Insomnia 146 Prolonged sleep 148 Sleeping sickness. Negro lethargy 148 Vertigo 149 Ocular vertigo 152 Aural vertigo. Meniere's disease 153 Neurasthenia 157 Hypochondriasis 173 The Traumatic Neuroses 182 Epilepsy 188 Myoclonus-epilepsy 207 I Icmicrania. Migraine 221 I Ieadache. Cephalalgia 234 The Neuralgias 242 Tic douloureux, prosopalgia 248 Glossodynia . . . , 250 Occipital neuralgia 250 Cervico-brachial neuralgia 251 Mastodynia 251 Lumbar neuralgia 251 Coccydynia 251 Pododynia 252 Visceral neuralgia 252 Spermatic neuralgia . 252 Urethrodynia 252 Eclampsia 252 Eclampsia infantum . . 25 2 Eclampsia parturientium 254 Eclampsia unemica 255 Te tany. Tetanilla 2^6 I .ocalized myospasms 262 1 a icalized Spasmodic Tic. Tic convulsif 264 I ; aeial tic A I asticatory tic 266 I [ypoglossal tic 266 Tic of the trunk and extremities 266 Respiratory tic 266 Spasmodic torticollis 267 Spasmus nutans 268 I reneral Tic. Tic impulsif 269 "Jumpers," Mirvaelnt, Latah 270 TABLE OF CONTEXTS 9 Paramyoclonus multiplex 271 Occupation Neuroses 272 Myotonia congenita 284 The Choreas 286 Acute Minor chorea. Sydenham's chorea 287 Symptomatic chorea 297 Chronic hereditary (Huntington's) chorea 297 Electric chorea 299 Paralysis agitans. Shaking palsy 299 b. The sympathetic neuroses. Angioneuroses and trophoneu- roses 306 The Sympathetic Nerve ; anatomy and physiology 306 Symmetrical gangrene. Raynaud's disease 310 Erythromelalgia 314 Acroparsesthesia 316 Angioneurotic oedema ' 318 Chronic hereditary trophcedema '. 321 Intermittent articular hydrops 321 Exophthalmic goitre 322 Athyrea 334 Myxcedema 335 Cretinism 337 Acromegaly 339 Gigantism 343 Adiposis dolorosa 345 Scleroderma 346 Progressive facial hemiatrophy 348 Ainhum 350 The System Diseases 350 Their nature and classification 350 Diseases of the Afferent or Sensory System 367 Locomotor ataxia 367 Diseases of the Efferent or Motor System 392 Progressive muscular atrophy 392 Amyotrophic lateral sclerosis 401 Progressive bulbar paralysis 406 Progressive muscular dystrophy 409 Pseudo-hypertrophic paralysis 416 Juvenile dystrophy 418 Infantile dystrophy 418 Spastic paraplegia of adults 421 Spastic paraplegia of childhood 425 10 TABLE OF CONTENTS Secondary spastic paralyses 426 Ophthalmoplegia 427 Myasthenia gravis 429 Occupation muscular atrophy. Craft palsy 430 Acute ascending (Landry's) paralysis 431 Periodical paralysis 436 Diseases of the Afferent and Efferent Systems 437 Ataxic paraplegia 439 Hereditary ataxia 444 Friedreich's disease 444 Amaurotic family idiocy 451 Multiple neuritis and neuromyelitis 454 Progressive interstitial hypertrophic neuritis 454 SECTION C THE NON-NEURONIC DISEASES. The Nature of the non-neuronic diseases 457 Part I. Non-neuronic diseases of the Spinal Cord 459 The Spinal Cord ; its anatomy and physiology 459 Spinal localization and topography 485 Lumbar puncture 494 Diseases of the Spinal Column 496 Dislocations and fractures of the vertebrse 496 Caries of the vertebrse. Tubercular spondylitis 499 Tumors of the vertebrse 507 Syphilitic disease of the vertebrse 509 Aneurismal erosion of the vertebrse 509 Spinal hydatid disease 509 Lateral curvature of the spine 509 Arthritis deformans of the spine 510 Diseases of the Membranes of the Spinal Cord 511 Ansemia and hypersemia 511 Inflammation of the spinal membranes. Meningitis 512 External pachymeningitis 512 Chronic cervical hypertrophic pachymeningitis 514 Acute spinal leptomeningitis 516 Syphilitic spinal meningitis. Spinal syphilis 519 Spinal meningeal hemorrhage. Hematorrhachis 524 TABLE OF CONTENTS II Diseases of the Spinal Cord 526 Anaemia 527 Hyperemia 527 Embolism. Thrombosis. Endarteritis 528 Spinal hemorrhage. Hematomyelia 528 Myelitis 531 Acute anterior poliomyelitis of infants 541 Poliomyelitis of adults , . . . 552 Abscess of the cord 555 Tumors of the spinal cord 555 Cavities of the spinal cord 562 Hydromyelia 563 Syringomyelia 563 Malformations of the cord. Spina bifida 569 Caisson disease 570 Diseases of the Cauda Equina and Filum Terminate 573 Part II. N on-neuronic diseases of the Peripheral Nerves 576 The Peripheral Nerves ; their anatomy and physiology 576 Neuritis 581 Multiple neuritis 589 Alcoholic multiple neuritis 592 Lead multiple neuritis 595 Arsenical multiple neuritis 597 Argentic multiple neuritis 598 Mercurial multiple neuritis 598 Diphtheritic multiple neuritis 599 Rheumatic multiple neuritis 602 Puerperal multiple neuritis 602 Beriberi or Kakke 603 Akatama 603 Leprous multiple neuritis 604 Tumors of the Nerves. Neuromata 600 Multiple neuromata 609 Fibroma molluscum multiplex 6og Plexiform neuroma 610 Diseases of the Spinal Nerves 613 Phrenic nerve 613 The brachial plexus 614 Erb's palsy 616 Klumpke's palsy 616 Total plexus palsy 616 Obstetrical palsy 617 12 TABLE OF CONTENTS Posterior or long thoracic nerve 617 Circumflex nerve 618 Musculo-cutaneous nerve 61 8. Suprascapular nerve 618 Musculo-spiral nerve 619 Median nerve 621 Ulnar nerve . 622 The dorsal nerves 624 The lumbar plexus 624 Obdurator nerve 626 Anterior crural nerve 626 The sacral plexus 626 Sciatic nerve. Sciatica 627 Plantar nerves » 635 The muscles of the body, their functions and nerve supply 636 Diseases of the Cranial Nerves 643 Oculomotor or third nerve 646 Trochlear or fourth nerve 648 Abducens or sixth nerve 64S Trifacial or fifth nerve 649 Facial or seventh nerve 652 Auditory or eighth nerve 657 ( ilossopharyngeal or ninth nerve 658 Pneumogastric or tenth nerve 659 Accessorius or eleventh nerve 660 1 Iypoglossal or twelfth nerve 661 I 'art III. Xon-neuronic diseases of the Brain 662 The Brain, its anatomy 662 Weight of the brain 664 Membranes of the brain 1 Embryological development 668 Lobes, convolutions, fissures, sulci 672 Cranio-cerebral topography 677 Minute structure of the brain 680 Cortex 68l ( Ufactory lobes (>S<> Corpora striata 6 ( )ptic thalami 691 ( )ptic tracts and retina . . . .692 Regio subthalamic^ 695 Corpora quadrigemina (105 Peduncles «»i" the brain 1 TABLE OF CONTENTS I Cranial nerve nuclei -. 698 Olivary bodies 705 Cerebellum -...■, . . . 705 Cerebellar cortex 707 Conducting tracts of the brain 710 Circulation of the brain . 710 Brain physiology and cerebral localization 714 Cortex ...;.. 717 Internal capsule . 736 Corpus callosum ......: 736 Corpora striata 737 Optic thalami 737 Corpora quadrigemina, geniculate bodies and pulvinar 73S Red nucleus, etc 739 Corpora mamillaria 739 Hypophysis cerebri ............. 739 Epiphysis cerebri ,.-..■ 739 Crura cerebri 739 Pons and medulla . . ..»....: 740 Cerebellum .....:...;...... 740 Olivary bodies 741 Symptoms of diseases of the brain . . -749 General symptoms . . 751 Psychic symptoms - 753 Disturbances of language . 763 Motor symptoms 772 Sensory symptoms .....:.... yyy Olfactory symptoms 779 Optic symptoms 780 Gustatory symptoms 792 Auditory symptoms , . . . 792 Diseases of the Membranes of the Brain 793 Pachymeningitis 793 Hsematoma 794 Leptomeningitis 798 Acute leptomeningitis .798 Epidemic cerebrospinal meningitis ........... 808 Tuberculous meningitis 814 Chronic meningitis 820 Circulatory diseases of the Brain 821 Cerebral anaemia 822 Cerebral hyperemia 825 14 TABLE OF CONTENTS Cerebral hemorrhage 828 Encephalomalacia. Embolism and thrombosis 845 Thrombosis of the intracranial veins and sinuses 850 Infantile hemiplegia 854. Encephalitis 864 Acute hemorrhagic encephalitis 865 Acute suppurative encephalitis ; cerebral abscess 867 Acute delirium. Bell's mania 874 Cerebral syphilis 877 Dementia paralytica. General paresis 888 Multiple sclerosis 901 Tumor of the brain 909 Intracranial aneurism 934 Hydrocephalus 937 Serous meningitis 939 Acquired chronic hydrocephalus 941 Congenital hydrocephalus . . 942 Diseases of the Pons Varolii and Medulla oblongata. Acute superior polioencephalitis 951 Acute inferior polioencephalitis 952 Apoplectiform bulbar paralysis 952 Diseases of the Cerebellum 954 Malformations of the Brain 956 SECTION D. GENERAL MALADIES WITH LEADING NEUROLOGICAL SYMPTOMS. Alcoholism qcq The opium and kindred habits ofo Tetanus q^c Hydrophobia 053 Arthritis deformans q5q Other toxaemic troubles q 7I SECTION A. INTRODUCTION. GENERAL CONSIDERATIONS UPON THE PECULIARITIES OF THE NERVOUS SYSTEM AND ITS DISEASES. The Nervous System is a unique and complex apparatus for the performance of unique and complex functions. Neither its anatomy nor its physiology is like that of any other organ of the body. When rough- ly contemplated as a mass of tissue, constituting the center, as it were* of the entire organism, it has more or less of a uniformity of structure and a singleness of function. When regarded minutely in its multitu- dinous relations to all the various parts of the body, it is a very com- plex, compound organ with a large variety of functions. Nervous matter per se consists of a single element and its support- ing environment immensely multiplied. The two functions of this ele- ment are self-nutrition and the manifestation of a special form of irrita- bility. A study of nervous matter, whether found in the brain, the spinal cord or the peripheral nerves, exhausts itself when the nervous cell with its' processes is analyzed. Its functions are summed up in the two words, self-nutrition and irritability. Its supporting environment of neuroglia, connective tissue and blood vessels is, strictly speaking, extra neural, or at least is not primarily involved in the manifestations of neurility. From this point of view the nervous system is a very simple affair. It is a point of view to keep always in mind, for in certain congenital diseases, inherited defects and degenerative, systemic maladies it offers the only clear explanation. It is difficult for us to keep this simple, uniform conception of the nervous apparatus clearly before the mind's eye, because we are accus- tomed to seemg its activities manifested in so many different ways. Mental, motor, sensory, trophic are the ways in which we usually re- mark the exhibitions of nerve force. A moment's thought, however, will bring home the fact that motion, sensation, mentality, nutrition are not nervous phenomena. Motion belongs to the muscles, sensation to particular end-organs, nutrition to special embryonic cells and men- tality to the interplay of various different processes. In other words, the great variety of outward presentations of nervous activity is due not to any variety in the structures and functions of the intrinsic nervous l8 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES elements themselves, but to the different organs and tissues of the body with which they are in intimate relationship and which they bind to- gether in perfect harmony of action. This also is a most important point of view from which to contem- plate the nervous system and one which emphasizes the peculiar differ- ence between it and the other organs of the body. In the localization of the lesion this point of view from which to regard the entire nervous apparatus and its functions is a very important one. From these standpoints it must be obvious to the casual reader even that a thorough knowledge of the histology and physiology both of the nervous elements themselves and of the nervous elements with their various connections and end-organs must be presupposed before any profitable advance can be made in the study of nervous diseases. This, of course, is largely true of all the organs and tissues of the body, but nowhere in the whole range of medicine, except in neurology, is a perfect knowledge of normal structure, normal relationships and normal functions so absolutely necessary for the accurate appreciation of ab- normal changes and abnormal manifestations. The oft-quoted aphorism that disease is nothing but perverted physiology comes more nearly to expressing the whole truth in neu- rology than in any other department of medicine. Much of the pessi- mism, lack of interest and ignorance in regard to the diseases of the nervous system spring out of unfamiliarity with the anatomy and physiology of the normal nervous system. In many other diseases of the body there are new secretions formed, new sounds produced, new appearances presented that enable one to form a diagnosis along with the aid of a good memory. For example, an ascitic fluid, a valvular bruit, a crepitant rale, a peculiar intestinal discharge may be so remote from all normal physiological exhibitions that its existence alone will quite determine the diagnosis. These un- physiological symptoms afford to the examiner a most ready and avail- able means of diagnosing disease. On the other hand, there is not a symptom belonging to disease of the nervous system that is not in its last analysis a simple perversion of a physiological manifestation. Fagge was therefore right when long ago he declared that the symptoms of nervous disease were almost without exception those of perverted nervous function. They reveal themselves always as an increase, a diminution or a modification of what would be a normal nervous exhibition. This is a most important fundamental fact to grasp in beginning the study of these diseases. It emphasizes the truth that a clear con- ception of the normal anatomy and physiology of the entire apparatus is an absolute prerequisite to the further comprehension of its pathology and symptomatology. Another peculiarity in regard to the nervous system is that most of its external manifestations are secondary in character and extraneu- ral. At no point except at the fundus of the eye do we at any time obtain a direct view of normal, functionating nerve tissue. Hidden behind the other organs and tissues, it reveals its own changes of activ- ity by the changes of activity in these organs and tissues. Motor, GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 10 vasomotor, sensory and trophic disturbances may all be directly due to nervous disturbances, but all that we can observe are the functional disturbances of muscles, blood vessels and special cutaneous end-organs and the nutritional changes in the related tissues. Occasionally we can observe changes in the cptic nerve at the fundus of the eye to corre- spond with changes in the vision, but more often even visual dis- turbances occur without any discoverable alterations in the fundus. Practically all objective nervous manifestations therefore are extraneu- ral in character. We can only infer corresponding changes in the nervous matter from the changes that we see in the non-nervous. Even mental and subjective symptoms are practically non-neural in their manifestation. The changes in the mental functions are recognized only by the indi- vidual himself as being related to motion and sensation. A change of volition, for instance, is to the subject himself a conceptual change of muscular movement ; an alteration of some, special or general sensa- tion is identified with the actual or conceptual alteration in the activity of some special end-organ such as the eye, ear or skin. Practically, therefore, all nervous manifestations, whether subjec- tive or objective, are secondary in character and involve extraneural tissues and organs. This is a startling and peculiar fact to remember always in con- sidering the semeiology of nervous diseases. So far as we know, irritability and self-nutrition are the only pri- mary physiological manifestations of unrelated nervous matter. When we speak of motor, sensory and trophic symptoms we refer merely to secondary, extraneural phenomena. Our analysis of the nervous activ- ity involves only inference and indirect reasoning. There is a third peculiarity in regard to the nervous apparatus that must be noted by one who wishes to understand its diseases. Though we often speak of it as a single apparatus, and though all its parts usu- ally functionate together in harmony, it is really a most complex and compound organ, made up of a great conglomeration of little organs or deposits of nervous matter, each having its own little sphere of activity and each exercising different functions according to the particu- lar end-organ with which it happens to be in connection. In the brain, in the spinal cord and in the nerves are clumps of elements which sub- serve motor, sensory and other functions, and yet lie in the closest sort of juxtaposition. Many observations in connection with the systemic diseases, with the progressive degenerations, and with the changes that sometimes take place in widely separated but functionally similar parts of the nervous system, lead us to suspect that the nervous elements are not all exactly alike in structure. \\ nether they differ chemically, physically or func- tionally we cannot guess. That they differ at all is only a matter of inference. So far as our present means of examination lead us, we can merely say that in structure the nervous system seems to be the same everywhere, though in function it is like a multicolored kaleidoscope. Xo other organ in the body is thus constructed. As a consequence the localization of disease in the nervous svstem is a characteristic and 20 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES diagnostic feature. The same lesion in different parts creates very dif- ferent clinical pictures, and the mystery of the so-called selective action of poisons and disease processes is in part dissolved. Viewed independently, then, the nervous organism is seen to fall into a category of its own. Under the impulse of a strong ancestral and biological influence it appears in the embryo. The continuation of this same prenatal or hereditary influence, coupled later on with an environmental and postnatal force, guides and directs its further de- velopments. In structure it is made up of innumerable elements whose chief powers seem to be functionally those of self-nourishment and the exhibition of a high degree of irritability. Such is the nervous system apart from all its structural and func- tional connections. It represents a mass of protoplasmic cells evolved from primitive embryonic cells and differentiated to a high degree for the performance of special purposes in the organism at large. It is always well to keep this conception of the nervous apparatus in mind, for it makes easy of comprehension some of the remarkable things that are observed in the nervous system in connection with heredity, de- generative influences, general infections and special diseases. As a matter of fact, however, the nervous apparatus is practically never an independent and unrelated organ. We have already seen how it is so intimately associated with the other organs and tissues of the body that its own changes of action can only betray themselves by cor- responding changes of action in these other organs and tissues. So close is the connection and so mutually dependent upon each other are the nervous elements and the rest of the organic tissues that the latter can even react upon the former almost as vigorously as the former can upon the latter. Nervous lesions can produce muscular atrophy ; but muscular disease often reacts in a way to cause wasting of the nervous elements. Optic atrophy causes the visual organ to undergo marked changes, while permanent shutting out of light from the eye results in a wasting of the optic nerve. This phrase mens sana in corpora sano is expressive of the truth that even mental and physical processes are mutually interdependent. If the nervous apparatus consists essentially, as I have previously indicated, of a mass of highly differentiated cells, it is subject not only to the tissues and organs with which these cells are functionally con- nected, but also to the tissues and organs which surround and support them. The latter, as we know, consist of neuroglia, connective tissue, lymphatics and blood vessels. These, together with the nervous ele- ments, make up what in gross anatomy is called the nervous system. Sometimes the nerve cells are spoken of as the noble and parenchy- matous elements, while the nutritive and sustentacula r structures that surround them are referred to as the ignoble or interstitial. A large number of diseases of the nervous system originate in these sustentacular tissues and secondarily damage the nervous apparatus in their way, just as diseases of the functionally related organs and tis- sues do in theirs. These diseases are literally as much extraneural as if they were located in the surrounding bones or membranes. The only primary diseases of the nervous apparatus are those that originate GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 21 and have their primary seat in the noble or true elements ; all others are secondary. The terms primary and secondary used to be employed in a grosser sense, the former being applied to all diseases that originated in the nervous system as distinguished from diseases that began in the bones, membranes and other coarse structures in the neighborhood. From the histopathological standpoint only the primary diseases of the nervous system are the parenchymatous diseases. The vascular and interstitial diseases are literally as much outside of the nervous apparatus as would be, for instance, disease of the spinal, vertebral or cerebral membranes. They affect the nervous apparatus secondarily and cause the appearance of nervous symptoms by their pressure or destructive influence upon the parenchymatous elements. Locomotor ataxia is a primary nervous disease ; cerebral syphilis of the gummatous type is not a nervous disease, but a disease of the blood vessels. The nervous changes in the latter are all secondary. For the proper appreciation of the intraneural diseases and the extraneural this conception of their primary and secondary charac- teristics should be insisted upon. Etiologically, pathologically, symp- tomatically and therapeutically there is the widest difference between the two sets of affections. The primary diseases are more subject to hereditary influences than are the secondary. They are degenerative in nature and are usually provoked by toxic, infective, cachectic, nutritional and other depressing causes of a general character. Their symptomatology is purely neu- rotic and as a rule is more or less uniform and progressive from the start. The localization symptoms are comparatively clear and well de- fined. Their prognosis is generally fair as to life, but bad as to recov- ery. Their treatment is most discouraging. They are both organic and functional, inherited and acquired. Inherited defect is at the bottom of a good many of them and accounts largely for the unsatisfactory character of their treatment. The secondary diseases are the result of traumata and various noxious agents. Heredity plays but an insignificant role here. The primary seat of these troubles is strictly extraneural, and hence the lesions represent all sorts of general and specific vascular and connec- tive tissue changes. Inflammation is at the foundation of most of these troubles and by pressure and otherwise the inflammatory process damages the parenchy- matous elements or the true nervous apparatus. The symptomatology of these diseases bears a double countenance — namely, the symptoms of the vascular disturbance and the symptoms of the nervous. The former are varied in character, are more or less febrile and are the same wherever the lesion may be located ; the latter are more uniform, reveal progressive irritation and destruction of the neighboring nerve elements, and are varied according to the location of the lesion. As these diseases are usually diffuse, the localization of the lesion is not a prominent feature and is possible only when the lesion happens to be of a certain character. The prognosis of these diseases depends upon the extent of the 22 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES damage done to the nervous elements and to some few particular in- herent features of their own. As a rule it is hopeful. Their treatment is generally effective and sometimes is rewarded with most brilliant success. They are practically all organic diseases, though the nervous elements for a time may be only functionally dis- turbed. The minimum of influential heredity amongst them accounts very largely for their more hopeful management. As illustrative of what is meant by primary nervous diseases may be mentioned tabes, Friedreich's ataxia, primary Little's disease, some types of progressive muscular atrophy, amyotrophic lateral sclerosis, progressive bulbar paralysis and most of the so-called neuroses. Among the secondary group I would place all of the inflammations, hemorrhages, neoplasms, and vascular, connective tissue changes, whether they occur in the tissues immediately surrounding the nervous elements or in the more remote membranes, bones, etc. Myelitis, en- cephalitis and neuritis occur here. All forms of vascular diseases, such as arteritis, hemorrhage, embolism, thrombosis, must be included. Tumors are all extraneural primarily, even including the unique glio- mata. Primary sclerotic processes are extraneural, being due to con- nective tissue changes. It hardly needs to be mentioned that diseases of the meninges and of the osseous coverings are pre-eminently extra- neural. These illustrations of what is meant by primary and secondary nervous diseases do not include all of the known affections by any means. The principle underlying the distinction having once been rec- ognized, it will be an easy matter when the diseases are studied in detail to assign them respectively to one or the other class as we learn their pathology. The Classification of the diseases of the. nervous system is still in a most chaotic condition. There is an obvious reason for this. Hith- erto all tlassifications have been based upon such varied and hetero- geneous factors as gross, arbitrary anatomical subdivisions, upon par- ticularly prominent symptoms, or upon strange etiological and thera- peutical distinctions. Some of these classifications were so absurd that they have long passed into the limbo of oblivion. Our knowledge of the nervous system is so incomplete that we still find justification for the calling of a special symptom a disease and giving it all the dignity of a special chapter. Though we no longer head a page in our modern text- books with such meaningless terms as paralysis and apoplexy, we still treat chorea and hydrocephalus and hemicrania and headache and vertigo as though they were diseases. By the later elimination of the names Landry's Paralysis, Meniere's Disease and others and the proper assignment of these diseases to the places which their pathology indi- cates, it is seen that we are steadily though slowly progressing towards a more accurate neurological nosology. Some day symptoms will not be employed at all as the basis of classification. It was long ago recognized that the pathology of a disease should constitute the only guide for its name and classification. Pathology, however, involves both the location and the character of the lesion. Many authors adopt both factors in their classification. In one pah of GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 23 the book will be found the discussion of the system diseases. Here the location of the lesion is the prominent feature. In another part of the book there will be found a discussion of tumors of the nervous system and syphilis. Here obviously the character of the lesion is the principal consideration. It must be frankly admitted that in the present state of our knowledge such incongruity is not altogether blameworthy. In fact, it has its advantages for the present. Some clay, however, this also will be avoidable. A very gross, yet convenient, classification, based upon location, is that which divides the diseases into those of the brain, spinal cord and peripheral nerves. Modern histology, and especially its recent magnifi- cent generalization, the Neurone Doctrine, has demonstrated that it is coarse and arbitrary to divide the nervous system into brain, spinal cord and nerves. It has shown us that there are no lines of separation between these so-called parts of the nervous system. Processes from the brain cells pass far into the cord from above, while processes from the nerves extend high up into it from below. From the cord cells pass, in opposite directions, processes into the brain above and into the nerves below. In other words, most of the nervous elements lie partly in one and partly in another of these so-called divisions of the nervous system. Our ignorance of this fact has hitherto caused some most erroneous conceptions and classifications of the nervous affections and the incubus of it we even yet find hard to shake off. We still sometimes refer to locomotor ataxia as a spinal cord disease, though its lesion is chiefly found in nervous elements that extend farther outside of than inside of the spinal canal. There are certain bulbar diseases that present a symp- tomatology more nearly related to spinal cord than to brain phenomena. It is a question in the minds of some pathologists whether certain forms of so-called peripheral neuritis are not really degenerative processes in the peripheral nerves due to trophic disturbances in the anterior horns of the cord. Certain eye symptoms have long been recognized as attrib- utable to disease in the lower part of the cervical cord. So far as the tracts and cellular processes are concerned in diseases of the nervous system, there are no demarkation lines between the brain, cord and peripheral nerves. As we will see when we come to discuss the neurone and neurone theory, the diseases that attack the true nervous elements primarily, the true nervous diseases, extend from brain into cord and into nerves and vice versa. They do not confine themselves to any one of these arbitrary divisions of the anatomists, but follow the course of the nervous elements. They may be classified, therefore, as Neuronic Diseases. They are the most truly nervous of all the nervous diseases. They are organic and functional, the former be- ing quite sharply localized, the latter being more diffuse and including to a large extent the so-called Neuroses and Psychoneuroses. The Neuronic Diseases and the Neuroses, therefore, will be treated of in sections by themselves and no attempt will be made to classify them as brain, cord or peripheral nerve affections. An examination of the gray matter of the nervous system reveals the fact that it is made up of a series of aggregations of cells extending all along the cerebro-spinal canal. These ganglia, or little brains, as it 24 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES were, are to a certain extent independent in their individual activities and control without much interference various related organs and parts of the body. These ganglia are numerous and are found everywhere, though regularly situated in all parts of the nervous system. They are in the sympathetic system, in the posterior spinal roots, and in many of the cranial nerves. They constitute the central gray matter of the cord as explained in the chapter on die minute anatomy of the cord. They are found in the nuclei, basal ganglia and cortex of the brain. Since their functions differ on account of their connection with differ- ent end-organs and different parts of the body, when they are anywhere attacked by disease, the location of the lesion can be at once determined by the peculiar symptoms presented. When the intraspinal ganglia, for instance, are affected, the symptoms are quite different from what they are when any of the intracranial ganglia are implicated. There- fore, for obvious reasons, we still find it convenient to speak of dis- eases of the brain, of the cord, of the nerves and of the sympathetic system. In each of these divisions there are many distinct ganglionic masses with sharply defined functions. This enables us to carry the classifica- tion of diseases on the basis of location to a still higher point. \\ e subdivide those of the brain, for instance, into those of the cerebrum, the cerebellum, the pons and the medulla. We might carry the division even still farther, but as a matter of fact do not find it specially con- venient to do so. We might even speak of the diseases of the motor cortex, of the visual cortex, of the sensory cortex, of the cervical cord, of the lumbar cord, etc. All this, however, can better be left to the con- sideration of the cerebral and spinal localizations in the chapters devoted to the anatomy of the brain and cord. In the following work I will adopt this provisional classification of the diseases of the nervous system, believing it to be justified by the present state of our knowledge, and realizing perfectly that newer dis- coveries will from time to time transfer some of the diseases from one group into another. Neuronic Diseases. (Parenchymatous Degenerative Troubles.) a. The Functional Neuronic Diseases. i. Cerebro-spinal. 2. Sympathetic. b. The Organic Neuronic Diseases. i. Of the Afferent System. 2. Of the Efferent System. 3. Of Both the Afferent and Efferent Systems. Non-Neuronic Diseases. (Interstitial and Vascular troubles affecting the Neurones secondarily.) a. Of the Spinal Cord and its Membranes. b. Of the Peripheral Nerves. c. Of the Brain and its Membranes. General Intoxications with Special Nervous Symptoms* GENERAL CONSIDERATIONS UPON NERVOUS DISEASES GENERAL CONSIDERATIONS UPON THE ETIOLOGY OF DISEASES OF THE NERVOUS SYSTEM. It is premature to formulate an exact etiology or classification of the causes of diseases of the nervous system. They are so many, so varied and so indistinct sometimes that we are quite at a loss to give to them always their deserved valuation. For many reasons, however, an attempt should be made to gener- alize, so far as known data will warrant, the etiology of these affections. In the first place, the causation lying behind the pathology serves as the guide to the proper therapy. Our management of these dis- eases, even more so than in others, cannot proceed along logical and scientific lines as to the best interests of the patient until we have a clear conception of their etiology and pathology. It seems like a truism to say this and yet the pre-eminence given to therapeutics, to the neglect of etiology and pathology, is the folly of the quack, the ignor- ance of the layman and the weakness of the physician. In the second place, we are so rapidly eliminating the innumer- able causative factors harped upon by the writers of the past, and discovering so much more positively the comparatively few real causes, that the time is getting ripe for an attempt at a systematic arrange- ment of the latter. In the third place, our knowledge of the pathology of nervous diseases has lately grown so and our conception of its relationship to all etiological antecedents has clarified to such a degree, that we are beginning to occupy a position to speak with a high tone of positive- ness of the latter as being the undeniable causes. In the fourth place, a study of the etiology of these affections points out a line for the classification of the diseases themselves, enables us to calculate the possibility of the removal of the cause and of the disease, and helps to determine the prognosis as well as the hopeful- ness or hopelessness of treatment. As all diseases of the nervous system naturally fall into one or the other of the two groups — namely, those that start primarily in the neurones and those that start primarily outside of the neurones, we must remember this in studying their etiology, for the latter is not exactly the same in all particulars for both. This will be noticed as we proceed in the discussion of the causes and especially when the particular diseases themselves are taken up for consideration. Another useful, because practical, division of the causation of nervous diseases is into those that are endogenous or developed within the body, and those that are exogenous or exert their influence from without. An anaemia is an endogenous cause of nervous degeneracy ; a blow on the head is an exogenous cause of meningitis. It would be perhaps too fine a distinction to speak of the endogen- ous and exogenous causes in relation to the neurones or true nervous elements; and yet such a division would be both scientific and prac- tical, for all of the hereditary diseases are practically due to endogenous 26 GENERAL CONSIDERATIONS UPON XLPVOUS DISEASES influences within the neurones, while all other diseases, whether merely congenital or prenatal, natal or postnatal, are exogenous. A grand starting point for the study of the etiology of nervous diseases is the division of the causes into the predisposing and the exciting. The former are inherent, of course, and include everything that involves the patient's individual body and personality. They there- fore take cognizance of his heredity, his intrauterine development, his birth and his constitutional state and his subjection to other diseases up to the moment of examination. They are concerned with his age, sex, nationality, occupation, mode of life and habits, his climatic, hygienic and social environment. The exciting causes are not neces- sarily inherent in the patient. As a matter of fact, they are more fre- quently external, all more or less temporary, and invade an organism rather abruptly. They include the traumata, the intoxications and the infections. Among the reflex causes so much referred to by many writers, some are of the nature of mere predisposing factors, some fall into the category of direct traumata. I will discuss them later on. For convenience we may formulate the following scheme /)f the causes of the diseases of the nervous system : 1. Predisposing causes. Heredity. Age. Sex. Nationality. Occupation. Mode of Life and Habits. Climatic, Hygienic and Social Environment. 2. Exciting causes. Traumata. Intoxications. Infections. 3. Reflex causes. I will now discuss these a little more in detail and explain the omission in the schema grossly outlined above, some causes, as, for instance, tumors, parasites, etc. As has been long recognized, heredity plays its most brilliant role in connection with the nervous tissues. So recently has the nervous system, at least in its highest and most complex elaboration, been evolved in the course of biological development that we can trace an- cestral phenomena, both anatomical and functional, better in it than in any other organ or tissue of the body. Zoologists recognize this so forcibly that they arc beginning to use the nervous apparatus for the basis of their higher classifications and to trace out with its aid the lines of descent in the various classes of animals. In neurology we have come to acknowledge that heredity is the most important factor biologically for modifying the nervous apparatus for good or for evil. Note carefully here the use of terms. I snid the nervous apparatus. Strictly speaking, this includes, we now know, only the neurones and the neuroglia. Tt follows logically and it is clearly demonstrated em- GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 2J pirically, that in the neuronic and neurogliomatous diseases only do we find heredity exerting the most transcendent force. Many of the dis- eases of this class are purely and entirely hereditary. They are of the nature of inherited defects. Such, for example, are Friedreich's disease, some of the progressive muscular atrophies, the so-called con- genital neurasthenias, hysterias, epilepsies. There are other diseases of the neuronic class, however, that are provoked into existence by one or more exciting causes, but are local- ized and otherwise determined by the patient's heredity. In these there is a tendency toward the disease, but it remains latent until some ex- traneous cause starts the latent influence into action. It is believed that this explanation applies to many of the acquired systemic diseases like locomotor ataxia, to most of the gliomatous conditions and to a large number of the acquired and secondary neurasthenias, hysterias, -epilepsies and other so-called functional neuroses. It is going too far, however, to affirm, as some have done, that a true neuronic disease cannot develop, whatever the exciting cause may be, unless there is an inherited neuropathic weakness of some sort present. It is not at all improbable that monotonous overexertion, intoxication or infection may expose itself in a disease of neurones that may originally have been endowed with perfect health. These cases, however, are not as com- mon as is generally supposed. It is astonishing the amount of strain the perfectly healthy and well-developed nervous apparatus will bear. Most of the breakdowns that we observe in actual life, whether of the organic or functional type of neuronic disease, owe a large part, of their misfortune to the inheritance of an inadequate strength and vitality. In the diseases that start outside of the neurones heredity plays such a small part that it amounts to practically nothing. In certain vascular troubles, such as the cerebral hemorrhages and possibly in some of the tumors, there is a bare trace of hereditary influence. In the traumatic, toxic and infectious troubles that cause inflammation and all its sequelae, it is practically nil. The diseases that occur as the result of hereditary syphilis are not hereditary nervous diseases. Hence what I will say in regard to heredity will apply more particu- larly to the neuronic degenerations and neuroses. By direct heredity is meant the transmission of the same disease from parent to child. Such heredity is not common. More frequently a nervous weakness or tendency is handed down. This is known as indirect heredity. When the hereditary influence is towards the pro- duction of the same disease in the child that was in the parent, it is spoken of as an inherited tendency. Even this is not so common. Much more frequently the progeny is presented with a neuropathic taint merely, which, upon provocation or without, may develop a simi- lar or dissimilar disease from that observed in the ancestry. A neu- ropathic diathesis is so frequently traceable in these cases that no ex- amination is properly conducted in which it is not promptly and most persistently inquired into. It is not always necessary that there should be a well-defined dis- ease in the parentage. Nervous strain may be sufficient to cause the 28 GENERAL CONSIDERATIONS UPON XERVOUS DISEASES children to inherit a constitution that soon revels in the most elaborate forms of nervous disease. I have seen a girl go insane with a primary form of degenerative dementia for which no other possible cause could be detected than a congenital neuropathic weakness. When she was conceived and being carried her mother was under excessive worry and a physical and mental strain striving to make a living for a large and healthfully growing family. Hard brain workers, those who dis- sipate and indulge freely in alcohol and tobacco, often transmit to their children a vitiated nervous apparatus. The latter surfer from neu- rasthenia, hysteria, epilepsy and various psychoses. If both parents are neurotic or subject themselves to causes that deteriorate the nervous system, the chances for the children are bad. The intermarriage of blood relatives is dangerous for the offspring. I have under observa- tion now a middle-aged man, the father of two vigorous, grown-up daughters, who has been the victim during the last eight years of typical disseminated sclerosis. His habits have always been exemplary and there is no known exciting cause for the disease. His paternal grandparents, however, were first cousins. His own father and a paternal uncle and aunt were all congenital deaf mutes. His own mother became a deaf mute from scarlet fever in childhood. The pa- tient himself ts the younger and weaker of twins. Mental troubles not infrequently follow close blood intermarriages^ Dean concludes from his examination of 181 cases of eye troubles in an institution for the blind that of the congenital cases fourteen per cent were the result of consanguineous marriages of the first degree. He- believes from his statistics that the greater the inheritability of a con- dition the more liable it is to be the result of consanguineous marriage. Sometimes the parent will be afflicted with a typical disease while the child will reveal only a general neuropathic depreciation. A mother is epileptic, her daughter becomes hysterical. It may be vice versa A father has all his life had attacks of typical hemicrania ; his child has epileptic fits or becomes the victim of dementia prsecox. Chorea breaks out in the children of markedly neurasthenic parents. The neuroses are more frequently transmitted by the mother than by the father. The heredity has been seen to be direct, if I may say so, in alter- nate generations, indirect in succeeding generations. Epilepsy, for instance, has been among the grandparents ; genius with some of its oddities and psychic peculiarities appeared in the next generation ; epilepsy again, with terminal insanity, reappear in the third genera- tion. This is sometimes called a manifestation of atavism, a reversal to earlier forms of nervous exhibition. Atavism is a remarkable and not such an uncommon phenomenon in the animal world. It is not frequent, however, in connection with nervous diseases. Indeed, it may seriously be questioned whether the mere repetition of the same disease back of the preceding generation is a true form of atavism. A disease in the ancestor is not necessarily an earlier or more primitive type of that seen in the progeny. It is really the same disease probably occurring in the same sort of a neu- ropathic constitution. As a phenomenon, however, this atavistic ten- dency in nervous disease, whether real or apparent, is interesting and GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 29 ^worthy of further study. The same may be said of those diseases that sometimes appear in collateral branches or in several members of the same family, so that they are spoken of as "familial diseases." These are largely of the nature of developmental defects or anomalies. Fried- reich's disease and some of the atrophies may be cited as illustrations. Glioma is a congenital defect. Diseases that reappear, and the morbid conditions that occur, later in life in the descendants than they did in the ancestry, may be looked upon as waning in the family. Under the head of heredity the modern doctrines of degeneracy must receive some consideration. The term is one that is much abused and misunderstood. Degeneracy is the result of a biological taint. It is a product of hereditary and congenital influences. It is not the immediate result of environment. It therefore does not mean a falling back of the individ- ual from a previously normal state, but a falling back of the racial or familial development, as shown in the anomalous and primitive stigmata of the individual. A degenerate is what he is because of his ancestry, and not because of anything he himself has done. It is therefore a misfortune, not a disgrace. In the physical sphere it shows itself by various stigmata, the value of which have not all been positively estab- lished. No man is perfect physically and yet we do not consider all men as degenerates. Just where degeneracy stops and so-called nor- mality begins is a hazy region ;- hence the many extreme and ofttimes absurd notions put forth by those who believe and those who reject the doctrines of Lombroso. Asymmetry of the skull, protrusion of the lower teeth, irregularities about the eyes, anomalies in connection with the ears, disproportion between different parts of the face are all often signs of physical imperfection. Still greater degeneracy is seen in such conditions as hare-lip, cleft palate, irregular teeth, retinitis pig- mentosa, albinism perhaps. And yet too much must not be inferred even from such coarse defects as these. Most epileptics reveal more or less stigmata of degeneracy. Cranks and queer people very often pos- sess them. Genius has been accompanied by them. On the street scarcely an individual is seen who does not exhibit some similar phys- ical anomaly in greater or less degree. -» There are parallel stigmata in the neurotic and psychic manifesta- tions. They may range all the way from a slight eccentricity of thought and manner down to gross mental debility, imbecility and idiocy. As no man is perfect physically, so no man is perfect mentally. This is all too obvious when a definition of what is meant by nor- mal mentality is attempted in court during a trial for insanity. So many factors enter into the make-up of one's mental manifestations that what is craziness in one man is perfect mentality in another. The points of view vary. Hottentots are not insane, nor even degenerate, because they do not think and act as Englishmen do. A genius, an artist, may well appear to be erratic, even crazy, beside a dull financier with his one idea of money-getting. Their opinions of each other in regard to their mental exhibitions will probably be mutually uncom- plimentary. We may, if we please, call the average man normal. Only in 30 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES comparatively coarse defects, however, can the positively abnormal be recognized. Genius is eccentric, even crazy at times ; so seems the dull monotony occasionally of the average man. It behooves the true scientist to be conservative in his conceptions- of mental and nervous degeneracy, just as he must be conservative in deciding who are afflicted with the stigmata of physical degeneracy. Along coarse lines we can recognize both psychic and physical de- generacy. We can even note a remarkable parallelism running be- tween them. A wholesale slashing about of the doctrine as some en- thusiasts have made, attributing degeneracy to all geniuses and bril- liant intellects, and to all deviations from their preconceived notions of physical and mental perfection, will lead, in the present state of our knowledge, like a boomerang, back to their own imperfections and de- generate reasonings. The age of the individual exercises a considerable influence in determining the nature of the disease. This is partly on account of the differences in the patient's own organism and in his habits and en- vironment in different years. It may even be entirely secondary so far as the nervous system itself is concerned, for the primary disease may entirely determine the age at which the nervous troubles appear. The nervous sequelae of scarlet fever, for instance, occur in childhood merely because scarlet fever is a childhood disease. In childhood and early life generally the congenital troubles that result from prenatal and natal injuries usually declare themselves. Hereditary troubles also are apt to appear early. The infectious fevers that occur in children are responsible for a large number of nervous diseases. Early malnutrition exerts its influence, as well as unwonted strains. The child's nervous apparatus is, of course, a growing and immature one, a fact which exercises some force in the determination of the kind of disease, as well as of its symptomatology and prognosis. In middle and later childhood we observe a predominance of motor troubles, such as chorea, paralysis and convulsive seizures. The ex- cessive use of the motor apparatus in learning to talk, to walk and to adjust the young creature to his environment is possibly the cause of this. About the time of puberty the sensory and emotional disorders are in the ascendent. Under the awakening of the sexual life and all that it means both in the individual's inner sensibilities and in his relation- ship to his surroundings, hysteria, neurasthenia, epilepsy, sleep troubles, hypochondriasis and inherited tendencies, nervous and mental, are apt to break out. From puberty on to the climacteric the nervous system is more « r less stationary and developed, but it is subjected to all of the strains of an active adult life. Traumatisms of all sorts now come into play. So do the intoxications and infectious diseases of adults. After the climacteric the troubles of old age, the degenerations of all the tissues, nervous and vascular, are to be counted upon. Hence the senile weakness, the apoplexies, the softenings, the arteriosclerotic and atheromatous degenerations, vertigos, neuralgias and paralyses are common. GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 31 The influence of sex is a knotty question. Women are more emo- tional than men and are undoubtedly subject to different physiological influences. But they also lead very different lives from men. The neuroses and sensory disorders predominate among them, whereas the organic and motor affections lead among men. Just how much of this is to be attributed to their difference of organization, and how much to their difference of mode of living, it is not easy to say. More study is needed before the influence of sex upon the development of nervous disorders can be dogmatically spoken upon, Nationality seems to have some bearing. Whether this is a racial, a biological or a mere geographical factor is not entirely known. The neuroses, for instance, are more prominent among the Jews and the Latin races of Europe. They are old races and have been historically subjected to most unhappy conditions. A degree of degeneracy there- fore may account in part at least for their proneness to the functional troubles. The organic diseases are more common in the Germanic and Anglo-Saxon peoples. Locomotor ataxia is a rarity in the Negro and the Jap. The insanities of childhood are almost considered American diseases, but they are probably due to the strenuous lives we live here. Certain immigrant races, such as the Bohemians and Poles, probably owe their susceptibility to epilepsy and other neuroses to their de- pressed conditions in life as much as to their nationality. France is the home of hysteria ; as a people the French live a comparatively sensuous, unrestrained and highly emotional existence. All occupations that are in unhygienic surroundings, that are monotonously prolonged, that necessitate irregularity of habits, that interfere with sufficient exercise, fresh air and relaxation, that, in a word, destroy. the nice balance between all the functions of the body necessary for its health, predispose towards the development of nervous troubles. Much neurasthenia is attributed to hard work, especially mental work, in these strenuous days, whereas it is not the work that is to be blamed, but the monotony of it and the one-sided development which it enforces. Hysteria is often provoked by the narrow mental lives that many women lead, as well as by their want of self-discipline. Occupations of some kinds are particularly prone to lead to nervous troubles on account of the exposure to accidents, to intoxica- tion and to infection. Bridge builders are liable to the caisson disease ; saloonkeepers suffer from alcoholic degenerations ; lead workers some- times develop lead palsy ; and traveling men get syphilis and develop tertiary consequences. It is said that the unmarried suffer more from nervous diseases than the married. The freer life and greater dissipation of the former may be the proper explanation for this. The whole subject of occupation, environment and the special dangers therein is a large one and can only be referred to briefly here. The influence of the mode of life that an individual lives has already been hinted at in the consideration of the age, sex, occupation and nationality. It is very great. It involves the mental as well as the physical life. Overindulgence in eating, in the use of spirituous beverages, tea, coffee, tobacco, is a prolific source of nervous trouble. $2 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES Bad sleeping arrangements, bad bathing customs, bad methods in everything will surely lead to misfortune. It is astounding how some of the simplest intimations of nature are misinterpreted and unheeded by those who ought to know better. A physician, in a high degree an irritable neurasthenic, came to me and declared that he did not seem to get well, but worse, though he stimulated himself up every day with a cold plunge bath in the morning, full doses of strychnia and long, exhausting walks. I told him I was not surprised : Excessive sexual indulgence is undoubtedly a source of much nervous breakdown in both married and unmarried. Athletics and the indulgence in outdoor sports at the present time are so excessive that they become a source of danger by putting unusual strain upon the vascular system. On the mental side, sensational literature, narrow thinking and the habit of flaring up in anger at every trifle are highly conducive to the development of the neuroses, as well as being often a symptom of them. Why is it so frequently forgotten that the mind is as much in need of proper exercise as the muscles are? The mind is a product of brain activity and the brain grows or wastes just as any other tissue of the body if it is exercised or neglected. We do not know what parts of the brain subserve particular faculties of the mind, but we do know that as a sort of counterpart to the biceps and triceps and lumbar mus- cles of the body there is a memory and an imagination, a logical and a speech faculty. How pitifully lop-sided is that man who toils daily after business hours in the gymnasium with his dumbbells and Indian clubs in order to develop the muscular system, but who never once thinks of exercising specifically his imagination or his memory. The dull round of his routine life does not afford the opportunity for very wide mental development. I am convinced that one-sidedness in mental development as well as one-sidedness in physical is responsible for some of the neurasthenia and hysteria which we observe about us. A volume could be written upon the influence of habits and the mode of life in the fostering of nervous affections. Climate, apart from mere racial and geographical influences, is not an important factor in the etiology of nervous diseases. The fact that these are more common in the temperate zones is because there the strenuous life and extreme competition are found. This suggests the truth that civilization is responsible for much nervous and mental trou- ble. Neurologists and alienists would find their services at a discount among barbarous races. In the centers where civilization is burning at white heat, as it were, namely, the great cities, there the nervous trou- bles are in abundance. In fact they are on the increase. The two great- est causes of these affections were included in that happy epigram of Krafft-Ebing, "syphilization and civilization." We must be careful, however, not to charge civilization with what is to be blamed upon squal- or and vile conditions in some of our cities. To argue as Rousseau did that we need to return to barbarism is a bad mixing up of cause and effect, of separate and distinct forces, that is wholly inexcusable. Civi- lization in its true sense means culture, refinement, comfort and mental GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 33 elevation. Because ignorance, stupidity, greed and poverty happen to loom up where civilization is existent, it merely shows by the contrast that much good has been accomplished though much is yet to be ac- complished. Not civilization but the abuse of the opportunities offered by civilization is the cause of the apparent increase of nervous troubles. Among the predisposing causes of nervous affections, certain diatheses must not be overlooked, especially the gouty and rheumatic. What passes under the general name of the lithaemic condition is read- ily recognized clinically, though the nature of the defective process is not fully understood. Faulty metabolism with faulty elimination is perhaps the real difficulty. How this reacts upon the nervous system to produce functional and peripheral diseases is not clear but that it does do so is amply demonstrated both clinically and therapeutically. I come now to the exciting causes of nervous diseases, and first and foremost I place in the list the traumata. These include all kinds of physical and mental insults. A terrific blow on the head may pro- duce less serious effects than a terrible fright. Bodily injuries of all sorts, especially about the head, may set up profound nervous and mental deterioration. Primarily or secondarily the delicate neurones may be disturbed. From a gross fracture of the skull, all the way to a mere concussion, may the traumata range. The former lacerates, compresses, invades with hemorrhage, inflammation and infection the cerebral tissues; the latter jars and disturbs the functions of the neurones in such a way as to give rise to neurosis. Exposure to cold and damp is of the nature of a trauma. So are sudden excitement, unnatural coitus and many other causes given in the books. Almost any form of infection may give rise to disease of the nervous organism. Usually it is of the inflammatory type of disease though in some chronic infections it may be non-infiammatory and purely a neuronic degeneration. It is generally believed that not the germs themselves but their toxic products are the immediate causes of the disease process. Syphilis, both acute and chronic, acquired and inher- ited, is highly blameable. Scarlet fever, measles, influenza, pertussis, erysipelas, gonorrhoea, septicaemia, pyaemia, etc., all enter into the etiol- ogy of nervous affections. Tuberculosis, near and remote abscesses and other germ troubles may provoke metastatic lesions in the nerve cen- ters. Some tumors and parasitic growths should probably be classed here, though in a way they act more as traumatic causes by compress- ing and mechanically irritating the nervous elements near which they are located. Until we know more about the pathogenesis of these neo- plasms we can only say that infection and traumatism are the imme- diate causes of the nervous disturbances when they are present. Of the intoxications that provoke nervous maladies, the alcoholic far outranks all the others. Lead, arsenic, mercury, aniline, copper, carbon dioxide, tea, coffee and tobacco are a few of the intoxicants. Here should be mentioned also the drugs that are sometimes used as a habit, morphine, cocaine, chloral. Autointoxication together with malnutrition is probably the explanation of the etiological bases of the various cachexias, of anaemia, of diabetes mellitus, of nephritis and certain alimentary troubles. 34 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES Finally, a word must be said upon the much-discussed reflex causes of nervous diseases. To deny all etiological influence of a re- flex character is to deny the reflex phenomenon of the normal nervous apparatus. To attribute disease in the otherwise unaffected nervous centers to a mere reflex activity, which is a normal function of a nor- mal nervous system, is illogical on the face of it. Therefore, we as- sume that a constant reflex excitement of the nervous apparatus pro- duces the manifestation of disease symptoms in only one of two condi- tions ; either as an excitant to an already abnormal and diseased nervous organism or as an excitant to an exhausted and wearied one. In the first case the reflex is not the primary cause of the dis- ease but is clearly a mere irritant to it. In the second case, it may be seriously questioned whether a mere state of exhaustion ever becomes transformed into a specific type of disease. Let me illustrate. There are ophthalmologists to-day who attribute epilepsy to eye-strain. Where there is epilepsy, eye-strain as well as a painful corn on the toe, a phimosis or an impacted bowel may provoke the attacks. If there is a tendency to epilepsy even, eye- strain may start the disease into activity. In all cases of epilepsy therefore the eyes should be corrected if need be, but don't charge the eyes with the central nervous trouble. When it is contended that eye- strain produces epilepsy de novo in a previously healthy nervous appa- ratus, it is incumbent upon the ophthalmologist to explain how it does it. He declares that it does it by exhausting the nervous centers. He has been betrayed clearly in his pathology by the use of the unfortu- nate word functional. Epilepsy, hysteria and neurasthenia we may grant for argument's sake are all alike in being functional but their very symptomatology proves that they are not alike in their origin and physical basis wherever and whatever that may be. The eye-strain of the ophthalmologists' pathology acts always in the one way, namely, as an exhausting strain, and yet strange to say it produces a variety of distinct specific diseases of a different character in different indi- viduals. Knowledge of the pathogenesis of these various diseases is not as clear as it should be but it certainly is clear enough to reveal the illog- ical reasoning of those who attribute a series of distinct, sharply-de- fined, different affections to a mere peripheral wearying reflex that is always the same in character and points to no direct connection between these various diseases or between them and itself. The causation of different distinct pathological conditions in a previously normal system by the mere constant exercise of a single and uniform reflex irritation is a step backward and brings us face to face again with a pathology that is hazy and antiquated and from which we had fondly thought mod- ern neurology was redeemed. In my opinion, nervous diseases are not reflex diseases though they may be aroused into expression, or excited into greater activity when so aroused, by a dozen or more sources of peripheral reflex stim- ulation. GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 35 GENERAL CONSIDERATIONS UPON THE PATHOLOGY OF NERVOUS DISEASES. In order to have a clear comprehension of the pathology of the diseases of the nervous system, one must keep well in mind the com- plex character of the nervous tissues and their relationship to all the other tissues. Many diseases are secondary to extraneous affections that cause pressure or by extension and metastases invade the nervous system. Therefore in these diseases frequently there are pathological findings of great importance in other parts of the body. For instance, intracranial embolism is usually associated with valvular heart dis- ease ; tuberculous meningitis is accompanied by tuberculous foci in the lungs; abscess of the brain is very largely due to suppurative otitis media; damage to the spinal cord frequently results from caries of the spine; disease of the muscles and various end-organs in direct functional connection with the neurones not infrequently sets up de- generative changes in the latter. It is not our province to refer to all of these extraneural troubles but merely to the pathological changes which they and other causes set up within the nervous apparatus. It will be remembered that nervous tissue consists of two dis- tinct sets of elements, developed from different embryonic layers in the ovum. The true nervous elements are the neurones and the neuroglia cells. Though these differ in function, the former being the excitable elements while the latter are merely sustentacular or supporting, both are found only in the nervous system, and developed from the epiblast are non-vascular. The false nervous elements are the connective tissue cells, the blood vessels, the lymphatics and the epithelium lining the ventricles. The function of these is to nourish and furnish a matrix for the true nervous elements. They are found in other parts of the body as well as here, are mesoblastic in origin and are vascular. Pathologically then the nervous system is liable to two great types of change. The non-vascular elements undergo degeneration, the vas- cular elements undergo inflammation. Degeneration is the only change that ever occurs in the neurones. The neuroglia cells may proliferate, form tumors and then undergo degeneration. Inflammation with all its attendant conditions, hemorrhage, vas- cular obstruction, infective processes, connective-tissue and parasitic new-growths, and sclerotic changes may occur in the vascular con- nective-tissue of the nervous system just as they may occur in the vascular connective-tissue of other organs and parts of the body. Un- der such circumstances the true or nobler nervous elements are damaged secondarily. It is doubtful whether a simple, primary degeneration in the true nervous elements ever sets up inflammation secondarily in the vascu- lar structures. I know that in locomotor ataxia, a true degenerative disease, and in some degenerative forms of polyneuritis inflammation 2,6 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES of the meninges and of the interstitial tissues is often respectively ob- served but it is a question whether this inflammation is not due pri- marily to the same general infection or intoxication that gives rise to the degeneration. Degeneration as it is seen in the nervous system is a unique pro- cess. It gives a very characteristic appearance to the affected tissues, and its presence and progress render the symptomatology of nervous lesions clinically almost pathognomonic. Hence its appreciation is most important. As the distinctive lesion of the nervous apparatus, it is more important than the grosser lesions of vascular origin. When a nervous element undergoes degeneration it dies, wastes and dis- appears. The cell-body swells up, then becomes granular and fatty, and finally is absorbed. The process is sometimes called chromatolysis. In the early stage and temporarily, if the degeneration is not severe or complete, there may be a shriveling up of the cell-body; its edges then become serrated, its nucleolus disappears, its nucleus shifts to one side (decentralization), the chromophyllic particles diminish in amount and closely hug the nucleus while open spaces appear in the substance of the cell-body (vacuolation). Such cells sometimes regenerate, pass- ing through a reverse order of events. In acute and complete degeneration the neuraxones participate in the cellular destruction. The white substance of Schwann breaks up into fatty globules, the nuclei of the neurilemma proliferate, and the entire process becomes a hollow tube containing the axis-cylinder and degenerated white substance. At length the axis-cylinder breaks down ; absorption takes place, and nothing is left. Into the hollow space vacated by the process, or neuraxone, the neuroglia and connective- elements crowd and thus render the tissue firmer and denser, more vascular and less puctuated from nerve fibres on cross-section (scler- osis) than it was before. Degeneration may be an acute or chronic process, severe or mild, partial or complete. Most important is the fact that it may be primary or secondary. Under certain circumstances it may involve the entire neurone or only a part of it. It may spread directly from one neurone to the next one in the same nervous pathway, provided the second neu- rone is functionally subordinate to the first. This explains the morbid anatomy of the progressive system diseases. It probably never passes directly from one neurone laterally across to another lying beside it. The simultaneous degeneration of the two neurones under such circum- stances is in all probability due to the simultaneous action of the same cause. The origin of these degenerations lets a flood of light in upon the etiology of nervous diseases. The primary degenerations may be due to hereditary, congenital, toxsemic, nutritional and traumatic influ- ences. When the vitality of the neurones, transmitted from the parent to the child, is so vitiated that they cannot develop properly, function- ate normally, or resist the deleterious influences of life, they are neuro- pathic and break down into the condition of disease. In this way the developmental and familial diseases are eyolved. Friedreich's ataxia and the progressive atrophies furnish capital illustrations. In all prob- GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 0/ ability, it is only degree that distinguishes the neuroses such as epilepsy, migraine, congenital neurasthenia and hysteria from the cited ex- amples. Congenital primary degenerations are undoubtedly due sometimes to shock and malnutrition of the mother and then of the child while the latter is in the uterus. One must not confuse with these cases the infantile hemiplegias in whom secondary degenerations occur as the result of hemorrhage, inflammation and other factors. The con- genital primary degenerations are non-hereditary for they are intra- uterine in origin. Most of them, like most of the natal, postnatal and adult primary degenerations, are due to general or local traumatic, nu- tritiona.1 and toxsemic influences. Profound shocks, blows and concussions, without causing any discoverable lesion, are not unfrequently seen to provoke degenerative troubles, particularly of the mild type of the neuroses. Some of these even go on to severe degenerative alterations pronounced enough to be manifested in permanent paralysis. Malnutrition as a cause of degenerative trouble needs but to be mentioned. Local malnutrition occurs from vascular obstruction by emboli, thrombi and arteritis obliterans. Many senile forms of nerv- ous degeneration are due to inefficient nutrition and metabolism. The supreme cause of the primary degenerations in adult life are the intoxications and infections. I might have said only the intox- ications, for in the infections it is the poisonous products of the germs and not the germs themselves that provoke the degenerative changes. Under the head of etiology in the previous chapter I have referred to the various kinds of intoxication. Just how these intoxications act upon the nervous elements is not clear. Some probably damage them directly ; others doubtless affect them indirectly by lowering the nutritive value of the blood. Much has yet to be learned upon this question. Perhaps the greater number of nervous degenerations are sec- ondary in origin. The function of self-nutrition for the entire nervous element seems to reside in the cell body. Therefore if the cell body is damaged, all parts of the neurone undergo a parallel wasting. If a part of the neurone, say a part of the neuraxone, is severed from its cell-body, all that part that has been cut off wastes away. The latter is the well-known Wallerian type of denegeration. Now the factors that may damage the cell-bodies of a mass of neurones, or lacerate and sever their processes from them, are innu- merable and as a rule belong to the diseases and lesions of the con- nective tissue, the vascular and sustentacular elements generally. It is not necessary to describe these in detail but it is important to note that the localization of the lesion is rendered ofttimes very definite by the limited and definite area of destruction. As these lesions impli- cate more or less transversely the nervous paths, the degeneration travels in opposite directions away from it. Remaining always in the same system wherein it started, it advances upwards in the sensory and downwards in the motor paths if the lesion is anywhere between the cerebral cortex and the posterior spinal ganglia. If the lesion be 38 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES outside of the posterior spinal ganglia and include the paths of both the anterior and posterior roots, the degeneration will be downward in both the motor and sensory fibers in the nerves beyond the seat of the lesion. There will be no degeneration on the proximal side of the lesion. Secondary degenerations being due to damage of the neurone bodies or severance of the neuraxones from their nutritive centers, they are confined more distinctly and prominently to the neurones imme- diately involved. The next adjoining lower or higher neurones are but slightly affected and then only when they are functionally subor- dinate and dependent. In this respect there is a difference between the primary degener- ations due to such general influences as heredity and intoxication, and the secondary due to separation of a neurone or part of it from its nutritive center. Though localization symptoms may appear with the primary de- generations and in some particular cases be well-defined, they will be most pronounced and therefore most valuable in the secondary degen- erations. The whole question of localizations will be discussed however elsewhere. The constant characteristic of all those lesions, whatever their original cause may have been, that eventuate in secondary degenera- tion is that they involve vascular tissues and sooner or later are ac- companied by inflammation. Perhaps the only exception to this rule, and that is more apparent than real, is the case wherein a local area of nerve substance undergoes softening from the shutting off of its blood supply by an embolus or a thrombus. In this case the degenera- tion is of the primary type, though the lesion is grossly local and vas- cular. In all other cases, vascular trouble enters into the lesion with the appearance of more or less inflammation and secondary degenera- tion. Inflammation, therefore, is a most important feature in the pathol- ogy of the nervous system. The inflammatory process itself is iden- tically the same as it is elsewhere in the body. There is the same ini- tial hyperemia, extravasation of red corpuscles, infiltration of leu- cocytes, redness, swelling, softening and oedema. All this of course damages the delicate neurones lying in the midst of it. They undergo the degenerative process which usually terminates in complete func- tional and structural annihilation. The number of neurones that may be implicated is always a varia- ble one, depending upon the virulence of the cause and the violence of the lesion. Many neurones weather the storm and regain their func- tional integrity after the inflammation subsides. As inflammation is the reaction of the organism to an irritant, whether traumatic or infective, it is really a reparative process, an effort of nature to rid herself of noxious conditions. It is therefore always regressive rather than progressive and tends to limit itself so soon as it conquers the baleful cause which brought it into existence. In the warfare which it is carrying on against the agents of harm, it causes damage itself, some of which remains as a sort of a scar long after- GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 39 wards in the conditions of sclerosis, cavity-formation and atrophy. Among the last of course are to be included the secondary degenera- tions. Usually we recognize three types of inflammation in the nervous system, just as we do elsewhere. They are the simple exudative, the purulent and the so-called productive or proliferative inflammation. The former two are acute or subacute processes and differ from each other in regard to the presence of pus. The last is a chronic process ; is characterized by little congestion and exudation but rather by the slow formation of connective tissue. The cause of all these inflammations is not the same of course, some being due to mechanical, others to chemical, and still others to bacterial factors. It is unnecessary to discuss these further here as they are not at all peculiar. Among the residua and after-effects of inflammation in the nerv- ous system there are several of great importance. The secondary de- generations I have already spoken of. There is often local softening, general destruction and necrobiosis in the vicinity, with a mass of de- bris consisting of broken down nervous tissue, and blood elements. This undergoes absorption and leaves a scar or cyst in some cases. In others, especially when the inflammatory process has been chronic, sclerosis takes place. By this term is meant a hardening of the tissues from an overgrowth of the connective tissue elements and the replac- ing of the wasted true nerve cells and processes by them. The neu- roglia usually proliferates also. In sclerosis then we observe an absence or diminution of the num- ber of neurones with an increase of the neuroglia and connective tis- sue. Whether the overgrowth of the sustentacular tissue is first, caus- ing destruction of the neurones by compression, or whether the neu- rones degenerate first and the sustentacular elements proliferate to fill up the vacuity, is net always easy to determine. It is generally con- sidered, however, that the latter method occurs in the primary degen- erative diseases, whereas the former mode obtains in the inflammatory and secondary degenerations. From what has been said under the head of degeneration, it is easy to infer that when the neurone-cells are destroyed they are never regenerated. This is a fact of common observation and accounts for the perma- nency of many forms of paralysis and trophic disturbance. A neu- raxone that is destroyed may, however, completely regenerate. Just how it does so is not minutely known. All evidence seems to point out that the neurilemma and white substance of Schwann grow again in situ, but that the axis-cylinder extends forward from the proximal or cellular end into the new nervous tissue. The former reappear some- how from the local elements, whereas the latter steadily pushes for- ward like a branch growing from the trunk of a tree. It grows of course always from the trophic center. So'me recent experiments made at the University of Chicago demonstrate in the lower animals that neuraxones can thus extend 40 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES across lacerations and other forms of solution of continuity in the cen- tral nervous system. An interesting pathological condition which belongs solely to the nervous system is what is known as gliosis or gliomatosis. It is the basis of a certain form of tumor and cavity formation. It is essen- tially a proliferation of the glia cells and neuroglia tissue. Being non- vascular and epiblastic in origin it undergoes both primary and sec- ondary degeneration. In certain states of embryonic defectiveness, it proliferates, breaks down, is reabsorbed and leaves a cavity. Syringo- myelia is such a case in point. Sometimes with or without apparent cause, it spreads out and creates a tumor-like formation. These are the well-known gliomata of the nervous system. Finally it joins in the proliferative process of the connective-tissue elements in the production of the scleroses of the. cerebrospinal axis. In regard to the diseases of the nervous system in which no patho- logical changes can be discovered we can only argue, form hypotheses, and balance our logic. It is a time-honored custom to call such diseases functional, and to describe them as depending upon change of func- tion without change of structure. I must confess that I am incapable of comprehending such a paradoxical condition. I cannot grasp the idea that an abnormal manifestation can be the outgrowth of a normal structure. It is argued, however, that malnutrition, and possibly slight blood changes, are the cause of the functional disturbances. When pushed to the wall, those who argue thus say that they presume there may be some temporary physical or physio-chemical change in the neurones as a result of the malnutrition or reflex irritation. That, however, is included in what I conceive of as change of structure. It is too fine for our present means of detection undoubtedly but logic can penetrate often where physical means of research fail utterly. Logic seems to me to affirm beyond all question that an attribute of a thing cannot alter in the slightest degree without some alteration in the the thing itself. An attribute does not exist apart from the thing. It is but another point of view from which we behold that thing. A func- tion is an attribute of a functionating body. It has no more real ex- istence apart from that body than has its color, consistency or shape. Not one of the latter can vary without some variation having taken place in the body. In the same way, the function cannot change, it seems to me, without a change having taken place in the functionating substance. Of course this has no reference to what may be called sec- ondary functions or functions resulting from the action of one normal structure upon another. Psychosis is a secondary function, for it is the result of the interplay of certain elementary impulses. An abnormal psychosis may be a functional trouble with a normal physical basis. This is further discussed under hysteria. Therefore, in my opinion, there is a pathology for the so-called neuroses and functional diseases. ( )ur knowledge of it, like our knowl- edge of a good many other things, is still wanting. In their last analysis, functional diseases are as organic as any degenerative disease but not to so great a degree or so grossly as in what are commonly denominated flic organic diseases. GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 41 As a resume of the pathology of diseases of the nervous system the following schema may be useful : A. Pathological changes distinctively and primarily Neural (in- cluding the Neurones and the Neuroglia). a. Malformations ; Hereditary and Congenital Defects; In- complete Development or Agenesis ; Defective Development or Dysgenesis. b. Degeneration, Primary and Secondary. c. Gliosis and Gliomatosis. B. Pathological changes, not distinctively nor primarily Neural (including the connective tissues, blood-vessels, lvmphatics and epithe- lia). a. Inflammations. b. Hyperemia, Anaemia, Hemorrhage, CEdema, Arterial and Venous Diseases generally. c. Connective Tissue Sclerosis. d. Infective Processes like Tuberculosis, Syphilis, etc. e. All Neoplasms, Tumors, Parasitic Growths, Cysts, Ab- scesses, etc. (excepting Gliomata). GENERAL CONSIDERATIONS UPON THE SYMPTOMAT- OLOGY AND DIAGNOSIS OF DISEASES OF THE NERVOUS SYSTEM AND THE EXAMINA- TION OF THE PATIENT. It will be most appropriate to discuss the general semeiology of diseases of the brain, spinal cord, nerves and sympathetic system at the head of the sections devoted to these diseases and immediately after the consideration of the anatomy and physiology of the respective, parts of the nervous system. In this chapter I will take up, merely in a gen- eral way, the symptoms that belong to the nervous system as a whole, and will attempt some practical suggestions in regard to the proper method of examining a patient. On account of the inaccessibility of the nervous apparatus to direct examination, except in the one little spot back of the eye, and on account of the multiplicity and interrelations of its functional activities, the examination of it is a supreme test of the physician's natural ingenuity, skill and knowledge. One must ap- proach it with his mind completely unbiased, alert, and flexible. New points of view must be taken when unexpected hints are thrown out by the patient or his guardians. No opinions must be formed until the examination is finished, and even then it is sometimes well to with- hold them until after two or three future examinations. Snap diagno- ses are especially dangerous in neurology. There are geniuses, ex- ceedingly rare and far between, however, who can instinctively grasp the full situation after the patient has uttered a few sentences and ex- hibited a few signs. Even they sometimes make outrageous blunders. The more experienced a man is and the more learned in regard to the 42 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES nervous mechanism of course the more swiftly and accurately will he arrive at his final diagnosis. It is the safest plan for every one, how- ever, to be patient and painstaking, to omit no details and to weigh and test every phenomenon even to the point of tediousness. A mere glance at the anatomy and physiology of the nervous sys- tem demonstrates how absolutely necessary that every organ, tissue and function must be closely interrogated. In this respect a neurological examination differs pre-eminently from every other that the medical man is called upon to make. Only in cases of the greatest emergency should a hurried manner and a quick diagnosis be adopted. It is un- fortunate, though necessary of course, that such has to be done even then, for it is sometimes the cause of some very sad mistakes. When there is grave doubt, or not a reasonably clear symptomatology under these circumstances, the physician will do well to do nothing. Under such conditions nature is less liable to err than is human blindness. When there is no emergency or need of special haste, the phy- sician should first note carefully all of the attendant circumstances sur- rounding the patient. If he visits the latter at his home he should ob- serve the environment as closely as possible, for hints are sometimes thrown out in this way in regard to the mental and social status, the mode of life, the joys and the sorrows. The relatives and companions about him may be highly suggestive. Alcoholic coma might not in- appropriately be thought of in a saloon ; hystero-neurasthenia in a girl might justly be suspected if a brutal father and a termagant mother are present ; trouble from excessive mental study would hardly be ex- pected where there were no signs of a book. The patient's own attitude must be quickly remarked and the changes it undergoes during the examination. By the attitude I mean both the physical and the mental. It is wise to examine the patient alone and in the presence of oth- ers. In hysteria it is sometimes risky to remain alone in the room with the patient, but the door can be left partly open while the friends and relatives are in a distant room. The presence of one of the latter when distasteful may cause all sorts of irregularities in the symptoms. A temporary change of environment often astonishingly changes the complexion of things. I remember a woman who was sent to a pri- vate hospital several times by me. It was almost ludicrous how reg- ularly as clock-work she flew into her hysterical attacks at home while during weeks at the hospital she was as docile and reasonable as any creature. Syphilitics will often admit to the consultant, who charges them a good fee and for whom they have therefore perhaps an exag- gerated reverence, the disease that they have constantly and strenu- ously denied to their family doctor. Get the patient always, if possible, under other conditions and examine further before forming a final opinion. Avoid leading questions in eliciting the history of the case. Neu- rotics are particularly open to suggestion. I have seen diagnoses made that were really constructed on the semeiology suggested by the doc- tor and acquiesced in by the susceptible patient. This is an easy and common pitfall and should be carefully guarded against GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 43 Let the patient first tell his own story, often in his own verbose way, for thereby most valuable hints may be dropped and leading lines of examination suggested. After he has said all he has to say, then let the relatives and associates tell their story. Even let them give their opinions. Be most patient with them for they are reasoning, albeit ignorant laymen ; and the very picturesqueness of their descrip- tion, the energy and interest with which they tell of the patient's strange exhibitions, the biased yet unbiased attitude of mind in which they are, may go a long way toward giving a complete clinical pic- ture, whereas a too close limitation to the mere scientific data may lead to some confusion. In the examination of hysterics, epileptics and the insane we have to depend very largely upon the accounts of the family and of the friends. We must, however, be always guarded against letting their views determine ours. A nice adjustment between the medical and the lay prejudices will here reveal the keen examiner and the skillful physician. During the telling of the history of the case by the relatives note the trend of their ideas and the degree of their intelligence. The pa- tient may have moved intellectually beyond their sphere, and his en- vironment being uncongenial he may act in a way that seems queer to them but is not entirely illogical. This is the origin, in a large meas- ure, of the popular conception of the insanity and eccentricity of geniuses and of artists. After acquiring all the information he can from the patient and "his friends, the physician takes up the examination himself and while allowing them to interject an occasional remark that may add new light to some things, he avoids being diverted from his own course of proce- dure. He should have a general systematic method whereby to con- duct his examination, else he will be certain to overlook many things. Even though he may not have to go through the whole form even- time before arriving at a diagnosis, it is nevertheless good to do so because important matters may thus be brought out, time may really "be saved in the end, and the practice of being systematic may develop a desirable facility for examining future patients. A scheme some- what like the following may be adopted, even printed out in one's case book : 44 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES Name Age Sex Nationality Occupation . Marriage Temperament Culture Date. . . Family History: Grandparents Parents Brothers and Sisters Other near Relatives Personal History : Habits as to Diet Habits as to Alcohol Habits as to Tobacco and Drugs . Habits as to Venery Inherited and Acquired Dis- eases : Syphilis Gonorrhoea . . Other Infections Condition of the — Lungs Heart Arteries Alimentary Tract Genito-Urinarv Tract Traumata Deformities Pulse Temperature Respiration Urine Gait Attitude Patient's Account Trouble in Brief of Pre? em Motor Symptoms . Sensory Symptoms Reflexes Electrical Phenomena Vasomotor and Secretory Symp- toms Trophic Symptoms Eye and Vision . . . Ear and Hearing. . Nose and Smell. . . Mouth and Taste. . General Remarks . Voice Speech Handwriting Diagnosis Psychic Symptoms The diagnosis of disease of the nervous system depends upon both etiology and symptomatology. It must be made from aJl available phys- ical and mental data. Its end is the determination of the actual exist- ence of a nervous disease, the character of the lesion and its loca- tion. The real presence of a nervous disease is decided almost wholly by etiological factors and a few observable changes like a choked disc or GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 45 muscular atrophy. In other words not all motor and sensory and psychic' abnormal manifestations represent actual disease. Simulation and malingering may be present. Suggestibility may provoke mislead- ing exhibitions. Secondary irritation from extraneous conditions of all sorts, both in and out of the body, may give rise to the exaltation or suppression cf nervous functions that closely imitate inherent nerve trouble. It is sometimes the hardest part of the diagnosis to determine whether there is an actual disease of the nervous system present. No fast rules can be laid down for determining this. It is all a matter of the logical ability, the shrew T dness, the skill, and the knowledge of the diagnostician. When it is recognized that an actual disease of the nervous sys- tem is present, the nature of it must be decided by a careful study of both its etiology and symptomatology. The location of the lesion is inferred almost wholly from the symptoms, though the etiology helps in determining it slightly in some cases. In the preceding chapter upon pathology, I have pointed out some general facts in regard to the nature of the various lesions and how they may be inferred. Localization symptoms will be taken up at the head of the various sections devoted to the brain, the spinal cord and the nerves. The gait of the patient in walking is a most obvious symptom sometimes, and to one experienced in nervous troubles will alone often suggest the diagnosis. The hemiplegic, the tabetic, the victim of dis- turbed equilibrium of the cerebellar or other type, and of spastic para- plegia, can almost instantaneously be recognized. The attitude, with or without the peculiar gait, is frequently very significant. Who does not recognize the characteristic stoop and posi- tion of the hands in paralysis agitans? Even the laity notice the list- less, fixed, indifferent position of the victim of melancholia. If atti- tude were given more consideration, hysteria, neurasthenia, many forms of psychosis and not a few organic troubles would almost be suspected at once sometimes. The attitudes of disease are not infrequently path- ognomonic. While the patient is giving his account of the first appearance of his present trouble and of its course and symptoms up to the present mo- ment, note carefully the character of his voice and the mode of his speech. Does he speak loudly or softly? Is his voice rasping or smooth? Is there aphonia and does he have to whisper? Observe whether he trips in his speech or runs his words together or scans his sentences as if he were scanning a line of poetry. Does he omit or repeat words or introduce irrelevant words into his sentences? Ask him to read and note if he does the same thing when reading. Aphonia, dysarthria and aphasia will all be discussed after the consideration of the physiology of the brain. They are mentioned here simply to im- press the importance of noting them carefully in the course of the ex- amination. Agraphia, trembling and other impediments to writing will also be described elsewhere. Psychic symptoms are hardly of less importance in neurology than 46 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES they are in psychiatry. The mind is the product of cerebral activity, and cerebral activity is provoked by afferent nervous impulses. It may be interesting but hardly profitable to discuss such questions as the immateriality of mind and the duality of man's nature. We have no proof, that can be called scientific, of a consciousness apart from cerebral function. To say this is not to deny the mind's possible im- material existence ; it is merely affirming the scientific limitations of the question. To escape all such gross notions as mental telepathy, mental trans- ference, mind-reading, clairvoyance, spiritism, etc., one has but to recollect that psychic phenomena are never known to occur without simultaneous cerebral phenomena. Dead brains have never, in all the ages, given the slightest evidence of the presence of mind, and mind has never been observed apart from living brains. This interdependence is further shown in the parallelism that ob- tains between physical disease and mental aberration. In many cases actual gross changes are observed in the brain ; in others the changes are so slight that our present means of investigation cannot detect them. It is not improbable that a disturbance of the normal flow of inpouring sensations, by means of various nervous and other diseases, may underlie a large number of the phenomena of psychiatry. At all events, as the nervous system is the seat of the mind pre-eminently, and as it is the apparatus upon which it seems immediately to depend both for its own portrayal, activity, and communication with the outer world, it is obvious that mental symptoms are of immense significance in neurology. A detailed description of these symptoms will be more appropri- ately considered along with the physiology of the brain. It may be noted here, however, that chief among them are the mental stigmata of degeneracy, abnormal emotional states with exaltation or depres- sion, delirium, maniacal violence, illusions, hallucinations and delusions, loss of consciousness, or coma, double consciousness, amnesia, aphasia, word-deafness, word-blindness, agraphia, etc. Motor and sensory symptoms have very dissimilar diagnostic valu- ation. The motor symptoms, being objective and capable of being ex- amined apart from the patient's consciousness, are the more definite, distinct and reliable to base a diagnosis upon. The sensory symptoms are almost wholly subjective. To be sure we can infer a good deal objectively in reference to them by varying ourselves the stimuli that provoke them and by testing them in the reflex arcs; but nevertheless even here it is practically impossible f o eliminate entirely the patient's consciousness during their examine tion. Under ether narcosis they are not in evidence even in relation to the reflex arcs. Too much stress, therefore, should not be laid upon sensory phe- nomena except in association with others. They are. however, the most important symptoms to the patient and sometimes the only ones. Their greatest value, after their reality has been positively determined, is in the localization of the lesion. The psychic nature of hysteria is all but positively affirmed by the peculiar distribution and prominence GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 47 of its sensory symptoms. A moving myelitis can sometimes be beau- tifully watched by the rise and fall of the border-line of anaesthesia. The symptomatic significance of the various forms of sensory dis- turbance will be referred to again in the introductory chapters to the special diseases. Only some general features will be noted here. There are five forms of sensation, commonly spoken of as gen- eral and special sense. Biologically they are all evoluted out of com- mon or simple touch sense. So far as the nervous apparatus itself goes there are nothing but afferent impulses. The naming of these impulses, touch, pain, heat and cold, sight, smell, taste and hearing, is the result of the special end-organs in connection with the nerves. A nervous atrophy that produces blindness, for instance, is no different from a nervous atrophy that produces cutaneous anaesthesia ; the form of nervous lesion that causes auditory hallucinations may be exactly the same as the form of nervous lesion that causes a paresthesia. With all the sensory phenomena, therefore, there should be a most careful determination as to whether the lesion resides in the outer end- organs, the receiving brain centers, or the intervening nervous pathway. It would be ludicrous, if it were not so tragical, to note how frantic- ally and persistently glasses are worn and changed, even upon the recommendation of careless and ignorant opticians, for a fading eye- sight, when an examination of the eye-ground would reveal a progres- sing optic atrophy. It is a common fact for the pains of tabes, a central disease, to be mistaken for the pains of rheumatism, a peripheral trouble. The diagnosing of the presence of a sensory disturbance is not enough. It must be determined why and wherefore the disturbance is present. In the skin are located three forms of common sensation, namely, touch, pain and temperature sense. In the muscles, tendons, fascia, joints and contiguous structures there is what is called a muscular sense, a sense which gives us some- what an idea of the location of the limbs. The nature of the latter sense is not clearly established yet and some authorities even doubt its existence as a special sense. The cutaneous and muscular senses are as much dependent upon special end-organs for their differentiation as are the so-called special senses of hearing, smell, taste and sight. When a sensation is excessively or abnormally acute we speak of the condition as hyperesthesia. When it is abnormally dull we re- fer to it as hypcesthesia. If it is absent entirely it is anesthesia. Per- verted sensations such as numbness, "pins-and-needles" feeling, formi- cation or the sensation as of ants crawling over the skin, are known as paresthesia. These terms have been so universally applied to the tactile sense alone that, I am satisfied, it has had a good deal to do with keeping alive the common notion that special sense is in some way something extraordinarily different from the sense of touch. Nerve blindness is nothing but optic anaesthesia ; visual hallucinations, like flashes of light, fortification figures, are really, when of nervous origin, only instances 48 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES of optic paresthesia. This should never be forgotten as it will save one from propounding such absurd questions as, Why should blind- ness occur in hysteria along with cutaneous anaesthesia, as a notable symptom? A proper knowledge of the basic character of all sensa- tion would cause the question to be asked, Why shouldn't it occur? Disease, it must be remembered, may attack the end-organs and produce hyperesthesia, anesthesia or paresthesia; a cataract produces blindness ; a scar in the skin causes local loss of sensation ; the loca- tion and lesion are unlike but the manfestations are the same, anes- thesia, but not pure neural anesthesia. Atrophy of the optic nerve and atrophy of the spinal sensory nerves cause respectively loss of vision and loss of the sense of touch ; the lesion is the same but the location merely is different. That is pure neural anesthesia. Destruction of the visual centers in the occipital cortex and destruction of the sen- sory centers in the parietal cortex alike cause sensory anesthesia. The universality of irritability in the nervous system is a most im- portant fact for it helps to give valuation to these phenomena as a means of localization, since it is thus varied merely by reason of the connections which the nervous apparatus makes. In this respect the sensory apparatus is the same as the motor wherein we localize lesions chiefly by the particular muscles with which it is connected. For the alterations in the pain sense we use the terms hyperalgesia and analgesia. Sometimes pain arises spontaneously in an analgesic area. This is known as analgesia dolorosa. Thermancesthesia is of course a loss of the temperature sense; hot and cold are not felt at all, only the touch of the heated or frozen ob- ject. In syringomyelia this is a remarkable and troublesome symptom. A localized pain without a known cause is a topoalgia. Causal gia is a pain like that of burning. Polycesthesia refers to successive repetitions of a sensation after one stimulation. In macrocesthesia objects feel larger than they actually are. A sensation may be quite normal in all respects except in being delayed. This is not an uncommon phenomenon and must always be looked for. Transference of a sensation is a curious exhibition sometimes called allochciria. A stimulation on one side of the body is referred to a similar spot on the other. Ataxia or incoordination of movement, astereognosis or loss of the sense of the shape of objects when held in the hand, and loss of reflex, are some of the sequele of disturbed sensation that will be discussed more in detail elsewhere. A knowledge of the sensory innervation of the skin is necessary for localization purposes. To test the sensations demands but a little ingenuity. Touch can be examined by passing lightly over the skin, with the patient blind- folded, a camel's hair brush, a piece of raw*cotton or other light, soft object. Note the quickness of the response, the accuracy of the local- ization and how it feels to the patient. GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 49 The temperature sense is proved by the application to the skin of a piece of ice or a test-tube filled with hot water. The use of excessive heat, pricking with a pin, or pinching will tell whether the pain sense is lost or not. The muscular sense is noted by placing the limbs in different positions, while the patient is lying in bed, and asking where they are without his looking at them. Place various weights in his hands and note whether he remarks the difference. This tests the pressure sense. Any small object like a watch, a key, a match-box, placed in the hand will aid in the detection of astereognosis. The patient gives an erroneous description of its shape or says he cannot describe it at all. • The costhesiometer is a compass-like apparatus with a graduated scale on it for the accurate measurement of the tactile discrimination in the skin. The two points of a hairpin or pair of sharp scissors may even be conveniently employed. The tongue, tips of the fingers and lips are the most sensitive parts of the body and serve well for tests with the sesthesiometer. In order to have a normal standard ready at hand for purposes of comparison, the following table from Weber, indicating the shortest distances and the parts of the body on which two points can be recognized, is presented. Tip of tongue 1-24 inch. 1 Aim. Palmar surface of third phalanx of forefinger. . . 1-24 inch. 1 Mm. Palmar surface of second phalanges of fingers. . 1-12 inch. 2 Mm. Red surface of underlip 1-6 inch. 4 Mm. Tip of nose 1-6 inch. 4 Mm. Middle of dorsum of tongue 1-4 inch. 6 Mm. Palm of hand 1-3 inch. 8 Mm. Center of hard palate 5-12 inch. 10 Mm. Dorsal surface of first phalanges of fingers 1-2 inch. 12 Mm. Back of hand 7-12 inch. 14 Mm. Dorsum of foot, near toes 1 1-6 inch. 25 Mm. Gluteal region 1 1-2 inch. 37 Mm. Sacral region 1 1-2 inch. 37 Mm. Upper and lower parts of forearm 1 1-2 inch. 37 Mm. Back of neck near occiput 2 inch. 50 Mm. Upper dorsal and mid-lumbar regions 2 inch. 50 Mm. Middle part of forearm 2 1-2 inch. 62 Mm. Middle of thigh 21-2 inch. 62 Mm. Mid-cervical region 2 1-2 inch. 62 Mm. Mid-dorsal region 2 1-2 inch. 62 Mm. Such wide variations of judgment occur among normal individuals that not much reliance can be placed on the sesthesiometer findings un- less they are very gross and constant. In general it may be consid- ered abnormal if the points have to be separated double the above dis- tances before they are able to be distinguished apart. The pain sense may be approximately measured and the differences observed by the employment of different strengths of the faradic cur- rent. Of course here as in all tests for sensation the eves must be covered, normal parts of the body must be compared with abnormal, 5C GENERAL CONSIDERATIONS UPON NERVOUS DISEASES repetitions of the test must be made at different times and false touches and deceptions must be practiced to note the amount or presence of sug- gestibility. It is well to outline an anaesthetic area sometimes with a blue pencil or iodine brush and note the changes from time to time. Blanks with printed outlines of the body upon them are useful for keep- ing a record of such data. The special senses have their own particular ways of being exam- ined which will be noted under the heads of the respective cranial nerves. Motor phenomena usually owe their initiation to some form of efferent nervous impulse. Nerves and muscles make up a very compact and closely united neuromuscular apparatus. The nervous tissue in- cites the movement, the muscle executes it. Only under exceptional circumstances do muscles contract from other than neural stimulation. Direct irritation by blows or other means may in some instances cause the muscular fibrillse to contract, and undoubtedly muscular paresis re- sults from inherent muscular disease*. Nevertheless it is so true that the vast majority of muscular contractions are due to nervous stimu- lation, that it may be generally affirmed that disturbance of the former is indicative of disease of the latter. The impulse that provokes a mus- cle into activity usually has its origin in a nerve cell or neurone-body. It is not probable that direct irritation to the neuraxones of the motor neurones awaken muscular phenomena. In the cases in which irritation of the peripheral nerves provokes spasms, the sensory neuraxones are in all likelihood irritated and in a reflex way the motor side of the arc is put into functional activity. Motor impulses therefore always start from the cell-bodies of the motor neurones. They may arise de novo there or they may be the result of stimulation by the related sensory neurones. In all forms of motor activity, therefore, normal or exalted, the cell-bodies of the related motor neurones, are more or less implicated. If the activity is diminished or annihilated the trouble may be anywhere in the motor neurones, their cell-bodies or their processes. Hence mus- cular over-activity has a much wider origin than has under-activity. This is an important distinction when it comes to a question of diag- nosis. ■ Spasms are due to far more numerous causes than are paralyses. Beyond the mere statement that stimulation is present, we can offer no explanation as to how nerve force passes over into muscular force, how excitement merely provokes contraction. Much more im- portant is it to know that all muscular movements of neural origin are due to impulses from two sets of neurones, the upper and the lower. An examination of the neuronic architecture of the nervous system re- veals the fact that the muscles are under the direct and immediate con- trol of the neurones whose cell-bodies are in the gray matter of the cord and its homologue the cranial nuclei, and that they are under the indirect or mediate control of the neurones whose cell-bodies are in the cerebral cortex. Individual muscles therefore are the executive agents of the lower neurones ; generalized movements, inhibition, and control of the lower neurones constitute the primary purpose of the activity of the Upper neurones. GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 5T So far as we know, the essential nature of nerve activity, the ex- pression of neurility, is identically the same in both sets of neurones. They differ in function solely on account of their respective end rela- tionships. Thus, as will be described more in detail in other sections of this book, they lend themselves by reason of the difference of func- tion to focal diagnosis. Individual muscle disturbances will point to the location in the cord or cranial nuclei. The disturbance of bodily movements will indicate quite as sharply just where the trouble is located in the cerebral cortex or its subsidiary structures. The symptoms upon which this localization diagnosis is to be made will be discussed at the head of brain and cord diseases. The character of the motor disturbance is indicative of both the location and nature of the lesion. As the cerebral set of neurones ex- ercise normally an inhibitory control over the spinal, when they are diseased that control is relaxed and the resulting motor disturbance is rigid and spastic in character. Where the disease involves only or pri- marily the spinal neurones, the disturbance is flaccid and limp in char- acter. In either upper or lower neurone disease, the lesion may be only such as to irritate and cause overaction or irregular action in the mus- cles. The irritative influence usually has its origin entirely outside of the neurone, either from disease in the sensory apparatus or in gross forms of trouble in the mesobiastic tissues. Lesions that are de- structive of the motor neurones are therefore usually inherent lesions and directly abolish the function of the neurones. Lesions that are merely irritative are more generally extraneous, affect the motor neu- rones secondarily, and exaggerate or otherwise disarrange their func- tion. The common evidence of destructive lesions is therefore paralysis, of irritative lesions spasm and inco-ordination. It is extremely important that the term paralysis or akinesis should be limited to the abolition of muscular movements from neural lesions alone. As I have already intimated, immobility may be due to many other causes, as for example ankylosis, muscular disease, rheumatism, frac- tures and dislocations. Both the voluntary and involuntary sets of muscles may be par- alyzed. There are degrees of paralysis so that we speak of complete and incomplete paralysis. The term paresis is sometimes used for the lat- ter. Individual muscles may be paralyzed or physiological groups of muscles concerned in particular forms of movement. A flaccid paralysis, is one in which the part hangs dead, limp, immobile ; a spastic paraly- sis, one in which there is stiffness, rigidity and tension in the part. A paralysis may have its origin in the higher psychic centers even. This is commonly spoken of as pseudoparalysis. The distribution of the paralysis is indicated by the use of s/ich terms as monoplegia, hemiplegia, paraplegia and crossed paralysis. As these belong to localizing signs, they will be referred to again. In testing a paralysis the patient should relax himself as completely 52 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES as possible and should be examined in various positions, in and out of bed. The normal physiological movements should be well known and the diseased side should be carefully compared with the well. First let the patient show what he himself can or cannot do. If the paralysis is only partial, take hold of the part and note the force of resistance exerted by the patient. The dynamometer is a graduated oval spring which can be used, by pressing it, to measure the comparative power of resistance. Its use is limited, however. Remember that the left side is normally weaker than the right. Paralysis of individual muscles can be sometimes tested thus, though in most cases it has to be noted by the attitude of the parr and by the electrical tests and associated atrophy. Tests for paralysis should be made repeatedly and varied in everv possible way. It should be noted whether there is the slightest shade FIGURE i. Dynamometer. of associated movement in the paralyzed member when the well one is stretched out. A little ingenuity and a perfect familiarity with the normal func- tions of the muscles are all that is needed for testing paralysis. Various instruments have been devised but immediate examination without in- struments of any sort will give a careful, skillful and experienced ex- aminer more and better information than an}' form of mediate test. Spasm or hypcrkinesis is an exaggeration of motility. Its real na- ture is hidden somewhat in obscurity, though it is probably due to exag- gerated or incessant nerve stimulation. It is not necessarily due to disease in the motor neurones. In fact, it is usually the result of irri- tation of these neurones by disease outside of them. The latter may be of a vascular or connective-tissue type, of a toxsemic nature, or a le- sion involving the sensory neurones. Muscular spasm is very often a mere reflex phenomenon and so far as the nervous system is concerned may not be the expression of disease at all. We do not know that there is any essential difference between tonic and clonic spasms except that the stimulating impulses in the former are so short and rapid in succession that the muscle fibres have no chance to relax and the whole muscle remains in prolonged and constant contraction. In clonic spasms there is an alternation of con- traction and relaxation so gross as to be easily observed. A cramp is nothing but a painful spasm. A convulsion is a spasm that involves the entire musculature of the GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 53 body or parts of the body. Convulsions may be tonic or clonic, co-ordi- nate or inco-ordinate. Unconsciousness usually accompanies a con- vulsion. Choreiform movements are spasmodic jerkmgs, in certain groups of muscles, irregular, inco-ordinate and more or less violent and par- tially under the control of the will. What is called convulsive tic is a very localized form of choreiform spasm, possibly even in one muscle, but more generally in a group of muscles that perform a certain physiological act. Athetoid movements, athetosis, consist of slow, ceaseless, waxy- like flexion and extension of the hands and feet and their various parts. Other forms of muscular spasm are the excessive muscular tension and contracture seen in some diseases. These are more or less perma- nent or at least chronic conditions and are consequently accompanied by a certain degree of organic change. If either one should disappear during sleep or under etherization, it would show that it was merely a functional trouble, probably hysterical. A forced movement is a sort of convulsion in which a patient in- voluntarily turns and moves in various peculiar but not altogether un- natural ways. He may whirl about suddenly or start off in a particular direction. Associated movements are another sort of curious, spasmodic phe- nomena. A limb lying at rest involuntarily performs the same or simi- lar movement that is voluntarily performed in the other limb. Hemi- plegic limbs sometimes thus move in more or less imperfect unison with the movements in the sound limb. As with paralysis so in cases of hvperkinesis, the localization of the lesion can be partly inferred from the distribution of the phenom- ena. We speak therefore of monospasms, hemispasms and other types. The symptom is so obvious that it requires no special skill to distin- guish it. Tremor is a fine clonic spasm, rhythmical, constant, independent of the will and due to faulty innervation. There is an ataxic form of it revealed upon volitional movement. The parts affected are kept con- stantly oscillating, usually without pain or marked exhaustion. The oscillations vary from 3 to 12 per second. When they are from 3 to 5 per second the tremor is coarse, when from 8 to 12 it is fine. Weak- ness is usually associated with it. The ataxic tremor is badly named intentional tremor. Fibrillary tremor is one in which the phenomenon seems to be lim- ited to particular fasciculi in bundles of muscular fibres. It is often- times so delicate that it can only be detected by resting the linger on the part affected and feeling it. Tremor is often increased by voluntary effort, as witness the tremor of dementia paralytica when the patient attempts to speak or write. Incoordination of movement, or dyskinesis is a most important neu- rological symptom. We have already seen that incoordination of move- ment may be due 10 sensory disturbance. Such is the cause of it in locomotor ataxia for instance. The various attitudes assumed by our bodies, the gait adopted in our walking, the physiological movements of 54 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES all sorts, are the result of the harmonious combined action of a number of muscles. There is normally a nice adjustment between antagonistic or opposing sets of muscles so that action and counteraction are never allowed to pass beyond the point necessary to accomplish a given move- ment. Flexion is always nicely opposed to extension, pronation to su- pination, dilatation to contraction. This is an unconscious adjustment, though it is obviously submissive to the will. We can overflex the arm but we cannot release the extensors from their entire control of the ac- tion at the same time. We can close the orbicularis oris but we cannot prevent the other facial muscles from taking part in the performance. In some of the sphincters the will is entirely inoperative, and yet the sphincter functionates under an opposing inhibition. Whenever this adjustment is broken, the movements become most indefinite, irregular, ludicrous and bizarre. The volition starts the muscles to contract, but what they will do not even the patient himself can imagine. Anarchy seems to rule among them and most surprising exhibitions are pre- sented. Such is incoordination or ataxia. It is from its very nature not a disease of one anatomical structure, though it is the product many times of a more or less anatomically limited lesion. We have said that movements are the results of activity in the cell bodies of the motor neurones and that this activity may arise de novo, or as the result of sensory stimulation. When sensation is disturbed, one important cause of muscular action is at fault and as a result that muscular action itself is confused. Again, for most of the automatic and involuntary movements, such as walking and the contractions of the uterus in parturition, there is somewhere in the central nervous system an arrangement of neurones for the adjustment of the various muscular activities that go to make up the completed movements. And finally in all voluntary movement the adjustment is uncon- sciously managed so that all muscles work harmoniously together, for the accomplishment of the particular end in view. Ataxia therefore may result from a disturbance of afferent im- pulses, from disease in the central coordinating apparatuses, or from the upsetting of normal mentalization. In all of this there may be no essential motor disturbances, no paralysis, no spasm, but merely sen- sory and reflex and mental trouble. The movements are bizarre because their sources of stimulation are in an abnormal state. There may, how- ever, be a purely motor incoordination. One set of opposing muscles may be functionally disturbed, whereby the normal balance of power between the two sets is broken. Indeed the different parts of the same muscle, and even different sets of fibrils, may be so affected that an intramuscular incoordination obtains. This doubtless is the underlying cause of many forms of coarse and fine tremor of irregular character. As I have intimated ataxia is the typical form of incoordination. It may be sensory or motor, usually sensory, in origin or due to trouble in the central organs that subserve the maintenance of coordination and equilibrium. In ataxia the disturbance of the certainty of purposive GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 55 movements is most obvious or with a little ingenuity can be easily elicited. Sensory ataxia is beautifully seen in tabes ; sensory and motor ataxia is probably observed in multiple neuritis and disseminated sclerosis. Cerebellar ataxia, in which the reeling gait of acute alcoholism is most accurately imitated, is due to trouble in the central coordinating apparatus. Vertigo is a form of central ataxia, so also is the confusion some- times observed in the psychoses of mild and severe degree wherein the ideas of movement are concerned. It requires a harmonious action between muscles to maintain an attitude as well as to perform a movement ; hence there is static as well as motor ataxia. All the senses help in the maintenance of equilibrium, therefore shutting off the visual sense by closing the eyes causes a static or motor ataxia to be increased. All one has to do to test for ataxia is to ask the patient to per- form some complicated movement — walk a chalk line on the floor, touch the end of his nose with his finger while his eyes are shut. Speech may be ataxic in character and it may be of motor or psychic origin. This will be referred to again in the section on the brain. A most important nervous manifestation is that of reflex action. In fact, reflex action, which means in its last analysis nothing more nor less than stimulation and return response, is the fundamental raison d'etre of the nervous system. To be cognizant of external irritants and to send back appropriate counter effects to the part or parts related to the source of irritation, is what nervous tissue was biologically evolved for. There is thus an ingo and an outgo as the fundamental fact in all primary nervous manifestations. Every move in life, even thought itself, is but a reflex or a modification of a reflex. Impulses in some low forms of animal life may travel in and out over the same pathways. In the higher forms the afferent are different from the efferent pathways. Between the two, at their central ap- proximation, there may be interjected special cells or neurones to modify the impulse in its round from afferent to efferent path. A reflex action therefore is seen to involve a more or less compli- cated arc consisting of afferent^ central, and efferent elements. The first originates in the skin or o'ther special end-organ of sense and terminates in some central gray matter. This gray matter contains the modifying intervening neurones and is usually under the inhibitory control, to a certain extent, of the higher or cortical neurones. The efferent pathway starts in the central gray matter, and terminates in the muscle, gland or secretory organ. An irritation in the skin there- fore sends an impulse inward and around the arc until it expends itself finally in muscular action or altered secretion. In its course this im- pulse, under certain circumstances, may be halted by a central im- pulse sent down from the brain or it may send an impulse itself up to the brain. 56 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES That is in brief the whole picture of a reflex action. It is clear that a lesion anywhere in the arc, in the afferent, the central or the efferent tract, may put an end to the reflex. Moreover, disease in the controlling inhibitory path reaching down from above will undoubtedly modify the activity of the reflex arc. If the inhibitory control is destroyed, the reflex will of course be released ; if it is in- creased the reflex may be completely throttled. According to the loca- tion of the center of the reflex arc, and according to the terminations of either end of it, will the localization manifestations be in connection with it. Therefore the detailed description of the individual reflexes will be postponed until the discussion of the special parts of the ner- vous system is taken up. It may be well to state here, however, that there are recognized in neurological symptomatology, four kinds of reflexes, the skin, or su- perficial, reflexes ; the tendonous, or deep, reflexes ; the idiopathic mus- cle reflexes, and the visceral reflexes. Reflexes may be absent, or they may be merely exaggerated or diminuished. Reflex phenomena occur wherever there is a neuro-muscular ap- paratus, hence these phenomena are observed in connection with the sympathetic as well as the cerebrospinal system; in the organs, and the vasomotor apparatus of the body. The superficial or skin reflexes are best elicited by a slight touch, a gentle pinch, a pin prick, a tickling or a momentary application of cold or heat to the skin. The value of these reflexes as localization symptoms is variable, as will be pointed out elsewhere. The more im- portant of them and the method of provoking them are as follows. About the head many are obvious and are seen in the winking and the twitching of the facial muscles, and in animals in the shaking of the ears to remove insects and irritating objects. The scratching of the skin of the face or chin causes the pupil to dilate. Irritating die skin over the back muscles, as for example over the erector spinas and scap- ular muscles, causes the latter to contract. The same is true of the muscles of the abdomen and thorax ; cutaneous irritation at the sides causes them to contract, especially the rectus abdominis. The cremasteric reflex consists of the drawing up of the testicle when the skin of the inner thigh of the same side is pinched and tickled. The scrotum alone is not affected. The palmar reflex is of very little importance, but in sleep some people move the fingers when the palm of the hand is touched. The plantar reflex is usually well marked and in fact enters into the Babinski sign. This observer noted that when the sole of the foot is stroked, there is a difference between the movement of the foot and of the toes. Normally when the plantar skin is slightly irritated, the foot is thrown into dorsal flexion and the toes into plantar flexion. That is the ordinary, long-recognized, fa- miliar plantar reflex. Babinski noticed thai in disease, when the sole is tickled, dorsal flexion of the foot lakes place, but the toes, especially the great toe, undergo dorsal reflexion also, or what is the same thing, extreme extension. This is a must important criterion oi disease. though its fullest significance we have yet to learn. GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 57 Oppenheim's reflex is close to that of Babinski's. It consists of a dorsal flexion of the toes and foot when the inner surface of the leg is stroked from above downward. The comparative importance of the plantar over the palmar reflexes illustrates a cardinal law that is true of all the spinal reflexes appar- ently, namely, that the nearer they are to the brain, the less active they are. The presence of these skin reflexes leads to the inference, of course, that their arc through the cord is normal. Their absence, how- ever, does not signify very much, because the mind of the patient is FIGURE 2. Method of testing the patella reflex. so intimately implicated in them, that it is always a matter of uncer- tainty as to the physical or psychical cause for their non-appearance. Among the most valuable symptoms in neurology are the deep, tendon reflexes. It is not necessary to discuss here the many views in regard to their essential nature. Investigators of equal reliability hold that they are true reflex phenomena on the one hand, or mere ex- pressions of muscle tonus, itself a reflex phenomenon, on the other. For the clinician it is enough that they are regularly modified in dis- ease and that they represent a reflex impulse. The muscular contraction is provoked by a stroke upon the muscle or its tendon. The examiner's ingenuity will suggest Che best attitude of the patient for placing the muscle-tendon in a condition of slight 58 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES tension withcut being pressed., stretched or otherwise prevented from acting. A slight blow with the percussion-hammer, or edge of the hand, will then cause the muscle to suddenly contract and throw out the limb. There are many conditions that might interfere w T ith the demon- stration of this phenomenon and of course they must be removed or given due consideration. The patient's mind may interfere. This is particularly the case in children. Hence distract their attention in some way or keep repeating the test while talking to them and in a moment FIGURE 3- Method of testing the patella reflex. of distraction it will suddenly appear. Disease states, in and about the related joint, in the muscles, and many other conditions, may interfere. As a general rule it may be stated that diminution or loss of the reflex means disease in the lower or peripheral motor neurones ; in- crease of it means disease in the upper or central neurones. In this way the deep reflexes become invaluable as localizing signs. In accordance with the muscle involved the symptom also helps us to localize the lesion in the segmental levels of the cord. The most important of the deep reflexes is that of the patella. A GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 59 child to be examined is placed on the edge of a chair ; an adult may sit upon a table. The legs are allowed to hang down at a right angle to the thigh. The examiner passes his arm under the knee of one leg and rests his hand upon the knee of the other. The patient is told to relax his muscles and to let his leg hang dead, as it were, and with full weight on the examiner's arm. I prefer this method to the common one of crossing one knee over the other in the ordinary sitting position. By the latter method there is sometimes considerable interference with the movement of the limb on account of the stoutness of the patient, the excessive tension put upon the tendon, or the occasional general incon- venience of the attitude. In the former method the patient can relax FIGURE 4. Method of testing the patella reflex with reinforcement. his muscles better, his mind is not centered upon the performance by the discomfort and the examiner can vary the tension of the tendon by slightly raising or lowering his arm. The tendon just below the front of the knee or just above it should be given a quick, sharp tap with the hammer or edge of the hand. The quadriceps femoris will then undergo a short, vigorous contraction and throw the foot out. If the patient ilexes and grips to- gether the fingers of his two hands, and then keeps trying to pull them apart, or if a child is given a small object to squeeze in either hand, the reflex response will be rendered considerably more distinct. This is what is known as reinforcement. 6o GENERAL CONSIDERATIONS UPON NERVOUS DISEASES Apparatuses have been devised to measure the force of this reflex but they are too complicated for ordinary use and do not add much to the information when employed. The explanation of the phenomenon is that a genuine spinal reflex is present. The muscle — not the tendon and hence the error in the term tendon-reflex — is by the blow put into a state of sudden tension, FIGURE q. Method of testing the ankle reflexi myotatic irritability. An impulse rushes to and from the cord causing the muscle to undergo the sharp, quick contraction. Disease anywhere in the reflex arc therefore is indicated by diminution or loss of the jerk. I have once seen it absent in a woman who was apparently in perfect health and by reason of her early outdoor life enjoyed a vigor of physique above that of the average. Oppenheim says lie has known two or three such individuals. In a neuropath with various anomalies of developmentj be assumed that the absence o\ the knee-jerk was a GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 6l hereditary stigma degenerationis. These exceptional cases only prove the rule, that less of the knee-jerk means disease. Xext in importance among the deep reflexes is the ankle reflex and ankle clonus as it is called. The heel-phenomenon or Achilles ten- don reflex is constant in health and is well elicited by asking the pa- tient to kneel easily on a chair and then tapping the tendo Achilles. The -calf muscles contract and the foot is suddenly extended. This ankle reflex is much like the knee-jerk and is subject to the same general exceptions. The ankle clonus is a symptom of disease and never occurs in FIGURE 6. Mode of eliciting an ankle clonus. health. It can be elicited by suddenly or gradually putting the calf muscles under excessive tension. The patient sits down and extends his leg. The examiner grasps the foot and forcibly flexes it upon the leg. If clonus is present, it immediately begins to oscillate with rapid and regular and rather wide excursions. These oscillations usually number about eight or ten a second. They cease when the examiner lets go of the foot and the muscles are relaxed again. Though most of the authorities say that this is a phenomenon of the calf muscles, some recent investigations seem to point out quite definitely that it belongs only to the soleus and not to the gastrocnemius. This is in line with the fact long ago pointed out by Duchenne that in spite of their common insertion, these two muscles have distinct pur- poses. In hysteria and neurasthenia there is occasionally seen a slight, brief pseudoclonus. 62 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES A wrist reflex is obtained by stroking the tendons of the wrist when the hand of the patient is placed supine on the hand of the ex- aminer. A t^p on the styloid process of either the radius or ulna pro- duces the radial or ulnar periosteal reflex. Much more important than any of these among- the arm reflexes- is that of the triceps, which is elicited by letting the patient hang his forearm, while at right angles to his arm, loosely over the fingers of FIGURE 7- Method of testing the triceps reflex. the examiner and by tapping the tendon sharply just above the ole- cranon process. Bechterew has described a scapulo-humcral reflex. With the per- cussion-hammer taps are made along the entire inner edge of the shoulder-blade, and beneath the inner angle of the same. Especially marked is the response when the stroke falls on the inner edge of the scapula near the inferior angle. There is adduction and slight outward lotation of the corresponding humerus. Both a jaw reflex and a jaw clonus are observed. With the mouth held loosely open, the muscles relaxed as much as possible, a pencil* small ruler, or paper cutter is placed transversely on the lower teeth GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 63 and smartly tapped. The muscles of mastication contract, especially the masseters ; the jaw leaps upward. In disease when forcibly de- pressed a clonus or rhythmical up-and-down movement takes place in it. Among these deep reflexes must be included the pupillary and ac- commodation reflexes of the eye. A bright light focused into the eye causes the pupil to contract. If the patient is asked to gaze at some distant object and then at another object brought to his usual reading FIGURE 8. Method of testing the scapulo-humeral reflex. distance, or if he is asked to keep his gaze fixed on the examiner's finger as the latter is gradually carried towards his nose, the pupils will be seen to contract. Where the former or light reflex is lost, while the latter or accommodation reflex is retained, the pupil is referred to as that of the Argyll-Robertson type. The various visceral reflexes are of such familiarity tint scarcely a word need be said of them here. The vasomotor and secretory reflexes are visceral in a sense. Nausea and vomiting caused by rapidly re- volving objects before the eyes are of the same nature. The heart, the stomach, the liver, the kidneys, the rectum and bladder may all be affected by some form of remote sensory disturbance. We do not make use of these reflexes to -any extent in diagnosis. Idiopathic reflex irritability of the muscles is seen in neurasthenic 6 4 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES and many other states. Diminution and increase of irritability are both observed. Percussion of the muscle awakens contractions that are lively and enduring, for a time. The same is observed when elec- tricity is the stimulant. In degenerative states both the mechanical and electrical irritability are usually reduced. When a muscle is suddenly relaxed and undergoes a slow tonic contraction, Westphal spoke of the condition as a paradoxical muscular FIGURE 9. Showing the mode of tt the jaw re Ilex contraction. It is best observed in the tibialis anticus when the foot is suddenly flexed on the leg. The mechanism is not known, nor arc we acquainted with its full significance. It is a rare phenomenon and never occurs in health. A somewhat similar exhibition has been seen in hysteria Electro-diagnosis in neurology is much more satisfactory than electro-therapy, in the fact that it is much more definite and accurate, And yet it is less resorted to than it should be, partly I suspect from the GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 65 fright produced in the minds of some by the vast array of symbols and formulae presented in certain works upon the subject. Neither the apparatus nor the principles upon which electro-diagnosis depends, are at all complicated. All that is needed in the way of proper paraphernalia are a good faradic battery, a good galvanic battery capable of furnishing a current of thirty or more milliamperes, a galvanometer for accurate measure- ments, though for general examinations even this is not necessary, good conduction cords, a rheostat and a number of electrodes of vari- ous sizes, in the handle of one of which is conveniently arranged a cur- rent interrupter. As time and space will be saved by the use of symbols, the follow- ing definitions are in order here. An will be used for the anode, or positive pole of the battery ; Ca will be made to stand for the cathode ; C is employed to denote a contraction of the muscle. CaCC in electro-diagnostic parlance means cathodal closing con- traction, which interpreted further means that, the anode being on some indifferent part of the body, when the negative pole is placed over the nerve or muscle to be examined there is a muscular contraction. In a similar manner CaOC is the formula for the contraction that would take place upon the opening of the current by removal of the negative electrode. AnCC stands for anodal closure contraction and AnOC for anodal opening contraction. To still further facilitate matters T is used to represent a strong continuous or tenatic contraction in the muscle, DeR the reaction of degeneration, and D the closed circuit or flowing current. Every examination for abnormality presupposes a knowledge of the normal. The same is true in electro-diagnosis. The normal polar formulae read thus : i. CaCC; 2- j InOC ; 3 ' CaOC ' These are varied and even reversed in disease. Moreover certain conditions must be observed and certain sources of error must be carefully avoided. Adults are more easily examined than children. In very early life, especially in the newborn, the skin, which is always highly resist- ent to the current is particularly so, and the peripheral nerves and muscles are incompletely developed. Adult results in electrical exami- nation are not observed in children until they are well along toward their fifth year. The resistance of the skin is overcome by moistening it and the electrodes freely with water in which a modicum of salt has been dis- solved. The passage of the current itself by stimulating the glands anil increasing the moisture of the skin in a few minutes increases the latter's conductibility. If one side of the body is diseased and to be examined, the other side should be simultaneously tested as a control. When both sides are affected resort must be had, unsatisfactory as it is, to a third per- son for the control tests. 66 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES In adults, and especially in children, great care must be exercised not to confound voluntary and reflex movements with those produced by the electric current. The distinction can be detected by close watchfulness and frequent repetition of the tests. Allowances must be made for variations in the size of the electrodes used, the current being dense in direct proportion to its strength and in inverse propor- tion to the diameter of the electrode, and for such natural conditions as over-development of fat, etc. The limb to be examined should always be so placed that the FIGURE 10. (After Erb.) 11. frontalis Oberer Facialisast U. coring, anpercil. M. orbic palpebr. Nasenniuskeln M. zygomatic! M. orbicul. oris Mittler. Facialisast M. masseter M. levator menti M. qnadr. meuli M. triang. menti Nerv. hypogloss. Unter Facialisast M.platysma myoid. Znngenbein muskeln 11. omobyoideus N . thoracic, anter. til. pector.) W. pbreaicu Snpraclaviculax- pankt. (trb'scher Pnnkt. M. deltoid., biceps, bract ml. in- tern, n sopinaW long) Tlexu* bra Gegend d»r CeotralwioduBgco Gegeml d. 3. Stirn- windung n. Insel (Sprachcentiumt M. temporali* Ober. Faciatisast vor dem Obr N. facialis Stauiro) N. auricul. post Mitll. Facialisast Vnter. Facialisast M splemns M. sternocleido- mastoideus N. accessonus M. levator auguli' scapul M cucalaris N. dors, scapulae N. thoracic, long. (M. serratns aotic maj) effect of the stimulation can be easily manifested. A large electrode is thoroughly moistened and placed firmly against the lower part of the sternum. It is not to be moved during the entire examination. The other electrode, the small one with the interrupting attachment, is pressed firmly down, after having also been thoroughly moistened, on the nerve or muscle to be tested. The accompanying illustrations indicate the most favorable points at which to place this electrode to obtain the clearest responses from the respective nerves and muscles under examination. A weak current is employed first and then made stronger until there is a beginning twitch in the muscle. That indicates that the re- GEXERAL CONSIDERATIONS UPON NERVOUS DISEASES 67 quired strength of current has been attained. Each nerve of the limb should be examined above and below, using the points in the illustra- tions as guides. The first great fact to note in regard to the use of electricity for diagnosis is that it applies only to the lower or peripheral neurones. We can get no information of the condition of the upper or central neurones by the aid of electricity. Moreover, in regard to sensation it FIGURE 11. (After Erb.) If. tncepi (caput Ion gum) tt. triceps v caput intern ) A7r». ulnar ill M. fiexoi carpi olnaris M Sex. digitor. commun. profnnd. M. lex. dkrltor »oblira. M ilex. diglu»»bl (digit. indicii et minimi) Htn. utnaru M p.ilmaris bre? M. abductor digiti min M. flexor digit, inin M. oppoaene digit, min atst. lumbricalesJ H. deltoldeos (vord. Halfte) M. Uic9p» bracUii H. bracb. interuus M. supinator longns at. pronator teres at. flex, carpi radiaiU If. flex- digitor. eubliia. M. flex, pollicls longus Nerw. mtdianut M. abductor pollic. brer. M. opponene poUicla M. flex. poll. brer. JO, adductor pollic can afford us but very little knowledge that is at all definite enough to be of service. ' Therefore, electro-diagnosis is pre-eminently con- cerned with giving us information as to the condition of the lower motor neurones. In all diseases of the cord, the roots, and the nerves in which these neurones are implicated, electricity furnishes most valu- able diagnostic criteria. Let us now consider what are the normal and abnormal exhibi- 68 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES tions. We have many distinct points of view from which to conduct the examination and mark the differences in the results. In the first place it should be noted whether the responses to any form of current are greater or less than normal. This is what is called the quantitative test. It is easily made and not of very great value in diagnosis. Mere increase or diminution of nervous irritability and muscular excitability, as we have already seen under the head of the reflexes, can be determined by mechanical means almost as well as by electrical stimulation. However, it is always wise to make the quan- F1GURE 12. (After Erb.) N. deltoideos (hinterr" HilfU) If. radialis M brachial, intern. M. supinator long 11 radial, eit. long. M. radial, est. bre». it. extensor digit, f communis \ 11. extenaor indicia M abductor pollic long. M. extensor pollic brer. U.lotero.i. dorsal. I JL trlcepi (caput longnm) liLtricepi (caput extern.) M. ulnar, extern, al. supinat. brer. M. extend, digiti M. eaten*, indicia M. extern. polL long. M. abdnrt. digit, mlw 1 M. lntarojs. dormJ* I HI et IT titative tests and to record them with the galvanometer readings. Of course care must be observed to use always the same sized electrode, usually a small one, and to remember that normally different nerves and muscles differ in their quantitative responses. Stintzing used an electrode with an area of 3 sq. cm. Neglecting the muscle averages as of less importance, he found that for the nerves the averages were as follows, the galvanic being given in milliamperes and the faradic in centimeters on the scale attached to the sliding coil of his battery : GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 69 Galvanic. Faradic. Ma. Cm. Facial nerve 1.75 121 Frontal branch 1.45 128 Mental branch 0.95 132 Accessory nerve 0.27 137 Median nerve (in arm) 0.9 122 Ulnar nerve 0.55 130 Musculospiral nerve 1.8 105 Crural nerve 1.05 1 1 1 Peroneal nerve 1. 1 115 FIGURE 13. (After Erb.) M. adductor mtgnoj U. adduct longai V. thIm ioUrnu M. tensor fwciM l«tM it. qaidricips femorU (gemeinscbafU. Pankl) M recUt femoru U. tutus external Muscles vary more widely even than do the nerves. Even if we were to duplicate Stintzing's apparatus and methods in every particular, there are so many sources of error in the conditions of the skin, etc., in different people, that only wide variations from the above comparative figures can be taken as evidence of a pathological condition. Diminution in quantitative excitability is much more common, definite and available for purposes of diagnosis than is increase. In only one disease, tetany, does increase of excitability to the galvanic current figure prominently as a symptom. Much more definite and reliable are the qualitative changes that occur in the electrical responses as the result of disease. Here we have to consider the nature of the current, faradic or galvanic, em- ployed in the examination; the strength of the current; and the tissue undergoing the test, whether it is nervous or muscular. When for any reason a muscle or its nerve is severed from its 70 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES nutrient center in the cell embedded in the anterior horn of the cord or cranial nucleus, it undergoes degeneration. This causes a change in the electrical responses. The result is spoken of as the reaction of de- generation and symbolized by ReD. It is obvious, therefore, that re- action of degeneration appears as a symptom in a great variety of diseases affecting the cells in the anterior horns of the cord and nuclei of the cranial nerves, the neuraxones belonging to those cells, and the muscles to which the neuraxones are attached. In such affections as poliomyelitis, the progressive atrophies of spinal origin, other diseases that damage the anterior cornual cells, bulbar nuclear paralyses, dis- eases that by compression or otherwise injure the anterior spinal roots, FIGURE 14. (After Erb.) I. tibial, antic ILextens. digit, comm. long. M. peronMaa breri* U extensor QiUociB long. Bm. InWiMMl donalM Ntrv. ptronaeut If. gestrocnem. extern. M. peromelia longu K. flexor htJlweU long. tt. extent, digit coma brent M. ebdnetor dlgiti min. and all severe degenerative diseases of the peripheral nerves like the infectious and toxic polyneuritides, ReD is a most important symptom. It never occurs as a sign of any affection that remains limited to the cerebral, upper or central motor neurones. In differentiating the two sets of paralyses therefore its value is obvious. There are various degrees of degeneration and the value of the symptom is furthermore emphasized in the remarkable fact that there is a direct parallelism between it and the degree of neuro-muscular degeneration. As the latter doesn't occur until about a week after the damage is first done, the electrical symptom will not be elicited be- fore that time. The immediate cause of the reaction of degeneration is the fact that the degenerated nerve terminals in among the muscle fibres re- quire a stronger and longer current than normal to excite them. GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 71 As a very late phenomenon the same electrical manifestations may obtain in pure muscular affections wherein the muscular fibres are wasted. The reaction of degeneration reveals itself in two ways. There is a gradual loss of contractility to stimulation, and there is an actual change or reversal of the polar responses to galvanic stimulation. If the faradic current is employed, and first the nerve and then the mus- cle tested, it will be noticed that there is a gradual diminution of the rapidity and force of the contraction. In other words a stronger cur- FIGURE 15. (After Erb.) Hero ischiadic** K. bicepi fern. (cap. long.) K. bicepi fan. (cap. brer.) N prrontut al. gastrocnem. (cap. extern.) M. nuor'hallicU lOSgV f M. gluueus maxima* M. adductor magnoa M semiteadioosas SI. semimembranosus M. gulrocnem (cap. lot) M.. neior digitot. coma tongas rent of electricity will have to be employed to get a contraction anything near like that of the normal. Finally as the degeneration advances, no contraction whatever can be obtained, no matter what be the strength of the current that is tolerable. If regeneration occurs there is a re- turn of the faradic response in both the nerve and the muscle in the reversed order in which it disappeared. When the galvanic current is used and the nerve is tested the phenomena are about the same as they are with the faradic stimula- tion. In other words the galvanic excitability of the nerve is lost. If now the muscle be tested with the galvanic current, there is for a time a remarkable increase in the contractions or what is the same thing a weaker current is merely needed to awaken the normal contraction. 72. GENERAL CONSIDERATIONS UPON NERVOUS DISEASES As the degeneration advances, finally the galvanic current loses its power of stimulation and then differs in no respect from the faradic. In regeneration of the nerve exactly the reverse order of events takes place until the normal status is reached upon the complete restoration to health. The following diagram will perhaps illustrate the characteristic features of the reaction of degeneration better than the above detailed description. It is taken from Erb to whom we owe much of our knowledge along this line. There is a change in the character of the polar responses also to galvanic stimulation of the muscles in the reaction of degeneration. The contractions are slow and worm-like instead of being sharp and lightning-like upon the closing and opening of the circuit. Moreover the normal superiority of the cathodal closing response over that of the anodal closing is reversed and the formula reads AnCC>CaCC. Even the cathodal opening contraction undergoes a greater in- crease than, and in very exceptional instances may even become equal to or stronger than, the anodal opening contraction. That these polar alterations are not as important reactionary signs of degeneration as are the differential phenomena between the faradic and galvanic re- sponses described above, is shown by the fact that we can even speak of the condition as a reaction of degeneration when the CaCC is equal to or exceeds still the AnCC provided the contraction is slug- gish in character. In some cases of partial reaction of degeneration there is a decrease of nerve and muscle irritability to both currents, whereas with direct stimulation of the muscle with the galvanic current, a slow contraction and reversal of the polar responses still obtain. The phenomena of the reaction of degeneration may be conve- niently summed up thus: 1. Loss of nerve irritability to the faradic current. 2. Loss of muscle irritability to the faradic current. 3. Loss of nerve irritability to the galvanic current. 4. (a) Early increase of muscle irritability to the galvanic cur- rent with later decrease, (b) Modification of the polar responses, even to the degree of complete reversal from those of the normal, the formula becoming AnCC equal to or greater than CaCC, with slug- gishness of the contraction as the most important feature of all. It may be mentioned that electricity is one of our best aids in detecting the feigning of disease, whether sensory or motor in type, and by it we can distinguish hysterical or pseudo-paralysis from the lower neurone types of organic paralysis. Consult the accompanying diagram for the best points to apply the poles. The vasomotor, secretory and trophic symptoms of nervous dis- eases call for but the briefest notice here as they are so generally local- ized in character that they can be better treated of under the head of brain and cord manifestations. The vasomotor and secretory phenomena are very closely inter- related and are supposed to be largely under the control of the sympa< thetic system. Flushing and pallor of the skin and mucous membranes, local and GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 73 -W-ft- — 1 pa n fU a. < n o -t !-+> n> j. a z a *> O O O 3 O <"D 3 oq P QTQ < ? i /,' )'r •Q- V m m N •^ NQ 74 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES general, may be frequently observed. Sometimes they can be elicited by causing a mental shock to the patient or by irritating the skin. Dermographia is where one can write with the back of his nail on the patient's skin and the tracing remains a long time. The tdche bleuatre of the French authors is an unimportant vasomotor manifes- tation in the skin. Any of the secretions may be increased or diminished. This is usually due to associated vasomotor trouble. Special names are em- ployed for the various forms and localizations of the secretory exhibi- tions. They are such as hyperidrosis and anidrosis for disturbances of the perspiration ; salivation for increase of the saliva ; diuresis for excessive flow of urine. The trophic symptoms are usually due to spinal disease and will be described at the head of those troubles. True atrophies must be carefully distinguished from pseudo-atrophies due to non-use. True hypertrophies and pseudo-hypertrophies must be carefully differen- tiated. The former must not be confounded with enlargements from other adventitious causes of enlargement. A hypertrophic joint for instance is not the same as a swollen and ©edematous joint. Trophic disturbances can be observed in any or all of the tis- sues of the body but they are extremely obvious at times in the epi- blastic elements such as the skin, the hair, the nails, etc. Ulcerations, pigmentations and many other changes are observed in the skin as a result of its diseased innervation. It is beginning to be recognized that many skin affections are really nervous troubles. Herpes, urticaria, scleroderma, pemphigus are neurotic in origin. General trophic diseases like arthritis deformans are, in some cases at least, of the nature of trophoneuroses. Just how the nervous centers preside over the nutrition of the body we do not clearly know ; whether their influence is direct or indirect, whether it is physical or chemical. We do know, however, that most important trophic centers are located in the gray matter of the spinal cord and that they oper- ate upon the tissues largely through the sympathetic and spinal nerves, together or separately. Trophic symptoms need no tests as they are wholly a matter of observation. The trophic changes in the muscles are accompanied by the peculiar phenomena described under the reaction of degeneration in the paragraphs devoted to electro-diagnosis. Trophic skin changes are less responsive to local medication than are those due to inflam- matory troubles, and this sometimes aids in their diagnosis. The visceral symptoms of neurology are motor, sensory, secretory or trophic in character and may therefore be passed here. They arc important, however, and should always be carefully noted in the ex- amination of every case. Local thermometry is sometimes a valuable assistant in diagnosis. The vasomotor condition, inflammatory or spasmodic, usually under- lies it. Thermometers of various types have been devised for resting against the head and elsewhere. Tumors and abscesses of the brain and focal inflammations have sometimes revealed spots of slightly altered temperature in the scalp. The same has been less frequently GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 75 observed in severe, focal cord troubles. The findings, however, are not sufficiently uniform or definite to make them very satisfactory as diagnostic criteria. General thermometry is the same in nervous as in other forms of infection and inflammation. The pulse may be due to a vasomotor disturbance ; otherwise it follows the same laws that it does in other pathological states of the .general organism. Skiagraphy, or shadow pictures made with the Rontgen ray, has added a most valuable aid to the diagnosis of some superficial and even deep lesions in the nervous system. Bullets and other missiles have been located by this method. Exostoses, spiculse, depressions and fractures in the inner table of the skull have been thus discovered when there were no external signs of any sort. Many tumors and growths can, if not too deeply buried, be thus outlined. Cortical growths are especially discoverable by skiagraphy ; basal growths are perhaps the least so. The examination of the cerebrospinal fluid and the value of the findings will be referred to again under the head of lumbar puncture in the chapter given to the anatomy of the spinal cord. GENERAL CONSIDERATIONS UPON THE TREATMENT OF DISEASES OF THE NERVOUS SYSTEM. I hesitate very much to write the following chapter, because liter- ally there are no general principles of treatment that apply to all pa- tients alike. Human beings are not automata nor machines built upon exactly the same plan and with the same measurements. Each in- dividual has his own peculiarities, so that it becomes literally true what is one man's meat is another man's poison. In the nervous system, wherein resides the very center of the individual's personality, the constitutional and functional differences between men are more marked than they are in the other organs of the body. The nervous system is the immediate source of all human activities. From the creation of a Paradise Lost to the sweeping of a public highway, the nervous system is the beginning and source of the phenomena. All other organs de- velop and functionate nearly in the same way in all men. but in regard to their nervous functions the differences are enormous. It is the fail- ure to recognize this fact that leads to so much dissatisfaction in the management of these affections. The greater the need of individualiz- ing in any kind of work the more patience, thought and concentration is demanded of the worker. It is much easier to think mechanically and machine-like, to have a single formula by which to treat all hys- terias alike and another by which to label always certain general groups of symptoms by a given name. It saves time and the expendi- ture of mental force. But, alas! it is usually disastrous to the patient's welfare and to the physician's reputation. Under such circumstances the former usually turns to quackery, while the latter rails at the hor- r< rs of nervous diseases. It is notorious that in general practice pessimism in regard to the j6 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES prognosis of these affections, and nihilism in regard to their treatment are all too common. The reason for this is often too obviously in the temperamental and mental inadequacy of the practitioner. He either lacks the proper amount of patience, his trend of thought is too mechan- ical, he is mentally lazy or he is ill-informed in regard to the newer discoveries in neurology. I was recently asked by a gentleman of large practice what a neurone was ; another informed me that hysteria was nothing but devilishness in the woman ; a third wanted to> know what I usually prescribed for paralysis ; while a fourth emphatically declared there was no such thing as neurasthenia. Such exhibitions make one feel that there are worse things than nervous diseases, and chief among them are the dogmatism of ignor- ance and the assumption of egotism. On the average the prognosis and treatment of nervous diseases- are about the same as they are in other troubles. All of the self- limited infections are beyond the pale of cure in the sense of ceasing at the behest of the physician. The latter merely controls their symp- toms, wards off complications and helplessly lets them run their course and terminate themselves. We can do pretty nearly as much as that in locomotor ataxia and a great deal more in brain tumor. If hys- teria is incurable, and can only be controlled when it breaks out in exacerbations, as it were, what are we to say of the rheumatic diathe- sis, of gout, of diabetes and the other constitutional diseases? Is val- vular disease of the heart ever cured, or advanced pulmonary tuber- culosis, or certain forms of renal disease ? As among other affections so among the nervous, we have the in- herited, congenital and constitutional troubles that can only be con- trolled and treated symptomatica! ly, whose course is chronic, and whose termination is only with death. On the other hand, there are many acquired diseases, like chorea, neurasthenia, syphilis of the central nervous system, multiple neuritis, that give most brilliant results under proper treatment. A pessimistic view of neurology is unwarranted and exposes the holder of it to the charge of being too badly informed to make an ap- proximately correct diagnosis, too inappreciative of the close relation- ship of the nervous system to the personality of the patient, too gross and mechanical in his mode of thinking, too empirical in the use of his remedial agents, and too oblivious to the important distinction be- tween inherited and congenital unalterable disease states and alterable and acquired temporary states. The purpose of this chapter will be to point out a few general principles of neurological therapy in order to more particularly empha- size the unreasonableness of therapeutic nihilism. It is most assuredly not written to enable any one to concoct a fixed scheme of treatment for all cases of nervous disease alike and thus to shirk the labor and responsibility of studying and managing each individual case upon its own merits. There are three prominent indications in the treatment of nervous disease. GENERAL CONSIDERATIONS UPON NERVOUS DISEASES JJ In the first place, the prevention of the disease itself, its return, or its exacerbation should always be attempted by the aid of proper hygienic and prophylactic measures. In the second place, every endeavor should be made to remove or render inactive the cause. Thirdly, the symptoms should be controlled in the hope of thereby removing their cause, or at least of mitigating the distress produced by them. In striving after these various ends we find that we have at our disposal various classes of agents. They are the psychic, the physical and the medicinal. I include surgery among the physical measures. Psychic, physical and even medicinal means may have to be em- ployed in the prophylaxis. The putting of an individual into the best condition to ward off disease means the putting of him into the very best mental and physical hygienic state. Mind and body must be trained to make for health and not for disease. Good physical and mental food is a necessity. Proper and varied exercise is a prerequisite. Cleanliness, system, regularity, moderation, serenity and freedom from irritation and exhaustion are desiderata. Ignorance of the laws of health is not so widespread as indifference to them. Ignorance in re- gard to the dangers of their infraction is the cause of much of this indifference. It becomes the duty of the physician, therefore, to warn as well as to instruct, to arouse by awakening alarm as well as to calm by conveying correct information. In doing thus his duty he may have to touch upon every minutia in the diet, the clothing, the habits, the occupations, the amusements, the mentalization even of his protege. Nothing is too insignificant in the prophylaxis of nervous diseases. Much of the advice w T hich he is to give is commonplace and well known, but people are thoughtless and forgetful and he has assumed the role of conservator of their health ; it behooves him, therefore, to iterate and reiterate his injunctions, however trite they may seem to be. One of the most important questions, though unfortunately not one of the most frequent, that he will be consulted upon is that of marriage and the possible effects of heredity. In no branch of medi- cine is heredity so prominent a factor as in neurology. Heredity is a tremendous feature in etiology. In the prevention of these troubles it must receive high consideration. If not for their own good, at least for the good of society and future generations, the unfit should not marry. It would lead me too far to mention all the forms of nervous disease that ought to restrain those contemplating marriage from that which will most assuredly give to the world more defective and more diseased offspring. The intermarriage of near blood rela- tions should be interdicted. Two nervously afflicted individuals should not enter into wedlock. An individual who has a form of nervous trouble in which heredity is recognized as a marked element ought not to beget children. Syphilitics should not marry. Members of fami- lies in which there is a clear psychopathic taint would not marry if they considered the welfare of others besides themselves. Members of families in which there is a traceable neuropathic taint should be slow about contracting union, or if they do marry they should unite in a yS GENERAL CONSIDERATIONS UPON NERVOUS DISEASES family where there is a history of exceptional health and vigor for several generations back. The children of a neuropathic ancestry need close observation, but coddling should be most strenuously guard- ed against. These children are ofttimes unfortunate in being the vic- tims of both a bad heredity and a bad discipline. They are sometimes brilliant, precocious, entertaining and physically weak. They are the center of the family interest and sympathy. They are therefore over- indulged, injudiciously humored and spoilt. Their physical and mental lives are all but riotous. They live as they please, eat as they please and think as they please. The consequence is inevitable. On the other hand, I sometimes wonder if it is not a greater evil to be the child of self-opinionated, narrow-minded, ignorant disciplinarians. I am con- vinced that in too many cases not neglect and overindulgence are the bane of these children so much as too much regulation — nagging, in fact — by anxious and loving but woefully stupid, ill-informed and short-sighted parents. I have frequently seen young neurotic girls driven almost insane by the imperious attentions of a mother who meant well, but who was incapable of seeing things from her daugh- ter's point of view. A school for parents would be a godsend for many- neuropathic children and its curriculum would include training in what not to do as well as in what to do. These children of neuropathic parentage need, as a rule, sys- tematic, wholesome, evenly balanced lives. All shocks, unusual or one-sided strains, freakish habits, intemperance in thinking, in eating, in drinking and in everything should be prevented. A steady moral purpose in life, a wholesome ambition in some one direction and a good regime in living should be inculcated. Nerves are marvelously built up and kept strong by systematized and regular mental and physi- cal activity. The same applies to adults as to these children. Dissipation, irregularity, extremes are disastrous to the health of the nervous tis- sues. Proper exercise of the entire personality, mental and physical, in a manner conducive to a well-balanced organism is a necessity. It is more solacing, but less truthful, to say that hard work is the cause of much modern nervousness than it is to say it is due to the distractions, dissipations, fuss and fume that are allowed to accompany the hard work. Hard work may injure its hundreds, but excessive, purposeless motility, mental flightiness, silly rush and roar, false and foolish identifying of mere mental and physical bombast with real ac- complishment injures its thousands. The use of alcoholics, tobacco and noxious beverages, the diet and the mode of eating it, the frequency and the style of bathing, the hours of sleeping, the whole life of the adult must be inquired minutely into, and proper suggestions made along well-known lines to insure against the outbreak of a possible or threatened nervous affection. The dietetics of nervous diseases has its greatest field of operation in neurasthenic and allied states. As I have treated of the subject in detail under the head of neurasthenia, I will not speak of it further here. It should be noted, however, that for neuropaths generally a wholesome nitrogenous diet, with the drinking of abundance of water GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 79 between meals, is to be employed. It includes meats, fowl, fish, eggs, milk, buttermilk, green vegetables, bread and butter. As a rule I find that neurotics do not eat enough or too much of some particular sort of food. Especially fats do they seem to eschew. They overindulge in stimulants, tea and coffee. I am convinced that in these cases it is not so important to advise a selected diet as it is to urge a generous one. Whims and notions often confront the advice of the physician, and if he pays much attention to them he will have to change the dietary every few days. I therefore try to discover, after a short period of observation, about what agrees best with the patient apart from any notions that he may himself have. I then urge that this food be taken in large amount, in several small meals a day if necessary at first. Even less digestible food is better for neurotics than little or no food. The stomach can always be assisted in the disposing of it with proper agents. Food, then, the best, the plainest and the most wholesome if possi- ble, but always food, more food, should be the guiding suggestion in regard to the dietary of most nervous people. The exceptions are relatively few and capable of being easily met. Athletes are not, as a rule, good brain workers, nor, on the other hand, is it necessary for people whose nerves are under a high tension to strive to become athletes in order to preserve health. It is amazing the amount of ignorance there is among the laity in regard to the mat- ter of physical exercise. Unused muscles, of course, with an over- worked brain produce an unbalanced organism. A certain amount of exercise, therefore, is absolutely imperative. Among real brain workers there is not enough exercise taken, as a rule, and among a large class of pseudo-brain-workers there is entirely too much exercise indulged in. No exercise is of much benefit that fails at the same time to inter- est the mind. To wearily stand and swing dumb-bells or Indian clubs in a close, stuffy gymnasium in the hope of gaining health is almost ludicrous. Long forced walks in which one is longing all the while to return home are not conducive to health. Such exercises merely weary the muscles and depress the mind. It is my observation that in a good- ly proportion of cases too much mere physical exercise is taken. I am often consulted by persons who are on the verge of profound nervous collapse and yet who wearily force themselves every day to take a cold plunge bath and a monotonous measured walk. What such per- sons need is usually rest and less task-work ; in a word, very moderate exercise with plenty of amusement. And this leads me to state another point I also believe to be true, namely, that many business men who are monotonously confined to their offices all day do not need physical outdoor exercise so much as they need relaxation and change of brain activity. The grind of busi- ness develops but one side of their mental faculties. They need, there- fore, psychic change even more than physical change. All sorts of outdoor games and sports are commendable therefore. Moderation should be the rule always even here. The bath is a powerful agent for good or for evil, hence the ground for the extremists who on the one hand spend half their lives in water, 80 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES or on the other indulge in the luxury once in two or three years. Here as everywhere moderation and good judgment are necessary. Cleanliness is, of course, next to godliness and should be a law ; but except under some special conditions -it is not necessary to spend the greater part of one's existence in the bathtub to keep clean. I am of the opinion that many people bathe overmuch as well as that many bathe not enough. Here again each one must be a law unto himself. As the bath is, by reason of its delightful after-effects, a sort of luxury as well as necessity, it can easily be overindulged in. Human beings are not water animals. I know of instances where the frequent and prolonged immersion of the body in water, the over- stimulation and excitement induced in a delicate organism by too fre- quent cold plunges, the tenderness and enervation fostered by too much employment of the hot water, and above all by too frequent rasping of the skin with harsh brushes, rough towels and dirty soaps, have done positive harm, though the practice had been persisted in because it gave a temporary pleasant feeling and held out a fatuous hope of increased health. Therefore, while a daily bath is to be commended, it is to be adjusted to the individual. His constitutional needs, his power of reaction and his general vigor must be taken into account when the questions are raised as to the frequency of the bath, the proper tem- perature of the water, the duration of the immersion and the various after procedures. Remember, too much and injudicious bathing are as harmful as too little or none at all. When a physician is consulted by an individual who already mani- fests the symptoms of a definite nervous trouble, it becomes his first duty to search for and remove, if possible, the cause. This may tax his ingenuity to the utmost and call upon all of his mediate and immediate resources. All of the therapeutic forces may have to be brought into play here to banish the cause of the disease, as well as the disease itself. Psychic, disciplinary and pedagogical measures can remove a false mentalization which underlies some cases of the psychosis hys- teria. A calming manner, an assurance that everything is all right, an assistance on the part of the physician in banishing trifling worries and at times an autocratic severity with positive commands, will do more than bottles of medicine. A weak personality, a hesitancy and uncertainty begotten of want of knowledge, or an exposure of the fact on the part of the attendant that he is not perfectly master of the sit- uation will be sure to heap trouble upon trouble for him and will send the patient's symptoms into an exalted degree of manifestation. It is the difference between doctors themselves in this respect that often causes the apparent differences between patients in regard to their responsiveness to treatment. This is so strikingly true that it under- lies the wide variations of opinion among medical men in regard to nervous diseases and their management. It is so true that a practitioner who finds he is mentally set all awry and meets with constant discour- agement in his neurological practice, whose cases go to his rivals and remain under their treatment and even get well, will do wisely to blame himself and endeavor to find out and correct his own shortcomings. Change of environment, change of companionship, change of GENERAL CONSIDERATIONS UPON NERVOUS DISEASES Si thought, suggestion, and even occasionally hypnotism, may have to be resorted to in order to lift the patient out of the mental state upon which his psychosis and neurosis may largely depend. The physical causes of nervous diseases are so numerous and the methods of attempting their removal are so commonplace that noth- ing but a bare mention of them need be made here. There are the intoxications, for instance, such as alcohol, arsenic, lead, morphine, cocaine; the infections, like syphilis, tuberculosis, pneumonia, malaria, influenza. These will all have to be attended to and removed before their nervous sequelae can be expected to mitigate. Surgical treatment will be called for in removing distant sources of infection, like abscesses. Tumors and deformities and exostoses press- ing upon the nervous tissues will demand the first consideration. I have seen simple lateral curvature uf the spine cause a severe hystero- neurasthenic condition. The dependence of Jacksonian epilepsy upon injuries to the skull is well recognized in some cases. The origin of intracranial abscess from mastoid and ear disease is all but universal. Many nervous diseases need no further treatment than simple re- moval of the cause. It is positive malpractice to go on plying the pa- tient with analgesic and antispasmodic remedies without attempting to discover and remove the cause. Only when the latter is absolutely impossible is symptomatic treatment justifiable. The symptomatic therapy as applied to nervous diseases involves psychic, physical and medicinal measures. I have already referred sufficiently to the general psychic influ- ences, which are the same here as they are when employed in the pre- vention of disease. Suggestion and hypnotism will be treated of more in detail in another part of the volume. I will mention here, however, that psychotherapy is of wide application and includes much more than is usually credited to it. I do not refer to direct psychotherapy, for that is acknowledged by every one to be a powerful weapon against diseases of a certain order. AYhat I mean to say is that even indirect psychotherapy is frequently quite as forceful in accomplishing good. I am convinced that not a little of the benefit obtained by both physical measures and drugs is due in some cases to the change of mentaliza- tion which they indirectly induce in the patient. Metallotherapy, elec- tricity, massage, mechanical exercises, baths are almost as efficient in affecting the patient's mind, in awakening new hopes, in dispelling despair, apart from their mere physical effects, as is music or change of companionship or general environment. In fact, they constitute a part of the change of environment and as novelties cause a novel train of thought. They are all legitimate means to use, in properly selected cases, for their psychic as well as for their physical influence. The physical agents at our disposal include those of hydrotherapy, me chanic other apy, electrotherapy, massage, gymnastics and surgery. To treat exhaustively of any one of these would take more space than can be allotted here and the reader is therefore referred t<> the numer- ous special treatises upon them. Some few general principles may not be improperly enunciated, however, at the beginning of a volume upon nervous disea 82 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES There are many kinds of baths in vogue to-day for the treatment of disease, as, for instance, the water, the electric and the light baths. Even mud, paradoxical as it sounds, is now the constituent of a bath. Hydrotherapy means the employment of hot and cold water to stimulate, quiet and otherwise modify the functions of the nervous elements. The ice bag is to be included, as that is a sort of local appli- cation of cold hydrotherapy. There is much refinement, some of it unnecessary in my opinion, in the division and subdivision of baths in works upon hydrotherapy. Two great forces underlie all forms of the therapeutic use of water in this way. One is the thermal force, the other is the mechanical. To be sure, they are practically both the same, for thermal force is believed now to be due to the mechanical activity of molecules. The modes of applying hydrotherapy involve the consideration of the application to and the abstraction from the body of heat, and stimu- lating, benumbing and other effects produced upon it by the weight, force and shock of the water. As a general rule cold baths, however applied, are stimulating. If too long in duration or if the patient's reactive powers are weak, stimulation passes over into exhaustion. Cold baths are not tonic in the sense that they are frequently supposed to be. If brief and employed in persons of vigorous constitution, they first contract then dilate the peripheral vascular apparatus. This sends the blood bounding through the body and hastens all the vital processes ; hence the exhilaration that usually succeeds a plunge into a tub of water at 60 or 70 degrees F., followed by a brisk rub. In a way a bath of this sort is like all other stimulants, even alcohol. It is not a tonic per se. It uses up quickly pabulum, it heightens all of the tissue ac- tivities, it excites into exalted power the vital organs, it enlivens the whole being even as alcohol does. The value of such an agent is incalculable, but, like a double-edged sword, it can cut both ways. It is pitiful to see how often nervous sys- tems that are worn out and in need of rest, that are crying loudly for more nourishment and less stimulation, are whipped up by all sorts of irrational methods of cold bathing. Great harm has been done, I am certain, by the promiscuous use of the cold bath, and all of its variations, in neurasthenia and other states of depression in which malnutrition is a prime factor. The mechanical effect of the water is added to that of the cold by applying the bath in the form of a shower, a solid jet (Charcot douche), a rain or a rapid spurting. Alternation of a hot and cold jet thrown against the body from a distance of ten or twelve feet constitutes the Scottish douche. Other modes of applying cold hydrotherapy are the wet sheet, the cold compress, the cold rub, the cold pack and the ice ba s- As a general rule all such applications, even the full or partial cold bath, should be brief and followed by thorough drying and toweling to provoke a reaction. Two per cent, of brine may be judiciously added to the water sometimes, thus simulating it to a natural sea bath. Many of the medicinal springs where baths are employed owe their virtue to the coldness of the water. GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 83 Extremely hot baths may be stimulating, but warm baths as a rule are sedative and tonic. The temperature of the water should be from 90 to 100 degrees F. Full baths, partial baths, sitz and foot baths, hot wet packs, hot compresses, sprays, douches, fomentations and hot water bottles are some of the ways in which heated water may be employed. In the Turkish bath the body is first exposed to a dry tem- perature anywhere from 130 to 200 degrees F., and then plunged into cold or gradually cooled water. In the Russian bath steam vapor is used and with higher temperatures even than those of the Turkish bath. A method of applying heat locally dry, even to the height of 400 degrees F., is by means of Beck's various apparatuses. The virtues of the hot mud, sand and other baths, as well as those of hot springs geaerally, are due entirely to the heat. According to the degree of heat there is first a contraction then a dilation of the peripheral circulation, which, however, lasts longer than it does after the cold bath. The sensibility is lessened and hence hot baths are sedative in irritable and painful states. A feeling of complete relaxation, languor and sleepiness usually follows them. The metabolism is slightly increased and the urea is more freely excreted. The pulse and respirations are at first rendered more rapid, but they soon become calm and steady. As a sedative, therefore, a warm bath is most valuable. In its power of provoking gentle and prolonged increase of the vital forces without causing excitement and high stimulation, the warm bath is more tonic than the cold. If it is taken too frequently, however, too hot or too long, it is enervating. As a continuous therapeutic measure it is more generally useful than cold baths in weakened states. Its effects may not be so immediately noticeable, but they are more lasting and nutritional. Mec hemic 0- therapy, massage and passive and active gymnastics all belong to the same general category. The basic principle underly- ing them is muscular and vasomotor movement by compression, irrita- tion and stimulation. The variations upon the principle are innumer- able and involve all sorts of mechanical methods and contrivances. Exercise, local and general, passive and active, is the foundation of these muscular methods of cure. In various institutions throughout the land are elaborate machines whereby patients are rubbed and shaken and twisted and slapped and punched and rolled and vibrated, until one wonders whether human beings after all are nothing but masses of impressionable dough. One cannot entirely gainsay these methods, elaborate and ludicrous as they sometimes are. In supplying that which the mental inertia of the patient himself makes wanting, they do indeed somewhat stimulate the organs and tissues of the body. Like the Kentucky colonel's infallible cure for a bad liver — namely a ride on a bad saddle horse — they do shake one up, and even a shake has its advantages. One wonders what sort of a pathology, except that which goes with natural laziness and acquired aboulia. is that which can he cured by shakes and slaps. ]f the patients enjoy it, however, and their faddish propensities are satisfied, we can at least attribute a large amount of the benefit to the psychic effect. Much of the talk- about the dissolution of adhesions, of the absorption of adventitious 84 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES matter and the revivification of the tissues is the mere twaddle of ad- vertisement and has no basis in fact. Massage and gymnastics are real and valuable aids in the treatment of nervous troubles and so far as the steam-engine, mechanico-machine methods can be made to imi- tate intelligent massage and normal gymnastic movements they are not to be condemned. Massage is a local exercise of the muscles entirely passive in char- acter. It has been systematized and more or less elaborated. In pro- voking fine muscular movement, in eliminating local products of metabolism and in hurrying on the flow of the blood current it lends itself powerfully to the relief and cure of nervous diseases. By blunt- ing the sensibility it relieves pain, warmth is created in the muscles and on account of the circulatory phenomena in them the latter maintain their normal development. Massage should never be employed except with a full realization of the underlying pathological condition and the end to be attained by it. In applying it the hand or hands of the operator, previously oiled with olive or cocoanut oil or not, are used and the strokes are always made toward the heart or centers of the body. In a general massage it is always best to begin at the periphery and work towards the body. The movements consist of gentle, steady, regular rubbing, pinching, squeezing, pressing, kneading and rolling of the muscles always in one direction. The pressure may be gentle or firm, superficial or deep, brief or prolonged. The patient, of course, remains all the while abso- lutely quiet. Gentle stroking or effieurage is perhaps the most generally used manoeuvre to begin with. It consists of quickly and lightly tapping the muscle. When the strokes are more energetic with one hand, while the other hand rubs the surface to and fro, the process is called massage a friction. Tapotement or percussion with the fingers and kneading are a couple of the frequent ways of employing massage. The patient must never be wearied by the treatment. As a rule a seance need rarely last longer than a half or three-quarters of an hour, often much less. Usually a restful and relaxed feeling follows. If the patient is inclined to sleep, he should be encouraged to do so. It is a good thing to give the massage usually after the morning bath. It should not be employed too soon before or after a meal. If given in the evening it is liable to disturb the night's rest. Gymnastics are active or passive. This is not the place to give an elaborate description of them. Passive movements of all sorts may be given while the patient is in or out of bed. He may or may not be told to resist the operator. Szvedish movements are of the nature of gymnastics. Active gymnastics, calisthenics are all well known and need but to be mentioned here. The use of rubber bands, dumb-bells. Indian clubs are advantageous at times. Moderation, gradual increase, en- tertainment and plenty of good fresh air must be enjoined while under- going gymnastic treatment. As soon as possible die patient should GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 85 leave gymnastic exercise for the final and more natural exertions that accompany outdoor walks, games and sports. For a discussion of the nature of electricity, the apparatuses em- ployed in its generation, and the laws in regard to its physics the reader should consult special works upon the subject. Here only a brief outline of its use in therapeutics can be introduced. There is much glamor surrounding the subject of electrothera- peutics and much mystery kept alive by the use of a lot of unnecessary details and formulae. Not so much harm is done by the want of knowl- edge in regard to volts and ohms and watts and electromotive force as by the inability to make a sufficiently correct diagnosis so as to know when and when not to use the electricity. As a natural force it is the same always, however it may be generated. The strength and character of the current are variously modified so that a slightly differ- ent effect is produced upon the tissues by these variations. The necessary electrical outfit for neurological work is not elab- orate. As a part, and a large part in my judgment, of the benefit of electrical treatment is to be attributed to its psychic effect, an elaborate display of apparatus has its advantages. The physician may, if he chooses, imitate the quacks and obtain some of the good results which they thus sometimes obtain. For actual physical electrotherapeutics, however, as I have said, not much of an apparatus is needed. The first requisite, of course, is a good generator of the electricity. Static electricity is obtained from the well-known static machine. Faradic and galvanic electricity are secured from the zinc-carbon, the dry silver-chloride or other form of galvano-faradic battery. In an office illuminated by electric light the electricity may be obtained by tapping the wires and using the commercial current after it has been passed through and is under the control of an appropriate rheostat. A milliampermeter is necessary to measure the dosage, and various electrodes and an insulating stool with the static machine, are needed to administer it. All kinds of ingenuity have been displayed in the creation of electrodes. What will be actually needed, however, are a couple of plain handles, one about 10 cm. and the other about 40 cm. long, a third handle with an interrupting attachment to it, an electrode about 5x15 cm. square, another about 10 sq. cm. in area, and a third about 1 sq. cm. A soft wire brush and a rheostat should also be among the fixtures. With most static machines a regular set of electrodes is furnished. High frequency currents, now being used with some degree of success, as well as the X-ray, need special apparatus. Perhaps a few definitions here of electrical terms may be allow- able. The anode is the positive pole; the cathode is the negative. A current is spoken of as stabile when the electrode is held steadily in one place, as labile when it is rubbed or moved over a part. An ascending current is one that is in a direction contrary to the course of the usual nerve impulse; a descending current follows the course of the nerve impulse. In regard to the modus operandi of electricity we know little be- yond the fact that it is excitant and stimulant. In spite of the at- tractiveness of the thought, it is probably not true that nerve force 86 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES and electric force are the same. As an irritant it is more likely that electricity excites, and unites with or completely annihilates nerve forces according to its strength, duration, etc. In this way it probably acts precisely as any other physical agent. In moderate dosage it is a stimulant tonic ; in large dosage it is a violent excitant ; in overwhelm- ing dosage it is a physical destroyer. In administering electricity this mechanical effect of it must never be lost sight of and the nature of the pathological condition — in a word, a correct diagnosis — must be established. I once saw a complete paraplegic who had been rendered so immediately after the second application of a strong galvanic current to his spine by a physician who diagnosed his trouble as rheumatism, though the entire history of the case for many weeks previously showed that a myelitis of traumatic origin was present. The patient died less than a year later, presenting a typical clinical picture of transverse dorsal myelitis. Only in some of the neuroses, neuralgias and diseases of the lower or peripheral neurones is electricity of much avail. In the neuroses and neuralgias all forms of electricity may be usefully employed. In the degenerative and trophic troubles of the lower neuro-muscular ap- paratus galvanism and faradism are the chief forms to be relied upon. Treatment should be given as a rule daily or every other day for six weeks or longer. In the neuroses the middle of the afternoon seems to be the best time. Seances, should average about thirty minutes ; longer under some circumstances, shorter under others. The proper dosage and other details of administration will be mentioned under the head of the particular diseases in which it is recommended. It may be stated here, however, that for its psychic effect and superficial influence the static current with its spark and other modifications is probably the most useful. Therefore it is to be commended in hysteria and certain other mild psychoses, in some neurasthenias and neuralgias. The galvanic current is probably the mest generally useful. It reaches the deeper parts, though not to the same degree as is some- times imagined, especially about the head and in the spinal cord, when employed in moderate or safe dosage. It is well to remember always that the anode is rather soothing in its effect, whereas the cathode is exciting and irritating. Only moderate and accurate!) measured cur- rents should ever be employed. The faradic current is more superficial in its influence and is generally useful in peripheral nerve and muscular troubles. There is no great difference between the effects of the electrodes, but the sec- ondary current is stronger and more irritating than the primary. The dosage of the faradic current is usually regulated by the sensations of the patient and the amount of muscular contraction. Dana tabulates the special methods of applying the galvanic and faradic currents thus : 1. General galvanization and faradization or general electriza- tion. 2. Local electrization by galvanization of the brain, of the neck. of the spine, of the special senses, limbs and viscera. Or by faradiza- tion of the neck, spine, limbs and viscera. 3. The combined faradic and galvanic currents. These are given GENERAL CONSIDERATIONS UPON NERVOUS DISEASES 87 by means of the De YVatteville switch. General and local electrization can be given in this way. 4. The polar method. This is employed chiefly in using the gal- vanic current. The indifferent electrode is placed on the sternum or back and the other electrode applied wherever indicated. 5. Cataphoric electrization by means of Peterson's electrode. 6. Electrolytic applications are used in enlarging strictures and affecting inflammatory deposits and neoplasms. Electro-diagnosis is referred to in the chapter on symptomatology and the examination of the patient. The factors that enter into elimatotherapy are largely physical in character and involve barometric pressure, purity of air, temperature, moisture, sunlight, wind, social and endemic conditions. As a rule nervous troubles are not much directly affected by these conditions, though the underlying diatheses upon which they depend may be very greatly so. In a general way neurasthenics prefer a warm, marine climate. Sea voyages are good for them, particularly on account of the enforced rest of mind and body that they involve. If stimulation is needed, high, rarified inland climates are to be recommended. Health resorts, such as those of Colorado and southern California, the West Indies, the Riviera and central Germany, are popular, but a new and virgin soil is sometimes better, away from the sight and thought of the presence of other invalids. A wild ranch life, a roughing of it in the forests of Canada, even a year or more on a good Mississippi Valley farm, is to be preferred to any popular resort, with its crowded cara- vansaries, its social competitions and its gaudy and shallow artificiali- ties. What a neurasthenic generally needs above all else is radical change of environment and of thought with rest. This is hard to ob- tain at the established resorts. Little need be said here in regard to the medicinal treatment oi" diseases of the nervous system. Except for the control of symptoms. medicines play but a minor role. Infections and underlying diatheses, such as tuberculosis, syphilis, gout, rheumatism and malaria, call for their special medication. So do anaemia and other blood conditions. The laity have not yet learned, and some physicians ajso, how little medicine does in some diseases; for on goes the expenditure of money and the swallowing of all sorts of patent nostrums, in spite of the fact that the pathological conditions show the utter uselessness of it. A badly disciplined girl is plied with valerian and asafcetida ; an ad- vanced tabetic takes hundreds of pills of so-called alterative.^ : and hemiplegics go on begging for some magic elixir which will relieve them of their imperfect speech. It is astonishing what crudity of ideas and credulity of belief are exhibited thus in connection with dis- eases of the nervous apparatus. A man is reconciled to an amputated limb, but for a lost memory he hunts the world up and down for a restorative. When he is told by competent authority that he has an incurable valvular heart lesion he seeks for relief, but not cure: when equal authority tells him he has an incurable optic atrophy, ho offers a million dollars for a remedy and consults with the m rant and superstitious. 88 GENERAL CONSIDERATIONS UPON NERVOUS DISEASES In no class of diseases is the doctrine of laissez-faire and the maxim of nil nocere more to be remembered than in those of the nervous sys- tem. There is always less danger in doing too little than in doing too much. The power of self-restraint and the maintenance of a masterly inactivity should be learned by the physician here if anywhere at all. If patients are clamorous, as they usually are, that something should be done, be honest with them, or at least with their family, and state the utter uselessness of treatment if it is useless. One's reputation is thus guarded and his conscience put at ease. If still the patient de- mands something,, inform the family that you will prescribe a placebo for its psychic effect. Everybody will be happy then, so far as hap- piness can be attained under the circumstances. Under no circumstances carry on a deception with yourself on the one side as the deceiver and the patient and all his family on the other as the deceived. Let the burden be shared partly by the family. It is legitimate to proceed in this way often to keep a foolish and self- willed individual from the hands of ignorant, greedy and unscrupulous quacks, both medical and religious. Serum Therapy and organotherapy seem to have a limited field of usefulness in neurology. They are still, however, in the experi- mental stage; except perhaps the use of the thyroid gland and its ex- tract in cretinism and myxcedema, where organotherapy has afforded most brilliant results. SECTION B. THE NEURONIC DISEASES. Parenchymatous Functional and Degenerative Troubles. THE NEURONE AND THE NEURONIC STRUCTURE OF THE NERVOUS SYSTEM. I have already referred to the fact that the nervous system, as it is known in general anatomy, is a large, complex, compound organ with a more or less uniform structure and function throughout all of its parts, but with an apparent difference of function in different parts on account of the different sorts of end-organs and tissues with which those parts are respectively connected. Viewed thus as a single organ, we find the nervous system composed everywhere of three distinct sets of histological elements dividing between them two important func- tions. The exhibition of nerve force is the specific function of the nerve- cells which with their processes are now called neurones. The nour- ishment and support of these nerve-cells, and the holding of them to- gether in ganglionic masses, constitute the specific function of the neurogliar and connective tissue, blood-vessels, lymphatics and epithe- lium. The former are the real functionating parts of the nervous system and so far are to be regarded as the neural tissues. The latter does not functionate in any specific way and accordingly may be looked upon as a non-neural tissue. Neuroglia is not found outside of the nervous system and will therefore need a little further explanation after I have considered the nerve-cells. The connective-tissue, blood-vessels, lymphatics and epithelium do not differ from the same elements in other parts of the body and hence need no further description here. The various end-organs and tissues with which the nerve-cells are in communication, and through which they reveal their activities, are included in the descriptions of the skin, the muscles, the organs of special sense, etc., in works upon general anatomy and histology. The Neurone and Neurone Doctrine. ( )f all the generalizations of science none have been more brilliant. more illuminating, more practical than the Neurone Doctrine. Like 90 THE NEURONIC DISEASES all these great scientific generalizations, as for instance, the law of gravitation, the atomic theory of chemistry, evolution, the nebular hypothesis and the undulatory theory of light, it is an inference merely, drawn from and propounded to harmonize a great mass of seemingly anomalous and unrelated facts and phenomena. It is not put forth as an infallible truth. It is open to future modification, and if need be to entire annihilation, should future discoveries be made that contra- dict its present teachings. Science, to be worthy of the name, must be something more than a catalogue of disjointed observations. If it hopes to escape chaos, or to make real progress, it must have a working basis, a theory, a plan, a rational and well-founded idea whereon to advance. Such is the neurone concept first clearly stated by Waldeyer, of Berlin, in 1891, and modified and elaborated by the work of Golgi, Raymon y Cajal, Van Gehuchten, Schafer, Sherrington, Bethe, Apathy, Batton, Held, Hodge, Vas, Barker and others. The special value of the doctrine lies in the fact that it puts many hitherto unexplained phenomena in the nervous system upon a satis- factory anatomical basis ; and it enables one to comprehend and to teach better than has hitherto been possible, the various nervous pro- cesses whether physiological, pathological or psychological. Neurosis and psychosis, organic and functional, inflammatory and degenerative, are some of the terms, bandied about in neurological pathology that indicate the unsettled state of much of our knowledge even to-day of the real nature of neurological processes. Like a mariner without a compass, we have been buffeted about upon the parenchymatous and interstitial, the nuclear and protoplasmic, the fibrous and cellular, the vasomotor and molecular waves until we have almost despaired of ever reaching terra Urma. The simultaneous ap- pearance of peripheral and central manifestations in tabes, the galvano- faradic dissimilarities between the central and peripheral palsies, the very raison-d' etre of the so-called systemic and non-systemic diseases have hitherto received but scant explanation. And as far as the pathol- ogy of the bcte-noire of medicine, hysteria, the imagination has toyed in our ignorance riotously with it or in hopeless despair left it severely alone. The neurone theory, with all its imperfections and unanswered questions, and founded as it is upon a limited number of facts, sends a brilliant ray of light into this mass of darkness and promises con- gruity where all has been incongruous. The following statements in connection with the theory seem to be warrantable at the present time : a. The nervous system is not a uniformly continuous, united entity, but is made up of a mass of distinct and disconnected nervous units. Discontinuity is the rule. b. These nervous units (neurones), each consisting of a cell- body with processes, have taken the place of the cells and fibres of the older histology. c. No cell-body is connected with any other cell-body : nor do its processes anastomose with those of its neighbors. d. The terminal processes of neighboring neurones intermingle THE NEURONIC DISEASES C)[ without anastomosing. Sometimes the terminal branches of the pro- cesses of one neurone surround and immesh, as in a net, the body of a neighboring neurone. This is spoken of as an arborization, a teloden- vum. The figure after J. Kollmann shows admirably the different stages of the development of the neurones. The neuroglia consists of a peculiar variety of cells which found only in the nervous system and which, are developed from the spongioblasts of the embryo. These cells seem to have no other fuirc- 9 6 THE NEURONIC DISEASES tion than to form a supporting network, scaffolding as it were, for the neurones. Until quite recently the neuroglia was spoken of as a variety of connective tissue. It is unlike the latter, however, in be- ing developed from the epiblastic instead of the mesoblastic layer of the growing ovum, and in containing the peculiar chemical substance neurokeratin, which % a variety of the keratin seen in the hair, nails and epidermis. The neurogliar cells are sometimes called "spider cells'' because of the relatively small size of the body and the very numerous and FIGURE 21. Axones of {mo- 1 tor) ventrul root. Medullary tube Neuroblast. Transverse section of the spinal cord of the chick to show the neurones in the different stages of development. Right side is from a five day chick ; the left side from a two day chick. (After Kollmann.) highly ramified processes. The body is varied in size but is more or less round in shape. It consists of granular protoplasm in which is imbedded a large nucleus with a nucleolus. So fine and numerous are the processes, that where the neuroglia is found in abundance, as for instance, around the central canal of the spinal cord, in the periphery of the cord, beneath the ependyma of the ventricles of the brain and below the pia mater, the tissue resembles almost a homogeneous matrix. According to Weigert the processes stain differently from the cell- body and thus would seem to have a different composition both chem- ically and physically. In states of inflammation the neurogliar cells are seen to swell up and serve as scavenger cells in carrying off noxious products. The Neuronic Architecture of tlie Nervous System. Having now studied the elements that enter into the composition of the nervous system and learning that the functionating neurones, according to Donaldson, make up fifty-three per cent, of its bulk (at least in the brain and spinal cord), while forty-seven per cent, con- sists of the sustentacular tissue (neuroglia and connective tissue net- THE NEURONIC DISEASES 97 work), it remains to be seen how these elements are arranged in rela- tion to one another in the architecture of the sum total or completed apparatus. Imbedded in the sustentacular tissue everywhere are the innu- merable neurones. Each neurone constitutes a unit in itself, so that it is quite improper to speak of nerve-cells and nerve-fibres as distinct and separate structures. The aggregate of all these countless neurones constitutes the real functionating nervous apparatus and their disease alone constitutes real inherent nervous disease. They are not scattered promiscuously, however, throughout the meshwork of supporting tissue but are more or less regularly grouped and arranged, both in relation to particular parts of the body and in relation to one another. FIGURE 22. Glia cells from the cerebral cortex of a rabbit. (After Benda.) Upon this grouping we have established certain artificial gross anatomical divisions of the nervous system, such as the brain, the spinal cord, the peripheral nerves and the sympathetic system. These divis- ions, however, are arbitrary, for the neurones which we find in them are always the same in all essentials. Somewhat more significant than these gross anatomical sub- divisions of the nervous system, is its obvious arrangement into masses of gray and white matter. A close examination of this reveals the fact that what is called gray matter is only the anatomical bunching together of the bodies of the neurones, while the white matter represents the assemblage in close proximity of their neuraxones. in the sympa- thetic ganglia, in the ganglia on the posterior spinal nerve roots, in the gray matter of the central cord, in the ganglia and nuclei of the cranial nerves, in the optic thalami and corpora striata, in the cortex, and in fact wherever this so-called gray matter is found, there are congre- gated in close quarters the bodies of the neurones. In the spinal nerves, in the white columns of the cord, in the centrum ovale of the brain, and wherever else so-called white matter is observed, there 9& THE NEURONIC DISEASES run the neuraxones extending out from the cell-bodies of the neu- rones. It is obvious, therefore, that there is no very great signifi- cance to be attached to this subdivision of the nervous tissue, namely, into gray and white matter. It is clear that the anatomical and functional relationship of the individual neurones themselves to one another is the great and im- portant underlying fact in the structure and specific activity of the nervous system. There is no single neurone anywhere in the body whose extent, with the aid of its neuraxone and dendrites, is so enor- mous that it can reach from one extreme end of the nervous system to the other. And yet we know that both afferent and efferent im- pulses traverse the entire length and breadth of the body. They must clearly, therefore, pass over several successive neurones in their long journeys. There is an end-to-end arrangement of the neurones of the various functional pathways along which the impulses travel between center and periphery. It is necessary to be familiar with the construc- tion of these afferent and efferent pathways, to know just where and how their constituent neurones begin and terminate, and to comprehend what functional relationship one neurone bears to its neighbor when both are constituents of the same direct and single pathway. In an earlier paragraph I described three typical forms of neu- rones and showed that whereas their differences were not essentially important, they subserved respectively, motor, sensory and sympathetic functions. Other types of neurones might have been described, espe- cially in the cortex, the commissural, and the association tracts of the brain. In all essentials, however, they are practically the same. If I describe the afferent and efferent projection paths upon the basis of their neuronic constituents, it will suffice to indicate the general neuronic architecture of the entire nervous system. The principle underlying it will have been portrayed. Future discoveries may en- large our ideas but it hardly seems probable that any very great alter- ation in the general principle of construction will ever hereafter be made. The entire length of the pathway for all motor impulses from brain to muscle seems to be made up of two long neurones, placed end-to-end. Of course it must be understood that the aggregate motor tracts are constituted of a great number of neurones lying side by side. Longitudinally considered, however, each individual outgoing impulse must, after it leaves the brain, traverse two neurones before it ex- pends itself upon the appropriate muscle. The first or central neurone has its body imbedded in the cortex near the fissure of Rolando and projects its neuraxone down through the cerebral crura and the pyra- midal tracts of the cord, to its termination in an arborization around some cells in the cranial nuclei of the medulla or in the anterior horns of the cord. The second or peripheral neurone has its body nestling in a cranial nucleus or anterior horn and extends its neuraxone out through the nerve to the end-plate of the muscle it is created to sub- serve. The two sections of this pathway may well be named cortico-spinal THE NEURONIC DISEASES 99 and spino-muscular respectively. The central neurone inhibits, stimu- lates, controls and regulates the action of the peripheral neurone. It moreover assists in maintaining its nutrition. The peripheral neurone on the other hand stimulates the contractions of the muscle and pre- sides over its nutrition. FIGURE 23. A.C.N Multipoint Cello/ Ant Horn Schema of the relationship of the neurones in the sensory and motor path- ways between the brain and the periphery. (After Halliburton and Mott.) The pathway for the afferent or sensory impulses is more com- plex and as a rule is made up of a number of end-to-end neurones. i ne outermost or peripheral neurone has its body hidden away in the ganglion on the posterior spinal nerve-root. As a modified dendrite one of its processes extends outward in the nerve to the skin, or some special end -organ, while the neuraxone passes inward and upward to LolC. IOO THE NEURONIC DISEASES the spinal cord to terminate in an arborization around the cells at the top of the column of Burdach and Goll. These ceils constitute the bodies of the next succeeding neurones whose neuraxones again pass upward to terminate in an arborization around the cells found lying in the optic thalamus. These cells once more extend their neuraxones up- ward to terminate and arborize around the cell-bodies of other neurones imbedded in the cerebral cortex. There are great variations in the neuronic arrangement of the sensory paths. They will be discussed somewhat more in detail when the anatomy and physiology of the brain and spinal cord are taken up. Here I merely wish to call attention to the fact that the afferent and efferent paths between the brain and the periphery are formed of a series of neurones placed end-to-end, that make the progress of the impulse a step-like one. There are special neurones and neuronic paths that do not emerge from the central nervous system at all but serve to connect different parts of it together and to approximate the inner ends of the various projection paths. In fact we are only on the threshold of knowledge in regard to the bewildering interlacement of all these countless neurones. It is hardly probable that what we denominate commissural, associative and projection tracts contain all of the neurones that it is possible for us to know later. Indeed we have still a goodly amount to learn in regard to these even, and the future will doubtless reveal to us greater complexities in the innumer- able pathways by which impulses travel hither and yon and in and out of the nervous system. When we will have learned more of these pathways, we will doubtless be able to understand some diseases that are now un- explainable. Already we are sufficiently acquainted with some of them to enable us to classify some organic and functional troubles in accord- ance with their location. There are some diseases that seem to have their seat entirely within the neurones. Pathologically and symptomatically they are, in the literal sense of the word, nervous diseases. The surrounding tis- sues may not be affected at all in them or only secondarily so later on. Etiologically, heredity, toxaemic and general cachectic states play the most important role in their development. Sometimes the heredity is direct, at other times it is indirect. The inheritance may range all the way from a mere neuropathic tendency to an actual and gross de- fect. So influential are the inherited and congenital conditions in the development of these diseases that it is doubted by some whether with- out such a neuropathic predisposition these diseases would ever appear as a result of the exciting causes usually given for their appearance. It is not believed by many that mere strain, exposure, overexertion, intoxication or infection would ever induce a pure neuronic disease if the neurones themselves were not previously predisposed by an inher- ited weakness. It is contended that such active causes would produce in those who were endowed with a vigorous heredity, diseases of the other tissues also, such as hemorrhage and inflammation. We divide these diseases nevertheless into the inherited and the acquired. This is a convenient division for practical purposes but it THE NEURONIC DISEASES IOI is about as strictly scientific and logical, in view of what we already know, as would be their division into organic and functional. All diseases in their last analysis must logically be assumed to be organic. A change of function to that which is non-function presup- poses by all the laws of reason, a change m the functionating substance. Organic and functional are relative terms to indicate the efficiency and inefficiency of our present means of investigation. The pains of tabes differ only in degree, and sometimes not at all, from the pains of idiopathic neuraglia, and yet merely because we can see the degen- erated conditions in the former we speak of it as an organic disease, while the unseen changes in the latter we denominate functional. Pathologically there is in all probability a mere difference of de- gree rather than of kind between the so-called organic and functional neuronic diseases. A neurosis is a degenerative trouble as truly as is a wasted condition of the neurones in some form of systemic disease. It may be a more widely diffused or a lesser degree of degeneration, but that it is a condition of functional and therefore inherent, degen- eracy can not be reasonably gainsaid. To conform with popular usage, however, particularly as we are in need of many data to make our con- tention absolutely undeniable, I will continue to divide the neuronic affections into the organic and functional. As some of the former con- fine themselves to, or at least begin in, a particular set of neurones, they may be further subdivided. In a gross way the same is true of the neuroses. The following seems to me to be a warrantable classification of the pure neuronic diseases: The Functional Neuronic Diseases. (Neuroses and Psychoneuroses.) i. Those of the Cerebrospinal System. 2. Those of the Sympathetic System. The Organic Neuronic Diseases. (System Diseases.) i. Those of the Afferent System. 2. Those of the Efferent System. 3. Those of the Afferent and Efferent Systems. FUNCTIONAL NEURONIC DISEASES. THE NEUROSES. All disease must be functional in its incipience. Some dis< remain functional; others go on to cause slight or minute changes un~ discoverable by our present means of investigation; while still others advance to the production of gross microscopic and macroscopic altera- tions, much destruction, and even annihilation of tissue. The latter changes we can see and so we call them Organic ; the former we cannot see, and so we still must denominate them Functional. This use of the term functional has cine to be specific. We do not include the gross organic diseases in the class of functional trouble-. 102 THE NEURONIC DISK ASKS though as a matter of fact they involve even greater functional dis- turbances than do the latter. The specifically called functional diseases are sometimes denomi- nated the Neuroses. As these diseases are, so far as we can deter- mine, the result of hereditary, nutritional, hygienic and general toxaemic influences, they implicate all of the neurones. They are diseases, therefore, of the nervous apparatus more or less in toto, and from their inherent nature are the most specific of all those of the nervous system. They are diffuse affections within the neuronic network. They there- fore are sometimes called the Psychoneuroses. They do not all present, for some reason unknown to us, exactly the same degree of vividness in their mental, sensory and motor manifestations. Some of them, like hysteria, take on a psychic complexion ; others, like hemicrania, lead with sensory phenomena ; still others, like the myospasms, seem almost to be entirely motor. The characteristic of these neuroses or psychoneuroses is that they all present a clinical picture in which all forms of nervous symp- toms appear, though not in all patients nor in all periods of the disease. Their diffuse localization is well represented in the name psycho- neuroses. The hereditary psychoneuroses are of the nature of biological defects. They are instances of racial or familial degeneracy. They bespeak a functional inadequacy in the neurones as a result of an embryological degenerative inferiority in their structure. As diseases they are therefore not hopeful from the standpoint of prognosis or therapy. The congenital psychoneuroses are hardly much more favorable. The acquired psychoneuroses are somewhat more encouraging, though as some heredity, less in intensity perhaps, plays a role in their evolution, they are at times obstinate and need both judicious and decisive management. Their prognosis under given conditions is ex- ceedingly bright. We can conveniently divide the psychoneuroses into those whose symptoms point most prominently to the cerebrospinal apparatus on the one hand, or to the sympathetic system on the other. The functions of the latter are so markedly distinctive that when they are deranged we sometimes refer to them as angioneuroses or trophoneuroses. It goes without saying, of course, that this classification is liable to modi- fication under the development of future knowledge. A. THE CEREBROSPINAL NEUROSES. PSYCHONEUROSES. HYSTERIA. Hysteria is recognized at the present day as a mental trouble, which, unlike the forms of psychosis usually classified under the old term insanity, reveals itself more distinctively through the motor, sensory and trophic apparatuses than they dc. The paralyses, anaesthe- sias and other stigmata of the disease are clearly mental in origin. Moreover, the disturbances of the memory, volition and intellect , cans- THE NEURONIC DISEASES IO3 ing a greater or less transformation of the patient's personality, the ego, indicate the psychic nature of the disease. The resemblance of many of the phenomena allied to hysteria, such as the histrionic manifestations, the mimicries, the hypnoses, the catalepsies, the somnambulistic actions, as well as the character of the typical hysterical symptoms themselves, have inclined most authors to regard the trouble as largely a disturbance of the higher psychic or cortical inhibition, with a relaxation of the lower or subcortical, or ganglionic activities. It is assumed that there are, so to speak, two minds, the conscious and the subconscious, the seat of whose activity is not known positively, though the former is supposed to be dependent chiefly upon the cortex and the latter upon the lower layers of the cortex or basal ganglia. Janet speaks of hysteria as being the result of a "contraction of the consciousness." From this point of view hysteria may be not inaptly compared to the well-known spastic paralyses of organic cerebral disease; only in hysteria the failure of inhibition from above and the spasticity from below are entirely and completely in the mental or psychic sphere and not in the organic. While admitting that much of this is purely theoretical, the theory explains and covers a larger amount of hysterical phenomena than any explanation hitherto ad- vanced. Etiology. — The first thing that must be observed under the head of the etiology of hysteria is that most of the causes assign to oy the older writers are no longer regarded as such. The uterus, on account of which the disease received its name, is not the cause: nor are any of the sexual organs, or the sex of the individual. Not upon any single organ or tissue of the body can the blame be laid for the hysterical condition, though upon one or all of them may depend the outbreak of an attack. The primary cause of hysteria is to be found in the patient's mental make-up, his temperament, his mental instability, which is more or less congenital and constitutional. The secondary causes that may precipitate an attack of hysteria or arouse the latent temperament into a more or less continuous objective reality are in- numerable and include such factors as early education, discipline, en- vironment, physical abnormalities and local diseases. From what has just been said, ir will be readily surmised that heredity plays the greatest role among the predisposing causes of hys- teria. The heredity is usually direct and transmitted through the maternal ancestry. The mother, or grandmother, or maternal aunts of the patient will be found to have been hysterical. Frankly admitting the force of suggestion and close companionship between mother and child in the development of hysteria in the latter, it is nevertheless true that the hysterical predisposition is chiefly pre-natal in origin. The heredity is not always direct and occasionally it is transmitted by the father. Chorea, epilepsy, extreme neurasthenia or mental disease may have been the trouble in the ancestry. Chronic alcoholism, excessive use of tobacco and dissipation involving nervous strain may transmit to the offspring the neuropathic temperament that needs but an exciting cause to provoke a genuine attack of hysteria. 104 T HE NEURONIC DISEASES Next to heredity the great predisposing cause is a false early edu- cation. The child being more or less neuropathic, its irritability, over- sensitiveness, flashes of what seem like mental brilliancy, high emo- tionalism and general abnormality are fondly mistaken by parents for evidences of exceptional talent or even genius. It is pampered and coddled, all its whims are humored, its doings and sayings are lauded, and all systematic discipline and irksome routine are completely omit- ted. The child becomes egoistic and tyrannous ; it loses all idea of the proper relationship it should bear to society ; it feels aggrieved at every little opposition it meets ; and as a result it grows into adult age com- pletely unfitted to meet the disappointments, discouragements and trials of life. This provokes a spirit of revolt and a tendency to be moody and unsocial. It may break into a violent and emotional excitation. For a long time it was believed that hysteria was a disease of women only. This was the result largely of locating the cause of the trouble in the uterus. The same symptoms in men were not deemed hysterical. Since we have given up this old pathology and pathogenetic explanation of the symptoms, more care has been taken in studying and comparing them and as a result we find that hysteria is a disease of both sexes and of all ages. The trouble appears more frequently among women than among men in the proportion of about ten or six to one, not because of their sexual but because of their nervous and mental differences. Woman's nervous system is more mobile and less inhibited normally than is man's. Some of the most typical cases of hysteria that I have seen have occurred in men. The dependence of the disease upon nervous instability is further emphasized by the fact that it so frequently breaks out in its most violent forms about the time of puberty, and from this period up to the thirtieth or fortieth year. Many of the neurotic symptoms of the climacteric are hysterical. It occurs in childhood and has been seen as early as two years of age. Passionate outbursts of temper and such manifestations as chorea and certain subjective sensory phenomena in childhood are occasionally hysterical in character. Hysteria is rare after the climacteric and in old age, though some authors speak of a senile hysteria. Certain races and nations seem to be more prone to the disease than others. It is very common among the Latin races, especially the French, from whom we have learned most about it. The Jews are very liable to it and it has been observed among certain barbarian tribes. The Anglo-Saxon is probably the least subject to it among the civilize 1 races. There are certain exciting causes that may arouse a latent hysteria. Foremost among them are emotional shocks and strains, such as terror, anxiety, disappointed love or what may be termed psychic traumata of every sort. The symptoms following lightning stroke, earthquakes, railroad accidents and collisions are often purely hysterical. Sudden irritative and imitative impulses may excite a hysteric outbreak. This occurs in epidemics and among masses of people. The THE NEURONIC DISEASES 105 mediaeval semi-religious pilgrimages, dances, the New England witch- craft, modern revivals, stampedes, religio-medical followings of quack- ery and peculiar beliefs of all sorts, many strange antics of the inmates of monasteries, convents, boarding schools and factories are all more or less attributable to the hysterical tendency. The history of these epidemics and outbreaks affords most interesting and sad reading. The folly and weakness of human nature is its most obtrusive lesson. Organic diseases and traumatic injuries may worK through the mind and nervous system to provoke and maintain a hysterical at- tack in one who is already predisposed. By themselves they are not causative of hysteria. This should be carefully remembered, for it explains how a removal of these exciting causes, by surgical or other means, often results in a return of the hysteria or hysterical tempera- ment into a latency which is too often interpreted as a cure, but which only awaits another powerful excitant to awaken it into activity. Just as hysteria is often symptomatically imitative of known forms of organic disease, so in those already predisposed it is etiologically imita- tive of them. Symptoms. — As Mobius well says, hysteria is a state in which ideas exert an influence to bring about morbid changes in the func- tions of the body. The ideas or mental state of the patient constitute the most, important symptoms therefore, and though not always the most obtrusive or the most easily elicited, they should in every clinical examination be most earnestly and persistently sought for. Certain facts stand out very prominently in regard to a hysteric's psychic condition. They are irritability and instability, abnormal emotionalism, altered volition and inhibition, and increased power of imagination. The mental power is not weakened, the memory is usually intact, and the will and intellect are about up to the patient's normal capacity. This leaves the imagination as the chief faculty at fault, and yet the increase in the power of the imagination is rather the result of the weakened power in the higher or controlling inhibition. Abnormal irritability with changeability of ideas, all the result of a congenital defect in the cortical structures underlying the function of mentaliza- tion and of an educational failure in the development of the associational paths supporting the proper concepts of life and its thousand and one relationships, is the hysterical mental condition. This abnormal irritability is both centripetal and centrifugal in its action. It is provoked abnormally by every slight inpouring impulse and it provokes abnormally every function of the body, motor, sensory and vasomotor. As Oppenheim points out, this abnormal irritability reveals itself in the sudden variations in disposition and in the pathological increase of normal actions. Laughing and crying become spasmodic. The transient normal inhibition produced by tear, anxiety, becomes paraly- sis. Indifference and insensibility from the same cause become Stupor, delirium and anaesthesia. Imaginary thoughts develop into hallucina- tions. Motor activity passes into spasms, contractures and cataleptic rigidity. This irritability and heightened influence of the mind are produced 106 THE NEURON 1 C DISEASES by the most insignificant circumstances and often by no known reasons. It shows itself in unwarrantable outbursts of anger, joy, vexation, depression and terror. The memory may participate in this irritability, causing past events and sights to be recalled with such vividness as to throw the patient into a confused and dream-like state and to make it appear as though he were living in another world or in another period of time. With all this irritability there is a most obvious loss of the power of mental inhibition and self-control, especially over the emo- tions. There is not strictly any loss of volitional power, or aboulia, but rather perversion. When the patient declares she cannot act, she frequently exhibits at the same moment an astonishing power of voli- tion in other ways. The ideas which are passing through her mind at the instant have so altered her motives for action that her power of volition is shown not in the desired but in other and unexpected ways. Instead of loss of will, therefore, it is nearer the truth to speak of perversion of the will. There is no distinct weakening of the mental power, though feeble-minded people may be attacked by the disease. As a matter of fact, hysteria occurs more frequently among intelligent than among ignorant individuals, a truth which has its bearing, in the management of these cases, upon the relationship of the physician to the patient. The alterations of character, the oft-times senseless and, purpose- less acts, the prevarication, the instability and unreliability, the tout ensemble picture which so often arouses disgust and even anger among the bystanders, are not the result of mental enfeeblement, but rather of the abnormal irritability, rapid changeability, perverted memory and vivid imagination which cause a swift passage of ideas through the patient's mind. There is no fixity in her motives and she is the prey to every gust that whirls down upon her mental horizon. She acts logi- cally in accordance with her motives, albeit the acts themselves appear to be bizarre, willful and without rhyme or reason. To understand, therefore, the attitudes, the speeches, the sensa- tions, the whole outward manifestation of these patients one must closely recognize the state of mental irritability and then as far as possible trace the underlying ideas to their source. Hysteria being in that ill-defined borderland between sanity and insanity, its mental manifestations, which are always present, assume the most varied forms. Some hysterics are undoubtedly insane in the common acceptation of the term, while others are only weak and irre- sponsible, especially in the moral sphere, but would not be commonly regarded as insane. Hysterics exhibit the hallucinations, delusions and illusions of the most profound forms of mental alienation, down to mere prevarica- tion, secretiveness and other slight forms of moral delinquency. It is in the ethical and emotional spheres that hysteria indicates its relationship to mental troubles most distinctly. Certain mental mani- festations that occasionally occur with hysteria should not be looked upon as a part of the disease. They are mere complications; the two conditions are merely associated forms of disease due to the same cause. A melancholia, mania, paranoia will sometimes break out in THE NEURONIC DISEASES IOJ the course of hysteria and be erroneously named hysterical melan- cholia, hysterical mania, etc. The prognosis of such cases depends not upon the hysteria, but upon the insanity, and as a rule it is bad for recovery. A clear appre- ciation of this distinction will lead to better results in the management ■of hysteria and to more prompt asylum confinement in the cases com- plicated by insanity. Other symptoms, often classified as hysterical, such as the impera- tive conceptions, phobias, like agoraphobia, pyrophobia, manias, like dipsomania, morphino-mania, kleptomania, are complications and should not be regarded as a part of the disease we are now referring to. They are expressive of a phrenasthenia and represent a mental de- pression, congenital or acquired. They often occur in hysteria, but they are not symptomatic of it. As mental troubles they may be modi- fied and colored by the hysteria that is present, and the association of the two sets of symptoms may be so close- that one may have much difficulty in determining which is the predominating and more im- portant disease. Here doubtless belongs the astasia-abasia so much studied in recent years by European neurologists. Standing and walk- ing are impossible, though there are absolutely no signs of disturbance of motility, of sensibility or of coordination when the patient is lying •down. Only when he gets up and attempts to walk docs the difficulty reveal itself. Moving on all fours, swimming and other forms of movement may still be performed. There are several possible explanations. It is clearly psychic in origin and may be a sort of specific psychic ataxia or special form of mental incoordination, loss of the memory pictures for walking. A somewhat similar condition obtains in some of the occupation neuroses. True hysteria, uncomplicated, is characterized by three particular forms of psychic disturbance observed mostly in paroxysms. The first is an indefinite sense of fear, unaccompanied by definite conceptions such as occur in the well-known phobias. With this sense of fear is a feeling of precordial oppression and cardiac palpitation. The second is a state of hallucinatory delirium, in which the patient shows the manner and fancies of one under the influence of frightful dreams or visions. He slinks away or strikes at the attendant. From this half-dreaming state a dash of cold water or other forcible form of stimulation may arouse him. The third state is that in which somnambulism, hypnotism, lethargy, catalepsy, etc., obtain. All of these three states are typically hysterical and arc dependent largely upon the abnormal activity of the affective faculties, the feel- ings, emotions and imagination. Next to the mental state in frequency and importance are the presence and the character of the sensory symptoms. Though tin— symptoms are subjective, like the mental., and are therefore that much less valuable as positive indication- of disease, they can be examined and tested objectively or at least made to provoke objective signs that ro8 THE NEURONIC DISEASES FIGURE 24. Hysterical anaesthesia. THE NEURONIC DISEASES IOQ are most valuable in indicating not only the presence of, but the real nature of the trouble. The first great law to be remembered in regard to hysterical sen- sory phenomena is that they are physiologically and anatomically mental and not neural. They do not localize themselves in accordance with nerve distribution or cortical limitations ; they involve those parts of the body known only to the patient's ego, or his consciousness. They are therefore more or less co-extensive with the segments of the body and not with the mere cutaneous area of this or that set of nerves. The hand, the arm, the leg, the half of the body, the glove or stocking type of distribution indicate the areas affected. The special senses show the same peculiar mental disturbance. In hysterical blindness it is mental vision, not actual vision that is affected. A second law to be remembered in regard to these sensory phe- nomena is with regard to their great changeableness and instability. They come and go with rapidity and uncertainty. They may be mild or marked throughout their entire extent and continuance, or they may be mild in one part of the body while severe in another. One side may show hyperesthesia, the other anaesthesia. The visual field of one eye may be immensely more altered than that of the other. A reversal of the conditions may even take place at a most unexpected moment. It is this fact that renders hysterics the willing and self- deceived dupes of religio-medical quacks, to whom they give fulsome testimonials of astounding cures. All forms of sensation may be affected in hysteria. Smell, taste, hearing and sight of the special senses and tactile, temperature and pain of the cutaneous senses, and even the muscular sense may be altered. The sense disturbance itself may be exactly that of an organic disease ; the only way it can be determined as hysterical, mental or functional is by carefully considering its distribution and noting its great change- ableness and instability. The pain sense is probably the one most frequently reported. In fact, more or less pain is complained of in every case of true hysteria. To attempt to give a complete description of the pains of hysteria would be to write a volume upon every kind of pain known to the human mind. The pains of hysteria may be of every sort and of every degree of severity; they may be located in any or all parts of the body; and they may act exactly as any other sort of pain acts. Then how are we to determine the hysterical character of these pains ? Often we cannot. Less frequently we can do so only by their association with other symp- toms and conditions suggestive of hysteria, and still less frequently by certain peculiarities that in a few hysterics do characterize and dis- tinguish them from other forms of pain. In the head there is with the pain an occasional hyperesthesia. The patient dislikes combing of the hair. Often there is a distinct clavus or sharp circumscribed area of pain at the vertex, a |x>sition not commonly selected by headaches from other causes. Rarely thi- pain is dull and spreads over the whole cranium or is limited to one side. In such instances neurasthenia or migraine should be carefullv no THE NEURONIC DISEASES FIGURE 25. 1 [ysterical anaesthesia. THE NEURONIC DISEASES IIT eliminated before a diagnosis of hysteria is made. With hysterical clavus, lasting several hours or days,- there is not unfrequently vertigo, nausea and vomiting. Remember that hysterical headache, whether it is vertical, occipital, temporal cr of the entire head, shows a tendency to be influenced by the mental condition of the patient. In this it differs from other forms of cephalalgia. Spinal pains are a part of the hysterical condition. I have not found them equally distributed along the entire spine so regularly as I have in neurasthenia. In hysteria they are more focal in character, a choice location seeming to be between the shoulder-blades. When tested by percussion or by the passing of a hot or cold sponge up and down the spine the patient winces or at least indicates suffering when certain spots are passed over. In this the trouble resembles the focal meningitis of the spine, from which it must be differentiated of course by other symptoms. Pains are frequently complained of in the region of the ovaries and mammary glands. In all cases of genuine hysteria these areas, the hysterogenic zones, are highly sensitive and may be painful upon pres- sure. I have seen hysterical men who suffered from sharp pain in the testicles, usually the left. Trunk pains, resembling intercostal neural- gia, gastralgia, cardialgia and even the cincture phenomenon of organic spinal disease, may be present. Pains are not frequently complained of in the extremities. Here the altered sensation more often assumes the character of numbness or "pins and needles." Once I saw in a young woman a painful toe that simulated gout. Under treatment it soon felt like a needle which the patient declared she had accidentally swallowed when an infant. Here, under suggestion, hyperalgesia was replaced by paresthesia. Cure followed further suggestion and the skiagraphic proof that there was no needle present. Mobius has described under the name akinesia algera a form of hysterical immobility caused by the pain produced in every form of movement, voluntary or forced. The pain is purely psychic in origin. It may involve every movement of the body and at times prove a most serious obstacle to treatment. Sensations of heat and cold, of formication, of alterations in size and strength, are much more common, in my experience, as hysterical symptoms in the extremities than are pains. This difference between the sensory phenomena of the trunk and the extremities is probably due to the psychic character of hysteria, the finer and more complicated physiology of the extremities having a more varied psychic representa- tion. The sensation of pain is more primitive and elemental than are the hallucinatory sensations like formication, worms, pins and needles. All of these sensory disturbances are of central or psychic origin. They may occasionally be awakened or localized by some form of peripheral irritation, but they are mental hallucinations. They arc the expression of the direct excitation of the pain-perceptive centers of the brain. Next to pains, so important are the paresthesias, hyperesthesias and anaesthesias in the symptomatology of hysteria that they must be considered a little more in detail. 112 THE NEURONIC DISEASES Formication, the feeling of ants and worms crawling over the body, and numbness are probably the most frequent paresthesias. It is a question whether some of the strange delusions of the hysterical may not be due in part at least to internal as well as external forms of paresthesia. A patient who was a typical hysteric had the delusion that there was a fish in her head which swam about there and came forward to drink whenever she swallowed liquid. Hysterics not unfre- quently complain of toads in their stomach and of worms in their bowels, which delusions may well be due to visceral paresthesia. Perverted sensation is in the very nature of things psychic and is therefore almost pathognomonic of hysterical or Other forms of psycho- sis. It may be provoked by various peripheral stimuli, but the per- version can only be the result of central trouble. In this it differs from hyperesthesia and anesthesia which may depend upon peripheral nervous disturbance as well as central, and are not so necessarily indica- tive of a psychosis. Hyperesthesia and anesthesia, being merely degrees of sensory disturbance and not perversions, are not pathognomonic of a psychosis and therefore are less valuable as symptoms of pure hysteria. They may both be due to actual peripheral nerve disease and thereby be asso- ciated with probable hysteria. On the other hand, they may not repre- sent any actual organic disease at all, but may be entirely central in origin and therefore indicative of a pure psychosis. In the latter case their distribution, their favorite location, their variability and change- ability and their modification under the patient's ever-varying mental state indicate their purely hysterical origin. It is rare for hyperesthesia ever to be complete. It is more fre- quently seen in spots and circumscribed areas or in conjunction with certain viscera and deep structures like the muscles and fascia. The hyperesthesia frequently coincides with the hysterogenic zones, a fact which is made use of to initiate or check an attack of hysteria, by making pressure upon them. These hysterogenic zones on the anterior part of the body are the areas just under the mammary glands, just above the ovaries, along the sternum, at the outer end of the clavicle, at the lower, lateral edge of the thorax and at the vertex of the head. The spinous processes of the back are often hyperesthetic as well as the tip of the coccyx. The left side of the body being the sensitive side, hyperesthesia as well as anesthesia selects it most frequently when they are of psychical origin. The special senses also frequently submit to an hysterical ex- aggeration of their function. Flashes of light, flickerings and other visual phenomena are among the common signs of hysteria. An in- crease of the visual field (Freund) in all directions is much rarer than a diminution. The "tubular field of vision'' is a condition in which there is a constriction and the absolute area is the same, no matter at what distance it is measured. I have seen it once. Partial increase, however, with partial diminution and the increase of some of the color fields, which virtually amounts at times to crossing or reversal of the color fields, is a form of partial hyperesthesia that I have not tin- THE XETROXIC DISEASES 113 FIGURE 26. Hysterical anaesthesia. (After Thomsen.) 114 l'BE NEURONIC DISEASES frequently seen. Double sight with one eye, not otherwise accounted for by optical conditions, is hysterical. Taste and smell and hearing may all be exaggerated. The hyper- sensitiveness of these nerves, however, is clearly psychic. The per- version of their function is probably more common in hysteria than is hyperesthesia. The ancesthesia of hysteria is clinically the most valuable of the sensory symptoms. It is the most frequent, is less intensely subjective than the others and can be examined with a higher degree of objec- tivity. Like the other sensory symptoms, it is purely psychic, as shown in its distribution and variability. As Bernheim well points out, all psychic anaesthesia, however, is not hysteria. Hemianaesthesia may be directly added to hemiplegia by pure psychic dynamism. It may occur from various causes in patients who are not the least hysterical. Hys- terical anaesthesia is observed in connection Avith all forms of sensation, special as well as general. The most common form of cutaneous anaesthesia is that which involves one entire half of the body, hemianaesthesia. Next in fre- quency is that which is limited to the conscious segments of the body, the hand, the forearm, the arm, the leg, the top of the head. This is the glove-type or drawers-leg type of anaesthesia. Its boundaries usu- ally correspond with the transverse folds and joints of the body or with the so-called transverse amputation lines. Both the hemianaesthesia and the glove-type reveal the psychic nature of hysteria, for they correspond to the mental conceptions of the parts of the body and not with the anatomical distribution of the sen- sory nerves. The least frequent of all the forms of anaesthesia is that in which the insensibility is scattered in patches or circumscribed areas about the body. And yet even this form is not in correspondence with the strict arrangement of the sensory nerves. It is bizarre and irregular. Its areas are located in and about the hysterogenic zones. It is deter- mined by such numerous local influences upon the patient's mind as pressure from the clothing, exposure, physiological function. Its bor- der lines are sharper than are the border lines of organic anaesthesia. This diffuse form of anaesthesia should be always most guardedly pro- nounced upon, for it is the type that is most liable to cause hysteria to be erroneously diagnosed for such organic troubles as multiple sclerosis, polyneuritis, etc. It may even occur with these diseases. This cutaneous anaesthesia is chiefly a pain anaesthesia, the tactile sense being normal or only slightly blunted. It closely resembles in this respect the dissociated sensory phenomena of syringomyelia. It is fre- quently tactile, however, and then the pain sense is alsc usually entirely absent. Occasionally the touch sense is merely blunted, hypccsthcsia. It is never merely delayed as in certain organic troubles. Loss of, or diminution of, the temperature sense, thermanaesthesia, may be observed. All these superficial forms of sensory disturbance are met with in the mucous membranes as well as in the skin. The mouth, the vagina, the rectum may all be involved. The superficial reflexes will HE NEURONIC DISEASES US FIGURE 27. Hystcric.il hemianaesthesia. (After Charcot.) n6 THE NEURONIC DISEASES FIGURE 28. Hysterical anaesthesia. (After Charcot.) THE NEURONIC DISEASES 117 be correspondingly involved, though at times a reflex response may be obtained in spite of anaesthesia, if the test is made suddenly and when the patient is not expecting it. The deep sensations, as those of the viscera, the joints, the fascia, are also subject to anaesthesia. Hysterical astereognosis and loss of the sense of position may be present from implication of the muscular sense and other deep senses. The special senses undergo hyperesthesia and anaesthesia. Upon this many of the curious phenomena of hysteria depend. The visual disturbances are the most common and important among the special senses. Complete amaurosis is very rare and loss of central visual FIGURE 29. — : 1^- f - Hysterical contraction of the visual fields. Private case. Charts drawn by Nance. acuity almost never occurs. Usually there is a more or less concentric narrowing or contraction of the field of vision, which of course must: be determined by a perimetric examination. The perception of colors may be lost or diminished, or the limitations of the color fields may be reversed so that they will overlap. These phenomena are usually more marked on one side than on the other. When unilateral the eye affected is usually the one on the same side as the cutaneous anaesthesia, which is mostly the left. Whenever the order and extent of the fields for white and colors are reversed, the evidence is positive of the existence of hysteria. It cannot be simulated. It does not occur in any other ocular disorder. Ring scotomata are particularly suggestive of a psychoneurosis. That the loss of vision is mental and not organic is shown by the fact that, unlike organically blind people, these patients avoid obstacles placed in their way. It can also be proved to be mental by the use of the stere iscope, by prism- ingeniously arranged and by a box in which the lines of vision from the eyes are made to decussate by means of small mirrors. The preservation of the pupillary reflexes in- dicates its non-organic character, and even though the pupils are at" n8 THE NEURONIC DISEASES fected, as they sometimes are by hysterical spasm, their appearance and mode of action is not at all in correspondence with an organic loss of the visual sense. All forms of anaesthesia may attack the retina, so that there may be hemianaesthesia or quadrant anaesthesia or focal and diffuse spots of anaesthesia. The same may be said of the achromatop- sia or dyschromatopsia that may exist. It is to be remembered that ocular hysteria is common in children and men. Organic disease of the eye may accompany the functional disturbance. The taste and hearing are sometimes lost on one or both sides. The alterations of taste explain the readiness with which these patients will resort to such filthy habits as swallowing urine and eating dirt. Moreover it accounts for their hallucinations and absurd declarations in regard to the articles of food that are given them to eat. Hysterically FIGURE 30. LEFT Ring scotoma in a hysteropic boy. (After Wood.) deaf patients do not as a rule incline their head in an effort to hear like those affected with organic deafness. Osseous conduction is usually absent. There is often cutaneous anaesthesia in and about the meatus of the deaf ear. When the olfactory sense is involved, the patients do not com- plain of disgusting odors and they may even reverse normal opinion and seem to enjoy that which nauseates the attendants. Hallucina- tions and illusions are common enough here. It is a curious fact that sometimes the patient himself is not aware of his various anaesthesias and only realizes them when they are demonstrated by the examining physician. Their instability, peculiar distribution, sudden transference and general bizarre character make them important, almost pathognomonic symptoms of hysteria. Janet thinks they are but the objective manifestations of the contraction of the field of consciousness. All forms of motor disturbance may be observed in hysteria. Spasms, both tonic and clonic, arc more frequent than are the con- tractures, pareses and paralyses. These disturbances are never as com- THE NEURONIC DISEASES IIQ plete as, nor do they simulate identically, the organic motor disturb- ances. Their psychic origin is noticeable in their peculiar functional character, their incompleteness, their localization and distribution, their duration and their instability and abrupt changeability. The absence of other distinguishing symptoms that accompany organic motor dis- turbances indicates the psychic or hysterical nature of these. For ex- ample, respiratory spasm of hysterical origin is not accompanied by the usual signs of the need of air ; hysterical paralyses do not exhibit the typical flaccidity or spasticity, the atrophy, the reflex and electrical changes which are associated with the organic paralyses. The motor disturbances of hysteria are due to an alteration in the patient's con- cepts of motion. Just as a hysteric really feels and sees and hears but does not know that she does, so she really can move her limbs but fails to do so because she does not know that she can. For this reason it has often been erroneously taught that aboulia, or paralysis of the will, was the underlying factor in hysteria. In the words of Paget the hys- teric says "I cannot" ; it looks as if she "will not" ; in reality, she cannot will. Such a view of hysteria is, however, not correct. There is plenty of volitional power, as is so often shown by the persistence and deter- mination with which the patient carries out her special plans and de- sires. The aboulia of hysteria is only an apparent loss of will power due to a perversion of the appropriate concept of motion. Suddenly restore this lost concept by suggestion, or mental shock, and the aboulia will vanish. Chief among the hysterical spasms, if indeed it is to be regarded as the result of a spasm and not a form of paresthesia, is the well-known globus hystericus. The patient complains of a lump in the throat, a ball which may or may not seem to have risen from the stomach and which feels as if it were held in the grip of the pharyngeal muscles. This sometimes produces great distress and together with spasm of the glottis may cause so severe a spell of choking as to call for the opera- tion of tracheotomy. The muscles of respiration are frequently in- volved. In fact the emotional and respiratory spasms are among the most common motor disturbances of hysteria. The crying and laugh- ing, often so meaningless, are of this nature, as likewise are the attacks of singultus (sometimes epidemic according to Oppenheim, Abelcs and others), eructation, of yawnings and of sneezing. Instead of tachypnoea there may be aphonia from implication of the vocal cords. Hysterical aphonia and aphasia are both common, especially the former. Cough (tussis hysterica), when present, is peculiar. It is dry, short and sharp like a dog's yelp and is absent during the night. Twitching of the eye- lids, blepharoclonus and blepharospasm, with photophobia and water- ing of the eyes may be present, but is more common in neurasthenia. a disease which often complicates hysteria. Any of the voluntary muscles of the body may be subject to hys- terical spasm. Twitching and convulsive movements, all forms of vio- lent motion, tonic as well as clonic, may attack the limbs and hurl them about or hold them in a rigid attitude. When the back muscles are in- volved there will be Opisthotonos or rhythmical arching 01 the body. All degrees of muscular spasm arc possible, and this, taken in con- 120 THE NEURONIC DISEASES junction with the fact that the movements are the same, only more vigorously performed, as normal voluntary movements, has formed the basis of an attempt to classify them. Even with the most violent spasms, consciousness is never entirely lost ; usually it is entirely present, though in the more severe cases it is clouded and disturbed with hallucinations. A curious hysterical phenomenon is the Saltatoric spasm, first described by Bamberger in 1859. It is rather more fre- quent in males than females. The symptom appears usually suddenly and consists of very rapid alternating contractions in the flexors and extensors of the legs, causing the patient to jump entirely off of the floor. It occurs only on attempts to stand and ceases when the patient sits or lies down. Scarcely ever does it attack the arms, and then only slightly. Hopping, jumping and running around, whenever he touches FIGURE 32. Major hysteria. (After Hamilton.) the floor, is the predicament of the patient. The spasm is clonic and involves ehiefly the calf muscles. Gowers, Kast and Eilenmeyer be- lieve it is a form of reflex. I am inclined to regard it as hysterical. A consideration of the various periods of a grand attack: of hys- teria, as outlined by Charcot, forms an interesting commentary upon the psychic nature of the disease and upon the manner of the, action of the mind. In the first stage, the consciousness being fully awake and realizing that something dreadful is impending, there are charac- teristic prodromes. There is irritability of temperament, fear, depres- sion of spirits, palpitation of the heart, restlessness, anxiety, aurse from the ovaries or stomach, paramnesia? of various sorts (tinnitus) eructa- tions, globus, visual mistiness and. partial clouding of the mental facul- ties. After this premonitory stage follows in a short time the second or epileptoid stage. The consciousness becomes still more obscure and perceiving, as it were, its gradual loss of inhibitory control over the lower or subconscious faculties, it becomes involved in a struggle in which it is more or less conquered. In almost every particular, except the suddenness of onset and the completeness of unconsciousness, this second stage of grand hysteria resembles an epileptic attack. It has been called hystero-epilepsy. It is pure hysteria and has nothing in common with epilepsy but the resemblance. The patient falls to the THE NEURONIC DISEASES 121 ground, the eves are closed, the head is rigidly drawn backward or to one side, the muscles of the jaw are clenched, the tongue is protruded and bitten, the face is red and then cyanotic, asphyxia seems imminent, arms and legs are strongly extended and adducted, the hands are tightly closed, the back is arched and the whole body is rigid and stiff. In a few minutes this rigidity passes off and a stage of clonic spasm follows. The face assumes horrible grimaces, the limbs and body un- dergo rapid alternations of rigidity and relaxation, the patient rolls from side to side or makes salaams or throws her legs into the air and does all kinds of absurd and purposeless things. She cries, screams, laughs, bellows, barks, scolds, yells and seems to be in a perfect de- lirium of mind and muscle. There is in all this a slight return of the mental faculties, but the consciousness is far from being awake, only realizing as it were its FIGURE 33- H vstero-epilepsy. Arc de Cercle. (After Richer.) powerlessness and want of control over the subconsciousness. It struggles to regain its normal ascendency, but failing to do so, the patient passes into the third stage in which the subconscious dominates and controls the physical movements, not in the delirious and spasmodic way of the unconscious epilptoid stage nor in the rational, purposive way of the fully awakened higher consciousness but in a hazy, semi- rational, dream-like manner that harmonizes perfectly in all probability with the ill-defined, ill-regulated, evanescent, incongruous multitude of hazy ideas floating en masse here and there through the patient's sub- consciousness. This is the period of the passionate (plastic) attitudes. The hallucinations of which the patient is the victim, may almost be positively suspected by the attitudes she assumes. Fear, auger, ecstasy, eroticism, meditation, religious emotion, jealousy, love, hate, are all marvelously depicted. The ecstatic and divine possessions of the mediaeval saints, nuns and monks, the enraptured attitudes often painted by the great artists in their pictures 'if the early fathers of the church. and all that class of tales, biographies, legends and art in which high and ecstatic emotionalism is represented, is now explained upon a hvsterical basis. 122 THE NEURONIC DISEASES The hallucinations of the patient in this third stage of grand hys- teria may be modified by external stimulation through any one of the senses. She is in a sort of hypnotic state, much as a normal person is when half asleep. Inpouring impressions are received and acted upon as if they were original. Finally, as a last stage, there follows a quiet delirium as the pa- tient is emerging from her subconscious condition into one of full consciousness. In this terminal delirium certain animal hallucinations play the most important role. When the entire attack has passed off there may remain some disturbance of sensation, movement, etc., for a time. These attacks may last altogether from ten to thirty minutes. When they follow one another with great rapidity for several days, the condition is known as status hystericus. Comparatively few cases of grand hysteria, as described above, are seen in this country, or outside of France and the Latin countries. FIGURE 34. Hystero-epilepsy. (After Mills.) Here the attacks are more apt to be mild and the symptoms more or- ganic-like and localized. There may be slight premonitory feelings of depression and exaltation, irritability, restlessness and gastric sensa- tions, which, however, are hardly ever obtrusive enough to deserve the name of aurae as it is used in epilepsy. Then follows the emotional- ism shown by crying, laughing, screaming, attitudinizing. Pains and hyperesthesia are loudly complained of. Tremor and vibratory move- ments involve the whole or parts of the body. Even local paralysis and contractures may appear. All of these manifestations occur under complete consciousness, with more or less volitional preservation and with great changeability. A sudden change in the patient's mental state may cause them to change suddenly or disappear entirely and the attack be thus brought to an end. On account of this, willfulness and obstinacy seem to be at the bottom of the patient's doings, and the attendants becoming thereby" angry and impatient, she is created with some form of punishment. This is a serious error and shows that the attendants have no real con- ception of the nature of the disease. The harsh and painful methods so often adopted in the management of these cases produce their results THE NEURONIC DISEASES 123 not by reason of their punitive characteristics, but by reason of their influence upon the diseased mentality. The tremor of hysteria is peculiarly frequent and characteristic. It may involve the whole body or only the extremities. In the more common forms the oscillations (five to seven a second) are rapid, rhythmical and of large amplitude. The tremor may vary so as to simulate paralysis agitans, or it may assume the appearance of the jerking movements of multiple sclerosis, chorea or myoclonus. Hys- terical chorea is pure hysteria that is sometimes supposed to be chorea. Various forms of local paralysis or limited paresis are met with in hysteria. A close stud}' of the paralysis reveals its psychic origin, for it seems to be clearly due to a sort of aboulia, the loss of will power, FIGURE r?. Hystero epilepsy. (After Mills.) however, being itself due to the loss of mental conception of motion. It disappears under ether narcosis and electrical stimulation. It i> changeable and is never accompanied by atrophy or other trophic symp- toms. Instead of being a complete paralysis it is more often a kind of loss of energy. The grasp of the hand is feeble, but changes even while the attendant is holding the hand. The weakness is volitional in origin, as shown by the fact that automatic movements are often retained. All forms of organic paralysis, monoplegia, hemiplegia and para- plegia may be simulated in hysteria. The monoplegias are probably the most frequent, the hemiplegias the lea- .inic paralysis is best differentiated by exclusion, though as a rule there will be slight varia- tions in hysterical paralysis that upon very careful examination will I2 4 THE NEURONIC DISEASES render its diagnosis usually easy. For example, hysterical hemiplegia scarcely ever includes the face and tongue, as organic hemiplegia usu- ally does ; and the contracture upon the hemiplegic side may be in the reverse direction from what it would be in organic disease. In the consideration of the various organic paralyses, further details in regard to their differentiation from the hysterical form will be mentioned. In all these paralytic conditions the knee-jerks are never lost; as a rule they are increased. Hysterical aphonia is a paralytic symptom so common as to de- serve special consideration. The voice being so closely involved in the mentalization of the patient, its loss is one of the most frequent forms FIGURE 36. Hystero-epilepsy. (After Mills.) of hysterical paralysis. The paralysis is usually sudden in onset, com- plete, and involves both vocal cords. It is volitional in origin, as shown by the fact that in singing, counting or in any other form of automatic vocalization, as well as in coughing and hawking, it is sometimes mo- mentarily restored. Usually the patient can only whisper. At other times the paralysis is not so complete, the voice being altered and rendered harsh and unnatural. In rare cases even the power of whis- pering is lost. This is known as hysterical dumbness or mutism. Un- like aphasics, a hysterical mute preserves more naturally her panto- mimic actions and cannot even articulate a single word. Close to hysterical mutism, either preceding or following it. is hysterical stutter- ing. Hysterical deafness has also been observed with the mutism. The ocular paralyses of hysteria are the subject of much dis- cussion. Ptosis, or pseudoptosis, due to a spasm ^i the orbicularis palpebrarum is not uncommon. Sometimes the eyelid is kept widely open by a spasm of tin levator palpebrae. The diagnosis of these con- ditions from organic paralysis is difficult, but powerful psychic sug- gestion, with the aid of electricity, will often diagnose correctly and cure the paralysis. The muscles of the eyeball are rarely paralyzed. ( )ppenheim says they are generalh simulated by contracture of the antagonists, as, for example, spasm of the internal rectus, causing an THE NEURONIC DISEASES 125 appearance of paralysis of the externus. He further declares that in his experience a true hysterical paralysis of the Ocular muscles never occurs. It is admitted by others, however, that a myosis, paralysis of the muscles of accommodation, and pupillary rigidity may occur. Monocular diplopia or polyopia may result from closure of one eye. or defective accommodation. It may be associated with micropsia or macropsia. As in all hysterical paralyses, these of the eye are char- acterized by their lack of permanency, their alteration under psvchic influence and their association with other hysterical manifestations. Dysphagia, with or without disturbance of articulation, is an oc- casional symptom of hysteria. It is due to paralysis of the velum FIGURE 37 Hysteric flexion contracture of one leg. (After Richer, taken from Tourette's work.) palati and deglutitorj muscles. It is generally complete and must therefore be most carefully distinguished from organic paralysis. Diaphragmatic paralysis is rare in hysteria, though it is often simulated by malingerers. Cases have been reported by Gilles de la Tourette, Petrazzini and others. It i< very alarming sometimes. Ac- cording to Wernicke, there is an insufficiency of the diaphragm, which is common in both hysteria and neurasthenia. In my opinion it belongs to the latter neurosis, which, as is well known, sometimes complicates hysteria. Paralysis of the bladder is very rare. The retention of the urine and the occasional dysuria are due to hysterical spasm of the sphincter vesicae. Disturbances of the Alimentary Canal. — Loss of appetite, anorexia hysterica, is sometimes a serious condition, for if not overcome after a time, it will lead to inanition and even death. It sometimes altera with excess of appetite or bulimia. Eructations of gas from tin- stom- 126 THE NEURONIC DISEASES ach are not uncommon, especially in cases of hystero-neurasthenia. Vomiting is more frequently a purely hysterical manifestation than are the eructations. Mucus and saliva are expelled, usually with the food that has just been swallowed. In spite of this the appetite and general physique do not fail. Stercoraceous vomiting may occur and according to Fazio may be spasmodic or paralytic in origin, but is not due to sim- ple antiperistalsis. The vomiting may not be so purely a hysterical symptom in some cases as it is the result of the ingestion of nauseous substances, like urine, taken purposely to provoke sympathy. As a rule vomiting is not a particularly dangerous symptom in hysteria, whatever may be its origin. Both constipation and diarrhoea are observed in hysteria. The former is so common as to have been considered a predisposing cause, chiefly through autointoxication. A temporary diarrhoea, after some violent emotion, may occur in hysteria as well as in some normal states. Abdominal distension, known as meteorism and tympanites, with intestinal gurgling and borborygmus, occur in nervous people and may be very annoying in hysteria. The distension often subsides without the evacuation of the gas that caused it. Disturbances of the Circulatory and Vasomotor Systems. — In the paroxysm the pulse is apt to be accelerated. When there is syncope it is retarded. Cardiac palpitation is usually a mere subjective sensation and may be associated with difficulty of respiration. Under high emo- tion the palpitation may be real. The purse often remains normal. Spontaneous haemorrhages from various parts of the body of a hys- terical patient should be looked upon with suspicion. As a rule it is brought on by secret means to provoke attention. This is confirmed by the fact that such haemorrhages rarely occur from the internal organs which are beyond the reach of the patient's machinations. It is de- clared, however, upon good authority, that spontaneous bleeding from the forehead, breast, foot and other superficial parts of the body does occasionally occur. Metrorrhagia may be very profuse, but curiously the general health is but little affected. Much more common, reasonable and objectively demonstrable is the occasional local asphyxia sometimes associated with hysterical anaesthesia. Oppenheim once excised a piece of muscular tissue from a hysteric without any. bleeding. Vasomotor spasm is the explanation of this phenomenon. Pallor, flushing, cyanosis may all be observed, often in rapid alternation in the same patient. Souques has reported a case of hysterical Raynaud's syndrome, in which mere suggestion with a placebo caused the paroxysms of local asphyxia, which up to that time had averaged eight a day, to disappear entirely. Of course. in this hysterical type of Raynaud's disease, or syndrome, gangrene does not occur. (Edematous-like swellings, non-pitting, with decrease! local temperature, are sometimes seen. On account oi the (edema, pain on movement, etc., a hysterical joint may closely simulate joint- disease. The vasomotor disturbances may be responsible largely for the associated hyperidrosis, polyuria and even diabetes insipidus that occasionally take place. Hysterical skin affections are vasomotor in character. Gangrene has thus been seen to follow an urticaria, an cry- THE NEURONIC DISEASES I2J thema or more frequently pemphigoid blebs. Occasionally a gasteri- form eruption was the first symptom (Van Harlingen). The very nature of the disease, with its exalted emotions and imagination, would presuppose frequent disturbances in the sexual sphere. Doubtless this is the reason why the disease was so long re- garded as primarily due to sexual aberrations. The effect was mis- taken for the cause. Impotence and loss of sexual desire may be due to local anaesthesia. With a local hypersesthetic condition, or without, there may be abnormal desire amounting almost to nymphomania or satyriasis. Perversions and semi-hypnotic delusions in this sphere are not only possible, but are so common that no physician who regards his own comfort and reputation would think of examining a hysteric without the presence of a third party. Hysterical fever has been reported. As a rule it is the product of some form of deception. If it does occur it must be exceedingly rare. Hysterical attacks as a rule do not occur during the rise of the temperature. If changes in the pulse, respiration and renal secretion accompany the elevation of temperature, there is probably some obscure inflammatory -condition present. However, pure hysterical tempera- tures of 108 and 113 degrees F. have been recorded. A large question mark is to be put after the statement that Teale once found the tem- perature in a hysterical patient 122 degrees F. They are said to be characterized by large daily curves, to be but little influenced by the usual antipyretics, and to vary considerably when taken in different parts of the body. Pathology and Pathogenesis. — Much confusion surrounds the pathology and pathogenesis of hysteria. This is not because we have not complete data upon the disease, but because there is so much differ- ence of opinion as to the nature of psychic processes, both normal and abnormal. It is practically universally admitted nowadays that hysteria is a psychic manifestation with secondary neuro-muscular phenomena. It is primarily and essentially a psychosis. This psychosis is dependent of course upon the nature and course of the patient's individual ex- periences as brought to her through and by way of her five senses. Hence the role played by these senses in the causation of the disease and the long list of physical etiological factors that have hitherto been assigned at various times as the cause of hysteria. Up to the present time the pathology of hysteria has been made to include almost every possible alteration in about every possible organ and tissue of the body. One thing is certain, namely, that there are no constant pathological findings of any sort, and in all of the cases there are no changes in the central nervous system upon which a pathology can be erected. For this reason some deny that hysteria should be considered as a disease at all, looking upon it as a mere mental and nervous disturb- ance caused by the changes and diseases in any or all of the tissues of the body. On the other hand, some authorities regard it as the result of some hypothetical changes in the molecular substance of the nejve cells, causing them to manifest an abnormal functional activity, which clas fies hysteria as a dynamic disease without discoverable lesions. 128 THE NEURONIC DISEASES In both of these explanations it will be noted that a physical basis, a pathology in the general sense of the word, is assumed for the dis- ease. It is a strange fact that the great variability, instability and sud- den changeability of the clinical manifestations of hysteria have not long since led the authorities to relinquish the idea of a physical basis for these changes. So materialistic is the average trend of thought that men find it hard to conceive of a disturbance of mentality without an underlying change of physical structure. This is scarcely to be won- dered at, for logically it would seem to be a necessity that every change of action presupposes a change of some sort in the acting substance. And yet there are ways by which activities may be changed and the underlying substance remain both physically and functionally undis- turbed. This is true not only in certain abnormal states, but is open to daily observation in perfectly normal states. Mental activities are complex in character and depend upon the play of their elementary constituents upon one another. These constituents may be acting in- dividually in the way that they should in all their normal perfection, but by means of an exaltation of some and a diminution of others or even by a simple rearrangement among them, the resulting complex and final activity may be very different from what was expected, or be sud- denly changed from what was at that moment in performance. A rough illustration may make this clearer. Suppose in a great railroad office all the employes are doing their usual duties in their usual way ; the great complex system will move in the usual way. The elements of the system, the individuals, will be working normally, and so the sum total of activities, the working of the entire railroad from end to end, will be normal. Suppose, however, some unusual factor is introduced among the activities of the individual employes ; suppose the president of the company or the manager, with all his authority to command, should give to one of these individual employes an erratic order to pass on over the system. The employe takes the order and does with it in his usual way what his chief told him to do; so does the next employe and the next; until finally the whole system is sud- denly disturbed in its usual routine of activity. The individuals, even the chief, were normal in structure, were well within the limits of their functional activity, and yet by an unusual play of these activities as individuals upon one another, there has resulted a most unusual work- ing of the entire complex activity of the whole system. Hysteria is a psychosis primarily and essentially. It involves in its last analysis a disturbance of the mental faculties — namely, the memory, the imagination, the will and the intellect. These so-called faculties, as well shown by that great teacher of modern physiological psychology, Wilhelm Wundt, are not actual entities or things with a physical habitat in the brain or elsewhere. They represent merely processes or forms of activity. They are merely convenient names for particular kinds of complex and compound movements. Accord- ing to Wundt, sensations and feelings are the primary and only ele- ments of all psychic manifestation. These sensations and feelings are dependent, of course, upon the physical changes in the nerve cells and fibres. These sensory and aesthetic phenomena combine or separate THE NEURONIC DISEASES I2Q among themselves in such a way as to produce that complex activity which we denominate the mind. The feelings, by becoming stronger, develop into emotions ; emotions overflow into muscular action if they are strong enough, and we call the process volition. Percepts are awakened by sensory stimulation and being brought into juxtaposition •or combined with other percepts form concepts. The comparing and contrasting of concepts is the process that underlies imagination, un- derstanding and judgment. Thus the whole mind is formed by the interplay of certain ele- mentary processes based upon sensation and feeling. These sensations and feelings may, of course, be disturbed by physical lesions in or about the nerve elements and the processes which underlie psychic action will be disturbed. This is the explanation of the way in which physical causes often give rise to hysteria, as well as to many other forms of mental trouble, such as polyneuritic psychosis, the psychoses of the infectious diseases, etc. But it is evident from Wundt's point of view or from the stand- point of modern voluntaristic psychology, that the mental faculties, so-called, may be disturbed by a disturbance in the mere interplay of the elementary, constituent processes that make up these faculties. These elementary constituents may not necessarily be abnormal indi- vidually, but if some of them be thrown into a state of unusual activity, the routine balance between them ail is so far disturbed as to cause a marked disturbance in the sum total of all their activities — namely, the mind. This explanation makes of hysteria a psychic disturbance de- pendent upon either physical or functional causes. The physical causes may lie in disease of the ultimate nerve elements, thus changing the elementary sensations and feelings that form the ultimate substrata of mind. ( )n the other hand, the functional causes may reside in an unwonted disturbance in the usual interplay of what may be quite normal sensations and feelings. Among the physical causes there may be found all sorts of lesions giving rise to hysteria. For this reason physical diseases of all kinds may provoke among those neurotically unstable an outbreak of hysteria. On the other hand, fright, sorrow, excess of joy, mental strain, may develop into hysteria in those neurotically unstable, not because these mental states are abnormal, but because they are so unusual as to dis- turb or at least represent a disturbance in the usual form of mentaliza- tion. No explanation of hysteria, in which disease there is admittedly no pathological findings, so harmonizes all the clinical phenomena and shows the half-truth contained in most of the explanations hitherto offered, for example, the old view that it was due to a paralysis of the will is true in part only. In some cases aboulia is present because the psychic process which we call the will and which is a product of the emotions, which emotions in turn are dependent upon the elemental feelings, is disturbed by the disturbance in these underlying proces Certain feelings are too weak or too strong; the corresponding emo- tions have caused the rise ( >f weak or altered motives; the resultant motor activity fails entirel) or is entirely perverted, [anet speaks of a "contracted consciousness" underlying hysteria. In some cases this I30 THE NEURONIC DISEASES may be ttue, just as the volition process may be rendered paretic. Others have thought that hysteria was due to an excess of mental inhibition. It is, in some cases. Still ethers find that emotionalism and an excess of imagination enter most largely into its pathogenesis. In some cases they undoubtedly do. And finally, not a few believe that it is merely the result of a vicious education and discipline in a neu- ropathic and unstable individual. In a large percentage of cases this also is most emphatically true. In all of these views, however, it will be remarked that the trouble is primarily a pure psychosis. The explanation which we have given, based- upon a rational and physiological explanation of mind, affords the best generalization for the harmonizing and clarifying of all these apparently diverse but really disjointed parts of one and the same view. Diagnosis. — In an ordinary, uncomplicated case, hysteria is not difficult to detect. The large majority of the cases, however, are not of the ordinary type or uncomplicated ; hence the diagnosis of hysteria is nearly always a matter demanding caution, thoroughness and experi- ence. It should always be diagnosed by the exclusion of all other pos- sible functional and organic diseases ; and in arriving at the diagnosis the psychic origin of hysteria should never be lost sight of. Even though the most careful and experienced physicians may be at times entirely at sea, the remembrance of the two facts just stated will go a long way toward making a diagnosis accurate. The mental origin of the motor, sensory and other symptoms of hysteria can nearly always be determined by the character and loca- tion of these symptoms and their alteration when the patient's attention is distracted, or his mind is put out of action, as it were, by chloroform or ether narcosis or hypnotism. An experienced examiner sometimes learns to detect hysteria almost intuitively, and this is not altogether surprising. Psychic manifestations of all sorts, or rather symptoms of all sorts that owe their origin to the mind of the patient, when collected and presented en masse, present a more or less characteristic picture differing from a collection of symptoms that owe their origin to simple organic changes in the nervous structures. The voluntaristic explanation of mind — namely, that it is merely a term to cover certain interdependent and interrelated processes among the elementary sensa- tions and feelings — accounts for the variability, distribution, change- ability and other peculiar characteristics of the symptom-complex of this psychic disease, hysteria, and emphasizes, on the other hand, the fixity, the anatomical distribution and the greater permanency and continuity of the symptoms of the organic diseases with which it is liable to be confounded. The psychic nature of hysteria makes plain why it is so often associated with organic diseases. For its activity, mind is dependent upon the activity of its primary elements, the sensations and feelings^ and these in turn are dependent upon the action of the histological nerve and other tissues of the body. Given an organic disease or dis- turbance of any kind anywhere in the body, and it is only a question of temperament, time and severity of this disease before a psychic dis- THE NEURONIC DISEASES IJI turbance will be provoked that may eventuate in a more or less severe attack of hysteria. One must not get the idea from this that hysteria is only a mental disturbance symptomatic of some organic disease, for such a notion would only represent a half truth. The mind is the result of two sets of underlying factors. On the one hand it is de- pendent upon nerve integrity and their normal sensations and feelings ; but on the other hand it is a process, a mere form of activity, dependent upon the normal interplay of the elementary processes of sensation and feeling. Hence, not all cases of hysteria are the product of gross organic diseases or are even associated with them. The association of the two diseases may obtain even without any relationship other than the mere association. All these facts must be taken well into account when differentiating hysteria from organic diseases and emphasize the importance of diagnosing the hysteria by the exclusion of every other possible disease. It is safer to overlook a hysteria than it is to pass organic symptoms as being hysterical. The condition of certain organs that are entirely beyond the control of the mind, as, for instance, the pupils, the heart and respiration, etc., may all be invoked to exclude hysteria. Probably the disease that is most frequently mistaken for hysteria is multiple sclerosis. Indeed, Westphal described a general neurosis, classified by Charcot and his school as a form of true hysteria, which could not be distinguished from multiple sclerosis either in its course or its symptoms, and which he called pseudo-sclerosis. True multiple sclerosis, however, may be distinguished from either hysteria or West- phal's pseudo-sclerosis by the presence of nystagmus, the condition of the optic nerve, the voluntary intentional tremor and the absence of psychic influences. The scanning speech of multiple sclerosis is more uniform and consistent than is the irregular speech sometimes observed in hysteria. In the latter disease some words are scanned and others are merely jerked out irregularly, quickly or slowly. The paraplegia of multiple sclerosis usually reveals a suggestion of spasticity, and clonus when present is absolutely involuntary, regular and intense. Remember, however, that hysteria and multiple sclerosis are often associated, and one must be careful to determine the symptoms that belong to either of the respective diseases. In brain tumor and cerebral syphilis there are symptoms that hysteria at times closely simulates. Moreover, both conditions may develop or at least be associated with hysterical phenomena. An error in diagnosis is therefore not uncommon. When there are the usual ophthalmoscopic signs of tumor or syphilis present, there can be no possibility of mistake. The headache of the organic troubles is differ- ent from that of hysteria in being more continuous, less sharp, more definitely localized and more prominent in the early hours of the morn- ing. With the cephalalgia of the organic diseases the mind is heavy, dull and apathetic, the countenance is expressionless and the pulse is retarded. There may be vomiting, Usually the other symptoms of hysteria are sufficiently pronounced to render a differential diagn clear. Hysterical paraplegia may be mistaken for the paraplegia of 132 THE NEURONIC DISEASES myelitis. In the latter disease, however, the character and distribution of the sensory disturbance, as well as the bladder and rectal troubles, are sufficient to differentiate the two diseases. In myelitis the absence of all psychosis and the non-implication of the special senses are to be carefully noted. In the same way vertebral caries may be distinguished from hysteria, the sensitiveness of the former disease being more cir- cumscribed and less like a cutaneous hyperesthesia. Hysterical joints are often mistaken for arthritic disease. A careful examination of the joint for organic disease and of the patient for other hysterical stigmata will make the distinction clear. Hysterical pains ought not as a rule to be hard to differentiate from neuralgic pains or pains due to organic disease. One has but to remember that hysterical pain is a psychic phenomenon and therefore has no definite, fixed, anatomical location as have the pains of organic disease. On the other hand, the latter pains are not so susceptible to suggestion and diversion of the attention as are the psychic pains of hysteria. The other symptoms, both of hysteria and organic disease, are usually sufficiently in evidence to give very material assistance in the diagnosis. The pains of angina pectoris, cephalalgia, etc., may thus give rise to some confusion, but a careful application of the above principles in making the differential diagnosis will render the latter less difficult. The same may be said of the differential diagnosis between hys- terical and other forms of spasm, such as chorea, tetany, hystero- epilepsy, etc. The psychic complexion of the hysterical spasm is want- ing in the muscular phenomena of these diseases. Hysterical chorea, so-called, is a rhythmical movement, slower and more regular and not so incoordinated as are the jerkings of true chorea. Chorea magna, as I have explained elsewhere, is nothing but hysteria. Likewise, hystero-epilepsy is merely a severe form of hysteria in which the state of consciousness and convulsions resembles somewhat that of epilepsy. As there is no connection between hysteria and epilepsy but this superficial resemblance in a few cases, it would be well to drop entirely the use of the term hystero-epilepsy. It is as misleading and unwarranted as the term typho-malaria. Hysterical patients often become neurasthenic and vice versa, f >ccasionallv both troubles coexist in the same individual without any other connection. They both have their distinctive signs, which should always be carefully separated, as I have pointed out under the head of neurasthenia. Hysterical vomiting has not infrequently given rise to the errone- ous diagnosis of gastric ulcer, peritonitis, perforation and appendicitis. As a rule hysterical vomiting takes place so soon after the swallowing of food that the latter comes up quite unchanged. The absence of characteristic pains, elevated temperature, local tenderness and the general history and psychic nature of the trouble, will help to deter- mine easily the hysterical origin of the vomiting. Oppenheim points out that there are persons with a congenital (perhaps due to a developmental inhibition) or early developed weak- THE NEURONIC DISEASES 1 33 ness in some particular part of the central nervous system, say the cerebellum, the medulla or particular cortical centers. Later in life these fail to functionate normally and disorders arise that are not strictly organic nor yet are purely hysterical. They may cause con- fusion in diagnosis, however, and should be guarded against. It is undoubtedly true that there is a strong inclination in hys- terical patients to practice deception. The exaggeration of the egotism with its necessary tendency to selfishness, the diminution in the inhibi- tory influence of the intellect and higher faculties and the consequent overactivity of the emotions, imagination and more elementary facul- ties, lead to the adoption of means and measures to accomplish ends that are not on a very high plane of justice and morality. A moment's consideration will convince one, however, that such deceptions are not criminal, as they would be if resorted to by a normal or non-hysterical person. Psychically the hysteric individual is a child ; mentally she has fallen back to an almost ape-like condition. To punish her in a vin- dictive spirit is both cruel and unjust ; to administer corporal pain with the hope of reforming her is useless and unscientific. Her mind is at fault. It is diseased. It works differently from the normal mind. It is under the control and influence of abnormal, strange and bizarre percepts and concepts. On account of this it may be acting most logically though the outward manifestations appear so extraordinary and provoking. The partial consciousness, the emotionalism, the screaming, the dramatic attitudes, the pugnacity may all be due to hallucinations and illusions of a frightful character, or to unusually active memory and imagination processes rather than to any nerve influences. The deceptions of true hysteria may be identical with the deceptions of malingerers. The difference between the two, however, lies in the underlying motive, and the cause and origin of that motive. In both it is a strong desire for some form of self-gratification. In the hysteric, however, it is usually a desire to gratify the mere personal ego, the love of self, sympathy, praise or some other form of spiritual self-aggrandizement. In the malingerer it is a desire for the acquisi- tion of some physical enjoyment, money, physical comfort, presents, etc. In this sense "hysterics" due to mere bad discipline, is a sort of mild form of malingering and being not the same as true hysteria may, unlike the latter, be treated at times advantageously by severe punitive measures. True malingering may be distinguished from true hysteria 1>\ various symptoms and measures, one of the best of which is chloroform or ether narcosis. Spasms of hysterical origin disappear and the sensory reflexes undergo change as the narcosis deepens. The paraly- sis may be tested by electricity, rlysteria being a psychosis, its mani- festations are so largely subjective in character that the examiner has but few objective signs by which to determine whether the patient is feigning or not. Nevertheless, it takes an exceptionally shrewd in- dividual to imitate all of the symptoms of hysteria or even a part of them, and to portray them with all their variability, changeableness, peculiar distribution so that they will continuously and constantly depict the picture of true hysteria. It is much easier to simulate organic 134 THE NEURONIC DISEASES disease than it is hysteria ; so that if the simulator of hysteria be closely watched he will portray some of his symptoms, such as the paralysis, contracture, etc., in a way that will resemble organic disease rather than hysteria. The moment he does that his game is lost, for the symptoms which simulate organic disease, as, for instance, fixity of the paralysis, anatomical distribution of the anaesthesia, the vomiting, etc., do not fit in well with the other symptoms that he is feigning, and therefore it is evident that he is afflicted with no disease, either organic or functional. As I have before intimated, hysteria is always diagnosed best by the exclusion of all other possible diseases, because it is possi- ble for it to imitate all other diseases so closely ; and by a careful study of its general complexus rather than of its individual symptoms alone, because it is a disease of the personality, a psychosis, and not a disease of special anatomical structures. Prognosis. — The prognosis of hysteria in regard to life is good. In regard to recovery it is favorable for the attack, unfavorable for the general constitutional trouble. Hysterical conditions secondary to organic disease depend upon the removal of these organic lesions. The patient's congenital, physical and mental constitution, her age, her environment, all must be taken into account in determining the possibility of recovery. Medicine cannot hope to alter natural traits and inherited defects. It can do much, however, to mitigate their effects. Exciting causes it can often remove and consequent bad habits it can change. While a physician may offer a generally favorable prognosis in regard to particular factors of hysteria, as, for instance, the recovery from a sudden acute outbreak, he should frankly admit the probability of relapses and he must be very guarded in holding out hope of a complete and permanent disappearance of the disease. In a small number of cases, complete and permanent cure has been ob- tained. Treatment. — In the treatment of hysteria the guiding thought should always be its psychic nature. It is primarily a disease of the mind; in a large number of cases it is the result of a vicious habit of mentalization. This must be modified in some way before any real benefit from treatment can be hoped for. All forms of mental disci- pline are, of course, most effective in the impressionable period of childhood and their most brilliant results are secured in hysteria when adopted as prophylactic measures. Nevertheless, mental discipline and psychic treatment are imperatively called for by an hysteric at all times, whether young or old, whether during the convulsive attacks or during the intervals between them. In other words, first, last and all the time the sheet anchor in the management of hysteria is the psychic treatment. Typical hysteria occurs at all ages and in general exhibits itself in the same way in children as it does in adults. The treatment of the hysteria of childhood differs in no special respect from the treatment of hysteria in the adult. The hysteria of the adult is, as a rule, the expression of a peculiar form of constitution, and usually is preceded by certain premonitory indications in childhood that should put the physician on his guard and THE NEURONIC DISEASES 1 35 urge him to give some advice to the parents as to the education and training of the child. Of course, the science of medicine cannot change the peculiar, inherited temperament which forms the background for the later hysterical outbreaks, but it its teachings are listened to and followed it can do wonders in forestalling these outbreaks and in keeping latent the ever-threatening tendency towards them. The whole field of child training, including a knowledge of psychology and mental development, of pedagogy and educational discipline, and of the psychological law T s of growth and evolution, is an exceedingly broad one. Either because parents, and alas, too often the family physician, are unwilling or unable to prepare themselves for so extensive a sphere of thought and activity, they fail to manage hysteria either from the prophylactic or the therapeutic standpoint. Unfortunately, much of the advice given under the head of the treatment of hysteria in text- books of even very recent issue is erroneous, because of the erroneous conception of the true nature of hysterical physical phenomena and the peculiar aberration those phenomena undergo in hysteria. Without occupying too much space here, let one illustration suffice. Nearly all the text-books suggest that hysteria, whether in the child or in the adult, is largely an aboulia and that therefore the patient's will should be strengthened in every possible way. As a matter of fact, the voli- tion of the patient is not paralyzed so much as it is perverted. Hys- terical patients only too often show a most vigorous will-power in the line of their perverted motives. Even when they are paralyzed or in a cataleptoid state, the will is not so much in abeyance as that it is working, perchance, too vigorously, in a direction entirely different from the ordinary channels. An overwrought imagination with reminiscences of intense fear, or of religious ecstasy or of sexual exalta- tion, may so absorb the patient's inner consciousness that the ordinary channels of mental activity are quite neglected and the patient is there- fore the apparent victim of such outward negative manifestations as anaesthesia, paralysis, etc. In the light of modern physiological psychol- ogy, so ably taught by Wundt, in which all mental phenomena are but composite psychical experiences resulting from the interplay and inter- connection of the two elementary processes, sensation and feeling, hys- teria like all other forms of psychosis, is the expression of an unusual, and so far unnatural, balance and harmony between these elementary processes. Its cure is the restoration and preservation of this balance, the establishment of the normal, or what is universally accepted as the normal, interplay of the primary processes or occurrences that go to make up the so-called memory, will, imagination and intellect, by the regulation and discipline of the physical elements, sensation and feel- ing. This conception of the nature of hysteria on the basis of modern physiological psychology, while it does not lessen the magnitude of the task involved in its prevention and treatment, gives to the task a more hopeful and rational ground whereon to work, than any explanation hitherto afforded. To ward off hysteria in one who has been born of neurotic parents, who shows in herself early in life a congenital neuropathic tempera- ment, or who has begun to reveal hysterical manifestations as the re- I36 THE NEURONIC DISEASES suit of a too close association with a hysterical mother or of a too close confinement to a predisposing environment, the indications are clear and involve the making of all such changes in the child's life as will change her psychic elements of sensation and feeling. Removal from home, the avoidance of too much sympathy, the regulation of studies and reading, the adoption of system in all life's activities, and, in fact, the employment of all measures, persuasive, punitive, educa- tional, social, disciplinary and of every other kind that will tend toward a regular, even, systematic, well-balanced mental and physical existence, in which no set of sensations, no set of feelings, no special faculty or organ of the mind or body is exercised at the expense of or more than any other. As most of these children, for example, are of the so-called artistic temperament with its preponderance of the imaginative and emotional faculties, they should be given games, studies and a general regimen that involve little or no exercise of imagination and feeling, but rather the intellect and reasoning faculty. The exercise of the imagination and emotions should not be prohibited entirely, as is too often done, for that merely reverses the unbalanced state of mental activity ; but they should be kept in partial abeyance by the giving of more work to the other faculties. Passing on to the consideration of the treatment of hysteria in the adult, the subject may be looked at from various standpoints. In the first place, the same sort of prophylactic treatment and discipline for the suppression of the disease is as applicable as it is in the child : its results, however, will not prove so happy because the adult cannot be controlled as a child can, and too often will not submit to proper self-discipline, and because in the adult the hysterical habit has usually become fixed more and more firmly m the course of the preceding years. Nevertheless, given a willing patient, prophylactic discipline along the lines suggested for neurotic children will do a very great deal in pre- venting the hysterical outbursts or in mitigating their severity when they have once broken forth. A volume might be written almost upon the details of such prophylactic treatment, for it would involve the consideration of all kinds of cultured and uncultured individuals and all forms of mental and physical activity known to the human race. The whole matter may be axiomatically slated in the one sentence, restore as far as possible the balance in the mental, moral and physical activities of the individual patient to whom the advice is being given. Physical diseases with which hysteria is so often associated must, of course, be removed. Ill health must be corrected with tonics, exer- cise, massage, baths, electricity, proper diet, sleep, etc. The mind must be appropriately ministered to as well as the body. All of which can be only suggested here on account of the vastness of the subject. In the second place, the treatment of the hysterical outbreaks. the paralytic and convulsive periods and their special symptoms, needs some consideration. The means at our disposal for combating this manifestation of hysteria are psychic, mechanical, medicinal. 1 am so convinced that hysteria is entirely a psychosis that I believe what benefit we obtain from such measures as are involved in all the hydropathic, climatologic, medico-mechanical and electric procedures THE NEURONIC DISEASES 137 that have been recommended and are legitimately employed, is due to the effect upon the patient's mind. Of course, I do not mean by this that remedies applied to organic troubles with which the hysteria may be associated do not help to remove the psychosis, but they do it indi- rectly by removing the Organic disease which may be aggravating the hysteria. In a word, if a case is one of pure hysteria, free from all forms of organic trouble, the remedial measures that so often seem to banish the hysterical symptoms do so by directly influencing the pa- tient's mind. I take it that the entire treatment of pure hysteria is in its last analysis psychotherapy, whatever the means employed to influence the patient's mind. Even in an hysterical attack, therefore, the first thing to do is to win the patient's mind so far as possible. To do this the physician must first of all impress his personality, his mastery over the patient and the situation and his ability to handle the case, not only on the patient, but upon all those in her presence. He may have to be harsh or gentle, commanding or persuasive, indifferent or sympathetic ; each case will require its own method and the physician himself must alone decide what course of action to take. The patient's confidence must be ob- tained and after that there must be no temporizing with her. Ofttimes this can only be accomplished when the patient is away from her home and among strange surroundings. The hospital is the only place and the Weir-Mitchell method of seclusion, with a daily systematic pro- gram, modified, of course, for each individual case, is often the only means of obtaining the desired influence over the patient. The physi- cian must frequently impress the patient with not only his mastery, but also with the superiority of his mind and mental attainments. He dare not be boastful, but he must command the patient's respect, admiration and even awe. To do this he must often be a highly cul- tured man, for these patients are themselves frequently highly cul- tured. If the physician's mental endowments are such as to cause an accomplished hysteric to hold small respect for him, it is obvious that he will have but little of her confidence and so much the less mental control over her. It is well, therefore, to be able to meet the various tastes and mental endowments of these patients by a broad knowledge of science, literature and art. Moreover, out of such a broad knowl- edge, suggestions can judiciously be made in regard to the patient's reading, studies and artistic occupations. I make it a rule to include in the written program for the use of the nurse, the books to be read. the amusements to be indulged in, the general lines of conversation to be prohibited, and deem them quite as important as the directions which I give in regard to the medicines, food, baths, electricity, etc. Psychotherapy involves the question of the employment of hypno- tism and suggestion. What has just been said about the relationship of the physician to his patient is of the nature largely of suggestion. As to the use of artificial hypnosis there is some question. Hypii itself is a hysterical condition, and the substitution of one hysterical state for another may be inadvisable. Hence, hypnotism had better never be resorted to except as a final incisure after all other means have been tried and have failed. And yet it must be frankly admitted I38 THE NEURONIC DISEASES that for the removal of paralyses, anaesthesias and contractures, it is not only to be recommended but sometimes proves highly efficacious. One must be careful always to make appropriate post-hypnotic sug- gestions, so that the patient does not come out of her sleep in a worse hysterical state, with other and more serious stigmata, than those with which she entered it. I believe it makes very little difference in the treatment of hysteria as to the character, mode of application or time of employment of such excellent measures as baths, massage, electricity and gymnastics. The essential point is to insist upon their being taken with regularity and sys- tem, and not to allow the patient's mere whims and changeableness to in- fluence the use of them. To overcome her egotism and self-regard, to strengthen her higher mental inhibition, and to direct her thoughts into channels that lead away from herself and her own elementary sensa- tions and feelings, it is wholesome to render the taking of the baths, massage and electricity somewhat of a slight task, possibly unenjoyable but absolutely necessary. In this way cold baths, electric shocks, tire- some exercises, apart from their tonic and stimulating effect upon the physiological processes of the body, accomplish their happy effects when adopted as a part of the treatment of hysteria. In regard to the use of medicines in hysteria, it may be generally stated that they are of little avail. Hysteria is a psychosis which re- veals itself most prominently at times through the nervous apparatus ; hence it is sometimes called a psychoneurosis. This neurosis is seen in such symptoms as the muscular spasms, vasomotor disturbances, con- tractures and such automatic acts as laughing, crying, etc. To reach such conditions the antispasmodics are sometimes useful. Valerian and zinc are the best in the list. Asafoetida, the bromides, quinine, sumbul, methylene blue, may all be tried, but without psychotherapy will usu- ally be found wanting. Narcotics, such as opium and choral, must be rigidly avoided; when once administered to the patient, the comfort of the attendant is at an end. Chloroform inhalations may be used to relieve certain cases of convulsion and contracture. Means of combating special symptoms are innumerable and will readily suggest themselves to the physician who is fully cognizant of the purely psychic nature of the disease. As soon as possible hysterically paralyzed patients should be en- couraged to walk. The disappearance of special symptoms does not mean that the disease itself is cured. Remissions and relapses are to be expected in such a constitutional disorder. The means of avoiding these were in- dicated under the discussion of the prophylaxis of the disease. PSYCHONEUROSES THAT ARE RELATED TO SLEEP AND TO THE HYSTERICAL STATE. There are certain psychoneurotic states about which there is a great deal of confusion and much honest difference of opinion. I refer to all that class of phenomena which is variously spoken of under the head of hypnosis and hypnotism, dreams, somnambulism. THE NEURONIC DISEASES I39 catalepsy, ecstasy, lethargy, etc. Some authors speak of these as dis- orders of sleep; others rank them as manifestations of hysteria and mental aberration. They are not exactly normal phenomena, of course, and yet many regard them as merely disturbances of a normal function, sleep. Others look upon them as absolutely abnormal and in that sense not to be spoken of as related to sleep at all. According to the latter they are phases of hysteria, possibly evidences of a severe, or at least positive, psychosis of fundamental origin. In my own opinion much of this confusion is quite unnecessary and is the direct outgrowth of certain old-time misconceptions in re- gard to the nature of mind and in regard to the limitations or exact meaning of the words normal and abnormal. I presume there is no dispute in regard to the fact that both sleep and hysteria represent a mental change associated with and founded upon some physical or physiological change in the nervous mechanism. If we start from the standpoint of the state of wakefulness as we observe it in the average man, then sleep and hysteria are both anything but a state of normal wakefulness. The mind, the consciousness, if you please, is more or less in abeyance in both states. We speak of sleep, however, as normal and of hysteria as abnormal. Justly so, for though the terms normal and abnormal are very elastic, they are used not in the sense of repre- senting absolutely what is right and what is wrong, for human knowl- edge is not definite enough yet for us to assume that critical standpoint, but in the sense of representing what occurs in the average or majority of people. All animals sleep, therefore that is normal ; only a few animals have hysteria, therefore that is abnormal. Such is the logical method adopted by both the scientific and unscientific world. Up to a certain point it is a correct and safe method. There is a glaring weakness, however, in such reasoning when we strive to arrive at exact knowledge. Averages and mere numerical pre- dominence do not confirm facts. There must be other features to establish them as absolutely undeniable. They must be fixed and unalterable ; they must always be identical in every detail ; they must appear absolutely always the same under the same circumstances ; and we must know absolutely everything about those circumstances before we can pronounce the law that they are normal or abnormal. As so few of these conditions are made to surround many of the phenomena which we now call facts, our assertions in regard to the normality or abnormality of the latter are only approximately and pre- sumptively correct. By striking the average we come as near as we can in fulfilling the above conditions and that which seems to approxi- mate most nearly to abiding by them we call facts and declare them to be normal or abnormal. Sleep is such a normal fact; hysteria is such an abnormal fact. When we begin to verge away, ever so little, from the average, we at once find that our normal and abnormal facts are a little shaky and at once seen to be open to variation and exception. All men sleep, but how? Some longer, some shorter; some profoundly, some lightly. There are degrees of sleep then, and perhaps other characteristics about it that are not necessarily exhibited in the average. At once they be- I40 THE NEURONIC DISEASES come abnormal, these exceptional sleep variations. A man who sleeps a whole month or one who does not sleep at all for a month are both abnormal because the average man does not do either. But how about the sleeping itself, the prolonged abeyance of consciousness — is that abnormal? The prolongation of it, of course, is exceptional and abnormal, but so far as we can determine the sleep, the mere abeyance of the consciousness, is not abnormal. Again, few men have hysteria, but those that do have it exhibit also variations and exceptions in their manner of revealing it. There may be such a profound yet partial abeyance of the mental faculties that the patient for the time being seems to be in another world entirely ; on the other hand, the condition may amount to only a high degree of egotism or emotionalism, such as even so-called normal men exhibit in periods of unwonted excitement. There are degrees of hysteria or psychosis, then, and variations from the extremes of madness down to the normal manifestations of mere excitement. Now it begins to be clear why opinions differ as to the normality or abnormality of certain mental exhibitions. There is a midground which shifts from normal to abnormal according to the reasoning, the predilections and the personal equation of the investigator. All of these psychoneurotic phenomena then we will grant are forms of mentalization, extending from the complete abeyance of it on the one hand (temporarily in sleep, permanently in death), to the ex- treme riotous and decidedly exceptional exhibition of it temporarily in hysteroid conditions, permanently in certain forms of insanity on the other hand. To avoid all confusion in regard to the middle-ground of the psychoses, the terms normal and abnormal had better be dropped. These troubles, then, of mentalization, falling between sleep at one end of the line and extreme idiocy, dementia and insanity at the other, involve the mind and its anatomico-physiological basis as well as all its variations and their particular anatomico-physiological bases. A definition of mind has been the goal of philosophers of all time. Physiological psychology defines it as a function, a compound of the interplay of certain well-recognized sensory-motor functions or im- pulses. It is therefore not an entity itself, but a mere phenomenon, a process, representing the complicated action and interaction of certain anatomico-physiological functions upon one another, or all together. That is the physio-psychological definition and conception of mind. It does not oppose the discussion of any other conception or object in the least to metaphysical debates in regard to dualism, in regard to the mind being a separate entity apart from brain, or in regard to the existence of immateriality or any other such knotty problem. It simply doesn't enter that sphere of research, but merely declares that so far as it is concerned, cessation of brain function means cessation of mind,, partial cessation of brain function or disturbance of it means partial cessation or disturbance of mentalization, an abnormal psychosis. With this brief attempt at clearing away the confusing factors in regard to the physiological meaning of the terms mind and normal,. THE NEURONIC DISE VSES 141 we are in a better position to analyze and understand the psychoneu- roses mentioned at the head of the section. Sleep is a function which, when performed under what are called normal conditions, represents a complete abeyance of all mentalization. with complete absence of consciousness. There is no proof for the belief held by some that mentalization never ceases, even during- sleep, and that we merely are unable to remember when awake what our thoughts were doing when we were asleep. The most reliable evidence all points to the fact that in healthful sleep the mentalization is abso- lutely at a standstill. The mere nutritive and vegetative functions that underlie it go on. Healthful sleep being a physiological phenomenon, it hardly calls for further description here* Like ail physiological functions, how- ever, it is sometimes disarranged. The disturbances of sleep are many and varied. Dreaming is probably the commonest disturbance. In fact, so> common, so near to the experience of the average, is dreaming, that the world looks upon it as a normal condition, another evidence of the fallacious method of arriving at knowledge by identifying that merely which can be predicated of the average with normality. Dreaming represents an incomplete sleep, a partial cessation of cer- tain high neuro-physiological functions, a partial awakening of mind. Dreams, of course, are innumerable and in their partial character repre- sent all phases of mentalization. In a broad way, however, it may be said that the imaginative, creative faculties and the memory are active, whereas the volitional and reasoning functions are in a state of in- activity. The inhibitory control of the latter over the former is with- drawn and thus is accounted for the rapidity, the strangeness and the incongruity of the actions in our dreams. Most wonderful combina- tions and thoughts and doings arise in this state. They remain uncon- nected by the higher psychic forces. They are so marvelous that primitive, ignorant and superstitious people look upon them as super- natural visitations, as flights of the soul into space, and as telepathic, clairvoyant, spiritualistic and prophetic agencies. They are purely psychic phenomena, of course, and doubtless depend upon an abnormal interplay for the time between the basal anatomico-physiological func- tions of the brain. They have their counterpart metaphorically in the physical sphere in the spastico-ataxic manifestations of certain diseases of the upper motor neurones, which withdraw the inhibitory control of these neurones from those seated in the cord below. Pavor nocturnus, the night terrors, seen in both adults and children. but more especially in the latter, is usually a psychopathic manifesta- tion that occurs when sleep is passing, according t<> the experiments of Kohlschittter, from its maximum intensity to a lesser degree of depth. It is thus a sort of a dream, but occurring in an individual with a neu- rasthenic or hysteric tendency it assumes the depressed, distressing. psychalgic form which eventuates in fright and terror. Nightmare, so-called, is ;i dream state in which the fancy revels in the creation of paralytic conditions or in which the volition especially is suppressed while the other facilities are partially awake, especially 142 THE NEURONIC DISEASES the apperceptive faculties. Sometimes in these dream states the apper- ceptive functions are so far awake that ideas and questions may be presented and be taken in by the sleeper. There is not a complete closure here of the pathways for afferent impulses. The whole dream and its direction may thus be guided by a touch on the body or a sound in the room, or a sudden flash of light. The stimulus may be an auto- stimulus and come from the body within. Thus are accounted for the dreams that are provoked and directed sometimes by an over-loaded stomach or a full bowel. In all of these states the sleep is abnormal ; the dreaming is an abnormality and rests on an abnormal anatomico-physiological basis. It is the result of an abnormal interplay between the inpouring and in- poured afferent impulses. Some of the mental functions are active,, some are inactive. Thus a strange and abnormal mentalization obtains. Sometimes these partial mental activities include the emissive or motor centers. A psychoneurotic reflex, a sort of low volition, or rather ideo-volition, with a reflex, is set up and the individual does things in his sleep. Thus we have the somnambulistic phenomena accounted for. Hypnotism is nothing but a form of artificial somnambulism. Partial abeyance of the mental faculties is the basis of all these exhibi- tions. An automatism characterizes them and just as the withdrawal of certain mental forces, and the concentration of certain others, causes the wonderful ideas that are enacted in the simple dreaming state, so the same disproportion between them enables the somnambulist to do the remarkable feats that he sometimes does. A sleeper, for instance, can walk on the perilous edge of a roof because, unlike one who is awake, he is not embarrassed by any observations or thoughts of the dangers of his situation. In regard to them and other attendant circum- stances his mind is asleep. All that it is taking in are the impulses pouring up from his feet or his. hands, his ears or his eyes. In a reflex way these impulses pass through the subconscious or lower coordinat- ing apparatus and out again to the appropriate muscles. The sleep walker is thus a veritable automatic mechanism ; a most delicate one, to be sure, but a completely unembarrassed mechanism. Automatism is a trait of all animals and is seen in many common acts of life. The developmental stage in regard to the intelligence, the habits and the mental capacity all determine a man's degree of automa- tism. Automatism sometimes gives one credit for heroism. A man rushes into danger often because he is a stupid automaton. The old adage that fools rush in where angels fear to tread is another way of defining automatism in men. What is vulgarly called "nerve" is a semi- automatic performance which a higher degree of intelligence would forego. Habits establish automatism. Most of the common acts of life, like walking, speaking, writing, many of the rarer accomplishments, like piano playing, tight rope dancing, trapeze swinging, are perform- ances practiced as habits until they have become automatic. They are the result of a subconscious reflex and during their performance the mind, so far as they are concerned, is partially asleep or wholly diverted. That somnambulism is but the same sort of automatism is shown THE NEURONIC DISEASES I43 in the fact that the habits of the individual often determine the char- acter of the sleep performances. A writer gets up in his sleep and writes ; a singer sings ; a mechanic goes to his tools. In ordinary somnambulism the stimulus to the mind comes from within. It is an auto-automatism. In hypnotism, on the other hand, the stimulus comes from without. The suggestions of the hypnotizer enter into the partial mentalization of the patient and their origin not being recognized by the higher reasoning faculties whicn are asleep, they enter into the mental reflex, give it its direction and so cause the subject to do what the hypnotizer suggests. This is mere artificial somnambulism. It is an abnormal sleep phenomenon, an automatism given directly by an external ideo-motor suggestion. It is certainly not a normal condition in the sense of being an average experience. It is a pure functional disturbance in the psychic sphere and so far as it is allowable to speak of functional dis- turbances or of the underlying inherited or acquired nervous defect upon which such disturbances are usually based, as conditions of dis- ease, hypnotism and all its allied phenomena are disease manifestations. They resemble hysteria and other psychoses in being abnormal or ex- ceptional forms of mentalization. Hypnotism is a subject that has been much written upon, and about which many divergent views have been expressed. The history of the development of the subject is interesting in the extreme, but would not be especially pertinent here and would take up too much space. Suffice it to say that at the present time it is founded upon suggestioii and all of its phenomena are the result of the influence of the suggestions of the hypnotizer made to the hypnotized. There is no transference of any physical force or influence. The subject acts in response solely to his own mental mechanism, but that mental mechanism is given its direc- tion of activity by the suggestions offered to the susceptible subject. To-day there are two schools whose opinions upon some features of hypnosis and hypnotism are somewhat dissimilar. The Nancy school, at the head of which is Bernheim, and the founder of which was Liebault, does not regard hypnosis as an. abnormal state in the strict sense of the word, for it argues that all men are more or less influenced by suggestion and it is only a question of degree as to the susceptibility of the individual. It declares, therefore, that taking human beings just as they come, eighty per cent, of them at least are hypnotizable. The Paris school, at the head of which stood Charcot, insists that hypnosis is a decidedly pathological phenomenon and that it is allied to genuine hysteria. It maintains that only the weak-minded and un- balanced, those who tend toward the hysteroi i state, can be hypnotized in the legitimate sense of the word. Ah men, of course, they admit. are open to suggestion, but hypnotism is something more than mere suggestion. It needs a defective organism on the part of the subject for its proper revelation. It seems to me that the difference between the opinions of these two great schools rests upon some fundamentally different ideas apart from the subject of which they are the prominent exponents. Their 144 THE NEURONIC DISEASES debate is really around the word disease, or that which may be con- sidered normal or abnormal. That all men are open to suggestion is a truism insisted upon by the Nancy school ; few men have hysteria is the truism uttered by the Paris school. Is all suggestion in its influence hysterical, or, on the other hand, is hysteria nothing but a form of suggestion? If the two schools can be brought to a point where they can answer these ques- tions in exactly the same way, their difference, it seems to me, will have vanished into thin air. The mind is a mobile phenomenon evolved by a very mobile anatomico-physiological basis. It is never the same for obvious reasons at different times in the same man, and, still more especially, never the same in two different individuals. Its mobility and changeability vary according to the stimuli that provoke it into existence, and the condition of the physical basis upon which those stimuli act. It is therefore always more or less susceptible to change under these condi- tions. If the stimuli are strong or if the physical basis is unstable, just in the same proportion will the susceptibility of the mind be to change. This may occur and yet the condition not be regarded as being abnormal. There is an indeterminate point, however, beyond which the susceptibility does deserve the name of disease. When the instability is so great that every form of stimulus, whether from within or from without, controls and directs the mental operations, wholly or partially, so that the individual seems to have no self-control whatever, the state becomes hysteroid, may even become an actual hysteria, may pass into a profound form of pure insanity. As I have pointed out in the section upon hysteria, this disease is one of mental unbalance, a true psychosis, in which a certain form of suggestion, egoistic auto-suggestion, plays a prominent role. In hys- teria the patient's mental state is a sort of waking somnambulism, if I may use the apparent contradiction. One part of the mental mechan- ism is out of balance with another part. The inactive part may not be asleep or below the normal waking state, but the active part is too vigorously awake and in excess of the normal waking state. To use metaphors, it is not that one part of the mind is so much paralyzed as it is that another part is in a state of spasm. This unbalance is so pronounced and so intense that it affects the ingoing and outgoing impulses even, and we call the condition a psycho- neurosis on account of the marked physical and mental clinical mani- festations. If hysteria be thus a psychosis, a mental unbalance of a particular sort, it simulates the condition of the unbalanced individual ' who is swayed by every suggestion that is poured in upon him. If hysteria be a disease, as it undoubtedly is, then the mental unbalance is a sort of disease, for it is but a mild degree of the same thing. Psychosis thus admits of degrees and variations, but we have not decided as yet upon the exact line where the normal condition ends and the abnormal begins. No man's mind is absolutely impermeable to changes of stimuli, or in other words suggestion, for it is itself the very representative result of those multiple stimuli. When does the yield- THE NEURONIC DISEASES I45 ing to these stimuli become so great that there is no longer self in the process ; or when do the stimuli set up such a powerful, overwhelming activity in one direction that there is apparent inactivity in another direction ? Who can answer ? He that can will point out to us exactly the line between what should be called normal and what should be called abnormal. Xear that line, extending on either side of it, in the one direction toward perfect mentalization and in the other direction toward insanity, will be found the weak and the susceptible individuals who are hypnotizable. On the one side they will be regarded as normal and possibly number seventy or eighty per cent, of the community : on the other they will be considered abnormal and will constitute a much smaller percentage. At either end of the line will be found the individuals who cannot be hypnotized at all, for either they will have too stable or too unstable minds. The conditions for producing hypnosis are clear enough. There must be susceptibility in the subject and there must be forceful self- confidence in the hypnotizer. Infants and insane people cannot obvious- ly be well influenced. Weak, neuropathic, rather neurasthenic and hys- teroid people make the best subjects. Marked hysteria, like insanity and dementia, is not readily amenable to hypnotism. The hysterical state between the paroxysms is, however, capable of hypnosis. Two things are aimed at in hypnotism. First the patient must be put to sleep. This is done by getting him to gaze at a bright object, a little above his eyes, thus wearying his eye muscles ; by gently stroking the brow outwards with the two hands ; and by urging him to keep his attention fixed en the thought of going to sleep while the operator, in a soft, monotonous tone of voice, keeps commanding "go to sleep, go to sleep ; now you are asleep, sleep, sleep, sleep," etc., repeating this, or some similar phrase, over and over until the patient is asleep or rather in a state of hypnosis. Ofttimes many trials have to be made before success is attained. If hypnosis is accomplished, suggestions may then be made to the patient to do this or that ; or he may be told that this ill or that ache is gone, etc. Be sure always to make the suggestion that it will not return when he awakes. A quick puff of breath or a command to "awake" will be enough usually to revive the subject. Of course, it goes without saying that only mental symptoms can be thus influenced and not very many of them. Imaginary and self- suggested ills are the most successfully managed thus . Hypnosis may in a particularly weak individual be set up by inter- nal stimuli. This is what is called autohypnosis. It is capable of (level- ing from habit. Hence since hypnosis is itself an abnormal condition. oping from habit. Hence since hypnosis is itself an abnormal condition. hvpnotic state, the practice of hypnotism should be interdicted by law except when in the hands of physicians, and by them it should be re- sorted to only when other means have failed. In certain conditions it is a powerful and legitimate therapeutic agent, but it has been very much overvaunted. Another manifestation of the somnambulic state is the condition I46 THE NEURONIC DISEASES known as catalepsy. It is closely related to hysteria and is dependent upon the overaction of certain mental activities, to the detriment of others. It can be sometimes produced artificially by a loud noise, a sudden flash of bright light or a fixation of the eyes upon some bril- liant object. The patients become statuesque in appearance. Their limbs are waxy-Lke so that they can be placed in any position, and they will remain there, without signs of exhaustion. All sensation seems to be lost, but the special senses are but little affected. The eyes remain open and staring. At times the rigidity of the muscles is so great that the patient may be placed with only his head and feet resting on chairs and considerable weights laid upon his body. In lethargy a still higher degree of abnormal abstraction occurs, all the senses, general and special, being abolished. These patients cannot be reached by suggestion. The eyes are half closed and the muscles are relaxed. Percussion of the nerves may still cause some muscular contraction, showing that the neuromuscular excitability is still present. It is said that this state can also be produced artificially by exerting pressure on the eyeballs in particular individuals, or by closing the eyes of one already in a state of catalepsy. All of these mental states are hysteroid in character and indicate a profound psychic unbalance with enormous overaction in some direc- tions and corresponding underaction in others. What are the physical changes underlying the phenomena we do not know, and probably never will know until we are more familiar with the normal mechanism of the mind. We have now seen that complete, profound sleep, involving all of the mental processes, is a normal condition periodically undergone by the nervous system. We have also seen that partial sleep, involving only a part of the faculties, may occur in such a mild form, dreams, as to be regarded as normal, and yet vary up through the many stages of somnambulism, natural or artificial, catalepsy and lethargy to apparent death. We have noted the similarity of all these conditions, especially the latter, with the psychospastic and psychoparalytic phenomena of hysteria. The last disorder of sleep is its entire absence. This is known as insomnia. It is a rare condition and is indicative almost of a form of insanity. Life cannot be maintained longer than about three weeks in the absence of all sleep. What is often called insomnia is merely a mild and relative form of the trouble. Drowsiness, short naps and mo- mentary lapses that practically amount to light sleep, are experienced by those who from various diseases declare they are the victims of insomnia. The treatment of these relative forms of the trouble consists of the removal of the cause and the management of the underlying habits and disease. In some insane states a prolonged insomnia sometimes oc- curs until the patient dies of sheer exhaustion. As I have said, it is impossible to live without sleep: it follows that every form of unnatural wakefulness, therefore, has some under- THE NEURONIC DISEASES I47 lving, continuous cause. Absolute insomnia does not exist except for a few weeks in certain mental and fatal cases of disease. Relative in- somnia, which is the common form complained of, is not a causeless trouble. In most cases the cause is obvious ; in a few it may be very obscure. Every case of insomnia, therefore, should be most exhaustive- ly examined for the cause of it before a moment's thought is given to its treatment. There is no mere symptom in neurology that has been more often managed as a disease per se, and that as a consequence has led to more disastrous habits with drugs and other agents than sleeplessness. Even physicians themselves have been their own victims in this stupendous piece of folly. Chloral taking and the steady use of hypnotics shows on the face of it that a symptom is being wrestled with while the hidden cause is still going on exercising its deleterious in- fluence. It shows also in too many cases that the adviser is too lazv or too ignorant to find out and to combat the cause. And, alas ! it sometimes terminates in mania and death. The common causes of insomnia are easily grouped under certain general heads. In the first place, many people inherit an irritable, weak and neuropathic constitution which refuses to functionate normally, or as the average, healthy constitution functionates. It is prone, there- fore, to be wakeful, with or without adequate provocation. These cases are the inherited and habit insomnias. They are purely nervous in origin. Some of them may show the nervous insufficiency in vaso- motor disturbance which is the cause of the cerebral activity and wakefulness. Certain heart and vascular troubles can obviously disturb the intracranial circulation. Lithaemia, gout, uraemia and other auto- toxic and diathetic conditions can irritate and interfere with normal functional activity. Fever is a disturber. Simple exhaustion sometimes produces irritability that prevents the much-needed sleep. Various substances that are ingested are sleep-destroyers at times, such as cof- fee, tea, alcohol, etc. One must also think of the intoxications with lead and tobacco, the infections like syphilis, malaria, etc. All of these conditions must be earnestly searched for in every case. Only as a dernier ressort should drugs ever be employed. Before turning in hopelessness to drugs, all sorts of dietetic, hygienic, hydrotherapeutic, electrotherapeutic and other mechanical measures should be tried first. I always urge that after a light supper all earnest mental exertion should be stopped. The evening should be passed in as indifferent a manner as possible. Quietude on the couch, with a minimum of excitement, such as reading, games, conver- sation, should be attempted. Occasionally a slow, short walk in the open air for a few minutes in the middle of the evening will be helpful. Never take a brisk walk just before going to bed. Some cases do well to retire in half an hour after coming in from outdoors. Others prefer a slight tepid bath and a little light lunch, such as crackers and warm milk. One must experiment here. The great desideratum is to quiet the nervous apparatus and to avoid every possible vasomotor dis- turbance. Music has a quieting effect nn some, irritating on others. A monotonous noise, such as dripping water in a neighboring room, has sent an insomniac sometimes to sleep. Monotony of thought, such as I48 THE NEURONIC DISEASES ' counting, humming, listening to dull reading and repeating senseless rhymes, may be helpful to some, but to many others I have found such measures to be simply exasperating. There are a hundred ways of fostering monotony and quiet and sedation. Each one must learn them by experimentation. If medicines must be resorted to, I give the preference to trional, sulphonal and the bromides. Chloral I consider a dangerous drug. More habitues are probably produced by chloral than by any other remedy. Hyoscyamus, usually in combination with the bromides, has often served well. Other hypnotics that may be useful to fall back upon are camphor, paraldehyde, methane, amylene hydrate, lupulin, chloralamide, small quantity of alcohol, cannabis indica, chloralose, ethylene bromide, gelsemium, hypnone, scopalamine hydrobromate, snmbul, tetronal, musk, asafcetida and valerian. The very length of this list suggests that the drug treatment of insomnia is at best only a makeshift. In intractable cases, few as they are, and where the cause cannot possibly be discovered, the symptom must at times be temporarily looked upon as being the disease itself. Never use opium or chloral in these cases except in extremity. They will surely become habitues. I have more than once heard a physician's reputation torn to pieces by one of these drug takers when perchance all he did was to write a single prescription for chloral or an opiate. Though the emergency may have demanded it at the time, the weak-minded patient seized upon the opportunity to begin a drug- habit which had finally driven him to seek a neurologist. The treatment of insomnia is not an easy matter. It is one of the most difficult tasks in medicine. Its general principles only have I here indicated. Firmness, tact and a careful study of the trouble as a mere symptom and not as a disease will accomplish the best results in the end. Temporary measures, such as the quick, injudicious use of hypnotics, will be sure to lead to more or less misfortune. Cases of prolonged sleep, lasting from a day even to several years, belong to the condition of lethargy, or cases in which a facile habit has developed of undergoing autohypnosis. Probablv the longest so-called sleep on record is the instance re- ported by Herbst, of Bremen, of a woman who was in a state of lethargy seventeen years. Ingestion of food was normal except that it had to be specially prepared for her. There was a semi-conscious state, as she moaned and otherwise indicated when she wanted atten- tion. The "sleeping" and "fasting girls" of the clime museums are lethargic hysterics. The so-called sleeping sickness, sleeping dropsy, maiadie du som- meil, negro lethargy is a parasitic trouble found occurring endemically in Africa, chiefly along the West coast, and limited entirely to the negro. The trypanosoma gambiense, recovered from the cerebrospinal fluid of the victims, with a species of tsetse fly, Glossina palpalis, as the intermediate host, is probablv the cause of the trouble. No distinctive pathological changes have been found, though in THE NEURONIC DISEASES 149 one instance there were evidences of a subacute leptomeningitis, chromatolysis of various degrees in the cells of the ventral cornua of the cord and a slight falling out of the nerve fibres (Warrington). The prominent symptom of the disease is the prolonged sleep or lethargy, more accurately coma, in which the patients waste away and die after several weeks or months, always within a year. They rarely recover. Treatment seems to be ineffective. VERTIGO. Vertigo is a symptom and accompanies many diseased conditions. It is only worthy of special consideration because it is such a frequent symptom and because it occurs sometimes by itself. The imperfection of neurological science is glaringly exposed in the way we still have to regard in some instances such mere symptoms as vertigo, chorea and hydrocephalus, as though they were special disease entities. Vertigo is a loss of the sense of equilibrium. It may be sub- jective or objective. The victim may imagine his environment to be stationary while he himself is in a whirling state, or it may seem to him that he is stationary while his surroundings are in a condition of unnatural movement. Vertiginous sensations involve incoordination as well as a loss of equilibrium. Some have thought, from the classical experiments of Flourens and the phenomena observed in aural vertigo, that the semicircular canals were the special and sole organs that subserved the maintenance of equilibrium. As stated by Edinger, J. Richard Ewald removed the labyrinth from both sides of a dog. The result, of course, was a loss of muscular tone and of the power to maintain vertical equilibrium. Walking and standing were impossible. Note, however, that these troubles were re- covered from, "the tracts for tactile and other impressions making up for the loss." The ablation of the cortical motor areas was next made, with a resulting incoordination and the loss of the power for making any regular movements. But even this was slowly recovered from. Suspicion being awakened that all movements were dependent now solely upon visual control, the mutilated dog was placed in a dark room. He immediately fell helpless to the floor. If this experiment means anything, it means that coordination and the maintenance of equilibrium depend upon several factors, each one of which is capable, up to a certain point, of assuming the whole func- tion.* The motor areas of the brain, the visual organs and the semi- circular canals all pla\ an equally important part. If the semicircular canals alone were the organs of equilibrium it is difficult to understand how the maintenance of equilibrium could even in the slightest degree be reacquired after their removal, in spite of the preservation of the tactile and visual senses. As I have shown elsewhere, vertigo and the loss of the sense ^\ equilibrium are the result of a disturbance in a more extensive sensory- motor field than is represented 1>\ the semicircular canals. In view <>! J^ " THE NEURONIC DISEASES the fact that in certain of the lowest vertebrates (fishes) whose move- • ments require the nicest sort of equilibrium there are no semicircular canals ; in view of the fact that experimentation has shown, as in the dog, that equilibrium may be recovered and maintained after the com- plete removal of the labyrinth ; in view of the fact that both anatomy and physiology point out that the sense of equilibrium is essentially a complicated sensory-motor phenomenon ; and finally, in view of the fact that the anatomy of the apparatus of equilibration, of which the semicircular canals form only a part, offers a more rational basis for its sensory-motor character than does so limited an organ as the semi- circular canals alone: I think that we are still justified in asserting that the semicircular canals are not alone the organs of equilibration, and that the vertigo of Meniere's disease is not due solely to injury of the canals, but to the injury done to the elaborate and complicated apparatus of equilibration of which the canals constitute a part. One object in presenting all this is to show that vertigo, if our knowledge of the anatomy of the apparatus of equilibration is at all correct, may be produced by irritation of any of its other ramifications, as, for instance, in the eye and in the stomach as well as in the ear. Hence, it seems to me that Gowers' statement referring to other forms of vertigo than aural vertigo is too much of a pure assumption as it stands : "It is certain that in the majority of cases in which such vertigo has been ascribed to other causes, these have only had an exciting influence and the symptom has been essentially due to the effeci of unobtrusive labyrinthine disease, bringing the center into an unstable condition." The study of vertigo is one of the most intricate in medicine, and though we are quite familiar with its subjective and objective mani- festations, its immediate cause remains yet a profound mystery. The maintenance of equilibrium cannot be satisfactorily assigned to any single center, though its disturbance may easily depend upon a number of distant reflex disturbances. There is a multiplicity of impressions constantly pouring in upon the brain through the various avenues of sense which must necessarily keep the mind in a state of extreme ten- sion, so that the slightest exaggeration one way or the other will result in mental confusion and more or less vertigo. We learn instinctively how to control and marshal these ever-passing sensations before the mind, but if for a time they become too frequent, rapid or sudden, th* controlling force of the intellect is weakened and we are confused an J overcome by them. Everybody knows how quickly vertigo is estab- lished by rapidly revolving mirrors, peculiar whirring sounds, electrical sensations upon the skin and such distant irritative conditions as dys- pepsia and constipation. Physiological psychologists arc agreed now that our knowledge of the relationship of things, as well as of space and its dimensions, is the result of experience gained from the use of our muscles. The infant learns these dimensions by the frequent application of its hands to the objects which it sees; and the sight, which is the only other sense that reveals to us an extended object, and that never shows us more THE NEURONIC DISEASES 151 than two dimensions at one and the same time, does so entirely through the "muscular sense" exercised in the use of the muscles of the eyeball. Now when we study the nature of equilibrium we discover that it is dependent upon a normal consciousness of the proper relationship of things outside of the centers of consciousness. It is not purely pyschic, as some have imagined. There is the sensation of a motor process in it. Dr. Hughlings Jackson has long insisted upon the im- portance of this fact, while Dr. Reynolds wrote as long ago as 1854 that "the feeling of equilibrium results from the harmony of our differ- ent sensations among themselves and with the motor impulse which is their combined effect. When any one group of the sensorial impres- sions is distorted or removed, the balance is disturbed, and as these impressions are themselves the stimuli of muscular action, attempts are made for its restoration . . . producing vertiginous or allied move- ments." The principal factors m the preservation of equilibrium are there- fore consciousness and normal sense impressions. A vertiginous im- pression reflected through consciousness may end in a complete or in- complete motor act. In the latter case there is merely a residual dis- turbance of past impressions without a fulfillment of the present impulse in complete motion, while in the former the patient is uncon- trollably moved in one direction or another. If the views of Spitzka and Starr in regard to the sensory paths in the medulla be correct, they bear strongly in favor of this sensory- motor theory of vertigo. Thus Spitzka concludes that impressions from the cochlea reach the cortical centers in the superior temporal gyrus by way of the posterior division of the eighth pair of nerves, the trapezium of the same side, part of the lemniscus, posterior pair of the corpora quadrigemina, external geniculate body and the corona. Ac- cording to some observations of Starr, which prove the correctness of the views of Flechsig and Von Monakow, the lemniscus tract is asso- ciated with the so-called "muscular sense'' and is chiefly distributed to the inferior quadrigeminal body. Some phvsiologists think that it is even continued up as far as the corona. It is believed that this tract is associated with the sense of sight, as well as with the coordination of movements. Flourens found that injuries to the corpora quadrigemina of one side caused ''forced movements," and that their complete removal resulted in incoordination of movement. The point which I desire to insist upon is the close relationship of all these tracts for sight, hearing and the "muscular sense" in the corpora quadrigemina and their more distinct termination in the cerebral cortex than in the cerebellum. If vertigo were a kind of en- cephalic ataxia, and due simply to disturbed coordination, it might readily be attributed to the cerebellum. While the function of the cerebellum may be that of coordination and the maintenance of "continuous tonic muscular contractions," and while as Spitzka tersely remarks, it may be the center where "impres- sions of touch and position 'ire associated with those of time and space," I find no authority for assuming that it is in any way the cen- ter for the institution of movements or the maintenance of these move- 152 THE NEURONIC DISEASES merits in a regular, methodical manner, which, to my mind, is the un- derlying phenomenon, objectively or subjectively, of true vertigo. Vertigo is something more than simple incoordination, though incoordination may enter as one of its principal elements. In vertigo there is a disturbance, not merely of the reflex functions, but of the higher powers as well. The motor and sensory phenomena of vertigo, in typical cases at least, are not mere sequences of abnormal motor and sensory impulses, but they are the impulses themselves. Simple incoordination manifests itself in irregular unmethodical movements and thus shows that it is due to an affection of some sub- ordinate regulating center of the brain; true vertigo manifests itself in regular methodical movements and thus reveals disease of a higher independent center. It is a significant fact, when considered in this connection, that the vertigo which is so characteristic of cerebellar disease and which is identical with the vertigo of Meniere's disea.se, is caused only by lesions in the middle lobe, or that part of the cerebellum which is most liable to involve the sensory-motor tracts of the medulla; and furthermore, that lesions of the lateral lobes not pressing upon or involving the middle lobe do not give rise to any known symptoms. And it is still more significant that vertigo is not pathognomonic of cerebellar disease, for it may be caused by lesions in other parts of the brain as well. Even in its coodinating function, it is more than probable that the cerebellum, instead of sending out direct coordinating impulses to the muscles, merely coordinates the impulses sent down from the cerebral centers above. Indeed, as Ranney has said, "it is a curious fact that most of the effects of cerebellar lesions are attributable, to a greater or less extent, to irritation of the crura." According to Goltz, Serres, Cayrade and others, the function of equilibrium is profoundly affected by destruction of the corpora quadrigemina. The experiments of Ferrier upon monkeys, fishes and rabbits, and of McKendrick upon pigeons, revealed the same effects. The corpora striata have also been shown by physiological experiment to preside over the maintenance of equilibrium, especially after the removal of the hemispheres. Every indication points to the fact, then, that we must look for the center of equilibrium not in one particular part of the brain, but in the harmonious action of the various sensory and motor centers one upon another. Vertigo then being merely a symptom no systematic discussion of its etiology and pathology is possible for we would have to discuss all the diseases in which vertiginous sensations occur. I have already shown the nature and general causation of the phe- nomenon and now will mention a few of the more common forms of vertigo and their probable etiology. I do this with the special desire of forestalling the various views which some promulgate in regard to the cause of all vertigos. Ocular Vertigo. The eves and the optic nerves constitute a most important inlet for afferent impulses. Any disorder in the visual ap- paratus must necessarily upset the orderly procession of these impulses THE NEURONIC DISEASES 1 53 both in relation to one another and to those from other sources. Hence we find in nearly all diseases, whether functional or organic, that exer- cise the slightest influence on the optic apparatus and the function of sight, more or less vertiginous sensation. It is a mere repetition then to say that muscular insufficiencies, refractive errors, reflex functional disturbances, intoxications, brain lesions, are all more or less accom- panied by vertigo. Aural Vertigo. The same may be said of diseases of the ear that was said of those of the eye. One variety of aural vertigo has long been dignified with the special name of Meniere's disease. The latter term is employed very loosely nowadays and many writers speak of all forms of vertigo whether due to internal, middle or external ear abnor- malities as types of Meniere's disease. In true Meniere's disease only the semicircular canals are affected. At least that is what Meniere himself seems to have believed. His second and fourth propositions are that certain functional troubles, having their seat in the internal auditory apparatus, may give rise to cerebral symptoms such as in- tense vertigo, uncertainty of gait, turning to the right or left and fall- ing, all of which may be attended with nausea, vomiting and syncope, and that all this tends to confirm the belief that the lesion which is the cause of these functional troubles is in the semicircular canals. In his original paper he describes ten cases, in only one of which was there an autopsy. This case, the tenth of the series, had no disease of the nervous centers, but the semicircular canals were filled with a "reddish, plastic material." It was on the strength of this single observation that he declared the canals to be the cause of the cerebral symptoms. Aside from the unscientific character of such a hasty generaliza- tion, this one case, as Bremner points out, will hardly explain those in which the cerebral symptoms come and disappear rapidly. And if the hemorrhage into the semicircular canals were the cause of the trouble here and in the majority of the apoplectiform cases, as Knapp argues, it remains to be shown why the hemorrhage is bilateral, since the aural symptoms are so frequently bilateral. Both Steiner and Sewall carefully removed the semicircular canals from the shark, whose auditory apparatus is practically identical with man's, without obtaining any disturbance of movement. The former experimenter believes that the loss of equilibrium results from lesions of the brain or its membrane, causing alterations of pressure, for he has again and again destroyed the canals in sharks, frogs and lizards without injuring the brain, and discovered no disturbance in the power of equilibration. After careful investigation Bottcher and Baginsky have come to the conclusion that the cause of the rotation of the head in Flourens' and Goltz's experiments was the injury done to the brain, and not merely to the semicircular canals. Bottcher marks the fact that the auditory nerve is not bound down at any point between the brain and the labyrinth, and that if. one is careful not to pull upon this nerve the vertiginous sensations are not awakened by the injury of the semi- circular canals. The slightest traction upon the auditory nerve in- jures its attachment at the medulla, and s< rise to the symptoms described by Flourens, Cyon, and Goltz. fn the experiments of 154 THE NEURONIC DISEASES Bottcher the head, furthermore, did not always rotate in accordance with the particular canal injured, as Goltz had formulated. The iden- tity of these canals with the cochlea in the embryo, their continuity with the epicerebral space through the aquaeductus vestibuli, and their ever- shifting relations amongst themselves in the quadrumana,' which go about sometimes in the horizontal and sometimes in the erect position, argue strongly against the special function of these canals being the maintenance of equilibrium. In the discussion of my paper upon the same subject before the Philadelphia Neurological Society, Nov. 24, 1890, Dr. Dercum re- ported some observations he had made of the comparative anatomy of the labyrinth for the purpose of securing new light, if possible, upon the function of the semicircular canals. He said : "They bear a dis- tinct relation to the lateral line organs of fishes, the nerve hills of the latter being identical in structure with the maculae acousticae. Just as the inclosure of these nerve hills in tubes seems to have for its object the gaining of distinctness and definiteness of impression so the in- closure of the maculae within the semicircular canals seems to have the same object. It is significant that each macula is situated at the end of a canal, and this suggests that the canal is a kind of conduit to carry off vibrations after they have made their impression on the macula ; and further, that if interference of vibration occurs, it occurs at a point distant from the macula. Confusion of sound is thus avoided. The in- ference from physiological experiment that these structures have for their special object the maintenance of equilibrium, has always seemed to me open to most serious objection.'' It is a well-known fact that many fishes have a utricle and three semicircular canals, while some have only one and two canals. Amongst the lowest vertebrates the lamprey has a saccule with audi- tory hairs and otoliths in communication with two semicircular canals, while the myxine or hag has only one canal. Doubtless there are im- portant sensory impulses of a special character which pass through these organs, but equilibration, due as we have seen to the fusion of a vast complex mass of sensations pouring in upon the brain from all sides, cannot possibly be the result of merely a single set of impulses from such an end-organ as the semicircular canals. The very nature of vertigo involves the idea of more or less loss of consciousness, and this alone would seem to indicate a higher cause for the trouble than sim- ply these canals. If it be granted that the pathological cause of this vertigo is to be found in the semicircular canals, it is hard to understand how in all cases the cochlea or the cochlea branch of the auditory nerve should necessarily be affected. Tn Flourens' experiment, the entire removal of the canals did not impair the hearing-. So strikme was this fact, that Flourens, as well as others, was led to believe that the branch of the auditory nerve which supplies the semicircular canals subserves motor purposes only. On the other hand, if Gellc's experiments arc to be trusted, the cochlea is in no way the source o\ vertiginous impressions. Simple continuity of structure will not account for the loss of hear- ing when the lesion is a hemorrhage into the canals, and pressure alone THE NEURONIC DISEASES 1 55 will not answer as a cause in all cases of so-called .Meniere's disease. In the first place, there is no regular correspondence between the amount of deafness and the intensity of the vertigo ; secondly, with total loss of hearing in Meniere's disease all known methods of diag- nosis prove that the nerve itself, and not merely the internal ear, is affected ; thirdly, physiological experiment shows that simple pressure does not cause total loss of hearing when no injury is done to the cochlea or cochlea branch of the auditory nerve ; and finally, no case has ever been reported in which the cochlea was primarily affected with consequent involvement of the canals and vertigo, though in blood supply and anatomical structure the cochlea and canals are similar. Hence, in Meniere's disease, I conclude that the lesion must concern the whole labyrinth or lie entirely outside of it. It was Dr. Wilks, of England, I think, who held that Meniere's disease was a neurosis of the centers of audition and equilibrium, and in its paroxysmal form at least was much like migraine with its eye -complications. While this explanation cannot be adopted for all cases, there is little doubt about the central nature of the disease in many of its as- pects. Burnett declared that "the neuropathic diathesis is well marked in most cases of aural vertigo." The grouping of the symptoms, as well as the proximity of the tracts and centers involved, point to the encephalic origin of the affection. Especially is this indicated, how- ever, by the sensory-motor nature of the vertigo, whatever be the peri- pheral source of the disturbance, and its association with the state of consciousness. Sudden or paroxysmal vertigo, causing the patient to fall some- times as in a stroke of apoplexy, followed by nausea and vomiting, headache, difficulty of hearing, stubborn tinnitus or even deafness, bilat- eral or unilateral, with various other possible accompaniments such as facial paresis, nystagmus, constitutes the clinical picture of Meniere's disease. According to Meniere himself it is due to hemorrhage into the semicircular canals. As the syndrome occurs in other ear troubles, and in cranial injuries and brain diseases, the semicircular canals may be only secondarily and functionally disturbed. In most of the cases the nausea and vomiting soon cease, the ver tigo passes away in the course of a few weeks or appears only at re- mote intervals, while the hearing remains more or less permanently damaged. The vertigo of the neuroses bears a close similarity to many c of Meniere's vertigo. In hysteria, hystero-neurasthenia, epilepsy, un- usual and unexpected sounds, sights and other sensory irritations arouse a vertiginous condition. The feeling of distress and impulse- to leap which many persons 'nave upon high places is a mild form of hystero-neurasthenic vertigo. Many irritating and destructive diseases of the nasal cavities, of the larynx, and elsewhere about the head often provoke vertiginous sensations. Intoxications, such as those of alcohol, lead, nicotine, caffeine. I56 THE NEURONIC DISEASES autointoxication from alimentary disorders and intestinal parasites, in- fectious states are all prone to manifest among their most prominent symptoms nausea and vertigo. In this class probably falls the so- called paralytic vertigo which is endemic in the Geneva canton, Switz- erland, and which is attributed by Geslier to misasmatic influences. The Kubisagara of Japan is probably the same sort of a trouble. Anaemia produces the symptom. Malnutrition of the nervous ele- ments may provoke the same functional disorders in them sometimes as does intoxication. Atheromatous conditions of the arterial walls and circulatory disorders, especially sudden changes in the intracranial circulation are characterized by attacks of giddiness. The climacteric vertigo is probably largely circulatory in origin though other factors play a role also. Disseminated sclerosis with its apoplectiform seizures is notable for its vertigo. A moment's reflection upon the pathology of this affec- tion makes clear the raison-d'etre of the vertiginous phenomena. Vertigo may even have a psychic origin. It is not uncommon in hypochondriac neurasthenia. Oppenheim even goes so far as to assert that it is his belief that every one may produce in himself a feeling of dizziness by thinking of incoordination and by depicting to himself the feeling. Prognosis. The hope of recovery from attacks of vertigo is en- tirely dependent upon the curability or removal of the pathological state out of which it arises. Intoxication and alimentary vertigoes are prac- tically all curable. The dizziness that accompanies arteriosclerosis very often disappears as the disease progresses. Sometimes that oc- curs in other cerebral troubles, though as a rule these vertigos are ex- ceedingly persistent. Removable conditions in the nose, eyes, ears or alimentary tract may all be expected to carry with them when removed the giddiness which forms a part of their symptomatology. Meniere's vertigo is very resistant to treatment. It often disap- pears leaving behind it disturbance of hearing. Treatment. This should always be toward the removal or amelioration of the cause or underlying disease. There is no need to say that intoxications, infections, reflex irritants, dyscrasiae and cachexias of all kinds, and anatomical and functional anomalies, should all be corrected and removed as far as possible. It would be a mere waste of space to recapitulate here all of the measures for doing this when reference to them can be made to the various chapters devoted to the diseases with which vertigo is associated. The symptomatic treatment of vertigo should only be temporary and when all efforts to remove its obvious cause have signally failed. Amyl nitrite, nitroglycerin, the bromides, phenacetin, hydrobromate of quinine, alkalies, digitalis, strychnia, etc., may be employed when the appropriate condition is present. Glyerinophosphates, gold, the ferruginous preparations, bichloride of mercury may be thought of for their tonic, and alterative effect and be administered for some time in small doses. The bromides and belladonna have relieved some cases of Meniere's vertigo under my care. The treatment with massive doses THE NEURONIC DISEASES 1 57 of quinine, suggested by Charcot, has ignominiously fafled in a num- ber of cases in which I tried it. To be sure the vertigo left after the use of the alkaloid for several weeks but Meniere's vertigo not infre- quently ceases thus without any medication. Charcot recommended that the quinine be given up to the point of cinchonism. At first the trouble may be made temporarily worse. On account of the danger to the hearing, aurists urge great conservatism in the use of quinine. If the patient is willing to exchange deafness for the vertigo, Charcot's suggestion to puncture the membrana tympani may be put into prac- tice. Blood-letting is heroic for vertigo but it has done good in some cases when intense congestion was present. In other cases sodium salicylate or hypodermics of pilocarpine even- other day have relieved most distressing attacks. After all it must be admitted that the only treatment of this symp- tom is the treatment of the malady to which it belongs. All other at- temps to relieve it are unscientific, haphazard and usually disappointing. NEURASTHENIA. If the term disease is to be made to cover all forms of abnormal manifestation, then neurasthenia is entitled to be regarded as a dis- ease as well as cancer or syphilis. No manifestation has been more happily named, for nerve-tire, nerve-exhaustion, nervous inadequacy express exactly the essential condition. It is a nerve-tire, however, that is abnormal in the sense that it is not immediately recovered from as in the temporary nerve-tire that results from a slight over-exhaustion. The nerve-inadequacy has fallen below such a point, either by the severity of the cause, the prolongation of the trouble, or the peculiar susceptibility of the patient, that certain functions within the neurones and in those tissues in direct relationship with those neurones are affected. As a consequence a more or less definite symptomatology is presented. The exhaustion is characteristic of the entire nervous system. It is a disease of the nervous apparatus in ioto. Though in different pa- tients it may manifest itself more prominently through the cerebral, spinal or peripheral nervous apparatus according to the peculiar con- stitutional and temperamental predilection of the patient, there is no good reason for subdividing neurasthenia into cerebrasthenia. myelas- thenia, sexual neurasthenia, gastric neurasthenia, etc. One does not divide typhoid fever into diarrhoeal, febrile and delirious types be- cause individual patients exhibit more prominently enteric, febrile or neurotic symptoms. The only logical and practical subdivision of neurasthenia is into the congenital and acquired forms. The former is a true nervous in- adequacy, a defect of prenatal origin, a failure of biological develop- ment; the latter is a true nervous exhaustion, a postnatal depreciation, a failure of physiological function from depressing influenc Etiology. Heredity plays a very important role in the develop- ment of neurasthenia. In the congenital types of the disease it is prac- I58 THE NEURONIC DISEASES tically the sole cause. The patient is born with an unstable and inad- equate nervous apparatus which later on reveals its symptoms of irri- table weakness not as a result of the trials and struggles of life directly but rather because it cannot meet and override, by reason of its inher- ent weakness, these trials and struggles which to a normal nervous ap- paratus would be as nothing. In these cases the environment is not the cause of the trouble but rather the patient's inherited nervous ina- bility to accommodate himself to what would be a normal environment. These cases are hard, if not impossible, to treat, because every sort of social condition provokes the neurasthenic phenomena in their irri- table and unaccommodating nervous organism. The large majority of the cases of acquired neurasthenia are en- dowed by heredity with what is called a neuropathic constitution. They have a nervous apparatus which under proper Conditions may never reveal any neurasthenic symptoms but which under other conditions leads it to break down rapidly and seriously. Unlike the former class of individuals, these are merely unstable. With some of them even work and strain that would be unfavorable to perfectly normal indi- viduals do not produce deleterious effects. These are cases that are always neurasthenic until they are placed in a position or are given tasks that harmonize with their tastes, their training, their mental calibre and their feelings. In the development of these neurasthenics their environment is a most important element but it has as a co-operating factor the inherited, unstable, unresisting nerv- ous organism which is prone to topple over when that environment be- comes particularly inharmonious. Many artists are of this class and it is among them that the so-called artistic temperament is mostly found. Their nervous organization is not exactly abnormal but it is so mobile, sensitive and responsive that very slight causes provoke it into revealing abnormal symptoms, which if long continued may result in a complete nervous breakdown. These cases are amenable to treatment but from the very nature of things, their management is difficult, taxing and tedious. I should say that from eighty to ninety per cent, of the cases of typical neurasthenia fall into one or other of the above classes in which heredity plays so important a part. Among the antecedents we find various nervous troubles, such as neurasthenia, hysteria, migraine, epilepsy, melancholia, insanity and other neuroses and psychoses. The family taint runs along the nerv- ous line. Gout and rheumatism are not infrequent in the ancestry. Alcoholism in the parents, excessive use of tobacco, dissipation, over- indulgence of the sexual appetite, are all frequently observed in the heredity. Severe and prolonged mental strain without a correspond- ing or appropriate attention to their physical vigor and functions In the parents will result in a neuropathic constitution in the child. The same may be said of all forms of monotonous, one-sided activity whether mental or physical. These neurasthenics occasionally expose their bad inheritance in slight physical stigmata of degeneracy. In a word all factors that tend to lower the mental and physical health of the parents, tend to confer on the children a nervous consti- THE NEURONIC DISEASES 1 59 tution that is neurasthenic or prone to become neurasthenic upon the slightest occasion. The purely acquired cases of neurasthenia, cases in which the con- stitution was originally strong and the heredity excellent, do occur though they are comparatively rare. In such cases only exciting causes can be charged with the disease. Among the exciting causes of neurasthenia we recognize both the mental and the physical. Mental causes include continued and exalted emotionalism, exces- sive use of the intellect, and all forms of mental strain, especially it associated with worry and anxiety. It is a fact, general opinion to the contrary notwithstanding, that mere overwork or hard work does not result in mental breakdown, if the individual's inheritance is good, the work is not disagreeable and his habits as to sleep, rest, nutrition and systematic physical exercise are carefully regulated. If properly guarded in this way and if properly exercised in regard to all of its faculties equally so as to avoid an unbalanced activity, the human brain is capable of bearing a greater strain than is ever put upon it by the average work of man. It is the worry, the fret and the one-sided strain and unsystematic, unwholesome use of the brain ; and not the mere amount of the work imposed upon it, that enter chiefly into the mental causation of neurasthenia. Close to the mental causes are the nervous strains. Usually the two go together. Thus sexual irregularities, excessive venery, and masturbation frequently develop neurasthenia though at times they are but the expression of a neurasthenic irritability. Cause and effect here must be carefully differentiated in each individual case. Many of the causes of neurasthenia are kept alive by the neuras- thenic state, so that a "vicious circle" obtains and must be broken some- where. Traumatic neurasthenia is usually due to a psychoneurotic shock. The same may be said of the prolonged irritation superinduced by the many reflex causes that are mentioned in the books. A local nidus of disease, a physical defect which disturbs the associated functions, or a physiological abnormality of any sort may well be conceived of as an exciting cause, acting in a reflex way upon a predisposed neuro- pathic apparatus. In this manner eye-strain, pelvic disease, gastro- intestinal trouble with associated malnutrition may all provoke and prolong an attack of nervous exhaustion. Infectious diseases and all forms of toxaemia may act as causes. The toxin may act directly upon the neurones so as to diminish their nutritive and specific function, or it may act indirectly upon the pri- mary assimilation of the alimentary tract and circulatory apparatus. Syphilis for instance injures both the functions of nutrition and the neurility of the individual neurones. Any of the infectious disea as well as intoxication by alcohol, nicotine, lead and arsenic, may thus cause neurasthenia. Autointoxication can be invoked as a factor, and in close connec- tion with it is the enteroptosis and gastroptosis referred to in Glen- ard's theorv. l60 THE NEURONIC DISEASES A severe loss of blood may very obviously produce neurasthenia, the blood being the source of the nutrition of the nervous elements. Some careful examinations of the blood of neurasthenics by Holdam, Smith Lodor and others show that it is almost constantly deficient in oxygen carrying capacity. This of course affects the in- ternal metabolism and suggests some form of toxaemia as an underly- ing cause. Finally it must not be forgotten that even severe and prolonged physical exercise sometimes terminates in neurasthenia. Many a mild neurasthenic is made worse by adopting, under a mistaken idea, a vig- orous regimen of outdoor games and indoor gymnastics. The one- sidedness of all such measures, apart from the fact that they only add more strain to an already strained nervous apparatus, would seem to be enough to condemn them for the treatment of neurasthenia. Euro- pean trips, incessant golf and tennis playing, long constitutional walks may in this way, when undertaken at the wrong time and without ad- vice, be a most fruitful cause of severe nervous prostration instead of a remedy as was hoped for. Pathology and Pathogenesis. As long ago* as 1869 Beard stated his conviction that there were definite structural alterations in the nervous system in neurasthenia though undetectable with our pres- ent means of research. To call the trouble a functional disturbance and deny all possibility of change in the functionating structure is an illogical contradiction. If the term neurasthenia is to include all forms of secondary as well as primary nervous disturbance we will have to admit that there is more or less truth in all of the theories of the disease based upon the many and various extraneural pathological findings. The etiolog- ical substrata of the affection will in many cases have to be included in its catalogue of pathological contents. Some consideration will have to be given then to the cellular vibratory theory of Beard, the spinal hyperaemia and anaemia account of Hammond, the dyspeptic malnu- trition of Hayem and Winter, the faulty cellular metabolism within the sensory tracts of Jewel, the vasomotor irregularities of Dumas, the gastrointestinal disarrangements of Leven, the enteroptoses of Glenard, the autointoxication theory of Bouchard, the cerebrospinal hypotonia of Cheron, and the altered nutrition in nerve elements of Erb and others. None of these theories and findings are universally applicable or sufficiently constant to make them the basis of a true pathology or pathogenesis. In simple primary neurasthenia there are no discoverable perma- nent pathological alterations in any of the internal organs of the body. We are forced to conclude, therefore, that the entire pathology of neu- rasthenia as a disease is to be found in the molecular and chemical changes, brought about by various causes, within the nervous elements, the neurones themselves. To C. F. Hodge is due much credit for revealing the changes which the neurones undergo as the result of prolonged exercise and stimulation. Basing his conclusions upon a series of experiments upon THE NEURONIC DISEASES l6l the lower animals, he affirms that the cell-nuclei are invariably shrunk- en, displaced within the cell and bounded by an irregular, jagged edge. They lose their open and reticulated appearance and become darker upon staining. There was observed a regular quantitative relation be- tween the strength of the stimulus and the amount of nuclear shrink- age. The nucleolus in some instances contained granules, which moved slowly about and were extruded into the nucleus. The move- ments of the nucleoli had every appearance of being amoeboid. After they had become somewhat broken up they were rapidly dissolved. Under the same conditions the size of the entire cell was diminished and its power of absorbing stains was greatly lessened. Vacuolation appeared within the cell protoplasm, a proof that the cell substance was used up in the action. In this process "the cell tires rapidly at first, then more gradually to a condition of fatigue." Vas observed that mild stimulation caused a swelling of the cell body while the central part of the cell was rendered more or less clear by the move- ment of the chromatin bodies toward the periphery. Similar observa- tions have been made by Mann who states that it requires prolonged and adequate rest to restore these exhausted cells back to their original appearance. The shrinking of the cell at its nucleus, together with the vacuo- lation of its substance, may be made to take place also by the injec- tion of various toxic substances into the blood, as has been done with absinthe in guinea pigs (Starr). Toxaemia not only affects the cell body. In all probability the earliest changes brought about in this way in the neurone occur in the delicate protoplasmic endings (Thom- son). From experiments made with potassium bromide Wright con- cludes that the inhibiting power of this medicament upon intellectual and motor processes depends upon its effects upon the ends of the protoplasmic processes, while the cell bodies themselves are only made to suffer after prolonged exposure to its influence. If the blood supply to the nerve cells be diminished or its nutri- ent quality be impoverished, changes similar to those just described occur with singular constancy in the cells. It is a fair conclusion that the same causes, overstimulation, toxaemia and malnutrition which with an inherited neuropathic consti- tution we see play so important a part in the etiology of neurasthenia, produce the same or similar alterations in the nerve cells of the victims of this latter disease. Symptoms. I am in hearty accord with Dana when he says that fifty per cent, of the cases of neurasthenia so-called, may be classed under other heads of disease. The term is much abused. The symptoms of neurasthenia are not only numerous and varied but they never group themselves exactly in the same way in all pa- tients. This is not to be wondered at when it is remembered that the disease is one involving the whole nervous organism and that its clin- ical presentation is modified by the peculiar constitution and tempera- ment of the victim. In spite of all this, however, there is a degree of uniformity in the clinical picture that makes it worthy of a special place in medical nosology. This clinical picture is sufficiently distinct 1 62 THE NEURONIC DISEASES even to render possible a differential diagnosis of neurasthenia from the many other troubles which it often resembles, and with which it is frequently associated, though at times it is extremely difficult. In a general way it may in the first place be stated that all of the symptoms of neurasthenia reveal and depend upon a condition of irri- table weakness. Whether in the mental sphere or the nervous, whether in the field of sensation or motion, whether vasomotor, secretory or trophic, the symptoms that present themselves always represent ab- normal fatigue, instability from weakness and diminished power of re- sistance, and an utter lack of sustaining force. This striking characteristic of the symptomatology of nervous ex- haustion is constant and uniform. It is pathognomonic. Analyze every manifestation of neurasthenia and if it represents a fatigue symptom it points to nervous exhaustion and not to any other neuro- sis. It differentiates this disease. In all neurasthenics certain symptoms appear with a remarkable degree of persistency when one remembers the generalized and ex- tensive character of the trouble. Among these may be mentioned a peculiar type of headache, and backache, vertigo, insomnia, melan- cholia, depression of spirits, bad dreams, cardiac palpitation, nervous dyspepsia with constipation and general restlessness and fear of im- pending ruin. I will now discuss these and some other indications more in detail. The patient's mental sphere is always invaded by the disease and hence we find that all of his psychic occurrences are depressed. His memory is poor, especially for names and the more recent events. His power of attention and concentration are annoyingly diminished. When reading his mind wanders off upon other subjects though his eyes may go on taking in the printed words. He cannot sustain, as he used to do, a continuous line of argument. His thoughts at times seem to vanish and thus for a moment cause him to lose the thread of his discourse. His will power has lost its old vigor; he plans this and that but he finds it hard to muster the energy to start his plans. His imagination is irritable and active in depicting disagreeable and hor- rible scenes. His dreams are all of an unpleasant and depressing char- acter. This vicious activity of his imagination is often the cause of the partial aboulia. The patient imagines all sorts of disasters and unfavorable condi- tions that may arise and so dreads to begin any undertaking. His judgments are all tinctured with this gloomy, negative view of life. He sees everything, as it were, through blue glasses and therefore all his acts and thoughts and conversations are suggestive of gloom, pes- simism and depression. His whole mind is in a state of psychic pain, mild psychalgia. Often the melancholia is so severe that insanity is thought of by the patient, his family and family physician. Occasion- ally it leads to suicide. These cases must be carefully separated from dementia precox, attacks of melancholia or manic depressive insanity, and such phrenas- thenic conditions as dipsomania, hysteria, obsessions and the exhaustion psychoses. THE NEURONIC DISEASES 163 Strange to say, neurasthenics with all their melancholy and de- pression are not hopeless of getting well as the hypochondriacs are. I make it a rule to question all of them, and gloomy as they may say they feel at times, they almost invariably declare they believe they will get well if the proper line of treatment can but be hit upon. I almost regard this in itself as a valuable sign of true neurasthenia. The feeling of dread which these patients have is usually indefinite and unaccountable. Often, however, it assumes the definite form of a mild phobia. The phobias of neurasthenia are innumerable so that only a few of them need be named by way of illustration. Agoraphobia (fear of crossing a large open square), potamophobia (fear of crossing a run- ning stream), claustrophobia (fear of being alone in a narrow place), astrophobia (fear of storms), pantophobia (fear of everything) are a few of the special varieties. It is unnecessary to dignify these phobias as special "fear neuroses,'' as Hecker, Freud and some others have done, for they do not occur alone or without some other neurasthenic symptom being present. They are of the nature of an obsession or fixed idea which is not an uncommon symptom of neurasthenia. I had a neurasthenic lad under my care who saw the body of his drowned brother shortly after it was taken out of the water. He was so much the victim of potamophobia and fear that he would himself drown some day that he never could be induced to cross over water. When inveigled into approaching a bridge he would suddenly turn and run to his room, lock himself in and suffer from severe trembling and fright. He recovered in about a year, the phobia and obsession dying out as his other neurasthenic symptoms disappeared. A young clergyman under my care was harassed with the fixed notion that he was offensive and ridiculous in the eyes of every human being he met, though as a matter of fact he was most agreeable and much sought after. These patients are all mentally irritable. They are oversensitive and easily offended ; hence they appear egotistical at times and lead a more or less solitary life. Their lack of sustaining power makes them often appear changeable, frivolous, weak and inconsistent. Nearly every neurasthenic complains sometime of vertigo. This is not a continuous symptom, nor is it very severe. It is usually of the objective type. The patient feels as if he were stationary, while objects are whirling about him. Occasionally in severe cases it is sub- jective, the patient feeling as if he himself were the spinning object. It is provoked generally by sudden and unexpected movements. Often the floor seems as if it were rising up. It is not accompanied by vomit- ing often, though it is associated not infrequently with a feeling of nausea. It never occurs at night nor when the patient is lying down. It is experienced in the morning soon after rising. It is felt when the patient ascends high places, stands upon the edge of a precipice or looks at rapidly moving objects, such as a passing train. Under these conditions it sometimes takes the form of an impulsive desire to jump from a high place, to rush in front of the train or attempt a sort of bird-like flight. 164 THE NEURONIC DISEASES These vertiginous impulses are occasionally very distressing and I have known people to be in constant dread from them of committing suicide. It is not at all improbable that some cases of unexplainable self-destruction by leaping from high places and plunging into deep cataracts may have been vertiginous attacks of this sort. Insomnia is a remarkably constant symptom of neurasthenia. The patient undoubtedly loses a great deal of sleep, but not as a rule so much as he imagines he loses. Trie sleep, such as it is, is broken and fitful and more or less disturbed with troubled dreams. Sleep during the day is almost impossible, even when the conditions are made par- ticularly inviting. The early part of the night may be occupied with sleep, but about two or three o'clock in the morning the patient awakens and thereafter remains awake until the next night. More rarely the patient passes the night in short, cat-like naps. Such sleep does not bring refreshment and as a consequence neurasthenics always feel tired and reluctant to get up when morning arrives. Among the subjective symptoms the sensory phenomena, both general and special, hold an important place. As aids to neurological diagnosis all subjective symptoms, and especially the sensations, must be studied with great care. The value of the patient's statements in regard to himself must be weighed with caution. Nevertheless, so constant and so uniformly similar are some of these symptoms that we cannot but help accepting them as distinct and valuable signs of the disease. The sensations of neurasthenia are generally of the nature of paraesthesia, very rarely hyperesthesia or hyperalgesia, and most rarely anaesthesia or analgesia. Nearly every neurasthenic complains of some form of pain. Headache is the most frequent. Even the headache is not a well-defined pain, but rather a form of cephalic paraesthesia. The patient complains of the head feeling loaded and full; or it is compressed as in a vise; or it seems as if it were covered with a heavy, leaden, tight-fitting skull- cap. One patient described it as though there were a thousand strings attached to her brain and some one were steadily pulling upon them all at the same time. Another corrected me every time I used the word headache, for she wanted me to distinctly understand that this distress was nothing at all like the distinct and painful headaches which she frequently suffered from. These neurasthenic headaches vary in severity, though they are always more or less present. A close observation of them reveals the fact that they exacerbate almost always after some unusual or pro- longed exertion. Therefore they usually appear shortly after the pa- tient rises in the morning. As the stimulation and distraction of the day's duties proceed they seem to partly die out, or at least are not so noticeable. But in the latter part of the afternoon, when more or less physical and mental exhaustion begins to come on the headache re- turns with greater intensity even than it had in the early forenoon. No other form of organic or non-organic cephalalgia occurs in ex- actly this way or possesses exactly these characteristics; hence the headache of neurasthenia is a valuable symptom of the disease. THE NEURONIC DISEASES 165 Backache is another very constant sensory symptom. It also is fairly characteristic, at least enough so to aid materially in making a differential diagnosis from other affections in which dorsal pains are present. The entire spine of the neurasthenic feels sore and tender, not more so in one part than in another. Pressure and percussion do not specially provoke tender spots nor does the passage along the spine of a hot sponge or a piece of ice. The pain seems to be eminently subjective, being more like a feeling of heaviness, burning, chilliness or aching ; it is of the nature of paresthesia rather than a hyperalgesia. Some patients complain of a numbness or stiff feeling in the spine and its related muscles. They attribute this feeling often to cold, rheumatism, or lumbago. It is usually relieved by the recumbent posture. The nerves of common sensation are implicated in nervous ex- haustion. The patient complains of ill-defined feelings of distress all over the body and in the viscera; of pressure, of flashes of cold and heat; of ill-defined, fugitive pains here and there. The very indefi- niteness and indescribable character of these sensations, their varia- bility in intensity and their dependence upon the patient's feelings and his condition of weariness are their characteristic attributes. There are such pains about the heart, leading to a fear of organic heart disease. This fear, with the neurasthenic trait of introspection, superinduces palpitation. Pains in and about the stomach are frequently referred to. As they are not influenced especially by the ingestion of food or associated with nausea and vomiting and other signs of organic gastric trouble, but are accompanied by nervous dyspepsia and troublesome eructations and feelings of distension, their nature is easily determined. The cutaneous as well as the visceral paresthesia: are sometimes very curious. Formication is common. It seems as if ants or worms were crawling under the skin. Pinchings here and there and flea-bites are imagined and give much annoyance. More frequently there is a feeling of coldness in the hands and feet, which the patient invariably attributes to poor circulation. Sometimes this peripheral coldness sud- denly alternates with a sense of heat as if hot irons were touching the skin. Among the visceral paresthesia? are such feelings as rectal con- striction and paralysis. I have seen some ludicrous cases of this. Pruritus ani is in most cases a cutaneous paresthesia of neurasthenic origin. The bladder may seem to be overdistended, obliging the patient to get up unnecessarily often to empty it at night. These visceral paresthesia?, when associated with morbid fear, sometimes give rise to most amusing situations and lead the neuras- thenic in his ignorance to suggest very humorous anatomical and physiological explanations. One told me once she knew she was losing her mind because she could actually feel her brain slowly but steadily softening. Patients have insisted that they had a tape-worm, that their lungs 1 66 THE NEURONIC DISEASES were entirely rotted away, that their wombs were misplaced or their testicles were shrunken. Such patients go the rounds of the physicians' offices and too often, alas ! find men who treat them for this or that local ailment so long as their money holds out. Cutaneous and visceral hyperesthesia is not infrequently met with. Simple increase of sensitiveness, without special perversion, may be neurasthenic in origin ; it is frequently, however, a sign of organic disease. Care must be exercised when making a differential diagnosis in the study of sensations that show a simple increase. In neurasthenia hyperesthesia is revealed by the patient's abnormal sensitiveness to cold and heat, the distress caused by the pressure of his clothing. The ill-defined internal sensations associated with the viscera are undoubtedly due sometimes to hyperesthesia. It is a very rare thing for the victims of true neurasthenia to com- plain of hyperesthesia or anesthesia; such sensory feelings belong more to hysteria and organic diseases. As hysteria and neurasthenia are so often combined in the same individual, the anaesthesia is more apt to be a symptom of the former. Nevertheless I have seen cases of pure neurasthenia of a profound type in which objective signs of a localized hypaesthesia (never anesthesia) were complained of. In these cases examination with the faradic brush seemed to show that there was not a real loss of sense, at least to an}- marked degree. The symptoms dependent upon involvement of the special senses are among the most important in neurasthenia because they are so uniformly present. Tinnitus aurinm, ringing, buzzing, whistling sounds in one or both ears are very common. Frequently the left ear is the seat of the trouble and at times it is so annoying that the patient declares it will set her crazy. In spite of removal of cerumen and local treatment for chronic otitis, which these patients so often insist upon, the annoyance continues. Aural hypersensitiveness, on account of which the patient starts at every little noise, is rather a psychic trouble than an auditory nerve weakness. Visual disturbances are represented by spots flying before the eyes, so-called musce volitantes, by swimming and vibrating and blur- ring of the printed page during reading. The eyes tire easily and water freely. Before a glare of bright light they may even pain sharp- ly. Vision itself may remain normal ; in fact, it may even be ex- ceptionally keen. Taste and smell are rarely if ever affected. I have seen a hyper- sensitiveness of these senses which was probably a mental or psychic symptom. The motor symptoms of neurasthenia are of minor importance as compared with the mental and sensory symptoms, but they are oi value on account of their objective character. They include chiefly weakness, tremor, rapid exhaustion and fatigue. The patient never puts forth his entire strength when he attempts to perform a muscular movement. He shows a marked disinclination and thus in part reveals the psychic nature of his trouble. Sometimes, on the other hand, he exerts considerable force, but it soon relaxes. THE NEURONIC DISEASES 167 This weakness of muscular action in neurasthenia never advances to the point of actual paralysis. In fact, it bears no similarity to or- ganic paresis. It is generalized throughout the entire muscular ap- paratus and is unaccompanied by atrophy or electrical reactions. Muscular overaction or true spasm never occurs in neurasthenia in the common acceptation of the term. The tremors and twitchings are not so much of the nature of spasms as they are of perversion and irritability of normal muscular contraction. Blepharospasm, or twitch- ing in the outer corners of the eyelids, is common. It is in part a sensory disturbance and in part a fine fibrillary tremor, myokymia. Tremor is a frequent symptom and usually consists of rapid vibra- tory movements of narrow excursion. Mental excitement and active movements in general are accompanied by this tremor. The patient says his hands shake when he writes, his head trembles, or his lips or his knees, when he becomes unusually excited. Fibrillary tremor in the muscles of the leg is often a most distressing symptom. It may attack any of the muscles of the body. Many people have cramps in the calves of their legs, especially when they are suffering from night- mare, but such cramps are not specially indicative of neurasthenia. Closely related to the sensory and motor symptoms is the state of the reflexes. As a rule the deep reflexes are exaggerated, both those of the leg and those of the arm. A foot clonus is sometimes elicited. Oppenheim declares that all other signs of muscular rigidity are absent, notwithstanding the statements of Binswanger and others. The myotatic irritability as well as the nerve excitability are both increased. In the severest forms of emaciation the knee-jerks may be diminished. Though largely true, it is not absolutely so, as Oppenheim asserts that the absence of the knee-jerks means always and only a symptom vji organic disease. I know a woman, perfectly healthy in every respect except for a slight nervous dyspepsia, in whom the patella tendon re- flexes are completely absent. The pupillary reflexes are somewhat exaggerated, though the con- dition of the pupils is generally normal. The irritable weakness of neurasthenia is revealed symptomatically in the vasomotor, secretory and trophic mechanisms also. Flushing and a rush of blood to the head are spoken of by nearly all patients. This is often associated with the vertiginous feeling of faintness and cardiac palpitation. The condition is observable objectively, for the skin is suffused with a general redness and its temperature is slightly elevated. Under the name of erythrophobia has been described a most stub- born and troublesome form of morbid fear of blushing. As Hartenberg has pointed out, the blushing disease, erythropathy, should be distinguished from the vicious neurotic circle set up by the fear of blushing, or erythrophobia. Shyness or shame, not related to fear, causes the flushing. This is a well-defined and frequent phenome- non. When fear is added to it, the two emotions influence and react upon each other. Blushing creates the fear of blushing and the fear creates the blushing. The latter becomes a writable obsession. The vasomotor disturbance mav even be so great as to cause l68 THE NEURONIC DISEASES wheals, as in urticaria factitia. I had a highly emotional young man, a professional musician with a high order of poetic temperament, once under my care, who almost nightly, as soon as the irritation and warmth of the bedclothes encompassed him, suffered from a form of neuras- thenic hives. Redness, local oedema and itching were marked. At first I thought it was a case of angioneurotic oedema. He fully recovered under the treatment for neurasthenia after a number of months. Certain special functions are so frequently affected in neurasthenia that they have come to occupy a place of considerable prominence in its symptomatology. I refer especially to the alimentary, circulatory and sexual functions. These troubles are largely subjective in char- acter and yet are not without certain objective features. Nervous dyspepsia in the broad sense of the term is practically always present. The patient complains of not being able to digest his food. It often seems to lie in his stomach like a heavy weight. His appetite is capricious ; usually it borders well upon anorexia or the entire want of appetite. The taste for certain articles of food may be changed. There is a craving for stimulants, like tea, coffee and alcohol. Shortly after taking the food there is a feeling of distension and fre- quently an annoying eructation of gas. Pyrosis or heartburn with water brash then follows. Finally this passes into a feeling of rawness and irritation in the stomach, with which is associated the old gnawing, empty feeling so unlike normal hunger. Nausea is not common and vomiting still less so. Persistent thirst and dryness of the mouth are common, especially at times of special nervous excitement. It is strange that there is not more deterioration of the physical health with all these gastric troubles! To be sure, the patients do lose flesh from a failure to eat, but the anorexia is the result of the dread of these vague sensations of gastric distress coming on, and even in the severest cases, wherein there may be vomiting, suffocation, cardiac palpitation and meteorism, it is out o>f all proportion to the actual loss in the physical condition. The slightly coated tongue, the eructations and the frequent hyperacidity add objective confirmation to the patient's un- doubted distress. Constipation is almost universal, depending upon peristaltic torpor and muscular atony. Borborygmi and rumbling in the bowels are frequent. Occasionally there may be attacks of temporary diarrhoea and in some cases there is a constant tendency to diarrhoea, which must be guarded against by the avoidance of every sort of emotional excite- ment. Scybalous fecal and mucous masses appear in the stools. In very rare cases these symptoms lead to a high degree of ema- ciation. This emaciation with pallor and loss of strength can be initiat- ed by the nervous dyspepsia and continued insomnia. Nevertheless it is a fact that a large number of neurasthenics do not suffer from bad general nutrition. Some of the greatest sufferers I have seen have been people who were outwardly the very picture of health. Palpitation of the heart is a symptom very commonly complained of. To a certain extent it is subjective in character and is associated with feelings of suffocation and precordial pain which may shoot down THE NEURONIC DISEASES 169 the arm. I have noticed that a fear of organic heart disease has often been the only thing- that sent these patients forth for medical advice. The so-called "tobacco heart'' is a good example of a neurasthenic heart It is subject to tachycardia, every wave of psychic activity causing it to vary in its rhythm, Intermittency of the beat is not uncommon. Allorhythmia and arrhythmia and even bradycardia have all been at- tributed to neurasthenia. Anaemic and systolic murmurs may be heard, but they are not sufficiently common to make them very valuable as signs. Vascular murmurs are spoken of. It is claimed by Gerhardt that more than one-half of those who had consulted him for palpitation of the heart had some functional disorder of this organ and not valvular disease. As a result of all these cardiac manifestations a special form of neurasthenia — neurasthenia cordis — has been, somewhat arbitrarily, established. Attempts have been made to locate the trouble in the sympathetic nerve or in the vagus, but without any very satisfactory results. For the present the irritable and weakened nervous apparatus is enough to account for the irregularity of action in an organ so directly under the influence of the nervous apparatus as the heart is. In the sexual sphere neurasthenia reveals itself in various ways. In regard to some of these manifestations it is a difficult matter ro determine always the proper relationship as to cause and effect. There is no good reason for segregating the sexual symptoms into a special form of disease and calling it sexual neurasthenia. The other mani- festations of the defective nervous system may be less noticeable on ac- count of the prominence of the sexual aberrations, but they will always be found if carefully sought for. In congenital neurasthenia there are weaknesses and perversions in the sexual sphere that show themselves early and are difficult to overcome. Masturbation is sometimes an early evidence of the nervous instability and irritability in both the mental and physical constitution of the congenital neurasthenic. Indeed Op- penheim makes the assertion, and I am inclined to agree with him, that the tendency to masturbate can be inherited. The habit once established, and ever increased as it always is, puts a strain upon the nervous mechanism and reacts upon the patient's mind through fear, shame and other depressing mental processes in such a way that the neurasthenia out of which the habit originally grew is all the more developed. Thus the vicious circle is again established, just as it is in the gastric and other special troubles. Sometimes these congenital neurasthenics, whether addicted to the habit of masturbation or not, are chagrined to find themselves im- potent. Usually this is psychic in origin and is due to local irritability. Such individuals find that they have their erections, emissions, nocturnal pollutions and even the orgasm, all more or less associated with what seems to be a normal sexual desire, but under peculiar and abnormal conditions, such as when out in society or under the strain of prolonged work, or of intense emotion. When they attempt to cohabit normally the erection fails to take place, or it is too brief, or ejaculation with relaxation occurs before penetration. If the intercourse is accom- I70 THE NEURONIC DISEASES plished there is such a sudden and complete reversal of the feelings from pleasure to disgust that the act becomes one to be avoided rather than indulged in. All of this still more depresses the patient and in- creases his nervous weakness and irritability. Many of the cases of sexual perversion are probably congenital neurasthenics. Sadism, Masochism, fetichism, pederasty and all forms of abnormal modes of cohabitation may in some instances be due to the neurasthenic congenital twist of the mind ; in other words, the result of a distinct psychosis : or they may be due to the efforts of a congenital neurasthenic to gratify his passion in some way, since he is deprived, by reason of his abnormal irritability, of gratifying it in the normal way. These abnormal excitations all increase the original trouble and the patient becomes still more neurasthenic. Spermatorrhoea, prostatorrhcea and all forms of seminal emission may be the result of a congenital or acquired neurasthenic weakness. Many patients have such discharges every time they defecate. Often the urine contains some spermatozoids. All of this worries and cha- grins and acts as the real depressing agent and causes the nervous ex- haustion more than does the physical effect of the seminal loss. Fiir- bringer speaks of an onanistic neurosis and Krafft-Ebing makes the attempt of dividing sexual neurasthenia into the three stages, a genital local neurosis, a lumbar or spinal neurosis and a general cerebro- spinal neurasthenia. This, it seems to me, is an unwarranted and con- fusing refinement of classification. The sexual phenomena are but one phase of a general cerebrospinal neurasthenia. The prominence of the sexual manifestations is due to many factors, such as the con- genital or acquired nature of the neurasthenia, the mental characteris- tics of the patient, the particular conditions and environment in which he lives. The sexual symptoms are distinctly psychoneurotic in origin and are dependent upon both general and local influences. They are always in part both symptomatic and causative of neurasthenia and therefore enter into the constitution of a vicious circle. The recogni- tion of this fact has an important bearing upon the treatment of the cases in which the sexual features seem to be so prominent. The condition of the urine in neurasthenia is not pathognomonic. As a rule it is more or less condensed and reveals variable evidences of a defective metabolism. The daily amount, as well as the specific gravity, is apt to be somewhat below normal. An excess of phosphoric and oxalic acid is frequent, but not characteristic, as is too often as- sumed. Indican in pathological quantity occasionally occurs and the relation of uric acid to the urea varies from I to 40, or 1 to 30, instead of from 1 to 45, or 1 to 60, as in normal urine. A transitory glycosuria is occasionally met with. Less frequently a temporary albuminuria without casts or other signs of renal disease is seen. There is no alteration of temperature in neurasthenia, though of course vasomotor disturbances may modify the local heat. Hypericin) sis is common enough to make it a valuable symptom when taken in conjunction with others. Sometimes, however, the skin is dry. Alopecia and. according to Beard, a tendency to an early decay THE NEURONIC DISEASES 171 of the teeth are to be noted among the trophic general disturbances. I have seen oedema as a hystero-neurasthenic symptom In spite of the frequent anaemia and loss of weight in many neuras- thenics, their blood is not found to be markedly abnormal. The cor- puscular richness may be up to the normal and, according to Hosslin, there is a healthy amount of haemoglobin. A thoughtful consideration of the entire symptomatology of neu- rasthenia, I think, will convince any one that it is a disease of the whole nervous apparatus. Confusion in the mind of the practitioner may result from the fact that all of the symptoms do not stand out with equal prominence in every case. In fact, each neurasthenic is a law unto himself in regard to the outward expression of his nervous ex- haustion. If his inclinations and previous mode of life have been intellectual his psychic symptoms will be the more obtrusive ; if he has been a mere physical laborer his symptoms of physical weakness will "be the more prominent ; if his environment and education have been immoral his sexual and other moral delinquencies will give the prevail- ing tone to the picture. In all of the cases, however, other manifesta- tions will be discovered upon careful examination and thus show that the predominant symptoms are but a part of a general symptom-group. It is this symptom-group that we must always search for, because it possesses certain more or less uniform and persistent characteristics that justify the establishment of neurasthenia as a specific entity. The subjective characteristics of this symptom-group are the more con- stant and regular ones and include the insomnia, bad dreams, melan- cholia, vertigo, headache and other pains, paresthesia?, gastric, cardiac and sexual symptoms, general weakness and irritability. The objective traits of the group are second only to the subjective in prominence and constancy. They are sometimes coequal to them in value as additional signs whereon to base a diagnosis. They include increase of tendon reflexes, cardiac, vasomotor and secretory disturb- ances, tremor, increase of muscle and nerve excitability and the general history of mental and physical irritability. Diagnosis. — The diagnosis of neurasthenia is usually easy ; at times it is extremely difficult. It is the safest plan to always diagnose it by exclusion, because it so often simulates other affections and not infrequently is a part of the clinical manifestation of distinct organic disease. For example, the cardiac symptoms at times resemble those of various heart diseases; and again in the earliest appearances of syph- ilis of the central nervous system the clinical picture is very frequently that of simple neurasthenia. Much assistance is afforded the diagnostician when endeavoring to determine whether a symptom is neurasthenic or not by remembering that a neurasthenic symptom is always the expression of an irritable weakness; is one of a number of similar symptoms; is variable and changeable both as to location and time of appearance; and is a per- version of the normal function rather than a mere increase or diminu- tion of it. For example, in neurasthenia the sensory phenomena run more to bizarre and variable paresthesia? rather than to actual anaesthe- sia or hyperesthesia ; psychic and cerebral disturbances assume the 172 THE NEURONIC DISEASES character of melancholy rather than true melancholia, of vertigo of psychic origin rather than true organic cerebellar vertigo or ataxia; the motor weaknesses are the result of volitional inertia or absence of psychic initiative rather than the expression of a true neuromuscular paralysis, paresis or spasm. Neurasthenia should never be diagnosed upon too small a number of symptoms or upon a set of symptoms limited to one sphere of physiological activity. It may be convenient at times to speak of gas- tric, sexual, cerebral, spinal and other forms of neurasthenia in which certain symptoms stand forth with special prominence, but it is highly injudicious and unsafe to diagnose neurasthenia from such prominent symptoms alone. The disease is one of the entire nervous apparatus and the tout- ensemble of its symptomatology must be more or less discovered before- a clear and decisive opinion can be formed. The same may be said of the diagnosis of those arbitrarily classified forms of neurasthenia some- times spoken of as primary neurasthenia, hystero-neurasthenia, cli- macteric neurasthenia, traumatic neurasthenia, anxiety neurosis, an- giopathic neurasthenia, neurasthenia gravis. Some further points in regard to the diagnosis of neurasthenia itself will be brought out in the discussion of its differential diagnosis. The disease with which nervous exhaustion is perhaps most fre- quently confounded, because some of the symptoms appear so nearly* alike in both, and because they both frequently exist in the same patient at the same time, is hysteria. Neurasthenics sometimes develop hys- teria, and hysterics are nearly always more or less neurasthenic. Never- theless, when both affections are present at the same time it is possible often to clearly determine which of the symptoms are due to the neu- rasthenia and which to the hysteria. The etiology of the two diseases has so much in common, as for example heredity, environment and education, it is not to be wondered at that a certain resemiblance should obtain between the two sets of symptoms. On the other hand, the differences between their patho- genetic bases — namely, the purely psychic nature of true hysteria and the purely physiological and histological nature of neurasthenia — ex- plain and emphasize most clearly the differences in the symptoms — even the same symptoms — of the two diseases. As Wundt, the great exponent of modern physiologic:.! psychol- ogy, has shown, mental phenomena are compounds representing inter- connections between certain elementary sensations and feelings. Con- cepts, judgments, memory, imagination and volition are not actual entities with a local habitat in the brain ; they are mere processes,, occurrences resulting from the interplay of the psychic elements, sen- sations and feelings. The latter are the only psychic phenomena known to have a distinct, local, anatomico-physiological basis. This being so, it is obvious how the symptoms of a pure psychosis, as, for instance, hysteria, should be so changeable, variable and abrupt, and should represent more or less faithfully normal physiological activities, but in an exaggerated or diminished form rather than in an actual perversion. In hysteria, therefore, we observe an irritability with a heightened sug- THE NEURONIC DISEASES 1 73 ;gestibility and the individual symptoms assume the character of a psychoneural anaesthesia or hyperesthesia, paralysis or spasm, exalted imagination or absent volition. The striking peculiarity of these symp- toms is their psychoneural distribution, and above all their incom- parable changeableness and sudden variability. They are utterly de- void of anything like stability and permanency ; they are mere increases or diminutions along normal lines ; they are the results of an unin- Jierited or overinherited clash of two or more psychic forces represented in the elementary psychic sensations and feelings. They are the ex- pression of a true psychosis and can only be effectively reached and managed through psychic means. Neurasthenia, on the other hand, while necessarily revealing itself in part through the psychic sphere, is not a true psychosis. It is an organic disease, using the term organic in the sense that disturbance of function necessarily presupposes disturbances of the nervous elements. It does not matter whether our present means of investigation fail to reveal to us the nature of the molecular, chemical or other dis- turbance, whatever it may be, in the neuroses or not. The fact remains that they are altered, else they would have functionated normally. The outward expression of this alteration of the elements in the neurasthenic state is that of an irritability with inability for sustained activity ; in other words, an irritable weakness. This organic basis of neurasthenia, about which we know so little positively as yet, lends a certain degree of permanency and persistency to the symptoms, a more or less definite anatomico-physiological dis- tribution to them, and a tendency towards positive aberrations and perversions in them, rather than mere exaltations and diminutions of normal psychic and sensory-motor activities. One or two brief illus- trations will make this clear. Headache is a symptom of both hysteria and neurasthenia. In hysteria it is a distinct pain, often in the vertex, when it is known as clavus. The pain sense is one of the normal psychic elementary senses. In hysteria this normal elementary sense is psychically exaggerated. In neurasthenia the headache assumes more of the character of a paresthesia. It is described as a pressure, a constriction, a pulling, a vise-like cap, but never as a distinct pain. Many times I have had neurasthenics stop me when I have used the word headache, in referring to their head trouble, saying that they had had headaches, but felt sure that this feeling was not like their distinct attacks of headache. Again, anesthesia of psychic origin is pel haps the typical symptom of hysteria ; no such anesthesia ever occurs in even the most profound state of nervous exhaustion. The rare form of numbness sometimes complained of by neurasthenics, if examined objectively, is seen to be not an anaesthesia at all, but rather a subjective form of paresthesia. And so on through the whole list of symptoms common to both ■diseases. A careful consideration of them from the two standpoints just described will enable one to determine whether they are hysterical or neurasthenic in origin, even when they appear in a patient afflicted with both diseases at the same time. Hypochondriasis, a mental malady in which the notion n\ dlSi 174 THE NEURONIC DISEASES is persistently and definitely localized by the patient, often resembles neurasthenia with anxiety and fixed ideas. The hypochondriac insists that he is never going to recover, but, on the other hand, is steadily growing worse, while objectively he is seen to be steadily improving. The neurasthenic, when asked, always feels that he is going to get well if he is only fortunate enough to get the right treatment. Hypochondriacs brood over their supposed trouble, but they are not wearily depressed or typically melancholic as neurasthenics are. Moreover, they do not reveal the weakness, the bodily emaciation, the inability to take prolonged exercise and the other sensory, motor and vasomotor symptoms of the latter. In a word, hypochondriasis is a pure psychosis of a restricted sort ; the somatic and anatomico-physio- logical functions are but little disturbed. The form of insanity commonly known as melancholia is some- times hard to differentiate from the melancholy of neurasthenia. In the former there is a strikingly persistent insomnia, a severe bodily emaciation, great restlessness, a high degree of causeless emotionalism,, hallucinations and delusions, and a tendency toward suicide. Melancholia is a pure psychosis with secondary physical mani- festations ; it is subject to abrupt changes in its symptomatology, es- pecially outbursts of mania. The face often shows an insane expres- sion and the acts of the patient, if closely watched, reveal a mental derangement. In melancholia the psychic predominance of the clinical picture is emphasized ; in neurasthenia the psychic depression is merely a part of a symptomatology that includes an irritability and weakness of the entire nervous apparatus, motor, sensory, vasomotor and sympathetic. And yet it must be frankly admitted that there are cases of melancholia and neurasthenia that lie on the borderland between the two diseases and in part actually overlap. These are the cases that give the real trouble in diagnosis. It should not be forgotten that a melancholia patient may at the same time be the victim of a neurasthenia and vice versa. Time and close observation alone can solve the true nature of some of these cases. Many gross organic diseases depress the patient into a state of neurasthenia, especially in their incipient stages. Among such diseases may be mentioned syphilis of the central nervous system, multiple cerebrospinal sclerosis, the parasyphilitic diseases, locomotor ataxia and dementia paralytica, cerebral tumor or abscess, and certain forms of toxaemia involving the nervous apparatus and resulting in gross lesions later on. The differential diagnosis of these will be discussed under the head of the respective diseases. Myasthenia gravis occasionally suggests neurasthenia. The weak- ness is here, however, more decidedly neural. It verges more upon a distinct paralysis. The bulbar prominence of the manifestations, their remarkable remissions, and the absence of sensory and psychic mani- festations are to be noted. Neurasthenic symptoms may be provoked in a reflex way In gross lesions and disease outside of the nervous system. The constant THE NEURONIC DISEASES 175 and severe pain of cancer, for instance ; the ceaseless distress caused by an inflamed uterus or bladder ; the anxiety and worry incident to a knowledge of organic heart disease may so strain the patient's nervous inhibition and self-control as to wear it out functionally and thus set up a condition of neurasthenia. The value of this observation lies in the direction of urging the examiner to spare no pains in searching for all such possible extraneural sources of the neurosis. The mere removal of this extraneural trouble may not cause the neurasthenia to immediately vanish, for the latter has grown into a distinct entity and needs attention solely on its own account. Neurasthenia is a slow on-coming disease. Even those cases which seem to develop suddenly or rapidly after some violent mental shock or physical traumatism do not at first exhibit the typical neurasthenia that they do later on. Sometimes the disease steadily progresses for a number of months or years and after reaching a degree of moderate severity remains sta- tionary. In all this long period there may be remissions and exacerba- tions of the trouble, due to various influences acting upon the patient, but there is scarcely ever any absolute return to health unless there is a more or less complete change of habits and environment. Exceptionally there are instances of neurasthenia gravis which run a very rapid course, and in spite of everything sooner or later die of inanition and complete exhaustion. Prognosis. — The prognosis of neurasthenia is generally favorable. It is never a fatal disease per se, though, as Oppenheim, Thoma, Regis, Frsenkel and others point out, it may through continual fear and emo- tion set up incessant acceleration of the heart's action until an organic disorder may result or favor the early development of atheroma of the heart and blood vessels, which ultimately may be the cause of death. When a neurasthenic worries himself into committing suicide the mental condition is probably the expression of a pure psychosis. Every case of uncomplicated neurasthenia is amenable to treat- ment, and under proper conditions most cases get well. This is par- ticularly true of the acute acquired cases. It is even true of the chronic cases. The congenital cases, of course, are only partially amenable to treatment. Medicine cannot recreate an inherited neuropathic con- stitution, but it can do a great deal in preventing and overcoming the acute outbreak of the disease in such constitutions. The character of the heredity, the patient's station in life and means for accepting what may prove to be an expensive method of cure, and the absence of all signs of other mental and physical disease obviously determine the prognosis. Given certain conditions, some of the most brilliant results in neurology are obtained in the cure of neu- rasthenia. Complete recovery from the most distressing condition- often obtained and gratefully appreciated. This is shown by the fact that such patients, knowing their neuropathic tendency, guard them- selves, when once they have recovered, and often live to a long and happy old age. I76 THE NEURONIC DISEASES Treatment. The successful treatment of neurasthenia depends upon several conditions. In the first place there must be a clear comprehension of the na- ture of the disease and of its underlying physical basis. In other words a clear diagnosis must be made, not merely of the patient's condition in general, but of the individual symptoms as well. For example a psychic symptom may be due to nerve-cell malnutrition (neurasthenic) or to disturbed psychic processes (hysteric). To attempt to combat it always in the same way will undoubtedly lead to frequent failure. The paraesthesiae of neurasthenia whether cerebral or neural in origin are quite different from the psychic anaesthesias and hyperesthesias of hysteria. In neurasthenia there is an anatomico-physiological derangement either within or without the nervous system dependent upon malnu- trition, toxaemia, exhaustion or reflex irritation. In the treatment of neurasthenia, therefore, the center of attack must be made against one or more of these factors. Psychic treatment is of no avail except in those cases in which there are hysterical tendencies. In the second place, the successful management of neurasthenia demands the absolute -confidence and ready acquiescence of the patient, on the one hand, and the highest degree of tactfulness, authority and wisdom of the physician upon the other. Failure to obtain a cure not infrequently results from the non-fulfillment of one or both of these conditions. From a want of confidence and obstinacy the patient fol- lows directions in a half-hearted manner, or delights in combating everything that is done or suggested, or listens too readily to all sorts of counsel from neighbors and friends and religious cranks. The result is obvious. The physician sometimes offends from want of tact, or he attributes to hysteria, a pure psychosis, symptoms that are neurasthenic and anatomico-pathological in origin and adopts a manner and mode of treatment that quite insults the patient, such as questioning the veracity of her statements; or he is less intellectual, less well informed and less cultured than his patient. All of which foretells the inevitable result. The third and too often the most difficult condition to secure for the successful treatment of neurasthenia is one that involves the ques- tion of the patient's finances, time and general ability to carry out an expensive and prolonged course of treatment. If the same unlimited freedom were given the physician treating a case of severe neurasthenia that is necessarily given to one who is managing a case of typhoid fever or pneumonia, the results would not only be better for neurasthenics, but the art and science of medicine would shine more brilliantly in the management and cure of the neurosis than it now does in the manage- ment and cure of the infection. The latter diseases are not cured by medicine, but inevitably run their course. The physician merely guards them from certain possible complicating conditions. In neurasthenia the disease in toto is made to right-about face and THE NEURONIC DISEASES I J J start upon an upward grade the moment the requisite environment and regimen are obtained. The failure of medicine to make a better showing in the treatment of neurasthenia is largely due to the fact that the patient's financial condition, or his inability to leave his business, obliges us to temporize and substitute therapeutic measures that are not absolutely the best. In all candor and as a matter of personal comfort and professional repu- tation, when the charge of such a case is undertaken, the practitioner had better tell the truth about the temporizing character of the treat- ment. There is no routine treatment with established formulae for neu- rasthenia. Each case must be managed in accordance with its own individual peculiarities. Rest and nutrition, both terms being used in the broadest sense, are the two words that cover the whole line of treatment for pure neurasthenia. Every amusement and exercise, every prescription, must be suggested with only those two ideas in view if the case is one of pure exhaustion. Xot many cases are purely neurasthenic. Certain hysterical mani- festations of a mild character usually develop along with the neuras- thenic. Therefore many of the therapeutic measures adopted for the purpose of procuring more rest and better nutrition of the nervous elements may in part be made to subserve the purpose of combating the psychic or hysteroid manifestations. In this way we sometimes hit two birds with one stone by changing the patient's environment, by pre- scribing particular forms of electricity, baths, gymnastics, outdoor games, travel, reading and other occupations. Even the medicines and dietary ordered may be made to apply to all three demands, rest, nutri- tion and psychic change, when all three are combined in the same patient. The mode and means of combating the third demand will be found discussed under the head of hysteria. Here the discussion will be limited to the treatment of pure neurasthenia. A little ingenuity will enable the practitioner to combine, when necessary, the two lines of treatment. The first great requisite is rest. Now rest is a broad term and is not synonymous with idleness. Ofttimes rest means change of occupa- tion, and occasionally a change that may involve greater occupa- tion. The rest must be mental and physical. It is rest for a man en- grossed in the cares and worries of a great financial undertaking to arrange his affairs temporarily so that he can go out into the woods and exercise his mind and body close to the heart of nature. It is resl for a woman worn out with the physical exertion of caring for a Large household of children to lie for a time idle on a bed and have her tin- used faculties exercised with a bit of literature, art or music. Et is resl for one who has always lived upon lower mental and physical levels to climb up the heights of mental and physical mountains where the horizon is larger, the skies more expansive and the play of color greater. A small back room in a private hospital with a congenial nurse an<|a small but well-selected library will, with other accessories, afford 178 THE NEURONIC DISEASES the highest type of rest to one. To another a trip up the Nile, a jaunt through Europe, a climb up the peaks of Colorado, a hunting expedi- tion through Montana, canoeing in Canada, farming in Illinois or even golf, tennis and ball in the park may be genuine rest. By not carefully considering the patient's previous mode of life, his temperament, his mental and physical demands, mistakes have often been made when change of environment has been recommended. A European trip has only aggravated some cases of neurasthenia; the so-called secluded "rest-cure" in a hospital has brought still more misery upon others. Rest in the broad sense of change, mental and physical rest, is the sine qua non for beginning the treatment of this disease. When all things are taken into consideration, perhaps the largest number of neurasthenics can obtain the required rest in a private hos- pital or home. In this way the expense of travel, the distant separa- tion from family and medical adviser, and all the annoyances of de- parture and return are obviated. A change of the mental and physical atmosphere is thus secured with the least amount of discomfort. There- fore most cases of neurasthenia are benefited by a preliminary sojourn away from their usual environment in a rest cure sanitarium, home or suburban hospital. It is assumed, of course, that the management, location and fitting of these places are all that they should be, otherwise they are to be shunned absolutely. To Weir Mitchell a debt of gratitude is owing for the thorough manner in which he has studied and outlined the rest cure. Its main features consist of more or less seclusion, physical and mental rest and forced nutrition. Only a small percentage of cases of neurasthenia, and those the more severe ones, need the absolute rest cure carried out in all of its details. The general purpose and principles of the treatment being kept well in mind, it may be varied by the attendant so as to be adapted to the particular needs of the case in hand. The details of the treatment may be briefly discussed under the heads of hydrotherapy, massage, electricity, diet and medicines. In a general way my own method of procedure with a moderately severe case of genuine neurasthenia is about as follows : After get- ting the patient away from her usual environment into a private hos- pital or home, or, if circumstances will not permit otherwise, into the home of some friend of the patient, where there is a sunny, well- ventilated room fitted up with furniture, pictures, books, flowers, etc., that suggest quiet and cheerfulness, I limit, sometimes prohibit, the visitation of friends and relatives. A willing and refined nurse is selected and requested to continue in constant attendance so long as she proves congenial to the patient. A few simple games and a list of a dozen or more books selected from the essayists, historians, travelers, poets, philosophers, novelists and humorists to meet the patient's particular mental requirements are recommended. For the first week or so very little is done in the way of active treatment. The patient is requested to remain on the bed or couch THE NEURONIC DISEASES 1 79 most of the time, to receive a few friends only, to pass the time chat- ting and getting acquainted with the nurse and otherwise becoming familiar with the new surroundings. During this first week the bowels are freely moved daily with calomel or enemata ; the kidneys are slightly stimulated with diuretin ; and the diet is quite closely limited to milk and broths given in small quantity every hour or two. Sleep is enforced by nightly doses of trional or sulphonal. A certain amount of psychic suggestion is made upon each visit to the patient, which visits are daily or every other day at first. This is to inspire confidence, to increase the docility and hopefulness. At the end of the first week or ten days of this preliminary treat- ment the patient usually recognizes that her physician can dominate the disease, for she finds she has slept and that though she is apparently more collapsed and ready to take to her bed, she is slightly less irritable and melancholy. Now the patient is ordered to keep her bed constantly. The calo- mel and diuretin are stopped and only the morning enemata continued ; the trional and sulphonal are gradually diminished each night; and a slightly more generous dietary is allowed. By this time the particu- lar requirements of the case are so well revealed that a daily program is carefully written out and left with the nurse to execute. The follow- ing program suggested by Dr. John K. Mitchell may be taken as an illustration : 7 a. m. Cocoa. Cool sponge bath with rough rub and toilet for the day. 8 a. m. Milk. Breakfast. Rest an hour after. 10 a. m. 8 oz. peptonized milk. 11 a. m. Massage. 12 m. Milk or soup. Reading aloud by the nurse. 1 130 p. m. Dinner. Rest an hour. 3 130 p. m. 8 oz. peptonized milk. 4 p. m. Electricity. 6 p. m. Supper with milk. 8 p. m. Reading aloud by nurse one-half hour. 9 p. m. Light rubbing by nurse with drip sheet. 8 oz. malt extract with meals, tonic after meals. 8 oz. peptonized milk with biscuit at bedtime and a glass of milk during the night if desired. Such a program as the above should never be adopted as a routine for all cases. Its chief value is in giving the approximate time fur each of the manoeuvres and in forming a skeleton ground plan whereon special indications maybe made to meet special requirements. Not the least advantage in the use of such a program is the regularity and system which it establishes. In regard to the dietary suggestions for such a program, it should be well understood that a forced nutrition is the desideratum and that l8o THE NEURONIC DISEASES therefore the only limitations surrounding it are the digestibility and nutritious character of the food and the digestive powers of the patient. -Sometimes I find it necessary to study closely the patient's idiosyncrasies in regard to food ; at other times I urge the use of all kinds of food, in- discriminately, that the patient will enjoy most, assisting its digestion, when necessary, with the artificial digestants. I get the food into the patient, sometimes with the regular meals, sometimes with small, fre- quently repeated meals, knowing that in spite of some temporary dis- tress, with digestants some assimilation of it will be accomplished. In the early dietary milk plays almost the entire role. Even throughout the whole six weeks' or two months' course of treatment milk is made a staple article. The quantity (two to four quarts a day), mode of preparation, frequency of administration and method of rendering it continuously palatable are subject to variation. The nitrogenous foods are usually considered the best for neuras- thenics. Meats, fish, eggs, green vegetables, fruits, with occasionally a little tea and coffee, and at all times abundance of water, may be taken. It is well for this class of patients to avoid such viands as pork, stews, hashes, veal and salt meats in general, thin soups, cooked oysters and clams, turkey, thick gravies, pickles, pies, pastry and preserves and all made dishes. Howbeit as nervous starvation is so largely at the base of nervous exhaustion, I would in some cases even allow some of these undesirable articles of food if the patient's idiosyncrasy called for them to the exclusion of the more digestible ones. The need of nutrition is so great that for the time being a certain amount of gastric distress may be risked if thereby more food stuff is accepted by the patient. Stomachic tonics, acids and digestants can do much towards aiding the stomach under these circumstances. Hydrotherapy is a most valuable factor in the treatment of neu- rasthenia. It should be employed for its tonic rather than its stimulat- ing or its psychic effect. Therefore I prefer warm or tepid baths daily, either upon rising in the morning or just before the massage in the forenoon. The tepid sponge bath in bed every morning, or the drip in the evening, may be employed in the early period of the treatment. In exceptional cases and sometimes late in the treatment when stimulation is indicated I use the cold bath or the cold wet pack. 1 am convinced, however, that the vast majority of the cases of neurasthenia are better off without such vigorous stimulation, at least until the)' are well along in convalescence. Whether the water is applied cold or hot, in sprays, douches or packs, in full baths, half baths or sitz baths, for a long or short time, are details that must be decided by the physician in individual cases. Massage, especially for women, is a most grateful form of passive exercise. It should not be too vigorous at first, though it may be administered daily. Gentle rubbing of the entire body towards the heart, sometimes with the use of olive or cocoanut oil, is usually all that is necessary. Occasionally pinching, stroking and slapping are beneficial. Later on active and passive gymnastics, Swedish movements THE NEURONIC DISEASES l8l and other forms of medico-mechanical treatment should be systemati- cally employed. About this time short walks with the nurse, or rides in the open air, may be indulged in, always under the physician's in- structions, however. Electrotherapy is sometimes abused in the treatment of neuras- thenia. It should never be begun too early, say before the second or third week in the hospital. It should be used at first rather tentatively, and had better be dispensed with entirely if it tires or irritates. Above all it should never be used in pure neurasthenia under the notion that its good effects are psychic upon the patient. Central galvanization of the spine with general faradization of the whole body, the former for five minutes and the latter for three-quarters of an hour, in some cases produces a marvelously restful feeling and provokes the fine fibrillary contraction of the muscles. I never saw any benefit from the use of static electricity in pure neurasthenia. High frequency currents, it is said, are being used with a certain degree of success. The same is claimed for the light-cure. Psychotherapy from the very nature of things has some influence in the management of neurasthenia, though that influence is infinitely less than it is in hysteria. It consists chiefly in the confident and assur- ing manner of those who are in attendance upon the patient. Medicines are valuable only as adjuvants to the other lines of treat- ment. They are chiefly tonics, stomachics, chalybeates, carthartics and hypnotics. Before speaking of them more in detail I want to protest against the too frequent use of stimulants, such as strychnia, alcohol, etc., in the early treatment of this disease. To whip up an exhausted and badly nourished nervous system is not only illogical, but positively harmful. Xot only in patented nervines, but, alas ! too often in the prescriptions of intelligent physicians do we find these stimulants when neurasthenia is the disease in hand. Among the tonics available are small doses of mix vomica, the mineral acids, quinine, arsenic, phosphorus and iron. Any preparation of these may be employed that is most easily assimilable and that upon trial seems to fit the case best. Among the cathartics the salines and cascara are the best for continuous use. I always gradually reduce the laxatives and depend for a while longer on gradually diminished ene- mata. The bromides, sulphonal and trional are the hypnotics of choice, though these should only be used early in the treatment and should soon be replaced by other means of inviting sleep. I deem it unwise to make specific suggestions for the treatment of individual symptoms. Besides emphasizing the one-sided conception of his trouble already held by the patient, it is apt to cause the attend- ants to lose sight of the fact that neurasthenia is a disease of the entire nervous apparatus and therefore always needs treatment along general lines. This statement applies especially to the treatment of the special organs at fault in what is called sexual neurasthenia. After the patient leaves the hospital or home it is well for him to still keep under the observation of his physician for several weeks longer, and above all to avoid returning into the same environment from 1 82 Tl L1C NEURONIC DISEASES which he emerged at the beginning of the treatment. His exercise, diet and tonics still need some oversight. Moreover the medical adviser can even at this period, when the patient is starting a new sort of a life, as it were, offer suggestions, propose restraints and lend encourage- ment that may prevent a much-dreaded relapse. During the long course of hospital treatment a bond of friendship, closer than a mere profes- sional association, has not infrequently sprung up that reveals itself when the patient has returned to his normal course of life in a mutual confidence which, if properly exercised, will keep the patient from ever falling into his old state of distressing invalidism. THE TRAUMATIC NEUROSES. A few years back the discussion about these troubles was loud and heated. To-day it has retired almost wholly into the courtroom where it is unfortunately mixed up with damage suits. From the very nature of the case it will probably continue to be heard there. By a traumatic neurosis is meant the more or less sudden appear- ance of abnormal manifestations or symptoms of disease in one who has recently passed through a severe accident, railway or other sort, and who shows nowhere upon his person any outward sign of physical injury. It is evident that a condition such as this definition would cover opens the way for simulation, fraud and malingering. The heat of the debate in the past was undoubtedly fanned to an exalted degree by the differences of opinion as to whether all such conditions outlined in the definition were not simulations. In most damage suits it is obviously the interest of the plaintiff to establish beyond question the actual reality of his trouble ; whereas it is the interest of the defendant to belittle, to prove the unreality of, the disease, and even to establish the simulation of it. The first ques- tion that always presents itself in regard to these manifestations, there- fore, is whether there is any abnormality present or whether the com- plainant is not a malingerer. This, of course, involves detective work of all sorts, medical as well as lay. As cases of trouble following accidents of all kinds continued to appear, some in search of damages and some not, it became evident that there was actual disease of some sort in them and that the ques- tion of malingering should be set aside as an entirely distinct and separate one. Can there be a disease from shock or other accident to the nervous system and yet no change be found in the tissues of the body? At last the question was narrowed down to something that scien- tifically was worth while answering. It was a question, however, in which it seemed there could be honest differences of opinion between equally learned and scientific investigators. In other words, it was the old, old problem looming up again, but under another guise, as to whether disturbance of function can occur without change of structure. There were those who answered "No" to this, but there were also those who answered "Yes." The reality of the symptoms was granted. THE NEURONIC DISEASES 183 Those who held that disease always meant some discoverable change in the tissues at once started to search for such changes in these accident cases. In some of them were found effusions, small hem- orrhagic foci, minute degenerative areas, meningeal alterations, vascu- lar obstructions, etc. On the strength of these few findings and upon the character of some of the symptoms, a pathology was erected. The influence of the distinguished surgeon, Erichsen, was so great that he led the medical world into adopting the view that the trouble was a spinal concussion with early molecular changes and secondary inflammatory retrogressive processes in the cord. He, Riegler and others regarded the condition as essentially organic, as a chronic meningomyelitis. Page then took up the subject and in a volume argued against Erichsen that no such damage to the cord could possi- bly be produced by railway accidents. Thus the ball was set a-rolling again and as investigators lined up on one side or the other we were edified with a vigorous scientific de- bate on the question of the causation of organic and non-organic dis- eases. But still the cases appeared in which the symptoms were unde- niable, but in which the usual signs of organic disease of the cord were wanting or were woefully confused. The pros and cons having all now been heard, it is to-day prac- tically conceded by the authorities of most reliability that the acci- dents which are supposed to cause these diseases can and do cause them, but that they are not by any means new diseases. The troubles which we observe after a severe shock, physical and mental, are of the nature of psychoneuroses, though they may have, in the beginning or later on, associated with them vascular, meningeal, inflammatory, sclerotic and other changes of the brain, spinal cord, nerves and sym- pathetic system., It is at last recognized that the traumatic nervous troubles are of many types. There are, first, the gross surgical cases with their fractures, luxations, hemorrhages and compressions of the spinal cord. Then there are the true neuroses or psychoneuroses. These arc- classified in modern neurology as neurasthenic, hysterical and hypo- chondriacal cases. Associated with and sometimes following later on these neurotic and psychoneurotic states, there are the hemorrhagic, inflammatory and degenerative troubles, or their combinations, in the nervous centers. The symptomatology of all these is well known, and as I haw described them in detail elsewhere they will not be repeated here. A traumatic hysteria is the same as any hysteria. A traumatic com- pression of the cord needs no further description than what has been given it under the head of spinal diseases. Were these traumatic cases never to enter court or be mixed up with damage suits, their discus- sion as a separate and peculiar set of troubles would disappear from the text-books. In court, however, there is still much debate as to what is meant by a neurosis or a psychoneurosis. Why? Because the law as il framed does not concern itself with hypotheses, however thev ma\ seem 184 THE NEURONIC DISEASES' to be well founded ; and those who administer the law are not willing or well enough informed in medicine to fully comprehend what is meant by a functional disease. The law and the lawyer demand what are commonly called physical proofs, evidences that can be actually per- ceived by our senses in some way. They do not care, when in court at least, if they are told that a large amount of scientific knowledge, even the ultimate foundation of all human knowledge, consists of inference drawn by a logical deduction from given data. How often the witness in a criminal trial is reminded to tell only what he actual-ly saw, or what he can swear that he perceived in some way through his five senses ! The court does not want to know what he thinks or infers. It cares nothing for his forms of reasoning or the logic of his argument. It wants from him merely cold, bare facts which it can itself behold or could have perceived under the same circumstances. This, of course, is the only stand to adopt in legal matters where the conflicts of human judgment are brought to a maximum, but it is an absurd one to adopt as the ground for the determination of knowl- edge. Upon such a basis we could not swear that we knew anything positively. Distant planets would have to be brought into court, atoms and molecules would have to be magically enlarged, gravitation would have to be materialized and weighed, and the vibrations of sound would have to be caught and held up for the jury's personal inspection. Facts as the law defines them are not the same as facts constantly recognized in the scientific world. Among these facts which -science accepts but the law does not is the one that we can have a functional disturbance in a nervous cell and yet not be able to discern it directly by means of any of our five senses. Logic can penetrate where the latter fail. It is just as legiti- mate to reason from effect back to cause in medicine as it is in astron- omy or biology. It is just as legitimate to accept the existence of inherent changes in cellular structures of the nervous apparatus when the manifestations are constant and uniform, as it is to infer the ex- istence of an unseen world when certain constant and uniform perturba- tions are observed in the heavens. In medicine we have long since advanced to the point where we recognize a neurosis or psychoneurosis as an entity, a disease, an abnor- mality, due inferentially but logically, to some molecular, chemical or nutritional change in the minute nervous elements. At the present day it is not incumbent upon the victim of the disease to defend that proposition. If the lawyer of the defendant wishes to deny it, he has the whole scientific world of medicine and of logic to combat, and to permit him to waste the time of the court in such a stupendous task is sheer folly. At the present day to discuss and deny the reality of the neuroses because forsooth we can't minutely define their pathological basis is as ludicrous as it would be to discuss and deny sonic forms of insanity in which no known pathological changes have yet been observed. In a word, then, these court trials should, if properly conducted, narrow themselves down to the question of the existence of or simula- tion of the neurosis; and in the event that the former is proved to be THE NEURONIC DISEASES 1 85 the case, they should have regard to the relation of the disease to the accident as one of cause and effect, and they should determine the severity of it as regulating the amount of damages to be awarded. In fine, the medico-legal consideration of the traumatic neuroses relates at the present day only to the establishment of their presence, their etiology and their prognosis. Etiology. — One of the questions always brought up in court, in fact the question upon which the awarding of damages actually de- pends, concerns the general and the particular causes of the neurosis. Can a neurosis be produced by shock in an individual previously well in every respect, or has the plaintiff's condition been caused solely by the admitted accident? Another question that also arises is, after granting that the acci- dent has served merely to awaken a latent diseased state in the plaintiff, how much damages should be awarded for a disease simply aroused into activity, in comparison with one actually originated? It is admitted by all neurologists at the present day that shock, both physical and psychic, can set up an abnormal state in an individual sufficient to be termed disease. Lightning strokes, railway collisions, concussions can and do produce symptoms which sometimes betray an actual physical basis and sometimes do not. In the latter case they constitute almost universally the clinical pictures of hysteria and hystero-neurasthenia of variable degree. YYe call these diseases or symptom-complexes functional for want of a better term, but we assume that they are dependent upon molecu- lar alterations, involving change of metabolism. Severe fright and excitement, mental and physical shocks of all kinds have been followed by these troubles when there could have been no possible thought of damages. It is perfectly reasonable, there- fore, that they might occur when a damage suit is possible, whether it be probable or not. It can no longer be doubted that a profound psychoneurosis, hys- terical or neurasthenic, with all that we include in those disease states, may be caused de novo by a severe accident in which there has been a severe mental and physical shock. Nevertheless it is true that in the majority of cases there has not been a state of ideal health before the accident. A shock that would produce a pronounced psychoneurosis in a previously healthy indi- vidual would be not unlikely to produce a severe physical lesion of some sort. Not infrequently such is the case, though it is not often rec nized until some weeks after the accident. Embolic and hemorrhagic, inflammatory and sclerotic conditions of insidious character are thus often initiated and bring in their train a long series of grave mani- festations. These are the organic ca.-es, and as a rule are the type that occur in patients who are caught by the accident when they are in perfect physical and mental vigor. Very often, however, the victim of the shock lias been the pos- sessor of a highly neuropathic constitution. He may even have been all his life, or recently, a hysteric or neurasthenic. Hi- nervous ap- l86 THE NEURONIC DISEASES paratus may have been already on the verge of collapse with alcoholic or nicotine poisoning. Meeting with an accident under such circum- stances, an unusual psychoneurotic state is suddenly evolved in him and he is profoundly prostrated, violently hysterical or pitifully hypo- chondriacal, though he may show no physical sign of injury and may even not have been as badly shaken up as some others in the accident. The latter is, therefore, a definite exciting cause of his trouble, though not the primary originator of it. The results may be lasting and later on even evidences of physical degenerative conditions may make their appearance. The relative amount of damages for an accident that originates and for one that merely awakens a latent disease state must be decided by the legal authorities. Symptoms. — There is nothing to be said of the symptoms of these neuroses more than what has already been said under the heads of hysteria and neurasthenia. The detection of malingering depends almost wholly upon symp- tomatology. Familiarity with the diseases that are simulated and nat- ural shrewdness alone will enable the examiner to detect a fraud. To lay down rules of procedure would be absurd. Each case must be studied by itself. The whole clinical picture must be taken into view as well as the various manifestations in detail. Most simulators are dishonest and fraudulent. A hysteric may be an incorrigible simulator and yet not be dishonest or fraudulent. The examiner must be careful to differentiate the two. One of his most difficult tasks, if the case goes into court, will be to convince the jury that there is a difference between the hysterical and the fraudulent simulator. It is a fact that the laity still look upon hysteria, with its high degree of susceptibility to suggestion, as a sort of a make-believe on the part of the patient, that gives strength to the defendant's side in the courtroom. If a case is hysterical I urge against all litigation. A prompt settlement, though small, will be better than to run the risk of trying to explain, in the presence of an opposing lawyer, to the average panel of layman, what is meant by hysteria and to prove to them that it is an actual disease as much as typhoid fever or pneu- monia. The task is herculean and even though heavy damages be really deserved, the chances are strong that the verdict will be against the plaintiff. A very good principle to remember in detecting malingering is that it is safer for the malingerer to imitate an organic disease than to imitate a neurosis, but that it is easier to diagnose a simulated neurosis than to diagnose a simulated organic trouble. The reason of this is obvious. The neuroses are so much more changeable in their symp- toms and have so many more of them than the organic diseases, that the chances of inattention on the part of the malingerer are better in the former than in the latter. A local paralysis or anaesthesia such as we observe in an organic disease can be imitated by a shrewd criminal. His attention being confined to so limited a phenomenon, he cannot be thrown off his guard by the tests of the examiner. On the other hand, scarcely a simulator is so well posted on disease as to be able to present THE NEURONIC DISEASES 1 8/ a complete and typical picture of a neurosis. He either overacts or underacts his part. One of the shrewdest simulations that I was ever asked to unmask was that of a woman who for years had been representing herself as an epileptic. She had clearly studied the disease well from the books. Several physicians who had seen her in her fits had pronounced her -epileptic. The family history was neuropathic and there had been a trauma. In all details she acted her part to perfection. When, how-* -ever, the entire history and total picture of the case were brought into the light of scrutiny it was obvious enough that the case was not one of typical epilepsy. It was not hysterical, nor was it organic. I pro- nounced it one of simulation and the later results proved the correct- ness of my suspicion. Another case that I saw was one of genuine hysteria in which imitation had played such a strong role that in the absence of certain stigmata of hysteria some of my colleagues thought it was one of pure simulation. The total picture of the case formed the basis of my ■opinion. I recently saw in consultation a traumatic hysteria in a man who could not move his left arm, could not hear with his left ear and was afflicted with complete aphonia. A stroke of lightning had hurled him across the room and he was examined about a week later. No organic lesion would have given the peculiar complexus of symptoms that he presented. Simulation was excluded by the rapid action of the pupils to light, with sluggish action to accommodation, and by the entire early and late history of the case. Simulators who attempt to imitate the neuroses do not render the picture changeable enough. They adopt a type of the disease and then give its manifestations such a permanency and a uniformity as belongs only to organic disease. Of course, electricity, a psychic shock, etherization and the state of the pupils will serve when in doubt as invaluable aids in differen- tiating organic from functional disease and distinguishing either from pure malingering. Prognosis. — the prognosis of the traumatic neuroses is always an important question in court. Much is said on the side of the defense about the rapid recoveries that occur after the payment of the damages. The insinuation, of course, is that deception has been practiced. Thai is undoubtedly the case sometimes. However, there is something to be said in favor even of those who thus recover after damages and yet are not simulators. It is a fact that the recovering of a large award and the satisfaction of having won one's case is a powerful stimulant. A neurosis is a functional depression, a continuous autosuggestion in some cases. Let such a case be given an experience that would make almost any human being glad, honestly glad, and it is nol surprising that sometimes it takes a turn and recovers. Psychotherapy is a potent agent when administered by a court as much as when administered by a physician in the hospital. As a matter of fact, however, there are numerous cases "I trau- matic neurosis that do not recover immediately or at any time after l88 THE NEURONIC DISEASES the awarding of suitable damages. Indeed, some of them even get worse and not a few develop in the course of a year or so distinct indi- cations of serious organic trouble. If the outlook of every case could be foretold, the amount of damages to be awarded could be nicely- adjusted. Unfortunately there are no safe guides in this respect. The traumatic neuroses have a prognosis that is a little worse than other forms of neuroses. If there has been a latent neuropathic state prior to the accident, the prognosis is rendered very grave. It is to be remembered that tabes, dementia paralytica, disseminated sclerosis, or spastic paraplegia may have been latent or just beginning to show themselves with mild neurasthenic symptoms about the time of the accident and that they may be suddenly started into a full and rapid progress by the nervous shock of the accident. . Aside from such facts as these the prognosis of the traumatic trouble follows that of the disease, however it may have originated. Treatment. — For treatment, see the sections on hysteria and neu- rasthenia. EPILEPSY. There are some twenty or more synonyms for this disease, so long has it been known and so varied have been the explanations of it. Its protean manifestations have caused students, from ancient times down to the present, to regard it all the way from a divine possession to a mere gross anatomical defect. The numerous names of the disease in a general way either describe some particular feature in its symp- tomatology or suggest an imaginary pathology for it. Falling sickness,, fits, apoplexia parva and morbus sacer are among the more striking synonyms. Epilepsy, from a Greek root meaning to seize upon, is now the recognized title of the disease everywhere. It. is easy to say that epilepsy is a condition in which loss of con- sciousness with convulsive movements constitutes the principal phe- nomenon. Such a definition, however, is inadequate to cover all of the various types, many of the aberrant forms and the general mental state, while it gives no clue whatever to the underlying pathological anatomy. In fact, it is not probable that any short definition of so pro- found and varied a malady will ever prove entirely satisfactory. This is especially important to remember when the physician is placed upon the witness stand in court and pressed to give in a few words a ae- scription of the disease. Preceding his testimony with this explanation, he may, upon the urgent request of the court, attempt a definition somewhat in this wise, remembering all the while that the two cardinal features of the clinical ensemble are the sudden loss of consciousness and the impairment or loss of motor coordination : Epilepsy is a dis- ease of the brain in which paroxysmally and somewhat periodically. abruptly and for a brief period of time, the consciousness is wholly or partially lost and the muscles are or are not thrown into a condition of incoordination with the tonic and clonic convulsive movements. There are four well-recognized types of the disease based largely upon their symptomatic differences. They are the grand mal, the petit null, the partial (Jacksonian) type and the psychic type. THE NEURONIC DISEASES 189 There are certain neuroses that are believed by many to be minor manifestations or types of epileptic seizure, such as migraine, certain forms of angina pectoris, asthma, nocturnal pollution, gastralgia, enteralgia, etc. These will be again referred to under the head of epileptic equivalents. Status epilepticus is a culmination of the disease, but hardly a special type. The old division into primary or idiopathic and secondary, organic or symptomatic epilepsy is rapidly being obliterated. As our knowl- edge increases we are realizing more and more that an organic change of some sort in the cortical structures underlies the whole trouble. The reason for and the exact nature of this change are the problems still awaiting solution. Etiology. — The one great truth that stands out beyond all others in regard to the causation of epilepsy is that a hereditary taint, more or less pronounced, lies behind it. By this I do not mean to say that it is a hereditary disease directly, though it is often observed itself in the progenitors. Various influences of a neuropathic sort, such as hys- teria, migraine, gout, insanity, alcoholism, nervous strain and dissipa- tion, syphilis, etc., in the ancestors often reveal themselves in the off- spring in the production of epilepsy. In this broad sense, therefore, genuine epilepsy is an inherited disease. The predisposing factor in its etiology is an inherited, developmental defect in the nervous system whereby certain exciting prenatal, natal and postnatal accidents and influences cause the cortical neurones to functionate in this abnormal manner. A clear heredity can be traced in about one-third of all cases. In two-thirds of these the inheritance is direct and of like sort. In the remaining cases an indirect heredity is so constantly, though ofttimes remotely, discovered that one is almost justified in saying that the so-called exciting causes of the disease depend for their potency upon the existence of this predisposing element. Sometimes where the heredity cannot be elicited clearly from the ancestral history, collateral defects and stigmata of degeneracy in other children will point it cut. A woman whom I saw recently had never had any trouble until she was forty-seven years of age. The family history, so far as it was confessed to me, was entirely negative. A sis- ter of the patient, however, had cleft palate. Another patient claiming to come of a strong and sturdy German stock had had migraine of a most seyere type until her first fit, and volunteered the information that her sister, as well as herself, were the victims of paroxysmal asthmatic attacks. Injuries, depressing influences and other factors that ma) affecl the growing brain in its early years will cause congenita! defects that may express themselves in epileptic seizures. Freud goes so far as to say that all epilepsy is due to the developmental defect left in the brain by the ordinary lesions of the cerebral palsies of childhood. In these cases, of course, what hereditary influence there is must be of an indirect sort and probably vascular in character. The traumatic cerebral palsies which provoke brain atrophy, sclerosis and other defects out of which epilepsy is evolved cannot be spoken of as hereditary. 190 TPIE NEURONIC DISEASES The disease may declare itself at any age. Partly on account of the hereditary and congenital nature of it, three-fourths of the cases- begin to show symptoms under twenty years of age. 1 have often noticed that an epileptic equivalent, such as migraine, asthma or neu- ralgia, will suddenly cease and epileptic seizures take its place at about the age of physiological stress, as puberty and the climacteric. It is said that nearly fifty per cent of the cases occur in the second decade of life, when the awakening of the sexual function and the consequent emotional strain are overwhelmingly dominant. Partly as predisposing and partly as exciting causes infections and toxaemias are particularly significant. The acute infectious fevers of childhood, and above all scarlet fever, are responsible for the disease. As the cerebral palsies of childhood, from which so many cases of epilepsy are to be dated, are also largely due to the acute infectious diseases, it is difficult to say whether the epilepsy is the direct result of the infection or the secondary result of the brain defect. Pertussis, thermic fever, malaria, pneumonia, influenza, diphtheria, measles, typhus, typhoid and yellow fever and meningitis have all been credited with the disease. Gastro-intestinal disorders with auto-intoxication may well be the cause of epilepsy, in view of our modern nutritive conception of its pathogenesis. Toxaemia from lead poisoning, exces- sive use of tobacco, renal diseases, alcohol and such poisons as cocaine, antipyrin, arsenic, physostigmin, chloroform, etc., are to be remembered as possible instigators of epileptiform and probably epileptic fits. Acquired syphilis may cause epilepsy, though as a rule the seizures are indicative of gross lesions of a vascular nature so familiar in cerebral lues. Inherited syphilis is undoubtedly responsible for some cases. Other chronic infectious states that, like syphilis, predispose to the disease are rheumatism, scrofula, rickets, gout, severe anaemia, cancer, drunkenness of parents at the time of conception, diabetes and possibly chorea. Dentition in a predisposed child may provoke the disease. In- fantile eclampsia may be the beginning of it, a fact which should warn us not to make light of infantile convulsions about the period of teeth- ing, as is so often done. Traumatic epilepsy is usually focal, partial and Jacksonian in type. Nevertheless injuries to the head may provoke general epilepsy in various ways. Depressed bone, fracture of the inner table, hemorrhage, embolic obstruction, degenerative focal encephalitis or meningo-en- cephalitis may be the immediate cause of the trouble. Mental shock is another factor and has often been the starting point of the severest types of the disease. Mental strain, such as pro- longed anxiety, worry, grief and exertion in those whose heredity is unfavorable, are of the nature of mental traumatic causes. I place here the influence of masturbation. Usually this is an evidence, in early cases at least, of a neuropathic condition, rather than the cause of it. And yet it is clear that the shame, the fear of detection, the periodica} excitement and the popular notion that the practice invariably leads to insanity, is* of the nature of a mental rather than a physical cause. THE NEURONIC DISEASES 191 The mental power of the old Roman eunuchs shows that the loss of the vita sexnalis alone is not the cause of mental deterioration, whereas the worry and continued distress that such a condition may provoke may be. The horrible results liable to follow masturbation may be depicted to a susceptible lad in order to frighten him out of the dis- gusting practice, but they ought not to be set down in a scientific work to that which is not the real cause of them. Indeed it is questionable, in my opinion, whether we do well to play upon the mind of the patient in order to coerce him into a better state of morality. The remedy may be worse than the disease and by reason of the fright and dread set up, a serious mental shock, with all its evil consequences, may be put in place of a mere filthy habit. Masturbation, I can well understand, may as a secret and vicious practice well provoke in a neuropath epileptic seizures. It should be discouraged, therefore, as strenuously as possible, without, however, resorting to measures that still more tend to disturb and depress the mind. Psychic outbursts, ungovernable passion, intense anger and electric shocks have formed the starting point of the disease. Heart disease is so often found in company with epilepsy that a causal relationship is more than a suspicion. Mitral regurgitation was the chief cardiac symptom in the 1,070 cases examined by Spratling. Some form of heart trouble occurred in 238 of them. Reflex epilepsy is still a battle ground of argument and opinion. No one believes in the sole causation of the disease by peripheral irritation. There must be already present a strong predisposition. The unstable equilibrium of the nervous elements must be already in existence. That being so, it is both conceivable and dem- onstrable that irritating diseases of the internal organs, intestinal para- sites, elongated prepuce, laryngeal polyps, carious teeth, eye strain and foreign bodies in the ear may provoke the seizures. That is very differ- ent, however, from the statement ihat reflex irritations are the cause of epilepsy. Putting a stop to the fits is not always curing the epilepsy. The removal of these various sources of reflex irritation usually puts a stop merely for a time to the seizures. Too often the patient's early delight and belief in the cure are transformed into bitter disappoint- ment and hopeless indifference at finding that the fits are beginning to return again. These may be due to some new source of peripheral irritation, and had the exact value of these causes in the production of the disease been honestly presented to him, he would be willing to continue further treatment instead of basing his resisting obstinacy against all further attempts at relief upon the false hopes that were raised by the earlier treatment. Senile epilepsy has been attributed to an atheromatous condition of the blood vessels. It is just as liable to be due to the general senile decay and toxaemia from functional insufficiency of the various organs as from anything else. It is obvious that predisposing causes play the initial role in the etiology of epilepsy and that innumerable exciting causes may provoke the former into an outward expression. The great predisposing causes are heredity, infection and physical 192 THE NEURONIC DISEASES defects in the brain of a congenital or acquired nature. Cerebral in- stability is thus established and constitutes the essential disease. Its presence may be discovered and typical epileptic seizures may be pre- cipitated, however, only when one or more of a long list of possible exciting agents begin to bear upon it. Pathology and Pathogenesis. — It would be interesting but un- profitable to rehearse all the explanations that have been vouchsafed in the past in regard to the pathogenesis of epilepsy. Some of the scien- tific accounts of it were no less fanciful and no more founded upon actual pathological findings than were the superstitious and religious belief that it was clue to some divine or devilish form of possession. As in all the great neuroses the, number and variety of hypotheses have been in inverse proportion to the number and constancy of the post mortem findings. It is only within the last few years that we have approached anything like a scientific and well-founded explanation of the disease. Much still remains to be discovered, but what we already know indicates in the clearest manner along what lines future investi- gations should proceed. It would be well in discussing the modern explanation of epilepsy to always keep a sharp line of demarkation between the actual patho- logical findings and the mere surmises and logical deductions that are based upon those findings. The former may always be referred to with a certain degree of positiveness ; the latter must always be looked upon with a certain amount of reserve. After noting briefly the actual macroscopical and microscopical changes commonly found in epileptics who have died, as well as some functional abnormalities frequently observed during life, I will follow the recent excellent work of Prout and Clark and indicate the prob- able relationship of these various pathological conditions to the cardinal symptoms and the disease itself. In the gross pathology of the disease we find various more or less constant changes in and about the head and the brain. Some of these are of the nature of stigmata of degeneracy, some are the residua of old diseased conditions, others are the results of arrested development, and still others are the direct products of the epileptic seizures. In all of this the one fact that stands out with embarrassing prominence is that these changes, whether macroscopic or microscopic, cannot always be determined as the cause or the result of the disease. Almost every sort of lesion of the cerebrospinal axis, its mem- branes and their bony envelope, has been seen in epileptics. Deformi- ties of the skull, thickenings of the bones, anatomical changes in its minute structure are almost constantly observed. They may have something to do with the production of the disease, but it is more likely that they are the expression of a physical degeneracy of which the epilepsy is in part also an expression. The membranes are opaque, adherent, thickened in many cases, showing signs of an old inflamma- tion, hemorrhage and other vascular troubles. The brain itself not infrequent lv shows a partial or general atrophic change with alterations in its contour and convolutions. Like the skull, it is often undersized and deformed. On the other hand, it may appear perfectly normal. THE NEURONIC DISEASES 1 93 The most frequent findings in it are the remains of the old lesions of an infantile cerebral hemiplegia. Spots of softening, old hemorrhagic scars, cysts, depressions, porencephalic deficiencies and atrophy may be discovered. These are the remains of pre and postnatal cerebral hemorrhages, embolism, thrombosis and meningo-encephalitis. Freud, as before mentioned, believes that all epileptics owe their disease to the lesions that cause the cerebral palsies of childhood, so frequently are they found in the post mortem examinations. With or without atrophy, the brain is sometimes hardened by an over-production of neurogliar tissue, a general gliosis. This is thought by many to be a result and not a cause of the fits. A sclerosis of the cornu ammonis was at one time dignified by Meynert and others as the pathogenetic factor in the disease. It cer- tainly occurs frequently, but is only a part of the general cortical sclerosis or neurogliosis. Cerebral tumors are not commonly the cause of epileptic seizures, but that they may be influential in the production of the disease must not be lost sight of. They provoke epileptiform rather than epileptic seizures. So varied, irregular and disconnected are all these gross conditions that one can only mention their existence in, without being able to postulate definitely their relationship to, the disease proper. They may be the outgrowth of the same etiological influences that establish the epilepsy, while, on the other hand, they may by pressure and other- wise so disturb the circulation and nutrition in the brain as to set up the epileptic state and habit in the cortical cells. There is no question at the present day but that epilepsy is a dis- ease of the brain cortex. For this reason I pass by the older theories, such as that of Kussmaul and Nothnagel, which places the primary trouble in the medulla and pons, where the vasomotor centers are located, that send out impulses which cause spasm of the cerebral vessels and cortical anaemia. Through its symptomatology and by means of experimentation it is settled that the source of the manifesta- tions of epilepsy resides in the cortical cell-bodies. Attempts have been made to determine the immediate cause and character of these cell changes. Here again theory has been rife, while the demonstrable proofs have been wanting. Among the more notable theories that have been propounded upon the slight histological basis of the cortical findings may be noted the cerebral gliosis irritative explanation of Fere. Chaslin and Soimner; the undemonstrable fine molecular changes in the cells assumed by Binswanger, Cowers, Jackson; the autointoxication view and all its minute possibilities upheld by Voisin, Agostim, Coloban, Krainsky ; and the views of those like Prus, Hering, Redlich, BischofT, Rothman and Von Bechterew, who have attempted to localize the origin and extension of the fits. By the work of all these investigators many isolated and sug- gestive facts have been brought to light, in uniting and comparing of which certain broad deductions may be made which will lead us t<» a fairly definite conception of the modern pathogenesis of the disc 194 THE NEURONIC DISEASES The examination of the cortex of twenty epileptics who died at the Craig Colony in New York warranted Prout and Clark to assert that the most striking changes were found in the cells of the second cortical layer, cells distinctly sensory in type. This, as well as the general symptomatology, favors the view that epilepsy is primarily a sensory disease with certain secondary motor disorders. In these cells the nucleus was most affected. It was granular, swollen and poorly outlined. The nucleolus was often absent, many hundred times more so in sections from status epilepticus cases than in sections from normal brains. The nucleus being damaged, the cell nutrition is injured, and as a result chromatodysis, often spoken of by other investigators as well, occurs. The cortex is invaded with leucocytes, especially after status and serial fits, which assume the phagocytic property of removing the products of the tissue metamorphosis. A neurogliosis accompanies the process, filling up the spaces vacated by the wasting cells. Upon these histological findings and the chemical changes and alteration of secretions noted but not positively established by others in the bodies of epileptics, Prout and Clark deduce that epilepsy is a cerebral disease attended and followed by profound and diffuse cortical degeneration ; that the morbid changes concern chiefly the destruction of the nuclei of the cells of the sensory type from which the primary departure of the disease originates and often and in the course of which a progressive and more or less marked and diffuse gliosis oc- curs ; that it is as a disease essentially a sensory phenomenon with a secondary motor manifestation ; that its etio-pathology rests with a variety of toxic or autotoxic agents not as yet definitely isolated or determined; and finally that it is engrafted upon a cortical organic cellular anomaly which is induced largely by a faulty heredity, the exact anatomic nature of which is not known. The two chasms still to be bridged before the circle of our knowl- edge of the pathogenesis of epilepsy can be said to be complete are the nature of the heredity and the change it produces in the cortical cells whereby they become epileptogenous, and the nature and source of the toxic agent that provokes these predisposed cells into the actual state of disease. In regard to the former we know absolutely nothing except what is hidden beneath the phrase developmental inadequacy. In regard to the latter the work of many investigations upon the blood, the urine, the sweat and the secretions of the ductless glands is suggestive and worthy of more than a passing notice. There is a hypotoxic state of these various fluids of the body in the interparoxysmal periods of the disease, a hypertoxic state just before, during and after the fit. Krain- sky and Weber attribute the toxicity to carbonate of ammonium, Donath to cholin and neurin, Haig to uric acid. In this connection it is worthy of note that the disease has been attributed to acetone, to leucomaine poisoning and to a diminished alka- linity of the blood. There is no conclusiveness or uniformity about the autointoxication basis of the disease as yet to warrant more than a passing brief mention. Symptoms. — The symptomatology of epilepsy may be studied in THE NEURONIC DISEASES I95 an orderly manner from several different standpoints. Types of the disease have been constructed out of the predominance of certain symp- toms so that there is a marked difference between the clinical account > of grand mal, petit mal, status epilepticus, partial or Jacksonian epilepsy, psychic epilepsy and the so-called epileptic equivalents. A disconnected account of the seizures may be given as though they alone constituted the disease, and the patient's condition between the attacks be assumed as one of health. Emphasis may be laid upon the slim data which we possess in regard to the exact etiology and pathology of the disease and the clinical description be varied so as to suit the so-called idiopathic or primary and secondary or symptomatic cases. In my own opinion the best plan upon which to study the outward manifestations of epilepsy is to regard all cases as practically the same, with the same general etiology, the same pathological basis and the same general clinical exhibition. I will regard the disease, when once established, as a continuous and more or less persistent one with mere exacerbations, as it were, in the seizures. In other words, an epileptic is such not because of his fits merely, but because he has a cerebral lesion or disorder which reveals itself occasionally by certain violent outbursts. The patient is as much of a victim of disease between attacks or fits as during them. He is in no sense in perfect health until all evidence or possible evidence of the disease is removed per- manently and completely. I will make no distinction between idiopathic and symptomatic epilepsy, for in their essential pathogenesis they are the same ; nor between the general and localized forms, for they too are practically the same, except for extent of symptomatic distribution. In other words, in the following description I will assume that epilepsy is a disease, one and uniform, characterized by a more or less orderly series of events in »11 cases and with only some slight variations in its clinical presentation as the result of some unimportant variations in the extent, origin and location of the lesion. I will note these variations, but I will not emphasize them as special forms, separate types or distinct diseases. Syphilitic meningitis that focalizes itself in the cervical part of the cord is not spoken of as a different disease from syphilitic menin- gitis that focalizes itself chiefly in the lumbar part of the cord, and yet the symptoms of the one are radically more unlike those of the other than are the symptoms of the various so-called types of epilepsy from one another. The essential characteristics of epilepsy are certain premonitorv warnings, a fit or sudden exacerbation with peculiar mental and motor phenomena, a group of sequelae and a quiescent interval until the next exacerbation. This is the uniform series of events that makes up the clinical picture. There may be and usually are many variations in the details of this picture. The premonitory symptoms, for instance, may be of every sort in which involvement of the cerebral functions are implicated. They may be so slight as to be practically nil. The mental disturbance may be profound and accompanied by I96 THE NEURONIC DISEASES other most violent and appalling demonstrations, while, on the other hand, it may be so slight that it alone seems to constitute the entire disease. The sequelae may be so pronounced that they seem quite like essen- tial traits of the disease; but, on the other hand, they may be entirely wanting. Sometimes they assume the role of the so-called equivalents. Finally the quiescent interval may resemble perfect health or con- stitute a mild degree of general physical and mental invalidism or even be occupied by an extension of the exacerbation into the serious con- dition of status epilepticus. So-called symptomatic epilepsy has no place in this description, unless it sets up and is accompanied by the form of the disease now under consideration. Symptomatic epilepsy due to gross irritative lesions is nothing but an epileptiform symptom of the particular disease in which it occurs, and should so be referred to under the symptomatol- ogy of that disease. The first evidence of the disease is always a fit. A person may have been born of epileptic parentage with all the predisposing heritage of the disease, nay, more, he may actually be the victim of the disease, but no decisive evidence of it occur for years or until he is well along in middle life. If the fits should be mild and nocturnal, not even his family may know of his trouble. Whether they occur during the day or night, it sometimes happens that the patient has no very distinct knowledge of them, particularly when they begin or are first discovered by the friends. In some forms of the disease in which the psychosis is the predominant element, the patient's own diary written by him while in the epileptoid state is a surprise to him. In petit mal he is usually unaware of his attack and when it is over goes on about his business as though nothing whatever had taken place. Very often early in the onset of even the seizure types of the disease the patient has only a vague sort of idea that something has happened. I have actually been asked thus by epileptics, especially those who have had their attacks at night, what could have happened to have caused their tongue to be so sore and jagged. It is almost pathetic at times to observe, especially in the young, this wonder, mystery and confusion, nay, even total ignorance and indifference sometimes, displayed by those who are apparently unaware of the nature of their trouble. In such cases the testimony of friends and witnesses of the attacks alone has to be depended upon for an account of the disease. The signs are all objective, the subjective being so slight or swallowed up in the period of overwhelming unconsciousness that the patient himself can give no intelligent account of himself. If the attack is thus capable of being verified and is typical, epilepsy can be positively diagnosed. That does not mean, however, that the attacks alone constitute the disease and the patient may not be the victim of it without the actual occurrence of the typical fits. There are cases that are undoubtedly epileptic, as are shown by the later appearance of the typical fits which in the meantime have gone on exhibiting for years periodical attacks of severe headache, asthma, cardiac disturbances, local and visceral neuralgia, unaccounta- THE NEURONIC DISEASES 1 97 ble moods and spells of eccentricity, vomiting, nocturnal pollution, nightmare and other so-called epileptic equivalents. In such cases these manifestations may awaken the suspicions of the medical at- tendant, but cannot in any way constitute the basis for a* diagnosis of the disease. Only the typical fit can afford such a basis. How often have we all sought in vain for an earlier evidence of the disease in which the first typical outbreak occurred well along in adult life, knowing all the time that it was a hereditary trouble and should have given some warnings in the earlier years of the patient's life ! And how often have we been almost inclined to doubt the pos- sibility of finding any pronounced underlying neuropathic premonition in the history of the patient's life until we were volunteered the state- ment that an abnormal periodical hunger and freakishness of appetite, a dysmenorrhcea without any apparent organic cause for it, an oc- casional insane outburst of ungovernable temper, or a peculiar, irresisti- ble impulse to do and say certain things, have been noted ! Epilepsy is a much-abused term and many and varied are the phenomena that have been recorded under it. Many diagnosticians have come to deny the wisdom even of ever declaring the presence of the disease until more than one fit has actually been observed by him who is called upon to make the diagnosis. This seems to be rather a tightly drawn position to take, and yet it was inevitable almost from the loose manner in which every form of convulsion and mental oblit- eration was looked upon as epileptic. My own position is that epilepsy is present in the patient as a distinct, positive disease even when there are no fits as yet in evidence ; that in such cases the equivalents of the disease may awaken suspicion and put the physician on his guard; but that only upon the occurrence, all other things being considered, of the actual typical attack, varied even as this may be, should a posi- tive diagnosis ever be founded. If possible the physician himself should see the attack, and better still, more than one ; nevertheless if those describing it to him are honest, observant and intelligent indi- viduals, he may base a positive diagnosis of the disease if their account presents a clean-cut, typical picture. Too much caution, however, cannot be exercised by him in making his diagnosis in such a way, for epilepsy is a disease often simulated by malingerers and so closely resembles other diseases, or is so closely imitated by other diseases, that even the most intelligent of the laity will give a description with all honesty of it such as to mislead the medical man into pronouncing it genuine epilepsy. In nearly all cases there are premonitions of an attack. These may be very pronounced and exist for several minutes, hours or days. On the other hand, they may be so slight and brief that they are not recognized by the patient or spoken of until after a most searching inquiry is made by the examiner. In some cases no premonitory signs can seem to be discovered, though I have found that these cases have gradually diminished in number as I have learned to make my exam- inations more searching. These forewarnings may be studied under the heads of sensory, psychic, motor and irregular aur?e. I98 THE NEURONIC DISEASES The term aura is particularly applicable to the sensory premoni- tion, for there is a suggestion of a sort of ascending "spirituous vapor" which Pelops, the master of Galen and first user of the name, sup- posed passed up through the extremities to the head by way of the veins. A peculiar sensation starting in the toes or tips of the fingers, from the stomach or region of the heart, and rapidly and lightly sweep- ing up towards the head, is very often felt just before the stroke of unconsciousness falls. The sensation is varied and uncertain. Many patients declare it is indescribable, especially the very frequent gastric form of it. It may resemble the globus of hysteria or retching. At other times it may consist of actual pain, especially in the vicinity of the heart. In the limbs it consists of some form of paresthesia, such as tingling, pins-and-needles sensation, formication or numbness. There may be slight dull headache. Not always does this sensation ascend ; occasionally it descends. It may not appear at all or in aborted attacks it may die out without the onset of any other symptom what- ever. When it was thought at one time that this sensory aura was somehow the direct cause of the fit, bands were tightly drawn around the arm and leg to check its progress. As in a certain percentage of cases this manoeuvre was followed by an aborted attack, the belief in the causative influence of the aura was strengthened as the result of a sort of post hoc propter hoc reasoning. It is usually stated that these sensory forerunners of the attack occur in about half of all the cases. I am skeptical of all statistics founded upon subjective phenomena, and especially upon such varied, elusive and intensely personal phenomena as are the sensory aurae of epilepsy. I have a suspicion they are present in more than half the cases, for I have not infrequently learned of them in long-subsequent visits upon the patient or from the testimony of friends with better memories, or in other direct and indirect ways when the closest ques- tioning of the patient himself, who may have belittled or forgotten them, failed to elicit their existence. After the general sensory aurae the most frequent and important are those of the special senses. Of these vision furnishes the largest number. Flashes of light, fortification figures, stars, lightning-like lines, increase and diminution in the size of objects, bright colors and darkness are all mentioned. Actual hallucinations and real pictures, curious illusions, the vanishing and approaching of persons and things are epileptic aurae that go a long way to explain some of the wonderful assertions of mystics, religious devotees, hysterical recluses and other superstitious neuropaths. Much fascinating and romantic literature, especially in the Middle Ages, when religiosity was rampant and ignorance was its handmaid, has been written on the basis of these visions. In the sphere of smell and taste some extraordinary phenomena are sometimes met with, though usually the patients who experience them describe them as bitter, sweet, pleasant, disagreeable, metallic, etc. Numbness of the tongue and palate may occur. One of Spratling's patients spoke of the aura as "a snuff of bad air." THE NEURONIC DISEASES I99 Xext to the visual the auditory aurae have furnished the strangest and most weird descriptions. Xot only noises, but the ringing of bells, blowing of whistles, sweet music, voices, human speech and other sounds, both in and out of nature, pleasant and terrifying, are heard. It is easy to conceive how these may be the basis of heavenly com- mands, spiritualistic whisperings and divine inspirations. The range of the auditory aurae stretches all the way from intolerable roaring and shrieks to actual deafness. Were these special sense aune only more frequent than they are, and did they occur relatively more often in the grand mal type of the disease, they would probably be less mysterious and therefore less misleading to the ignorant and superstitious laity. The visual aurae are twice as frequent as all the other special sense aurae combined ; the auditory are one-quarter as frequent as the visual ; the olfactory and gustatory are the least frequent of all. Usually only one sense is affected; rarely, however, two or more senses may exhibit aurae simul- taneously. Still more astounding at times than the sensory aurae, though they are less frequent, are the psychic. The mental processes, especially the imagination and intellection, are unusually quickened. Emotions of a high degree, and a rapid succession of ideas, spring into being and continue until suddenly obliterated by the paroxysmal stroke. It is to be remembered that the sudden, unaccountable actions of the patient may be due to the nature of these exalted feelings and ideas. Fear and horror may cause him to suddenly run away or strike at a neighbor, or accomplish his own destruction. It must be undoubtedly true some- times what we are told of the sudden illumination and inspiration which these patients experience when they are composing, inventing or attempting the solution of some abstruse problem. The fine frenzy of the poet may sometimes be closely allied to a psychic aura. Memory may be exalted, so much so that the far past is regarded by the patient as being actually the present. These aurae are at times delirious and seem like a veritable pre- epileptic insanity. The}- are mostly of the depressive and terrifying type, though they may be of the exalted and ecstatic type with rapture, joy, lightness or immense serenity. Spratling quotes from Dostoievsky, the Russian novelist, who was himself an epileptic and described his own feelings in the character he has drawn in "Besi". "There arc moments, and it is only a matter of five or six seconds, when you sud- denly feel the presence of eternal harmony. This phenomenon is neither terrestrial nor celestial, but is an indescribable something which man in his mortal body can scarcely endure. The terrible thing is the frightful clearness with which it manifests itself and the rapture with which it fills you. During those few seconds I live a whole human existence." It is but a step from this to the belief in and proclamation of one's power of disembodiment and visitation to the supernatural. Psychic aurae, like the sensor}-, are to be awarded the credit of giving us many strange beliefs, surprising mental exhibitions and what 200 THE NEURONIC DISEASES seems almost like omnipotent clairvoyance. It is just here where genius unto madness becomes closely allied. The motor aurce consist of jer kings, local spasms, trembling, twitching in one or more limbs. They are very brief as a rule and are more characteristic of partial or Jacksonian epilepsy. Complicated coordinated movements of an automatic sort are occasionally observed. Procursive epilepsy is that form in which the patient runs forward just before the fit. Some epileptics turn rapidly around and go around in a circle. Gould and Pyle relate the case of an epileptic workman who, upon one occasion, ran from Peterboro to Whittlessy. A patient of Echeverria's resorted quickly to violent exercise on a trapeze to control the spasms. A patient of mine would jump up and run around the dinner table. There are many other premonitory signs of the disease classified under the head of irregular aura. A sudden pallor, general erythema or spotted appearance of the skin may represent a vasomotor aura. Profuse perspiration has introduced a fit. A case reported by Moyer was wont to have sudden attacks of uncontrollable sleepiness. This somnolence has been noticed in a number of cases. Elevation of general and local temperature, and sphygmographic evidence of increased arterial tension have been observed during an aura. Immediately succeeding the aura or, as in some cases, without it, the patient abruptly falls in a state of complete unconsciousness. He may fall in any direction, though usually it is forward. He may strike any near object, such as a hot stove or sharp point and thus acquire a wound that may leave an ugly scar. A young woman patient of mine was found dead, having dropped while making the bed in such a way that her throat and chin became caught on the sideboard of the bed- frame and she was asphyxiated. With the fall and the sudden coma there is a tonic spasm or sudden tetanic stiffening of all the muscles of the body. The teeth are clenched, the arms are rigid and flexed, the thumb is pressed tightly down into the palm of the hand and the fingers firrnly flexed on top of it, the legs are stiff and flexed or extended. The limbs are not all contracted in the same way. The patient appears therefore contorted and hideously deformed. The trunk is twisted or arched. The head is turned to one side, the facial muscles drawn or distorted, the eyes fixed with the lids closed or open and the pupils immoble, or the eye- balls rolled up into the head, the lips compressed and distended laterally, the cheeks bloated and disfigured and the color pale and cyanotic. Frothy saliva, mixed with blood if the tongue happens to have been caught between the snapping teeth, sometimes oozes between the par- tially opened lips. The muscles of the neck are rigid and marked in outline. On account of the spasm of the laryngeal and respiratory muscles a short, sharp, piercing cry, or perhaps only a loud sigh, is heard. The breathing ceases or becomes a mere inspiratory gurgle. The faeces and urine may be involuntarily extruded. After a few seconds or fraction of a minute this first or tonic stage is followed by the second or clonic period. The unconsciousness con- THE NEURONIC DISEASES 201 tinues or may even clear up a little, or may have been only partial from the beginning. The face becomes flushed. The muscles of the entire body twitch and jerk forcibly and rapidly in a rhythmical sort of a way. On account of this the patient may be thrown violently about, some of his joints be dislocated or even a bone be broken. These movements are at times very powerful, symmetrical and regular. The jaw snaps and the tongue, being pushed forward, causes the teeth to lacerate its edges. The eyeballs roll about. The rectum, bladder and seminal vesicles may all be evacuated. The respiratory movements FIGURE 38. m\ WBE^jH P"^^^^^E. g W^fr *m» fcii >^r r^P ^^^^ f Grand mal seizure, with partial retention of consciousness. (After Spratling.) become heavy and sighing. Vomiting and retching may occur. The pulse increases in frequency along with the respirations. Perspiration breaks out over the whole body, and the general appearance of the patient shows a beginning relaxation. The vise-like hold upon him in the tonic stage is clearly letting go dining the clonic stage. Towards the end of the clonic stage, which lasts from one to five minutes, the muscular movements gradualh quiet down, cease entirely in some muscles and in all of them later on, and the patient lies limp and exhausted. Sometimes he opens his eves, looks dazed and eon- fused, and then sinks into a profound slumber; at other times lie seems to suddenly regain complete consciousness, bul is surprised and com- plains of an intense headache and of feeling very tired and sleepy. •Somnolence may last several hours and constitutes what is some- 202 THE NEURONIC DISEASES times called the third stage of the attack. The sleep is so natural and physiological after such a profound expenditure of nerve force, while the rest of the patient's condition is so little disturbed, that the pro- priety of calling this the third stage of the paroxysm may well be FIGURE 39. The order of progression of attack in a case in which the initial spasm — 519 times in succession in forty-nine hours — was confined to the first phalanx of the right thumb. (After Spratling.) questioned. From this sleep the patient usually wakes up refreshed, with his normal consciousness completely restored and only a slight, decreasing headache and possible soreness of the tongue indicating to him that something unusual has happened. THE NEURONIC DISEASES 20} Such is the general sequence of events in a typical grand mal attack. There are variations upon this clinical picture that must now be noted. In the tonic stage of the fit the tetanic rigidity of the muscles may be so powerful, and the cessation of the respiration so prolonged, that •death will occur under the most extraordinary circumstances. A woman once w r ashing dishes at a sink made a noise that attracted attention, and when discovered a little while after was found dead rigidly standing in the same position. Relaxation occurred after they laid her on the floor. Sometimes in this stage a fine series of fibrillary contractions can be detected in the muscles. All muscles of the body may not be equally stifT and in exceptional cases only those of one side have been involved. It is not so very uncommon, even in the so-called idiopathic types of the disease, for the spasm to begin in one limb or on one side of the body. This peculiarity, with others, wdiich indicates a close resemblance between idiopathic and symptomatic epilepsy, gives ground for the contention of Starr that epilepsy is an organic disease and that there is no such distinction as the above between types. The ciliary muscles of the eye are also sometimes included in the spasm (Knies). When they can be tested the reflexes are found to be abolished during the tonic stage. They have been seen to be normal and even exaggerated but in these cases the unconsciousness and general sup- pression may not have been profound. The conjunctiva is insensitive and the pupils are dilated and irresponsive. The fundus oculi has been observed to be pale, then flushed. Thomsen and Oppenheim have shown that not rarely concentric contraction of the visual field follows true epileptic attacks. Transient paralysis, aphasia and dysarthria may immediately succeed a fit. The skin and mucous membrane may be red in spots and exhibit minute hemorrhagic extravasations. After the attack the urine may be discharged in excessive amount, may contain an increase of urea and some albumin. A slight elevation of temperature may be noted during the fit. High temperature belongs to the symptomatology of status epilepticus. Cases in which there are marked and persistent post-epileptic phenomena, such as aphasia, paralysis, tremor, conjugate deviation of the eyes, blindness, deafness, diarrhcea and vomiting, should always be regarded with a high degree of suspicion. True epilepsy may not be the cause of them but rather some gross lesion such as tumor, thrombosis or hemorrhage. After these grand mal attacks have once started they tend to re- appear periodically, and increase in number and severity. For a time they are nocturnal, occurring mostly in the early hours of the morning while the patient is sleeping and the physical forces are at their lowest ebb. Sometimes they take place just after retiring at night. As the disease continues they appear in the daytime and often cease ;it night. Usually they are single but two or three attacks in succession ma\ cur the same day or on successive days. These serial attacks may be so numerous, rapid and close together that a condition of >t-un- ■set up. Some patients have grand mal attacks every few weeks or 204 THE NEURONIC DISEASES months, with many fits of the petit mal type in the intervals. Rarely a patient may experience a fit only once or twice in a year or two. A most interesting and singular form of epilepsy is that which appears in the petit mal type. This is characterized by frequent, brief disturbances of the consciousness, entirely unknown to the patient and without aurae and convulsions. The attacks come on without the slightest warning and may occur several times during the day. The patient suddenly stops his work, becomes fixed and staring and seems to be in a profound condition of mental abstraction. This lasts but a few seconds and when it is past the victim proceeds with his occupa- tion just as if nothing whatever had happened. Bystanders, however, can note the fixity of the attitude, the partial or complete absence of the consciousness, the momentary cyanosis, the strange appearance of the patient and even the slight momentary spasm. In rare cases there may be a feeble aura of some sort, or the spasm may appear transiently without loss of consciousness. In other cases the mind is not so much absent as diverted. The patient responds but his answers are irrele- vant. He may stop in the middle of a sentence and mumble words about something entirely different, or he may automatically get up from his desk and do something else. If he is directing envelopes, for instance, he may suddenly awaken to discover that he has directed an unnecessary number all exactly alike. In such attacks there is usually partial amnesia, the patient dimly remembering that he was doing something different, but what or why he cannot understand. There is an automatic, dream-like state under such circumstances in which the sufferer may do all sorts of queer things, such as undressing himself in public, striking those about him, walking off of dangerous heights and writing long compositions. These states may be prolonged for several days, weeks or months,, enabling the patient to travel long journeys, transact elaborate busi- ness and do 1 a lot of strange things, of all of which he has only the vaguest sort o>f remembrance when he suddenly comes to and finds him- self in the midst of strange surrounding sand anomalous positions. The cases of so-called double consciousness such as that of Alary Reynolds,, reported by Weir Mitchell, and of lima S., studied by Krafft-Ebing, are of this character. The general mental state of these patients, except when they are in the fit, is not as a rule much below the normal. In fact the posses- sors of the most brilliant minds have sometimes been the victims of both grand mal and petit mal attacks. Caesar, Mahomet and Napo- leon are the classical illustrations. As a rule, however, there is apt to be more mental deterioration among the petit mal than among the grand mal epileptics. In the former there may be terrific outbursts of passion and violence, with the commission of great crimes. A veritable delirium and mania may even occur, or there may be a pro- found dementia. Instead of violence a low cunning treachery may be exercised in the attainment of their strange purposes. There is an obvious medico-legal interest surrounding these cases> The degree of consciousness or its perversion should, of course, deter- mine such a patient's criminal responsibility. This problem being so THE NEURONIC DISEASES 205 -difficult, one need not feel surprise at the unfortunate wrangles that sometimes mar the court proceedings when so-called expert testimony is being taken. Some of these attacks are less of the type known as petit mal than they are instances of what are known as the psychic equivalents -of epilepsy. The whole question of epileptic equivalents is still under discus- sion. By the term is meant something that is not so much like an epileptic fit as that it is a substitute for it, equivalent in value, force, meaning, compensation, etc. Most writers recognize the existence of ■epileptic equivalents, though Magnan, Le Grand du Saulle, Tomlin- son and others deny them. The psychic equivalents do certainly at times seem more like states of insanity, perhaps post-epileptic insan- ity. The propriety of characterizing these states as epileptic is doubt- ed for the reason that when they are not associated with typical fits they are rarely if ever so characterized but are then looked upon as ■distinct psychoses or pure mental disorders. However, as epilepsy is in some respects so largely a mental manifestation, it is not easy to deny that a psychosis ma}' be truly of epileptic origin. Hence for the present, at least, it will be well to speak of the epileptic equivalents, -especially those in the psychic sphere. Spratling desires to keep a sharp distinction between true psychic epilepsy and the psychic epileptic equivalents. The distinction rests upon the presence or absence of other epileptic signs such as the motor •disorders. Among the equivalents, the psychic always far outnumbering all the others, may be mentioned the absences, the strange acts, the in- decent exposures, the mild manias, such as nymphomania, klepto- mania, etc., certain phobias and obsessions involving unprovoked as- saults, automatic laughter, a form of angina pectoris, diaphragmatic spasm, sleep states, asthmatic affections, spasm of the glottis, nocturnal pollution, certain forms of pertussis, neuralgias, migraines, the hunger -evil, sudden outbreaks of perspiration, verbigeration, etc. There is such a thing as reflex epilepsy but not in the sense of the old writers. A dormant epileptic condition may be aroused into activity by various exciting peripheral irritants, but a true epilepsy can hardly be attributed to the mere excitation produced by such peri- pheral disturbances. In a person predisposed it is conceivable that a phimosis, a cervical uterine scar, a mass of intestinal parasites, a nasal polyp, a pharyngeal adenoma, an accumulation of wax in the «ar, and muscular anomalies of the eye, may be the cause of initiating the attacks. To say that such conditions can be the cause of epilepsy in non-predisposed individuals would have to be followed by an planation, before the assertion could be accepted, as to why more suf- ferers from these common conditions do not have epilepsy, and why in those that do have epilepsy from this condition there are always found the great predisposing factors of heredity, degeneracy, intoxi- cation or trauma. Oppenheim notes that binding a limb <>n which there is a peri- pheral cicatrix will often check an epileptic fit. Hie same thing will 206 THE NEURONIC DISEASES often occur, however, in so-called simple idiopathic epilepsy without any such peripheral irritant. The scar in such a case may indeed be a sort of epileptogenous zone, but that does not establish it as the sole or prime cause of the disease. I find there is not a little confusion in the minds of some of the profession between partial, or Jacksonian epilepsy, and what is called symptomatic epilepsy. Every form of local convulsion is not partial epilepsy. It may not be epilepsy at all but merely epileptiform. In a way it may be physio- logical rather than pathological, for it may represent the normal over- action of a vigorous and healthy sensory-motor apparatus to an ex- ternal source of constant irritation. So-called symptomatic epilepsy is therefore but a convulsive symptom of the disease of which it is declared to be symptomatic. A compressing lesion of the foot, say, causing pains and spasms in the area of the nerves caught in the lesion does not necessarily cause those pains and spasms to be symptoms of a nervous disease. This is an important distinction to bear in mind when speaking of partial, or Jacksonian, epilepsy. This, unlike mere symptomatic epileptiform manifestations, is a true nervous or cere- bral disease and is epilepsy. It may originate remotely from the irri- tation, disturbed circulation or other influence from an extra-neural growth or lesion of some sort. Gradually, however, a local change is set up by this extra-neural influence so that the pathological basis of a true epilepsy is established. This change may remain local, more often it spreads throughout the entire cortex. The great characteristic of Jacksonian epilepsy, therefore, is its local distribution, either during the entire fit or at its commencement. The sensory or motor phenomena begin in one arm or leg, one fin- ger or side of the face, then increase with or without the loss of con- sciousness through the entire epileptic cycle. This is not a mere aura, though it may partake of the nature of one if the fit passes into a general one involving the mind and body, as in the ordinary grand mal type. It is, in the purely local cases, the epilepsy itself. Hence we see the propriety of the term partial epilepsy. As I have said it may remain partial and be free of any asso- ciated disturbance of the mind. In most cases, however, the loss of consciousness comes on, somewhat more slowly than in the grand mal seizures of the idiopathic type, and then the patient experiences the typical generalized convulsive seizure. The pre-eminent fact in re- gard to it is that the localization of the phenomenon never changes. These cases are usually followed by more or less persistent local physical defects such as paresis, paresthesias, aphasia, etc. Their causes are numerous and may be purely local or general. Among those that have been enumerated I may instance injuries to the skull involving the brain, cerebral hemorrhage with its pathological sequela?, thrombus, embolus, syphilitic meningitis, tumor, alcoholic ox- cess, old cicatrices and visceral sources of irritation. This does not exhaust the list but indicates a few ways in which, the cortical cells may be rendered epileptogenetic. There has always been a discussion among writers as to whether THE NEURONIC DISEASES 20J simple tonic or clonic convulsions alone can constitute epilepsy. Prich- ard described, in 1822, a violent and powerful form of epileptic seizure in which a tetanic state of the muscles occurred. These cases are rare, fortunately so, for they are very fatal. It seems to me that Prichard described simply an exceptionally severe type of grand mal in which death occurred during the prolongation of the tonic stage, and before the appearance of the clonic stage. The myoclonus-cpilcpsy, described by Unverricht in 1891, is an association disease or trouble like hystero-epilepsy, choreic epilepsy, etc. The two diseases may run together in the same patient, with alternation or combination of the symptoms. The association, how- ever, does not constitute a new disease or special type of either. Occa- sionally the disease is really one thing with a mere resemblance in some of its phenomena to the other. Hystero-epilepsy, for instance, is usually hysteria whose outbreaks resemble at times epileptic seiz- ures. It serves no useful purpose, and is only confusing, to establish new diseases or even new types of mere associated or similarly appear- ing affections. Much stronger is the ground, if types are to be noted, for sepa- rating status ipilepticus as a special form of the disease. Clark de- fines it as the maximum development of epilepsy in which one paroxysm follows another so closely that the coma and exhaustion are continuous between seizures. Sooner or later there is a marked eleva- tion of temperature, pulse and respiratory movements, with profound exhaustion and death. Status occurs most frequently in the grand mal type of the disease and partakes of all the features of the disease except in regard to the special points above noted. To treat it as a separate disease with its own etiology, pathology, symptoms and treatment is quite unnecessary. It is a culmination of epilepsy and is distinguished by differences of degree rather than kind. The patient remains continuously unconscious. The muscles appear to be almost continuously convulsed. The temperature may rise to 109 degrees F. and even recovery occur, as in a case reported by Spratling at the Craig Colony. About half the cases that attain the condition of status die. Among the sequela of epileptic convulsions are to be noted the scars upon the tongue and about the face and body, extensive burns, fractures, dislocations, hemorrhagic extravasations, exhaustion, paraly- ses, elevation of body temperature and certain changes in the blood and the circulation. Some of these, especially the apparent increase of toxicity in the blood and elevation of the blood pressure, need fur- ther investigation. The mental sequelec of single and multiple epileptic seizures is always a matter of great anxiety to the patient and his family. It would not seem reasonable to expect so profound a disturbance of the cerebral functions to continue long without some deterioration of the mental faculties. Depression, mistrust, irritability, melancholia, and increased emotionalism are usually noticeable in these patients. The mental condition may get no worse than this, and may indeed even 208 THE NEURONIC DISEASES seem to be not in the least affected for years. Later on the memory grows weaker, amnesia occurs, the intelligence becomes blunted, more or less stupidity supervenes and ultimately simple dementia appears. Hypochondriasis is often present. The mental deterioration charac- teristic of the disease I am convinced has been too often charged to the use of bromine. When the disease occurs in childhood the mental results are for obvious reasons much more disastrous. Imbecility and idiocy are espe- cially liable to occur. All forms of the disease without distinction are responsible for the lowering of the intelligence. Some have thought that there was more danger in this respect in the petit mal than in the grand mal, but it is to be remembered that the petit mal type of the trouble is almost always entirely psychical. It represents as it were a considerable degree of mental degeneracy in itself. As bearing on the treatment it is to be noted that sometimes epi- leptic seizures are followed by a disappearance of a certain amount of stupor and mental indifference. I have long taught in my lectures that it is not always wise or safe to suppress completely and perma- nently the fits even when that is possible. Occasionally an epileptic who shows himself amenable and responsive to treatment is better off for a fit now and then. Diagnosis. Epilepsy is usually easily diagnosed. In doubtful cases it should never be hastily decided. The longer a case is ob- served the more the diagnosis becomes conclusive. It is when the dis- ease is first discovered, and when it is wanting in some typical mani- festation, that the diagnosis sometimes becomes problematic. For this reason and because of the serious prognosis attending the disease, the physician will do well to rarely make a positive diagnosis until he has been able to watch the patient in one of his attacks. The laity are so easily misled, being untrained and excitable, that it is never wise, ex- cept possibly in certain very obvious instances, to risk a diagnosis upon the description of friends or relatives. The earlier it is in the disease and the more atypical the account of it seems to be, the more impera- tive is it that the diagnostician should see the fits for himself, or should place the patient where he can be under the continual observa- tion for a time of a trained assistant. Under such circumstances every detail, however insignificant it may seem to be, should be made note of. The presence and character of the aura? should receive the most scrutinizing attention, for the very nature of the disease and its source may be indicated in the aura. Then the mode of onset should be care- fully observed. This will include the relative period at which the unconsciousness took place, its depth and duration ; the location of the first convulsive manifestation, its extension over the rest of the body, its tonic and clonic character, its duration and its mode of dis- appearance ; the state of the pupils ; the condition of the eyes ; the strength and frequency of the respirations and pulse beat; the cyanosis. followed by flushing of the skin ; the involuntary evacuations of the bowels and bladder. The character of the after effects will in some cases help to estab- THE NEURONIC DISEASES 2(X) lish the diagnosis. They are the return to a dazed and surprised state of consciousness, the headache, the profound exhaustion, the transient paralyses, local muscular soreness and even slight mental aberration. In every suspected case of epilepsy, whatever the type, look for some disturbance of the consciousness. This is undoubtedly the car- dinal symptom of genuine epilepsy. It may be the sole symptom, as in some cases of petit mal, psychic epilepsy and the psychic equivalents of the disease. It is even detectable in the cases of partial or Jackso- nian epilepsy in which the motor manifestations ma}- be almost over- whelmingly dominant. Xote the character of the disturbance in every particular and the order of its progression for it is pathognomonic of the disease and is never exactly the same as it is in other diseases. If it is accompanied by the convulsions and other usual symptoms of a lit. the diagnosis is absolutely easy and certain. A good deal is written about the fades epHeptica. Many epileptics show in their faces the indications of a degenerative heredity, as I have often pointed out to my classes. They also exhibit scars acquired by falls and injuries sustained in previous attacks. They may even re- veal the facial acne from the overuse of the bromides. All of this, however, is of no very great value in the matter of diagnosis for with such conditions present the case is apt to be a pronounced and ad- vanced one, and doubtless already easilv diagnosed from the other symptoms. As the Upical facies epileptica is not an early, sudden acquisition, but rather the result of a slow development, it cannot as- sist us much at that period in the disease when such assistance is most needed, and when the diagnosis of the disease is of the supremest im- portance. There are a number of conditions in which mental and motor disturbances occur, with which an epileptic attack is capable of being confused. They are hysteria, alcoholism, drug intoxication, syncope, uremic poisoning, cerebral apoplexy, general paresis, vertigo, tetanus and simulation. The differentiation between hysteria and epilepsy is at times a matter of great importance and difficulty. There are convulsive seiz- ures sometimes in hysteria so like those of epilepsy that, for a long time the}' were studied apart under the name of hystero-epilepsy. The one manifestation that can be absolutely relied upon to establish the distinction is the pupillary reaction. In hysteria this is not lost as it invariably is in epilepsy. Hysteria being a psychosis primarily the whole complexion of the case is dominated by the mentality of the pa- tient ; in epilepsy the mental disturbance is the result of a frank cerebral lesion and is therefore often accompanied by marked localization phe- nomena. In other words the mental side of the picture and a certain suggestiveness of active volition are more noticeable in hysteria, whereas in epilepsy the convulsions or motor phenomena and appear- ance of complete absence of volition stand prominently forth. The suddenness of the attack, the initial cry. the involuntary twitchii the injury from the fall, the mvoluntarv evacuations, the subsequent exhaustion, stupor, headache and somnolence all point t<> epilepsy. 210 THE NEURONIC DISEASES Hysterics do not injure themselves by falling unconscious nor do they often bite their tongues. Involuntary micturition practically never oc- curs in hysteria. Hysterics yell and cry and toss themselves about and complain of pains and develop their attacks at the time of day, early evening, when they are likely to be observed. Their attacks are longer in duration than those of epileptics and are sometimes amenable to sharp suggestion. Their symptoms vary during the attack and have prominently among them sensory phenomena. The mental criteria are of some value as hysterics are usually keen and intelligent, whereas epileptics, especially of long standing, are dull, somnolent and even demented. The facies epileptica is not exhibited by hysterics nor do they ever have the post-epileptic paraly- sis, hemorrhagic extravasations, wounds, mental confusion and auto- matism that epileptics often show. They never develop a temperature of a degree or two, such as occurs in the epileptic. On the other hand they recover promptly, pass large quantities of urine after the attack and assume an entirely different manner when the causes of the initia- tive a emotion out of which the attack grew are removed. A motive lurks behind many outbreaks of hysteria, and as the attack is thus somewhat in the sphere of volition, painful pressure upon the ovaries, threats of discomfort and objectionable manoeuvres will often bring a hysterical convulsion to an abrupt termination. The coma of acute alcoholism is not so complete as it is in epilepsy and there are no typical convulsive movements. The odor of the breath, the flushed face, the maudlin semidelirious state, and the ab- sence of most of the characteristic symptoms of epilepsy, such as the pallor, the turned-up eyes, the bloody frothing at the mouth, the rigid condition of the muscles, etc., all distinguish the one disease easily from the other. Urcemic, drug and lead intoxication are to be distinguished by the usual history of disease in other organs, of drug-taking and occu- pation in lead. Syncope occurs in feeble individuals and under special surround- ing conditions such as an over-heated room, shock, over-exertion. Heart disease is often present. Syncope is wanting in the usual signs of epilepsy, such as the aura, the muscular spasm, the initial moan, the evacuations and the usual sequelae of stupor, headache and somno- lence. The faintings come and go, several in succession, like a soft zephyr without causing much disturbance in the patient's attitude. The paralytic symptoms, the history of the onset, the action of the pupils, the respiration, the duration of the coma, with the absence of many of the symptoms of epilepsy, will help to distinguish the cerebral apoplexia from the latter. Aural vertigo, or Meniere's disease, is accompanied by aural symp- toms such as tinnitus and deafness. In the early stages of dementia paralytica the convulsions are apt to be unilateral, and not accompanied by a loss of consciousness. There are other well-known symptoms, such as the tremor, the emotionalism, the eye symptoms, that enable us to recognize general paresis. However, it is to be remembered that in general paresis, tumor, THE NEURONIC DISEASES 21 1 cerebral syphilis, nephritis and arteriosclerosis the epileptiform con- vulsions are more apoplectic and symptomatic than are the evidences of genuine epilepsy. Therefore, always search well for the symptoms of these diseases and regard the convulsions as wholly one of these symp- toms. In other words, the examination is not so much to determine the differential diagnosis between an idiopathic and symptomatic epilepsy, so-called, as it is to determine the diagnosis of the disease of which the epileptiform manifestation constitutes one, and perhaps a minor, evidence. In tetanus there is generally a history of an injury, and the jaws are characteristically locked. Simulation sometimes causes quite a little trouble. To escape punishment for a crime, or to win large damages from a railroad or other corporation, are two powerful motives to tempt cunning persons to study and imitate epilepsy. Usually their detection is easy, but I have seen the fraud so skillfully practiced that the shrewdest exam- iners were deceived. If closely watched a simulator will omit or perform wrongly some one manifestation among the many that he has to remember. For instance he will not close his thumb down and clench it beneath his fingers, or he will forget the cry, or fail to bite his tongue. His re- flexes will be lively, especially his pupillary reactions. He will ex- hibit no scars, no hemorrhagic extravasations, no petechial spots. There will be no involuntary evacuations, nor will he suffer afterwards with an exhaustion paralysis, a stupor and prolonged somnolence. His body temperature will not rise. He will tend to overdo the part and elaborate symptoms that should be kept in abeyance, or make little of those that should be very prominent. Without seeing the patient, so-called, I detected a very cunning simulator once in a long hypothet- ical question constructed to correspond with the case, because one part of the clinical picture did not harmonize with another part, whereas if each had been presented alone without the other the deception would have been much harder to detect. Mai ret emphasizes the importance of a urinary analysis. The toxicity of the urine is said to be decreased after the attack, and ren- dered hypotoxic in the intervals of true epilepsy. Spratling points out that a simulator can reproduce the tonic bet- ter than he can the clonic contraction. Heller has suggested that the thigh, for instance, be firmly grasp- ed, and in a simulator the muscles will relax or be immediately con- tracted again if an attempt is made to flex the limb. Finally the simulator cannot cause the loss of the knee-jerk and ankle clonus, which occurs after a severe epileptic attack. Prognosis. There is so much uncertainty about the course and termination of epilepsy that it is risky to assert an opinion. It may continue for years and the patient die of some other disease, such as pulmonary tuberculosis. ( )n the other hand it may suddenly de- velop into the dangerous and fatal condition of status. Some patients have attacks only once or twice a vear, others have them several 212 THE NEURONIC DISEASES times a day. Often they appear in a series of several attacks during several days and then there will be none for a considerable time. It is not usual for the attacks to remain always of the same type. They tend as a rule to increase gradually in frequency and severity. In the more common form of the trouble the early attacks will be nocturnal and for that reason long unrecognized. They will then be discovered, by the roommate of the patient, and if he is not told of them, may continue as nocturnal and unknown to him. Usually, how- ever, attacks begin to appear in the daytime along with, or instead of, the nocturnal ones. They occur in the grand mal form about every two or three months and several times in the interval in the petit mal form. The minor fits may occur many times a day. There is always danger of accidental death in a seizure. Falls may result in fracture of the skull, severe and extensive burns, chok- ing and asphyxia by compression of the throat from extraordinary position, drowning and in a hundred other ways. Continuously nocturnal attacks are more dangerous than diurnal because they occur more frequently in feeble persons and overtake the individual when his vital forces are at their lowest. Though the immediate danger to life in epilepsy is not great, it is nevertheless a serious disease. While not so often killing the patient directly, it greatly jeopardizes his existence by the numerous fatal pos- sibilities it surrounds him with. Tuberculosis of the lungs 'terminates a considerable number of cases. Status epilepticus comes next as a cause of the fatal issue. About half of the cases of status die. Sudden death in a fit may be due to rupture of the heart, passage of food from the stomach into the trachea, asphyxia from position, sudden paralysis of the respiratory center and a prolonged tetanic con- vulsion. Spratling furnishes the following ratios : Of every one hundred epileptics who die, about four do so as the result of a single seizure ; twenty-four as the result of status ; twenty-four as the result of some lung disease, chiefly tuberculosis ; twelve as the result of accident in- cluding suffocation in bed ; ten as the result of some organic heart trouble: and about twenty-six from all other causes. Epilepsy, apart from its fatalities, always tends to shorten life. Early cases rarely pass beyond middle life. Later cases may continue into old age. De^th is not the worse thing to be dreaded by an epilep- tic. The terminal dementia ; the occasional maniacal attacks ; the out- bursts of emotion and rage with the commission of crime: are possi- bilities that death might beneficently forestall. The petit mal and psychic epilepsies are particularly to be regretted and guarded on this account. The question of the cure of epilepsy is just now undergoing a lively discussion. A hundred years ago or so the prognosis in this respect was much better than it is to-day. The cures were rated then even as high as fifteen per cent. Tt is not improbable that sympto- matic and other amenable forms of the disease entered into the making of these very favorable statistics. Perhaps more attention of a dis- THE NEURONIC DISEASES 213 ciplinary and hygienic sort was given to the patient then than now. as the use of the bromides, undoubtedly overdone now, was unknown. On the other hand there are many to-day who regard epilepsy as an absolutely incurable disease. Such a pessimistic view is hardly justifiable. The term cure has a varied significance and while a res- toration back to a condition of perfect normality would perhaps be a Utopian dream, a cessation of the fits for years, and a gradual return to a better state of mind is certainly to be regarded in the light of a cure. If under the enlightened modern treatment of the disease with bromides and hygienic measures, and especially with the aid of the colonization method, all epileptics could be rigidly controlled, and if all the cases that improve, then wander away and never seek for further medical advice, could be gathered together, I am sure a set of tables could be constructed showing statistically a much more hopeful out- look for the cure of the disease. It certainly is not an absolutely in- curable trouble and the percentage of restorations, roughly and yet conservatively estimated under modern treatment as about five per cent, is enough to warrant the attempt in every case that offers the slightest degree of favorable indication. It would be a happy hit if we could always determine just which class of cases are the most amenable to treatment. Turner and oth- ers declare that senile epilepsy is essentially a tractable disease. The least favorable cases are those that begin under ten years of age. If arrest occurs between the fifteenth and twentieth years the outlook is particularly favorable. The chances for a cure then steadily decrease with the advancing' years but after thirty-five the intractable cases again begin to diminish. m Spratling says if there is a difference in the curability of the two sexes, he is inclined to think that it favors the male side. Contrary to the opinion held by some high authorities, the same author finds that the influence of the catamenia and marriage is not good but rather the reverse. The attacks have been unaltered in the least in most of the cases; in a few of them they have been increased in both frequency and severity. As is well known the grand mal type of the disease is more sus- ceptible of treatment than is the petit mal, and the diurnal than the 11 .cturnal. Acquired and inherited mental weakness renders the out- look less hopeful in all forms of the trouble. The previous duration of the disease will also determine the patient's future chances. The re- moval of certain exciting causes, such as alcohol, syphilis, local mal- adies and reflex sources of irritation may, if done early, diminish or even stop entirely the attacks, with all further progress of the dis< Spontaneous recoveries have been reported but they are ><> rare as hardly to he taken into the consideration of the prognosis. The strong tendency of the disease is always to perpetuate itself. The probability of imbecility and idiocy should always he kept in mind in connection with infantile epilepsy. TREATMENT. Some one has uttered the paradox that the way to cure epilepsy is to stop the fits. This is one of those half-truths 214 THE NEURONIC DISEASES which when lived strictly up to often do incalculable harm. Were the fits the whole of epilepsy, such an aphorism might establish good therapeutics. Unfortunately we are learning more and more that epilepsy is a deep-seated, continuous, obstinate disease of the brain, of which certain outbursts of a periodical sort constitute but one of many evidences. To stop the fits merely is, therefore, not the great- est good to be obtained from treatment. It does not in itself cure the disease. In some cases it has actually done harm. Every case of epilepsy, viewed from the therapeutic standpoint, consists of two elemental factors, the disease proper, and its violent exhibition in the fits. The latter are the outgrowth of the former and dependent upon it. By reacting upon a diseased and weakened nervous system, the frequent violence of the fits may undoubtedly increase the nervous weakness and underlying epileptogenous state. All treatment of epilepsy therefore must direct itself to checking the fits and to restoring the diseased nervous system. It must be both fundamental and symptomatic, general and specific, prophylactic and alterative — in a word it must attack the trouble at its source and it must restrain it in its violent outbursts. Any line of treatment that confines itself to one or the other of these two sides of the picture will surely fail of attaining the best re- sults. The administration of the bromides to suppress the convulsions, to the exclusion of all other treatment of the patient as a victim of a profound general disease, is as unscientific and illogical as it would be to neglect the fits entirely and to try and cure him by attacking the general underlying condition. The superior results of institutional and colonization treatment over those generally obtained in private practice are due to the fact that in the latter, for obvious reasons, medicinal control of the fits merely takes precedence over the general hygienic, pedagogic,, disciplinary and tonic measures, whereas in the former it is just the reverse. It is a platitude to state that the first step in the treatment of every case of epilepsy is to make a thorough search for, and remove, every possible source of irritation. This is absolutely necessary in every case no matter what may be its heredity, its previous duration or its type. Nothing that is abnormal about the patient, and that can be removed or remedied, should be allowed to pass. To mention all the possible abnormalities would require an enumeration practically of all the diseases, medical and surgical, to which the human body is liable. Every organ, every tissue, every function should be closely in- terrogated and if found to be wrong, should be righted as far as it is in the power of modern science to do so. When this is done the fits will diminish and ameliorate in a certain proportion of cases. This is the mirage, however, towards which many sincere and skillful in- vestigators have been beguiled when announcing to the world that epilepsy is due to certain organic peripheral defects and that they have cured it by removing those defects. Operations upon the eyes, nose and sexual organs have all been heralded as cures for the disease. Sooner or later, however, the fits have returned in spite of the operations, showing that the reputed THE NEURONIC DISEASES 21 5 cure was nothing but a temporary relief, not even a permanent ameliora- tion. All hope of curing the disease by thus removing mere peripheral excitants shows a lamentable misconception of its true central patho- genic basis. The eye-strain, the nasal polyp, the tapeworm, the phimo- sis, the constipation, or any other abnormal condition, must be re- moved as promptly as possible. It will not cure the disease but it will remove possibly an excitant of an epileptic central nervous system and so cause a diminution in the number and severity of the fits. The basis of epilepsy is a cortical change. If there is no change in the cortex and the fits are but a sort of a Round-Robin-Hood reflex manifestation from a peripheral excitation, I regard the epileptiform phenomenon in such a state of affairs as physiological rather than pathological. It is not true epilepsy though it may be highly epilep- tiform. It is obvious that the proper wearing of glasses, the excision of old cicatrices, the extraction of an ulcerated tooth, will put a stop to the reflex convulsive phenomenon. Nay more, if done early it may thus even be said to cure epilepsy, in some few cases, prophylactically, for it is undoubtedly true that the continuation of such a severe form of reflex action does sometimes set up an epileptic habit in the cor- tical cells, which may go on to the development of the typical degen- erative condition which underlies the true disease. It is never a trivial matter therefore to remove these various ab- normalities in epilepsy; it is trivial to regard the amelioration caused by such removal as a cure of the disease. Where a patient can best be treated is a question that always pre- sents itself. This cannot be answered until after many factors have been taken into consideration. If the disease is of a light form and the patient's intelligence is good he may get along well enough at home. Cases that first appear in adult life, after the mind has developed, as a rule do not need removal from home so much as those that appear in childhood and in which the mind is likely to be most seriously af- fected. In general it may be said that most epileptics, young or old, are better off away from their old haunts and companionships where they are neglected, continually looked upon as peculiar and are able to constantly draw invidious comparisons between their own unfortunate state and that of their friends and relatives. Institutions and colonies, and private sanitaria, when properly conducted, afford the best re- treat for them. Here their food, exercise, bathing, work and amuse ments can be regulated and systematized while they themselves can be protected from doing themselves or others harm if an insane fit should suddenly overtake them. If insanity complicates the case, of course removal to an asylum : - absolutely imperative, and though the insanity may be only tempo- rary it is better for the patient to remain there in case it should un- expectedly reappear. Even the mentally weak epileptics are undoubt- edly better off in an institution. The absolute control of the patient by the physician is a sine qua 2l6 THE NEURONIC DISEASES non to any degree of success in the treatment, and this alone estab- lishes the superiority of institution over home management. Colonies, such as those at Bielefeld, in Germany ; Chalfont, St. Peter, near London, and Craig Colony, at Sonyea in New York State, are growing in popular and scientific estimation. ■ The home-life, the preservation of his individuality, the absence of the competitions, ex- citements, allurements and comparisons incident to urban and subur- ban normal life, and the sympathetic companionship and uniformity of avocations here secured cannot but exert a better influence upon the patient. His invalidism is less emphasized in the midst of such sur- roundings and his entire mode of life and treatment is under closer supervision. He is educated intellectually and physically, trained in the use of tools, set to do attractive tasks, and in every way encour- aged to regard himself as a useful and responsible member of the com- munity. This is wholesome upon the mind and so checks one of the saddest possibilities of epilepsy, namely, the mental deterioration. All epileptics need outdoor exercise. This is forcibly demon- strated, as Spratling says, by the records at Craig Colony, showing a less aggregate number of fits in summer than in winter, and less among men than among women. The outdoor exercise must be systematic and non-exhausting. Gardening, farming and tending cattle are espe- cially commendable. Mild, unemotional indoor games and occupa- tions are to be encouraged. Marriage, for obvious reasons, had better be discouraged among epileptics. Alcohol, tobacco and other dissipations can be given up. Spratling says that in some cases he has known smoking to bring a calm that the individual stood in need of. The dietary of an epileptic needs supervision in only a few par- ticulars. It should be simple and include the breadstuffs, milk, but- ter, fruits and cereals. Eggs are good but meats should be indulged in sparingly. Seeded berries, cake, pastry, condiments, pork, veal, ham and grease-fried foods, alcohol, tea and coffee, should be avoided. Regular meals and abundance of easily-digested food should be the rule. The stomach must not be overloaded. The omission of sodium chloride from the diet of the patient is the latest suggestion that seems to have proved beneficial in a larg*e number of cases. Climatic, hydrotherapeutic and electrical treatment can only affect the disease indirectly by upbuilding the general constitution. The medicinal treatment of epilepsy is very old and consequently boasts of a long list of remedies. Some of these are still used, though they are probably without influence upon the disease. Others mod- ify the disease indirectly either by acting as general tonics, suppress- ing the nervous excitability, or influencing the circulation. We have no remedy that will make over the degenerated cortical cells into nor- mal healthy organs. We can only affect their abnormal function, ap- parently not their histological structure. If. however, we can check their excessive expenditure of nerve force, we may be able to put them in such a state of quietude that they can catch up, as it were, in their self-nutritive processes and this in the course of time will restore them THE NEURONIC DISEASES 2IJ to a more or less normal condition histologically. It is only within re- cent years that the medicinal treatment of epilepsy has been correctlv appreciated, put upon a sound basis and afforded the most desirable results. As we are ignorant of the minute cellular changes upon which the epileptic phenomena depend, and as we only know that certain medic- inal agents seem to have the power of suppressing these phenomena, without knowing how they do it, their administration is regulated by the changes produced by them on the fits. In other words the medic- inal treatment of epilepsy is purely a symptomatic treatment, and the chief end of our efforts by these means is the suppression or diminution of the paroxysms. This statement of course does not apply to the use of general ton- ics like iron and quinine, nor to the use of remedies directed against particular constitutional conditions like syphilis, malaria, alcoholism, and other forms of intoxication and infection. And it will have to be modified if in the future it should be discovered that toxaemia plays the chief role in the disease, and serotherapy is established on a posi- tive scientific basis. In the control of the paroxysms sometimes immediate measures are wholly or partially successful. These of course are only appli- cable when there is a distinct aura of sufficient duration. Patients can carry about with them the small glass pearls, each containing three minims of nitrite of amyl, and when they feel an attack coming on, can quickly break one in the handkerchief and inhale the vapor. A small bottle containing a bit of cotton saturated with the nitrite can be quickly uncorked and the contents inhaled. It is supposed that this flushes the brain with a rush of blood, or at least relaxes the vasomotor spasm of the cerebral circulation upon which the paroxysm is hypo- thetically believed by some to depend. At all events it is so often ef- fective in abating attacks that it is wise for all patients to carry the drug and use it if they have time to do so. When the aura begins in a limb, an old and sometimes success- ful manoeuvre is to suddenly constrict the limb with a tight band. Many patients wear a strap continuously and so arranged that they can quickly draw it tight themselves. Strong flexion, extension or traction on a limb may be tried. Cutaneous stimulation with a dash of cold water, tickling, pinch- ing, rubbing and slapping is also sometimes available. Two or more epileptics can travel together and serve each other in this way. Chloral hydrate in hypnotic dosage, or a teaspoonful of common salt, may abort an attack. According to Fancher and Spratling a few case.- have been checked by the hypodermatic injection of 1-10 or 1-15 of a grain of apomor- phine. Vomiting will probably follow but that often occurs in epilepsy. Brown-Sequard, Bravais, llirt and Buzzard have attempted to abort the seizures by maintaining a permanent blister about the limb. It is always to hv remembered that many patients are better for having a seizure, and hence it is not always desirable to check it. If 2l8 THE NEURONIC DISEASES they are somnolent, stupid, subject to headache and distressed in feel- ing after the abortion of the fit, it had better not be checked next time. In all cases it may be said that the aborting of attacks does not modify the disease, and is only commendable on the grounds of per- sonal comfort and the avoidance of the possible accidents. These, how- ever, are reduced to a minimum by having the patient under constant surveillance. When the attack has occurred there is not much to be done, ex- cept to guard against accidents. The clothing should be loosened, a piece of cork or rolled-up rag should be placed between the teeth to prevent biting of the tongue, the general attitude should be arranged so that if vomiting occurs food will not slip into the trachea, and the patient placed where he can sleep after the paroxysm is past. In status epilepticus rectal injections of chloral hydrate, forty- five to sixty grains, or amylen hydrate, five to eight grains, should be tried. Morphine is generally useless though it may be used in combi- nations. The following emergency formula is highly recommended for status by Clark : ^ Tr. opii deoderat. m. v. Potass, bromid. gr. xxv. Chloral hydrat. gr. xx. Liq. morph. sulph. (U. S. 5j. Sig. — One dose ; repeat in two hours if necessary. If these remedies fail to modify the attack, bloodletting should be resorted to. The condition is desperate and .the remedy must necessa- rily be heroic. Of all the remedies employed to control the fits the bromides still continue to enjoy the highest favor. They were introduced as anti- epileptic remedies by Laycock over half a century ago, and though they have been much abused and even when carefully employed do not constitute an ideal therapy, they are the best medicinal agents that we possess. Their mode of action is quite unknown and their ad- ministration must always be empirical. On this account there is even to-day, after these long years of trial, much diversity of opinion as to how they should be used. As drug treatment is necessarily so predominant in the home treatment of epilepsy, the abuse of the bromides is observed more in private than in institutional practice. As a rule the remedy is given in too large quantities, it is pushed after it has demonstrated its in- effectiveness, and it is not administered in proper time relationship to the expected paroxvsm. Moreover, when it is found to be effective and well borne it is not continued long enough. There are a number of bromide salts that are available, though in my own experience, as well as in that of others, the potassium salt is in the long run the most useful. Some physicians prefer the use of THE NEURONIC DISEASES 2IQ ■a. single salt, others a combination of two or three of them. The potassium, sodium, ammonium and lithium bromides may be used in this way, the relative dosage varying according to the idiosyncrasy of the patient and the predilection of the prescriber. Strontium bromide is said to disturb the stomach the least. Calcium bromide, gold bro- mide, zinc bromide, bromium and camphor monobromate are other -eligible preparations that may be tried when those first mentioned fail. As a rule these will fail, too, but in a disease that is so obstinate one is glad at times to have something to ring the changes on. The bromides may be used alone or in combination with other Temedies to be mentioned. Two cardinal rules are to be observed when they are resorted to. The one is to use them in the best way, and in only such quantity as will check the fit. The other is to continue them, when found to be .effective, for a year or more in spite of the absence of all symptoms. The average case takes from a dram to a dram and a half a daw This may be administered in several small doses or, better still, in one large dose timed so that the maximum effect of the bromide will cor- respond with the hour of the expected fit. When stopping the use of the bromides it is never safe to with- draw them all at once; this has occasionally precipitated a condition of status. Xo rule can be set down for the administration of these prepara- tions, as the greatest success is obtained in studying carefully their effect upon each individual case. Dilute them largely with water ; do not administer them within an hour before or after meals; give ihem to the point of tolerance; prepare the largest dose about five or six hours before the expected fit, whether it be nocturnal or diurnal, continue the remedy for one or two years ; withdraw it gradually ; and employ it with the expectation of the best results in grand tnal, and the least favorable results in petit mal and psychic epilepsy. Such are the main points to be remembered. Certain accidents are to be avoided in the use of the bromides. Of these the gastric disturbances and the facial acne are especially unfor- tunate. Sometimes these can be avoided by the administration of iron and arsenic during the course of the bromides. Mitchell has seen irritability, melancholia at menstruation, bad temper, suicidal or hom- icidal tendencies and temporary delusions produced by them. They do undoubtedly depress the mental faculties; so does the disease for which they are administered. It is not always assumable, therefore, that every case of mental deterioration during a course of bromism i.^ the result of the medicine. In the hypochlorization method of Toulouse and Richet the pa- tient's food is salted with bromide of sodium instead of chloride of sodium. It is said that ten grains of the salt given in this wa\ is equal in effect to twenty grains given in the usual way. The method is worth trying in some cases, though it is not always successful. Spratling commends highly bromipin, especially in feeble or 220 THE NEURONIC DISEASES asthenic cases, in children, and for hypodermic use in status epilepticus- For ordinary administration he recommends the following formula : 1$ Bromipin giv. Syrup, simp. giv. Spts. menth. pip. 3iv. Mucil. acacise ad o xv J- Sig. — One to one and a half ounces, for adults, three times a day r after meals. It does not affect the gastro-intestinal canal ; does not produce acne;, and does not cause abscess when given hypodermically. Opium has long been tried in the treatment of epilepsy but it has not retained popular favor. Lately it has been used again but in combination. In 1893 Flechsig formulated a treatment with opium and the bromides. For six weeks mild opium narcosis is maintained. The drug is then suddenly withdrawn and the bromide substituted in doses of 120 to 130 grains daily, and continued for two months. The latter is then gradually reduced and maintained at about 30 grains a day. The plan is of doubtful efficacy and its dangers are not insignifi- cant. In my own experience, though limited, it was not superior to- the use of the bromides alone. After the bromides there are two remedies about equally divided in popular favor. They are atropine and borax. Either one may be given in combination with the bromides. Small doses are to be em- ployed in the beginning, and gradually increased until tolerance is ob- tained. Both may be continued for a long time. The extract of bella- donna may be used instead of the alkaloid. The conjunctivitis and eczema that are liable to occur after the prolonged use of borax are best combated with arsenic. Its cumulative tendency seems to give rise to persistent attacks of furunculosis and this militates somewhat against its use. The bromides have also been used in conjunction with antipyrin, digitalis, adonis vernalis and chloral hydrate. Ethylene bromide in doses of one or two minims two or three times a day, in emulsion or capsule, seems to have been effective in a number of cases in controlling the fits. Amylene hydrate, in from a half to two dram doses daily, was extensively tried by Weber. Only five per cent, of the cases responded favorably to it, and these were disturbed in general health and made drowsy, sluggish and dyspeptic by it. It may sometimes act better than chloral in cases of status epilepticus. Nitroglycerin in minute, frequently repeated doses may act happily in senile epilepsy where there is marked arteriosclerosis. Zinc is a historical but not much used remedy nowadays. Ure- thane in doses of twenty to forty-rive grains has been commended by Jacobi and Spratling. Chloretone acts somewhat like the Flechsig treatment. According to Weeks, it should be pushed as rapidly as possible. About six grains three times a day is the proper dose for THE NEURONIC DISEASES 221 prolonged usage. Solanum carolinense (fluid extract, o l / 2 to 5j t. i. d.), and simulo (tincture 5ij to oiij t. i. d.), have had their advocates. They are useful sometimes when the bromides cannot be tolerated, are com- paratively harmless and are therefore always open to trial. Trional has been tested and found to be useless. The coal tar derivatives, anti- pyrine, phenacetine and acetanilid, affect the heart and fail to affect the epilepsy. In a small proportion of cases continuous chloroform narcosis may be a dernier ressort. It cannot be relied upon, however. The employment of electricity, hydrctherapeutics and other general meas- ures, except for their general constitutional effect, shows a want of appreciation of the true nature of epilepsy. The valerianates, aniline sulphate, silver nitrate, asafcetida, calabar bean, camphor, cannabis indica, chloralamid, conium, copper-am- monio-sulphate. duboisine, gaduol, hyoscyamine, ignatia, strychnia. lobelia, paraldehyde, phosphorus, physostigma, picrotoxin, rue, san- tonin, sodium fluoride, spermine, stramonium tincture, sulphonal. sumbul and many others constitute a list of remedies that may be tried when the usual medicines fail. These will doubtless fail, too, but their administration may be useful for the constitutional, tonic or psychic effect while other more general measures are being put into force. Bacterial products, serum therapy and other measures to produce immunity and to combat the toxaemia are being experimented with by those who believe in the intoxication and autointoxication theory of the disease. So far the results have not been very encouraging. Surgery for the relief of epilepsy is more especially applicable in so-called symptomatic epilepsy. Cranial depressions, spicuke of bone, old cicatrices, cortical cysts, meningeal thickenings and other cranial and cerebral abnormalities should always lead to the question of trephining. The draining of an abscess may put a stop to the disease. I have seen the fits stopped for a long time by the simple operation of trephining in a case of multiple abscess discovered during the operation. According to Sachs and Gerster symptomatic epilepsies that have existed for more than three years do not offer a very promising out- look from operative procedure. The more recent the injury the better the prospect for ultimate recovery from surgery. Surgical measures are often called for in the removal of some peripheral source of irritation that may be keeping up a reflex type of the disease. I have elsewhere stated that all such excitants must be removed, if possible, in even' case. The use of the seton in the nuchal region, the ligation of the carotid and vertebral arteries and the extirpation of the sympathetic ganglia, are among the historical and curious relics of the surgical treatment of epilepsy. HEMICRANIA. MIGRAINE. This is a paroxysmal neurosis, periodical and epileptiform in char- acter, in which headache and other special sensory symptoms constitute the prominent feature. If it were a mere headache, its classification 222 THE NEURONIC DISEASES would fall in the following section and be subject to the same criticism made there in regard to all forms of cephalalgia. Some authorities regard it as a special disease entity. -In deference to their views and on account of the uncertainty of our knowledge as to its exact pathology,, it may be wise for awhile yet to devote to its discussion a special chap- ter. I place it here, however, because, as I will show later on, I believe it is most intimately related to epilepsy, is possibly a sensory epileptic equivalent of a special type and should be considered under the general head of epilepsy. Etiology. — No fact is more patent in the etiology of migraine than that it is an inherited trouble. The inheritance may run through several generations and be either direct or indirect. How frequently we are told by the victims of this disease that their father or grandfather suf- fered from similar attacks of headache ! They often volunteer the in- formation that they have a brother or a sister who is also afflicted with it. The maternal ancestry may also have been subject to it, though it has seemed to me that direct heredity was less prominent on the maternal than on the paternal side. Gowers asserts that in more than half the cases distinct heredity can be traced and that usually it is direct. An indirect inheritance is not uncommon and usually consists of a strong neuropathic taint. This may come from either side of the house and may have sprung out of ancestral dissipation, excessive mental work or some related nervous disease. Epilepsy, hysteria, mild psycho- ses, neuralgia, gout, asthma, paroxysmal cardiac troubles are among the diseases noted in the family history. Given this inherited neuropathic constitution, it is obvious how the exciting causes of migraine may be both numerous and varied. All unwonted strains, whether mental, nervous or physical, may start up an attack. A great emotion, fright, prolonged mental labor, anxiety and worry will cause it. One of the worst sufferers I ever treated was a young girl, whose father, now dead, had had the disease, who was attempting to finish a college course during the absence of her lover to whom she expected soon to be married, and with the con- tinual presence of her mother, who was an ignorant, self-opinionated, nagging woman. Only the strictest seclusion in a hospital for a time saved the girl from what almost threatened to be complete mental breakdown. Peripheral reflex excitants may provoke an exacerbation of the disease. Masturbation, coitus, hypertrophy of the nasal mucous mem- brane and working in overheated rooms have been blamed for it. Autointoxication, with or without obstipation, is believed by many to be a most important factor in the etiology. Peripheral diseases and deformities may well play a role in exciting it. Eye-strain in one predisposed to the trouble is just as much a cause, no more, no less, than might be disease of the sexual organs. Anaemia, overlactation and other depressing influences will aggravate it. It has occurred in connection with diabetes and chronic nephritis. The similarity between the etiology of migraine and that of epilepsy is very impressive. It is an inherited neuropathic condition, a high degree of nervous instability upon which an innumerable list of ex- THE NEURONIC DISEASES 223 citants, chemical, physical and functional, operates to provoke an exacerbation or paroxysm. Weir Mitchell has lately compared the atmospheric storm curve with the headache curve and finds a relationship. March and April are the worst months, October and February the best. Pathology axd Pathogenesis. — There are no discoverable char- acteristic anatomical changes in hemicrania. This fact stands as an unanswerable argument against all theories in which this neurosis is attributed always and solely to a reflex irritation. If hemicrania is due to eye-strain, then eye-strain must always be found, with the ac- companying anatomical defect, in all cases of the disease. As a matter of fact, there is no pathology known. A neuropathic condition of the central nervous system is assumed on account of the heredity and other suggestive phenomena, while the exciting anatomical and physiological causes of the outbreaks of the disease are both obvi- ous and numerous. \\ "here so little is known, theorizing is always rife. Of all the pathogenetic explanations of the disease, two only are worthy of special consideration. The first is that hemicrania is but a form of epilepsy ; the other is that it is the expression of a vasomotor disturbance due to disease of the sympathetic nervous apparatus. It seems to me that if any reliance is to be placed upon compari- sons at all, the etiology, symptoms, course, occasional complications and other manifestations associated with migraine point most clearly to its relationship with epilepsy. The heredity in the disease, the de- pendence of the outbreaks upon exciting causes, the periodicity that occurs in these outbreaks, their onset, progression, sudden termination and sequelae are so strongly epileptiform that if they are not epilepsy, no two phenomena ever came so near being alike while yet being unlike. The only difference between epilepsy and hemicrania is that the former is characterized by prominent motor symptoms, while the latter is almost wholly sensory. This, however, is only an apparent and not a real difference. In epilepsy of the petit mal and psychic type motor symptoms are often wanting, while in hemicrania it sometimes happens that slight motor phenomena can be observed. To be sure, the mental disturbance is the cardinal feature in all epilepsies ; but in hemicrania the mind is very often seriously affected. This is seen in the amnesia, aboulia, aphasia, irritability, want of attention and stupor, which, however, never or very rarely passes into the complete unconsciousness observed in epileptic fits. The sensory character of migraine is, if anything", confirmatory in part of its epileptic nature in view of the more recent conception of the pathogenesis of epilepsy. The latter is by most modern pathologists now believed to be primarily a sensory disease. The sensory elements of the cortex have been found to show certain chromatolytic changes. A toxic degenerative condition attacking these sensory elements causes them to manifest the early signs, aura, etc., of the fit. Their close connection with the motor elements is the explanation of the severe secondary spasms and convulsions. The 224 THE NEURONIC DISEASES severity and wide extent of the nerve storm accounts for the profound coma. In hemicrania a somewhat similar succession of events takes place, but milder in degree. There are the sensory prodromic intimations, then the nervous explosion, which, however, does not overflow into the motor elements to the same extent that it does in grand mal, and finally the sudden cessation with more or less lingering sequelae. The outward exhibition of the two diseases being thus so much alike, it is but fair to assume that there is a similarity in the underlying pathology, whatever that may ultimately prove to be. In epilepsy the process is generally accepted to be located in the cortex. In mi- graine it is probably cortical also. On account of the prominence of the headache and of the gastric symptoms, some hold that the maximum foci of the disturbance are localized in the primary centers or ganglia of the trigeminal and pneumogastric nerves. This may be true, but on account of the eye symptoms, mental disturbances, aphasia and occasional motor phe- nomena we must still believe that the neurosis is in large part, if not wholly, a cortical trouble. One way, and perhaps the best way for the present, to express the relationship between the two diseases is to say that migraine is an epileptic equivalent. Under the head of epilepsy will be found a discussion of these so-called equivalents. Of all the explanations of migraine, the one that allies it to epilepsy is to-day the most generally accepted. It has the support of Liveing, Gowers and many others. The other popular theory of the disease is that a spasm of the cerebral arteries causes it. Whytt and Du Bois Reymond were the leading sponsors of this explanation. Mollendorf and others, on the other hand, urged that vascular dilatation rather than contraction was the basis of the trouble. Many writers, like Latham and Eulenburg, think that both spasm and dilatation of the blood vessels are the cause of the disease, and, with most followers of this theory, assign the pri- mary cause of the vascular trouble to the sympathetic nervous system. Latham thinks that the early symptoms are due to vascular contrac- tion, the later, and especially the headache, to dilatation. Eulenburg and others make two leading types of the disease, which they have denominated the sympathetico-tonic and sympathetico-paralvtic re- spectively. The weakness of this vascular theory is that the basis for it con- sists in the mere appearance of certain accessible arteries during an attack. It assumes that the same thing is occurring in the intracranial that is observed in the extracranial circulation ; that this is a cause rather than an effect of the disease, and that it is dependent upon some hypothetical change in the sympathetic nervous apparatus. In addition to assuming so much, the theory is not founded upon a single post mortem finding that would lend it color or support. Furthermore, it does not account for the remarkable similarity between the general e>> THE NEURONIC DISEASES 225 symptomatology and etiology of migraine and epilepsy, unless it is likewise adopted as the explanation of the latter disease. It seems to me clear that as migraine is so closely related to epi- lepsy, all theories that are advanced to explain the former disease must be employed to explain the latter. This the vascular theory does not do, and it is therefore inadequate to explain the nature of migraine. Migraine is a primary neurosis, of which the sudden vascular derangement is one of the manifestations. The exact primary seat of the neurotic storm is not yet determined, though most of the indications point to the sensory centers of the cerebral cortex. Symptoms. — Very many cases of migraine have premonitions of an oncoming attack. In others the onset is sudden and abrupt, some- times awakening the patient out of his sleep at night. The premoni- tory symptoms are not unlike the premonitory signs and aura that precede an epileptic fit. For a day or two, or several hours, the patient will feel tired, listless and indifferent. Somnolence and a fullness of the head are complained of. Sometimes there is unusual mental bril- liancy, with a surprising activity of memory, imagination and intel- lection. As a rule, however, the apathetic condition, with yawning inability to fix the attention, vertigo and depression obtains. An ab normal hunger or indifference to food, with slight feeling of nausea and cardiac palpitation, have been spoken of by patients before the onset of the headache. Immediately before an attack other patients experience visual disturbance, as, for instance, spots flying before the eyes, flashes of color, rainbow formations, star-like fortification figures, hallucina- tions of animals and strange creatures. Sometimes the visual field slowly increases or decreases, grows dark or light, becomes dark in segments, with transient hemianopsia and scotoma. A blurring of the vision more frequently occurs. Hippus or alternate contraction and dilatation of the pupil has been seen. The other special senses are only rarely subject to these aura-. Tinnitus aurium, parosmia, numbness of the mucous membrane of the mouth, have been noted. Paraesthesia and, more rarely, cramps and spasms have appeare I in the upper extremities just before an attack. Transient aphasia, more particularly of the amnesic type, has sometimes been premonitory of the headache. When these sensory premonitory manifestations arc marked, it its that the succeeding headache is less severe and less definitely localized than when they are slight or absent entirely. There is absolute rule about this, however. Some patients have actually had these aurse without the appearance of the headache at all. An attack of migraine or paroxysm of the disease presents a cer- tain constant clinical picture, the details of which are subject to much modification. Soon after the appearance of the premonitory symptoms, and letimes even without them, a headache occurs abruptly or gradually, lily increases to a climax, where it remain- several hours, accom- panied by certain sensory, motor and psychic manifestations, and then 226 THE NEURONIC DISEASES rapidly disappears with usually an attack of vomiting, leaving the patient exhausted, sleepy and depressed. It is to be noted that the attacks are more or less periodical and paroxysmal ; that they pretty closely resemble one another always in the same patient ; and that they are separated by intervals of apparent perfect health. The headache is their prominent, but not their only, symptom. They are often provoked by overexertion, indiscretions in eating, mental excitement and unusual use of the eyes. Sometimes they come apparently unprovoked. Paroxysmal headache with vomiting, visual disturbances and other less constant symptoms, appearing rather abruptly, rising to a maxi- mum of intensity, remaining there a certain number of hours, and then abruptly vanishing, constitutes the clinical picture, in brief, of an attack of migraine. As the minutiae of this picture are subject to much variation, I will now discuss them a little more in detail. Though it has given the name to the disease because it is so constant and prominent, the headache is not by any means its sole or most important symptom. In rare instances it is entirely absent, and in all cases the patient has become so accustomed to it that though it causes him the most discomfort, it does not alarm him so much as some of the sensory, motor and psychic phenomena that accompany it. This is to be remembered both on account of the proper concep- tion of the disease and its proper management. Hitherto in the profession, and even yet to a large extent among the laity, the headache has been regarded as the cardinal feature of the disease, and in a way the cause of all its other manifestations. The disappearance of the cephalalgia synchronously with an at- tack of vomiting and vertigo has fixed the impression in the minds of the people that the liver is somehow the cause of the headache, and that if the vomiting of bile can only be induced, the headache must of necessity vanish. They, therefore, commonly speak of migraine as "bilious sick headache," totally ignorant of the fact that both the head- ache and the so-called bilious manifestations are synchronous phenomena depending upon the same neurotic process. In a general way it may be said that the more pronounced the cephalalgia is the less obvious will be the other special sensory and motor symptoms. When the latter are marked, the headache is apt to be of a minor degree of severity. The character of migrainous headache is quite distinctive. As I have intimated, it appears rather suddenly, but develops gradually. Sometimes it bursts forth in all its excruciating intensity at the very beginning. It does not fluctuate, but continues steadily, after it has reached its climax, for a day usually — sometimes only a few hours, at other times for two or three days — and then rapidly vanishes. It is generally a unilateral headache, the seat of its maximum intensity be- ing in the temporal region. After remaining awhile in one temple it may cease, only to reappear in the other temple. It may extend from one temple over the front of the brow or across the top of the head to THE NEURONIC DISEASES 22^ the other temple. Not infrequently the pain starts in the occipital region, extends forward to the supraorbital region, or down the neck, even into the arm. In the same patient it usually occupies the same locality and extends in the same general direction with each succeed- ing attack. Occasionally it always appears in one way during a cer- tain period of life, and in another way during another period. Its localization, steadiness, intensity and paroxysmal character are among its striking qualities. The pain itself is of a burning, boring sort ; more often just a dull, incessant, sickening ache. At first it may seem to be superficial, but later on it feels deep in the cranium. Percussion aggravates it, as well as light, noise, or any peripheral irritation. Mere pressure does not increase it, but rather affords some relief. Movement, jars and other slight disturbances seem to awaken it to renewed intensity. The patient therefore instinctively seeks a couch in a dark and silent room and is irritable with those who disturb him even with their good offices. The skin of the head may be hypersesthetic, and while the pain is on al! the nerves of the body, but especially the fifth nerve, have their sensibility highly exalted. Hence the patient is in a wretched state, limp, irritable, excited or stuporous, and indifferent to those about him and their attentions. Frequent experiences have taught many of the victims of this disease to bear it in patience and to await in seclusion its early termination. There are all degrees of the headache, though as a symptom it is more or less always present. It may continue to be the chief mani- festation of the disease throughout life, or it may suddenly cease, to be replaced by some other neurosis. Two sisters whom I saw suffered all their lives with asthmatic attacks, except one of them who twelve years ago began having attacks of migraine in place of those of the asthmatic neurosis. It sometimes happens that the headaches stop and epileptic seiz- ures begin. I have seen the headaches alternate with hysterical outbreaks, and other spasmodic conditions. The headaches never occur alone, a fact which more than any- thing else indicates the protean and extensive nature of the disease. Next in frequency to the headache, and usually accompanying it, arc the gastric phenomena. These consist of anorexia, acute indie tion, nausea, retching and vomiting. That not one of these is in direct relation to the headache is shown by the fact that the latter is in no way modified by them when they occur early in its course. Distress in the stomach may precede the head pain, occur when it is at its height, or appear only when the latter is beginning to wane. Usually then some anorexia and nausea from the beginning of the attack, and then it the end violent retching and even vomiting of bile accompanies the sation of the headache. If food is taken it fails to digest and comes up with the retching. This symptom causes great distress and prostra- tion sometimes and is generally the terminal manifestation of the par- oxysm. Sleep and quietude follow it. 228 THE NEURONIC DISEASES In some cases there may be diarrhoea and polyuria towards the end of the attack. In about half of the cases there is some visual disturbance. This assumes a great variety of forms and usually indicates trouble in the occipital cortex. When both eyes are affected, the phenomenon usually occurs in similar parts of the visual fields. Complete or partial hemi- anopsia may occur or the vision in one or both eyes may be blurred. Flashes of light, star formations, highly colored zigzag lines or fortifi- cation figures are not uncommon. Actual hallucinations of animals have been observed. Moving phenomena are particularly frequent. Spots float across the field of vision, objects approach and recede, diminish and grow large, or become alternately brilliantly luminous and dark. Central scotoma ta have been seen. In rare instances double vision has occurred. All of these manifestations may appear abruptly or come on gradually ; they may be more or less constant, though as a rule they come and go or change from one to the other in the course of the attack. The functional character of these troubles and their dependence upon the higher or cortical visual centers are obvious. With the headache and stomach troubles they complete the tripod that most frequently makes up the clinical picture of an attack of migraine. The other symptoms to be mentioned occur much more irregularly and in- frequently than these three. General sensation is affected in many patients in such a way as to clearly indicate a cortical functional trouble. Unilateral or bilateral paresthesia and even anaesthesia may occur in' the arms, rarely in the legs, in the face, mouth, throat and neighboring parts. Numbness, tingling, pins-and-needles, a feeling as of water flowing over the skin, hot and cold, are the more common cutaneous disturbances complained of. As with the aurae of epilepsy, these sensations sometimes travel up the arms in gradation or in skips, and then appear in the foot and travel up the leg. They rarely last longer than a few minutes. The tongue and fauces sometimes feel numb and may become actually anaesthetic. Paraesthesia of the limbs usually precedes in time paraes- thesia of the face and lips. When unilateral it is of the hemiplegic type, involving the same side in the face and limbs. These cutaneous disturbances are far less frequent than those of the sight. In very rare instances the other special senses have been affected. Tinnitus aurium, parosmia and alterations of taste are possible, but not common. Transient deafness in one ear, hallucinations of hearing and taste and other bizarre observations have been recorded. The motor symptoms are never prominent and are usually entire- ly unnoticeable. Slight weakness of the hemiplegic type may be ob- served in the limbs, or they may exhibit a certain degree of spasm. These motor manifestations, when present, appear on the opposite side from that of the headache. They are always associated with sensory symptoms and are exceedingly transient in duration. Cerebellar inco- ordination was observed in one case. The vasomotor symptoms are much more commonly observed. THE NEURONIC DISEASES 220, Usually there is an initial spasm of the facial blood vessels with pallor. This may continue through the attack. In other cases it is replaced by a congested and flushed condition. Pupillary changes, unilateral sweat- ing, oedema, redness of the conjunctives and retardation of the pulse are some of the occasional observations. These phenomena are usually bilateral, though they may be one-sided. They point clearly to implica- tion of the sympathetic nerves, but are neither constant enough nor sufficiently indicative of the intracranial circulation to found a theory of the disease upon the sympathetic nerve, as some have attempted to do. Aphasia of the amnesic variety, more rarely of the motor, is a symptom liable to appear. It is rarely complete and in a case observed by Oppenheim was combined with agraphia. Hemianopsia may be associated with the aphasia. Word-deafness, aphasia, etc., are seen in only a small percentage of cases in which the cephalalgia is left- sided. Giddiness and vertigo, with loss of equilibrium and nausea, are sometimes present. More or less psychosis occurs in all of the cases and it is not en- tirely due to the pain and distress experienced by the patient. It is a part of the paroxysmal phenomena. There may be slight excitement, irritability, intellectual brilliancy just before the onset. More often there is emotional depression, weakness of memory, lack of power of attention. There follows in some cases actual stupor or confusion, or transitory insanity with marked hallucinations. There is never com- plete unconsciousness, but a state of double consciousness has been seen. The memory may recall far-off events with extraordinary vividness. The general prostration caused by an attack of migraine is severe. Not only during the spell, but for some time after it, the patient is dull, apathetic and somnolent. At the climax of the paroxysm some- thing seems to suddenly give way. Vomiting suddenly occurs, the headache rapidly dies out, ptyalism, perspiration, profuse micturition occur and the patient passes into a profound sleep, from which he usually awakens some hours later refreshed and feeling quite well again. It is useless to attempt to establish types of the disease out of the many peculiar forms and combinations that the symptoms assume in different cases. The disease is always the same and mere prominence of this or that symptom does not alter it. In the larger number of cases there are present only cephalalgia and the gastric disturbance. When the disease occurs in children there may be some elevation of temperature, even to 102 or 103 degrees F. The sensory and other manifestations of the disease may rarely occur without the hemicrania. Eye symptoms, transient aphasia, paresthesia in the limbs may thus appear alone or in alternate attacks with the headache. Gowers cites a case in which for years the only symptom was a visual disturbance like that which usually precedes an attack of headache. At no time was there any pain. These ca 230 THE NEURONIC DISEASES are exceedingly rare, but should be carefully studied and properly recognized. Some of these cases may belong to the class of hemicrani-al equiva- lents spoken of by Mobius. In this it is assumed that the disease is epileptiform and has equivalents just as ordinary epilepsy has. Among other equivalents noted are cardialgia, severe vertigo, gastroxynsis, circumscribed pains of a severe character in other parts of the body than the head, and attacks of hemiparesis. Temporary mania, mental confusion, dream states and melancholia have been seen in place of a migrainous attack. One of my patients would lock herself in her room, refuse food for days and do other strange things that led her own family to declare that she was insane, and to suggest her confinement in an asylum. During several months' treatment under my care in a private hospital she showed no signs of insanity, but had several unusually severe outbursts of headache. Mobius has made use of the occasional cases of constant hemi- crania to support the epileptic theory of the disease, calling these cases instances of status hemicranicus and comparing them to status epi- lepticus. Among the affections sometimes associated with hemicrania epi- lepsy stands prominent. Epileptiform attacks may mingle with those of headache or the latter may cease and be replaced by typical epilepsy. Writer's cramp and other occupation neuroses, as well as convulsive tic have been seen conjointly with it. Nearly all cases are somewhat either neurasthenic or hysteric. Both of these latter diseases may be developed out of the prolonged strain and suffering caused by the hemicranial attacks. As a rule, however, they are the product of the same neuropathic constitution that underlies the migraine. The im- portance of this fact is shown when one mistakenly attempts to treat a migrainous headache under the erroneous notion that it is but a symptom of the neurasthenia or hystero-neurasthenia that may be present. The latter may improve, but the hemicrania will probably remain. Disappointment will be then the portion of both the patient and physician. Diagnosis. — In fairly typical cases the diagnosis of migraine is easy. The points to be carefully noted are the heredity back of the dis- ease, the provocation of the attack by some unwonted strain, the pre- monitory symptoms, the somewhat abrupt onset of the unilateral head- ache, dragging in its train more or less marked visual and gastric dis- turbances, other sensory symptoms and even certain motor and 'vas- omotor manifestations. The comparatively brief duration of the attacks, their somewhat periodical and paroxysmal character and their sudden termination with severe vomiting should be carefully taken into consideration also. The association of the trouble with other neuroses, especially epilepsy, is quite confirmatory. However, other headaches are sometimes unilateral, paroxysmal, combined with other symptoms and in many ways may be confused with hemicrania. In these headaches assistance in making the diagno- sis must often be obtained solely from the other symptoms of the THE NEURONIC DISEASES 231 disease underlying the headache. The cephalalgia that often accom- panies hysteria, neurasthenia, uraemic poisoning, eye-strain, constipa- tion, etc., can thus be easily distinguished aside from their own char- acteristic features. In tumor of the brain the appearance of the fundus of the eve, the comparatively constant presence of the pain, the absence of the prodromes and peculiar visual disturbances of migraine, and, when present, the localization symptoms, will help to establish the diagnosis. The pulse may be retarded in migraine as it is in cerebral neoplasm, but in the former disease it is an exceptional symptom, while in the latter it is a common manifestation. On account of the elevation of the temperature migraine in child- hood may be mistaken for meningitis and other brain diseases. The paroxysmal character of the headache, the ancestral history and the absence of the usual objective signs that accompany these diseases will guide the diagnosis in the right direction. Petit inal may be overlooked as a case of hemicrania. The dis- tinction is sometimes a difficult one to make on account of the appar- ently close relationship between the two diseases. In ' epilepsy the visual aurae are not as prolonged as they are in hemicrania, the psychic manifestations are somewhat more prominent and the sensory symptoms are less noticeable. If the slightest convulsion occurs the diagnosis of epilepsy is of course assured. Tic douloureux appears in a particular nerve, the fifth, and has not the aurae, the eye symptoms, the general sensory manifestations of the hemicrania. Prognosis. — As inheritance is so important a factor in its etiology, migraine must be looked upon largely as of the nature of a congenital defect. This, of course, precludes the possibility of its complete eradi- cation. And yet it may cease suddenly at any time, though it is then likely to be replaced by some other form of neurosis or even psychosis. Most cases, however, continue having their paroxysms during all their lives. Cases that I have seen change have always been about the climacteric. In several instances I have seen migraine replace some previous neurosis about this period. The longer the disease has existed the smaller is the hope of its disappearance or even amelioration. The latter in many cases can be brought about by changing the patient's mode of life, environment and removing all possible causes of peripheral irritation. Hemicrania is practically incurable. The type of the disease in which the visual phenomena are especially prominent sometimes pre- cedes tabes dorsalis and dementia paralytica. When it is associated with hysteria, hystero-neurasthenia and other grave neuroses, especially epilepsy, the outlook is very unfavorable. Amelioration and the remote- possibility of a spontaneous cessation is about the besl sort of comfort we can hold out to these patients. The disease is decidedly chronic and in no way jeopardizes life, h- greatest danger, though that is not frequent, is its transition into epilepsy or insanity. 232 THE NEURONIC DISEASES Treatment. — In undertaking the treatment of a case of hemi- crania, the first requisite is to remove every discoverable excitant of an attack. Many patients have discovered this fact for themselves and when presenting themselves to the physician for advice declare that they have carefully avoided all habits and acts that have initiated their attacks. Nevertheless it is always well to interrogate the patient closely in regard to his environment, his occupation, his dietary, his custom in regard to sleeping, bathing, exercising, in regard to his sexual life, his bowel functions and his brain work. Not a stone should be left unturned in this search. Proper corrections, of course, should be made where errors and excesses are found in the habits and manner of living. As migraine is in many cases a reflex disease, the patient's body should be thoroughly examined for physical and functional defects. The eyes may be defective in one case ; the nasal passages obstructed in another. An enlarged tonsil or a mastoiditis may be causing the trouble. Anaemia may be present, or there may be some latent renal disease. The very multiplicity of the exciting causes of migraine, and their elusive character in so many of the cases, urge upon the diagnostician unusual care and thoroughness in his search for them. Their removal, of course, is a sine qua non to all further treatment of the case. When this is done many cases will undergo marked amelioration and some will be absolutely cured. As the neuropathic instability frequently continues in such cases, other existing causes may arise and provoke the attacks anew. Those who vainly attribute migraine to these peripheral causes alone do not have a proper conception of the disease and are disappointed when there is a return of the headaches in some of the cases in which the supposed existing cause has been carefully removed. All discoverable causes having thus been removed as far as possi- ble, there should be an effort made to combat the disease by continuous, prolonged, constitutional treatment. The dietary should be regulated and rendered as bland and nour- ishing as possible. A purely vegetarian diet has modified some cases. Stimulants, condiments, fats and rich pastry had better be avoided. Tea and coffee are sometimes harmful. Milk diet is commendable, though a large percentage of these cases declare they cannot take milk. I have found this to be due largely to the psychic impression that biliousness, which they think they suffer from in their attacks, is somehow associated with the ingestion of milk. I nevertheless in- sist upon their taking the milk, in small, frequent quantities, and have more than once overcome the prejudice. I urge these patients to drink abundantly of pure water at all times. As constipation is not uncommon, some natural water with cathartic properties, like Carlsbad, Hunyadi, Friedreichshalle, is to be used more or less regularly before breakfast. The general health should be built up if necessary. Arsenic and iron and other tonics should be given if the state of the blood and organs calls for them. Hydrotherapy, mechanicotherapy, massage, gymnastics and electricity may all be advantageously employed with this end in view. A change of climate and a sojourn in the mountains, or by the seashore, may THE NEURONIC DISEASES 233 so alter the general state of ill-health as to cause a marked ameliora- tion in the migrainous attacks. For the disease of hemicrania, like epilepsy, we have no specific medication. The prolonged administration of some remedies, however, has resulted in a number of complete and partial successes. Of these remedies arsenic, nitroglycerin and the bromides head the list. In my own experience arsenic has seemed to be the most successful. It very often fails, however. Any form of arsenic may be used, though arseni- ous acid is perhaps the most available. Fowler's solution and arseniat- ed waters may be used alternately. It must be continued for a long time, with proper precautions and intervals of rest, and in full physio- logical dosage. Iron may be advantageously combined with it in some cases. I have not been so successful with nitroglycerin. Gowers praises this remedy highly. It is to be given for long, continuous periods in doses of 1-200 to 1- 1 50 of a grain three times a day, after meals. Dur- ing the paroxysms it should be stopped. It is in the angiospastic type of the disease that it exerts its happiest effects, accomplishing it, as Gowers says, by regularly flushing the brain with new blood and so increasing its general nutrition. The continuous use of the bromides has helped some cases, but as a rule they are not satisfactory remedies. In spite of all we can do in most cases the headaches persist in returning. In such instances all we can attempt to do is to mitigate the patient's sufferings when the attack is on, or is about to begin. Many patients have become so discouraged at the futility of all medication that they refuse to be treated, seek a dark, quiet room, and await patiently the cessation of the paroxysm. Nitrite of amyl inhala- tions may be tried in the beginning of an attack. A cup of strong coffee or twenty drops of chloroform has checked it somewhat. Herter rec- ommends that the stomach be washed out with water at 105 degrees F., and a brisk saline cathartic be administered. The patient should keep a recumbent position in bed and a firm hot or cold compress applied to the head, and his feet placed in hot mustard water. Menthol may be rubbed over the temporal or frontal regions ; instillations of cocaine may be made into the conjunctival sac ; and a mustard poultice applied to the back of the neck. Electricity does no good and morphine must be withheld as long as possible. The drugs that are employed to combat the headache are numer- ous. In their effects they are very variable. What may be most suc- cessful in one case, or in one paroxysm, may fail utterly in another. Hence it is wise to have in hand a number of agents to experiment with. In most cases a thirty or forty grain dose of potassium bromide, sometimes with tincture of cannabis indica or tincture of hyoscyamus, repeated every two or three hours, gives considerable relief. Chloral produces sleep, but after this wears off the pain comes hack in all its pristine severity. Even morphine sometimes acts this way. The coal- tar preparations, antipyrine, acetanilid and phenacetine. enjoy an un- deserved popularity. In large doses they are apt t<> be dangerously depressing, and in small doses they are not rapidly effective. In mild 234 THE NEURONIC DISEASES cases, however, I have gotten some results from all three, administered in small, frequently repeated doses. Personally I prefer phenacetine, giving twenty or thirty grains, in five-grain doses, every quarter or half hour. While taking this remedy the patient should lie down and take some brandy or aromatic spirits of ammonia. A few grains of caffeine with potassium bromide makes an excellent combination for some cases. Sodium salicylate in thirty or forty grains, administered in a cup of strong coffee, sometimes suppresses pain. Caffeine may be combined judiciously with it. Guarana (paullinia sorbilis) is similar to and not much better than caffeine. It may be given in twenty or thirty grain doses, repeated every half hour for three or four times. The dosage of the alkaloid, guaranine, is one to five grains. Other medicaments worthy of a trial sometimes, and always use- ful as a reserve force when the others fail, are analgen (5-15 grains), exalgen (4 grains), methylene blue {V/2 grains), cytisin (1-20 grain), migranin (16 grains), cannabis indica, nux vomica, ergot, ammonium chloride, neurodin (15-25 grains), quinine valerianate, thermodin (15- 20 grains), triphenin (15-20 grains), valerian, aconite and belladonna. The drug treatment of migraine is far from being all that could be desired. If cannabis indica in full dosage, the bromides with hyoscyamus, sodium salicylate, the coal-tar preparations and caffeine fail, there is not much to be hoped for from anything else. Hence it is best to persist, even in the face of possible ultimate failure, with these than to rapidly change from one drug to another of this long list of uncertain and often inert preparations. As a last resort hypodermics of morphine may be turned to, though occasionally even these fail, and in doing so may save the patient from the greater danger of the drug habit. The seton has seemed almost to have produced a cure in a few cases. It is inserted in the back of the neck, the tape is changed fortnightly and the wound is dressed daily with one boric fomentation. Watson, Cornish and others commend the treatment in obstinate cases. It prob- ably acts by counterirritation. HEADACHE. CEPHALALGIA. CEPHAL^A. Headache is only a symptom accompanying many diseased states and the term ought to be regarded in the same way that paralysis and apoplexy are. As it is so prominent and frequent a symptom, as it is so often the only symptom of which the patient complains, and as its cause in a certain percentage of cases is quite hidden, it rises almost to the dignity of being a special disease, and so perhaps in a work on nervous troubles deserves a special section. In a book covering the entire field of medicine there would be no need of a special discussion of headache, for anything like complete consideration of it would include a discussion of the whole field of medicine. Not every pain about the head is included under the specific term headache. By it is meant a more or less diffuse soreness and pain in and about the vault of the head. Facial pains and pains following the THE NEURONIC DISEASES 2^ course and distribution of particular nerves are regarded as neuralgic. The laity sometimes use the term "neuralgia'' when referring to head- ache, but for the sake of clearness the above distinction between the terms should, for the present, be insisted upon by the profession. Headaches are pains of variable intensity, extent and duration that occur in the frontal, occipital, temporal and vertex regions of the cranium. Various classifications have been made upon the basis of location, cause, character and relationship to other diseases. All of these classi- fications are of some practical value ; none of them are complete or entirely satisfactory. The best are undoubtedly those in which the causes of the headache, considered on broad lines, are made the foundation. Etiology. — As I have said, a discussion of the causation of head- ache would cover the whole field of medicine. There are certain prominent factors, however, that may be profitably dwelt upon. Neurotics and people in a condition of nervous depression furnish the larger number of victims ; hence heredity, in the form of neuropathic transmission, plays a role. Age, occupation, environment, habits, dis- sipations, mode of life, all enter into the etiology in so far as they cause nervous inadequacy. Including migraine in his statistics, Dana finds that headache attacks ten to fifteen per cent, of school children, over fifty per cent, of women, and about twenty-five per cent, of men. The age at which the special varieties of headache make their ap- pearance depends upon the underlying cause. Early adult life for both sexes seems to be the favorite period of the disease. The com- petition and strain of urban life ; the dissipation and self-indulgence of wealth ; the mental overexertion in bad hygienic surroundings ; the barometric and climatic changes of various seasons and localities, are all elements in determining the frequency of headache. From the standpoint of etiology the following types of headache may be noted : I. Hcemic and Vasomotor. The brain being so abundantly sup- plied by blood, as the ultimate seat of consciousness and all painful sensations, it would naturally be expected to be affected when the integrity of the blood or the amount of its supply is any way disturbed. Anaemic and congestive headaches are therefore very real and not at all uncommon. The former may be associated with chlorosis, sudden and severe hemorrhage or the deteriorated condition following a long, exhausting disease. Arteriosclerosis may cause a local anaemia with nutritive depression, though Oppenheim suggests that the rigid vessels may irritate the meninges. Both active and passive hyperaemia will raise the intracranial blood pressure and thus provoke a cephalalgia. It may be arterial and caused by sudden emotional excitement, toxicity of the blood from alcohol, tobacco, coffee and traumatism; or, on the other hand, it may be venous and associated with cardiac, renal and pulmonary disease. Faulty position and tight constriction of the neck- may impede the circulation. The nutritive value of the blood ma) be greatly diminished by the presence of infectious material. Thus under the class of haemic headaches we must enumerate all those thai are 236 THE NEURONIC DISEASES associated with such diathetic conditions as gout, rheumatism, diabetes,. uraemia; such infectious fevers as malaria, influenza, typhoid; and such forms of autointoxication as have their origin in gastric dis- orders, constipation, hepatic and renal insufficiency, etc. Thus mechan- ically, nutritively and physiochemically are these haemic and vasomotor headaches produced. 2. Toxic. In a smaller number of cases certain poisons probably act as direct irritants upon the pain mechanism, causing minute func- tional disturbances in the nervous elements that, if continued long- enough, may go on to actual neurotic degeneration. In these intoxica- tions the effect of the poisons on the histological elements is probably partly direct and primary and partly indirect and secondary by way of the vasomotor disturbance which they set up. An alcoholic headache, for instance, may be both a purely toxic and a secondary haemic head- ache. Among the numerous toxic agents which we regard as of etio- logical importance may be noted alcohol, tobacco, coffee, lead, mor- phine, chloroform, ether and others. Foul air in badly heated rooms or from defective plumbing, impure water and tainted food may be dan- gerous on account of their toxicity as well as by reason of depriving the organism of its normal needs. 3. Reflex. The reflex causes of headache are numerous and have furnished the ground for many a sharp battle of opinion. It is- perfectly reasonable that peripheral abnormalities should at times pro- voke central disturbances, but it is absolutely illogical to affirm that all central disturbances are due to such peripheral conditions. Through its outstretching processes, the seat of consciousness is in close relationship with every tissue and organ of the body. It may well happen, therefore, that disease of any one of these outlying struc- tures may be such as to set up a high degree of distress in the pain- centers of the brain. To enumerate the sources of reflex irritation, one would have to give a complete resume of anatomy, physiology and pathology. Suffice it to say that they are most commonly found in connection with the eyes, the stomach, the sexual organs, the nose and. the ear. Errors of refraction and accommodation ; naso-pharyngeal hypertrophies, tumors and inflammations; sexual excesses, masturba- tion, uterine diseases and menstrual disorders ; indigestion, gastric catarrh, intestinal parasites, etc., are among the more common periph- eral reflex causes of cephalalgia. 4. Neuropathic. Under this head we place those headaches that accompany epilepsy, neurasthenia, hysteria, neuritis, neuralgia, chorea, etc. These partake of the etiology of the disease with which they are associated. In my opinion most of them are toxic or autotoxic in origin, and not a direct expression of the neuropathy in the way that the general neurosis is. For example, neurasthenia is a general neu- rotic deterioration; the headache that accompanies it is usually the result of the auto-intoxication that develops from the functional in- efficiency of the organs of assimilation and excretion. The pain of hysteria is usually the result of a psychic or physical abnormal reflex influence. And so on for most of these so-called neuropathic head- THE NEURONIC DISEASES 237 aches. Hemicrania, though discussed by itself in another section, falls into this class, being- related to epilepsy. 5. Organic. By organic headaches we mean those that are set up by gross, organic lesions easily discovered upon post mortem exam- ination, or even during life, and that may or may not be amenable to -surgical intervention. Disease of the bones of the skull, meningitis, -cerebral syphilis, intracranial neoplasms, arteriosclerotic productions are some of these organic causes. Under their respective heads the symp- toms will be further considered in detail. 6. Psychic. Once in a while we meet cases of headache which seem to have been brought on by pure psychic influences, and are certainly sometimes quickly removed by suggestion and other psychic means. Sudden emotion, fright, disappointment, prolonged intellectual action, dread, terror and other depressing psychic influences sometimes .give rise to headache. In a few cases of masturbation it has seemed to me that the headaches and other symptoms were due to the dread and shame and fear of insanity, more than to the mere physical stress. With Wundt I regard the mental acts as processes and not actual en- tities. They are not simple, but complex. They represent the inter- play of certain elementary sensory impulses in and among one another. If this interplay is suddenly disturbed or shocked it reacts injuriously upon the underlying sensory elements and causes them to manifest pain. These cases, however, are very rare. Pathology and Pathogenesis. — It is somewhat of an anomaly to speak of the pathology of a symptom. Only diseases have a patho- logical basis, and headache is not a disease. And yet there are some facts in regard to the pathogenesis of even a single symptom so promi- nent and unique as headache sometimes is that need a moment's con- sideration. Apart from the pathology of the underlying general abnormality upon which the headache depends, there is absolutely no known con- stant lesion that can by any stretch of reasoning be distinctly associat- ed with the latter. Hsemic, toxic, reflex, neuropathic and organic head- aches reveal nothing beyond the usual findings of these respective con- ditions which may be singled out as the specific source of the head pain. Various theories have been proposed and the cephalalgia has been assigned to the implication of the fifth nerve or the nerves of the dura mater, the nervi nervorum, the primary and basal ganglia or the cerebral cortex. One thing seems to be certain, however, and that is that true cephalalgia is not the result of disease in the peripheral nerves and their primary ganglia, for this produces the true neuralgias in and about the head. The headache pain is too diffuse and otherwise unique to be a mere nerve pain. There is nothing beyond mere guess- work to assign the pain to the dura mater. Well-known primary and secondary diseases of this or the other membranes, aside from the pres sure which their thickening may exert upon the cerebrum or related nerves, do not provoke true headache. The bead pains of syphilitic and tubercular meningitis are pressure pains. To attribute all h< aches to the dura mater we would have to assume that in all head- aches the dura was diseased and more or less thickened. This would 238 THE NEURONIC DISEASES be an unwarrantable assumption, especially in the total absence of all other indications of meningeal disease. - The localization of the original source of the pain in the basal ganglia, especially the optic thalamus, was strongly argued by many at one time. Our more recent knowledge of the physiological functions of the basal ganglia is not favorable to this view and it never was anything more than a gratuitous hypothesis without experimental or pathological proof. The only view that has a shadow of a positive foundation whereon to rest is that which attributes the pain directly to the functional or organic disturbance of the sensory elements of the cortex. Migraine, of course, is something more than a mere headache. It is a disease of the sensory cortex closely related to that other primarily sensory dis- ease — epilepsy. It has many symptoms, but its leading symptom, the headache, is in all essential respects the same as the various types of headache now under discussion. Therefore if migraine is a cerebral sensory phenomenon, why not the cephalalgias that so closely imitate its dominant symptom, the headache? In their immediate causation the same toxic, reflex and other factors play a significant role. In their diffusiveness, changeability and localization they are not unlike. Even in some of their manifestations other than the mere pain they bear a remote resemblance. And in their management the same gen- eral specific medication obtains. In other words, let the cause of the trouble be what it will, as well as the general nature of the disease or its collateral symptoms, the headache, every indication shows, is a functional disturbance of the sensory cerebral cortex. It is quite impossible to account for the location of certain head- aches in particular parts of the head. Psychic auto-suggestion may have something to do with it, as, for instance, the localization of the ocular reflex headaches just above the eyes. The location of the irritative lesion, as in some of the organic cases, may determine the location of the headache. In most of the cases there is no sort of imaginable way by which the concentration of the pain upon this or that side of the head, in front or on top, can be accounted for. This very indefiniteness is an argument in favor of its cerebral origin, it seems to me, for in this respect at least it resembles the psychoses, which are positively accepted as of cortical origin, but without a more definite seat within the cortex. Like the latter it is a functional trouble of the sensory cortex more or less in toto, rather than of special centers of sensation in that cortex. Symptoms. — Cephalalgia is a diffuse, changeable soreness or pain of the head or various parts of the head. It may be superficial and aggravated by slight percussion. Steady pressure often relieves it. Sometimes it is dee]), concentrated and boring. At other times it resembles a paresthesia and is described as burning, cutting, pressing. pulling, sticking like pins and needles. Usually the seal]) is hyper- aesthetic. The character of the pain and its action under different conditions are sometimes dependent upon the immediate cause of it. The same may be said of its location. For instance, anaemic headaches are dull THE NEURONIC DISEASES 239 and heavy, are not particularly severe and are spread more or less over the entire scalp, the brow or sides of the head. The recumbent position usually alleviates them. On the other hand, the congestive headaches are full and throbbing, combined with vertigo and aural disturbances, visual phenomena and redness of the face and con- junctivae. The recumbent position aggravates these. The location of the headache is often strongly suggestive of its cause, though no absolute rules can here be laid down. The larger number of the head pains are frontal ; next in frequency are the oc- cipital headaches. Vertical and lateral localization of the pain is not uncommon and often generalized combinations of these are met with. Pains that are more or less constant and are localized in the supra- orbital region are apt to be due to ocular defects or dyspepsia. If the pain here is absent in the morning and appears in the afternoon it is due to eye-strain. In neurasthenia I have often noticed that the patient will get up with a slight frontal soreness, which will disappear for a few hours, only to return later on in still greater severity. Auto- intoxication headaches, with constipation, are dull and constant over the upper part of the brow. Sharp, boring, burning pain on the vertex is a neuropathic headache. As clavus it is typical of hysteria ; as a full, compressing or lead-cap feeling it is probably neurasthenic. A steady, duller ache on the top of the head should cause us to look for anaemia, uterine disease, possibly stomach or bladder trouble. Occipital soreness is mostly uterine or gastric in origin and is often accompanied by nausea. There may be vertigo. Temporal headaches, particularly if they are unilateral, keen and persistent, are neuralgic or migrainous. In the diagnosis of the headaches of organic origin, say from tumor, exostosis or meningitis, one would not rely alone on the pain symptom. In every case a most thorough search ought always to be made for collateral symptoms. These will often interpret most con- clusively the cause and nature of the head pain. The persistency of the headache will suggest a persistent cause, like caries of the teeth, tumor; headaches that come and go may be merely neurotic or indicate their dependence upon conditions that are subject to changes, such as nerve tire, constipation, gastric activity and quietude. Diagnosis. — The mere diagnosis of headache is no diagnosis at all. The underlying cause or disease that provokes the pain is tin- ultimate purpose of the examination. It is always a confession of defeat, unfortunately sometimes unavoidable, to write a prescription with no better end in view than merely to suppress the pain. Head- ache is like the wreath of smoke that should lead at once to the threat- ening fire. The latter is not extinguished by blowing away the smoke. Hence of all the symptoms met with in the practice of' medicine none needs a more careful study. I am convinced that in making a diagnosis in headache the errors will be less in number just in proportion as the examiner keeps his mind off of the headache itself and turns his scrutiny upon the collateral symptoms of the possible disease that is causing the headache. In other words, let him minimize as much he can the cephalalgia in the drawing up of the clinical 'picture of the 24-0 THE NEURONIC DISEASES case in hand, and emphasize all the other symptoms that may be pres- ent. For instance, search keenly for the symptoms of neurasthenia, if there is an intimation that the head pain is neurasthenic in origin ; rather dwell upon the paresthetic, lead-cap character or what-not of the headache. Delay not with the dull, aching, diffuse character of a possible anaemic headache, but make a blood examination and deter- mine the presence of anaemia. Be not satisfied with a supra-orbital headache as of ocular origin, but study the case as though it had pre- sented itself primarily as one of eye trouble. In the books and in practice too much reliance is put upon the location and character of the headache as indications of its cause. They can only offer the barest sort of hints. So variable are the different types of headache that one may some- times easily be mistaken for another. Authors lead their readers into a feeling of treacherous self-security by telling them that each sort of headache has its own marked and distinctive characteristics. The characteristics are quite shadowy and scarcely ever more than barely suggestive. Exceptions are all" too frequent to the general rules laid down in the books. The only safe way to do is to search for and diagnose the primary disease on top of which lurks the headache that induced the patient to consult the physician. Prognosis. — This depends so absolutely upon the underlying cause that nothing further need here be said about it. Treatment. — The proper treatment of headache is the removal and treatment of the underlying cause. Anything short of that is a' mere makeshift. The shops are full of so-called headache remedies, sure-cures and patent nostrums. A physician who> treats a case of headache that presents itself at his office by prescribing a pain-killer or -shot-gun sedative mixture, without attempting to find out and re- move the cause, is exercising about as much intellectual acumen as the individual himself would do if he had first gone to the drug store and asked for one of the much-lauded headache cures. The laity rec- ognize this and reason to themselves justly and unanswerably that there is nothing gained, but something saved, by not consulting such a physician. Much of the rampant quackery and counter-prescribing of the day is thus fostered, and such physicians can take much of the blame for it to themselves. Every case of headache indicates some disease of more or less severity, and though a physician may at first attempt to give the pa- tient comfort by annulling the pain, his duty and his reputation both demand that he insist upon the patient joining him in regarding his trouble as something more than mere head pain, and in allowing him to search for and remedy so far as possible the disease of which the headache is one symptom. Under the head of etiology I have pointed out some of the more prominent of these troubles, and their treatment must be sought for elsewhere. I will mention here merely some of the means of relief to be employed temporarily. If the headache is at all severe it is well to minimize as much as possible all sources of peripheral, sensory irritation. The patient should occupy a dimly lighted, still room, comfortably warm and with THE NEURONIC DISEASES 24I free ventilation. The clothing must be loosened and, better still, re- moved entirely in some cases. The cutaneous sensation should be kept as quiet as the visual and auditory. A warm bath is sedative in this respect. If there is congestion of the head, mustard pediluvia are useful, but they must be employed for half an hour at least. Except in a few instances of occipital headache mustard to the nape of the neck is irritating and not equally compensating. In extreme cases two or three leeches behind the ears may have to be resorted to. Dry cups to the shoulders and upper spine have helped some of my cases. The effect was partly psychic, I am sure. Electricity is useless. Gentle rubbing and stroking of the head, the cold water coil, the ice bags or hot cloths to the brow and nucha may be tried. Rubbing the temples with the menthol stick or a twenty per cent, solution of menthol, or a chloroform liniment is sometimes useful. Only alleviation, however, can be hoped for from all such external applications. The stinging of the skin diverts the attention temporarily from the cephalalgia. In most cases much more can be anticipated from a brisk cathartic given early. Calomel, salines, jalap powder, elaterium and croton oil are available for this purpose. There are many remedies that may be given, but their effects will vary in different cases. Phenacetine has been the most generally useful in my experience. A ten or fifteen grain dose is given at once, and five grains every hour or half hour thereafter until about fifteen grains more are taken. Then I continue the remedy in five-grain doses every two, three or four hours. It is well sometimes to give a little brandy or aromatic spirits of ammonia at the same time, and to insist upon the patient retaining the recumbent position. Antifebrin and antipyrine may be used instead, though in my experience they have threatened collapse several times. An excellent pain sedative sometimes is a thirty or forty grain dose of sodium salicylate. Muriate o>f ammonia in doses of a dram or half a dram is recommended. Some of the remedies sug- gested under the head of hemicrania may be tried. Such medicines as aconite, cannabis indica, nitromuriatic acid, digitalis, belladonna, col- chicum, ignatia, nitroglycerin, sodium bicarbonate and valerian should be thought of when the particular conditions present call for them. Caffeine alone, or with full doses of the bromides, is available. Hydro - bromic acid, aromatic spirits of ammonia and valerian have assists 1 me in some cases. Butyl-chloral hydrate, camphor, chloralamid, ethy- lene bromide, gelsemium, guarana, hyoscyamus, kola and quinine con- stitute an experimental list which it may be convenient to have ready at hand when the case is obstinate. As a last resort the stronger nar- cotics may be used. Even morphine fails sometimes and there is always the danger of establishing the habit in these cases. So desperate are the cases sometimes that even blood-letting and the introduction of a seton have found approval. In no trouble is the physician's skill and resources put to a more severe test. The greater these are the happier he will he. I le should never allow himself to be so resourceful, however, as to thereby be rendered forgetful of the fact that the fundamental treatment of head- ache is first, last and all the time the steady and persistent treatment 2^2 THE NEURONIC DISEASES of the underlying- disease or defect out of which the cephalalgia is but a mere by-product. THE NEURALGIAS. The term neuralgia means literally nerve pain. In a broad sense every pain that is experienced is a neuralgia. In this way the laity still speak of headache sometimes as a neuralgia. In neurology we have restricted the use of the term to pains that can be definitely local- ized in the nerves of the body. Indefinite and diffuse pains and mere psychic distress we do not include in neuralgia. The explanation and definition of pain has been the problem of the ages, and we are almost as far from its solution as ever. Pain is a phenomenon, a manifestation merely. It is not a thing itself, but merely represents the activity of some thing. Just what this thing is, and at what point this form of its activity ceases to be normal and becomes abnormal, is the still unanswered riddle. The localization of the phenomenon we call pain and the determination of its relationship to what we are pleased to denominate normality would, if known, dispel a vast amount of confusion and contradiction in regard to all neuralgia. I will endeavor in as few words as possible to make clear the position of modern neurology in regard to these two important ques- tions, and I think it will help us to classify and comprehend better the specific forms of neuralgia now about to be considered. It is conceded by every one that the experience of pain is dependent upon the nervous elements. If the nervous elements of the body could be in some miraculous way dissolved out of it, without in the least disturbing the bones, muscles and other tissues, there would absolutely cease to be all pain or possibility of pain. In what part of the nervous system does this function of the pain sense reside? Both experimental and pathological observations prove that the neurones, the true nervous elements, the cell-bodies with their processes, constitute its seat. The neuroglia, the connective-tissue and the blood-vessels, the sustentacular elements interwoven with the net- work of neurones, do not subserve in any way the awakening or the transmission of the pain sense. By the aid of certain experimental and pathogenetic degenerative processes we have learned pretty ac- curately just which sets of neurones it is that are sensory. There are so many different forms of sensation, such as touch, temperature, pain and special sense, and the neurones that subserve them are all so closely intermingled, that only in connection with one or two of the special senses are we able to say just what sets of neurones belong to each respective form of sensation. We can pick out, as it were, the visual or the auditory neurones, but we cannot pick out the pain-sense, the tactile and the temperature-sense neurones. We can only speak, then, of general sensory neurones and special sensory neurones, and in a gross way we can point out where each respective set is to be found. When we examine, by staining and microscopic methods, these sensory neurones and compare them with what we have learned to be the motor neurones, we discover that apparently there is no essential THE NEURONIC DISEASES 243 difference in their intimate structure. I say apparently, because even yet we dare not assume that our modern means of investigation, mar- velous as they are, are absolutely perfect, reliable and ultimate. With our present means of investigation, however, we make bold to assert that all neurones of the body, sensory, motor and trophic, are, except in some obviously and functionally unimportant particulars, structurally alike. This assumption is strengthened in part, as I have pointed out elsewhere, by the fact that the manifestations of nervous activity are revealed to us not in the nervous elements themselves, but in their related end organs. Motion is not, strictly speaking, a nervous, but a muscular phenomenon. Vision, hearing and touch are manifestations or experiences evolved by special extra-neural apparatus connected in- timately w T ith similarly constructed nervous elements. As on the motor side, so on the sensory, then, we assume that the neurones are not only structurally the same, but functionally the same, and that their apparent differences of function are dependent upon the particular end organs with which they happen to be connected. Though the proofs from the laboratory are not yet absolutely be- yond question, they are so strongly in favor of the uniformity of nervous matter everywhere, both in structure and function, that it has almost become an axiom in neurology that there is a specific nerve- energy, a form of irritability merely, which is the same everywhere, but which is revealed in various ways through the various non-neural tissues and organs with which the nerve elements are in organic, functional relationship. If this is so, then we must look to the ends of the pain-sense neurones for the explanation of the nature of pain. Unless we add pain as an additional form of energy in the nervous elements to the one universal specific nerve-energy, irritability, we must account for the pain, just as we do for motion, for secretion, for vision, for hearing, for taste, for touch and so forth, by the presence of some extra-neural mechanism. If it be so added as merely another form of neurility, it would have to be a characteristic of motor neu- rones too. Analogical reasoning bears us out, therefore, in the contention that pain as a specific sense is the result of nervous irritability working in conjunction with some sort of extra-neural structure or mechanism. There are many gross observations in physiology and clinical prac- tice that lend color to this conception of the nature of pain. I will now refer to these somewhat in detail, as they will have to do with the relationship of pain to what we are pleased to call normality, and which I said was the second great problem to be solved in connection with this very commonplace phenomenon called pain. Having shewn that the trend of modern neurology is toward af- firming positively that pain is not a neural manifestation per se, but the expression of mere neural irritability, responding to the abnormal work- ing of some extra-neural tissue or mechanism, it behooves us to point out as far as possible where and what that extra-neural mechanism is. It will be conceded that sensory, like motor neuronic irritability. needs for its manifestation an external stimulus. It will doubtless be also conceded that for its proper termination into what we call pain, 244 THE NEURONIC DISEASES and not into some other form of sensation, the irritability must expend itself upon some specific organ or mechanism. The stimulus is from without and is in the form of an irritation. If the stimulus can originate in the neurone itself and provoke thus the specific sense of pain by expending itself solely within the neurone, apart from its central connections, we would be forced to go back again and believe that the evolution of pain is a specific form of nerve- energy. A stimulus may come from a degeneration or other nutritional molecular change in the neurone, or in a part of it, say at the outer end of its peripheral process, but the stimulus will have to pass out of the neurone in some way to provoke pain unless we believe that the pro- duction of pain is a function of the neurone, which would, of course, destroy the axiom that there is only one specific form of nerve-energy, mere irritability. Stimuli we find may be conceived of as extra-neuronic and intra- neuronic. The extra-neurcnic forms of stimulation, both in health and in disease, are of common observation and form the source of nine- tenths of our pain sensations. The intra-neuronic forms of stimula- tion are probably best evidenced in the peripheral degenerative troubles in the sensory nerves. These are not common, though they may be assumed to be the basis of some of the pains of certain general and localized, mild or severe, degenerative troubles, like pseudo-tabes of the Dejerine type, of a few cases of so-called peripheral degenerative neuritis from alcohol, arsenic, etc., and possibly from some forms of neurosis like neurasthenia. These peripheral degenerations are not uncommon, but it is always a question whether they are primarily degeneration and not secondarily degeneration from a compressing inflammation, new growth or other lesion in their close proximity. Granting that we do have an inherent peripheral stimulus in this occasional primary peripheral degeneration in the neurone that subserves normally the pain-sense, we have an ex- planation of the specific primary degenerative neuralg'ias referred to in the books. This stimulus must expend itself centrally upon some special cell, organ or mechanism to produce the specific sense which we call pain. We find the cell bodies of the sensory neurones scattered all the way up and down the nervous tracts. This I have pointed out in the earlier chapter on the neurone and the neuronic architecture of the nervous system. Are the central sensory cells individually the pain-sense centers? We find sensory cells in the posterior root ganglia, in the posterior oornua, in the bulbar ganglia, in the central ganglia of the brain and in the cerebral cortex. Which of them subserve the pain-sense; and were we able to determine which, would the cells themselves be the mechanism wherein the incoming sensory impulse is transformed into a pain-sense" It would hardly seem so, for if a sensory nerve he cut oft from its cutaneous connections and the proximal end of it be irritat- ed, pain is experienced. If under such circumstances the transforma- tion of this afferent impulse into the specific pain sense be accom- THE NEURONIC DISEASES 245 plished by the individual cell to which the impulse has centrally arrived, apart from this cell's connection with all other cells, we will again be driven to attributing to these cells both a specific structure and func- tion. We will then have to relinquish the idea again that nervous ele- ments have all only one and the same function, the manifestation of irritability, and that their differences of function depend merely upon their connections. This will not be necessary, however, it seems to me, for there are many clinical observations that go to show most sus- piciously that the sensory cells are not individually the centers for the transformation of mere afferent impulses into specific pain sensations. Chief among these observations are the following : 1. The obliteration of consciousness by ether narcosis abolishes all pain sense, without, so far as we can see, abolishing the functions of the afferent neurones generally. Of course, all sensation is largely subjective and the patient's ego being for the time in abeyance, there are no subjective results from peripheral stimulation. Some of the reflexes that are outside of the sphere of consciousness are still active ; for example, the visceral reflexes. Here the sensory cells are apparently still intact and so far as we know they are not essentially different from the cells that functionate in the conscious sphere. 2. In the same line of proof is the fact that memory retains spe- cific forms of sensation, when the usual sources of the afferent im- pulses are removed, as is seen in cases of amputation. There may be some question as to whether consciousness is co-extensive with the entire nervous apparatus or is to be confined solely to the brain. In regard to memory there is no such question, however. Memory is a brain function, or at least not a spinal cord function. Most de- structive diseases of the cord can occur without the slightest weaken- ing of the memory. In the amputations, therefore, in which memory retains sensory pictures, it would be demonstrated that the functions of the cord cells and brain cells are different. Cord cells don't memor- ize, consciously at least; cortical cells do. As the cells are the same functionally and histologically, though, we must search for another reason for the difference in their functions in this respect. It is to be found outside of the cells — namely, in their relationship and interplay with other cells. It is in the mind. In its last analysis, then, pain is a pure mental manifestation, h is not a specific form of nerve-energy. It is not a direct product of individual cellular activity. It is dependent upon the activity of certain cells for its expression. It is a psychic phenomenon, with all the variability and all the uncertainty that goes with a complex manifesta- tion of a complicated physical interplay of individual physiological units, such as the mind is. Pain is not an entity. It is a psychic experience ; one of what Wundt well calls the psychical elements. Being dependent for its pri- mary evolution upon the activity of a physical basis, it assumes a phys- ical appearance and we speak in common parlance of physical pain. On the other hand, being a psychical element and partaking of all the characteristics of mind, we speak of it as a psychical pain. There can obviously nor be two kinds of pain that are identical 246 THE NEURONIC DISEASES and yet different at the same time. The difference between physical and psychical pain is one merely of the nature of the stimulus and the underlying physical basis. Like the distinction between colors, which is a purely psychic or cerebro-psychic phenomenon, and which we project into the outer world by attributing the colors to the objects or sources of the visual stimulation, so the pain, which is a psychic experience, is projected outward and we say that the pain is in that nerve or this organ, whereas the latter is only the objective source of the stimula- tion. This has all a most practical bearing and if the general truth un- derlying it is fully grasped, it will not be difficult to realize that a psychic pain, such as may occur in hysteria and other psychoneuroses, is as much of a reality as is a pain caused by a corn on the toe. In neither case is the pain sense per se different ; in either case it is a psychosis due to different sources of stimulation. In the latter case we can reach the source of stimulation with the knife and salves ; in the former case there is a disturbance in the functional interplay of the psychical elements of such a nature that neither surgery nor medicine can reach, but only psychic measures which belong to the same sphere of action, the psycho-neurotic, that the disease does. Extra-neural stimuli of so-called physical pains are as a rule very amenable to treatment, and their prognosis is generally favorable. In- tra-neural (neuronic) stimuli which produce both the so-called physical and mental pains, are very obstinate to treatment and possess a rela- tively hopeless prognosis. As the pains of the first sort were the most obvious and the first to be recognized boldly, and as the particular nerves involved in the extra- neural lesion were clearly and easily discoverable, nerve pains came to be identified with individual nerves and the term Neuralgia acquired a very narrow meaning. So fixed is this meaning now that it would be hazardous in the extreme to use it in any broader sense. I will there- fore continue to employ the term as a synonym for a pain that can be referred to some particular nerve or nerves. As these nerves are sub- ject to inflammation, compression and destruction, they will be appro- priately discussed under their own individual heads in the section devoted to the non-neuronic affections in the latter part of this book. I [ere, however, ought to be noticed those neuralgias which are, or may be, referred to particular nerve trunks, but which reveal no discoverable changes in or out of the constituent sensory neurones, which are of central origin and which therefore partake of all the traits of a true psychoneurosis. These are what some writers mean by the primary or essential neuralgias. Like all similar neuroses they have a neuropathic heredity behind most of them, or occur as a part of a general neuropathic state; are probably the expression of a congenital, nutritive deterioration of greater or less degree in the general neuronic elements of the nervous system; are of doubtful, often hopeless, prognosis; and are not very amenable to treatment except through general tonics, powerful seda- tive^, isolation, disciplinary and psychic measures, by which they may sometimes be considerably helped, but not cured. THE NEURONIC DISEASES 247 Clinically it is not always possible to differentiate an idiopathic neuralgia from the pain caused by neuritis, reflex irritation or neigh- boring disease. This is because both may be of such a slight degree that the characteristic symptoms of either are not well marked. The difficulties of practice should not, however, be allowed to interfere if possible with the accurate presentation of the known facts of science. A neuralgia that is a mere reflex pain does not seem to me to be prop- erly classified as a disease, though it is proper to refer to it in a work upon nervous disturbances, just as we there also refer to other normal functions of the nervous system. A neuralgia, for instance, that is caused by a cicatrix, a bad tooth or loose internal organ, I would call attention to, not as a nervous disease, but as a disease overworking a normal nervous function. It is to be corrected by removing the cause, but it is certainly not a "true idiopathic" neuralgia in the sense of being a disease any more than the sudden touching of a hot plate or the prick of, a pin is a neuralgia in the sense of being a disease. This is an important distinction, for practically all of the so-called reflex neuralgias are of this character and need no treatment other than removal of the source of the reflex. Every nerve pain should be ex- haustively examined for a possible irritant of this sort. This leaves then only the inflamed nerves to be differentiated from the idiopathic pains. A careful consideration of the pathological condition in neuritis, and the absence of such a condition in primary neuralgia, will help one to realize the difference in the clinical presentation of the two troubles. The former is locally irritant and compressing ; the disease process is not uniform throughout the entire nerve length ; the nearby motor elements are implicated in the damage, along with the sensory ; and there is a rather sharp limitation of all the sensory and motor signs to the one nerve and its area of distribution. Idiopathic neuralgia, on the other hand, is a central neurosis, there- fore there are no marked locaf manifestations ; the nerve is sore, to be sure, but it is not sharply and distinctly locally sore ; the whole nerve is equally sore. The pain is a referred pain. I recently saw a case in consultation that had been vigorously treated locally with mas- sage, electricity, etc., as an instance of sciatica. The pains were paroxysmal and complained of almost entirely in the foot and a short way up the leg. Pressure on the nerve caused soreness, but not pain. I diagnosed intrapelvic secondary irritation, which proved to be abun- dantly present upon further examination by a surgeon of ability. Idiopathic neuralgia has no motor symptoms except what may be caused in a general way by the pain of movement. There are no electrical reactions. No atrophy appears except late in the disease. The paroxysmal character of the pain indicates its idiopathic nature, though it must be remembered that a mobile source of irritation to the nerve may cause a mere reflex pain to come and go like a paroxysmal trouble. A tender nerve can, of course, be made still more painful by sudden movements, jars, coughing and sneezing. I have found stretch- ing of a nerve that caused marked exacerbation of pain, say by forcible 248 THE NEURONIC DISEASES flexion of a limb, to be rather indicative of a neuritis than an essential neuralgia. Early motor, other sensory symptoms with the pain, and trophic manifestations point to neuritis. In fact, pain and only pain constitutes the entire clinical picture of neuralgia. Late trophic symptoms of a general character, like falling of the hair, etc., may be due to the same general neuropathic condition of which the neuralgia is itself one evidence. Changeability of neuralgia is in marked contrast with the fixity of the neuritis. It is not an uncommon thing for a primary neuralgia to suddenly transfer itself to the corresponding nerve on the opposite side of the body, or to some other remote nerve. Hyperesthesia of a slight degree, but never anaesthesia, sometimes occurs late in the area of the skin innervated by the affected nerve. The pressure points of Yalleix are as a rule indicative of neuritis, being the local spots in the nerve where the inflammatory spots are most exalted. In neuralgia, however, they may indicate where the sore nerve happens to pass over a bony edge or prominence. Prolongation of the pain has its general depressing effect upon the constitution, though it must be remembered that primary neuralgia is mostly observed in depressed, cachectic and neuropathic individuals. Mild psychosis, such as melancholia, is often present. Suicide is thought of in many severe cases. The causes of primary neuralgia are the general cachectic, infective and toxic states. Mental shock may cause it. Debility is the most frequent cause. It is doubtful if malaria is so much a cause as it is supposed to be. Neuralgia is not more common in malarious districts than elsewhere. One must not confuse the neuralgia of the general debilitated state induced by rheumatism, gout, Bright's disease, lead intoxication, diabetes or exposure with the neuritis often produced by the same causes. Women of neuropathic families are the greatest sufferers from the disease. Children and the aged do not have to endure it. It is prac- tically unknown before fifteen years of age. The following are some of the more prominent forms of idiopathic neuralgia and their treatment : Tic Douloureux. Trigeminal Neuralgia. Prosopalgia. This is a frequent form of idiopathic neuralgia. It should always be carefully distinguished from symptomatic pains in the fifth nerve, from various lesions and sources of irritation around and near it. The latter are very common and will be referred to again under the head of special dis- eases of the fifth nerve. In tic douloureux the patients are usually neuropaths in the middle period of life. All branches of the nerve may be the seat of the pain and the trouble may be unilateral or bilateral. The paroxysmal and intense character of the pain is a marked feature of it. Usually it is limited to one branch of the nerve and is most excruciating. It is described as a feeling like a boring piece of hot iron, or a hot knife cutting into the Face. All movement is avoided. Tenderness is felt and even twinges of pain in neighboring nerves. From the cheek, the lips and side of the THE NEURONIC DISEASES 249 nose, where the pain is usually at its maximum, it seems to radiate into the teeth, over the eye, up or to the head and- across the temple. The face becomes red, the eyes water, the flow of saliva and nasal mucus increases. The agony lasts a few minutes and then the pain partially recedes. It usually returns again and the new paroxysm may, like the first, be started by some simple irritation against the skin. Long standing cases may be accompanied after years of suffering by premature turning gray of the hair and in rare instances facial hemiatrophy. The disease usually involves the supra-maxillary branch on one side. The supra-orbital and inframaxillary branches are less often im- plicated. Changes of the weather sometimes affect the pains and they are worse as a rule in winter than they are in summer. The severe forms of these neuralgias that sometimes occur in the aged were called epileptiform by Trousseau. In the diagnosis of tic douloureux every possible reflex and symp- tomatic pain of the nerve should be eliminated. It is to be frankly admitted that this cannot always be done. It is to be remembered, then, that pure idiopathic neuralgia of the fifth nerve is far less fre- quent than any of the other forms. One should always be on the alert to recognize at any time symptoms that point to some other cranial disease which has caused the pains. If the patient is neuropathic, if there is a heredity, direct or indirect, behind the disease, if the pains are markedly paroxysmal and severe, almost epileptiform in character, and if they constitute the entire ciinical presentation in the case, a diagnosis of tic douloureux will probably be correct. The treatment of tic douloureux is not a very grateful task. It is a most obstinate trouble and often resists all measures. Sometimes it ceases all at once. Would that we could always take the credit for this. Every case must be managed along general lines and treated for the paroxysm. The whole physical and mental being must be built up. Change of scene, change of thought and entire change of environment may be necessary. The diet must be abundant, plain and nutritious. The functions of the body must be systematically attended to. Tonics like iron, quinine, cod liver oil and hypophosphites, and especially arsenic, may be judiciously administered. All defects, such as circula- tory weakness, poor blood, insufficiency of the kidneys, ocular anoma- lies, sluggish liver, must receive their quota of attention. In some cases general treatment along these lines will cause the paroxysms to cease entirely, or, what is more likely, to remain farther apart and be less severe. The treatment of the paroxysms will indeed tax the physician's resources to the uttermost. It is discouraging to read the long list of remedies that are recommended, and yet that generally prow Ii Still something must be done, and as amelioration does sometimes most unexpectedly follow the administration of a remedy, we must go on giving them, even though it be like a forlorn hope. In aged persons nitroglycerin, gr. 1-100 every three or four hours, has been effective. Anaemic cases may have their attacks aborted by an inhalation of 250 THE NEURONIC DISEASES nitrite of amyl. Sodium salicylate in twenty or thirty grain doses may be successful. Quinine, arsenic, gelsemium and potassium iodide in full dosage are occasionally available. The last should be given es- pecially if there is the slightest history of syphilis in the case. Co- caine instillations into the conjunctival sac, and painting of the nasal mucous membrane with five or ten per cent, solutions may afford tem- porary relief. Butyl-chloral, croton chloral, codeine, narceine, napellin, neurodin, stramonium, pyramidon, atropine, cannabis indica may be experimented with. The coal-tar preparations were useless in my experience. Duquesnel's aconitine has been useful in some of my cases. The employment of morphine must be instituted with extreme caution. Dana recommends for cases not over four or five years' standing rest in bed with massive doses of strychnine. I have seen this succeed in one case. The drug is given hypodermically in doses of gr. 1-30 once daily and gradually increased until gr. 1-6 or 1-5 is reached. This is kept up for four days, and then gradually reduced. Absolute quiet is enjoined in bed. After a month iron and potas- sium iodide are exhibited. The treatment can be repeated with less vigor. Certain local measures sometimes relieve the pain. I have referred to the use of cocaine. Local freezing with a methyl chloride spray, or the application of extreme heat, may bring relief temporarily. Schleich's subcutaneous local application consists of a solution containing cocaine gr. ] l / 2 , morphine gr. y±, sodium chloride gr. iv^2 and water giij. Compression of the carotid arteries may be tried. Electricity is often beneficial, but it may have to be used in various ways. Oppenheim curiously secured temporary good results with a powerful faradic brush applied to the soles of the feet. This was probably a psychic result. The electricity is usually directed as near as possible through the affected nerve, or it may be applied to the cranium or to the sympathetic nerve in the neck. Galvanic, faradic, static and cataphoric forms may all be employed. Galvanization of the Gasserian ganglion is the most generally useful method. Electrotherapy so often fails that I am inclined to attribute the supposed cures from it to the power psychic effect usually produces on these neuropathic individuals. Operative procedures often give prolonged temporary relief, but after them, just as in spasmodic tics, the trouble comes back. The very nature of the trouble, a central neurosis, would lead me to expect that sort of a result. However, in some of the frightful cases a tem- porary relief of several months amply pays for the risks of the opera- tion. Neurectomy, extraction of the nerve and intracranial resection of the nerve and the Gasserian ganglion are the procedures resorted to. Their description belongs to the works upon surgery. Glossodynia doubtless should be classified here. Occipital Neuralgia. As a pure non-symptomatic form this is ex- ceedingly rare. Usually disease in the upper cervical vertebrae, or their neighborhood, and hysteria, are the causes of the pains in the upper cer- vical nerves. When the neuralgia is idiopathic, unlike the other forms THE NEURONIC DISEASES 25 I here, it is unilateral, paroxysmal and intense. Tender points are elicited at .the exits of the nerves. The pains may alternate with those of tri- geminal neuralgia. The treatment is the same. Cervico-brachial Neuralgia. This is very rare idiopathicallv, most •cases being due to rheumatism and other obvious causes. In neuras- thenics and hysterics pains shoot around the shoulder and down into the arm and into the digits, following the course of the nerves, particu- larly the ulnar nerve. Most of the cases of cervico-brachial neuralgia that I have seen were cases of neuritis. The diagnostician must be on his guard in regard to these pains, for often they represent meningeal and other organic spinal cord lesions. Their treatment, after eliminat- ing all sources of irritation and all organic disease, is the same as for all idiopathic neuralgias. Intercostal Neuralgia and Mastodynia. This is second in impor- tance only to facial tic douloureux. The left side is usually affected and mostly in women. Several nerves are affected at the same time, so that the pain seems to be in the side of the thorax. Sometimes the radiation of the pain extends to the inner side of the corresponding arm. So many causes produce pains around the body that one must be most keenly on the alert for other diseases and troubles that may be the cause of the intercostal pains. Always remove these causes. If the pain still continues, then the usual measures recommended for all idiopathic neuralgias are to be employed. In mastodynia the neuralgia is limited to the nerves that go to the breasts. They are branches of the intercostal nerves. The pains are paroxysmal and intense and seem to spring from those nerves that come off from the fourth to the sixth intercostals. The skin is hypersesthetic and the breast tender. Herpes, of course, does not occur in idiopathic neuralgia, only in neuritis. The pressure points are here over the second to the sixth spinous processes. Women of middle age are usually affected ; men very rarely. Excit- ing causes may be pregnancy, lactation, fissures, trauma and mental excitement. It is a stubborn neuralgia and the treatment is difficult. Lumbar Neuralgia. True essential neuralgia of the lumbar nerves is so rare that it may be almost laid down as an axiom that pain in them means neuritis or inflammatory and neoplastic development in and about the pelvis. Sciatica I regard practically as always a neuritis or a mere sec- ondary pain. I will discuss this further under the head of the sciatic nerve. Pains that appear in the lower abdominal region, in the scrotum. along the perineum, in the buttocks and down the thighs will almosl surely be found associated with some pelvic disease. Coccygeal Neuralgia. Coccydynia. This is practically a woman's disease. It is an intractable neuralgia of the coccygeal plexus and interferes with sitting. It may be excited by walking or evacuation of the bowels. It is sometimes associated with a local inflammation, though usually it seems to be an idiopathic neuralgia. ( Opiates by Suppository, local irritants and finally operations have had to be resorted to. Electricity has often here proved effective, one pole being placed over the coccyx and the other in the vagina. 252 THE NEURONIC DISEASES Plantar Neuralgia. Pododynia. The pain here is in the heel and sole of the foot. Visceral Neuralgias. These occur in neurasthenic and hysterical women, especially in connection with the heart and the stomach. They must not be confused with the crises of tabes and other forms of symp- tomatic visceral pains. Pelvic neuralgia is very common in women. There is a nephralgia that may even lead to operation for renal calcu- lus. The intestines, the liver, the spleen may possibly be the seat of pure neuralgia, but if so it is very rare. These pains usually mean some organic disease and had better always be regarded in that light. Spermatic neuralgia is a very rare but obstinate affection. Urethrodynia is a rare and obstinate trouble. ECLAMPSIA. Convulsions that resemble epileptic fits, that tend to recur, and that are dependent upon causes outside of inherent brain changes, are now specifically designated by the term eclampsia. From one point of view they may be looked upon as a sort of symptomatic epilepsy, but as they are always acute and in most cases cease entirely after a short time, or after the removal of the cause, it is well to use another name for them than that of epilepsy. There are three prominent types of eclampsia which should receive some notice in a volume upon neurology — namely, infantile eclampsia (eclampsia infantum), parturient eclampsia (eclampsia parturientium) and ursemic convulsions (eclampsia urcemica). ECLAMPSIA INFANTUM. During their early years children are very subject to convulsive seizures upon various provocations. It has been suggested that this is due to the immature and undeveloped condition of their nervous apparatus, especially the w r eak condition of the higher inhibitory cen- ters, a reason which has some plausibility in it, but fails to account for the convulsions of childbed and ura?mic poisoning. Etiology. — The causes of infantile convulsions are numerous, though they may all be grouped under any one of the three heads, reflex, infectious and psychic. Rickets and general debility are very important factors in the production of the trouble. Just how much is to be attributed to the cachexia itself, and just how much to the various disturbances which the cachexia leads to, it is not easy to say. Rickets occurs chiefly between the sixth and eighteenth months of life. It is a condition that fosters or represents defective development just at the period when the development normally is most active. As a result it leads to bad dentition, to gastro-intestinal irregularities and to de- fective nutrition generally. A rickety child as a rule will exhibit a large number of local malformations and functional abnormalities. These-, taken in conjunction with the general defective nutrition, con- stitute a long list of causes of infantile convulsions. The laity always think of worms first among the possible causes, but they are not such THE NEURONIC DISEASES 253 frequent factors as auto-intoxication from gastro-intestinal defects, the distress of difficult dentition, overloading of the stomach, especially with indigestible food, profuse diarrhoea, etc. Phimosis, otitis media, inguinal hernia and other sources of peripheral reflex of an abnormal character acting upon a badly nourished and undeveloped brain, mav all be counted among the causes. Infectious fevers very frequently start in children with a convul- sion. It is probably of the same nature as eclampsia now under consid- eration, though as a rule it is not regarded as the same. It is thus seen in scarlet fever, measles, pneumonia, etc As a symptom of brain dis- ease, congestion, inflammation, hydrocephalus or meningitis it must be remembered. Natal hemorrhage may induce a convulsion immediately after birth, to be followed later on by all the usual sequelae, such as hemiplegia, epilepsy and mental deterioration. Mental shock, such as fright, night terror, fear upon being suddenly left alone, has provoked convulsions. No case of infantile convulsions should be regarded lightly, for be- sides indicating a dangerously susceptible, possibly cachectic, constitu- tion, it may be but the first indication of genuine epilepsy. -Out of 1,450 cases of epilepsy Gowers found that 180 began within the first three years of life. Osier analyzed 460 cases of epilepsy in children and found that in 187 the fits commenced within the first three years. This is the period of life in which eclampsia occurs. As a rule epilepsia con- tinues on past the period, whereas eclampsia ceases. Such, however, is not always the case. Convulsions have occurred in infancy and epi- leptic fits later in life. It would be desirable to know just what is the exact relationship between epilepsy and eclampsia, if there is any. It certainly is not true that early convulsions produce later epilepsy. Hysteria has occurred in adults who had convulsions in childhood. It seems more likely, therefore, that a neuropathic constitution is present, which yields easily in childhood to causes that provoke a convulsive seizure, but is able to resist the greater neurosis until a later age. Epi- lepsy as we have seen occurs in infancy. It is the mistaking of these cases for eclampsia that makes every case of the latter disease a mat- ter of serious consideration. Symptoms. — These are much the same as those of epilepsy. Pre- monitions are sometimes seen in an excessive irritability, with facial changes, slight twitchings about the mouth and rolling of the eyes. Most mothers can detect an oncoming convulsion when others see noth- ing wrong. The attack usually appears suddenly with fixation of the eyes, pallor of the lips and face, stiffening out of the trunk and ex- tremities, rotation of the head and disturbed respiration. The fit is epileptiform in every respect, except in its severity. In a minute or so the spasm relaxes, not to appear again for some minutes or an hour •or so, or it passes into a clonic condition which is usually slight and in- volves the face, eyes, hands and feet. In other cases, a moderate tonic spasm, interfering somewhat with the breathing, may continue for twenty or thirty minutes. Sometimes a series of brief convulsions oc- curs with partial recovery only in the intervals. A scream accompanies some of the attacks. In other cases only screaming, grinding of the 254 THE NEURONIC DISEASES teeth and a slight bilateral rigidity are observed. These convulsions- are to be distinguished on account of their bilaterality from the hemi- plegic spasm of brain lesions. ( rradually the attack subsides, and the child goes to sleep in a state of more or less stupor. In fatal cases the attacks are severe and run together in rapid succession, coma being present as the child dies. 1 )eath is particularly to be dreaded in weak children, in cases with great frequency of the attacks and in diarrhceal hydrocephaloids. Eclampsia usually ceases after a time never to recur. If the con- vulsions continue on throughout the second, third and fourth years they must be looked upon as being epileptic. They are also epileptic if they do not cease after the removal of what may be regarded as an obvious, possible cause. Diagnosis. — The chief differential diagnosis always to be made is between eclampsia, epilepsy and organic brain disease. The first comes on suddenly in a state of relative good health, is associated with some obvious cause, disappears with the removal of the cause and does not continue or recur later on. The second continues after having once appeared, in spite of the removal of apparent cause, and in every way more nearly represents a true epileptic than does the eclamptic seiz- ure. The third reveals characteristic organic symptoms, one-sided spasms that are more or less permanent, and other signs of intra- cranial hemorrhage, meningitis, etc. Laryngismus stridulus, rickety spasms and rigidity, and the hydrocephaloid state have all an eclamptic appearance but need not be considered here further. Prognosis. — This is always serious. Some children are able to pass through the ordeal, but weak and debilitated infants usually suc- cumb. Infant mortality is largely dependent upon eclampsia. Febrile convulsions are not very serious but as I have said they are regarded by some as not exactly the same as eclampsia. They usually occur in more robust children. Chronic diarrhoea with eclampsia usually means death. The prognosis also takes in the question of the seizures being those of genuine epilepsy. After the third year eclampsia is practically never seen. Treatment. — Remove causes that can be removed and treat those, like the rickety condition, in the usual way with syrup of the iodide of iron, cod-liver oil, hypophosphites, etc. Every mother knows the value of sudden immersion in a warm bath. Bromides, chloral and chloroform inhalations are available means for controlling the spasms. Morphia hypodermically, or nitrite of amyl may have to be tried in an attack. Bromides can lie continued afterwards. ECLAMPSIA PARTURIENTIUM. Etiology. — Young primiparae are the most frequent subjects of childbed convulsions, though women of all ages and with any preg- nane) may have them. They usually occur in the last two or three months of pregnancy, during the parturient act or soon after it. Many of the- patients are epileptics and the convulsion is only one of their usual tits. \ arious opinions used to he held in regard to the real cause THE NEURONIC DISEASES 255 of these convulsions such as reflex irritation in the womb, sepsis and cerebral anaemia from the loss of blood. All authorities agree nowadays that nephritis is the cause and that the convulsion is really uraemic. It is true that the coma of uraemic eclampsia is usually wanting in puer- peral eclampsia, whereas in the latter some credit must be given for the reflex influence from the uterus ; the statement is not therefore weakened that both uraemic and parturient eclampsia are due to the same cause and are therefore all but identical. Albuminuria, oedema, amaurosis and albuminuric retinitis may all be present and confirm the etiology. Infection may play a role in some cases from a specific bacillus. Symptoms. — These resemble an epileptic seizure in every respect. In addition there are uraemic manifestations such as intense headache, precordial pain, sudden blindness and oedema. The fit sometimes in- cludes a scream, profound coma, pallor, biting of the tongue, fixation or rolling of the eyes, and violent general tonic and clonic convulsions. The temperature often rises, the pulse becomes weak and the respira- tions assume the Cheyne-Stokes character. Prognosis. — About thirty per cent, of the cases die in coma, in an apoplectic attack or from the sepsis. Status epilepticus with a tem- perature of 109 degrees has destroyed some patients. Treatment. — To hasten the delivery, and how best to do it, are questions entirely for the obstetrician. Venesection that used to be so frequently practiced is now regarded as contraindicated. It weakens the patient merely, without modifying the source of the trouble in the kidneys. Chloroform inhalations, morphine, chloral hydrate in full dosage, venesection with transfusion, diaphoresis with vapor baths and pilocarpine, and free purgation are all to be resorted to according as the demands of the case and the vigor of the patient will indicate. ECLAMPSIA UR/EMICA. The cause of this is of course nephritis. The presence of the kid- ney disease is determined by the condition of the urine, albumin and casts being generally present, by the hypertrophy of the heart and by the albuminuric retinitis. Just what is the mechanism by which the state of blood under these circumstances causes coma and epileptiform seizures is not entirely clear. The convulsions usually occur suddenly though there may be some headache, restlessness, visual disturbances, twitchings of the mus- cles, even a comatose state and visual obscurity just before their onset. I have seen such symptoms in patients in whom I could only diagnose a renal insufficiency, and have observed them disappear with the free use of cathartics and diuretics. They certainly suggested a remote re- semblance to uraemia of mild degree. May not some of the cardiac irregularity, the pains, die general irritability, the fibrillary muscular twitchings of some severe cases of so- called neurasthenia be due to slight renal trouble, enough to provoke a faint degree of uraemic intoxication ? In typical uraemic convulsions the coma, the spasms, tonic and 256 THE NEURONIC DISEASES clonic, the biting of the teeth, the rolling of the eyes are all clearly epileptiform. Nystagmus is sometimes present. The pupils are gen- erally dilated and fail to react to light. They have been seen contract- ed for a brief spell and then dilated. Respiration is greatly embarrassed and right after the fit the knee-jerk is gone. Coma, sleep or drowsi- ness may all follow the fit. Treatment. — Purgatives and hot baths should be used when an attack is threatened. The bowel may be irrigated with water at a tem- perature of 150 degrees F., and nitroglycerin freely used. For the seiz- ure chloroform inhalations must be used, and morphine and chloral. Purgation, diaphoresis, with hot applications and pilocarpine, and mor- phine especially for the delirium are about all that can be done. TETANY. TETANILLA. This is a disease characterized by tonic, intermittent or continuous spasm of symmetrical distribution in the ends of the extremities ; by certain indefinite and irregular sensory and general phenomena; and by a causation that points strongly in the direction of a severe intoxi- cation of the spinal cord or lower neurones. Our knowledge of its pathology is not ample enough yet to enable us to classify the disease definitely. It is seen but rarely in this country. In Europe it is more common and in some places of Sweden and Austria appears to be almost endemic. It has occurred epidemically in the spring of the year. The first account of it was given by Steinheim and Dance, but it was through the work of Trousseau that it first became generally known. Many investigators have studied it carefully since, but their views are still unsettled and divergent. It has been called pseudo-tetanus, essen- tial contracture, and shoemaker's spasm. Etiology. — Tetany has been seen in every period of life. It is most frequent, however, in early childhood and in early adult years. Up to the age of about twenty males are far more subject to it than fe- males. After twenty, strange to say, the number of females is almost double that of the males. This argues emphatically against an occu- pation cause for the disease so strongly believed in by many. Children and girls are physically nearer together than are either to adult males. After fifty the males begin to predominate again. Occupation per se is hardly a cause of the disease though it is said that forty-three per cent, of the cases occurred among shoemakers in the German epidemics. The chances are that contact with some poison is favored by the hand- ling of leather. Certainly the disease is not the occupation neurosis known as cobbler's cramp. The toxic, infectious origin of the trouble is all but positively sug- gested by a large number of etiological factors. When it occurs in women, for instance, it is usually about the particularly exposed periods of pregnancy and lactation. It is seen in connection with many gastro- intestinal troubles, so that a gastric tetany is often spoken of. Diar- rhoea, intestinal parasites, constipation, the use of tainted food and pyloric stenosis with dilatation of the stomach have preceded it with suspicious frequency. One woman suffered immediately after eating THE NEURONIC DISEASES 257 some grapes and drinking soda water. Diarrhoea is a particularly com- mon antecedent in children. Autointoxication is probably the explana- tion of these cases. It is fostered by all depressing influences such as exposure to cold, fatigue, acute disease, rachitis and other diathetic and cachectic con- ditions. Be the factor what it may, if it exhausts and debilitates the constitution in general it favors the onset of tetany. Some of the gas- tric cases have been supposed to be reflex in character because the spasm appeared immediately upon the introduction of the stomach- pump. Why does it not occur more frequently then under such condi- tions, if it is a reflex and not a toxic disease? Cessation of the spasm by removal of intestinal parasites does not argue for the reflex any more than it does for the toxic hypothesis. In favor of the latter is the oft- observed fact that extirpation of the thyroid gland is followed by it. It was seen soon after the injection of spermine. Nephritic disease, the use of alcohol, ergotin and chloroform, the finding of toxic substances in both the stomach and the urine, are data enough to almost establish the toxic origin of the trouble. Among the infections that it has accompanied may be enumerated typhoid, scarlet fever, rheumatism, measles, diphtheria, malaria, influ- enza, pneumonia and smallpox. Generally the spasm appears during the convalescence from these diseases. Whether there is any special significance in the rather frequent association of hysteria and epilepsy with tetany it is doubtful to say. The question is one of importance from the standpoint of differential diagnosis rather than of etiology. It is easily conceivable that a type of constitution that tends toward the instability of hysteria and epi- lepsy would easily yield to a toxic irritant and present the clinical pic- ture of tetany. Pathology and Pathogenesis. — Nothing distinctive in the way of morbid anatomy has been found. Some poliomyelitic changes may or may not be due to the reaction upon the cord of the violent muscu- lar contractions. The same changes have been recorded in connection with other virulent intoxications involving the cord and lower neu- rones, as witness acute ascending paralysis of the Landry type. Hypere- mia and small hemorrhagic foci have been seen, with accumulation of lymphoid cells about the capillaries, and even minute spots of myelitis. The ganglion cells have been swollen or shrunken, cloudy and softened and full of vacuoles. In other words chromatolysis has been present. The spinal membranes, especially in the cervical region, have been con- gested. All of these occasional findings are too indefinite to establish a fixed pathology upon, but when we compare them with the similar findings of other forms of severe intoxication, particularly Landry's disease and some cases of alcoholism, we are impressed with the fact that if tetany be indeed a functional disease, a functional disease is not always one without any organic change whatever. Whether the spasms preceded or followed the few organic changes that have been observed is immaterial in comparison with the suspicious fact that the two phe- nomena are actually associated together. The association alone ren- ders very shaky the rather paradoxical notion that functional exhibi- 258 THE N ELKO N 1 C DI SEA SES tions can be existent without some structural, molecular or chemical, change. Until we get more positive data, it may be well to still speak of tetany as a functional disease and classify it among the neuroses. That the trouble is probably a toxic disturbance of the nutrition of the cells in the spinal cornua is suggested by the symmetrical character of the symptoms showing a central seat for the lesion, by the prominence of the motor manifestations over the sensory, by the similarity of the clin- ical picture in some respects to ergot and strychnine poisoning, and by the few poliomyelitic post mortem findings. The subsequent atrophy in the spinal cells seen in some rare cases and the almost total absence of inflammatory signs, suggest that the tn ul>le is a toxic primary neuronic degeneration. Symptoms. — The characteristic symptom of tetany is the muscular spasm . There may be sensory phenomena of an indefinite character and same other general disturbances. The mind is never affected. There may be premonitions of the disease but they are not com- mon. Usually they are of the constitutional and sensory type. Among them are a feeling of malaise, apathy, headache with nervous vomiting, pains in the back and down the limbs. Sometimes just before the spasm appears, various paresthesias will be felt, such as tingling, pins-and- needles sensation, burning and numbness in the extremities. At length the upper limbs, particularly the fingers and hands, more rarely the lower limbs, exhibit a condition of tonic spasm. Sometimes the feet are affected at the same time. The trunk is very rarely involved. So characteristic is the attitude of the hands that the disease can be diag- nosed from it alone. It is to be noted that the spasm is tonic and that it is symmetrically distributed. The appearance of the hand is much like that of one writ- ing. The ends of the fingers are pressed together and inclined toward the ulnar side. The thumb is strongly adducted and partially flexed beneath the index finger. The palm is hollowed and the wrist is some- what flexed. The condition of the fingers is due to flexion at the metaearpo-phalangeal joints and implication of the interossei muscles. Very rarely has extension of the wrist and fingers been observed. In the foot the ankle joint is extended, the toes flexed and overlapping, and the attitude that of equino-varus. The knees are generally extended though they may be flexed, and the thighs are occasionally adducted and flexed. This spasm may be intermittent every few minutes or hours or days. Sometimes it lasts many weeks. It can be provoked by irritation. The muscles are and feel rigid. Attempts to move them passively usually cause pain. Of course their condition interferes with all use of the hands or renders the movements and gait awkward and difficult. The strength of the contractions varies between wide extremes. It may amount to a slight stiffening or an immovable tetanic contracture. In the intervals the sensitiveness of the parts, and even the paresthetic sensations, usually continue somewhat. They rarely disappear entirely with, or as promptly as, the spasm. Other muscles besides those of the limbs may be involved. In THE NEURONIC DISEASES 259 the severe cases all or any of the muscles of the body may be thrown into a state of rigidity. Swallowing and respiration may be interfered with. The ocular muscles have been affected and nystagmus has been noted. - FIGURE 40. & Position of the hands in tetany. (After Oppenheim.) The patient's consciousness is not, except in the rarest instances, altered. The temperature and pulse rate during the attack are usually somewhat elevated. At other times they have been seen to be sub- normal. I UK NEURONIC DISEASES The electrical phenomena of tetany are remarkable and some- times unique. They are usually referred to as Erb's sign. There is an exalted degree of excitability of the nerves to both the faradic and galvanic current. With the latter there is a complete reversal of the polar reactions to a remarkable degree, cathodal closing contraction equaling, and in rare instances even surpassing, that of anodal clos- ing. In the average case, only a weak current is required to produce a cathodal closing contraction. The current that would be needed in health to cause this response, produces in tetany a violent tetanic con- traction. Anodal opening tetanic contractions and cathodal opening tetanic contractions may be provoked. The latter is never seen in any other disease than this. For the sake of brevity, these reactions may be represented by and compared with the normal formulae, thus: Normal— CaCC, An CC, CaOC, AnOC. Tetany— AnCC, AnOC, CaCC or AnOC, AnCC, CaCC, AnOTe. \n( )TE and CaOTE are never seen in any other disease in man. Along with this electric hyperexcitability there is an increase of the mechanical irritability. As Chvostek's sign it consists of a vigor- ous contraction of the muscles, especially those of the face, when the relate;! nerve is slightly tapped or irritated. Trousseau's sign is prob- ably due to the same mechanism. It consists in provoking the spasm by compressing the nerve trunks or bloodvessels in the limb in such a way that the arterial or venous blood is impeded. Trousseau's sign is not observed in any other disease, though Chvostek's has been seen in tuberculosis and simple gastric dilatation, but less marked than in tetany. Increase of sensory excitability to both mechanical and electrical stimulation is known as Hoffmann's sign. That the sensory nerves are hyperaesthetic is shown by the radiating paraesthesia that occurs in their areas of distribution when they are compressed. The supra- orbital, ulnar and major saphenous nerves are available for this test. Chvostek noticed the phenomenon in connection with the auditory nerves, these nerves reacting to comparatively weak galvanic currents, whereas in health only about fifteen per cent, of cases react and then only to strong currents. A clang is observed upon anodal closing, when the anode is held still, and upon anodal opening. What Bechterew calls the reaction of excitation is the progressive increase of the mechanical and electrical excitability by repeated stim- ulation. ( )ther inconstant symptoms are met with. The association with hysterical and epileptic manifestations has already been noticed. Myxcedema and exophthalmic goitre have been seen with it. Even psychic disorder with hallucinations and confusion have been noted as in other similar toxic diseases. Some of the vasomotor and trophic exhibitions are quite prominent at times. Hvperidrosis, polyuria, albuminuria, glycosuria, cedema, urticaria, herpes, pigmentation and of hair and nails have all been recorded. There is nothing of moment in connection with the reflexes. Sev- eral times the knee-jerks were lost. Incipient cataract, believed b\ Peters to be due to obstruction of THE NEURONIC DISEASES 26l the circulation near the lens from spasm of the ciliary muscle, mydria- sis and pupillary rigidity are among the occasional ocular symptoms. General chilliness, fugitive pains, nausea, vomiting, diarrhoea, in- crease of temperature and pulse rate are seen in the beginning of the trouble, and even later, and are probably mere evidences of the general intoxication. Diagnosis. — The clinical picture is so unique in fairly typical cases that there ought to be no difficulty in arriving at the diagnosis. The attitude, the intermittent tonic symmetrical contractions in the hands and feet, the electrical signs, the probable history of infection or intoxi- cation, should all be given due consideration. Even when the spasm appears in the muscles of the jaw, trismus, tetanus can be differen- tiated by the fact that in tetany the trismus is a later phenomenon, the spasms are intermittent, and are more marked in the extremities than they are in tetanus. In the latter the extensors, supinators and adduc- tors are more apt to be affected ; in tetany the flexors, pronators, ab- ductors and internal rotators. The finding of the tetanus bacillus clinches the diagnosis. Latent tetany of childhood, tetany without spasm but with parges- thesia, Erb's and Chvostek's signs, and even a pseudo-tetanus of in- fancy have been noted. The differential diagnosis between tetany and spinal meningitis, hysteria, epilepsy and torticollis is not difficult if the characteristic electrical and other signs of the former are conscientiously sought for and the symptoms other than the motor are inquired carefully into in the latter. Prognosis. — In most cases the prognosis as to life may be re- garded as favorable. Bad signs may develop suddenly, however, at any time. Therefore, the cases are always serious. The accompani- ments of the disease vary the prognosis. Thus in cases with gastric dilatation, in those in which a goitre has been cut out, in those sub- jects exhausted and debilitated by disease and where respiratory com- plications are present, it is grave. Delivery and the cessation of lacta- tion usually terminate these forms of the disease. Infectious and intox- icating cases frequently run a mild and rapid course to recovery. Some- times the recovery is tardy and relapses are not to be unexpected, es- pecially when pregnancy and the function of lactation recur. The course of the entire disease may be from a few days to many months, even, without remissions, to many years. Treatment. — All possible causes and irritants should be removed as promptly as possible. Intestinal entozoa should be gotten rid of, the foetus should be delivered, tainted food should be washed up. The termination of the pregnancy in the pregnant cases, and the adminis- tration of thyroid extract in the absence of the thyroid gland, usually cause a disappearance of the disease. For the rest, we must depend upon rapid elimination, diaphoretic treatment in cases following cold, and the use of nerve sedatives. Free diuresis, catharsis, and diaphoresis cannot certainly be as dangerous as the disease itself is. Bloodletting is hardly commendable, or the icebag to the spine either, in view of the high probability of the dis- 262 I HI". NEURONIC DISEASES ease being a general toxaemia. The stomach pump cannot be used and in all manoeuvres the danger of provoking spasm must always be remembered. Warm baths and warm wet packs are both sedative and eliminative. Of the sedatives potassium bromide is the most available. It must be given in full dosage, 30 to 60 grains, three times a day. For a severe and extensive paroxysm morphine may have to be used. It cannot be employed continuously, however, for obvious reasons. It is in the continuous cases that the bromide salt is applicable. Hyoscin hydrobromate, gr. T-25, hypodermically every hour, until full medic- inal effect is obtained, has helped in some cases. Curare and curarine and phosphorus have their advocates. Sanger Brown has been pleased with the use of chloral and chloroform narcosis. When the laryngeal and respiratory muscles are threatened, chloroform inhalations are especially to be recommended. Administration, of course, must be careful, especially in the beginning. Pilocarpine subcutaneously will also temporarily relax the muscles. Look out for exhaustion and do not carry the use of either chlorofrm or pilocarpine too far. One wonders how electricity had ever been thought of as a thera- peutic agent in such a disease, and yet a stabile, mild galvanic current has the approval of good authority. The faradic current is decidedly contraindicated. General tonics like iron, quinine and cod-liver oil should be given between paroxysms, especially in children. Moynihan urges the early adoption of surgical measures in cases of severe gastric tetany, which certainly seems reasonable when there is a mechanical impediment to the free working of the organ from pyloric stenosis. LOCALIZED MYOSPASMS. A spasm is a symptom. It is an involuntary and abnormal con- traction of a muscle, due to disease. It arises out of a multitude of conditions involving the entire neuro-muscular apparatus. Spasms may be classified upon an anatomical or a functional basis. As the anatomy and functions of the entire neuro-muscular apparatus are very extensive and complex it is obvious that such clas- sification must prove chaotic and confusing. This is just the condition of most of the classifications of this symptom at the- present day. And it can hardly be expected to be improved very much until we learn more of the patho- logical origin of the trouble. It is uesless to form a classification with such gn i.ss anatomical divisions as the head, the neck, the trunk and the. extremities for its guiding principle, for spasms in one part are very generally accompanied b) spasms in another part. It is generally futile to classify them in accordance with the particular nerves involved, for not always are the same nerves implicated nor is the spasm always a nerve trouble. In the production of a muscular contraction many structures are concerned. They are the muscular tissue itself, the neuraxone, the motor neurone-cell and under most circumstances the related sensory neurone with all its peripheral connections. In the THE NEURONIC DISEASES 263 voluntary and semi-voluntary muscular contractions the centers that subserve volition and the other higher psychic acts must also be taken into account. Thus it is clear that a normal muscular contraction is by no means a simple affair. Disorder of any part of this complicated mechanism may very reasonably provoke a disorder of the resulting contraction which may assume the character of an over-contraction. Direct irritation and withdrawal of inhibition are the two forms in which the primary disorder occurs out of which the spasm is evolved. The irritation may be in the muscular or the nervous part of the mech- anism. Disease of the brain and other inhibitory centers may cause certain sensory-motor mechanisms to be set free, as it were, to func- tionate in a manner far in excess of the normal. It is not correct, there- fore, to say that spasm is a symptom of spinal cord disease. It may be a symptom of disease in any part of the sensory-motor neuro- muscular apparatus. This at once suggests that these spasms might be classified into those due solely to muscular disease and those due to nervous disease. The latter could again be subclassified into those that are dependent upon lesions in the motor, the sensory or the cere- bral centers that subserve psycho-motor-sensory functions. We already use this classification for some of the spasmodic manifesta- tions and were we in possession of complete data m regard to all of the diseases in which they appear we would probably find that this classi- fication would prove the best. We distinguish even now between the spasmodic conditions of cerebral irritation and cerebral destruction. We recognize in our nosology a whole host of sensory reflex spasms. We acknowledge that psychic spasm may occur in hysteria and allied states. In a word we have conceived of an anatomical basis for most of the spasmodic conditions that we meet with. For want of a better term we call these spasms sometimes symptomatic, which means merely that we are fortunate in knowing just about what the lesion is that is causing all the trouble. These spasms being but symptoms of well recognized diseases it does not seem to me necessary to describe or refer to them again in this chapter. They will be found treated in detail under the head of the respective diseases wherein they appear. The spasms of which we have as yet no definite knowledge in re- gard to their pathogenesis but which are well recognized clinically will be considered here. For physiological reasons already spoken of, it will be assumed that in these spasms there is a functional disturbance which may in- volve the muscular tissue or the central neurone-bodies. The term functional has a broad meaning here. It may be allowed to indicate a nutritional, or at least, a physio-chemical, change of some sort in these elements or a disturbed innervation among them whereby they permit of unrestrained activity in the muscles. From this point of view the spasm that is not due to inherent mus- cular disease is a primary neurosis. In these spasms heredity, <>r in other words the neuropathic constitution, nutrition, infection and trau- mata play the largest etiological role. These spasms may be at times excited by reflex stimulation but they are not pure reflex spasms. They are generally accompanied by j(,4 I HE X ILL' RUNIC DISEASES other signs of a neuropathic state and very often by another distinct neurosis like hysteria or neurasthenia. In taking up for consideration then those spasms that are them- selves of the nature of pure neuroses, I discard all those that are symptomatic of, or form merely a part of, the symptom-complex of well-recognized diseases discussed in other parts of this book. I realize the risk I am running in possibly including here some spasmodic affections that may be merely symptomatic, but the uncertainty of our knowledge as to their real nature may serve as my apology for taking the step. These spasms may be regarded from the standpoint of their clin- ical appearance, which is useful incidentally for purposes of descrip- tion but which should never have been adopted as a basis for classi- fication as it unfortunately too often has been. They will be spoken of as tonic and clonic, as belonging to this group or that group of mus- cles, as rapid or slow, as coordinated or incodrdinated, as involving parts of the muscles or the whole muscle. Walton rightly urges that a more careful use of terms in describ- ing these spasms should be insisted upon. By way of illustration he recommends that the term myoclonia should be dropped as a collective designation for unrelated disorders, in which I heartily agree with him, and that myokymia should be limited to cases showing, without hereditary or congenital history, widespread muscular quivering with- out indication of progressive degeneration of the nervous system, with- out constitutional symptoms and without signs of present infection or other acute disease. And so on. It is not possible in the present state of our knowledge to always separate an essential myospasm from one due to some functional or other nervous trouble, any more than it is possible to separate an es- sential progressive muscular atrophy from a low grade chronic anterior poliomyelitis. It is not improbable that we will come to believe, as many are believing in regard to the atrophy and anterior horn trou- ble, that there is no sharp line of demarkation ; that functional muscu- lar disturbance is always more or less accompanied by a related neural trouble. L( >CALIZED SPASMODIC TIC. TIC CONVULSIF. A quick, sharp, short, electric-like, painless contraction in a muscle <>r group of muscles is known as a spasmodic tic. It may occur in any muscle of the body, though it is most frequently seen in the facial mus- cles, probably because they are the most active and intimately con- nected with mentalization of any in the body. \ tic may consist o\ one contraction. Usually there are several in rapid succession, after which there follows a period of quiescence. Sometimes a single nerve is involved or even single branches of a nerve. \\-r\ often, however, a number of nerves, nearby or widely separated, may be subject to the trouble. As a result different mus- cles, but always muscles concerned in the production oi related move- ments, an- contracted. THE NEURONIC DISEASES 265 The disease is a chronic disorder, has usually a neuropathic his- tory behind it, and occurs frequently in association with hysteria, epi- lepsy, and other neuroses and psychoses. A nutritional or molecular change of some unknown character in the nerve nucleus or its representative center in the cortex is assumed as the pathological basis of the trouble. These localized tics are often miscalled local or habit chorea. They have nothing in common with the latter disease and the manner of the muscular movements is not the same. The tics should always as far as possible be sharply differen- tiated from mere reflex and symptomatic spasms of the same muscles. The results of their treatment respectively are very different. The tics are not very amenable to treatment and in their management dis- ciplinary and psychotherapeutic measures have a large share. They represent a fundamental, hereditary, general nervous defect or the neu- ronic, residual change after the operation of some acute cause or dis- ease. They speak always for more or less central degeneration and are therefore apt to be of wide distribution or concerned with mus- cles and nerves that subserve general physiological movements. They may, however, implicate individual muscles and nerves. The facial tics are the most common and most marked. The rea- son, as I have said, is because the facial reflexes are so rapid and so closely linked with mentalization. The psychogenic origin of them in many instances is shown in the way that they suddenly begin after a high emotion or mental shock. Most of the victims are more or less mentally undergrade, or are at least neuropathic. A vicious habit may evolve the trouble. The trouble is only very rarely obtained by direct inheritance. They may resemble and even originate as an occupation neurosis. I saw such a tic that occurred in a young cornetist with both a family and an individual pronounced neuropathy. Any of the muscles of the face may be affected, and the symptom may be bilat- eral, though it is usually unilateral. Single muscles and groups of muscles may be involved. Perhaps the most frequently affected is the orbicularis palpebrarum and the least the orbicularis oris. Tn fact there is no rule and every possible form of the exhibition may be looked for. The occipito-frontalis, the chin muscles, even the external auricular muscles have each been seen to be affected by themselves. Both tonic and clonic have been the contractions, though the latter are the more usual. Mental and physical rest diminish the jerks, whereas excitement, effort and voluntary movements increase them. Cutaneous irritation usually evokes them. Sometimes they can be par- tially controlled by the wili. These spasms do not impair much the ordinary voluntary movements of the face, and as a rule they do not cause much subjective distress, sometimes not any. Hearing phe- nomena may occur when the stapedius muscle is affected. The velum palati and uvula have been supposed to be implicated, though these are more likely to be due to some gross organic lesion which may be diagnosed by collateral symptoms . There are no sensory, vasomotor, trophic or secretory symptoms. The objective signs, except the eon- tractions, are all negative and the trouble appears to be a pure neurosis. 266 THE NEURONIC DISEASES Of course every case of facial spasm is not a pure tic. Organic lesions of the nerves and their representative centers may cause facial spasm. Such cases must be always differentiated and in every case it is well to hunt first for some such organic cause. Tic patients are often the victims of hysteria, epilepsy, hemicrania, hystero-neuras- thenia and the psychoses. Stuttering is usually a form of tic, though it may have had its origin in a vicious habit. Tic may be spoken of as mimic tic but it is entirely wrong to name it chorea as is so often done. Facial tic is a chronic, sometimes remittent trouble, with a gen- erally unfavorable prognosis as to cure. Blepharospasm holds out the most hope of amelioration. No treatment but general tonic and disciplinary treatment will do any good. I have seen arsenic cause a bad blepharospasm in a boy almost entirely to disappear for a time. Bromides may help some. All other remedies usually fail. If the spasm be not a true tic, but a nerve reflex or symptomatic trouble, the treatment is both obvious and prom- ising. If tic douloureux be present with the motor tic it must be treated as a separate condition. A masticatory tic is sometimes seen that involves the muscles of mastication supplied by the motor branch of the fifth nerve. Trismus, hysterical, rheumatic and reflex spasms of the lower jaw must all be carefully differentiated. The reason that the prognosis of most of the cases of masticatory spasm is favorable is that they are not cases of true tic but are due to inflammations about the temporo-maxillary articulation, to diseased teeth, to hysteria, etc. What is said of masticatory tic is true of occasionally observed hypoglossal tic. The tongue here shoots rapidly in and out of the mouth, or from side to side.- The spasm is generally bilateral but a hemispasm has been seen. A tremor in the glossal muscle and the spasm of some neighboring facial muscles have accompanied this tic. Alternation of masticatory and hypoglossal tic is a curious phenome- non. Sometimes the trouble has resembled an occupation neurosis com- ing on upon attempts at mastication, cornet-playing, etc. Tics occur in the muscles of the trunk and of the extremities, but they are rare. Other forms of spasm such as occupation neurosis, re- flex, organic and symptomatic spasms, are often enough seen and must be clearly separated, both on account of the prognosis and the treatment. The causes of the spasms here that are not of the nature of true tics are innumerable and consist of trauma, local irritations, overexertion, varicose veins, and intoxications. Neurasthenia, hys- teria, epilepsy, chorea, writer's cramp and other neuroses may be pres- ent as a rule. Spasms of the trunk and limbs therefore are not un- favorable from the viewpoint of cure, for they are so seldom essential tics. Respiratory ties are various, always troublesome and sometimes very serious. Singultus, or hiccough, may be of this nature, and may or may not prove fatal. Tt is usually a mere reflex trouble, however, short in duration" and not alarming. The diaphragm and its innerva- tion is of course the seat of the phenomenon. Yawning and sneezing are scarcely ever of such a character as to be regarded in the lisrht of THE NEURONIC DISEASES 2t>7 disease. I recently treated a young man, with a syphilitic history, who suddenly began to have uncontrollable yawning spells that so alarmed him as to drive him to seek medical advice. Neurasthenic, hysteric, •epileptic individuals and those with organic brain trouble, meningitis, cerebellar abscess have been troubled with abnormal yawning. As an aura of epilepsy it also occurs. A snoring spasm of psychic origin has been recorded. A cough that is not of reflex or inflammatory orig-in is apt to be hysterical. Counter-irritation, internal sedatives and electrization of the phrenic nerve are the chief measures that we possess for combating these respiratory spasms. Fortunately they are rarely prolonged or serious. Closely related to the localized tics, if it really be not one of them, is the form of torticollis known as spasmodic zvryneck. This is not the same as congenital torticollis with its shortening of the sterno- cleido-mastoid muscles due to obstetrical injury or prenatal atrophy; nor as symptomatic wryneck with its rheumatic, inflamed muscle, tu- mors, abscesses, syphilitic lesions and other easily discovered causes ; nor as spurious wryneck due to mild caries of the spine. Spasmodic Torticollis is a central neurosis and is associated with the hysterical, neurasthenic and generally psychopathic constitution. The muscles supplied by the spinal accessory nerve are the ones usu- ally, or at least prominently involved. Other muscles, however, and other nerves are likely to be implicated. Heredity exists in most of the cases though it is rarely direct. It involves more especially a neuo- pathic taint. Women are much more subject to the trouble than men. It occurs in the middle period of life and often follows traumata of various sorts, extreme emotional excitement and sometimes no appa- rent exciting cause. Stigmata of degeneracy are sometimes seen. The disease is a central neurosis of the medulla, more probably of the representative centers for the neck muscles in the cortex. It is a degenerative trouble and resembles an occupation neurosis in being a defect in the coordinating mechanism for the movements of the neck. In this form of the disease the spasm is generally of the clonic type, tonic spasms being more characteristic of the other forms of torticollis. Irregular impulses are sent out from the degenerating and defective centers which cause the muscles supplied by particular nerves to undergo irregular and intermittent contractions. The stemo-cleido-mastoid muscle is the leading one to be involved, Dut others are very often implicated with it. The combinations thus effected are quite numerous. Some of them have received special names because they lead to special movements and attitudes. The most common combination is that of the sterno-mastoid and trapezius mus- cles. The splenius, scaleni and deep cervical muscles, platysma and omohyoid all may be affected. In its course the disease passes from one muscle to another. In the early days when this disease was not differentiated sharply from other forms of torticollis, especially the congenital form, tenotomy would be done on one muscle after another as they contracted after -each operation. 268 THE NEURONIC DISEASES The attitude of the head and shoulders will of course depend upon, the location of the spasm. When only one sterno-mastoid muscle is affected the face is turned towards the other side, the mastoid drawn down and the chin elevated. Contraction of the trapezius, usually the upper part only, which is supplied by the accessorius, causes the head to be rotated towards the side of the muscles affected and drawn strongly backwards. An alternation of contractions between the sterno- mastoid and trapezius muscles causes a sort of circular movement of the head that is very characteristic. Other movements are produced when other muscles such as the complexus, superior, oblique and splenitis are implicated. In most cases the inferior oblique, recti capitis postici, major and minor, are included and show involvement of the upper cervical nerves. Less frequently the contraction is bilateral. Retro-collic spasm is a drawing of the head directly backwards by the two trapezii. It is a rare condition. Spasmus nutans, salaam spasms, epilepsia nutans, the nodding spasm of children, is occasionally a neurosis of the eleventh nerve though it is more often a dentition reflex or due to digestive disorder, basal meningitis and gross brain disease. It may be associated in such cases with transient loss of consciousness and thus resemble or be accompanied by true epilepsy. Often it is a mere habit and is a veritable tic. It is revealed in a rhythmical nodding or oscillating of the head of various degrees of rapidity, even up to fifty and sixty times a minute. The eye and facial muscles often participate in the salaam. Nystagmus, strabismus and blepharospasm may thus be a part of the symptom-complexus. In time the muscles undergo hypertrophy in some cases. An atrophy of the opposite muscles, as observed by some, is probably due- to neuritis or other gross damage. The same may be said of the- pain that is occasionally present. A genuine tic does not have pain with it but the muscles may feel sore and ache. The spasms as a rule cease during sleep and are increased by mental excitement and fatigue. They are clonic and recur every few minutes so that it is impossible- to keep the head still long. In typical cases there are no objective signs of organic, motor or sensory changes. The psychic accompani- ments of the trouble occasionally include mental confusion, delirium and melancholia. The diagnosis calls for a sharp differentiation of spasmodic tor- ticollis from the other forms. The shortness of the sterno-cleido- mastoid and the fixity of the deformity indicates congenital torticollis. Tain, fever, local tenderness belong to the rheumatic type of the dis- ease. The usual signs of caries of the spines will aid in excluding the- spurious type. All forms of torticollis should, however, be most min- utely examined for possible reflex, traumatic, inflammatory or other source of the spasm. They must be excluded, of course, before a diag- nosis of spasmodic wryneck is permissible. General tic and paramyo- clonus may first appear as torticollis. Facial tic, echolalia, define the former, whereas associated spasm of the great trunk muscles, bilateral in distribution, is the determining feature of the latter. There is noth- ing choreic aboul torticollis and if choreic symptoms are present, the THE NEURONIC DISEASES 269 patient has the two affections simultaneously, just as he may have tor- ticollis with hysteria, epilepsy and other psychoneuroses. The prognosis and treatment follow the lines of that of all the tics. I would reiterate and urge especially again here that a most careful diagnosis be made to eliminate all possibility of the disease be- ing of any of the other forms than the spasmodic. When this is de- cided upon, only quiet and rest, sedatives and psychotherapy will do any good. I have, like most of my confreres, fallen into the error of recommending operative procedures but I have learned not to do so any more. The spasm invariably returned, though in some cases there was a grateful period of rest. I have seen it immediately reappear in other muscles. I have not been so unfortunate as to have seen any case made worse by operation, though such observations have been made. Electricity does no good except for its psvchic influence. Re- lapses follow it and cause disappointment and mental depression, which are bad for the neurosis. Apparatuses to hold the head fixed aie a mere useless torture. General tonic treatment, change of climate, change of mental stimulation, change of general regime and environ- ment with such occasional sedation as bromides and opiates can give, are as effective as any measures we can institute. The cures some- times reported as the result of operative and other mechanical meas- ures are doubtful because the disease itself is subject to remissions, and because most of such cases have not been observed long enough after the reported recovery. Of course the therapeutic story is an entirely different one for the other forms of torticollis. Tenotomy is the thing to do in con- genital torticollis. Antirheumatics, antiphlogistics, counter-irritants and later on electricity, massage, gymnastics, fixation apparatuses, setons, nerve stretching and nerve resection are all to be employed and with a legitimate hope of success. Here as elsewhere essential pri- mary neuronic degenerative troubles are beyond the power of medicine ■except so far as the latter can influence their nutrition. The extra- neuronic troubles that irritate and damage the neurones secondarily •are amenable to treatment — all except the damage to the neurones. The discussion of the other forms of torticollis will be found un- der the head of diseases of the special nerves (accessorius) in another part of the book. GENERAL TIC. TIC IMPULSIF. This is a remarkable psychonenrosis originally described by Guinon and Gilles de la Tourette and often known as the latter's dis- ease. Other names that have been given to it are maladie des tics con- vulsifs, maladie des tics impulsifs, myospasia impulsiva. Gray sug- gested the name palmus. Though the disease is confused by some with chorea it is really a psychoneurosis more nearly related to hysteria than to anything else. Its spasmodic exhibitions and their inherent neuronic origin make the discussion of the trouble in place here. When Gilles de la Tourette first described the affection in [885, he 27O THE NEURONIC DISEASES stated that first there appeared a motor incoordination which does not affect the general health at all but involves the face and upper extremi- ties gradually, the latter usually one-sidedly. Winking, drawing of the buccal commissures upward and outward, contraction of the mas- seters with grating of the teeth, projection and occasional laceration, of the tongue are some of the facial manifestations. The neck muscles become involved and there is alternate flexion and extension of the head with sometimes inclination to one side or the other by the sterno- mastoid spasm. In the upper extremities the fingers are flexed and extended and the shoulders raised and dropped. Soon the lower limbs participate in the extraordinary performance. In them the incoordi- nation is more extensive and involves larger masses of muscular tissue. It is unilateral or bilateral. The foot is stamped on the ground or the patient stoops over and suddenly becomes erect, or jumps off of the floor. The movements are all sudden, rapid and serial. During" these strange, violent antics, the inarticulate cry, like the sounds "hem" and "ah," occurs and is frequently repeated. Sometimes the sound is articulate and loud and resembles some particular word in each patient's case. Being repeated echo-like, the symptom has been termed echolalia. Oaths, obscene and profane epithets are often emitted,. coprolalia, a characteristic which Gilles de la Tourette thought was pathognomonic. Sometimes a gesture made to or before him will be imitated by the patient, a symptom denominated echokinesis; or he will suddenly and involuntarily give voice to some of his most secret thoughts, tic de pensee. Involuntary, irregular, violent incoordinated movements of par- ticular parts of the body, or of the whole of the body, explosive utter- ances, and obsessions or fixed ideas constitute the clinical complexus of Gilles de la Tourette's disease. It is believed that the French authors had their attention drawn to this singular hysterical trouble by the publication of Beard in 1880 upon "The Jumpers of Maine," and of Hammond upon the "Miryachit" or Meriatschenje of Siberia and Kamchatka. The Malayan Latah is the same thing, as no doubt is the saltatory spasm of Bam- berger, mentioned under hysteria. There is a neuropathic taint behind this general tic and it is most often seen in children between the ages of seven and fifteen. In rare instances the heredity has been direct. An outburst of emotion, a trauma or an infectious fever has been in some cases the starting point of the manifestation. Being a hysterical condition the patients are aware of their condition but they cannot help it. In fact they gladly yield to it to get rid of the irresistible impulse. It is a lifelong affliction as a rule though not absolutely incurable. Remissions occur fre- quently. The affection must be differentiated from localized spasmodic tic. from chorea, from hysterical spasms and paramyoclonus. A careful consideration of its complete symptomatology will make the differen- tial diagnosis easy. Medicinal treatment, as might be expected, proves to be futile. Chloroform inhalations, bromides, chloral, mav be useful during the THE NEURONIC DISEASES 27 1 attack to secure temporary respite. The same general treatment used for hysteria, with complete isolation as a prominent feature in it, is to be adopted here. PARAMYOCLONUS MULTIPLEX. This is a rare affection, first described by Friedreich. He gave it the above name. It has since been called, however, by various names, such as myoclonia or myoclonus multiplex, polyclonia, convulsive tremor, myospasia. It is characterized by bilateral clonic spasms which involve whole muscles or groups of muscles that belong en- tirely to the trunk, or are partly attached to it. We know so little about the disease that it is difficult to give it a positive classification. Not all of the cases that have been described as paramyoclonus multi- plex have corresponded exactly with the clinical picture presented* by Friedreich's case. This was a phthisical man, 50 years of age, who two weeks after a fright felt a stiffness in his body and developed a clonic spasm, ten to fifty a minute, in rhe upper arm muscles and mus- cles of the thigh, especially the quadriceps. The spasms were bilat- eral, were lessened by voluntary movements, occasionally ceased for a quarter of an hour and were absent during sleep. Reflex action and myotatic irritability were both increased. Galvanic electricity banished the spasm for a time but it soon returned. There were no findings in the muscles or the spinal cord. Since Friedreich presented and named this case others have been described that are extremely sug- gestive of tic, of hysteria and of electric chorea. As has well been said the moment we diverge from the clinical picture outlined by Fried- reich we get close resemblances to other neuroses. Because some writers have freely admitted other phenomena under the name para- myoclonus multiplex we find it extremely difficult to know just where to place the disease. Etiology. — Men are more often the victims of the trouble than women. It occurs chiefly in adults. A slight hereditary and familial influence seems to have been behind some cases. Weiss noted the trou- ble in four generations, Gucci in three generations. Its association with hysteria, neurasthenia, epilepsy and other familial neuropathic tendencies has often been commented upon. It has followed fright, ex- treme emotion, severe strain, traumata and infectious disease. Pathology and Pathogenesis. — We know 01 no pathological changes. The disease seems to be a violent spinal neurosis of some sort. Further than this we cannot go, and even this is but a tenta- tive statement. There are probably different types of the trouble or it may be but a symptom, variously modified, of other well-known dis- eases. It is certainly difficult to give the disease a perfect nosological independence. Mobius and Strumpell have doubted the independence of Friedreich's own form of the trouble. The former identifies it with chronic chorea, but his views are not generally accepted. Raymond groups it with electric chorea, with which Oppenheim identifies it. Others believe that it is merely a form of hysteria though it is usually wanting in the sensory, but has revealed seme of the psychic, stigmata of hysteria. 2"J2 THE NEURONIC DISEASES Symptoms. — Here again we meet with a confusing diversity of opinion. Just what symptoms should be included under the name of paramyoclonus multiplex has not yet been entirely agreed upon. There is no dispute about the location of the spasm bilaterally in the muscles of the trunk or those that connect the trunk with the limbs. It is not accepted that the symptoms can be limited to the limbs. The spasm may begin on one side of the trunk, or it may be more marked on one side but its bilaterality is one of its chief characteristics. The spasms are clonic and may be violent, sharp lightning-like, throwing the patient of! his chair and flexing and extending the body, or they may be so fine, delicate and tremor-like that they scarcely move the part. They rarely are sen by a monotonous, excessive or faulty manner of using the muscles concerned in certain special, complicated and coordinated movements THE NEURONIC DISEASES 273 and shown by the inability to perform these movements without the loss of other movements involving the same muscles. If the use of the adjective functional be applicable at all to disease, it certainly is ap- plicable to this disease above all others. An occupation neurosis is not only functional in origin, but is apparently itself nothing but a disturbed function. It must not be imagined that every sort of inability to per- form muscular movements that are caused by occupations are to be classified under the head of this neurosis. Many lines of work involv- ing cramped positions, pressure upon particular muscles and nerves, intoxication as with lead, or direct traumata may set up a neuritis or other pathological condition that will decidedly interfere with coor- dinated movements. These are not instances of the disease now under consideration. This is shown in various ways, but in none more em- phatically than in the fact that the prognosis of such is relatively fav- orable, whereas the prognosis of the occupation neurosis is decidedly unfavorable. As the disease occurs in connection with a great number and variety of occupations chiefly engaging the hands, a large number of names have been given to it. Writing seems to provoke it more than anything else and it has more particularly been studied since the intro- duction of the use of the steel pen in the early part of the last cen- tury. It is commonly called therefore writer's cramp, or scrivener's palsy. Occupation spasm, fatigue neurosis, coordinated occupation neurosis, professional neurosis are some of its synonyms. An inter- minable list of names for it 'is made up of those describing the partic- ular form of occupation in which it occurs, as for example, pianist's cramp, telegrapher's cramp, milker's spasm, sewing spasm, etc. Writ- er's cramp, graphospasm, mogigraphia, in a word, so-called occupa- tion neurosis, is a distinct disease, and should always be carefully differ- entiated from other conditions that may at times closely resemble it. Etiology. — The etiology has some remarkable features about it that are extremely suggestive. A neuropathic constitution and a par- ticular occupation strain cover the main points in regard to its causa- tion. The disease is rarely seen in one who inherits a perfect nervous constitution. In some cases there is a distinct nervous heredity, actual disease having appeared in the immediate ancestry ; in most of the cases there is merely a transmission of a neuropathic predisposition. I have never seen a case in which I could not trace this neuropathic inheritance more or less distinctly. Neurasthenia, hemicrania, epilepsy, neuralgia, convulsive tic, phobia, and tabes have been seen and recorded in association with it. Worry and intemperance that are said to predis- pose to it may be but a revelation of the same neuropathic basis upon which it likewise rests. The artistic temperament is generally neu- rotic, and this neurosis occurs frequently among artists when they overwork their muscles upon musical instruments. Several members of the same family have been stricken with the trouble. The influence of a nervous temperament cannot be doubted. Men are more frequently affected than women probably because the}- are more exposed to the overexertion, by reason of business life that brings it on. They suffer especially from the motor form of the trou- 274 I HE NEURONIC DISEASES ble. It occurs at all ages, though by far the majority of cases are seen between the years of twenty and forty. That there is something behind the disease of a constitutional nature that provokes it in addition to the mere overuse of the muscles is shown by the remarkable fact that in some occupations wherein women predominate, the men are still the greater sufferers. For ex- ample, Lewis has found that in this country telegrapher's cramp was seen in only four women out of forty-three cases, though women are employed in telegraphing in a much larger proportion to men than these figures would represent. The exciting cause of the disease is the overuse of a particular set of muscles. This is perhaps not a perfectly accurate way of stating it. As these same muscles respond to the will in other forms of move- ment than that which brought the trouble on, it should be stated that an excessive, monotonous employment of a particular form of com- plicated, coordinated movement causes it. This presents the affection in an entirely different light and begins to let some illumination in upon its manifest connection with a neuropathic temperament. It is the overuse of a movement, and not merely of the muscles, that underlies the trouble. Movements are primarily of brain or mind origin, whereas mere nerves and individual muscles are represented in the spinal centers. This etiological fact that complicated, coordinated movements rather than mere nerve or muscle activity is the cause of the occupation neuro- sis sharply differentiates this disease from the affections that simulate it but depend upon poliomyelitic and neuritic changes. Not every kind of movement provokes it, but those painfully and slowly acquired, educated movements, that are highly complicated and require an extreme degree of a novel form of coordination, that were and are purely voluntary, but by practice have grown to be automatic, and that bring into play an unwonted, antagonistic set of muscles. In writing, playing musical instruments, milking, beating a drum, manipu- lating a telegraphic key, there are several combined acts being per- formed at the same time and under the same strain. Not only is firm prehension exercised by some muscles while others at the same mo- ment are performing the required movement, but both are under an unusual tension that at first consciously and later unconsciously gives unwonted exercise to the cerebrum and the central coordinating appa- ratus. In writing, for instance, the pen must be held more or less firmly and it must be properly moved by both fingers and the arm. This is a compound, complicated act therefore. It means a remark- ably sharp disassociation of the movements of different sets of mus- cles all in the same extremity and at the same period of time. This requires an extraordinary adjustment of function between different of motor centers in the cord and brain. It opens up new path- ways of inter-communication between these centers and by the nature of the process puts these new pathways, and with them the new coor- dinating apparatus, into a condition of severe strain. Exhaustion takes place and though the associated motor centers may operate and cause the muscles to contract in other ways and along other coordinating THE NEURONIC DISEASES 275 paths, they cannot do so along these that have become thus wearied. That the mere overuse of the muscles alone is not the cause of the trouble is shown in the fact that neither authors nor stenographers who write a very great deal are victims to any great extent. Copyists and those who use a cramped hand in writing and pens that prevent a free movement of the arm are the great sufferers. Resting the hand on the little finger and moving the pen up and down with the other fingers while the arm remains comparatively quiet and poised seems to be a particularly dangerous method. Sharp steel pens require so much more stiffness and firmness in movement than do soft, loose quills that they have been in fact justly blamed for the trouble. The style of writing, the intensity of it caused by hurry to get through, the instrument employed and in fact everything that increases the demand upon the coordinating apparatus are etiological factors. To this must be added, of course, the monotonous exercise. What is said of writing applies with equal force to all other occu- pation movements from which the disease has developed. In the irreg- ular yet carefully regulated movements of the telegraphing arm and hand, we observe a strained coordination of a highly complicated character. The pianist, the violinist, the flute-player, the milker, the drummer, the cigar-roller, the seamstress, the tailor, the cobbler, the dancer and others are usually employed in an occupation that upon the face of it shows an unusually fine coordination between various com- bined and antagonistic groups of muscles. At first this coordination was practiced with difficulty. It had to be learned. It tends to become automatic, but has not yet reached the degree of complete automatism. It is subject to weariness and to depression if the guiding cerebral centers are vitiated by heredity and other predisposing factors. Mo- notonous and excessive exercise of it breaks it down. Local inflammatory conditions in the hand, injury, wet and cold, albuminuria, lead-poisoning and other causes of neuritic degenerative conditions have been blamed for it, though it is a question whether these cases should be regarded as typical instances of the disease. I am sure some of the latter are but cases of neuritic incoordination, spasm or palsy and are no more to be regarded as cases of occupation neurosis than the ataxia of tabes is to be regarded as the same as psy- chic vertigo. Emotion is an etiological factor. I ' athology and Pathogenesis. — No pathological changes have been found in the few cases that have been examined post-mortem. It will not do, however, to say with Oppenheim that none will probably ever be found and it is entirely a misconception of the disease to attrib- ute it to the neuritis and poliomyelitis evidenced in some supposed cases of the disease that were examined pathologically. It is undoubt- edly a functional trouble, but functional troubles must necessarily spring out of some minute nutritional changes, albeit the latter are too fine for our modern means of investigation. Consider for a moment the etiology of this strange disease. Note how a tendency, a neuro- pathic tendency, born in the individual or fostered by his environment, plays a leading role among its predisposing causes. Note the diseases 276 THE NEURONIC DISEASES with which it has been associated; neurasthenia, functional spasm, epi- lepsy, hemicrania, hay fever, neuralgia, chorea and a tendency to want of all self-restraint, sobriety and regularity that go with a strong and healthy mentality. Observe its frequent association with emotional states, with wasting diseases, depressing traumata and a mode of life that is unhygienic in its surroundings and duties. The background upon which the disease portrays itself is suggestive indeed and makes one think involuntarily of the hysteroid and other mild psychoses and subconscious, cerebral exhibitions. After considering the etiology of the disease, observe for a mo- ment how it reveals itself. It is preeminently a motor trouble. The disturbance occurs in a variety of movements that are of late acquisi- tion, are educational, are striving to become automatic and are already partly so, and are the result of a most complicated and refined form of coordination that is too coarse to be wholly cerebral in origin and yet not coarse enough to be spinal, but just of such a character to be sub- conscious, subpsychic, like some of the phenomena of hysteria, phrenas- thcnia and other forms of cerebro'-psychosis of the subconscious order. Place this beside the fact that the disease is not essentially neural or muscular because other movements can be readily made by the same muscles, as witness the disabled telegraph operater writing with great ease and fluency ; beside the fact that none of the usual signs of mus- cular or neural disease, electrical or otherwise, are observed in typical cases ; and finally, besides the fact that the prognosis is bad and the treatment discouraging, it seems to me we have enough data to sug- gest that the trouble is essentially central in origin, is a subconscious cerebration and is a functional weariness or exhaustion of the coordi- nating apparatus. It is easy to understand of course that the excessive use of the muscles which provoke the disease, but which does not represent it in its essential nature, for it is not muscle exhaustion so much as it is exhaustion of the central inhibitory, coordinating apparatus, may in a reflective manner cause atrophic conditions and even occasional fugi- tive nenritic and poliomyelitic changes of a slight character. Exhaus- tion of functional coordination with certain variable secondary changes of a grosser nature constitutes then, so far as logic can demonstrate to us in the absence of more positive physical findings, the pathogene- sis of the true occupation neurosis. Here again it is shown that this disease must not be confounded with the spasmodic pareses that often simulate it but that owe their origin to some limited form of muscle or nerve disease such as myositis, neuritis, poliomyelitis. SYMPTOMS. — The onset of the trouble is always gradual. It is first noticed that the use of the hand is becoming uncertain and be- yond the delicate control of the will. Not only does the writing seem to grow more difficult but it begins to change in character. There is a tremulousness about it. a little jerkiness, a disinclination to pro- ceed in the old regular way. Sometimes a feeling of soreness or weight or slight pain is felt in the fingers, up the arm and between the should- der blades. A little writing may still be done normally, but lengthy THE NEURONIC DISEASES 277 use of the pen produces a feeling of extreme tire and a stiffness and unnecessary rigidity in holding. The symptoms of the fully developed disease are necessarily varied because the many occupations in which this neurosis occurs demand the use of many different sets of muscles and nerves. There is no fixed clinical picture therefore for the neurosis in general though there is more or less of a definite manifestation in each special occupation. There are, however, three well-recognized types of the trouble, namely, the spasmodic, the paralytic and the tremulous. When there is much pain present, the type is referred to as neuralgic. So much more prom- inent are the motor symptoms that the affection may well be looked upon clinically as a motor neurosis. In writer's cramp the attempted use of the muscles at once de- velops a spasm. They become stiff and seize the pencil and the move- ments are awkward and uncontrollable. Sometimes other muscles than those merely necessary to perform the act of writing participate in the spasm. The left arm has thus exhibited an associated condition. More often when the left arm is employed to relieve the affected right, the latter immediately becomes spasmodic. More rarely even the legs have been seen to be spasmodic when only the writing with the right hand provoked the trouble. A hemispasm of the entire body has been reported and suggests strikingly the seat of the primary lesion. The spasm in the fingers is tonic and affects the muscles concerned with the performance of the act. Some authors insist that the latter is an essential indication of the disease, but Lewis does not. The flexors of the fingers, more rarely the extensors, are contracted. The thumb and index finger are especially marked by the spastic weakness. The thumb may be controlled and pressed firmly into the palm while the fingers are forcibly separated. Sometimes the spasm is so promiscuous and spread about that the whole hand seems to be in a state of riotous movement. As it begins to appear the patient changes the method of holding his pen and thus all sorts of bizarre and even ludicrous posi- tions are adopted in order to be able to go on with the writing. Per- sistence in the writing even with the new attitude causes the spasm to appear in other muscles so that the work has to be given up entirely. A condition almost of tetany may develop, with contraction of the flex- ors of the wrist as well as of the fingers upon the slightest attempt to use the muscles for writing or any other purpose. Mitchell described a lock-spasm in which the contraction of the fingers and hand was so violent for a time that the latter seemed to be locked. When the spasm wears off the muscles relax — feel a little tired and weak. In some cases there is during the spasmodic condition severe pain in the muscles, bones and joints. A significant fact is that the more the patient worries over his inability and the more frantic his efforts are to overcome it, the worse the disturbance is both motor and sensory. The paralytic form of the disease is less common than the spastic. Like the latter it comes on gradually and has other symptoms occa- sionally associated with it. The fingers, hand and arm are subject to a feeling of intense weariness and when writing is attempted they re- main immobile and like a dead weight upon the table. The will seems 27* THE NEURONIC DISEASES to have lost all power over them. Sometimes this paresis follows a spasm. Paresis in one muscle with spasm in another has been seen. Rarely a paralytic condition is followed by a spasmodic. The result in regard to the occupation is the same. Inability, awkwardness, with strange and frantic efforts to overcome and thwart it are so obvious at times that the attitude alone is almost sufficient to make a diagnosis upon. In the paralytic form a certain degree of muscular atrophy is occasionally noted. It is unlike the wasting of the progressive form of atrophy in the fact that it is less in degree and it is amenable to re- covery. If it is an early manifestation and is accompanied by local pain and paraethesia, neuritis is probably the cause of the trouble and not the neurosis under consideration. A mild, localized, permanent, late atrophy may rarely occur as the result of secondary damage down to the central cells of the cord by the severity and prolongation of the neurosis. None of the usual objective signs can be elicited by exam- ination. When electrical reactions, cutaneous sensibility, true objec- tive paralysis, tremor or ataxia is observed the disease is not one of occupation neurosis but some organic trouble of peripheral nerves or cord. Too much inportance cannot be given to this distinction, for the prognosis and treatment depend upon it. It is very rare for patients to complain of tremor and yet in some instances an irregular tremulousness has been observed. One must be well on his guard not to mistake dementia paralytica or disseminated sclerosis, which sometimes in their incipiency reveal only a tremor and partial inability to write, for this disease. As an exhaustion mani- festation, tremor, or rather trembling or unsteadiness, may well be expected, especially in the forefinger when the hand is at rest. An unusual form of nystagmus has been seen among miners and attributed by Nieden to the defective illumination of the mines causing eye-strain. The neuralgic forms of the trouble must be carefully studied in a skeptical frame of mind. Authors describe all sorts of painful condi- tions that are clearly due to neuritis. One writer speaks of pain over the nerve trunks elicited by pressure, of hyperesthesia, anaesthesia and paresthesia, all typical of a distinct neuritis. What is the logic of having two descriptions thus of the same disease in different parts of the lx>ok? If this author regards the occupation neurosis as an expression of a neuritis or other pathological change, it can and should l>e kept under the symptomatology merely of neuritis; if the occupation neurosis is not a distinct disease of such gross organic origin, then these symptoms of such organic disease do not belong to it and should not be referred to except as an evidence of a complication. Clearness of distinction and logical reasoning are in neurology as in every science coequal in importance to the mere accumulation of facts and observa- tions. Complications and sequelae may he easily understood as ac- companying an occupation neurosis. Organic gross changes and in 9ome advanced cases even undiscoverabk histopathological changes of a nutritional character may cause various vasomotor and trophic symp- toms. These, as a rule, will represent nenritic and poliomyelitic disease which will differentiate the trouble at once from an occupation neurosis. Diagnosis. For the sake of clearness we must in this disease THE NEURONIC DISEASES 2/9 maintain a sharp distinction between a functional and an organic trouble. It is confusing, unscientific and illogical to talk about a neurosis being a functional trouble and distinguished from organic, and immediately under the head of neurosis begin depicting the well- recognized clinical pictures of distinct organic diseases. And that is just what the older writers have done and alas ! what the later writers are still doing. Neurology cannot hope to be clear to the general reader when its followers thus wantonly jumble its well-known facts. I am not criticising the difference of opinion in regard to the real nature of the occupation neurosis, for I admit that there is much room for a difference of opinion, but I am criticising the method of calling a disease one thing, functional, and then describing it with the symptomatology of another well-recognized thing, organic. The logical contradiction, not the difference of opinion in connection with the disease, is the ground for my criticism. I have perhaps already sufficiently shown that in my opinion an occupation neurosis is not a neuritic or muscular disease itself, though as a complication or a sequel it may have accompanying it a neuritic or muscular organic trouble. Its etiology and its symptomatology, as I have previously shown, both loudly declare that it is a disorder of the higher conscious or subconscious coordinating mechanism. Y\ nether this mechanism resides in the cortex, basal ganglia, cerebellum, medulla or gray matter of the cord we cannot say. But its location is of little consequence in comparison with the fact that it is a highly compound, complicated function dependent upon the play of one source of nervous impulses upon another source. If the sources of these impulses them- selves are organically diseased, we may have spasms and pareses with incoordination. Such diseases will be recognized as well-known organic lesions of the brain, cord or nerves. On the other hand, the mere functional play of one source of impulse upon another source, the new, educated function as it were, is disturbed and exhausted. This is a true occupation neurosis. There are many diseases in which muscular weakness and dis- turbed coordination with spasm are seen and must be differentiated carefully from this disease. Every sort of disorder of writing is not writer's cramp. The former is seen in multiple sclerosis, in high tabes, in dementia paralytica, in paralysis agitans, in hemiplegia, in chorea, in progressive muscular atrophy and various injuries and lesions about the hand and related nerves. The differential diagnosis of these is easy as a rule and involves always the diagnosis of the organic or general trouble. The two diseases that have been most confused with writer's cramp are neuritis and poliomyelitis. In all these there are motor, sensory and other exhibitions that lead at times to a close simulation between them. Neuritis has very often been mistaken foran occupation neurosisand vice versa. A neuritis certainly interferes with complicated movements and it may spring from the same general predisposing and exciting causes in some instances as an occupation neurosis. The necessity for a clear recognition of a distinct difference between the two, how- 280 THE NEURONIC DISEASES ever, is indicated in the opposite character of the. prognosis of the two diseases. Neuritis is a local, distinct, comparatively permanent lesion with definite, constant symptoms. In neuritis the sensory symp- toms are in great evidence often and are always more or less present. They certainly hold a much more exalted degree of prominence in re- lation to the motor symptoms here than they do to the motor symptoms in an occupation neurosis. In the latter we often have muscular, crampy pains, general soreness and widely distributed fugitive aches, but we don't have the localized pains upon pressure in the nerve trunks, the localized spots of hyperesthesia and anaesthesia, the well-defined and unmistakable parsesthesise like numbness, tingling, burning, pins- and-needles sensation of neuritis. The sensory phenomena of the latter are prominently cutaneous and objective ; those of the former are largely muscular and subjective. Neuritis suggests clearly an organic, well-defined local lesion ; an occupation neurosis has a sort of resemblance to neurasthenia or hysteria. It is in the motor sphere that the difference between a neuritis and an occupation neurosis comes out most clearly. Incoordination, it is true, is prominent in both. In the former it is incidental, in the latter it is all but the entire disease. There is definite weakness and paralysis in individual muscles, so that for no sort of movement can the muscles be exercised in neuritis ; in the neurosis there is only ex- ceptionally paresis and then only as a result of prolonged and monot- onous exertion. In the neurosis the muscles may be powerless to move for one sort of movement, but apparently little affected for the per- formance of another sort of movement. A victim of pianist's cramp may be able to write normally ; an affected telegrapher can still play the piano; and one who can no longer write may still be able to draw with the greatest facility. When the trouble involves the lower ex- tremities other movements than those which caused it can usually be well performed. This argues against the old view that the muscles themselves are diseased. The electrical changes, so well known in neuritis, the reaction of degeneration, are not seen in the neurosis. The presence of atrophic, vasomotor and secretory local disturbances, loss of the reflexes, pareses and similar manifestations in other parts of the body point to a neuritis. Neuritic symptoms are bilateral, when they are bilateral at all, from the start. In the neurosis, an attempt to use the other arm may set up bilaterally the same trouble in it, but the disease is never bilateral from the beginning. As I have said, all of these neuritic manifestations may occur in the course of an occupation neurosis, but in my opinion they represent a complicating neuritis. Tf T thought the disease were entirely a form of neuritis, it would be referred to merely as a symptom of this disease described elsewhere. The frequent disappearance of the neuritic symp- toms while the incoordination and difficulty in writing continue shows also that a neuritis and an occupation neurosis are not the same thing. PoliomyeliHc symptoms may occur also in the course of an occu- pation neurosis, but they are easily differentiated. In poliomyelitis there is a febrile condition, a regressive paralysis finally limiting itself, al *ng witli rather rapid atrophy in particular muscles. Poliomyelitis THE NEURONIC DISEASES 28l occurs most frequently in the legs; the neurosis in the arms. Spasm, pain and true incoordination are wanting in poliomyelitis. The sore- ness of the muscles, the fatigue symptom, the appearance of the in- capacity upon the initiation of a particular form of complicated, ac- quired movement are also absent from the spinal inflammatory de- generative trouble. Tetan-v has been mistaken for an occupation neurosis. Tetany is provoked by any sort of movement, whereas this disease has its spasms initiated only by an attempt to resume the occupation that caused it. The "hcphccstic hemiplegia''' of Dr. Frank Smith seen among Sheffield smiths is declared by Gowers to have been cases of organic disease. of the hemisphere. Types of the Disease Based upon Occupation. — Practically the trouble is always of the same nature in whatever occupation it may have been acquired. It is useless therefore to go into any elaborate description of all the forms of the disease that have been seen and reported. The principal feature of investigation in each particular case is the character of the movement that has brought the trouble on and the particular muscles involved in the elaboration of that move- ment. The whole question is therefore one of location merely and of gross anatomy. This investigation will have, however, rather a scien- tific interest merely than a therapeutic one, for the treatment is the same in all cases. It may be of value diagnostically, however, if the occupation can be guessed from the symptoms of the disease alone. Pianist's cramp is chiefly a professional disease and seems to occur, possibly on that account, more among women than men. The spasmodic and neuralgic varieties of the disease, more particularly the latter, are seen. Precision in striking the key and prolonged extension of the fingers are troublesome manifestations. Pains are often felt in the fingers, up the arms, in and about the shoulder and between the shoulder blades. Gowers mentions a case that had pain in the outer border of each hand, but never any cramp except at night. Playing, of course, has to cease as soon as the symptom appears. Paralytic forms and others occur, but usually pain and exhaustion during playing repre- sent the complexus of symptoms. Violinists may be affected in either or in both hands. In flute players the left hand is more often involved than the right. In a young cornetist that I once treated who had failed to get much improvement from many previous lines of treatment and who improved, but I cannot say recovered, after a long course of treat- ment with me, the lips were slightly spasmodic and there was an in- definite soreness along the course of the supraorbital nerves. He was distinctly a neuropath, but exhibited at 110 time any other local symp- tom. Aldrich reports a case in a trap-drummer whose duty it was to beat a drum by the operation of a pedal manipulated with the right foot. Cramping pain and a sense of exhaustion seemed to be located in the tibialis anticus and peronei muscles. The symptoms appeared only when playing or when walking rapidly. I once had at my clinic for a long time what seemed to be a clear case of the disease in a painter. The possibility of lead-intoxici- 282 THE NEURONIC DISEASES tion was eliminated by the absence of the extensor paralysis, the abdo- minal colic, the blue line along the teeth and other well-known signs of saturnism and lead neuritis. He could move his arms somewhat, but there was no spasm and only soreness in them. He had been work- ing hard and continuously for a long time on a steady job and told me he had scarcely ever rested his right arm by using his left. To blacksmiths is known a very painful occupation cramp involv- ing especially the deltoid and biceps muscles. Seamstresses, tailors and others who do sewing, such as sail-makers, carpet-weavers, suffer at times from the disease. Runge believes he has seen it in waiters from carrying plates and glasses. Cigarmakers who roll cigars all day and milkers of cows have the cramp sometimes. Oppenheim has seen it appear in barbers every time they had to use the razor. It was spastic in character. Lapidaries are not free from the neurosis. A metal-chaser has suffered from it. Among the other numerous trades that at rare intervals cause it may be mentioned -Rower-makers, turners, watchmakers, knitters, engravers using the burin, masons handling the trowel, compositors, cnamelers, shoemakers and money-counters. A sawyer's cramp is described by Poore. These trade neuroses, so to speak, are not as frequent by any means as the neuroses caused by writing, music playing and telegraph- ing. Telegrapher's cramp seems to affect more particularly those who use the Morse system, which necessitates an up and down movement of the key. The typewriter may produce the trouble just as piano- playing does. Stammering and stuttering from much speaking is related to this disease, so is the painful exhaustion of the laryngeal muscles called mogiphonia by Frankel. A dancer's cramp is known among ballet dancers. It would seem that every form of human occupation, involving strained, continuous and complicated, coordinated movements is liable to provoke this neurosis. Still the caution must be repeated not to con- found neuritis with its degenerative symptoms and which lurks behind the occupation palsies with this neurosis. Prognosis. — Some authors affirm that the prognosis of writer's cramp is variable. It is if the symptom alone is made the disease. If the trouble is confounded with a neuritis the prognosis naturally will be favorable, for that is the prognosis of neuritis. Of the disease occu- pation neurosis, as I conceive it and have endeavored to describe it, the prognosis is decidedly unfavorable. It runs parallel with the sort of prognoses that we have to make in hysteria, neurasthenia and allied general disorders. Amelioration occurs after periods of prolonged rest. Absolute cures have been seen, but they are rare. As a rule the disease continues many years or for life, relapsing or rather breaking out in a sort of exacerbation every time the original movements that caused it are attempted. There is danger always in using the other hand, as writers and telegraphers so often do, for it frequently also becomes affected. Tt never destroys life. Treatment. — Prophylactic treatment is of great moment. The THE NEURONIC DISEASES 283 instant the first premonitions of the trouble appear cessation of the occupation must take place. All writing had better be given up for a time. The prophylaxis is summed up in the ore phrase, avoid all stiff, cramped, uncomfortable attitudes, style of writing and manner of hold- ing the pen. Place the body at the desk in writing so that an easy, loose and pliable position can be maintained. Adopt a style of writ- ing that is large, full, round and flowing. Use soft pens in thick •cork handles. It is good to vary the attitude and the penholder from time to time. Whether writing or telegraphing the entire arm should rest on the table. The vertical style of penmanship is said to be less liable to produce the trouble than is the slanting. Each case is to be studied from its own standpoint, but ease of attitude, ease of mind, largeness of movement and frequent relaxation, are among the im- portant preventive measures to be remembered. The rest should be absolute and not merely a variation of exercise. It is unwise to go from much writing, for instance, immediately to piano-playing. The two neuroses have been seen to develop thus simultaneously in the same individual. When the trouble has fully declared itself, only absolute and pro- longed rest is the safe plan to adopt. Those who must write some at all hazards may be guarded somewhat by the use of a special apparatus to hold the pen with. These are very numerous and consist of rings and bracelets for several fingers, in which the penholder can be fastened. The attitude of the hand is decidedly altered while the writing goes on. All instrument makers have several forms of apparatus of this sort," specially commendable among them being the Matthieu finger ring and thumb rest, the Nussbaum finger bracelet and others. There is no drug treatment for this neurosis. Of course the neurasthenic state may call for its special tonics. The very nature of the disease, an exhaustion of coordination, precludes any hope of benefit from mere medicinal agents. Electric treatment is also ruled out by the nature of the disease. Galvanism has been applied to the cervical cord in some cases with alleged benefit. As everyone knows electricity is a most valuable agent in the treatment of the degenerative neuritic troubles, both galvanism and faradism. It is to be noted, however, that those who claim such excellent results from the electric treatment of writer's cramp do not distinguish sharply between the pure functional occupation neuroses and the neuritic professional palsies. It is easy to understand therefore how even their results vary so extremely. Massage and gymnastics afford the best results. The former maintains the nutrition while the muscles and nerve centers are regain- ing their tone from rest, and the latter changes yet engages the activities of the central coordinating apparatus. Swedish movements of the hands and fingers with resistance on the part of the patient are particu- larly good. The fingers and wrist are flexed and extended, adducted and abducted. The pressure must be regular, the seances short and the practice several times a day. Gentleness, persistency and the end to be attained, the restoration of wearied muscles and exhausted co- ordination, are to be kept clearly in mind. 284 THE NEURONIC DISEASES Suggestion and psychic treatment has some influence, as psychosis enters to a certain extent in the loss of the coordinating power. Tenotomy and nerve-stretching are mentioned merely to provoke the wonder why they were ever thought of. MYOTONIA CONGENITA. This singular affection is sometimes called Thomsen's disease,, because this physician, who was a victim of it, and in whose family there were more than twenty cases in five generations, gave the first complete and accurate description of it. Before him Sir Charles Bell, Benedict and Leyden had each referred to it. Since the prominence which Thomsen conferred upon the disease it has been quite closely studied by Seeligmuller, Westphal, Mobius, Erb, Striimpell, Yig- ouroux, Hammond, Dana, Shaw and many others. It is not a common disease, though upwards of half a hundred cases perhaps have been reported. It is more frequently observed in Germany and Scandinavia than in England, France or America. Its exact position in the classifi- cation of diseases cannot be determined for reasons- noted in the dis- cussion of its pathogenesis. Etiology. — The predominant feature in its etiology is its heredity and occurrence in the same family. A few isolated cases have been observed in which the hereditary influence has been chiefly neuropathic. Males are said to be more frequently affected with it than females. The disease may appear in early childhood about the period of adolescence. It is not known to be dependent upon any exciting causes for its outbreak, though as it appears to be an expression of defective development, all depressing and exhausting surroundings may have something to do with hastening its appearance. Pathology and Pathogenesis. — So few cases have come to autopsy that our knowledge in regard to the essential basis of the trouble is far from being adequate. In the cases of Erb and others there was hypertrophy of the muscular fibers, increase of the sar- colemma nuclei and an overgrowth of interstitial tissue. The fibers have been reported as being diminished in size, though increased in number. All of the muscles, even the diaphragm, have exhibited this change. Dejerine and Sottas have confirmed these findings and added the statement that in their case the brain, cord and nerves were entirely normal. In some cases absolutely no changes have been observed either in the muscular or the nervous tissues. The symptoms of the disease, taken in conjunction with these almost negative pathological findings, favor the hypothesis that it is a functional trouble. Most writers following Leyden have described it .is a functional disease o\ the muscles, not including it at all under tin- head of nervous diseases. This is extraordinary in view of the neurotic and psychic relationships of the disease. And yet it is but fair to state that these authors look upon the mental deterioration and tin- nervous manifestations and influences often observed in the dis- ease :is of the nature of mere complications, The same developmental THE NEURONIC DISEASES 285 inadequacy that affects the muscular apparatus affects the nervous cen- ters. Other authorities classify the disease with the dystrophies, not merely because there is a certain similarity in some of the clinical fea- tures with the latter, but because they suspect that it, as well as the d}Strophies, are remotely dependent upon some minute central change in the central nervous apparatus, which may ultimately show that the> are closely related to the central progressive muscular atrophies. A third group ot writers are inclined, to regard the trouble as hysteroid in origin, though not in any sense hysterical. They explain that it is a sort of psychosis, a psychic incoordination, a connecting link between mental and bodily ailments. The absence of constant and distinctive pathological findings fa- vors the functional character of the disease. The hereditary and de- velopmental origin of the disease, its chief limitation to the voluntary muscles, its close relationship to the volitional faculty, the frequent mental defectiveness and the absence of most of the well-known signs of typical organic disease of the brain or spinal cord lend some sup- port to the mental theory. With the meagre data now at hand nothing definite therefore can be said in regard to the pathogenesis of this remarkable affection. Symptoms. — The prime symptom of Thomsen's disease is an intentional spasticity. I use the adjective intentional here in the same sense in which it is used when the intentional tremor of multiple sclero- sis is spoken of. In the muscles of the extremities and of the face the slightest voli- tional movement causes a slow, firm, irresistible contraction. This may occur at any time when the patient starts to use the particular muscle or set of muscles. It is usually, however, most prompt and severe after a period of rest. With a powerful exertion of the will the contraction can be retarded and even at times overcome. Thus it is seen to be a volitional phenomenon in part, albeit a markedly abnormal one. Some have endeavored to explain the entire disease on this per- version of the volitional muscular function. It is argued that to pro- duce a. desired movement the volitional faculty, so-called, must exer- cise a combined, normal control over every muscle of the body and its antagonist. It must preserve a balance between the two. If this balance is lost, the muscles do not work well together, action and over- action causes an unnecessary expenditure of force, and a sort of pur- poseless movement. As one author has put it, an inhibition of volun- tary movements due to a muscular rigidity sets in during the action. Influences like increased emotion that affect the will affect most strik- ingly the contractions. Often when the contraction initiated by the will is once started it cannot be stopped, even with the greatest exertion. After several such tonic contractions they become gradually weaker and finally cease altogether from exhaustion. They do not reappear until another voluntary movement is instituted in them. Warmth, mental quietude, non-observation of self, frequent exer- cise of the involved muscles and ingestion of a small amount of alcohol will tend to diminish the severitv of the contractions. 286 THE NEURONIC DISEASES All the muscles of the body except the viscera and involuntary muscles may be the victims of the disease. Swallowing, therefore,. may be difficult. Speech is affected. Protrusion of the tongue may cause it to be caught and bitten between the teeth. In grasping an object the hand closes like a vise. The act of walking becomes stiff > sluggish and irregular. The patient may even fall and remain rigid. The ocular and laryngeal muscles, with the sphincters, escape. In repose the muscles are normal to the touch ; in contraction they arc hard and firm. They may be normal in size or slightly enlarged or diminished. Sometimes their contour is abnormal from implication of a part of a muscle group. There are no disturbances of the re- flexes. In Erb's so-called myotonic electrical reaction in the muscles cer- tain changes take place in the responses to the galvanic current, those to the faradic remaining normal. There is a muscular contraction of long duration. The muscles show an increased excitability to the direct galvanic current. Only contractions upon closure are obtained. Anodal closing contraction is usually the more marked. This is somewhat of a reversal of the normal phenomena. With a stabile galvanic current a vermicular, rhythmical, wave-like contraction passes through the muscle from the negative to the positive pole. The contraction in the muscles may be started by any form of ex- ternal irritation. A slight tap or pressure will sometimes bring on the slow, worm-like movement in them. Sensations are normal. Only a slight aching pain is complained of sometimes. The mind as a rule is more or less weak, though in some instances it is bright and clear. Epilepsy, hemicrania, pseudo- hypertrophy, polyneuritis, tetany and other neural complications have been observed with the disease. Diagnosis. — The diagnosis is not difficult as the clinical mani- festation is so unique. Diseases in which similar spasms occur are to be differentiated by the existence of other symptoms which myotonia is singularly free from. Prognosis. — The prognosis in regard to life is excellent. The disease is one of years and even a whole lifetime. It is not pro- gressive in the true sense of the word. Remissions may occur during Its course. Treatment. — There is no treatment that will prove of any avail. Gymnastics and constant activity helps to make life bearable bv keep- ing down the severity of the spasms. THE CHOREAS. The term chorea is from a Greek word meaning a dance, and is employed to indicate certain irregular, involuntary, incoordinated mus- cular movements observed in various disease states. It is therefore not a distinct pathological entity ; it is merely a symptom. This fact should be carefully kept in mind, as the underlying affection of which the choreic jerkings form one symptom is the indicator for the proper treatment to be employed, and not the choreic movements themselves. THE NEURONIC DISEASES 287 The separating of idiopathic from symptomatic chorea, as some authors attempt to do, is entirely arbitrary and leads only to confusion. All the choreas are symptomatic, as will be seen in the further discussion of them. Acute minor chorea, the type of them all, is as much a mere symptomatic manifestation of an underlying toxaemia or other disease as are the choreas of hysteria, pre- and post-hemiplegic chorea, the pandemic choreas, Huntingdon's disease and the choreic phenomena associated with Friedreich's disease and other sclerotic processes. ACUTE MINOR CHOREA. Acute minor chorea, St. Vitus' dance, or Sydenham's chorea, is a disease of the central nervous system, frequent in childhood, charac- terized clinically by irregular, involuntary, incoordmated muscular movements, and by certain less obtrusive sensory and mental phenom- ena; and pathologically by a few scattered, circulatory, inflammatory and degenerative findings. Etiology. — Heredity does not play a direct role in acute minor chorea ; the so-called hereditary chorea, Huntingdon's chorea, is an entirely different affection. In almost one-sixth of the cases of this disease, however, a pronounced neuropathic taint is shown to run through the family. The child's forebears have been subject to epi- lepsy, insanity, hysteria, neurasthenia, narcotic poisoning with alcohol or tobacco, and even chorea itself. Race exhibits some influence, Jew- ish, German and Portuguese children being especially prone to the trouble. According to Weir Mitchell, the Negro race is singulany free from it. Later childhood is the favorite period for the outbreak of St. Vitus' dance ; and during this period girls are more frequently affected than boys, in the proportion almost of three to one. Nine-tenths of the cases, according to Gowers, occur between the ages of five and twenty. As puberty approaches the ratio of girls to boys increases. After twenty-five years of age the disease becomes more rare, and in old age it is very rarely seen as senile chorea. It is thus a disease of all periods of life, but especially of childhood, with a slight preponderance in the female. Ancemic, neuropathic and irritable children are often the victims of chorea and becoming hysterical, they sometimes manifest the com- bination of chorea and hysteria, which must always be carefully differ- entiated from the pure hysterical symptom known as chorea hysterica. The relationship between chorea minor, acute articular rheumatism and endocarditis is so striking as to call for more than a passing notice. It is estimated that approximately one-quarter of the cases of chorea give a history of acute articular rheumatism. The rheumatic symp- toms usually precede the choreic, though at times they may be coinci- dent with them, or even follow them. The rheumatic history is not so frequently obtained in the cases of early childhood as it is in the later and early adult cases. Another remarkable coincidence is the frequent appearance of endocarditis with chorea. Gowers affirms that it is within the truth to say that in one-half of the cases in which or- 288 THE NEURONIC DISEASES ganic disease of the heart exists this preceded the chorea. Occasionally the heart lesion develops during the course of the chorea. So promi- nent is this relationship between the rheumatic troubles and chorea that many authorities, chiefly among the English and French, regard it as distinctly causal. German and American authors do not accept so strongly the relationship as one of cause and effect. Many explanations have been offered for this association of rheu- matism, endocarditis and chorea. Some say that chorea is merely rheumatism of the central nervous system. In other words, chorea is rheumatism, or rather one of the occasional symptoms of the general- ized trouble known as rheumatism, of which endocarditis and the articular swellings are also symptoms and complications. The absence of any distinct climatic influence upon the development of chorea, while on the other hand its proneness to appear in the spring and fall seasons, when it is known that rheumatism is most frequent, adds weight to the suggestion that chorea is largely a rheumatic symptom. It has been argued that the chorea is the result of the same toxic state of the blood that in all probability provokes the endocardial and joint inflam- mations. The existence of a specific microorganism (Richter, Berke- ley, Dana, Meyer, Pianese) has been invoked, after some observations and experimentation, to account for the toxaemia which institutes the chorea, and not a few believe that the same, organism will be found to be the cause of the rheumatism. Such a view receives some further support from the fact that chorea has developed during and after well- recognized infectious diseases, such as scarlet fever, typhoid, measles, pertussis, acute pyaemia, gonorrhoea, syphilis, puerperal fever and even the distemper. All of this makes of rheumatic chorea merely an associated mani- festation of a specific form of intoxication. It has this in its favor, that it is more plausible and founded upon better established observa- tion than is the older view that minute emboli from the diseased valves of the heart are floated up to the brain, where they become lodged in the finer terminal arteries, and produce areas of cerebral softening, which in turn produce the motor symptoms of irritation. Before leaving the subject of the relationship of rheumatism to chorea, let me warn against confounding all pains associated with chorea with rheumatic pains. Chorea is not solely a motor affection. but not un frequently shows implication of the sensory apparatus as well. Tt is a well-attested fact that a severe mental shock frequently results in an attack of chorea; and the effect of mental strain in school, worry, and otherwise, is undefinable. Osier says that the prominent psychical element in the causation of chorea is certainly one of the most serious objections to the infectious theory of the disease, since there can be no doubt that ordinary chorea may rapidly follow a fright or a sudden emotion. It is no objection, however, it seems to me, but rather a confirmation of the toxaemic theory. It is beginning to be well recognized now by alienists that changes in the blood, cither of a toxic character or at least affecting its metabolic function, may precede or follow sudden disturbances of mentalization. THE NEURONIC DISEASES 289 The chorea from simulation, which has been observed endemically and reported from boarding-schools, etc., is like the true St. Vitus' dance epidemics of the Middle Ages. It is hysteria with choreiform manifestations. The occurrence of chorea during pregnancy is so grave a circum- stance as to demand a moment's consideration. Some think it is c. reflex chorea and is to be regarded in the same light as chorea rrom other peripheral sources, such as eye-strain, accumulation of wax in the ears, phimosis, intestinal parasites, etc. It is hard to imagine how a peripheral mechanical irritation can produce, by way of a reflex action, so unique and characteristic a symptom-complex as chorea is. Reflex action as a cause of disease has been heretofore too often ap- pealed to, and in view of many experiments and observations lately undertaken had better be dismissed except as a mere slight predispos- ing cause. In regard to the chorea of pregnancy, it is much more likely that an autotoxcemia is in some way responsible. This is shown by the fact that the chorea may appear before, during or after the pregnancy. The severity of the trouble and the frequent development of maniacal symptoms are also rather confirmatory. To sum up, the etiology of chorea, though varied and far from being established, points most suggestively to an infectious trouble associated with an unwonted mental strain, the underlying toxins af- fecting most vehemently the upper motor neurones and to a certain extent the sensory and psychic faculties, in that period of life, child- hood, and under that peculiar condition, neuropathic, when they are most susceptible. Pathology and Pathogenesis. — If we are contented with the view that chorea is merely a symptom of a more or less profound intoxication, the discrepancies in regard to the pathological findings may be easily accounted for. In a large class of cases there are no such findings and the disease is denominated functional. Unwonted susceptibility on the part of particular neurones in certain children, or the character of the causative infection, may well produce changes that will disturb the functions of those neurones and yet elude the power of our modern histological methods of examination. On the other hand, this infectious state, if sufficiently prolonged, or even the reaction caused by the violence of the disease in general, may lead to such scattered lesions as are sometimes observed at the autopsy. Of the latter there have been observed intense hyperemia of the brain, hemorrhagic and inflammatory foci, spots of softening, thromboses, emboli, etc., in the meninges, central ganglia and in the area sup- plied by the Sylvian arteries. Along with these circulatory findings must be mentioned the endocarditis, valvular disease and fatty degen- eration of the heart. All of these observations are inadequate as a basis for establishing a pathology of chorea, and thus regarding it as a distinct, uniform disease. The so-called chorea corpuscles are devoid of pathological interest in this disease, having been found in persons dead from other -causes (Wallenberg). Symptoms. — The most obtrusive symptom, and that which gives 290 THE NEURONIC DISEASES the name to this affection, is the irregular, involuntary, incoordinated jerking of the muscles. As it is the upper and not the lower neurones that are involved primarily, the jerking movements are not limited to individual muscles, but rather to groups of muscles, antagonistic or otherwise, that subserve generalized movements. Hence the head, or the arms, or the legs, or special parts of the body, like the tongue, the lips or the eyelids, twitch spasmodically. In other words, this symptom involves not muscles, but rather movements. For instance, the head will every few seconds jerk to the left or to the right; some- times it will be drawn backward or forward. The face will twist first to one side, then to the other; the lips will pucker or be drawn to one side; the eyelids will rapidly shut and open. All or any of the movements of the hand and arm may be affected. The thumb and fingers will snap shut and then open, or the whole hand will undergo a series of supination and pronation acts. The forearm may suddenly become flexed upon the arm and then relaxed, or the whole arm may be manipulated spasmodically from the shoulder. As with all intra- cranial or cerebral troubles, that part of the extremity in which the finer and more complicated movements take place normally will be more affected in the disease state. Hence the movements of the face are worse as a rule than those of the hand and forearm, those of the hand are worse than those of the entire arm, and the arms are worse than the legs. The trunk muscles usually escape, or when choreic movements attack them the disease is of another sort than the one I am describing. On account of the attitude of the patient, the jerking of the feet and legs is not so noticeable as that of the rest of the body. When the patient lies down or rests with the feet off the ground the leg movements become more obvious. With these movements ofttimes the whole body will be thrown violently about. This restlessness or incessant jerking may involve all parts of the body or only particular parts. It may therefore be confined only to the head or to one arm or one leg. More frequently it is bilateral in char- acter, implicating together the head, the two eyes, the two arms or the two legs. Occasionally the movements confine themselves to one side or the other, a condition known as hemichorea. The motor restlessness which comes on insidiously is peculiar in chorea and the symptom is not likely to be confounded with any other form of spasm. It is not rhythmical and it does not confine itself to any one muscle-group. It is now here, now there, rotating the arm or suddenly abducting and adducting it; then the legs jerk and cause the child to fall or at least to assume a disturbed gait. The entire body will turn from one side to the other. The eyes will twitch, the eyelid's snap, the tongue shoot in and out. The speech will be disjointed and difficult, and in extreme cases almost incomprehensible. These movements are all involuntary and are usually increased when under observation. The patient can control them 'for a few seconds, but not indefinitely. They occur mostly in daytime and dur- ing wakefulness. Sometimes they are present at night, during sleep. They are generally increased by associated voluntary movements,' though they themselves are always purposeless. Their constant vari- THE NEURONIC DISEASES - 29 1 ability in form, direction and location is their most striking character- istic. Respiration is affected sometimes in so far as it is a voluntary process. The diaphragm is the respiratory muscle most involved. The muscles of simple phonation generally escape. The violence of these movements varies greatly in different cases. In some patients they are so slight as to be scarcely noticeable, and to be elicited in examination by the production of some emotional ex- citement only; while, on the other hand, they may be so general and terrific as to give rise to the distressing condition known as muscular insanity (folie musculaire ) , in which the patient hurls himself violently about, leaps up and down, injures himself, bites his tongue and 'finds it impossible to eat or get a moment's sleep. Muscular weakness is not uncommon. Sometimes it amounts to a mild paresis. The paralytic chorea of Todd is characterized by ex- treme paresis, with but few movements. Rarely after the attack a permanent, local paralysis or weakness remains. It has been stated by some that the disturbed action of the heart, sometimes observed, is choreic. This is scarcely probable. In some of the cases it is undoubtedly one of the signs of an endocarditis. The contractions that are said to occur in the papillary muscles are not based upon adequate demonstration. In most cases the above symptoms begin insidiously and are mild at first. Some are mild throughout the entire course of the trouble. Another class of cases become rapidly violent and continue so until the disease reaches its climax in about four or six weeks. Others are so frightful as to resemble acute mania of a most active type, and still others exhibit paralytic rather than choreic symptoms. It is ob- vious, therefore, that chorea as a symptom may vary all the way from mere restlessness or slight "fidgets," for which the child is too often punished, up to an extreme degree of muscular activity or paresis with mental aberration. There is no good reason, however, for classi- fying these various degrees of the trouble into the distinct types, mild, severe, paralytic, maniacal, etc., as some authors do ; for as the chorea is merely one manifestation of a complex symptom-group, dependent rpon various diffuse lesions of a more or less infectious origin, the classification might be carried on indefinitely, since no two cases are ever exactly alike. So prominent are the motor manifestations that many other symp- toms, almost equally important at times, are entirely overlooked, or at least not sought for. Among these sensation is included. Trousseau long ago used to declare that the sensibility of the skin is almost con- stantly disordered, and yet many authors to-day affirm that the sensa- tions are never affected. I have found present not only formication, tingling and other paresthesia, but actual pain of a neurotic origin. These slight, fugitive, muscular pains, sometimes sharp, but more fre- quently dull and aching in character, are, I am convinced, not unfre- quently mistaken for rheumatic pains. Headache is quite a frequent form of pain. It must be admitted that hyperesthesia and ancesthesia, hyperalgesia and analgesia, and all the intermediate sensory phenomena 292 THE NEURONIC DISEASES may occur with the chorea. In all probability the sensory symptoms are provoked in the same way, and probably by the same sort of lesions, as are the motor symptoms. Another set of symptoms too often passed by in the examination of a case of chorea are the menial. There is rarely a case in which some change of mentality does not occur. In the mild cases and in the beginning of nearly all the cases there is a peevishness, slight irri- tability, heightened emotionalism, weakness of memory, lack of con- centration and power of attention. These symptoms often show them- selves before the muscular jactitations attract attention, and bring down upon the child the anger and corrective punishments of school-teacher and parents. The other extreme of the mental picture seen in some rare cases includes violent delirium, shouting, singing, incoherent chat- ter and all of the usual exhibitions of a severe acute mania. Accord- ing to Fagge, Dr. Hills of the Norfolk County Asylum stated that more than one patient suffering from chorea had been sent to the institution as insane. Such errors are likely to continue to occur so long as chorea is regarded as a distinct, substantive disease, and not as a mere symp- tom which may be present in, and form part of, or at least be associated with, other forms of mental and nervous disease. The chorea may be the chief trouble and the psychosis a complica- tion, or, on the other hand, the primary trouble may be mental and the choreiform movements may be the passing complications. A careful differentiation, difficult to make at times it must be confessed, will be the only means of positively determining into which category the case should be placed. The most severe cases of the maniacal form of chorea occur in association with pregnancy. Sometimes they last for several weeks, the mania being noisy and violent for a time, and then subsiding into depression or apathy, with hallucinations, ideas of persecution, etc., or even acute paranoia. The special senses are usually not affected in chorea. In some children who, as a rule, are of the neurotic class, and therefore would probably have the same troubles with or without the chorea, there is hypermetropia, astigmatism and muscular insufficiencies. The fundi do not reveal anything particularly abnormal, although optic neuritis has been observed in a few cases. The pupils not unfrequently are dilated, but they react promptly both to light and accommodation. Irregularity of the pupils was once noted by Gowers, the larger one being on the side opposite to the limbs most affected. The deep reflexes are generally normal, but occasionally they are diminished, and cases have been recorded wherein they have been ab- sent. This symptom should not be given much stress, as the deep re- flexes are variable among people in perfect health. It has been argued by some that the joint changes, sometimes seen in choreic cases and formerly attributed to the rheumatism, are true arthropathies of the trophic sort. No other trophic symptoms of cerebro-spinal origin are observed in chorea. According to Osier, herpes zoster occasionally occurs, and other THE NEURONIC DISEASES 293 skin eruptions, rheumatic in character, such as erythema nodosum and urticaria purpurica. As a rule the bodily temperature remains normal. A slight rise, however, is not especially exceptional. In maniacal chorea the tempera- ture may run as high as 104 degrees, just before death it has gone as high as 109 degrees. These elevated temperatures of chorea, even in the mild cases, are usually due to the complications, such as the joint affections, rheumatism or endocarditis. With the rise of tem- perature there will usually occur an increase in the pulse rate. The rapid pulse rate which some authors assign as a pure choreic symptom, indicating chorea of the heart muscle, is not necessarily associated with temperature. It is not due to chorea of the heart, but is merely the irritable pulse characteristic of neurotic children. In marked contradistinction to the renal function of hysteria, the urine of chorea is generally scanty in amount, high-colored and con- centrated and over-charged with phosphates and urea. Albuminuria is not a symptom of chorea, though it may be present when kidney dis- ease is complicating the trouble. In chorea nocturnal enuresis is not unfrequent. Chorea being a symptom merely) rather than a disease, it follows that it has numerous complications. Some of these are so frequent that they have been elevated by some authors to the dignity of special symp- toms. Such, for instance, are the heart complications. Others, I believe, are not so much complications of chorea as that the choreic manifestations are but additional symptoms of the diseases sometimes spoken of as the complications. This is illustrated well by some of the so-called rheumatic, syphilitic and other infectious accompaniments, the manias, and paralyses. This is a most important and practical distinc- tion to bear in mind, not only from the standpoint of diagnosis and prognosis, but especially from that of therapeutics. I will have more to say about it under the head of diagnosis. While at a loss to comprehend how the cardiac troubles, important as they may be, are to be considered among the symptoms of chorea, I will grant for the nonce that they may be complications and will discuss them under that head. It is my own belief that there is no direct relationship between the heart manifestations and the chorea. but that both are the direct result of the underlying toxic state of the blood, which toxic state of the blood sets up an inflammation in the one case and a functional neurosis in the other. Endocarditis is the most important heart complication. Pericarditis occurs occasionally. The former is almost invariably of the simple or warty form, according to Osier, and though not dangerous in itself. is apt to produce valvular incompetency. The usual sounds and signs of this heart complication are present. In chorea, however, there is an accidental heart murmur which must not be taken as indicative organic disease. It is anaemic in origin and is heard as a soft systolic blowing over the mitral and pulmonary valves. I have heard a l diagnostician assert that in his experience all cases of chorea exhibited heart signs, and upon the strength of this teach that chorea is always a rheumatic complication. Of the 140 patients with chorea examine* 1 294 THE NEURONIC DISEASES two years later by Osier, the heart was found normal in 51, disturbed functionally in 17, and exhibiting signs of organic disease in 72. In my own study of 100 cases, about forty-nine per cent showed heart trouble, and not more than one-quarter revealed mitral insufficiency. The mistake of regarding chorea as always a disease with heart trouble as a complication is well shown in the attempt, first made by Kirkes and afterwards by able authorities, of explaining the neurosis on the ground of a local ischsemia caused by the presence of an embolus washed up into the cerebral circulation from the diseased valves of the heart. It is true, even as Oppenheim says, that in the course of chorea such an embolus takes lodgment and produces paralytic symptoms. A moment's consideration will convince any one that such a cause for the chorea most emphatically proves that the choreic jactitations are but a symptom of a much more extensive trouble. The chorea in such a case is of the pre- or post-hemiplegic type, and is but one of the symp- toms of one of the well-recognized forms of cerebral apoplexy. The disease is not chorea with embolic obstruction as a complication ; it is cerebral embolism with chorea as one of its accidental symptoms. Rheumatic manifestations in the joints, as well as generalized acute articular rheumatism, are sometimes put down as complications of chorea, and, strange to say, often by the very same authors who argue that chorea is the direct result of the rheumatism. It seems to me that this ready interchangeability of two such sets of phenomena as acute articular rheumatism and chorea minor is proof sufficient that both are but symptom-complexes, coincident and alike dependent upon infec- tion. This is further supported by the occasional, though rare, asso- ciation of simple neuritis. Among the psychoses and neuroses associated with chorea should be mentioned the maniacal manifestations, mild and severe, as already mentioned, hysteria and epilepsy. I recently saw a case of severe acute delirium of childhood, caused by some playmates throwing some earth- worms down his back, in which there were for several weeks gradually- fading frightful hallucinations with choreic jactitations. The chorea vanished before the hallucinations had entirely disappeared. Here again the mere symptomatic character o-f the choreic jactitations was made obvious and the impossibility of referring to the psychosis as a complication of the chorea or vice versa. Convulsive attacks, cataleptic in character, persistent spasms almost like typical hemiplegia, and even paralysis, are all seen sometimes in association with chorea. As many of these are of hysterical or organic origin, they should more probably be regarded as separate diseases in which the choreic phenomena play merely an accompanying role. Diagnosis. — In a simple, uncomplicated case the diagnosis of chorea is easy. The jactitations are characteristically irregular, involun- tary and incoordinate. As the term chorea, however, refers only to the muscular movements, the disease, if it is to be regarded as a dis- ease, is scarcely ever uncomplicated. It is these complications or asso- ciated symptoms that sometimes render the differential diagnosis diffi- cult. So far as the jactitations are concerned, it would seem hardly possible to confuse the choreic movements with the slow, wax-like THE NEURONIC DISEASES 295 movements of athetosis, the rhythmical, somewhat more controllable movements of hysteria, the intentional movements of multiple sclerosis, or the peculiar tremor of paralysis agitans, etc. The distinction be- tween them will be pointed out under the head of these respective dis- eases. This distinction has only to be made, as a rule, in adults, as these latter diseases do not occur in childhood. There is a disease of childhood, the choreic athetotic form of cerebral infantile palsy, which may at times give much trouble. The muscular rigidity, particularly in the legs, associative movements and the athetoid nature of the twitchings will, if carefully sought for. be generally found and will indicate the cerebral palsy. Chorea has been mistaken for ataxia, especially when limited to the lower part of the body, and thus altering the gait. Oppenheim saw such a case in a boy three years old and was not led to make a diagnosis of chorea until he had observed the involuntary movements in rest. A general tic may be mistaken for chorea, or vice versa. The differentiation will be discussed under the head of the tics. Cases of Friedreich's ataxia were wont formerly to be reported as chorea. The slower character, athetoid-like jactitations of the former. the ataxia, the scoliosis, the scanning speech, the nystagmus and the familial nature of the disease, ought to make clear the differentiation. Choreic patients with marked maniacal symptoms have often been sent to the asylum as cases of insanity. The age in which insanity usually occurs, the garrulity and other mental symptoms and the rela- tive insufficiency of the muscular spasms will aid in making the diag- nosis of a primary psychosis. Prognosis. — The prognosis of chorea varies with the nature of the underlying cause. As a rule it is good, especially in those mild forms of the trouble known as acute minor chorea. These cases usually get well in two or three months. Occasionally they last only a few- weeks, and very rarely they last six months or a year, or even become incurable. The recovery, as a rule, is complete, but relapses are not uncommon. Only in the most violently severe cases, and even rarely in these, is the disease fatal. Death is the result of malnutrition caused by the inability to feed the patient. The dangerous signs of this seri- ous state of affairs are delirium, elevated temperature, irregular pulse and rapid emaciation. In most of these cases there is distinct heart trouble. About one-quarter of the choreas of pregnancy die, though the fatality is due to such factors as the severe psychosis, the cachexia, the heart complication and the abortion or unnatural parturition. Choreas late in life are more apt to become chronic or permanent than are those of childhood. Senile chorea is practically always per- manent. Treatment. — In the treatment of chorea three well-defined ob- jects are to be aimed at — the removal of the cause, the quieting of the mind and nervous system and the rapid nutrition of the patient. In most cases some form of infection will call for the first atten- tion. If it is rheumatism and its congeners, an antirheumatic line <>t' treatment is to be instituted, including; the regulation of the diet and 296 THE NEURONIC DISEASES the use of such remedies as the salicylates, alkalies, iodides, cardiac remedies, etc. This is not the place to discuss the treatment of rheuma- tism, but it is pertinent to remark that the same treatment is to be employed in the management of rheumatic chorea. Other infections should be attacked in the same way. A syphilitic history calls for the iodides and mercury. Autointoxications should draw the attention to the emunctories. Cathartics, diuretics and dia- phoretics are all indicated in such cases. If a thorough and honest search is made for the cause of the chorea, predisposing or exciting,, organic, functional or reflex, and that cause removed, the disease will get well promptly and without further direct medication. In chorea, however, there is always a more or less exalted excita- bility of the mind and nervous system that needs particular care. The patient should be removed from all mental strain. He should be taken out of school. Threatenings and scoldings should be avoided. Even his games and playmates should be selected with the purpose of lessen- ing mental strain. In some instances seclusion for a time, with a well-selected nurse and the adoption of a modified Weir Mitchell treatment used for hysteria and neurasthenia, may prove most useful. Of this nature is a sojourn on a country farm or other change of residence. Of course, all stimulants, such as tea, corre-2 and alcohol,, are to be prohibited absolutely, and milk given in place of them. Hypnotics and sedatives, such as the bromides, come into play and ofttimes give the happiest results. In extreme cases chloral hydrate may have to be administered continuously and even continuous inhala- tions of chloroform. Morphine hypodermically and antipyrine have been borne well and given good results in a few cases of extreme sever- ity. The zinc salts, conium, cannabis indica, hyoscin, physostigmine,. exalgin, salol, salophen, etc., may all be tried, but will probably be found more or less unsatisfactory. The exhaustion, whether general or neural, whether a primary or secondary factor of the disease, is the condition that in many cases seems to demand the promptest attention. The best and the most rapidly acting tonics are here loudly called for. Of this class of reme- dies, arsenic still carries the palm. So prompt and generally satisfac- tory is the effect of arsenic upon the diseased neurones that it has come to be regarded as almost a specific in chorea. Any form of arsenic may be employed, though the favorite forms are arsenious acid and liquor potassii arsenitis (Fowler's solution). I prefer the latter. Beginning with a minimum dose, the remedy should be rapidly in- creased until the first signs of intolerance show themselves — namely,, nausea and oedema under the eyelids. The medicine should be dis- continued for a few days and then renewed, to be gradually increased again. In this way I have administered as much as twenty-five drops three times a day. Arsenical toxaemia can be largely avoided by ad- ministering it well diluted in water, and when there is food in the stomach. A few drops of mix vomica will cause it to be borne well in some cases. And as anaemia is so generally present, some form of easily assimilated iron may be judiciously given at the same time. I have never had herpes develop and I have never observed in my own THE NEURONIC DISEASES 297 practice arsenical neuritis, though both are possible and have been reported. In some cases arsenious acid seems to act more powerfully. It is a convenient form to use with a chalybeate. OTHER CHOREIC DISORDERS. The desirability of dropping the term chorea as the name of a disease and of using it, like the term paralysis, merely to indicate a symptom, is shown by the multiplicity of choreas, spoken of in litera- ture and which, pathologically, ofttimes have absolutely nothing in common. We are not able to banish it entirely yet, because we do not know all that we should of these so-called choreas. Of the pre- and post-hemiplegic choreas, and one or two others, we have learned enough to cease speaking of them as diseases and to merely note the choreic manifestation as one of the symptoms of certain well-recognized patho- logical conditions. In time no doubt all the other choreas will be the same. Their division into essential and symptomatic choreas will do, temporarily, as a coarse classification, but it also will do when we learn positively whether the lesion is purely neuronic or extra-neuronic, whether the irregular, mcoordinated jactitations now referred to as choreic can be produced by intimate molecular changes in the nerve cells, as well as by the irritation of these cells by minute hemorrhagic inflammatory lesions. The former is what is meant to-day by essential, or idiopathic, chorea, and undoubtedly underlies the degenerative, hereditary, infec- tious and embolic types of the trouble ; the latter is referred to in the term symptomatic chorea. CHRONIC HEREDITARY CHOREA. HUNTINGTON'S CHOREA. Huntington, of Long Island, first described this affection in 1872. It was referred to, and probably Huntington's own cases described, by Waters in a letter to Dunglinson in 1842. In 1863 Lyon referred to it under the head of chronic hereditary chorea in a paper in the American Medical T lines. It is, therefore, not justly called Huntington's disease. It is an independent disease, with choreic manifestations prominent in its symptomatology and has no direct connection with Sydenham's minor chorea. It has been observed everywhere, here as well as in Europe, though it seems to be more common in and around New York. It is vulgarly called in the communities where it exists the "magrums" or "megrims," though it is as remote from migraine as anything possibly can be. The prime traits of the disease are its direct hereditary character. its late onset, its progressive, incurable nature and its association with pronounced mental deterioration. It is so distinctly recognized as a purely inherited disease tli.it children are interdicted in every possible way from intermarrying with families in which it occurs. Where a member <>t' an afflicted family escapes the disease and marries, his descendants, usually, though not always, escape also. 298 THE NEURONIC DISEASES Literature contains numerous reports now of some remarkable in- stances of the disease and its hereditary origin. The reports of Zacher, Hoffmann, Lannois, Sinkler and some others are of special note. D'Ormea has described it in eleven members of one family of four generations. I recently saw a case whose father had had the disease, but could get nothing further of the history. The age of the patient, the choreic symptomatology and the marked mental deterioration were all typical. The disease appears so uniformly between the ages of thirty *a<3 fifty that a member of a family cannot be said yet to have escaped it who has not arrived at that period of life. This late onset of the trou- ble is a marked characteristic and differentiates this from most of the other choreas of a general nature. It has been seen by Hoffmann, however, as early as ten years of age. It occurs about equally in both sexes. The families in which it occurs are strikingly numerous, eight and ten children being not un- common. In some cases it has started after a severe emotion. Gen- erally, however, there is no known exciting cause for it. When it has skipped a generation, epilepsy or hysteria has been seen in that gen- eration replacing it. The motor manifestations are typically choreic, consisting of ir- regular, sudden, incoordinate jactitations of all the muscles of the body. The arms and legs jerk and the face grimaces just the same as in ordinary minor chorea. The patient is to a certain extent rendered helpless by the move- ments when they are severe. His gait is affected, his speech is imperfect or even impossible, his respirations are embarrassed and he cannot use his hands in writing, hold knife and fork, etc. In mild cases the will can for a time repress the movements, but they are apt to be more violent elsewhere or later on. The reflexes are usually increased. The senses are not affected. The mind is always affected and its condition is one of the special features of the disease. There is a steady and progressive dementia, ending in complete idiocy in many cases. In the early stages, as in most dementias of the progressive, degenerative type, there is depres- sion, melancholia, suicidal tendencies and absolute apathy. The mental phenomena usually follow, though they may precede, the motor. The anatomical basis of the disease is unknown. In a few post- mortem examinations there were indications of a chronic meningo- encephalitis. Pachymeningitis, leptomeningitis, hemorrhagic foci, hydrocephalus and disseminated miliary inflammatory spots in the brain tissue were seen. The findings, according to Binswanger, were the same as in dementia paralytica. Some authors base their opinion upon these indefinite findings that hereditary chorea is pathologically a chronic meningo-encephalitis. It is hard to reconcile primary inflammatory changes with such pronounced direct heredity. To inherit inflammation and hemorrhagic foci without other cause for them is a new idea in pathology. In my own opinion the disease is a primary neuronic degeneration. THE NEURONIC DISEASES 299 an inherited developmental defect. The gross findings referred to above are purely accidental and secondary. This, it seems to me, har- monizes both the indefinite character of these findings and the marked heredity in the disease. The patient never gets well, but steadily progresses toward idiocv and death. It may last many years, however. All treatment hitherto tried has proved futile, a result one would almost expect from the developmental character of the trouble. Were it a primary inflammatory trouble, some hope from treatment might be entertained, if not towards producing a cure, at least in checking the onward progress. Such a hope has never been realized. ELECTRIC CHOREA. Henoch, Dubini and others have described forms of choreic mani- festations that occur at various ages, sometimes endemically, in various parts of the body and are especially characterized by the quick, light- ning-like character of the movements. In Dubini's disease there are short, rapid spasms, that involve successively different parts of the body, painful sensations, epileptiform and even paralytic symptoms. There are often atrophy and electrical reactions present. Heart failure and coma usually terminate them. Recovery is exceedingly rare. Bergeron's electric chorea is hysteria. Henoch's electric chorea is in all probability a form of paramyoclonus multiplex observed in children. It is obvious that the sooner we get rid of the term electric chorea the better, for none of the diseases described under it are chorea or have any relationship to the genuine, irregular, incoordinated, non- sensitive jactitations of the latter. What is called habit chorea is a coordinated movement more nearly related to the tics. Chorea major is hysteria ; so is what is technically called hysterical chorea. Laycock's procursive chorea, wherein young girls exhibit rhythmical rotating and procursive movements of the body, is clearly hysteria. It has been called chorea festinans by some writers. PARALYSIS AG1TANS. SHAKING PALSY. PARKINSON'S DISEASE. This is a disease of advanced age characterized by a peculiar tine rhythmical tremor, muscular rigidity, with a more or less distinctive attitude, and a striking modification of the gait; without a known constant pathological basis; and without any encouraging amenability to treatment. The largest individual statistical studies of this disej have been made by Cowers upon i 15 cases seen in private and hospital practice, and by Hart upon 2\<) cases seen in the clinic of M. Allen Starr. Upon these reports, as well as upon a few personal observations. the following description is based. Etiology. — It is true that paralysis agitans is a disease of the aged, for rarely is a case seen under forty and most of them occur 300 THE NEURONIC DISEASES much later. The decade between fifty and sixty is the favorite period for its appearance, about forty per cent, of the cases beginning then. Between sixty and seventy there were only twenty per cent, between forty and fifty, twenty-five per cent. Gowers says it is rare over sixty-five. It has been seen to occur as late as seventy-eight and per- haps later. That it is not solely a disease of advanced years is shown by the fact that Lannois recorded it in a child of twelve and Berger in a patient of seventeen. The youngest case that has been reported is that of Huchard in a child of three years. It is obviously a disease of the beginning degenerative period of life. Heredity seems to exercise so slight a role that it may practically be neglected. In about fifteen per cent, of cases a direct heredity has- been traced. A neuropathic tendency may underlie the disease and take the place of the direct heredity which is seen in the small number of cases so characterized. The influence of sex is probably not direct, but indirect, through exposure, occupation, etc. Males are afflicted with the disease about twice as frequently as females. The exciting causes that have been mentioned are numerous.. They include psychic shock and depression caused by worry, fright, anxiety, overwork, trauma, exposure, infectious diseases, such as grippe, malaria, typhoid fever, pneumonia, rheumatism, syphilis, poisons like lead and carbolic acid, intoxications with alcohol, tea and coffee, menopause, confinement, sunstroke, insomnia, abscess, piano playing, etc. As forty per cent, of the cases gave persistent worry as the prob- able cause, and as worry, dread and anxiety must have been more or less an accompaniment of the various other alleged causes, it may be assumed that psychic strain is the preeminent factor among the excit- ing causes. Many of the others may have paved the way for the gen- eral degeneration which seems to lurk beneath the disease process. On the other hand, it is always well to be mindful of the fact that worry is often a sign and result of, rather than a mere cause of, these degenerative troubles. The cause of paralysis agitans, therefore, it must be admitted, is obscure, though along certain lines a certain definite etiological picture can be outlined. Pathology and Pathogenesis. — Little need be said upon this subject, since so little is known, and what is known does not help very much in explaining the raison d'etre of the symptomatology. So far as any constant reliable findings, post-mortem, within the nervous structures are concerned, our knowledge is absolutely negative. The hypertrophy of the nerve cells of the pons Varolii described by Luys ; the induration of the pons, medulla and cord mentioned by Parkinson ; and even the more recent observations of Ketcher, Borgherini. Koller, Dana, Redlich and others involving sclerotic conditions of the blood vessels and the glia, and other findings similar to those noted in senile processes generally, are only worthy of historical interest. This total absence of any distinct discoverable lesions is remark- THE NEURONIC DISEASES 3OI able, especially in view of the fact that the symptoms are sometimes unilateral, monoplegic and otherwise indicative of a definite focal lesion. Gauthier seems to have hopelessly abandoned the nervous system en- tirely, for he looks upon the trouble as primarily one of the muscular system. Gauthier's position is not a plausible one, as Gowers has well shown. The character of the symptoms being so purely motor, and in a remote way similar to chorea, the source of the trouble must in all probability be looked for in the cerebral cortex and the motor centers. As Gowers well says, this is strengthened by the great similarity of these patients to a person under the excessive emotion of fear. There is a loss of inhibitory control with a regular explosion of abnormal nerve force from the lower cells, all due probably to certain physio- chemical intracellular changes as the result of a sort of senile-like degeneration and lowered nutrition. With Oppenheim, however, I agree that paralysis agitans should not be looked upon as a mere condition of presenility. It is a distinct disease of a more or less definitely localized pathogenetic process, and not a mere side issue to a general lowering of all the bodily forces and functions. Symptoms. — There are four special symptoms that characcerize •every case of paralysis agitans — namely, the tremor; the steady muscu- lar rigidity upon which the characteristic attitude depends ; the slightly paretic or retarded movements; and the gait. A typical case of the disease cannot be mistaken for anything else, -even at the first glance, for the symptoms in their combination are unique. With the head slightly bent forward and every joint in the arms, legs and body slightly flexed, the hands and arms trembling with a fine, rhythmical oscillation, the patient trips forward with a sort of mincing step, as if he were on the point of breaking into a run. The tremor and rigidity are usually seen in the same set of muscles. They may involve the entire body or only parts of the body. The move- ments of a voluntary sort in the arms and legs arc distinctly sluggish and delayed. I will now discuss the symptoms a little more in detail. The tremor is the special, obtrusive sign of the disease. It usually precedes, though it may follow, the appearance of the muscular rigidity. It may be limited to the fingers, which, being flexed, look as if the) were rolling a pea between them. It extends to the muscles of the arm or it may involve the arm and leg, usually on the same side. Winn all four extremities are involved, one side seems to be generally a little worse than the other. This characteristic one-sided distribution of the tremor, and its tendency to progress from the ends of the ex- tremities upward, indicates its hemiplegic nature and cerebral origin. The body sometimes oscillates, though for obvious reasons its tremoi is not so noticeable as that in the extremities. Some writers have de- clared that the head never shakes. That is certainly an error. I have seen it exhibit the same sort of tremor in both a to and fro and a rota tory manner, as the hands show. It is rare, however, in the head. Only in four instances among I I art and Starr's eases was the tremor) absent. 302 THE NEURONIC DISEASES That the parts of the body in which the voluntary movements are the most delicate and complicated are the preferential parts for the mani- festation of the tremor is shown by the greater number of cases that begin on the right side and in the fingers of the right hand. The upper extremities were involved seven times more frequently than the lower, and the right side a third oftener than the left. Sometimes the tremor advances very rapidly from one part of the extremity or body to another, at other times it will continue for years to be limited to FIGURE 41. Paralysis agitans. (After Charcot.) one part. Gerhardt and Hart both cite cases in which the tremor was intentional. In addition to the head, trunk and extremities, the tongue, the lips, the angle of the mouth, one side of the face, the eyeballs and the orbicularis palpebrarum have all been seen to be affected with the tremor. The tremor is a stereotyped one, fine, rapid and rhythmical. The oscillations are four or five to the second and the extent of the excur- sions is small. Sometimes the tremor is so fine that it is onlv observa- THE NEURONIC DISEASES 303 ble in the patient's penmanship ; at other times it is very large and coarse. Voluntary active movements usually put a stop to the tremor for a few seconds. The same may be said of passive exercise and the fixing of the patient's attention. Mental excitement always in- creases the shaking. In sleep it usually ceases, but not in all cases. Neither the attitude of the patient nor the position of the limbs at all modifies it. At times it shows a tendency to remit. Next to the tremor the muscular rigidity is the important symptom of paralysis agitans. It usually coexists with the shaking and reveals itself about the same time. In some cases it comes on later than the tremor, in a still smaller number it even precedes the latter. Schlapp and Hart both report cases with rigidity, but without tremor. It is the muscles of the neck and back that reveal the rigidity, especially in the patient's stooping attitude. The slight flexion of the joints in the members affected with the disease is due to the muscular rigidity. When the face is involved the appearance is statuesque and wax-like indeed. The rigidity is noticeable also in the resistance offered by the muscles when passive motion is attempted. The same cause explains the delayed active movements. It seems as though the muscles could not respond as promptly as usual to the will. The modification of the gait is a. striking feature of the disease. The patient takes short, mincing steps on the tips of his feet and seems as if he were about to start into a run or to stride forward as though he were on the point of falling at every step. This is termed propulsion. Sometimes he exhibits a curious tendency to walk backward, retro pul- sion. Lateropulsion has been noted, but it is not common. Contrac- tures sometimes occur and incomplete paralysis in the later stages. The deep reflexes may be diminished or increased. They are often normal, never absent. There is neither atrophy nor electrical altera- tions in connection with the musculature. In two of Hart's cases, typical in every respect, the pupils were of the Argyll-Robertson type. The speech is often altered and there are changes in the voice. Slowness and irregularity, but not true dysarthria, scanning or stutter- ing characterizes the former ; while the latter *is monotonous and whining. Oppenheim says that hypcridrosis is rather a frequent occurrence. It does not bear any special relation to the parts affected with the tremor, though it may not be a generalized sweating. General restlessness is a characteristic in a goodly number of cases. This, however, may be due to the fact that active movement puts a certain amount of quiescence upon the tremor and so makes tin patient feel more comfortable. Among the sensory symptoms occasionally spoken of by these patients may be mentioned slight darting pains, aching, pricking, numb- ness, tingling and heat and cold, all very changeable, evanescent and subjective. The mind remains unaffected, or if it is affected it is a complication and not a part of this disease. Slightly depressive emotional states may occur, but as a rule the patients are cheerful and satisfied. 304 THE NEURONIC DISEASES FIGURE 42. Paralysis agitans. (From photograph in the Pathological Museum of the Med. Depart, of the University of Illinois.) THE NEURONIC DISEASES 305 A slight elevation of temperature and an increase of the pulse rate have been observed in some cases. Diagnosis. — A typical case of paralysis agitans with its tremor, attitude, muscular rigidity, sluggish movements and peculiar gait can offer no difficulty in diagnosis. Other diseases may have a fon*i of tremor that may be suggestive of shaking palsy. Multiple scierosis has caused doubt in some instances. The earlier development of the dis- ease, the nystagmus, the intentional character of the tremor, the optic nerve involvement and the bladder disturbances wfll readily indicate the trouble as one of multiple sclerosis. Senile tremor is sometimes very much like paralysis agitans. The head is involved here more frequently and the shaking is always in- creased upon voluntary movement. Moreover, the other symptoms of paralysis agitans, the rigidity, the attitude, the gait are wanting. Oppenheim calls attention to senile paraplegia, a spastic paralysis of old age in which there is arteriosclerosis of the brain and cord, which presents a clinical picture not wholly unlike paralysis agitans. In the senile paraplegia, however, there are true paralytic symptoms, such as dysphagia, dysarthria, bladder disturbances, etc. Sometimes a form of traumatic neurosis occurs that is almost the counterpart of Parkinson's disease. These cases are difficult to differentiate at times, because it is a recognized fact that paralysis agitans sometimes follows trauma. However, in the traumatic neuro- sis hysterical or neurasthenic symptoms predominate and the charac- teristic symptoms of true paralysis agitans are not all present. Prognosis. — The prognosis of shaking palsy is good in regard to life, bad in regard to recovery. The disease usually involves the whole body in the course of a couple of years, then it may remain stationary for a great many years. Death often occurs from exhaustion. Apo- plexy sometimes closes the scene. A worse prognosis than death is that of the late paralysis, contracture and general bedridden, helpless state that sometimes obtains. Fortunately this is not a frequent condi- tion, in spite of the fact that the disease is most emphatically a pro- longed one. A mild delirium, a slight fever and a complete physical breakdown are the usual accompaniments of the end. Treatment. — The treatment of paralysis agitans is, if carried out on proper and conservative lines, attended with a fair degree of success, considering the chronicitv and fundamental nature of the disease. Mental and physical rest, quiet and nutrition are the main indica- tions of treatment. All sources of mental shock, disturbance and de- pression should be removed. Hence it is well to get these patients away into the country. Popular and fashionable resorts, sanatoria and all places where there is likely to be a crowd of people, with the at- tendant excitement, had better be avoided. Cheerful compan) . plea-ant occupation, light exercise, reading, simple games should all be urged. Good ventilation and a comfortable bed at night are important items. It is strange that because movement, such as carriage and railroad riding, makes these patients often feel better, as Charcot Ion- 306 THE NEURONIC DISEASES pointed out, that any one should think of introducing vibratory chairs and other apparatus for their treatment ! The nutrition should be plain and abundant. Alcohol, tobacco, tea and coffee should all be discontinued. In the words of Oppenheim, all forced therapeutic measures should be avoided, such as cold water cures, massage, electricity, nerve- stretching, suspension. Purgatives, diaphoretics and antipyretics are of little benefit and may be dangerous. The following drugs have been recommended in this disease : Salicylates, Dover's powder, arsenic, Indian hemp, nitrate of silver, curare, bromides, atropine, phosphorus, cod-liver oil, picrotoxin. They are practically useless. The most advantageous medications are those that have a sedative and hypnotic influence. The hydrobromate of hyoscine, the sulphate of duboisine, morphine, codeine are the best of the palliative remedies. Podack recommends sparteine. Veratrum viride, gelsemium and nux vomica in the form of tinctures have quieted the tremor in some cases. Erb speaks highly of arsenic. My own experience inclines me to favor the hyoscine or duboisine. B. THE SYMPATHETIC NEUROSES. ANGIONEUROSES AND TROPHONEU- ROSES. ANATOMY AND PHYSIOLOGY OF THE SYMPATHETIC 'nervous SYSTEM. The division of the nervous system into the cerebro-spinal and sympathetic is an artificial one. Both parts are intimately united anatomically and functionally and we cannot draw any sharp line of division between the diseases of the one or of the other. And it is probable that we never will, because it would seem, from both experi- mental and pathological observation, that the sympathetic merely regu- lates and controls in some way impulses that pass between the cerebro- spinal centers and the organs of vegetative life. In this fact, however, we can detect a slight specialization in the functions of the sympathetic. Symptoms of disturbance in the vegetative and nutritive organs indi- cate more or less involvement of the sympathetic, though doubtless conjointly with the cerebro-spinal apparatus. As the latter, however, cannot, it seems, produce certain manifestations without the conjoined activity of the former, we are justified in part in making a division be- tween cerebro-spinal and sympathetic diseases. The latter probabl> never occur without the former; the former very often occur without the latter. To illustrate, angioneurotic oedema is said to be a disease of the sympathetic nerve, but it always has associated with it cerebro- spinal symptoms. A localized myospasm is a cerebro-spinal nerve trouble, but it rarely has accompanying symptoms that point to the sympathetic. Much of our present knowledge of the Anatomy and Physiology of the Sympatlictic Nervous System we owe to the researches of Gas- kell, and in the following brief account I will follow his teachings. THE NEURONIC DISEASES FIGURE 43. Siwrior Cervical Ganglion of the Sympathetic; Its Connections and Branches. (Reduced from Flower.) ICtoIVC, Branches of commu- nication to lour upper cervical nerves. PS. Branches of communication to petrosal ganglion Vr, Branches of communication to ganglion of root of pneumogas- tric V, Branches of communication to gangnon of trunk of pneum >gastric. H, Branches of communication to hyp 'glossal nerve. C -\ Carotid plexus. C'P, Cavernous plexus. CA, Branches accompanying in- ternal carotid artery. OG, Branches to ophthalmic ganglion tk, To tympanic branch of glosso- pharyngeal. 3, to third nerve 4, to fourth nerve. 5, to fifth nerve, 6, to sixth nerve V, Vidian nerve to sphenopala- tine ganglion. S/», Large superficial petrosal from facial nerve. EAC, Accompanying branches of exiernal carotid artery. PP, Pharyngeal plexus, formed by union with branches of vagus and glossopharyngeal nerves. SG 7 Superior cardiac nerve. The Middle Ctrvical, or Thyroid Ganglion. IVC to VIC, Branches of com- munication with fourth, fifth, and sixth cervical nerve. IT, Inferior thyroid branches. MC, Middle cardiac nerve. ri , To recurrent laryngeal. The Inferior Cervical Ganglion. VI1C to VI1IC, Branches of communication with seventh and eight cervical nerves. IC, Inferioi cardiac nerve. C^, Cardiac plexus. GW, Ganglion of Wrisberg. LCP, Posterior, or left coronary plexus. RCP, Anterior, or right coronary plexus. CRL, Cardiac branches from pneumogastric or recurrent laryn- geal nerves. APP, To right anteiior pulmonary plexus. LPP, To left anterior pulmonary plexus. ID to IID, Branches of commu- nication from the first to the twelfth dorsal nerve. a, a, To a rta. vertebra, . oeso- phagus, and posterior pulmonary plexus. GSN, Great splanchnic nerve. SSN, Small splanchnic nerve. SSN', Smallest splanchnic nerve. D, Diaphragm. PN, Phrenic nerve. SP, Epigastii , or solar plexus. CLP, Cceliac plexus. C*, Cystic nerve. GSD. Gastro-duodenal plexus. CsP, Gastric, or coronary plexus. Pj>, Pyloric plexus. S/P, bplenic plexus. 1 GrE, Left g stro-epiploic plexus. Per, Pancreatic plexus. H/P, Hepatic plexus. V", Branches from pneumogastric. Dml\ Diaphragm- atic plexus. SG, Semilunar ganglion. SR*P, Suprarenal plexus. R»P, Renal plexus. S/P, Spermatic plexus. SMP, >ui eri^r mesenteric plex-nteiic plexus. LCI, 'e't colic plexus. Sz, Sigmoid plexus. SHw, Superior hem rrhoidal plexus IL to VL, Branches of communicati n with the five lumbar nerves. IS to VS, Branches of commu- nication with the five sacral nerves. C, Branches of communication with the coccygeal nerve. HP. Hypogastric plexus. IHP, Pelvic, or inferior hypogastric plexus, giving branches to all the pelvic viscera. 307 308 THE NEURONIC DISEASES The sympathetic apparatus consists of a double chain of inter- connected ganglia, lying on either side of the vertebral column and extending from the head to the pelvis ; of a number of more or less isolated ganglia, including the ophthalmic, the otic, the spheno-palatine and submaxillary in the head, various ganglia and plexuses in the thorax and abdomen, called cardiac, solar, hypogastric, aortic, sper- matic and renal ; and finally of various ganglia and plexuses in many of the viscera, such as the stomach, intestines and urinary bladder. Some physiologists regard the ganglia on the posterior spinal roots, the glossopharyngeal, vagus and sensory root of the fifth nerve as largely sympathetic in function. Rami communicantes, so-called, the visceral branches of the cerebro-spinal nerves, pass out from the latter into the lateral chain of the sympathetic, from which rami efferentes again emerge to enter the collateral ganglia, from whence pass still other branches to the various terminal ganglia in the organs of the body. Some fibres which can be traced back into both the anterior and posterior spinal roots are seen * to enter partly into the sympathetic chain and partly into the great splanchnic nerves. The white rami from the second to the fifth proceed upwards and join the superior cervical ganglion. Other white rami enter the lumbar and sacral plexuses. The gray rami do not represent, apparently, visceral branches below the second thoracic nerve-root and above the second lumbar. The white rami are, it would seem, the proper visceral branches. The white rami of the second and third sacral roots, however, com- mingle with the non-medullated fibres that pass from the ganglia to be distributed chiefly to the spinal column, the spinal membrane and the spinal nerve-roots themselves. All unstriated muscles receive branches from the sympathetic nerve ; hence all the viscera are innervated by it. The dilator of the pupil, the unstriated muscle of the lid, the erectores pilorum and the sweat and the salivary glands are all under its control. The cells are generally small and multipolar and possess an axis- cylinder process and numerous dendrites. The rami communicantes from the cord usually terminate in arborizations around the cell bodies in the sympathetic ganglia, making up thus the sympathetic neurones of the first order. The cell bodies lying in the sympathetic ganglia, with their neuraxones extending out to the unstriated muscles, con- stitute the sympathetic neurones of the second order. The following facts in regard to the distribution of the medullated visceral nerves emerging from the cord and entering the sym- pathetic and other nerves are of some clinical importance: They belong chiefly to the thoracic spinal nerve-roots. In the second and third sacral nerves they constitute the nervi erigentes, which pass directly to the hypogastric plexus and not into the lateral chain. From this plexus other fibres proceed to the inferior, mesenteric ganglia, to the bladder, the rectum and the generative organs. The white visceral rami of the upper cervical and cervico-cranial regions do not associate with the corresponding gray rami, but form the internal branch of the THE NEURONIC DISEASES 309 spinal accessory nerve. This branch passes into the ganglion of the trunk of the vagus. Visceral fibres are found also in the roots of the vagus, of the glosso-pharyngeal and in the chorda tympani, small petrosal and other cranial nerves. The origin of these visceral rami is presumed for many reasons to be in the cells of the posterior vesicular column of Clarke in the cord. They are also probably connected with the cells of the lateral horn of the spinal gray matter and its representative in the medulla, the antero- lateral nucleus of Clarke. The efferent nerve fibres of the sympathetic supply the muscles of the vascular system with vasoconstrictor, vasodilator, cardiac ac- celerator and cardiac inhibitory branches ; the visceral muscles with viscero-motor and viscero-inhibitory branches ; and the secretory gland cells with their special branches. Their names indicate sufficiently their functions. Xot much is known of the functions of the sympathetic ganglia themselves beyond that of modifying the histological structure and maintaining the nutrition of the fibres that pass through and out of them. They are probably not reflex centers such as are those in the spinal cord. How much of the metabolism of the body is directly pre- sided over by the sympathetic nerve is not known. Gaskell believes that in it are anabolic and katabolic, constructive and destructive fibres respectively, which go to every tissue and gland in the organism. The functions of the system may be said in general to be motor, vasomotor and secretory. The last may also be trophic, though that is still an open question. Symptoms of Lesions in the Sympathetic Nervous Apparatus. — Some of these have been long known from early physiological experi- mentation. Bernard many years ago severed the cervical sympathetic and observed dilatation of the blood vessels on the same side of the head, with increase of cutaneous temperature, contraction of the pupil, and occasionally retraction of the eyeball. Stimulation of the nerve produced contraction of the bloodvessels, decrease of the temperature, dilatation of the pupils, dilatation of the palpebral fissure, protrusion of the eyeball and sweating, all on the same side of the head as the nerve stimulated. Lesions and diseases of the nerve in which disturbance of the physiological phenomena belonging to it have been noted arc numer- ous. Traumata, compression from neighboring neoplasms, apical in- flammatory alterations of the lungs and pleurae and abscesses are among them. The symptoms are both irritative and paralytic. In such dis- eases as glaucoma and exophthalmic goitre we. cannot, as W< points out, say there is any constant peculiar change in the cervical sympathetic. In the aged, the diabetic and the syphilitic, in leukaemia, pellagra, tuberculosis and infectious diseases, changes have been ob- served of the nature either of atrophy, hyperaemia, sclerosis, pigmentary and fatty infiltration, amyloid degeneration, accumulation of colorless blood cells and microccocci in the ganglionic blood vessels. We know scarcely anything of the diseases of tin thoracic or abdominal sympa- thetic, either pathologically or symptomatically. 3IO THE NEURONIC DISEASES In associating the physiological disturbances sometimes observed in the vasomotor, cardiac and secretory spheres with disease of the sympathetic, one must remember that the same symptoms may be pro- duced by lesions in the cerebro-spinal system. Thus it is always an open question as to the exact location of the trouble in even fairly well defined sympathetic manifestations. Exophthalmic goitre, for instance, shows in many of its symptoms a possible relationship to sympathetic disease ; but those same symptoms might be well produced by a lesion in the cerebro-spinal apparatus, revealing themselves only secondarily through the sympathetic. The angioneurotic and trophoneurotic exhibitions generally are thus often only secondary sympathetic disturbances, the primary trou- ble being back in the cerebro-spinal apparatus. Tn universal inherited and acquired degenerative states, in general cachexia and infection, it would seem singular for the cerebro-spinal system to suffer and the sympathetic to escape. Hence we conclude that in these general neu- roses both parts of the nervous apparatus are implicated. We insist therefore that the nervous organism of the entire body is a unit and cannot be artificially divided physiologically as it is done anatomically. Among these sympathetic neurotic exhibitions we note flushing and pallor of the face, sensations of heat, rapid pulsations and excessive sweating. The heart palpitates, the head seems to suddenly fill up with blood, the ears ring, the eyes grow misty. It may be a sign of the sympathetic origin of these manifestations that through them all the conscious state is not disturbed nor are there any somatic muscular disorders or objective sensory phenomena. In hysteria, neurasthenia, profound nervous shocks, marked physiological strains such as puberty and the climacteric period involve, masturbation, sexual excesses and high mental excitement, the angioneurotic symptoms are not infre- quently observed. SYMMETRICAL GANGRENE. RAYNAUD'S DISEASE. This is a rare affection of the peripheral vascular system, charac- terized by a local asphyxia and gangrene, and dependent upon some disturbance of the vasomotor nervous apparatus. It occurs independ- ently or in the course of other nervous affections, such as hysteria, tabes dorsalis, syringomyelia, spinal tumor, epilepsy, exophthalmic goitre, scleroderma, etc. Etiology. — Heredity does not enter into its etiology, but a neu- ropathic constitution is probably a factor in every case. The young and middle-aged are most liable to it. Women are more frequently its victims than men. It has been observed in the nursing babe. A child three years of age completely recovered after losing, from this disease and spontaneous amputation, both arms above the elbow and the left leg below the knee. In children the disease occurs largely through the influence of the nervous system. In old people through the influence of the heart and circulation. Ancemia, chlorosis, exhaustion and physical defects, such as a congenitally small aorta, serve as predisposing causes. Exposure to THE NEURONIC DISEASES 311 cold, emotional stress, suppression of menstruation and traumata of various sorts have been invoked as causes of the disease. It has followed various infectious and cachectic and debilitated states, such as malarial fever, rheumatism, neurasthenia. It has occurred in asso- ciation with tuberculosis and nephritis and in morphine and chloral intoxication. Diabetes and syphilis have been blamed for i- Pathology. — Of the pathology of Raynaud's disease we know very little. Raynaud himself suggested that there was a local spasm or contraction of the finer blood vessels. The smaller arteries are com- paratively abundantly furnished with muscular tissue and so are capable of active changes in their calibre. Their nerve-supply from the sympa- thetic is under the control of the spinal cord and is subject to influences in the way of cold, heat and dampness from the periphery, or from central disturbances caused by a toxic and vitiated state of the blood, or from an abnormal and unwonted impression sent down from the brain and the higher psychical centers. These provoke a spasm of the arteries and veins. An endarteritis and an endophlebitis have both been seen by Dehio, while Pitres and Vaillard have found neuntic changes in the nerves of the fingers. These were all probably secondary effects and have no bearing upon the pathogenesis of the disease. Symptoms. — The chief symptom of Raynaud's disease is a pecu- liar vascular spasm, appearing suddenly, paroxysmally and symmetri- cally at the ends of the limbs. Usually two or three fingers of both hands or several toes on both feet experience a paraesthetic sensation of some sort, crawling, numbness, deadness (digiti mortui). They as- sume a waxy paleness, are cold and look shrunken. Some anaesthesia is present and often severe pain precedes and continues during the onset. Blood does not flow if the fingers are pricked with a needle. This is the stage of local syncope or regional ischavuia. As a rule it lasts about an hour or so, and is then followed by a reaction in which burning sensation, redness and heat are observed. This may disappear in the course of a few hours more and the limb remain perfectly normal. More frequently, however, the syncope returns and becomes more of a permanent condition. In a few minutes or hours cyanosis or local asphyxia takes the place of the initial pallor. The skin becomes dusky, mottled, blue-black and finally black. The pains become intense and unbearable in tin hands and arms. Anaesthesia is not usually present, though it may be. The fingers are swollen and subject to slight hemorrhages and bullae. On account of the tension of the muscles and skin the lingers are stiff and awkward. The gait may he affected or the patient may not at- tempt to walk at all. Any part of the body may be the seat of these manifestations. Usually, however, they appear in the lingers and toes first, <>ih- finger or toe being affected sooner and more extensively than its neighbor. After the extremities the ears, the nose and areas of skin upon the arms and legs are involved. Such attacks may occur paroxysiually for years, being provoked by exposure to cold, as in winter, or by gastric . mental and other forms of disturbance. During all this lime the general health remains good. 312 THE NEURONIC DISEASES More commonly this second stage of local asphyxia is followed by local gangrene. In the painful and mottled areas necrosis begins and may go on to the formation of complete sloughs, or it may retrograde and leave only a scar. Black spots first appear in the gangrenous areas, with vesicles and blisters which fill with bloody serum, burst and leave an exfoliating dark crust, or a slow-healing ulcer. The ulceration is superficial and when healed leaves a permanent scar. The repetition of this process leaves the tips of the fingers shrunken, irregular and unshapely from cicatricial formation. In severe cases, which, however, are rare, the necrotic process extends, mummifying the whole end of the finger or toe and establishing a line of demarkation at which spontaneous amputation may take place. Suppuration is slight and in the course of a little time the stump gradually heals. In this way a part of the foot or hand, the lobe of the ear, the tip of the nose or tongue may be lost. Symmetrica? areas upon the trunk ma}' be affect- ed and a condition even of multiple gangrene be set up. Such cases occur chiefly among young children, and on account of the intense suffering, the restriction of movement and the exhaustion, terminate in death in a few days. The relationship of hemoglobinuria to Raynaud's disease is re- markable and mysterious. It usually occurs in connection with the gangrenous stage. It may be present in the attack or it may take the place of an outbreak. The similarity of symmetrical gangrene and hsemoglobinuria is shown in the fact that both may be brought on by exposure to cold, the paroxysms often being preceded by a chill. In the one case the nerves and blood vessels are chiefly affected ; in the other the blood and blood-making organs. Albumin, haemoglobin, and red corpuscles are all found in the urine. Epilepsy has been seen in these haematuric cases. As a rule there is no fever, but the general strength and constitu- tional vigor are lowered by the continuous, unbearable pain, insomnia and loss of appetite. Mental dullness, temporary attacks of unconsciousness and other psychic and cerebral manifestations are not uncommonly observed. With depression they may even appear among the prodromes of the disease. Aphasia has been noted with transient right-sided hemiplegia. Delirium, delusions and mania must be added to the occasional cerebral manifestations. Sensibility is usually disturbed and conduction noticeably dimin- ished. Analgesia is probably the most common of the sensory modi- fications. Tactile sense is usually blunted, though the thermic sense remains intact. Trophic disturbances are exhibited in the ioint changes, including acute synovitis, swelling, thickening of the phalangeal articulations and anchylosis. These are multiple and attack several joints at the same time. Scleroderma may be associated with Raynaud's disease, and paronychia may develop between the attacks. The nails may be altered, the skin hypertrophied and the smaller muscles of the hand somewhat atrophied. Ocular disturbances, loss of hearing-, tinnitus aurium and modifier- THE NEURONIC DISEASES 313 tions of taste and smell are not unknown, and sympathetic paralysis has been evidenced in certain oculo-pupillary phenomena. Thus the symptoms and complications of Raynaud's disease point to an implication of the entire nervous system. The immediate cause of the symmetrical gangrene, however, is obscure, though the indica- tions point to a functional instability of the vasomotor apparatus. The remote cause of this instability is in all probability a constitu- tional and neuropathic one; the immediate cause may reside in some form of disease in the peripheral nerves, the cord, the brain or the sympathetic system. Diagnosis. — The diagnosis of Raynaud's disease must take into account the early age of the patient, the bilateral and symmetrical character of the symptoms, the successive order of local syncope, asphyxia and gangrene, the implication of sensory, motor and trophic FIGURE 44. Raynaud's disease. (After Affleck.) functions and the absence of primary disease of the heart and blood vessels. It is not difficult in typical cases. Erythromelalgia is closely related to Raynaud's disease, but it never produces gangrene and the pains are usually relieved by cold instead of being made worse by it, as in the latter affection. Endarteritis obliterans does not present in successive paroxysms the syncope, asphyxia and gangrene observed in Raynaud's symptom- complex. In lepra mutilans several fingers are simultaneously and equally affected with gangrene and fall off without the presence of any pain. Neuritis is not paroxysmal nor gangrenous, though it may give rise to confusing trophic symptoms in the skin. Moreover, the etiology and symptoms of neuritis are so characteristically different from those of this disease that a differential diagnosis can usually be easily made. In senile gangrene the age of the patient, the presence of cardiac and vascular disease and the non-paroxysmal course of the trouble is to be remembered. Ergot poisoning resembles symmetrical gangrene in some resp< and Ehlers has argued that this and the allied tropho-neuroses wen- 314 THE NEURONIC DISEASES all due to ergot intoxication. Tingling in the fingers and feet, cramps in the legs, arms and chest, dizziness and weakness, cold feeling all over the body, dilatation of the pupils and feebleness of the pulse, with retching, vomiting, diarrhoea and increased salivary secretion are the usual symptoms of ergot poisoning. Epidemics of gangrene have occurred from eating bread made from ergotized rye. Prognosis. — The prognosis is favorable in regard to life, except in the severe and multiple types of the disease. And yet patients even recover from these though mutilated and deformed. Death has very rarely occurred directly from this disease ; some complication has usu- ally been the cause of the fatal issue. No cure is known for it. Usually, therefore, it continues for years, appearing in periodical attacks. Treatment. — There is no known specific remedy. General hy- gienic measures must be instituted and the nervous system especially must be kept quiet and in a high state of health. The nutrition especial- ly must be maintained by gentle stimulation and abundance of easily- digested food. The bowels must not be allowed to become costive, the kidneys sluggish or the skin inactive. A regular and systematic out- door life and exercise must be adopted. The patient must be guarded from cold by suitable clothing and the removal to a warmer climate in winter. The administration of the iodides, chloral and nitroglycerin may be tentatively tried. Benefit from the nitrites is very doubtful. Hutch- inson advises the tincture of opium in three or four drop doses, three or four times a day, in combination with general tonics. Galvanism to the spine and sympathetic has been recommended. The pains are best combatted by local warmth, very gentle mas- sage and lukewarm hand and foot baths. Anodynes may be resorted to, but the hypodermic use of morphine may excite local gangrene. Cushing obtained relief from the intense burning pains, and improve- ment of the circulatory condition, by the gentle application of the tourniquet daily to one or the other limb, according as the symptoms indicated. ERYTHROMELALGIA. This interesting symptom-complex was described by Weir Mitchell in 1872 and named thus by him in 1878. Our knowledge of it is almost entirely clinical, in spite of the fact that there are now about fifty cases on record. It has been studied by Mitchell, Lannois, Collier, Hamilton, Sachs, Eisner, Rost, Dehio, Auerbach, Shaw and others. Mitchell regarded the trouble as morbus sui generis, but the opinion is growing that it is nothing more than a symptom of disease of the central nervous system, or peripheral arterial apparatus. Males are more frequently stricken with the trouble than females. The only known ec/uses for it are cold, a neuropathic diathesis and syphilis. The pathology of the trouble is but very little known and the pathogenesis not at nil. The only cases that have been examined satisfactorily post-mortem were those i^i Auerbach, Hamilton and THE NEURONIC DISEASES 315 .Shaw. Edinger's findings in Auerbach's case consisted chiefly of r thickening of the intima and media of some of the distal blood vcsseis ; some degeneration in the nerve bundles of the cauda equina; degen- eration of the lower part of the posterior columns of the cord ; and some degeneration in the median part of Coil's column in the thoracic and cervical cord. The peripheral nerves were normal. In Sach's case the nervous tissues were normal, but the arteries were all diseased in the limb that was amputated on account of gangrene. In Hamilton's case there was some neuritic degeneration, but it was insignificant in coi. parison with the arterial disease. He concludes that the disease is more nearly related to Freidlander's obiiterative arteritis than to a degenera- tion of the spinal cord. Shaw's more recent findings lend support to FIGURE 45. Section of an artery of the great toe in Erythromelalgia. (After Mitchell -and Spiller.) this view. Nevertheless it is believed by many that erythromelalgia is a symptom-complex indicative of organic or functional disease of the central or peripheral nervous apparatus. By some it is considered to be an angioneurosis ; by others a disease of the posterior and lateral spinal gray matter. Plantar neuritis and acroneurosis are two other explanatory hypotheses. In view of its uncertain pathology and hypothetical pathogenesis, we must still look upon it as simply a re- markable symptom-complex. Burning pain and reddening of the skin in the feet, and more rarely in the hands, are its chief clinical manifestations. The pains usually appear suddenly, with or without fever, especially in the soles of the feet and in the hands and fingers. 'They may extend into tin- legs and arms. The parts soon become intensely red, hot and swollen. The blood vessels pulsate and there is local sweating. The pain is •of a burning character, usually .severe and even excruciating. It is more or less constant, though sometimes it remits. It is relieved by cold; aggravated by heat. Recumbency affords relief, whereas walk- ing markedly increases it. Standing increases the pain, the pulsation •of the arteries, the enlargement of the veins and the mottling of the 3l6 THE NEURONIC DISEASES skin. (Edema is not uncommon. The objective sensibility is not very- much altered, though both hyperesthesia and slight hypaesthesia sometimes obtain. In this there is a suggestion of a peripheral neuritis.. Other symptoms besides these have been observed in conjunction with erythromelalgia, but they are not considered as part of the latter symptom-complex. There may be headache, vertigo, palpitation and attacks of syncope. Widespread arteriosclerosis and cardiac lesions have been found in some of the cases. Angina pectoris and functional disturbances of the heart have been reported in other cases. Unilateral sweating, thickening of the veins, enlargement of the bones, an atrophic: condition of the muscles of the limbs, hemiplegia, muscular dystrophy with cerebral tumor and other indications of disease of the centraL nervous system and of the peripheral nerves have all been noted in various cases. From the standpoint of symptomatology the conclusion of Lewin and Benda seems justifiable — namely, that there are among the re- corded cases three distinct classes: (i) Those with true organic dis- ease of the central nervous system; (2) Those with functional disease of the central nervous system ; (3) Those with peripheral disease of the nervous system, either functional or organic. Until more data of a pathological character are forthcoming to- uphold the view that this symptom-complex is dependent upon a primary disease of the arteries and their obstruction, we must accept the symptomatic evidence that it is a manifestation of some primary trouble in the nervous system. Obstinate and chronic, the trouble may last for years with remis- sions and exacerbations. The heat of summer aggravates it. During its course temporary convulsions and spells of unconsciousness have been witnessed. It is not dangerous to life in the absence of serious complications, but exhaustion, gangrene and complicating diseases add to the gravity of its prognosis. It is very resistant to all forms of treatment. Faradism, cold and the antipyretics of the coal-tar group have been tried for the pains. In exceptional instances warmth has seemed to give the most relief. In one case it disappeared after extirpation of the ulnar nerve. ACROPARESTHESIA.. This is a vasomotor neurosis of the extremities which sometimes may be regarded as a disease, though in the vast majority of cases it is a mere symptomatic trouble. Without objective signs of any sort, it leveals itself in paroxysmal attacks of numbness, tingling and other forms of paresthesia in the hands and feet. The condition was described by Putnam, of Boston, in 1880 and by Dana, of New York, in 1885. It remained, however, for Schultze* of Bonn, to name it acroparesthesia in 1893 and to awaken the pro- fessional interest in it. Many writers have since extensively treated of it, notably Laquer. Ormerod, Bernhardt, Berger, Friedman, Sinkler, Gallois, Charbo, Collins and others. Etiology. — Females are almost twice as often affected as males. THE NEURONIC DISEASES 317 Most of them are about thirty or forty years of age. The climacteric seems to favor it. Nationality or occupation does not seem to have any special influence. The heredity, if any, is only that of a milol, general neuropathy. Convalescence from disease, a general cachexia and a state of debility have underlain it. Syphilis is not a cause. The things to which it has been attributed are so numerous that one is forced to the conclusion that none of them are causative except as mere excitants or as factors in producing a general nervous debility. In most cases no cause can even be suspected. Exposure to cold, sudden changes of temperature, as in laundry work with the hands, sewing, darning, long bathing, miscarriage, free use of alcohol and tea, fright, pregnancy and so on, ad infinitum, have been the antecedents of the trouble even as in all other neuroses. Pathology and Pathogenesis. — Of the pathology we know nothing, and of the pathogenesis we merely guess from the symp- tomatology that there must be a condition of irritation in the vasomotor centers which affects the nutrition of the peripheral sensory apparatus. Neuritis may be excluded, for it reveals none of the symptoms. All that we can say is that it is a vasomotor neurosis. Where the primary trouble is would hardly be worth guessing. Symptoms. — These are entirely subjective and sensory. They come on suddenly, often at night, even awakening the patient out of her sleep. The sensations are as varied in number as the patients themselves. The term paresthesia covers them all. Most prominent among them are numbness, tingling, formication, a feeling as if the limb, or rather more often, the finger-tips, were asleep. In rare cases the toes are affected. Sometimes the condition is actually painful ; at other times it is a distressing, itching, cold, wooden, swollen, stiff sen- sation. The movements of the extremity are usually restricted on account of the feeling. The trouble is more or less continuous, with exacerbations. It has been known to endure from a week to somewhat more than a year. The trouble may be confined to certain parts of the hand and foot, or to one hand. The rubbing and warming attitude and performances of the patient are characteristic. There are no uni- form objective signs; usually none at all. Sensation may be some- what decreased in the fingers or there may be a pallor. There are no motor disturbances. Other angioneurotic manifestations have been witnessed, such as the complaint of the patient of blood rushing to the head, of palpitation of the heart, etc. Tin- disease Is chronic, and, like all the primary neuroses, has an unsatisfactory prognosis. The diagnosis involves its differentiation from symptomatic paresthesia, from vascular disease and from other central organic and functional troubles. Care in the examination will make the differen- tiation easy, however. Raynaud's disease may simulate it. but there is local asphyxia and gangrene in this serious affection. Tetany, hys teria, acromegaly may all at times exhibit a resemblance t«. acroparaes thesia, but the appearance of other symptoms will soon distinguish them. Treatment. — Remove, of course, any discoverable cause. Gen -eral tonics, such as arsenic, phosphorus, strychnine, quinine and iron 318 TllK NKUKONIC DISEASES are all useful on general principles in combating a neurosis. Ergotirr was recommended by Sinkler. Electricity in the form of central galvanization, medulla, cervical cord and sympathetic being brought within the current, and local faradization may be tried. ANGIONEUROTIC (EDEMA. Angioneurotic oedema, first definitely described by Quincke and his pupil Dinkelacker, is chiefly remarkable for the circumscribed swellings that appear on the face, neck and extremities, without ap- parent cause or previous warning. For this reason it has sometimes been called periodic swelling, urticaria tuberosa, giant swelling. There is almost always present gastro-intestinal disturbance, which is prob- ably of an (Edematous nature, like that of the exterior of the body. The mucous membrane of the larynx may be the seat of the sudden oedema and so cause an alarming dyspnoea or even death by suffocation. Etiology. — The etiology of the disease takes cognizance of the early adult age of the patient, its more common occurrence in the male sex, the occupations which tend to excessive fatigue of mind and body, and a certain natural or acquired neurotic state of the constitu- tion. Heredity seems to play an important role, as shown by the surprising series of cases, all in one family, reported by Osier and Milroy. In six generations of this family twenty-two individuals were the victims. Krieger's case was a young man twenty-five years of age,, whose mother was similarly affected. Cold and traumatism are frequent exciting causes of the attacks. The former in conjunction with lowered vitality may explain to a certain extent why, as in one of my own cases, the trouble was so prone to appear toward the early morning before the patient awakened. Unusual muscular exercise will sometimes precipitate an attack. I have seen a hard day's shopping, the patient passing from one store to another through the cold wintry air and climbing flights of stairs, do it. Alcoholism predisposes to this disease, likewise in some cases a sojourn near the sea. Nervous individuals are more frequently the victims. It has been observed in association with neurasthenia, exoph- thalmic goitre, hysteria and urticaria. Pathology and Pathogenesis. — The disease being so rarely fatal, very little opportunity has been afforded for making a thorough pathological examination. Its occurrence in markedly neurotic in- dividuals, and its many resemblances to a general neurosis, suggest that it probably has no discoverable lesion in the nervous system. It is probably related to hysteria, though the absence of the usual, well- known stigmata of the latter disease and other symptoms tend to prove that it is not pure hysteria. It is most likely a functional trouble of the sympathetic and central nervous apparatuses, but the cause and character of this trouble are, at the present time, entirely unknown. In this connection it is suggestive to note that Schlesinger reported a case in which this acute oedema was seen in combination with erv- thromelalgia and Raynaud's disease. Quincke himself regarded the THE NEURONIC DISEASES 319 basis of the trouble as a vasomotor neurosis, the vessels being suddenly dilated. Symptoms. — The patient may be apparently perfectly well and remain indoors all day. Then upon going out into the open air toward evening, or upon taking a longer walk than usual, there may come on suddenly and without the slightest premonition an attack of dyspnoea, completely frightening and unnerving him. The extremities become cold, perspiration ceases, a slight cough arises and a feeling as if the stomach were suddenly distended. Then quickly follows the characteristic ccdematous enlargements upon the arms and face and elsewhere. These swellings are circumscribed and resemble giant hives, except that they neither itch nor give any pain. They measure from two to ten centimeters in diameter, are pale in color or of a deeper red than the surrounding skin. They do not pit upon pressure, like ordinary dropsical effusions, but if firmly pressed with the tip of the finger they quickly rise to their former level. Some patients com- plain of a local sense of burning or scalding, otherwise these tumefac- tions do not produce any distress. This circumscribed oedema, which may attack the mucous mem- branes as well as the skin and subcutaneous tissues, is to be differen- tiated in this disease by the abruptness of its onset, its brief duration and its rapid disappearance. Any part of the body may become its site, especially the face and extremities. In a migratory sort of a way it may vanish from one part, to quickly reappear in another, ft rarely lasts over a few hours. It is strictly non-inflammatory. It is probably true that the alarming dyspnoea and associated gastro -intestinal trouble are the result of the same oedematous process going on within the mucous membrane. Krieger's case, a typical one, was found dead in bed one morning and an autopsy showed that death was caused by sudden oedema of the glottis. Collins finds that out of seventy-two cases three showed their initial symptoms to be located in the stomach and in thirty-four per cent, of them all, gastro-intestinal manifestations were of sufficient importance to attract notice. The throat was involved in about twenty-one per cent, of all cases, a fact which should be carefully remarked, as death has more than once resulted from oedema of the larynx. The stomach symptoms arc, gen- erally, a sense of uneasiness and extreme tension, loss of appetite. enlargement of the epigastrium, colic and sometimes profuse vomiting and intolerable thirst. Osier says the pains may become so severe at times as to require the administration of morphia. In one of my cases there was at no time an) marked pain, but an intolerable sense of fullness and of retained undigested matter, with a bloated appearance of the epigastrium. Nausea and periodical vomiting may occur and are thought to In- due to the internal urticaria. The patients are usually constipated, though a colliquative diarrhoea sometimes follows the subsidence of an attack. Often the urine is voided in large amounts. Albuminuria casionally occurs and in one instance led to the* erroneous diagnosis of renal disease. Oppenheim saw a case where the suddenly appearing 320 THE NEURONIC DISEASES articular swelling simulated an attack of gout. Higier reported a case in which the oedema of the velum palati led to attacks that resem- bled pseudocroup. Hemorrhages have occurred from the mucous membranes and in one case Joseph saw a paroxysmal hemoglobinuria complicated with this disease. The frequent association of angioneurotic oedema with hysterical or hysteroid manifestations is striking. For a long time I regarded one of my cases as pure hysteria. In some cases, as in one reported by Collins, hysteric attacks preceded for some years the outbreaks of the cedematous trouble. Irregularities of the menstrual function seem to perform as much a part in the disease under consideration as they do in true hysteria. Collins, Lewin and Quincke cite instances in which the onset and dis- appearance of the migratory cedematous swellings bore a certain rela- tionship to menstruation. Amenorrhcea was sometimes accompanied by a monthly swelling of the ankles, lips or eyelids. Complete exhaustion and nervous anxiety often terminate an attack of the oedema. It was with great difficulty that one of my patients could be persuaded that her heart action was normal, so weak and prostrated did she feel when about to recover. Between the at- tacks the general health is good. Diagnosis. — The diagnosis of angioneurotic oedema is not dif- ficult if the characteristic symptoms are present. These symptoms are especially the local swelling of the skin, the gastro -intestinal dis- turbance and the nervous depression. Giant urticaria is a disease so closely related to angioneurotic oedema that some have thought they were the same. The ©edematous manifestations of hysteria are usually associated with the well-known motor, sensory and psychic symptoms of this latter disease. Prognosis and Course. — Angioneurotic oedema is rarely fatal, in spite of the severity of the attacks and the nervous depression often associated with them. The fatal cases that have been reported were probably due to asphyxia caused by the closure of the glottis from the cedematous swelling. This oedema of the glottis is to be dreaded as a possibility in all cases. One of my own, a woman, had been seized one night with one of her usual attacks and died of suffocation before the physician in the neighborhood summoned in the emergency was able to do anything. The disease is essentially a chronic one, lasting sometimes a whole lifetime. The general health is not greatly disturbed. While much can sometimes be done to lessen the frequency and severity of the attacks, the underlying constitutional trouble, the dis- ease itself, so far has proved intractable to all forms of treatment. Treatment. — No remedy has yet been suggested for its relief. Neither the disease itself nor the attacks arc amenable to any known treatment. The salicylates, especially the salicylate of soda, have given the best results in the hands of others, as well as in my own. By them the intervals between the attacks appeared to be lengthened, but as for lessening the severity of the attacks, or in any way actually pro- THE NEURONIC DISEASES 32 1 PS. This is a condition referred to by Moore, who first described it. by Oppenheim, by Fere and others. Usually the knee, sometimes the other joints of the extremities, the spinal column or the maxillary articulations are effused with fluid regularly every few days or weeks. 322 THE NEURONIC DISEASES A mild fever has occurred in some cases. There are absolutely, how- ever, no other local or general symptoms. It is said to be an inde- pendent disorder or an accompaniment of one of the general neuroses, like exophthalmic goitre. Fere has described a permanent hydrops. The disease has been associated with trauma, malaria and other causes without good reason. It has no relationship to rheumatism, but is a pure neurosis. It is obstinate, but sometimes goes away of itself or alternates with polyuria, hyperidrosis or the symptoms of exophthalmic goitre. Treatment is not satisfactory, though it includes the salicylates, arsenic, quinine, ergotine, laxatives, electricity and hypnotism. EXOPHTHALMIC GOITRE. This is often called Graves' disease because this English practi- tioner wrote a clinical account of it in 1835, which attracted the atten- tion of the medical world. On the Continent the name of Basedow, of Germany, is usually employed, because he described it independently in 1840. If any physician's name is to be attached to it, that of Parry has the strongest claim, since he has left in his posthumous writings a very fair clinical portrayal of his first case, seen in 1786, and others observed later. The contention of the Italians that Flajani fully de- scribed it in 1800 is not well substantiated. Our present knowledge of the affection does not permit of its exact classification. Clinically it is a neurosis. Pathologically and pathogenetically there is some ground for suspecting that it is a thyroid disease. A strict definition, therefore, can only include a few meager facts in regard to its etiology and the symptom-complex tachycardia, enlarged thyroid, protrusion of the eyeballs and certain neurasthenic and vasomotor manifestations. Etiology. — Heredity enters as an important factor in the etiology of Graves' disease. In a few instances it is direct. A mother and two sons under my care have had it, the latter recovering completely. In another instance an aunt of the patient suffered from it. Similar examples have been observed and reported by others. In the majority of cases, however, the heredity is only indirect. Various other neu- roses, such as neurasthenia, chorea, epilepsy, hysteria, psychoses and nervous strains from excessive indulgence in alcohol and tobacco, can be recorded in the family history. A neuropathic condition is trans- mitted to the offspring and through the female line. The affection is sometimes called a "woman's disease," as it has been observed to occur ten times more frequently in women than in men. It appears at all periods of life, but most commonly in the second decade of women, and the third of men. Worry, fright, anxiety and extreme emotional depression have often been observed to precede it, but whether they were causative or symptomatic has not always been clear. All exhausting influences, general debility and anaemia predis- pose toward it. T have seen cases that followed pregnancy, abortion, the puerperinm and prolonged spells of illness. Abnormal menstrua- tion, cardiac disease, infection (scarlet fever, rheumatism, pneurrlonia, THE NEURONIC DISEASES 323 syphilis, etc.), diarrhoea, excessive use of alcohol, over-exertion, like mountain-climbing, and injuries to the head, have at various times been assigned as causes. If exophthalmic goitre is primarily due to disease of the thyroid gland, some of the above-named factors cannot reasonably be looked upon as causes, much less as symptoms. And yet they are too fre- quently and too intimately associated with it to be regarded in the mere light of accidents or coincidences. As etiological factors they can fit in with the conception of the disease as a primary neurosis — a conception which is further supported by such elements in the etiology as the sex, age and heredity. Pathology axd Pathogenesis. — The post-mortem findings are so indefinite that even at the present day nothing but hypotheses can be offered in explanation of the nature of the disease. Among the various theories only two are worthy of special consideration — namely, the one that regards the trouble as a primary neurosis, and the other that looks upon it as an intoxication from primary disease in the thyroid gland. In the former theory it is assumed that there is a functional dis- turbance, toxic or quasi-physiological, within the cellular elements of the central nervous apparatus, and that ail of the symptoms of the disease, cardiac, glandular and ophthalmic, are the direct result of the neurosis and are in no way dependent upon one another. Attempts have been made to localize the neurotic process in the medulla ob- longata (Filehne, Durdufi, Bienfait, Sattler, Fitzgerald), the cerebral cortex (Newman) or the peripheral apparatus, including especially the vasomotor sympathetic system (Abadie). None of these efforts have proved successful. The immediate cause of the neurosis is wholly unexplained. The other theory of the disease is the so-called "hyperthyroida- tioir' or "dysthyroidation" theory. It was first promulgated by Mobius, of Berlin, in 1886, and has been ably supported by Wette, Muller, Jofrroy, Brissaud, Segnault, Boinet, Bramwell, Murray, Adam and Greenfield. As a result of disease in the thyroid gland, an exces- sive or altered secretion is poured out into the circulation and this, either by its direct toxic effect or by its disturbance of the general metabolism, causes all of the nervous manifestations and their ondary results. Chronic iodism or thyroidism, depending upon the thyroiodin or iodothyrin, a normal substance found in the gland, both free and in combination with a proteid and containing 9.3 per cent, of iodine and 0.56 per cent, of phosphorus, is believed to be the essential trouble. The origin of this glandular disease is not explained, though a few authors fall back upon some form of nervous trouble, possibly a neu- rosis, in accounting for it. This thyroidation theory i^ popular just at present; is supported by various more or Jcal arguments; and affords a basis for the surgical treatment of the di& Hypertrophy and persistence of the thymus gland has often been seen. The other pathological findings, such as the degenerative changes in the sympathetic, the inflammatory and other foci of disease in the 324 THE NEURONIC DISEASES restiform bodies and elsewhere, and even the cirrhosis, hyperplasia and vascular alterations in the thyroid gland itself, are scarcely constant and decisive enough to be regarded as the histopathological foundation for the disease. Until more constant post-mortem evidence to the contrary is forthcoming we must still regard exophthalmic goitre as a mere symp- tom-complex and largely of the nature of a neurosis. The numerous hypotheses put forth to explain its pathogenesis, while interesting and suggestive, are none of them established as positive facts. Symptoms. — The onset of the disease varies. After some un- wonted exertion, hemorrhage or mental shock the symptoms may promptly appear and rapidly increase. After some prolonged physical strain, worry or infectious disease they usually come on gradually and develop slowly. Once developed they are more or less permanent, though they may be subject to occasional remission. In some mild cases they quickly appear and disappear. In the acute severe cases they usually subside somewhat and the disease becomes stationary and chronic. The clinical presentation of Graves' disease includes the tripod of symptoms tachycardia, struma and exophthalmos. Not all of these are present in every case, nor are they all equally prominent when present. There are other symptoms that are almost of like importance, as, for example, trembling, hyperidrosis and mental depression. After these are a long list of neurasthenic and hystero-neurasthenic mani- festations in regard to which it is not always easy to say whether they are symptoms or complications. The tachycardia, or irregular rapid action of the heart, is the earliest, the most constant and subjectively the most obtrusive of all the symptoms. So pathognomonic is it that when it cannot be account- ed for on the basis of any other organic or functional heart disease, a diagnosis of incipient exophthalmic goitre is allowable. It may come on abruptly or gradually. Often it is the only symptom that sends the patient to his physician ; at other times it is only discovered as the result of a most painstaking examination. The phenomenon is like that which occurs when a person is unduly excited. Subjectively the heart seems to throb violently and spasmodically without rhyme or reason. Nervous excitement may induce an exacerbation or increase it. Objectively it is observed to beat irregularly and with an exalted degree of force. In bad cases it flutters and pounds and seems like a caged animal struggling to set itself free. The impulse is sharp and noticeable over a wider area than normally. In mild cases the beats number about 100 to the minute; in the majority of the cases it rises to 120 or 140; in the more severe cases it may even go to 200. Rarely docs it ever subside to the normal, except in the mild cases. Some- times the pulse is regular and small, while the arteries beat vigorously. With all this energy the circulation is not actually hastened. The condition is merely a sort of delirium cordis, representing not strength. but rather lack of inhibition. Usually there is no indication of an organic lesion upon physical examination. Slight enlargement of the heart may be detected later THE NEURONIC DISEASES 325 on, this being clue to dilatation rather than to hypertrophy. Occasion- ally a systolic murmur is heard near the base of the heart. Graves stated that in one case this was audible at a distance of four feet from the patient. As the result of the dilatation a sound suggestive of mitral regurgitation may be heard at the apex. All of these are sec- ondary manifestations and when dependent upon actual organic lesions are to be looked upon as complications. Valvular defects from slight endocarditis have been occasionally found post-mortem. Severe paroxysms of palpitation, extreme violence of action, accom- panied by dyspnoea and signs of dilatation, without hypertrophy, are among the later cardiac manifestations. The arterial system becomes dilated from the lowered tone of the vascular walls and the increased pulsations of the heart. The carotid arteries especially, the thyroids and even the abdominal aorta, exhibit an exalted pulsation. A loud systolic murmur may be heard in the neck and is rarely ever entirely absent. A curious fact is that the smaller arteries beat comparatively less forcibly than the larger-sized vessels. The entire arterial system, however, is subject to the peculiar throb. In a few instances, which are exceedingly rare and in which the thyroid was markedly enlarged and variable, the exophthalmos slightly observable, and the tremor, sweating and neurasthenic symptoms were present, I could not detect the slightest alteration in the heart action. These cases sought medical advice solely on account of the increased size of the neck. Xext to the heart phenomena the enlargement of the thyroid gland is the most frequent and important symptom. Occasionally, as I have seen, the first and only thing to attract the patient's attention is the increasing tightness of her collar. In one or two instances the friends detected the enlargement before the patient did. Objectively it is the most noticeable symptom, and as it appears sooner or later in almost every case, it is to be regarded as a cardinal symptom. Those who believe that the thyroid is the primary scat of the entire disease find some difficulty in explaining the cases in which it fails to enlarge, and in the exceedingly uncommon cases in which it actually diminishes. They are driven to assume that the gland is just sufficiently diseased to produce an abnormal secretion without be- ing itself altered in size. The enlargement of the gland is gradual and slow. It begins usually after the tachycardia has existed for some time. It is not a uniform enlargement. Most frequently the right side is bigger than the left. Tt may be equal on both sides. The isthmus is rarely in- creased. Sometimes there are remissions, or rather the gland alter- nately enlarges and recedes. Later on it becomes permanent. The patient realizes the change in the gland by the tightness in her dl and occasionally by a distressing sensation as though something were about to choke her. The degree of enlargement is usually not ex- cessive and very rarely as much as it is in ordinary goitre. Tin- super- ficial veins over the gland are dilated. Palpation sometimes reveals a slight thrill and auscultation enables one to detect a soft, blowing murmur synchronous with the- cardiac systole. ( iuttmann decla that a double murmur is pathognomonic. 326 THE NEURONIC DISEASES The protrusion of the eyeball is a variable symptom, though highly characteristic of the disease when present. It is the least frequent of the three cardinal symptoms and it is often entirely absent. It usually appears with the enlargement of the thyroid. It rarely occurs alone. It may be unilateral, more commonly it is bilateral. The right eye is apt to be a little more prominent than the left. Generally the condition is slight and sometimes it is barely noticeable. It may be so marked, however, that the insertions of the recti muscles may be FIGURE 46 Exophthalmic goitre. (Eisendrath.) exposed. The lids are drawn back, the white sclerotic coat is widely visible and the patient exhibits a highly exaggerated surprise-look or stare. The movements of the eyeball are limited, the lids cannot close and the conjunctiva is slightly irritated. The eyeball is actually in- creased in size somewhat and may be painful as well as prominent Vision is not generally disturbed, though myopia has occurred, prob- ably on account of the increase in the size of the globe. The fundus appears normal, though the retinal arteries may pulsate abnormally. Secondary effects, like ulceration, sloughing and opacity of the cornea. THE NEURONIC DISEASES 327 have followed the insufficient protection of the eye. (Edema of the lids and even optic atrophy have been observed. A remarkable symptom, which, however, is only present when there is exophthalmos, is that known as Von Graefe's. If the patient holds her head still and is asked to follow with her gaze the finger of the examiner, carried slowly downward about twelve or fourteen FIGURE 47. Exophthalmic goitre. (Colburn.) inches in front of her eyes, it will be noticed that as the ball of the eye rolls downward the upper lid fails to follow it as it docs in health. This is not due merely to the physical obstruction, for the symptom does not occur when tru are protruded from any other cause. The upper lid is in a state n\ spasmodic elevation from implication of the fibers of the muscle of Miillcr. The presence of this symptom is 328 THE NEURONIC DISEASES therefore dependent in no way upon the exophthalmos and consequent- ly may be observed when the latter is very slight. It is usually bilat- eral and may vary in degree of severity. Winking does not take place as often as it does in normal indi- viduals (Stelhvag's symptom), and there is some retraction of the lids so that even in the mildest cases of exophthalmos the palpebral fissure is wider than would be expected. The internal rectus of one eye may be weakened, as shown by asking the patient to look at the tip of his nose. External ophthal- moplegia has been observed, though as a rule there is no paralysis of the ocular muscles in this disease. There may be a vibratory tremor of the eyeball, as is sometimes seen in other parts of the body. Lacry- mation may be a symptom. So constant is tremor that some authors add it to the three cardinal symptoms of the disease. I have not found it so frequently as this, though I have seen it many times. Basedow first described it. The ex- tremities and the head oscillate with a fine, regular movement, eight to ten oscillations to the second. As the symptom usually occurs early in trie disease, and does not always continue throughout its course, it is important always in the diagnosis. It is suggestive of the tremor of paralysis agitans. Mental excitement and exertion increase it, but it is never absent even during rest. Choreiform jactitations have been occasionally noticed in conjunction with this tremor. In addition to the four important symptoms of exophthalmic goitre just described there are many others, less constant, but often of much assistance in arriving at a diagnosis. Among these hyperidrosis has seemed to me to be ratlfer frequent. Nearly all of the patients complain of sweating more than they have been accustomed to doing, especially under the arms. Sometimes it is unilateral and even local, as in the palms of the hands, about the head, etc. On account of this dampness of the skin the local resistance to the electric current is markedly diminished. A psychosis of some sort is nearly always noticeable in exoph- thalmic goitre. It may amount only to a slight degree of irritability, anxiety, restlessness and melancholy — a mild phrenasthenia. The memory, the power of attention and the will may be somewhat weak- ened. Sleep may be disturbed so as to be either very light or very profound. Unpleasant dreams are not uncommon. In rare cases actual delirium, mania, melancholia, with distinct hallucinations and illusions, may break out. An effort must always be carefully made to determine whether the psychosis is primary or secondary. Exoph- thalmic goitre occasionally occurs among the insane. The psychosis that is a symptom of Graves' disease usually assumes an atypical form and its course is variable. Acute mania may appear and terminate in death in a few days. The general health of these patients is usually deteriorated. They are anaemic, weak, emotional and emaciated. In spite of a good appe- tite and the ingestion of abundance of food, they even go on to a condition of complete marasmus. The bowels become loose, attacks of vomiting take place, the temperature rises a little and there are hot THE NEURONIC DISEASES 329 flashes that sweep over the entire body. Muscular weakness, vertigo, headache, various paresthesias, slight alteration of the reflexes, spinal tenderness, want of energy and staying qualities all indicate a pro- nounced condition of neurasthenia. A true motor paresis does not occur, though persistent paraparesis in the legs, and even a monoplegic and hemiplegic condition have been recorded. Among the trophic manifestations a muscular atrophy is exceed- ingly rare. The skin, however, may be pigmented somewhat, as it is in Addison's disease. The hair occasionally turns gray and falls out. Leucoderma, urticaria, local oedema, myxedematous swellings and pruritus are some of the occasional and distressing associated phe- nomena. Polyuria is frequent enough to be regarded as an important symp- tom. In rare instances it may be associated with albuminuria or glycosuria. As in hysteria, epilepsy and other neuroses, the micturition may be excessive at times. With the polyuria and hyperidrosis, a cer- tain degree of polydipsia is apt to be present. Amenorrhcea has not been so rare in my observations as the authorities state. Delayed menstruation has been associated with the disease in some of the young girls that I have seen. The relationship between the two I am unpre- pared to explain. It has been thought that there is some connection between the sexual apparatus and the thyroid gland. The latter is said to undergo changes at puberty and often to vary in size with each menstrual period. The role played by sex in exophthalmic goitre is due, in my opinion, not so much to the sexual organs as to the differ- ences in the nervous constitution. The respiratory function exhibits some anomalies in Graves' dis- ease. Paroxysmal cough and dyspnoea are not to be made light of. Bryson has shown that there is a lessened expansion of the chest in inspiration, though Patrick has found this to run pari passu with the weakness of the muscular apparatus. It is not therefore a characteristic symptom in itself. Complications. — The complications of exophthalmic goitre are numerous and must not be confused with the symptoms of the disease. Among the nervous troubles with which it is sometimes associated are the psychoses already referred to, hysteria, epilepsy, tabes, tetany, dia- betes, rheumatoid arthritis, Raynaud's disease and migraine. The osseous system has been affected, as in osteomalacia, and has combined with or passed into myxedema. Optic neuritis and optic atrophy are complications rarely seen. Enlargement of th< lymphatic glands, vertigo and bronzing of the skin have- been noted by several observers. Valvular defects of the heart are complicating rather than symptomatic Organic lesions of all sorts, both in and out of the nervous system. except that of the thyroid gland, are so exceedingly rare that the) may all be put down as complications or mere secondary results. Diagnosis. — A typical ease of Graves' disease is easily recognized. Typical cases, however, arc not often seen and. moreover, the physician is usually called upon for a diagnosis early in the disease when tin- symptoms are few and not well marked. It is most desirable t«» make the diagnosis early, as then the disease is most amenable to treatment. 330 THE NEURONIC DISEASES The tachycardia may be the only symptom present when the pa- tient first comes under observation. If it has come on rather suddenly, after a severe strain or shock in a neuropathic individual, especially a woman with hysteroid tendencies and a neurotic ancestry, and espe- cially if it cannot be accounted for on any other organic or functional basis, a diagnosis of incipient exophthalmic goitre may be made with reasonable justness. The changeability, excited character and appar- ently causeless origin of the tachycardia are strongly suggestive of Graves' disease. It is less paroxysmal in its exacerbations in the latter than in any other neurosis. If neurasthenic symptoms of a marked character occur with a tachycardia that does not change after the patient has maintained quiet for some time, a strong suspicion of exophthalmic goitre should be formed. If any of the minor symptoms, such as a mild psychosis, local hyperidrosis, vertigo, vasomotor dis- turbances, trembling and muscular weakness, reveal themselves with this form of tachycardia, the diagnosis is all the more strengthened. A careful examination for, or at least an expectant anticipation of, enlargement of the thyroid and prominence of the eyeballs may re- ward the examiner with grounds for making an absolutely positive diagnosis. If only struma is present with the tachycardia it is to be remem- bered that ordinary goitre sometimes provokes cardiac overaction by pressure upon the sympathetic and vagus nerves. Ordinary goitre, however, occurs mostly endemically, has not a neurotic heredity be- hind it, is not associated with marked neurotic symptoms, appears gradually, grows larger and maintains its size more uniformly than does the goitre of Graves' disease. In ordinary goitre the exophthalmos is more apt to be unilateral, and the direct irritation of the sympathetic is shown in the marked unilateral mydriasis. It must be admitted that the differential diagnosis between exoph- thalmic goitre and ordinary goitre with pressure symptoms is some- times extremely difficult. In the exophthalmic goitre I have made the differentiation by placing the fingers well to the side, and behind, the tumor and experiencing a lateral pulsation or thrill. If the tumor is an ordinary bronchocele, throbbing from mere promixity to the great underlying blood vessels, there will be no lateral thrill, but the whole mass will pulsate forward. Acute iodism (Oppenheim) and cocaine intoxication (Durdufi) have produced a clinical picture resembling that of exophthalmic goitre. Some of the complications may lead to confusion in the diagnosis, but if the cardinal symptoms of Graves' neurosis be sought for, and if it be remembered that most of these complications have other symp- toms that belong only to them and not to the exophthalmic symptom- complex, the differential diagnosis will not be a matter of such extreme difficulty. Prognosis. — In its ordinary form the disease is essentially a chronic one. It lasts for years, with remissions and exacerbations. Though complete recovery takes place in only about one-quarter of the cases, and these the milder ones, death, on the other hand, need not THE NEURONIC DISEASES 33 1 be feared, except in the more rapid and severe cases. Intermissions oc- cur in some cases, so that the patient suffers from a series of attacks. The light cardiac cases are the most hopeful. These sometimes return to health in three or four weeks. They may become chronic, develop other symptoms and last for years. Gowers finds that the prognosis in these cases is a trifle better in women than in men and where the disease seems to run through the family. With the appearance of struma and exophthalmos the prognosis becomes more grave. Very serious is it if the apparent cause is irremediable, if there is marked emaciation, if the thyroid is unusually enlarged and if the muscular weakness is extreme. Organic heart disease renders the outlook unfavorable. Xo case, however severe and prostrated it may be, is absolutely beyond all hope of restoration to health. Some very remarkable and unlooked-for recoveries have taken piace. In those that do recover the heart disturbance is apt to linger or be the last symptom to go. Death is dependent usually upon the cardiac weakness. Gowers .c- marks that the average prognosis is worse among hospital patients than in those of better position in private practice. My own observa- tion confirms this. Treatment. — The treatment of exophthalmic goitre is still un- satisfactory, whatever the means employed. Every line of treatment has been followed by some cures ; no line of treatment has been tried without a certain percentage of failures. In view of this fact, one is inclined at times to believe in the self-limitation of some of the cases, especially when we recall how remittent and even intermittent the disease often is in its course. Credit must not be too hastily gJvcn, therefore, to any particular mode of treatment that happens to be fol- lowed by improvement. The treatment of this disease may be profitably discussed under the five heads, specific, hygienic, medicinal, electrical and surgical. There is no known specific remedy that will cut the disease short. Recently, however, some favorable results from the exclusive adminis- tration of milk drawn from thyroidectomized goats have been reported by Ballet. Enriques, Mobius, Lanz and others. The theory is that animals from which the thyroid gland has been removed develop in their bodies a substance that would fix or neutralize the poisons of Graves' disease. Goebel argues that in Graves' disease, depending upon a hypersecretion of thyroidin, iodin is advantageously excluded from the dietary and this Is accomplished by the giving of milk only from an animal deprived of its power of secreting any iodin through the removal of its thyroid gland. The duration of this treatment varies from several weeks t<> six months, and has been followed by very encouraging results. The serum from thyroidectomized goats and sheep has been used by Mobius, Burghardt and Blumenthal with some degree of success. It is administered per <>s and subcutancously. By the latter method I ccm. has been injected daily, while at the same time rodagen, a powder prepared from the milk of thyroidectomized sheep, was given three times a day in dos< s of 3 to 6 grams. I !ases with organic cardiac 3$2 THE NEURONIC DISEASES affections generally got worse and in some instances the serum pro- duced erythema, rheumatoid troubles and general depression. All forms of serum treatment have as yet been tried in too small a number of cases to be positively recommended or condemned. There are no prophylactic measures to be noted, as the disease usually makes its appearance more or less abruptly. General hygienic measures are of immense value when the trouble has once announced itself. First among these is rest and absolute quietude of mind and body. Removal of all sources of worry, a change of environment and a certain amount of rest daily in bed will cause an amelioration in the symptoms of nearly every case, and their disappearance completely in not a few of them. A sojourn, therefore, in the country where plenty of fresh air and good food, with quiet, can always be obtained, is judicious. The food should be plain, non-stimulating and largely vegetable. Alcohol, tea and coffee should be prohibited absolutely and meats largely restricted. Most patients do better at the seashore than in the mountains, though some have improved in high, dry altitudes. Sea-bathing and mountain-climbing are decidedly dangerous, as is also a sea-voyage, unless the patient is proof against mal dc mer. Gentle outdoor exer- cise and moderation in bathing are to be recommended. Sexual ex- citement, smoking and all forms of dissipation and unwonted mental and physical stimulation must be interdicted. The patient must adopt a most regular mode of life in regard to his sleeping, rising, eating, etc. Some cases do better when they keep their bed absolutely for a few weeks, though in the majority of cases this is not required. As advanced cases, in which operative measures have been sug- gested as a dernier ressort, have recovered when the above line of treatment has been rigidly and persistently carried out, too much stress cannot be laid upon the hygienic treatment of the disease. In addition to the sedative and tonic effect of these hygienic meas- ures, the effect of the physician's personality, the hopefulness of mind that can be inspired by the companions of the patient, and the remarka- ble influence of psychic suggestibility are important and rather favor the neurotic theory of the disease. It is a curious fact that Mobius, who first propounded the glandular theory of the disease, has reported some good results from the use of hypnotism. Oppenheim, who questions the thyroidation theory and favors the neurotic, has never seen hypno- tism do a particle of good. I am convinced, nevertheless, that a certain amount of psychic suggestibility plays a useful part in the management of the disease, and I am not sure that some of the vaunted cures from surgical as well as from other measures may not be due to profound mental impression. Tlie medicinal agents that have been employed to combat the dis- ease are many. Most of them are used to combat the symptoms. Suc- cesses and failures have followed all of them. None oi them can be recommended with certainty, though all of them should be given a trial before the ease is declared hopeless or subjected to the risk of a severe operation. Like all experimenters, T have my own favorite THE NEURONIC DISEASES 333 remedy, though at times I have found it to fail me. I have seen codeia, in gradually increasing dosage, continued for a considerable time, produce prompt and happy amelioration of both subjective and ob- jective symptoms. I give th ; s alkaloid a trial in every case before re- sorting to other measures. Sometimes I have had better results from the administration of asafcetida in full doses at the same time. The heart action has quieted down, the struma has seemed to lessen in size, and above all, the patient's general nervous condition has disappeared. The bromides have been less effective in my hands, though beneficial in some cases. I have seen no results from belladonna or its alkaloid, atropia, such as others have reported. I have given it until the physio- logical effects were decided. The administration- of iodine and the iodides is full of. uncertainties. The symptoms are less liable to be increased under the use of hydriodic acid. In a few instances good has followed all of them and they are open to trial if watched and given cautiously. Painting the skin over the gland with iodine, or rubbing into it daily the ointment of the red iodide of mercury, is urged by some authorities. Iron, quinine, arsenic and the mineral acids serve as useful general tonics. Oppenheim urges the avoidance of opium for the diarrhoea and says that he has had better success with Colombo. For their symptomatic effect tincture of aconite, aconitia, strophanthus, digitalis, cactus grandiflorus, veratrum viride and picrate of ammonium have received various indorsement. Phosphate of sodium daily in doses of two to ten grains has been praised by Kocher and others. The very multiplicity and diversity of these remedies suggest their own criticism. They are probable useful in combating certain condi- tions in individual cases, but as distinct therapeutic agents for the cure of exophthalmic goitre they are practically worthless. They arc merely adjuvants in fostering the nervous quietude and bodily nutri- tion upon which the real cure of the disease depends. Before leaving the discussion of medicinal agents, a word should be said in regard to the administration of thyroid extract, so much lauded a few years ago and now so extensively condemned. It is true that the symptoms of the disease are usually increased by it. and for that reason it must be given, if given at all. with great caution. It is curious logic to base the thyroidation origin of the disease upon this fact, as many do; for as the administration of thyroid extract in all neuropathic individuals will provoke more or less nervous dis- turbance (a quarter of a grain produced a disturbing degree <>t' excite- ment in a case of myxoedema, and in another of sporadic cretinism under my observation), the increase of symptoms produced by its administration in Graves' disease merely indicates the neurotic origin of them in this affection. T have seen ;i case in a young man w I mother had the disease in an advanved stage, and whose brother showed it in its incipience, completely cured and without any dish ing phenomena, during its intermittent administration in five-grain doses during a period <■(' almost eighteen months. I do not think. therefore, that it is open to absolute condemnation, though I would urge caution, and some hesitancy, in its administration. 334 THE NEURONIC DISEASES Electrical treatment should always be given a trial, as some very- favorable results have followed it. Galvanization of the cervical sym- pathetic is the method of choice. Two or three times a day, in ten- minute seances, and in doses of three or four milliamperes, the stable current should be transmitted along the anterior edge of the sterno- mastoid muscle. It is a question whether the effect is upon the sym- pathetic or the vagus nerve. The same mode of application in health does not cause any pupillary changes, which seems to indicate that the sympathetic is not very much involved. Strong currents must not be used, and it is better to err on the side of too small and too brief an application than vice versa. The patient should lie down during the treatment. Dana recommends placing the positive pole beneath the ear and the negative over the thyroid gland for two minutes, then over the cardiac region one minute, positive pole over the eyes, and finally the negative over the thyroid one minute. Vigouroux and Charcot long ago recommended the direct galvanization of the cardiac region. The static breeze in the same area is said to have done good. General faradism and central galvanization are useful for their general tonic effect. Electrical treatment often fails, and I am prone to think that some of the good accomplished by it is due to psychic suggestion, ex- pectancy, hopefulness and the systematization of the patient's general regimen inculcated by it. It therefore has its use. One enters a battlefield in discussing the surgical treatment of exophthalmic goitre. Here, as elsewhere, good reports are forthcom- ing. All of the operations are dangerous, and the danger increases with the stage of the disease and the inexperience of the operator. Whereas so great an operator as Kocher may report a hopeful per- centage of cures in carefully selected cases, physicians will be loath to regard exophthalmic goitre as purely a surgical affection until no cases are cured by other measures, and until the statistics and tech- nique of the average surgeon make a better showing. This is not the place to discuss the relative merits of medical and surgical treatment. Many analyses will have to be scrutinized and all the bearings taken fully into account before the question receives any- thing like a definite answer. Five operative procedures are to-day before the medical world for consideration. In the order of their popularity and usefulness they may be enumerated about as follows: Sympathectomy, or exsection of the cervical sympathetic nerve ; thyroidectomy, with a mortalitv ranging about fifteen per cent ; ligation of the thyroid arteries ; Jaboulay's exothyropexie (exposure of the gland to the air) ; and operations upon distant organs, as, for example, the uterus. By operative procedures good results are claimed in from sixty to eighty-five per cent, of cases. The mortality is so varied in the hands of different operators that at present nothing definite can be affirmed. MYXGEDEMA VXD CRETINISM. ATTTYREA. Myxcedema, cretinism and cachexia strumipriva are three condi- tions dependent upon disease of the thyroid gland and characterized THE NEURONIC DISEASES 335 by a peculiar condition of the skin and mucous membranes, by weak- ness and certain nervous manifestations, and by a psychosis of a more or less uniform and typical sort. Absence of the thyroid gland is the basis of the trouble, as shown both by post-mortem examination and by the brilliant results obtained from the administration of thyroids. The three diseases may therefore be well considered together, and on account of the prominence of their nervous symptoms be included in a work on nervous diseases. It was Sir William Gull, and later on Ord, who in the seventies first described a cretinoid condition in which mental failure and a myxedematous condition of the subcutaneous tissues were associated with atrophy of the thyroid gland. There were no pathological find- ings of any moment, though the pathogenesis of the disease was attributed to the loss of the thyroid gland. The suspicion was trans- formed into a certainty when a similar condition was produced by extirpation of the gland by several investigators. Horsley established the condition by a complete removal of the gland in monkeys, and Kocher and Reverdin showed that it followed complete extirpation in the human. This operative myxcedema, or cachexia strumipriva, is characterized by a tired feeling, pains and heaviness in the limbs, tetanoid contractures, sensations of cold, temporary swelling of the face, hands and feet, mental apathy and coma. A chronic state gen- erally follows this in which the mentality becomes sluggish, the skin cold, dry and scaly, and the strength decidedly diminished. The hair may fall out, the bones cease to grow and the patient die if not treated. This cachexia appears more promptly in the young than in the old and is believed to occur only when the gland is entirely and com- pletely removed. The identity of these symptoms with those of myxcedema in the adult and % cretinism in the child has confirmed pathologists in the view that the same pathogenesis is applicable to all these conditions. MYXCEDEMA. This is sometimes called Gull's disease. Charcot named it cachcxie pachydermique. It has been referred to as cretinism in the adult. The disease comes on insidiously and progresses slowly. Women are more affected than men in the proportion of six to one. It occurs mostly between the ages of thirty and fifty. It has been seen in in- fants, but not earlier than at six months of age. There is a slight hereditary influence on the maternal side, and several eases have ap- peared at times in the same family. Other neuroses have preceded it and exophthalmic goitre has been replaced by it. Alcoholism and syphilis do not predispose to it. Though it occurs so largely in women, the sexual accidents of women do not seem to have any bearing. Various exciting causes have he en assigned, such as exposure' to cold, emotional excitement, traumata, etc. Symptoms. — Trophic changes constitute the main symptoms in the clinical picture. There is a slow and gradual increase in the general bulk of the body. The head and extremities enlarge, assume a new contour and take on a new shape. The countenance especially 336 THE NEURONIC DISEASES appears swollen and puffy. The lines of expression are obliterated, the lips thickened, the nostrils broadened^ the mouth enlarged and the entire features rendered coarse and brutal. Apathy and stupidity are suggested by the physiognomy. The tongue is thickened and hard- ened. Local swellings occur in the neck and on the extremities. The tumefaction, especially in the supra-clavicular region, includes the skin and subcutaneous tissues. The hands and feet are stubby, the fingers and toes broad and separated. The skin is dry, hard and scaly, is rough and does not pit upon pressure as in ordinary oedema. It feels cold and dead, looks pale, seems elastic to the touch and does not perspire. The mucous membrane of the mouth is also infiltrated. Imperfect nutrition is shown in the falling out of the hair, nails and teeth. Muscular weakness and mental deterioration added to the physical deformity render these individuals awkward, ungainly and clumsy in all their movements. The psychic condition is the second important characteristic of this disease. There is a gradual impairment of the mental faculties. The thoughts and perceptions are slow, the speech is delayed and the general intelligence greatly lowered. At first the memory is weakened. The disposition becomes irritable and suspicious. Hallucinations and delusions that are inconstant and systematized, and suicidal impulses may all be observed. Sometimes there is merely inability to fix the attention and lack of mental energy. At other times there is a total lack of interest and a settled state of stupor. Finally a condition of complete dementia obtains, in which there seems to be an obstruction in all the avenues of both afferent and efferent impulses. The five senses are all more or less impaired. At times there is great depression and despair, which, however, is not identical with true melancholia. Mania, melancholia and hallucinatory paranoia have been observed. This group of mental symptoms differs in its course from those of the ordinary psychoses and is as typical in its general characteristics of myxcedema as are the physical signs. Headache, vertigo and coldness are often complained of by these patients. The bodily temperature is not unfrequently found to be sub- normal to the extent of one or two degrees Fahrenheit. The muscles are weak and sometimes atrophied. Incoordination and contractures are observed in rare instances. The electrical resistance of the skin is increased and it is often hypaesthetic or even anaesthetic. The voice is unnatural, hoarse and monotonous. The reflexes, the electrical re- action of the nerves and muscles, the functions of the abdominal and thoracic viscera are all normal. Hemorrhages are not infrequent. Albuminuria is observed in about twenty per cent, of the cases; gly- cosuria rarely. Synovitis of the knee-joint, optic neuritis and optic atrophy are among the rarest symptoms that have been noted. The physical deformity, the peculiar infiltration of the skin and subcutaneous tissues, the tncnlalization, the hoarse voice, the weakness and awkwardness are so characteristic a group of symptoms that the diagnosis of myxedema is never a very difficult matter. Were any doubt present, the therapeutic test with thyroid extract would soon remove it. THE NEURONIC DISEASES 337 The course of the disease is chronic and progressive, often ex- tending over five, ten and fifteen years. The prognosis without treatment is unfavorable ; with treatment it is extremely bright, so far as complete or partial restoration to a normal condition is concerned. The treatment, however, has to be ■continuous, as its cessation is usually followed by a prompt relapse. When death occurs it is usually the result of some cardiac trouble or .some intercurrent malady, such as tuberculosis. In view of the accepted pathogenesis of myxcedema, it is hard to explain those cases in which exophthalmic goitre is associated with it, as well as those in which an enlargement of the thyroid in young per- sons gives rise to an acute and temporary manifestation of it. I have seen it follow exophthalmic goitre, but this is not so difficult to ac- count for. Probably in all these conditions there is an enlargement of the thyroid generally with complete atrophy of its parenchymatous, secret- ing structures. CRETIXISM. This is a form of myxcedema occurring endemically and sporadi- cally, appearing congenitally or during infancy, having the same gen- eral symptoms as the adult form, and depending upon disease or ab- sence of the thyroid gland. Endemic cretinism occurs chiefly in Switzerland, Italy and France and is associated with goitre. It is "believed to be due to the loss of function in the thyroid gland. Sporadic cretinism is rare, especially in this country, and is in all respects identical with infantile myxcedema. Cases of so-called infantilism are probably mild grades of the same condition. Rarely before the age of six or seven months is the disease rec- ognized. Then the same general trophic and mental deterioration is noted. The growth and mentality of the child are seen to be retarded. The face assumes a bloated, coarse, stupid appearance. The tongue is large, the lips protuberant, the mouth increased in size, the eyelids thick and drooping and the facial lines obliterated. The body is dwarfed and thick, the abdomen pendulous, the extremities short, club- shaped and bowed. The hands and feet resemble paws. Hair is scarce, the teeth are undeveloped or decayed, the nails are deformed or absent. The skin is pale, sallow and waxy ; it is dry and leathery to the touch. Fatty tumors abound in and about the neck. The disproportion be- tween the various parts of the body is very remarkable. The mind fails of development and a condition of idiocy thus obtains. The muscula- ture is weak so that the child has to be supported when walking. Usu- ally walking, like speaking, is quite impossible. The fontanelles com- monly remain unclosed. There are mild degrees of the trouble in which the physical symp- toms are only slightly indicative of delayed development and the mental state is that of medium grade imbecility. Such a ease I have seen restored almost to a normal condition by the administration of thyroid extract. The thyroid gland is supposed to be affected by a transient functional disturbance. 338 THE NEURONIC DISEASES •A V o u ^ >- " * THE NEURONIC DISEASES 339 Death occurs early in most cretins, though some have lived to middle adult life. If treatment is instituted early and before puberty, or the age at which puberty should occur, most brilliant results in some cases may be obtained. Treatment. — There is only one remedy that is effective in all these forms of athyrea or alhyreosis, and that is the thyroid gland or thyroid extract. We are originally indebted to Murray for this remark- ably brilliant therapy, though much has been done in the way of es- tablishing it by Schiff, Bercher, Horsley, MacKenzie, Horwitz and others. Beginning with one grain three times a day, the dose may be continuously increased until fifteen or twenty grains three times a day are taken. Forty grains or more is about the maximum average daily dose. Unpleasant symptoms that are to be guarded against are those of general thyroidism — namely, paresthesia, restlessness, tachycardia, dyspnoea, anorexia, emaciation, delirium, etc. These occur, however, in only a small number of cases. On the other hand, the results of thyroid treatment are so positive and happy that nothing in the way of therapeutical success can compare with it. Relapses occur if the remedy is discontinued ; hence, as Murray himself suggested, full doses should be given at first to effect the cure and moderate doses should be continuously administered thereafter to maintain the normal or acquired metabolism. Thyroidin, thyroiodin, idiothyrin have all been prepared from the thyroid glands of sheep and tried in place of the thyroid extract. While some advantages are claimed for them, especially the iodothyrin, none of them equal in efficiency the whole gland or its extract. ACROMEGALY. As Marie first described this disease in 1886, it is sometimes named after him. It has also been called Pachyakria. As its more common name indicates, it is chiefly characterized by an enlargement of the ex- tremities and face. Etiology. — Heredity seems to exercise no influence in its etiology. All ages are liable to it, though the great majority of cases occur between twenty and forty. Most authors declare that it attacks both sexes about equally, though I am inclined to think that women are a little more prone to it than men. Race and nationality exert no deter- mining force. It has followed syphilis, rheumatism, the infectious fevers and alcoholism, though it is doubtful if these are in any way causative. Such recorded exciting causes as exposure to cold, fright, worry, emotional shocks and traumata are too indefinite to be given much consideration. Pathology and Pathogenesis. — The mosl constant post-mortem finding is enlargement of the pituitary gland (cerebral hypophysis). In practically every case this is found to be altered. The other findings in the disease are comparatively so inconstant the opinion has become quite universal that the primary seat of the trouble resides in this gland. 340 THE NEURONIC DISEASES Those who question this hypothesis base their opposition upon the fact that often the hypophysis is diseased without the appearance of the acromegalic symptoms.. Various neoplastic growths, such as sarcoma, glioma, adenoma, have been found as the reason of the pituitary en- largement, especially of the anterior part of the gland. The function of the gland is disturbed and a general nutritive disorder is thus estab- lished. The exact function is not positively known, though embryol- ogy,, evolution and pathology all seem to indicate that it bears a close resemblance to that of the thymus and thyroid glands. Many symptoms of acromegaly are comparable to those of myxoedema, especially in their tropho-neurotic characteristics. Evolu- tion, embryology and histology all teach that before the closure of the post-pharyngeal vault there was a direct connection between the mouth and ventricles of the brain by way of the infundibulum. The anterior part of the pituitary gland, having been caught in the intracranial cavity when the latter finally separated from the buccal cavity, its structures retained their thyroid-like metabolic functions, the whole gland became ductless and entered upon the role of an organ of internal secretion, and so in some way assisted in regulating the general nutri- tion of the body. In many cases of acromegaly, besides disease of the hypophysis, the thyroids are found to be atrophied and the thymus gland persistent and hyperplastic. The trophic changes found in other parts of the body include thick- ening and deformity of the bones, especially the lower jaw and distal bones of the extremities; arthritic conditions in and about the joints; hypertrophy of the skin, connective tissues, arteries and nerve-sheaths, spinal ganglia and sympathetic ; increase in the size of the brain, eye- balls and abdominal organs ; and asymmetrical degeneration of the posterior columns of the cord. From the pathological findings the dis- ease is well classified as a tropho-neurosis, whatever may be the seat of the primary trouble. Symptoms. — The disease approaches insidiously so that often the enlargement is first noticed by the patient's tailor or bootmaker. There may be at the commencement various paresthesia?, slight drawing pains, general debility and peculiar visual phenomena. In women the menstruation sometimes ceases. A persistent headache, vertigo, gradual failure of vision and other signs suggestive of intracranial tumor may awaken suspicion. Ere long, however, the characteristic symptom of the disease makes its appearance. This consists of a uniform, progressive enlargement of the hands and feet and certain parts of the head and spinal column. In the hands and feet the soft tissues, as well as the bones, are increased in size. The fingers and toes are not deformed, though they are big, uncouth and easily mov- able. They look like links of sausage. The skin is thick, but, unlike that of myxoedema, it is soft, moist and pliable. This enlargement rarely extends above the wrist or ankle. The same excessive development attacks the lower jaw so that a condition of marked prognathism is presented. The maxilla may pro- ject so far forward as to almost seem to encompass the upper jaw. THE NEURONIC DISEASES 341 The nose, lips and ears are enlarged. The whole face, especially the lower part of it, is both lengthened and widened. The cheek-bones are prominent. The eyelids are thickened and in some cases the tongue is excessive in size. While the long bones of the arms and legs, the metacarpal and FIGURE 51. Acromegaly. (After Marie.) metatarsal bones usually escape, the clavicles, sternum, ribs, patella and vertebral column are implicated. Kyphosis of the upper dorsal region is a very frequent symptom. The en si form process of the sternum is particularly prominent and the manubrium is so thickened that a per- cussion note sounds dull (Erb). The abdomen is enlarged. The skin under all these circumstances may continue i<> appear perfectly normal. As a patient of mine once expressed it. except for Ins failing eyesight and general change of shape, he couldn't sec that he was in any way 34-2 THE NEURONIC DISEASES abnormal. In rare cases the heart is enlarged ; the penis increased in size ; the testicles atrophied. Eye symptoms are not infrequent and on account of their early appearance are often of extreme importance. Progressive loss of vision from optic atrophy is the more common trouble. All signs of irrita- tion, compression and destruction of the optic nerves and chiasma may be expected. Optic neuritis is much rarer than optic atrophy. Hemi- anopsia of various types, hemiachromatopsia, unilateral blindness with hemianopsia in the other eye, are some of the more common visual disturbances. Fig. 52 portrays the visual fields of a patient of mine before he went totally blind. For a time he had horizontal hemianopsia, so that everything below the level of his eyes was invisible. This lasted about six months and gradually passed into the bitemporal type shown in the chart. The ocular muscles are sometimes paralyzed with transient or permanent attacks of diplopia. Flemianopic pupillary rigidity may be present. Exophthalmos has been observed, but is exceedingly rare. Pressure by the enlarging hypophysis explains easily these various transient and permanent ocular manifestations. The mental state of these patients is always somewhat affected, though not so characteristically as it is in myxcedema. They are apathetic, sluggish and devoid of energy. A sort of a drowsy air and heaviness belongs to them. Their movements are slow and awkward. There is a weakness of their muscles, though their electrical reactions are unaltered. The condition of the reflexes varies, but is of no special import. There are many other symptoms that may appear, but are not constant enough to be considered typical of the disease. Some of them are merely indicative of an intracranial new growth ; others are partly due to the disease itself and partly due to associated derange- ments. The thyroid may be enlarged, more commonly it is diminished in size. The skin may be thickened, but it is never myxcedemafous. The muscles are sometimes wasted. The larynx may be altered in shape and the voice rendered deep and rough. Its mucous membrane, like that of the mouth, is thickened. All of the five senses may be blunted. With the enlargement of the heart the pulse rate may be accelerated. Impotence is usually present. Sweats sometimes occur, along with polyuria and polydipsia. Bulimia or polyphagia is rather frequent. Pigmentation, moles, warts, keloids, fibromata and neu- romata are often observed. Dyspnoea, palpitation, vasomotor neurosis, general weakness and mental depression are not to be unexpected. Epileptic attacks have been recorded in rare instances. Diagnosis. — The diagnosis depends upon the onset of the disease, the peculiar enlargement of the lower part of the face and extremities. the kyphosis, the ocular symptoms and the mentality. Myxcedema and cretinism are to he differentiated by the limitation of the enlargement to the soft tissues, the infiltration of the subcutane- ous structures of the entire face, hands and feet, the cold, harsh, (In- state of the skin, the alopecia and the dementia. The hones are not THE NEURONIC DISEASES 343 hypertrophied as they are in acromegaly, ncr are the kyphosis and prognathism present. Gigantism is closely related to acromegaly, if it is not identified with it. Many giants become acromegalics. Massalongo. Klebs, Meige and others believe that the primary disease in both conditions resides in the hypophysis cerebri. Partial gigantism or hypertrophy of special parts of the body (hemihypertrophy, macropodia. macrocheiria, etc.) is a congenital trouble and is distinguished from acromegaly by its limitation to a particular part of the body and the great deformity of that part. Syringomyelia, adoposis dolorosa, elephantiasis, leontiasis ossea. diffuse hyperostosis, osteitis deformans have all their own characteristic FIGURE 52. Bitemporal hemianopsia in a case of acromegaly in which complete blind- ness ultimately occurred. Implication of the optic chiasm is clearly shown in the perimetric fields taken by Dodd. An autopsy being denied, I assumed from the history and other features of ;hc case that the hypophysis was the seat of a sarcoma. symptoms and can be distinguished from acromegaly by a close study of the total clinical picture of the latter. In osteitis deformans. Paget's disease, the shafts of the long bones and the bones of the cranium, not those of the face, are enlarge. 1 and softened. The long bones are curved from the weight upon them. Pains are present in them almost constantly. There seems to be a close relationship between this affection and malignant tumors. Noth- ing is known of its etiology and its treatment is without results. Leontiasis ossea is characterized by a hyperostosis of the cranial bones, sometimes also of the face. The head, face and neck, with both the hard and soft tissues, increase slowly and | vely. Stan- has named the condition megalocephaly. M acromegaly is curious as the antithesis of acromegaly. It was described bv Hutchinson and Gilford in [896. There is a defect 01 344 THE NEURONIC DISEASES FIGURE 53. Pulmonary osteoarthropathy of Mario. (From a photograph in the Pathological Museum of the Mod. Dcpt. of the University of Illinois.) THE NEURONIC DISEASES 345 nutrition, due to some form of nervous trouble, which is seen In the smallness and immaturity in some parts and consequently a relative largeness and apparent prematurity in others. Marie's hypertrophic osteo -arthropathy, which occurs in associa- tion with cardiac and pulmonary disorders, deserves special attention in the differential diagnosis of acromegaly. In this disease the bones are more involved even than they are in acromegaly. Arnold refers to the disease as a secondary hypertrophic osteitis. In this disease prognathism is not present and kyphosis, if at all present, is lower down in the spinal column. The end phalanges are peculiarly deformed, the wrist and ankle-bones are enlarged and the finger tips are club-shaped. There is no uniformity in the enlargements. Moreover, the tongue is not affected as it is in acromegaly, and the mind is clearer. Prognosis. — Sternberg notes three varieties of the disease in regard to its course. The first is benign and may last fifty yeais; the second is the usual chronic form and may continue from eight to. thirty years ; the third is the acute, malignant form that terminates in about three or four years. The disease is incurable, but at times it ceases to progress. Relapses occur. Death is usually the result of marasmus, diabetes, heart disease or some intercurrent trouble, such as cerebral tumor, bedsores, secondary disease of the genito-urinary tract. Treatment. — Treatment is unsatisfactory. Organotherapy has so far proved to be entirely ineffective. Thyroid and pituitary extracts have been administered without results. It has been reported that the iodides, mercury and arsenic have seemed to have arrested the disease — an open question always where the disease is prone to cease progressing of itself at times. The attempts of Caton and Paul to bring about a cure by extirpating the pituitary gland are not very en- couraging. Symptomatic treatment is about all that we can offer the victims of this disease at the present time. ADIPOSIS DOLOROSA. This is sometimes called Dercum's disease, because it was first de- scribed by Dercum in 1888. A considerable number of cases have been described since and even examined post-mortem. The appearance of painful, fatty tumors just beneath tlie skin in various parts of the body is its cardinal clinical manifestation. Changes in the thyroid and pituitary bodies, and a neuritic condition in the peripheral nerves, are its chief pathological findings. The disease usually appears in persons of a nervous temperament in the middle period of life. The majority of the cases have been over forty years of age. The women are often hysterical, and both men and women are highly susceptible to suggestion. A general asthenic and neuropathic condition usually characterizes them. The pathology and pathogenesis of the disease are not clear. The three prominent findings ire the fatty tumors, the neuritis and the changes in the thyroid gland. The fatty tumors occur anywhere on the body jusl beneath the skin, are variable in size and in no way differ from ail lipomata. hi 346 THE NEURONIC DISEASES the tumors the nerves seem to be increased in number and are in a condition of interstitial inflammation. The thyroid gland exhibits a state of cystic degeneration with partial atrophy and the acini filled with colloid material. Adiposis dolorosa is clearly a tropho-neurosis, but where the pri- mary source of the trouble is to be looked for we can no more deter- mine here than we can in any other tropho-neurosis. As Billings has said, it is as yet only an interesting clinical entity. Its classification must await further knowledge in regard to the pathogenesis of this disease as well as other tropho-neuroses, like acromegaly. No treatment of the trouble has been of any avail in checking the formation of the tumors. A few cases have been reduced in weight, the asthenia and mental depression removed, and the internal meta- bolism apparently placed on a somewhat better basis by the adminis- tration of thyroid extract. SCLERODERMA. This remarkable disease is believed by most authorities to-day to be a tropho-neurosis. The chief feature of it is a localized or diffuse induration of the skin. Etiology. — Females are attacked by it far more frequently than males. Kaposi afhrms that seventy-five per cent, of all cases are women. The middle period of life is the time of its appearance generally. Osier believes that it is more frequent in this country than statistics would seem to indicate. Its immediate cause is unknown. Some believe the disease is a tropho-neurosis of spinal or sympathetic nerve origin. It certainly seems to be closely related to progressive facial hemiatrophy, and it is not unfrequently associated with Raynaud's disease, erythro- melalgia, Addison's disease, rheumatic troubles and exophthalmic goitre. Others still continue to regard it as a true skin disease, due to some local affection. The old view that it was due to closure of the thoracic duct or other lymphatics and a stagnation of the lymph has not been confirmed by the more recent examinations. Pathology and Pathogenesis. — Beyond the obvious symp- tomatic changes, the pathological findings are not of special significance. The changes in the skin, the connective tissue over-growth, the disap- pearance of the subcutaneous fat, the increase of elastic fibers, have given the name to the disease. The vessels are numerous, but dimin- ished in calibre. The entire affection is in all likelihood a primary, connective- tissue hypertrophy of nervous origin or disturbance, ending in resolu- tion or atrophy. The neurotic nature of the disease is strongly sug- gested by numerous observations. Schwimmer's cases revealed disease of the peripheral nerves. Westphal saw significant changes in the brain. Eulenberg and others have reported the association of pro- gressive facial hemiatrophy and other tropho-neuroses with the disease. In some cases marked arthritic changes have been observed not unlike those seen in the Charcot joint. THE NEURONIC DISEASES 347 Symptoms. — In exceptional cases these begin with chills, fever and other signs of inflammation. More generally, however, the first symp- tom noticed is the growing stiffness and rigidity of some portion of the skin. This slowly increases during months and years. It is most noticeable and often limited to the upper extremity, though it may appear in the lower. It may occur in circumscribed patches or be diffused over a very large area. Sometimes the spots are disseminated at first and later on run together. The face and neck are not unfre- ouently involved. The trouble is usually bilateral, but is sometimes unilateral. The lips may be retracted or the gums exposed. The nose may be greatly attenuated, giving the physiognomy a very dis- agreeable appearance. The hands are tense and glossy, the fingers thinned and club-shaped and the metacarpophalangeal articulations .sometimes subluxated. As the subcutaneous fat atrophies the skin •clasps the bone and reveals sharply its outlines. The diseased skin is cold, thick, rigid and hard. It feels like the derma of a frozen corpse. It is not sharply delimited from the normal skin, though it often has a hypersemic tract around it. It is swollen in the neighborhood or shrunken. The surface of the epiderma is smooth, shining, scaly, whitish in color or pigmented in patches. In the beginning it is often merely (Edematous and hypertrophic. The mucous membrane of the mouth and vagina often exhibit similar alterations. Wrinkling, of course, ceases, and all actions implicating the »*m, like laughing, winking, breathing, become difficult and impeded. The muscles beneath are usually indurated, shriveled and atrophied. The bones are thinned. Peculiar constrictions, mutilations, even spon- taneous amputations and ulcerations have all been seen. Sclerodactyly or characteristic crippling of the fingers occurs. The sensibility is rarely involved, though it may be somewhat blunted. The tenseness is felt by the patient and sometimes pain, itch- ing or paresthesia. Paralytic symptoms occasionally appear. Hypo- chondriacal neurasthenia usually exists. There is general marasmus and the patient is depressed mentally. In some instances amyloid de- generation has been found post-mortem in the lungs, heart and kidneys. Besides the diseases mentioned under the head ^i etiology, sclero- derma has been complicated by eczema, acne, erysipelas, herpes zoster, variola, morphoea and keloid. Its diagnosis is not difficult. Prognosis. — Rarely the disease disappears by involution. The retrogression is gradual, often only in spots The trouble is always a chronic one, with remissions now and then during its long course. Most cases go on to the formation of ulcerations, mortification and other complicating conditions, suffering all the while with pains, insom- nia, bad nutrition, and finally die from marasmus. Treatment.— There is no known cure for scleroderma. Nutri- tion, rest, good general hygiene and such general tonics as iron, qui- nine, arsenic and cod-liver oil are m order in all cases. Salol internally in fifteen-grain doses three times a day has seemed to have been bene- ficial in a few instances. Improvement has occurred in many c 34^ THE NEURONIC DISEASES under thyroid medication. Galvanization of the sympathetic was urged by Schwimmer, and the local application recommended by Pif- fard, Fieber and others. Ointments and local medication are frequently grateful. Xaphthol. salicylate ointment, mercurial and iodine soaps and preparations, and copper oleate are thus available. To these should be added local baths and massage. PROGRESSIVE FACIAL HEMIATROPHY. This is a very rare affection in which one side of the face under- goes a remarkable progressive wasting, and is probably due to a dis- turbance of the trophic functions of the trigeminal nerve. The earliest accounts of it were given by Parry and Romberg. Etiology. — Little is known of its causation. It prevails more among women than among men. It is first recognized in early adult years, though it usually starts in childhood and in many cases seems to be congenital in origin. Being a chronic, progressive disease, it may run on into old age, but it is rarely ever seen to start after thirty. It has followed slight injuries to the face and head, acute rheumatism,, scarlatina, diphtheria and typhoid fever. It is not known whether the neuralgic pains in the fifth nerve that sometimes accompany it early in its course bear a causal or symptomatic relationship to it. A general inherited neuropathic taint has been detected in some cases. Pathology and Pathogenesis. — The only pathological findings that are of any significance are those which enter into the symptomatol- ogy of the disease. The pathogenesis of the disease is almost wholly conjectural. I am inclined to follow Gowers' view that it is an organic affection of the fifth nerve or its Gasserian ganglion. The marked limitation of the symptoms to the peripheral area innervated by this nerve is only ex- plainable on this basis. The trophic character of the predominant symptoms and the implication of both the sensory and trophic branches of the nerve point to the ganglion as the primary seat of the trouble. Its connection with slight injuries and local infective processes are highly suggestive of the nature of the disease process. Most authors regard the trouble as primarily a trophoneurosis. Those who try to class the disease with exophthalmic goitre and migraine are illogical. In some respects it resembles scleroderma and yet there is not that sharp trigeminal distribution of the manifestations in scleroderma that there is in this disease. Moreover scleroderma never follows trigeminal disease under any circumstances so far as we know. It is a more general trophoneurosis. Oppenheim leans toward the sympathetic hypothesis. Bitot thinks it is a primary skin disease. Mobius regards it entirely as an infec- tive process following some local injury or sore in the neighborhood. Symptoms. — The symptom of the disease is described in its name, progressive facial hemiatrophy. Gradually there is a diminution in the general bulk of one side of the face. In this all the tissues partake, the skin, the subcutaneous elements, the muscles and even the bones. THE NEURONIC DISEASES 349 The skin tightens down and clasps the bones so as to emphasize their outlines. The hairs fall of! and the sebaceous glands shrivel up. The cartilages of the nose waste causing it to be grossly distorted. The lips diminish on one side and are sunken. The perspiration may be excessive while the local temperature remains normal. The bones are decidedly smaller on the affected side. One side of the face is full and youthful, the other worn, haggard and aged looking. The strange physiognomy thus presented, a sort of double indi- vidual as it were, is so striking that the diagnosis can be made at a glance. The palate and tongue are usually unaffected. Sometimes, however, they also have been wasted on the same side. Xot in all cases are the muscles implicated, though the frequent paresis of the pteragoids, masseter, temporal, and the fibrillation and tremor in them, point to implication of the motor root. The electrical reactions are usually preserved, thus distinguishing the disease from a true progressive myopathy. Sensibility as a rule is unaffected though in some cases intense neuralgic pains occur early and anaesthesia or at least hypaesthesia later. The arteries and veins do not seem to vary in size. The saliva and tears are permitted to flow as usual. Tlie organs of special sense seem to be undisturbed so far as their functions are concerned. Bilat- eral atrophy has been seen occasionally and once atrophy of the skin and subcutaneous tissue in the back and arm of the same side was seen. The disease progresses steadily for a number of years and then remains stationary for the rest of life. Oppenheim notes the occurrences of the disease repeatedly with neuralgia, epilepsy and the psychoses, with chorea, spasm of the mus- cles of the face, jaw and tongue, once each with tabes, syringomyelia and disseminated sclerosis. The diagiwsis must be differentiated from congenital inequality between the two sides of the face, from facial atrophy in anterior poliomyelitis, and in hemiplegia of children and adults. Tt must be kept clearly apart from acquired facial hemihypertrophy, as describe 1 by Montgomery, which by contrast may lead to a diagnosis of hemia- trr phy of the other side. And it should be distinguished carefully from scleroderma anVl morphcea. If it is to be regarded as a result of trige- minal neuritis, according to Mendel's findings, then it should also be carefully differentiated from atrophy due to nuclear lesions and to sympathetic nerve paralysis The gross diagnosis is easy. The minute differential diagnosis is sometimes rendered confusing because we are ignorant of the exact pathology of the disease and its limitations. Some authorities un- doubtedly include more in progressive facial hemiatrophy than oth- ers do. Prognosis. — Favorable as to life. Bad as to recovery. The dis- ease is chronic and progressive but usually becomes stationary after :t number of vears. The pain> are sometimes wearing and tlie limita- tion of movements in the muscles of mastication is sometimes annoy- 350 THE NEURONIC DISEASES ing ; otherwise the patients are sound and well and suffer no very great discomfort. Treatment. — It is not amenable to any known form of treatment. AINHUM. This is a disease of the negro and is probably a trophoneurosis*. It was first described by Silva Lima, of Bahia, but cases have been re- ported by Hornaday and Pittmann, Duhring, Shepherd, and Morrison. It is rare in this country but has been frequently seen in South Amer- ica and somewhat in Asia. Its cause is not known. There is a singu- lar constriction o>f the skin formed about the base of one or more toes. Finally a deep furrow completely encircles the member, the bloodves- sels are obliterated and amputation takes place. The little toe seems to be the favorite victim. It is a chronic trouble and the toe does not drop off often for years. There is no sign of inflammation and usually no pain is connected with it. ORGANIC NEURONIC DISEASES. THE SYSTEM DISEASES. THE NATURE OF THE SYSTEM DISEASES AND THEIR CLASSIFICATION. In the preceding chapters the neuroses and psychoneuroses have been discussed. As commonly accepted, these are functional derange- ments of the neurones or functionating part of the nervous system. Our present means of scientific research is not such as to enable us to declare positively what the nature of the change is in the neurones or in their relationship to one another whereby the alteration of func- tion, upon which we call the condition one of disease, is brought about. And yet in regard to these neuroses and psychoneuroses, cer- tain facts stand out with a most suggestive prominence and upon these facts we can at least frame a working hypothesis. » In the first place it is to be noted that heredity, toxcemia, shock, strain, malnutrition and overexertion play the greatest role in their etiology. These causes are of a general nature and presumably act upon all parts of the nervous apparatus in the same way. Every neurone is subjected to the same deleterious influence. The nervous system is usually the victim in toto. Another fact that stands prominently forth is that no changes of any sort, whereupon the symptoms of these diseases can be explained, have ever been seen in the nervous elements. We have to assume that the primary trouble is inherent in the neurones themselves and entirely beyond our ken. It is born and has its expression there. While we cannot say what this inherent trouble is. we can very definitely declare that it involves the two primary functions of the neurones concerned. THE NEURONIC DISEASES 35 1 namely, their power of exhibiting ncurility and their power of main- taining their own nutrition. In the very nature of things the former function is dependent upon the latter. When a neurone functionates abnormally, it is mere truism to sav that its power of exhibiting sensibility is disturbed. "Whether this is always due to a disturbance of its power of maintaining its own nutri- tion, as it undoubtedly is in a large number of diseases, or whether shock, toxaemia, strain, etc., can directly cause it to act thus abnor- mally without affecting in the least its power of self -nutrition, seems to be a debatable question. Change of neurility without change of nutrition or structure would indeed be a true functional disease. It could hardly continue long, it seems to me, without some structural effect being produced upon the intimate physio-chemical constituents of the neurone. So intimately related are the neural and nutritional functions of each individual neurone, that it is all but inconceivable that one should long be changed without some change in the other. Underlying all functional diseases, I take it therefore, there is sooner or later some undiscoverable but structural change. The change may continue to be undiscoverable and the trouble continue to be spoken of as functional. There are changes in the neurones in some diseases, however, that are observable under our present staining and microscopic methods of examination. We speak of these as degenerative changes. Even- neurone in the body is liable to them and all parts of the neurone, cell and process, may be implicated. There are all degrees of degeneration from that which is scarcely detectable down to the complete atrophy and disappearance of the en- tire neurone. Every indication points toward the fact that the degeneration is a nutritive change and that it is dependent upon the failure of the nutritional function of the cell-body. This is called primary deg emtio n. When any part of a neurone is severed from its cell-body, com- pletely or partially, that part immediately begins to undergo degen- eration. If the separation is complete, it will disappear entirely. This is termed secondary, or Wallerian, degeneration. Two facts are obvious, therefore, in regard to all neuronic degen- eration, namely, that though the process be practically identical in both the primary and the secondary forms, in the former it is caused imme- diately by an inherent functional nutritive disorder within the cell bodies themselves, and in the latter by the gross separation of the de- generating parts from their centers of nutrition. The distinction is an important one. The primary forms arc thus the result of a profound neuronic deficiency. In accordance with all clinical evidence, this is largely due to hereditary, toxaemic, shock, exhaustion and straining influences just as the functional deficiencies in the neuroses are. only more so. En thesi the vigor of the cause or the limited resistance of the neurone constituents is such that the mal-nutritive process emerges from its obscurity and reach* 352 THE NEURONIC DISEASES stage where we can observe it with our microscope and other aids. When it has reached this stage — a stage, remember, which does not indicate the beginning or the end of a new process, but merely the limit of our powers of detection — we term the degeneration organic. A primary organic degeneration then is merely a gross, observ- able deterioration of the neurone resulting from a profound disturbance, inherited or acquired, in the nutritive function of the cell-body of the neurone. This would make it appear, therefore, that primary organic degenerative neuronic diseases are in close relationship to the neuroses. Perhaps the better way to state it would be that the neuroses are at the beginning of the organic neuronic primary degenerations or are earlier forms of organic troubles. As everybody knows the earliest clinical manifestations of all de- generative diseases are functional or neurosis-like in character. This is so common an observation that it needs but to be stated to be readily acknowledged. It is the reason for most of the diagnostic mistakes that are made in the earliest stages of the organic neuronic diseases. Two great forces underlie all of these primary organic degenera- tions. The one is the inherited and represents a diminished vitality, a lessened power of resistance against ordinary stimuli, or even extra- ordinary stimuli, in the constituents of the neurones; the other is the acquired and is revealed in the special virulence or vigor of the exciting cause. In most cases, both of these forces are in more or less operation at the same time, though in some affections, like Friedreich's disease for instance, the one will be the dominant factor, whereas in other affections, like locomotor ataxia, the other will be most in evidence. For the hereditary factor, with all its tendencies, Gowers has re- cently suggested the name abiotrophy and defined it as the "degener- ation or decay of parts in consequence of a defect of vital endurance." This defect of vital endurance characterizes practically all of the pri- mary organic degenerative diseases more or less. The process which seems to take place in the cell bodies is the same as, or is very closely related to, that which is technically called chromatolysis. This term is now employed to cover the alterations in the cell-body which we can observe and which are of the nature of a degeneration. They are not in any sense of the word inflammatory. Chromatolysis is a specific nervous phenomenon and is not wit- nessed in any other tissue of the body. There are various degrees of the process down to complete atrophy and annihilation of the cell. The accompanying illustrations show roughly, but forcibly the be- ginning of the chromatolytic process. The causes of these changes have already been dwelt upon in speaking of the degenerative process in general. Anything that profoundly disturbs the nutrition of the cell tends toward its chromatolytic modification. Hence an inheritance of poor vitality, a toxaemia of marked virulence, an exhaustive strain, or a tremendous shock may initiate it. A certain degree of chromatolysis usually occurs in cases of sec- ondary, or Wallerian, degeneration, as well as in those of primary degeneration. The proximal end of the neurone wastes somewhat while THE NEURONIC DISEASES 353 the distal or severed end is disappearing. This is especially seen after amputations and in the rare conditions of neuromyelitis and ascend- ing degeneration of the motor nerves. This upsets in part the Wal- lerian law that when a nerve is severed from its nutritive center, the distal or severed end only undergoes degeneration : for here we ob- serve the proximal end also undergoing degeneration. As early as twenty-four hours after the damage is done to the peripheral nerve, FIGURE 54. A. A. Normal nerve-cell, stained by the Nissl method. (After Marinesco-Ray- mond. B. Disease of the cell after section of the peripheral nerves. Chromatolysis with nuclear displacement. (After Oppenheim.) changes have been observed, chromatolytic in character, in the related cell bodies of the medullary nuclei and anterior cornua. Restitution sometimes takes place; not infrequently complete atrophy occurs un- der these remarkable circumstances. The explanations offered for this phenomenon are all hypothetical though plausible. For example, it was for a time supposed to b sort of retrograde process, a backward-going Wallerian degeneration. 354 THE NEURONIC DISEASES Many to-day believe, however, with Marinesco, Goldscheider, Len- hossek and others, that in health the nutrition of the cell is excited and maintained by the usual sensory and volitional impulses. When the cell is put out of action, as it were, by these peripheral degener- ative conditions, it is not called upon to functionate as it was wont to do, and is therefore not made the recipient of its wonted stimulus. Its nutrition therefore flags and it undergoes a chromatolytic deterioration. The observable phenomena in chromatolysis I have briefly de- scribed elsewhere. They consist first of a swelling and blurring of the cell, then of a shriveling up with a serration of its edges. The nu- cleus moves to one side (decentralization) and the nucleolus gradually disappears. The w r hole cell has its power to absorb staining reagents markedly lessened. The chromatin bodies move toward the periphery and the dumbbell-like bodies closely hug the edge of the nucleus. The latter bodies stain deeply. Vacuolation appears in the cell substance. As the process advances general atrophy of the neurone takes place and the latter finally disappears. If regeneration should occur, the reverse phenomena obtain, but much more slowly. The space that is left vacant is then filled with an overgrowth of neuroglia and connec- tive tissue (secondary sclerosis). In the secondary degenerations the cell-body undergoes a lesser degree or partial chromatolysis, though sometimes it may be complete and atrophic. The neuraxone, both that part of it "which has been severed from the cell and that which still re- mains attached, undergoes the well-known fibre degeneration; abso- lutely and completely in the former, relatively to the degree of the cel- lular chromatolysis in the latter. It is clear thus far that neuronic degeneration is a special patho- logical phenomenon and characterizes the nobler elements, the true nervous apparatus. The organic (observable) neuronic changes and the functional (non-observable) neuronic changes constitute the basis of the true nervous diseases, the diseases that belong to the nervous system per se. These diseases in all probability differ, so far as their being or- ganic or functional is concerned, in degree rather than in kind. There- fore, I have grouped these two great sets of diseases together and made a special class of them, calling them the neuronic diseases. We have just seen, however, that in the subclass of neuronic dis- eases, called the organic, the degenerative process is primary and sec- ondary in its origin. This of course is merely another way of saying that there are two great groups of causes that damage the nutritive function of the neurone and its several parts. The one group of causes go to the very fountain head of nutrition and overthrow it by directly hurting the cell-body. The other group of causes merely cut off, compress or otherwise mutilate the neurone and its processes so as to interfere with its vitality. Except in regard to heredity, we may say of both groups of causes that they are from without or are extra- neural. A toxin that sets up a primary degeneration is as much an ex ternal disease affecting the neurones secondarily as is a stab wound that severs the spinal cord and sets up a secondary degeneration. In both troubles the degenerations per se are the same, though their loca- THE NEURONIC DISEASES 355 tions and immediate causes are different. A poison that initiates a primary polyneuritis, so-called, is really as much of an extraneural cause as is a cerebral embolus that cuts off the blood supply of a cer- tain area of cerebral neurones. The peripheral degeneration, as a mere disease process, is as much of a degeneration as that which oc- curs in the central trouble. It is obvious, therefore, that our classification of these degenera- tions into primary and secondary is quite artificial and is made only for coarse practical purposes. This is only too well demonstrated, and to our chagrin, when we try to decide whether a certain degenerative disease is a primary or a secondary one. We tacitly admit in such in- stances that the degeneration is one and the same but we can't make up our minds whether the causative factor is the primary or the sec- ondary one. How often this is the case ! It is perhaps best illustrated, however, in the development of our ideas in regard to locomotor ataxia. One hypothesis has declared that the degeneration of the peripheral sensory neurone is the direct result of the syphilitic virus in some way. Another hypothesis argues that it is due to a slow compression of the posterior nerve roots by the specifically inflamed meninges. The former explanation makes of tabes a primary degeneration ; the latter relegates it to a secondary degeneration from meningitis. Take as another illustration multiple neuritis. A degeneration of the peripheral neurones is observed in practically all cases. In the toxic cases it is sometimes a primary degeneration and due to a polio- myelitic process ; sometimes a secondary degeneration and due to a compressing interstitial inflammation. Landry's paralysis may be an initial stage of primary or secondary degeneration in the peripheral motor neurones. Ophthalmoplegia is both a primary and a secondary degenerative disease. The point I wish to emphasize by these and numerous other illus- trations that could be cited is that in these organic neuronic degenera- tions we have a sort of borderline group of diseases which lie between the purely inherent neuronic troubles, like the functional diseases, and the non-neuronic troubles like the inflammations and neoplasms that only affect the neurones secondarily and by compression. Therefore, it is difficult to classify positively all of the organic neuronic degenerations, for sometimes we behold them as primary pro- cesses, at other times as secondary processes. And yet I feel that the best interests of neurology will be subserved by a general classification founded, so far as present data will permit, upon the distinction be- tween the primary and the secondary organic neuronic degenerations. The two sets of diseases in which the primary and secondary neuronic degenerations respectively appear an- so dissimilar in respect to etiology, prognosis and treatment mat for this reason alone it seems to me most practical to emphasize the distinction by thus making it the basis of a classification. A degeneration of tin- pyramidal tracts from an intracranial hemorrhage may be no less serious, ; ,s a degeneration, than that which we see in amyotrophic lateral sclerosis, but the sec- ondary nature of the former and the primary nature of the latter in- sinuate an important distinction between them when it comes to the 35^ THE NEURONIC DISEASES matter of their prevention by early treatment, or to the question of prognosis when they first begin to appear. Primary degenerations are usually hopeless, whereas secondary degenerations, especially if they don't implicate the nutritional function of the* neurone bodies, are comparatively hopeful. In taking up what I have termed the organic neuronic diseases in the next chapter, I have in mind especially the primary organic neu- ronic degenerations. The secondary degenerations will be incidentally referred to in the latter part of the book as the expected or accidental sequelae of the extra-neuronic diseases in which they happen to ap- pear. To illustrate, progressive muscular atrophy with its atrophic paralysis is regarded as a primary degeneration and is therefore classi- fied and treated here as an organic neuronic disease ; acute anterior poliomyelitis with a similar atrophic paralysis is regarded as an in- flammatory disease of the spinal cord and is therefore classified and treated elsewhere as a non-neuronic afTection. The neuronic degener- ation here is regarded as a secondary process, an early sequel as it were, of the infectious myelitis. Where a disease happens to be either a primary or a secondary degenerative trouble, it will be treated of most fully in the section wherein it would naturally fall by the greater prominence of the one or the other of its manifestations, and it will be only incidentally men- tioned in the other section. Moreover in this way, where the classi- fication O'f the disease is in doubt among the authorities, I will intimate my own opinion in regard to it. System Diseases. — The sharp localization of the lesion and its limitation to certain definite neurones is a remarkable characteristic of these organic degenerative diseases. The phenomenon has always aroused the greatest investigative enthusiasm among neurologists, and there have not been wanting plenty of hypotheses. To-day, however, we are as much in the dark as ever. Why such a general cause as heredity or toxaemia should provoke a degeneration in a particular set of neurones, and not in others, is still a profound mystery. And even in the secondary degenerations there is no adequate explanation as to why certain groups of neurones should be more implicated than oth- ers. If the explanation is to be found in a biological, evolutionary embryonic or developmental influence we are not yet in a position to adequately word our explanation. This is the same as saying that we do not know how heredity acts. I am convinced, however, that even in the inflammatory troubles with their so-called secondary degenerations, such as acute anterior poliomyelitis, though the location of the inflammation may be accounted for in some way by the peculiarity of the circulation in and through the anterior horns of the cord in this disease, the frequency with which certain of the cells, as those that preside over the tibialis anticus muscle, are affected, is due to some hidden biological fact, Mime spe- cial susceptibility conferred upon them by their evolution, their early use or their embryonic development. Certainly heredity plays a role, though perhaps a minor one. along with the infection, in these focal, inflammatory troubles with their THE NEURONIC DISEASES 357 sharply localized secondary and permanent degenerations. In the purely hereditary troubles, like Friedreich's disease, we can go no farther than to say that an inherited biological or embryonal influence is at work. It would be perfectly useless to try and imagine why the brunt of the damage falls upon some neurones and not upon others. The fact is as much of an enigma as is the origin of sex. In regard to the organic degenerative diseases that follow a gen- eral intoxication, such as locomotor ataxia, we can make a guess, but only a feeble one, as to why the lesion is confined to a particular set of neurones. In this case we can reason behind the intoxication, as it were, and found our guess upon a biological and physiological basis. In some of them there is undoubtedly a localizing influence exerted by the heredity, along with the general strain or intoxication. If in the pure hereditary degenerative troubles certain groups of neurones are constantly broken down, it is but a fair conclusion to suppose that in some other acquired diseases, this same sort of heredi- tary influence may lie dormant, and does thus lie dormant, until it is rendered effective by the added presence of the depressing intoxica- tion. The question is often asked, for instance, why all, or even more, syphilitics do not develop locomotor ataxia, if syphilis be indeed the cause of this disease. One legitimate answer would be, though it would amount only to an inference drawn from analogy, that in cer- tain individuals who acquire the infection, there is an inherited weak- ness in the sensory system. The infection lowers the resisting power of the sensory neurones and immediately the hereditary tendency re- veals its presence and force. Another answer to the same question could be made in accordance with the Weigert-Edinger hypothesis. In this it is assumed that those neurones or systems of neurones, which are normally called upon to do more work than other systems, have the nice balance between their combustion and repair, their de- structive and constructive metabolism, more quickly and more pro- foundly upset than those which are not subject to such strenuous du- ties. Edinger argues that the sensory nervous apparatus is. compara- tively speaking, never idle and that certain parts of it are even more constantly in action than are other parts. For example, in locomotor ataxia the peripheral sensory neurones of the legs and eves are the chief points of the localization of the lesion because normally these neurones are incessantly in action in all of the performances of the waking state, reflex and voluntary. Of these two hypotheses, I prefer the former for several reas Edinger's theory does not answer the question why all syphilitics do not develop locomotor ataxia. It is to be presumed that the plrj logical strain upon their sensory apparatuses is approximately the same in all. Moreover the hypothesis omits entirely to account for the purely hereditary cases and those that begin to show their sharp localization even before the special neurones are actively called ii|x>n to work. If overwork is to he employed in explaining the localizatioi the lesion in these acquired troubles of adult-, the theory cannot tainly be laid aside entirely when the question involve ime sort of a lesion that appears early in life. I can much more readily un- 358 THE NEURONIC DISEASES derstand how heredity, strong or weak, may provoke the degenera- tions in Friedreich's disease and ordinary tabes, though it be the sole cause in the former and the partial cause in the latter, than can I con- ceive of a force determining a lesion in one disease such as super- function does, as it is claimed, in tabes, but doing nothing in another disease with a similar lesion such as the posterior degeneration ob- served in the Friedreich type of the trouble. Heredity can cover both FIGURE 55- ttndl Capsule "RooTs "Post Schematic representation of the neuronic structure of the motor and sensory pathways. (Modified from Jakob.) diseases in part at least ; but super-function can only be made to apply to one of them. If it be answered, however, that in Friedreich's dis- ease, the influence of excessive function is the proper explanation just as it is in locomotor ataxia, then we must assume that it accounts for the accompanying pyramidal degeneration in the motor tracts as well. If this is granted, then by analogy we ought to have the motor neu- rones more often degenerated in locomotor ataxia than we do. THE NEURONIC DISEASES 359 On account of these objections, I do not think, therefore, that the Weigert-Edinger hypothesis i9 as well supported as is the hereditary in explaining why certain groups of neurones are so much more liable to degenerate than others are when the same exciting causes are in operation. If the reader will recall the preceding chapter upon the neuronic architecture of the nervous system, and the illustrations that accom- pany it, he wall remember that the neurones were grouped together into various motor, sensory, commissural, associating and storage sys- tems. Lying side by side their cell-bodies formed the ganglia while their processes constituted the tracts. Each tract was made up of an FIGURE 56. Schematic representation of the neuronic structure of the sensory nerve tracts. (Modified from Jakob.) immense number of neuronic pathways along which the individual im- pulses traveled from one end to the other. In their course we learned that the impulses had to proceed step-like over two or more neurones, placed end-to-end, as it were, by synaptase, before they reached their journey's end. We analyzed two of the pathways about which we know the most, namely, the projection sensory and motor paths. We found that the former consisted of a number of neurones in apposi- tion, whereas the latter was made up of only two, the upper and the lower neurones. Our acquaintance with organic neuronic degenerations is entirely limited to the diseases of these projection sensory and motor path- ways. Doubtless there are similar degenerations in the commissural, 360 THE NEURONIC DISEASES association and intercalary systems of the brain and elsewhere that, for aught we know, underlie many obscure psychic and other troubles. We are far from being in a position to affirm that we know all of the degenerative diseases of the motor and sensory projection sys- FIGURE 57- leics /VWscl< terns. Even of those we do know we have still much to learn. Never- theless as the neuronic construction of these projection systems, so well shown in the accompanying diagrams, is such that the division of them into brain, spinal cord and nerve tracts is simply untenable from the clinical standpoint, we must, if possible, make the lines of divis- THE NEURONIC DISEASES 361 ion correspond with the divisions between the individual neurones. On the motor side we can do this much more easily than we can on the sensory side, for the neurones that make up the individual pathways are much less in number and the points of separation between the two longitudinally related neurones are more uniformly and distinctly lo- calized. The upper neurones of the motor tract consist of a group whose cell-bodies lie embedded in the cerebral cortex around the fissure of Rolando and whose neuraxones pass down in a bundle through the internal capsule, the crura cerebri and the direct and indirect pyra- midal tracts of the cord. They all terminate in arborizations, but at various levels, in the column of gray matter that makes up the ante- rior horns of the cord and its homologous extension, the motor nuclei in the medulla oblongata. Their arborizations surround the cell- bodies found there. In this way, it will be observed that the cerebral cortex is brought into physiological communication with the cell- bodies of the lower motor neurones lying in the motor cranial nuclei and the anterior cornua. These variously lengthened upper neurones, taken all together, constitute what is called the Upper Motor Segment. The lower neurones of the motor tract are observed to have their cell-bodies where the neuraxones of the upper segment terminate in arborizations, namely, in the motor cranial nuclei and the anterior cornua of the cord. Their neuraxones pass out through the motor cranial nerves and the anterior roots and spinal nerves respectively. They terminate in the motorial end -plates of the various muscles to which they are supplied. In this way the entire somatic musculature of the body is brought into direct physiological connection with the an- terior horns of the cord and motor cranial nuclei, and into indirect physiological connection, through the upper motor neurones, with the cerebral cortex. These lower neurones, taken en masse, make up what is termed the Lower Motor Segment. The functions of these respective segments, though motor, are somewhat different, a fact which must be remembered when their dis- eases are clinically considered. The lower segment, through its indi- vidual neurones controls and stimulates the contractions of the indi- vidual muscles of the body. It also presides over their nutrition. \s its constituent neurones spring from various levels in the cord and from different motor cranial nuclei, the location of the lesion in a cen- tral disease can thus be determined by the particular muscle or the particular motor nerve that is symptomafically affected. Furthermore, disease of this lower motor segment, or of any of its constituent neurones, produces for obvious reasons a flaccid paraly- sis, a muscular atrophy and an electrical reaction of degeneration. Since these lower motor neurones serve as the efferent pathways t'<>r all the reflexes, their damage also causes a diminution or loss of all forms of reflex action. The principal organic degenerative diseases thai are limited to the lower motor segments, that we know of, and that will hi- discussed in the next chapter, are poliomyelitis, Wernicke's superior and inte- rior polioencephalitis, the acute ascending paralysis of Landry, asthenic 362 THE NEURONIC DISEASES bulbar paralysis or myasthenia gravis, and the primary degenerations of the cranial motor nerves, such as the ocular, facial, hypoglossal and motor branch of the trigeminal. FIGURE 58. N^uscle dcreie/l um The upper segment, through its constituent neurones, controls, stimulates and inhibits the activities of the lower segment. It does not influence, nor does it even know of, individual muscles. It pre- THE NEURONIC DISEASES 363 sides only over systematized physiological movements. These move- ments are produced by the combined action of a number of physiolog- ically related muscles. The distinction between the functions of the upper and lower seg- ments in regard to these physiological movements and the individual muscular contractions is of immense importance in clinical practice. I am in the habit of teaching that the upper motor neurones are psychic, so to speak, in their activities and, like the mind, are cogni- zant of general movements only, such as those of the leg or of the hand, whereas the lower motor neurones are wholly spinal and are cognizant only of individual muscles, such as the deltoid or the quad- riceps femoris. The upper motor neurones do not enter into the production of an ordinary reflex, though by reason of their inhibitory control over the lower neurones they inhibit the normal reflex somewhat. They do not exert any special trophic influence, though it is assumed that their normal stimulant action upon the lower neurones helps in a measure to preserve the latter 's proper nutrition. Remembering then the relationship, anatomical and functional, of the upper motor segment to the lower, we are not surprised to discover that when it is diseased and its inhibitory control is thus withdrawn from the lower segment, the muscles are thrown into state of spastic rigidity or spastic paralysis; that this paralysis implicates muscle groups which subserve the movements of various parts of the body like the face, the arm, the leg or the whole half of the body, but does not Implicate merely individualized muscles ; that the reflexes are exag- gerated ; that there is little or no muscular atrophy; and finally that the normal electrical reactions remain unchanged. The more important organic diseases of the upper segment thai we know of and that will be treated of here are the spastic paralyses of adults and of children, hereditary spastic paraplegia, Erb's spinal paralysis, some secondary spastic paralyses and briefly hysterical spas- tic paraplegia. If Strumpell's cortical polioencephalitis be listed, though there is some doubt as to its existence, it will have to be in- cluded here among the secondary degenerations. There are a number of diseases that involve the entire motor tract. both upper and lower segments. Foi obvious reasons the dominant symptoms will be those of the lower motor segment. To this, how- ever, there is one exception, namely, the type of progressive mil- iar atrophy which Charcot named amyotrophic lateral sclerosis. Here the disease process seems to begin in the lower end of the upper motor segment and hence for a time the dominant symptoms are those of an upper motor segment disease. The troubles that we usually classify as involving the whole mo- tor tract are the progressive muscular atrophies, progressive bulbar paralysis, the progressive muscular dystrophies and the amyotrophic lateral sclerosis. The sensory tract is far more complicated than the motor and in regard to its organic degenerative diseases we are not well informed. We are best acquainted with those that belong to the peripheral neu- 304 THE NEURONIC DISEASES rones of the system, namely, locomotor ataxia, tic douloureux, some neuralgias about which, however, there is considerable doubt, and the primary degenerations of the nerves of special sense, especially the optic. FIGURE 59- The best way to indicate the construction of the various pathways that make up the sensory system will be to trace an impulse along them from the skin to the brain. The accompanying diagrams will assist the reader in following the courses of the impulses. THE NEURONIC DISEASES 365 Leaving the skin and passing first along the sensory nerve, a stim- ulus arrives at the posterior spinal ganglion where the cell-body of the respective neurone is found. Up to this point the impulse has been traveling' by way of a long, modified dendrite. After it leaves the cell-body in the posterior ganglion it emerges along the neuraxone •of the same neurone that passes directly into the cord by way of the posterior root to arborize around a group of cells embedded in the pos- terior horn. From this second cell, and by way of its neuraxone, the impulse, crosses through the anterior commissure to the lateral column of the opposite side where it ascends in the antero-lateral ascending tract of Gowers to finally pass through the medulla and pons and to arborize around the ceil-bodies nesting in the optic thalamus. Con- tinuing on through this third cell and its neuraxone, it ultimately reaches the cerebral sensory cortex. This is what is called a direct sensory path and it consists of the three end-to-end neurones, the peripheral, the spino-thalamic and the thalamocortical. Another direct pathway is that up the columns of Burdach and Goll to the nuclei, cuneatus and gracilis, at their summits, thence to the thalamus, and so on to the cortex. The peripheral neurones in these instances are of enormous length and actually extend from the skin, through the posterior root, to the top of the posterior columns of the cord. There are certain sensory impulses from the muscles, the joints and the viscera that reach the brain through a very much more in- direct route than the two just outlined. The^e indirect pathways are not well known and we can only trace the impulses along them in a tentative sort of a way. Entering the cord by way of the posterior roots, the latter ascend the posterior columns to their respective nuclei, thence they cross to the opposite side in the sensory decussation, some ultimately reaching the cerebellar cortex where they terminate, others continuing on from the cerebellar cortex to the red nucleus, optic thal- amus and cerebral cortex. Other impulses of this sort ascend to the cells of the column of Clarke, thence to the cerebellum by way of the direct cerebellar tracts, and so on to the brain. These indirect paths may be summarized thus, peripheral sensory neurone, spinocerebellar neurone, cerebello-thalamic neurone, and thaJamo-cortica] neurone. Along the direct paths are supposed t<> pass all the sensations "i" touch, temperature and pain; along the indirect travel the impulses from the muscles, tendons and joints that have to do with coordina- tion and the maintenance of equilibrium and tin- activities of the vis- cera. My excuse for introducing here this minute account Off tin- sen- sory pathways is that they are all implicated apparent!) in the - sory system disease about which we know the most, namely, locomo- tor ataxia. Further details in regard to tin- anatomy and physiology of these various projection pathways will Ik- found in the chapters de- voted to the physiology of the brain and spinal cord. The considera tion of the neuronic structure of tin- special sense systems will not be 3^6 THE NEURONIC DISEASES taken up in detail here as it belongs to the description of the various- cranial nerves later on. There are quite a number of organic neuronic degenerations that FIGURE 60. Stniory Nerve jtom NVusclf etc. crxsory Nerve' j^onTViScerx occur in a combined way in both the sensory and motor systems. Chief among these are amaurotic family idiocy, some forms of hereditary cerebellar ataxia, Friedreich's disease, ataxic paraplegia, the primary THE NEURONIC DISEASES 367 combined sclerosis of Putnam, toxic combined sclerosis, progressive interstitial hypertrophic neuritis of children, multiple neuritis and neuromyelitis. Some authors are perhaps justified in classifying among these or- ganic neuronic degenerative troubles, because of the frequent prominence of their systemic manifestations, syringomyelia, infantile diplegia from agenesis, delirium grave or Bell's mania, and dementia paralytica. I prefer for the present not to do so, however, because it seems to me that the extra-neuronic manifestations are sufficiently prominent to still keep them entirely out of this class. Their neuronic changes, except perhaps in a few exceptional cases of Bell's mania are, in my judgment, emphatically secondary. DISEASES OF THE AFFERENT OR SENSORY SYSTEM. LOCOMOTOR ATAXIA. Locomotor ataxia is a better name for this disease than either tabes dorsalis or posterior spinal sclerosis. All three are objectionable however. It is not a motor trouble, as might be inferred from the first name. It is not a wasting of the back, as the second suggests. And it is not primarily a posterior spinal sclerosis. As the ataxia, shown in the gait, is the most obtrusive symptom, the name locomotor ataxia is perhaps the most convenient to employ. The disease consists of a slow, progressive degeneration in the peripheral sensory neurones, and their related structures, with a secon- dary sclerosis of the sustentacular tissues. Clinically it is character- ized by certain sensory phenomena, incoordination of movement, and trophic disturbances. It is notable for the absence of all true motor symptoms and mental aberrations. Etiology. — Since the initial work of Fournier, Erb and others, the opinion has been growing that syphilis is the cause of locomotor ataxia. Tersely stated, the hypothesis would be, — no syphilis, no loco- motor ataxia; or in other words, every case of locomotor ataxia pre- supposes syphilitic infection. Many do not accept entirely this dictum because no sets of statistics have yet been brought forward in which a positive history of syphilis was obtained in every case. Even Four- nier and Erb admit this, but as such a history is obtainable in at least 75 to 85 per cent, of all cases, they and others assume that it has 1>een overlooked in the remaining 15 or 25 per cent. This is not improbable as syphilis is sometimes innocently acquired and honestly denied. At other times it reveals such slight and evanescent primal*} and ondary manifestations as to be very easily overlooked. It is not abso- lutely true, however, as is frequently asserted, that it is in this class of syphilitics that locomotor ataxia is most prone to occur. While a con- servative opinion would not agree with Fournier that locomotor ataxia is parasyphilis or metasyphilis, a mere sequel of constitutional syph- ilitic infection; and much less with Lesser, who believes that it rep- resents a fourth stage of syphilis, thereby being in no sense a separate and distinct entity of itself, it does accept the Statement that syphilis is far and beyond all others a cause of locomotor ataxia. 368 THE NEURONIC DISEASES Some of the conservatives hold that the specific infection serves simply as a predisposing cause and in that sense may underlie most of the cases. Other causes, however, must come into play along with the specific to provoke the disease under discussion. A still larger number of conservatives argue that in the 15 or 25 per cent, of cases in which a history of syphilis is not obtainable, the other causes may and probably do produce tabes without the existence of an underlying specific, predisposing factor, such as syphilis. That syphilis precedes the appearance of tabes in a large majority of cases is undeniable. That it is an antecedent demonstrable in all cases, has not yet been shown. Therefore, that it is the only cause of locomotor ataxia is still an open question. The other causes that are believed to act in conjunction with syphilis and in occasional cases possibly alone, are excesses of all kinds, especially excessive venery, occupations, such as that of the syph- ilitic driver whose ataxia began in the right arm, exposure to prolonged cold and dampness, strains of all kinds, such as running a machine, climbing arduous steeps, difficult parturition with hemorrhage, pro- longed lactation, depressing emotions, and spinal injuries. Simple traumatic tabes is to be regarded with doubt. Apart from syphilis, other infections have been credited with the disease, namely, pneumonia, rheumatism, typhus fever. Most tabetics have had gonorrhoea but it is not a cause. Latent syphilis may have been present in all of these cases for where such causes are discov- ered apart from syphilis they, like ergot when used in excess, produce an atypical form of the disease or a combined type of degenerative sclerosis. Heredity does not play a significant part in the etiology though it is believed by many that an inherited neuropathic predisposition goes far toward explaining why some syphilitics acquire locomotor ataxia and others do not. Men are^more frequently attacked by the disease than women, in the proportion almost of ten to one. This may be because of the greater liability of men to the various causes spoken of above; and yet the difference is so marked that a special susceptibility is believed by many to belong to the male. The disease appears preeminently in the middle period of adult life. More than half of the cases occur between the ages of thirty and fifty. About thirty-seven seems to be a favorite year for its commence- ment. It has been observed as early as ten or twelve years of age, and as late as sixty-five. It is exceedingly rare, however, before twenty and after sixty. In some mysterious, probably biological, way, race and nationality appear to exert an influence upon its development. In the Negro, the Jap, the Egyptian, the Bosnian and the Herzegovinian, the Kirgizh and the natives generally of East and Southeast Africa, among whom syphilis is rife, tabes is all but unknown. This has been used as an argument against the syphilitic etiology of the disease ; but it may be counter-argued that it is because syphilis in these races has effected an THE NEURONIC DISEASES 369 immunity on account of its very excessiveness and almost universality, against its own tertiary accidents. Pathology and Pathogenesis. — If syphilis is the cause, medi- ately or immediately, of locomotor ataxia., the lesions found are not those commonly recognized as syphilitic. Hence, tabes is not a syph- ilitic affection in the strict anatomico-pathological sense. The degener- ation that is known now as the primary process of the disease is a remote result of the specific intoxication, the wreckage, as it were, after the storm. Most of the syphilitic cases reveal their first symptom in from five to fifteen years after the acquisition of the infection. In a large number of these cases the signs of syphilis have been slight and when the tabetic manifestations had begun to appear were prac- tically nil. Only the suspicion of an initial lesion can be formed some- times. In women usually this is wanting. Erb, Fournier and others have noticed that in a few case? in which no history of syphilis could be secured, the father was syphilitic. The term tertiary as applied to the disease in the sense of be- ing a late manifestation of syphilis, is misleading, for the primary infection may precede the outbreak of the trouble anywhere from one to forty years. The most prominent and constant post mortem finding in loco- motor ataxia is the degenerated appearance of the posterior spinal columns. Burdach's columns, especially in the lumbar and dorso- lumbar regions, and Goll's columns higher up. are seen to be graver, smaller, harder and less punctated than normally. A narrow ventral area just alongside the posterior commissure escapes the change. Clarke's vesicular columns, Lissauer's tracts, and the posterior corn u a are included in old cases of the disease. This can all be readily ob- served with the naked eye. This pathological picture is what misled the earlier investigators into giving the names to the disease that they did and in placing it among the spinal cord affections. Two things are to be distinctly noted in regard to the pathological basis of locomotor ataxia, as we know the disease to-day. They are, first, that the disease is not an inflammation, but a pro^ressiz'e defen- eration, and secondly, that this degeneration occurs in the peripheral sensory neurone, whose cell-body rests in the posterior root ganglion and whose processes extend out into the peripheral nerve on the one hand and up into the posterior tracts of the cord on the other. The degeneration may be looked for anywhere in the entire cutaneo-spinal extension of this neurone. It is more often found, however, in the outer or cutaneous end and the inner or spinal end than anywhere in the middle or in the cell-body. It is most frequently observed in the spinal end. As these spinal end- extend up int.) the posterior columns, it is clear why in the great majorit) s, these columns are the principal seat of the disease. Microscopically this symmetrical, bilateral posterior degeneration is seen to originate in the nerve fibre- and run through the usual c >urse. The neuroglia may remain normal but as a rule it proliferates along with the connective tissue. A few remaining fibre> may be seen run ning through the proliferated tissue. The bloodvessels 7 3 Marie has himself lately given up this hypothesis it still seems to be somewhat in favor in some quarters. Redlich and Obersteiner, noting the constancy and the character of the degeneration in the posterior roots and the frequency of the adjoining meningitis, propounded the theory that a chronic specific meningitis of the posterior parts of the cord is the primary disease. This meningitis, by constricting, compressing and otherwise injuring the roots where they pass through it sets up an atrophy in them and thus transmits the disease by contiguity up into the cord. This hypothe- sis brings locomotor ataxia into line with the established forms of spinal syphilis by making of it primarily a vascular disease, a spe- cific meningitis. Many of the earlier symptoms, especially the pains, are not unlike those observed in meningitis involving constriction of the posterior roots. The objections to the theory, however, are that it fails to explain the almost universal limitation of the meningitis and its associated degenerative sequelae to the posterior parts of the cord (though Marie and Guillain have latelv invoked the peculiar vas- cular and lymphatic supply here to solve this difficulty) ; that it ac- counts for a degenerative process that ought accordingly to appear more frequently in other forms of chronic specific and non-specific men- ingitis ; and that it fails utterly to explain the not infrequent associated degeneration of some of the cranial nerves, as for example the optic, and the peripheral cutaneous nerves, and of the posterior spinal ganglia. Every explanation hitherto advanced leaves without an answer the question as to why the disease, whether it be syphilitic or not in origin, begins and limits itself to the sensory neurones, and especially to the lower sensory neurones. Biology, embry- ology, evolution and heredity have all been invoked to show that there is a special, neuropathic weakness in these particular neu- rones and that therefore they yield to the virus of the syphilis. The Edinger theory endeavors to cover the ground. In it the assumption, first noted by Weigert, is made that certain parts of the nervous appa- ratus are worked more than others with consequently a more rapid disintegration and consumption of the •elemental metabolism. This occurs in the sensory system which is ceaselessly transmitting im- pulses from the periphery to the brain. Under the stress therefore of such a universal depressing agent as syphilis, the sensory system is the first to give way. Symptoms. — Xo case of locomotor ataxia presents exactly the same clinical picture as any other case. The variations in the combi- nation of symptoms and their character are innumerable. Tims arbi- trary types of the disease have been described, much to the confusion of its diagnosis. There is, however, a fairly definite symptomatology and keeping well in mind this typical picture, oik- can easily recognize the slighter shades of variation which i' may under I will first attempt a description of the typical presentation of the disease and then discus- a little more in detail a few of its varia- tions. It must be remembered that the symptoms in all cases will be those that one would naturally expect from a slow, progressive 374 THE NEURONIC DISEASES generation here and there in a widely extended, peripheral sensory apparatus. Progressive sensory disturbances form the basis of the sympto- matology of tabes. These may begin and remain in the legs ; they may extend to the arms; or they may commence in and be limited to the arms. They may involve the sensory nerves of the head and of the viscera. Later symptoms may diverge from the purely sensory type so that trophic, motor and vasomotor manifestations may be present. The uniformity of the symptomatology of tabes depends upon its sen- sory character and upon its steady progressive course; its variability depends upon the variations in the location of the degenerative pro- cess. While the former tempts us at times to make a diagnosis upon the barest indications, the latter should warn us not to do so unless two or more symptoms are present. A mere pain, a mere diplopia, a mere loss of knee-jerk or a mere loss of vision, without any other symp- tom to base a diagnosis upon, even when there is a history of syphilis, ought never to more than arouse a suspicion of possible tabes. Only in this way can mistakes be minimized in the diagnosis of a disease which assumes such a variety of outward forms. The first indications of locomotor ataxia usually make their appear- ance in from five to fifteen years after the contraction of the syphilis. Ten years is about the average. The patient notices a feeling of weak- ness and tiredness in his legs, especially after a slight exertion, not un- like that seen in neurasthenia. He observes that in writing or button- ing his clothes he has not quite his usual control over the fingers. Along with these suspicious manifestations there may appear here and there about the body slight fugitive momentary pains that are mistaken for cold and rheumatism. On the other hand, none ot he forgotten, however, th;it though the incoordination is the mOSl <>l>vi"iiv symptom, it i> n.t by any means the most important. Ataxia appears in the legs in about J$ per cent, of all cases, and in the arms in aboul 8 per cent. The symptom occur in othei affections than tabes, notably in multiple neuritis, disseminated cerebrospinal 37% THE NEURONIC DISEASES FIGURE 64. FIGURE 65 Trunk anaesthesia in locomotor ataxia. THE NEURONIC DISEASES 379 sclerosis and cerebellar disease. When it has appeared, however, in the course of tabes, the disease is usually so far advanced that there are other indications present which render the diagnosis a comparatively •easy one. The affection at this time has reached its full development. The movements of the arm or leg will be observed to be full and strong. In other words, there is absolutely no motor paralysis. In walking the patient exhibits a decided uncertainty, especially if the room be dark- ened. In fact, it is sometimes quite impossible for him to get about at all in the night. Occasionally the trouble is first noticed by the patient when he ascends or descends a flight of steps. He stumbles unaccount- ably. Not rarely it is noticeable objectively before the patient himself is aware of it. A momentary closing of the eyes, or a looking away from the floor or neighboring wall increases the ataxia so much that it is all but impossible to walk. In walking the patient lifts his leg "higher than it is necessary, rotates it slightly outward, jerks it forward, stamps it down upon the floor with the heel first and the toes last. When he is seated the ataxia is observed as soon as he is asked to kick the leg of the table. With the hand he finds it difficult to perform the more delicate and •complicated movements, such as writing, buttoning his clothes or touch- ing the end of his nose quickly when his eyes are shut. Ataxia may involve the facial and tongue muscles, though only very rarely. More significant still are the spontaneous movements, which are sometimes seen in the ataxic arms or legs. They are entirely involun- tary. The patient cannot keep his limbs quiet. The arm suddenly rises to an elevated position or the hand is lifted up, and all without the pa- tient's knowledge if he happen not to be looking. According to Hirsch- berg, these weird movements are not the result of a complication, but are a manifestation of the incoordination. Like the latter, they are increased when the eyes are closed. The duration of the ataxic stage may be many years and, like the preceding period, be characterized by intervals of apparent quiescence or slight remission in the progress of the disease. As the second period draws towards its close the incoordination becomes so bad that it is quite impossible for the patient to move about at all. In a short time he takes to his bed and the disease passes into the so-called paralytic stage, which, as a rule, is much shorter than either of the other two. The pains have at last ceased. The eyesight is almost entirely gone. The anaesthesia and the analgesia are extensive and profound. The bladder and the rectal function., are most seriousl) disarranged. Trophic disturbances result in obstinate ulcers, bed-sores, muscular wasting and especially softening and disease of the bones and joints. Distortions and spontaneous fractures are seen in this stage The muscular scum- is lost and the stereognostic mux- is gravely dis- turbed. Anosmia and deafness even may be present. With all this, however, there is still no actual paralysis if the case is an uncomplicated one. The mind unfortunately remains clear as a rule, though in some cases it happily becomes clouded or weakened. The patient grows abnormallv emotional Thus be lridden, foolish, wasted, a physical and 3 8o THE NEURONIC DISEASES FIGURE 66. I Locomotor ataxia. From a photograph in the Pathological Museum of the Med. Department of the University of Illinois. THE NEURONIC DISEASES 38 1 mental wreck, the victim lingers helplessly on a few months more until ■complete exhaustion from bedsores, cystitis, pyonephrosis or some other intercurrent malady mercifully puts an end to the sad picture. There are symptoms that may appear at any time in the course of a locomotor ataxia and be indicative of the disease : but they are not as constant nor as frequent as those just referred to. Though they are of less value in the establishment of the diagnosis, they are strongly con- firmatory when they constitute a part of the composite picture. Oc- casionally they may appear alone or early in the disease. They may be unaccompanied for a long period of time by any other symptom. Under such circumstances they should always awaken a suspicion of tabes and keep the attendant on the alert to detect the slightest manifestation of any other sign that will confirm the suspicion. Bladder disturbances ma}' be among the earliest symptoms. They occur in about 60 per cent, of the cases. Among my own cases this was a very frequent symptom. At first there is a little difficulty in micturition. The patient cannot void his urine when he feels he ought to. Then again he may feel a desire to void it too frequently. Grad- ually the trouble increases until in the later stages of the disease actual incontinence and retention occur. In some cases this trouble disap- pears in spite of the progressive character of the malady. An early bladder disturbance coming on several years after syphilitic infection should always be mistrusted. Constipation usually, and for the same reason, accompanies the vesical trouble. The early satyriasis and impotence I have already referred to. Oppenheim has seen an involuntary priapism continue for weeks at a time. Nocturnal attacks are not uncommon. Impotence usually follows all such manifestations and is generally complete inside of a year. I am convinced, however, that too much stress is placed by the pro- fession upon the sexual disturbances as a sign of tabes. I saw a case of advanced locomotor ataxia with all the typical symptoms, early pains, Westphal sign, Argyll -Robertson pupil and extreme ataxia pres- ent, which several physicians declined to pronounce tabes because there was not, and never had been, the slightest disturbance of the sexual function. In women a sort of nymphomania sometimes occurs par- oxysmally and is spoken of as a clitoric crisis. The so-called crises are important phenomena. They occur in the earlier periods of the disease. As their name in part indicates, they are sudden, unexpected, temporary disturbances of a more «>r less violent nature and sensory in origin, in the viscera and visceral function-. In the order of their frequency they may be listed about as follows: ( ias- tric, rectal, laryngeal, pharyngeal, intestinal, cardiac, renal and bladder. The gastric and laryngeal crises are the more important, the former on account of their frequency (36 per cent, in Mott's cases), and the latter on account of their alarming character. All of the other crises are comparatively rare. In the gastric crises the patient experieno sudden, twisting, wrenching pain in the region of the stomach. He nits first his food, then gall. He grows pale and weak, and shows by the rapidity of his pulse and generally that he is in great distress. 382 THE NEURONIC DISEASES The crisis passes after several hours, or it may be days or weeks, as suddenly as it came on. There is no foretelling when it may reappear. Trunk anaesthesia can be discovered in all cases with gastric crises. Usually these, as well as the other crises, alternate with the lancinating pains and sometimes are accompanied by a slight fever. The laryngeal crisis occurs much less often. When seized by it the patient chokes and coughs suddenly and for a few minutes experiences a paroxysm, with cyanosis, such as occurs in pertussis. The attack is sometimes very severe and distressing and the victim feels as if he were about to die from asphyxia. The laryngoscope discovers nothing ab- normal. Paralysis of the laryngeal muscles, especially of the crico- arytenoidei postici, has been observed late in tabes. A rectal crisis occurred in 8 per cent, of Mott's cases. It consists of sudden pain and evacuation, or of colic with diarrhoea. Abrupt disturbances of deglutition are regarded as pharyngeal crises. The implication of the vago-accessorius nerve is shown by the oc- casional cardiac and respiratory crises. There is a sharp, quick pain near the heart, with a sudden feeling of oppression and a rapid, irregu- lar pulse. Angina pectoris is not unfrequently suspected, so similar are the two phenomena. Pain in the chest, with disturbances of the respiratory function,, may occur. In a case observed by Egger in Dejerine's clinic a brachyp- nrea had lasted at least four years. The respirations were reduced to seven or eight per minute for two or three hours after eating. Vomit- ing, roughness of the voice and tachycardia were also* present. Renal and bladder crises are characterized by sudden, sharp pains in the region of those organs. Pel and Oppler have described respec- tively a temperature crisis. Optic nerve atrophy probably occurs more frequently than some collections of cases of tabes state. It is a symptom that is not objective- ly noticeable and is not often enough sought for. Mott found it in 30 per cent, of the cases of hospital tabes examined by him. Among" Starr's cases it was found in about 9 per cent. The average, calculated from the statistics of numerous observers, is about 20 per cent. It is sometimes a very early symptom and has been known to precede all other signs for many years. It may occur in any period of the disease. It is a serious trouble, as it is often the forerunner of brain involve- ment. The absolute reliability of Benedikt's dictum, that tabetic symp- toms usually recede or come to a standstill when optic atrophy makes its appearance, is open to grave doubt, though in some instances it does seem to be true. The beginning atrophic changes can sometimes be detected with the ophthalmoscope when the vision is but little inter- fered with. The atrophy is of the simple, progressive type and in uncomplicated tabes is never preceded by optic neuritis. There is no swelling or hyperemia of the disc. It generally is first visible in the temporal halves and gradually involves the whole field. At first the ncuteness of vision is good and there are no central scotomata for colors or white. The visual field is gradually, concentrically and sector-like narrowed. Sooner or later complete blindness comes on ; in the ma- THE NEURONIC DISEASES 383 jority of cases in about three years. One eye may be affected sooner than the other, but as a rule the degeneration is bilateral. Ocular palsies are the most frequent cranial nerve symptoms of tabes. Any ocular muscle, or muscles, may be involved, so that the paralysis is never £n associative one~ In the earlier period of the dis- ease it is apt to be transient ; later on it becomes chronic and permanent. Diplopia, ptosis, strabismus and even nystagmus are met with. Oph- thalmoplegia may occur, as well as myosis and mydriasis. The trophic phenomena that occur in the course of locomotor ataxia are important. They are late symptoms usually, though they have been seen in the pre-ataxic stage. Of these the arthropathies, Charcot joints, are the most significant and occur in about 10 per cent, of the cases. The knee-joint is probably the most frequently affected. The phenom- ena may, however, be seen in the hip-joint, the shoulder, the elbow, the ribs, the ankle and the spinal column. In many respects the trouble resembles an arthritis except that the latter is accompanied by pain, redness and fever. In the Charcot joint a sudden swelling comes on, with a profuse accumulation of fluid; the intra-capsular tissues are destroyed ; the capsule loses its power of contraction ; and a condition resembling a luxation or subluxation supervenes. New substance is found in the joint, and either as an excrescence or free causes a more or less marked deformity. Under these circumstances the long bones are brittle so that spontaneous fractures are frequent. The perforating ulcer, most commonly located in the ball of the foot and often bilaterally in both feet, is a trophic tabetic symptom. Other rare manifestations of this character are spontaneous gangrene, spon- taneous falling of the nails, nutritional disturbance of the skin, falling out of the teeth, ulceration of the mucous membrane of the mouth with alteration of the sense of taste, neuroparalytic keratitis and other trigeminal manifestations, herpes, facial hemiatrophy and forms of muscular atrophy. These are all comparatively rare symptoms. Some of them, as, for instance, the muscular atrophy, may be extensive and involve the whole body. Multiple neuritis (Dejerine) is probably the cause of them, though many hold that they are true spinal cord symp- toms. Unilateral hyperidrosis, ecchymosis or suggillation and various exanthems have been reported, though it is questionable whether they are true tabetic symptoms. Cerebral symptoms occur in the course of tabes, but they arc indica- tive of a complication. They should be noted, nevertheless, because they often modify the true tabetic symptoms. The anaesthesia and anal- gesia are naturally different when there are psychic aberrations pi cnt, from what they would be otherwise. Sudden attacks d\ ven congestive, apoplectiform, epileptiform and migrainous seizures, acute mania with slight changes of character, hallucinations, even visual hallucinations with complete optic atrophy, ma\ all occur in tain They indicate, however, a cerebral complication. Mich as cerebral syphilis or incipient dementia paralytica. Diagnosis. — The diagnosis of locomotor ataxia is n<»t difficult in typical and in advanced cases. Only in the early period of the disease 3^4 THE NEURONIC DISEASES is confusion encountered. One ought never to doom a patient to so hopeless a malady unless two od food, and all the forces that go toward the creation and preservation of con- stitutional vigor, with alternations of moderate exercise, with plent) of rest and sleep, are the chief elements in the general treatment tabes. Rut this is not all, for the symptomatic treatment is always an 388 THE NEURONIC DISEASES important factor and often the only part of the therapy that the physi- cian is called upon to exercise his special knowledge in. The drug treatment of tabes is extremely unsatisfactory. Many remedies have been proposed and tried, but none of them has retained the universal approval of the profession. Most of them were sug- gested long ago when our knowledge of the pathology of the disease was scanty. It was thought that somehow they would overcome or pre- vent the sclerosis which was believed to be the primary trouble. Now that we know the fundamental process is a slow, progressive, paren- chymatous degeneration, we are not surprised at the successive disap- pointments caused by one vaunted remedy after another. Of all these general alteratives the nitrate of silver. has remained in favor the long- est. It may be given by the mouth or hypodermically. It must be given for a long period of time, with frequent intervals of rest to prevent argyria. The dose is about a quarter of a grain in pill. Ergot, potassium iodide, the double chloride of gold and sodium, phosphorus, belladonna, arsenic and barium have been used. I have not seen any benefit that could be clearly attributed to any one of these numerous preparations. The nitrate of silver is the only one of the group that I would deem worthy of a trial. Some of them, such as arsenic, small doses of phosphorus, the tincture of iron, may be given for their gen- eral tonic effect. Erb counsels strongly against strychnine, and most justly so in regard to the use of it in large or even average dosage. On the other hand, I believe that strychnine in very minute doses, not over one two-hundredth of a grain three times a day, when added to rest and other general measures, is a most valuable remedy. In larger doses it may be absolutely disastrous. It must always be employed with caution and one had better err on the side of giving too small than too "large doses. The use of the extracts of gray matter, especially the gray matter of the sheep's brain, as long ago recommended by Constantin Paul, to regenerate atrophied nerves, moved Leyden to recall humorously the similar experiments with products furnished by the ass in Pliny's time. The same atmosphere of the comic surrounds the use of Brown- Sequard's testicular fluid, the goat lymph and the other startling agents now more or less in vogue. These primary, degenerative neuronic dis- eases, progressive and hopeless, offer a glorious field for the unscrupu- lous catch-penny and the ignorant quack. The largest opportunity for the drug treatment of locomotor ataxia is in the management of its special symptoms. The lancinating pains are the symptoms that the patient most vehemently demands relief from. They are sometimes excruciating, they are always wearing and ex- hausting. Ofttimes if they are mitigated but in part he can learn to bear them and for years may go about his ordinary avocations in spite of them. The remedies that have from time to time been recommended for their relief are now almost legion. Most of them will give rise to disappointment. Sometimes one will succeed in one case, only to fail in another; hence it is well to have a full armamentarium ever at hand. The opiates and morphine are to be avoided as long as possible, for fear of establishing a dangerous habit; and yet they arc the most THE NKI KON'IC DISEASES 389 effective agents we have to combat the pains with. At times they must be used, but always cautiously and for as brief a period as possi- ble. After the opiates the following remedies may be mentioned : Codeine, sodium salicylate, cantonin, chloride of aluminum, acetanilid. antipyrin, phenacetin, methylene blue, analgen, salipyrin, lactophenin, pyramidon, exalgine, phosphorus, solanin, cannabis indica, neurcdin and bicarbonate of sodium. Here as elsewhere the axiom holds true that the multiplicity of remedies is in inverse proportion to the benefit obtained from them. Most of the above drugs will prove disappointing in the majority of cases. Exceptionally this or that one may be found effica- cious. The crises usually need special attention. The local application of cocaine for the laryngeal crises, and the hypodermic injection of mor- phine for the gastric, are the readiest means of affording relief. So unsatisfactory are all other measures that one is disinclined even to try them, and yet morphine must be withheld from a tabetic patient as long as his endurance holds out. Bladder and rectal pains may be combated with suppositories of iodoform and belladonna, codeine, antipyrin, ichthyol, warm injec- tions, etc. Morphine and oxalate of cerium may be used for the attacks of vomiting. Nothing will prevent their coming on. For insomnia the usual remedies, such as the bromides, chloral, paraldehyde, hyoscyamus, are all commendable. In a word, the symptomatic management of tabes is no different from the symptomatic management of any other disease. It is only somewhat more resistant and therefore calls for more powerful reme- dies, such as morphine, which very remedies must be avoided. ( hie must not employ his strongest agents too early in the battle, but reserve them until later when the struggle grows fiercer and all other measures have completely failed. Hydrotherapy is highly commendable in all cases of tabes, if em- ployed intelligently and with a certain degree of regularity. The chief benefits from the water-cures of this country and of Europe are due to the systematic method of application usually carried out, and not to any special medicinal virtues in the water. Sometimes it is positively dangerous for a patient to go to one of these establishments, for, being absent from his regular attendant and under the care of those who are responsible for a large and varied assortment of patients, he is apt to be neglected or to urge forms of heroic treatment that may hasten the onward progress of his malady. The good that these establishments afford can just as well he obtained at home if the patient is tractable. For ten or fifteen minutes every day a tepid bath is both grateful and health-giving. Brine and carbonized baths are excellent. I lot baths must never be taken. Sweat and vapor baths, recommended by Leyden for early tabes, had better be employed with great caution. Not baths are one of the dangers of the springs and resorts that have been rei mended. As a rule the temperature of the water should never be al 95 degree^ I\ (old baths and cold douches are approved of by some authorities. Krb treated nineteen patients with cold baths, of whom 390 THE NEURONIC DISEASES sixteen were benefited. It is a question, however, whether the risk run does not outweigh the benefit obtained. Erb affirms that a bath with a temperature above 58.5 degrees F. is positively injurious. Cold wet cloths to the lower limbs, their immersion in cold water after a pre- liminary warming with warm, moist wrappings, are less objectionable than a cold full bath. The water may be from 48 to 66 degrees F. The time of the immersion should be not longer than a minute. The limbs should be vigorously dry-rubbed and kept at rest afterwards. Stimulation of the cutaneous circulation is the object aimed at in this manoeuvre. Cold spinal douches I would never recommend. They may prove harmful. Electricity has been happily used in some cases of locomotor ataxia. Care and moderation must be observed here also. All forms of elec- tricity and all modes of application have been tried. Central galvaniza- tion of the spine and general faradization is a combined method that exercises both a tonic and beneficial reflex effect upon the nervous structures. Three or four times a week, eight or ten milliamperes of the galvanic current may be transmitted through the spine with one pole at the neck, the other at the lumbar region. No sitting should be prolonged beyond five minutes and the treatment should be kept lip for many months. Static electricity and the faradic brush are some- times acceptable to the patient. Pains and anaesthesia have been re- lieved in this way. The current should be strong enough to provoke slight pain, but not muscular contraction. As a symptomatic remedy electricity has a wide field of usefulness ; as a curative measure it is about as valueless as all other remedies. There is danger in its use, as with all other measures, if it is applied too frequently and too heroically. The indication for massage in tabes is as a general tonic. Massage of the skin improves its nutrition and may partly relieve some of the sensory manifestations. Deep massage of the muscles is useless and debilitating to the patient. It is to be employed only when paralysis and atrophy begin to show themselves late in the disease. Passive motion is contra-indicated where the articulations are already too mobile, and the muscles are in a hypotonic state. There is not much of a field for massage in tabes and when it is tried it should be very gentle, and the seances very short. Exhaustion is a condition to be studiously guarded against in tabetics and massage is most dangerously liable to provoke exhaustion. The suspension treatment introduced a few years ago by Motschut- kowsky and popularized by Charcot has not fulfilled the promises it first held out. Temporary effects, such as relief of the pains and im- provement in the gait, follow its application in some, indeed in very many, instances. But they all relapse. The modus operandi of the effect of the treatment upon the degenerating nerve elements is any- thing but clear and in some respects the treatment is unscientific and irrational. However, it is still used to some extent under a modified form, such as drawing the legs forcibly up over the patient while he is lying on his back (Benedict), or approximating the patient's chin to his flexed knees (Blondel), or with the lees extended and adducted. bend- THE NEURONIC DISEASES 391 ing the upper part of the patient's body forcibly forward while he is seated on the edge of a table (Gilles de la Tourette). Operative nerve stretching, once popular, has been completely dis- carded as a therapeutic measure. The wearing of a corset has relieved the pains in some instances, and according to Bramwell tabetics with much ataxia sometimes walk better in India rubber soled shoes. Of all the mechanical measures used Fran J? el's re-education exer- cises are the most in vogue to-day and indeed do seem to afford the best results. They relieve, however, only the ataxia. Their psychic effect on the patient is good and they improve the muscular strength. They are useful in all stages of the disease. The exercises are simple in principle, though in details they may be greatly elaborated. Blocks of wood placed on the floor, short flights of steps, ladders for the practice of the legs, keyboards, simple buttoning and unbuttoning of the clothes, picking up of specially distributed articles for the exercise of the arms, are some of the simple forms of apparatus that may be extemporaneous- ly arranged. A little ingenuity will suggest means by which the patient for a few minutes every day can practice the function of coordination in his legs and arms. This, like all other methods of treatment employed in locomotor ataxia, must not be expected to accomplish too much. In some cases it seems to be without any effect. It should always, how- ever, be given a faithful trial in all cases. Even hypnotic treatment has been suggested in this disease. Beril- lon, of Paris, proposed it in 1895 because of certain functional psychical factors. The proposal is not such an absurd one in view of the fac that many believe the benefit that temporarily accrues from the use of suspension, gymnastics, Frankehs exercises and other measures is largely psychical. The restoration of confidence, the awakening of hope and the anticipation of improvement are all beneficial in this dis- ease and doubtless are in part the explanation for some of the g derived from treatment, whether the treatment be given by the scientific physician or the quack. This, taken in conjunction with the well- recognized characteristic of this disease to cease progressing at times, leads to the erroneous reports of cures. Unfavorable as the prognosis is in this disease, it is the duty of the medical man to use all his best efforts to keep its victims out of tin hands of frauds and religio-medical charlatans. He can do >" b) en- couraging the patient, keeping up hi> anticipation and strengthening his confidence in every way. The quack does this \^\- his <>wn persona] gain; the physician should do it as a part of his general management of the trouble because he will thus keep the patient in the hands of those best able to care for him — namely, the medical profession, he cause the nature of the disease is such as to warrant a certain amount of hopefulness, and finally because such a state of mind exercis< certain degree of positive benefit in minimizing the symptoms and in retarding their progress. OTHER SENSORY DEGENERATIVE DISEASES. We do not know of any other primary systemic sensory degenera- tive troubles. It is not improbable, however, that they ma) 39 2 THE NEURONIC DISEASES There are some forms' of psychosis and certain epileptiform manifesta- tions with pure sensory phenomena, like hemicrania sometimes, that suggest a possible intracranial sensory systemic trouble. A degenera- tive affection is rarely limited to the sensory cranial nerves. x\s a rule such degenerations constitute a part of tabes, dementia paralytica, mul- tiple sclerosis, syphilis and other general diseases, or they are a sec- ondary result of inflammatory and other extra-neuron ic processes. Primary disease, however, has been observed, though very rarely, in some of the cranial nerves. The optic and the trigeminal nerves have probably illustrated the condition most frequently. Perhaps some few instances of tic douloureux could be classified here. The neuralgias,, however, as I have shown elsewhere, are something more than mere nerve troubles of a purely local character, and this is preeminently true of tic douloureux. Primary, bilateral optic atrophy does occasionally occur, though it is much rarer than secondary or associated atrophy. The auditory nerve has been supposed to have been primarily affected,, but if so it must have been very exceptional. Primary disease in the glosso-pharyngeal, pneumogastric and sensory spinal nerves is prac- tically unknown. In general toxic and infectious states they are oc- casionally degenerated, especially the vagus. With secondary degenera- tion they are not infrequently affected. DISEASES OF THE EFFERENT OR MOTOR SYSTEM. a. DISEASES OF THE WHOLE MOTOR TRACT. PROGRESSIVE MUSCULAR ATROPHY. There are a number of affections in which wasting of the muscles,, with a resulting paralysis, is the dominant and often the sole feature. Unlike the association of the paralysis and atrophy observed in acute poliomyelitis and neuritis, in the diseases now about to be considered the combination is one of atrophy and paralysis. In other words, the wasting in the former follows the paralysis, in these the paralysis follows the wasting and proceeds pari passu with it. Many of the for- mer diseases, in fact most of them, are purely inflammatory in character and the neuronic degeneration is a mere secondary result or sequel. In these, however, the degeneration appears to be a pure primary process throughout. It will be remembered that the efferent or motor path extending from the cerebral cortex to the musculature of the body consists of two anatomically separated grand divisions. The upper segment is made up of a group of neurones whose cell- bodies nest in the cerebral cortex, while their axones, of various lengths, extend downward through the internal capsule and pyramidal tracts to their arborizations around the cells of the various intracranial motor nuclei and anterior spinal cornua. This is a compact segment and forms the pyramidal tracts. The lower motor segment consists also of a lot of neurones whose THE NEURONIC DISEASES 393 cell-bodies are to be found in the anterior horns of the cord, while their axones pass out through the anterior spinal roots and related nerves to their termination in the distant muscles. The center of nutrition for each neurone resides in its cell-body. Therefore the tone and health of the upper neurones are dependent upon the integrity of the related cell bodies in the motor cortex ; the tone and health of the lower neurones are dependent upon the integrity of the related cell-bodies in the anterior horns of the cord. Each of these two sets of neurones exercise a nutritive control over the respec- tive structures below them and with which they are in physiological relationship. The tone and health of the lower neurones are to a con- siderable degree dependent upon the integrity of the related cerebral neurones ; the tone and health of the muscular tissues are very greatly dependent upon the integrity of the related spinal neurones. These facts have been all elucidated and illustrated in an earlier chapter. They are repeated here merely to show how a muscular atrophy of high degree may occur from a diseased condition in the cell bodies of the lower motor neurones ; and furthermore, to make plain how a lesser degree of muscular atrophy may occur from a disturbance of the health and functions of the lower neurones by a disturbance in the health and functions of the upper. Every muscular atrophy of neural origin is due to disease of the lower or peripheral neurones. This disease may be primary, in which case it is an original disease of the anterior gray matter of the cord ; or it may be secondary, in which case it is due to disease in the pyramidal tracts. This at once establishes two great types of spinal progressive muscular atrophy, the primary, or what Charcot used to call the protopathic type, and the secondary, or what he termed the deuteropathic type. As in both there must be always some disease of the lower motor neurones, many authorities do not recognize the dis- tinction between the two types just mentioned, but refer to them all as chronic muscular atrophy, progressive (central) muscular atrophy, spinal progressive multiple atrophy, spinal progressive amyotrophy. In view of the immediate pathogenesis of the muscular atrophy, this contention that they are all practically one and the same disease is a strong one. However, there are certain features in the clinical picture of the secondary, or deuteropathic, forms so unique and striking that it will perhaps be just as well to consider the latter in a section by themselves. I will discuss them under the head of amyotrophic lateral sclerosis. There is much better ground for separating the amyotrophic lateral sclerotic cases from those that are not of this type than there is for the separation of the latter into such subclasses as progressive bulbar palsy, progressive muscular atrophy and progressive hereditary muscu- lar atrophy; for these all represent pathologically the same disc with a symptomatology varied merely in accordance- with the gl anatomical location of the lesion; whereas tin- former represents a characteristic pathology that establishes a particular and distinctive clinical picture. Etiology. — In both the Aran-Duchenne type of i ve mus- 394 THE NEURONIC DISEASES cular atrophy now to be considered, and the Charcot amyotrophic lateral sclerotic type to be considered later, the immediate cause of the atrophy is, as was long ago shown by Levy and Lockhart Clarke, disease of the cells in the ventral horns of the cord. The cause of this disease is practically unknown, though Charcot believed that in amyotrophic lateral sclerosis it was secondary to disease in the pyramidal tracts. This is doubted to-day and even in Charcot's type the deterioration in the ventral horns is thought to be primary. Men are affected more frequently than women with progressive muscular atrophy. In most of the cases it appears between the ages of twenty-five and forty-five. A hereditary influence reveals itself in a small proportion of cases and in these it seems to be merely of a neuropathic character. In rare instances the heredity seems to be direct. In these cases several members of the same family may be afflicted and the disease occurs at an earlier age than usual. Such cases of a hereditary or familial type of progressive muscular atrophy of spinal origin have been observed by Werdnig, Hoffmann and others. The legs and back are first affected and later on the other muscles of the body. The disease advances slowly and terminates in the usual way, even with bulbar symptoms (Werdnig) in about four or five years. It is not yet positively established whether Charcot-Marie's type of hereditary muscular atrophy beginning in the leg is a central, spinal or peripheral neuritic malady. If it is the former, as Dana and others think, it would seem unnecessary to elevate it into a special affection and devote a separate section to its consideration. If it is a neuritic affection it would naturally fall into the chapter on neuritis. In my own opinion it is but a form of the disease we are now considering, and I will so describe it. The causes to which progressive atrophy have been attributed are so numerous that one instinctively doubts the reality of any of them. Nevertheless mention must be made of cold, dampness, exposure, men- tal strain, over-exertion, trauma, infection and intoxication. Perhaps the last two have a stronger claim for recognition than any. At all events the disease is said to have followed measles, typhoid fever, cholera, rheumatism, syphilis and childbirth. The atrophy of lead poisoning is not strictly of the progressive type. This disease has oc- curred as a senile form at the age of seventy. Pathology ano Pathogenesis. — A slow, non-inflammatory, de- generative process in the anterior horns of the spinal cord constitutes the primary pathological basis of the disease. Usually this is most marked in the lower cervical and upper dorsal regions. From these points it may extend upwards and downwards until the entire cord is involved. The large pyramidal cells, especially the central and me- dian groups, are shriveled and wasted, are greatly reduced in number, or are entirely absent. The neuroglia and connective tissue are in- creased ; but there are no distinctive vascular changes, though here and there a blood vessel may be dilated. The processes from the wasted cells are also atrophied where they pass through the white matter out into the anterior roots. Sometimes in the course of the disease, and to a lesser THE NEURONIC DISEASES 395 degree usually, the white matter of the cord, particularly the pyramidal tracts, participates in the atrophic process. All parts of the lateral columns except the direct cerebellar and antero-lateral tracts, and also the antero-lateral ground bundle, may exhibit the atrophic condition. It was thought by Charcot that cases showing atrophy of the pyramidal tracts constituted a separate type of the disease, but in this he was in error, for many cases reveal upon post-mortem examination a consider- able atrophy of the pyramidal tracts, which during life manifested no spasticity, the symptom upon which Charcot largely founded his amyotrophic lateral sclerosis. The latter is only a clinical manifesta- tion of the general disease. In most of the cases there is sooner or later extension of the malady into the medulla. In some cases it seems to start and terminate in the medulla, constituting the type known as progressive bulbar paralysis. When the disease advances from the cord upwards, the homologous motor nuclei show the same general changes as do the anterior horns. Along the pyramidal tracts the degeneration lias been traced even as far up as the cerebral cortex. In the opposite direction the degenerative process has extended out through the anterior roots, into the peripheral nerves and even into the muscular tissue. In the latter the fibres are shrunken and diminished, their striations have disappeared, granulations and fat globules have taken their place and the interstitial connective tissue is increased. The entire muscle therefore is at first soft, then hard, and its appearance is pale, streaked and yel- lowish. Healthy fibres may continue here and there among the dis- eased ones and the small vessels among them may be dilated. A primary degeneration of the entire neuromuscular apparatus with its prominent focus in the anterior horns of the cord and their related structures, the muscles, constitutes the pathological basis of all forms of spinal progressive muscular atrophy. So uniform and con- stant, though not necessarily always to the same degree in all parts of this neuromuscular tract or apparatus, are these pathological changes found that were we to confine our attention solely to their anatomical side, all forms of progressive atrophy would be reduced to the nomen- clature of a single disease. Symptoms. — The moment we begin to attempt a classification of progressive muscular atrophy from its clinical presentations alone w< find that we must speak of different types, though in general the symp- tomatology of all types is about the same. If simple muscular atrophy. with a paralysis running along parallel with it, occurs without an\ other manifestation, we have the pure form of the disease. If spasticity accompanies the atrophy and paralysis, showing considerable involve- ment of the pyramidal tracts, we have Charcot's amyotrophic lateral sclerosis. If the simple muscular atrophy with paralysis appears firs! in particular groups of muscles, we have the Vran-Duchenne, the peroneal, the bulbar, tin ophthalmoplegic and other localized typ In all cases the disease develi ps slowly and insidiously. The tient may be affected for months and years, but not being particular!) discommoded, he either docs not notice the trouble or else fancies thai he is merely a little thinner than he oughl to be. I knew a case where the marked wasting of the upper limbs induced the pinna n r a long 3 ( /> THE NEURONIC DISEASES FIGURE 67. 1 FIGURE 68\ FIGURE 69. Fig. 67. — Chronic bulbar paralysis with amyotrophic lateral sclerosis. Section through the medulla. Fig. 68. — Amyotrophic lateral sclerosis. Section through the lower cervical cord. Fig. 69. — Spinal muscular atrophy. Section through the cervical cordL ( St riimpcll -Jakob.) HE NEURONIC DISEASES 397 -time to the belief that she was generally run down and abnormally thin all over the body, though as an actual fact her lower limbs were plump and normal in size. The disease shows itself first in, and often remains limited to. the •upper extremities. Sometimes one limb is affected more than another. FIGURE 70. Progressive muscular atrophy. Age of patient forty five yean reich.) ( After Fried In the beginning there may be some slight weakness and dull, rheuma toid pain in the muscles of the arm and shoulder; or there ma\ be a mild sort of rigidity causing the finer movements to reveal a traci awkwardness. Usually about this time the muscles have commenced to show some degree of wasting. In the more common Aran Duchenne 39& THE NEURONIC DISEASES type the small muscles of the hand are the first to become atrophied. The balls of the thumb and of the little finger flatten ; the hollows be- tween the tendons on the back of the hand deepen ; the lumbricales and interossei seem to disappear and the fingers are scrawny and prominent at the joints. The entire hand gets flat and the thumb stretches parallel with the fingers, giving it the ape appearance. The adductor longus pollicis is soon involved. Adduction, abduction and flexion of the thumb is extremely difficult. Gradually the fingers are drawn back- ward and are abnormally flexed on account of the extension of the dis- ease up into the arm, thus giving them the well-known appearance of the main-en- griff e. As the disease advances on up the forearm, arm and shoulder it is noticed that the anatomical nerve distribution is not followed, although in many cases the ulnar nerve area is prominently involved. All possible changes are seen in the course of the disease. One arm may become far advanced before the other is attacked. On the other hand, the trouble may appear in the shoulder first, particularly the deltoid muscle, thence pass to the biceps, less frequently the triceps, and so on all the way down the limb. The appearance of the entire arm is very characteristic ; thin, wasted, with the grooves deepened, the bony prominences exaggerated „ it hangs limp and gaunt by the side. Other parts of the body beside the arms may be implicated. In fact, it is a peculiarity of the disease to thus skip about. The entire shoulder girdle may suffer and with it the deep muscles of the back. The legs are rarely affected, and then as a rule only late in life. The muscles of the pelvic girdle, particularly the glutei, the extensors and the abductors of the thigh, are occasionally attacked. The diaphragm becomes involved and ultimately the intercostals. Last of all the mus- cles of the neck and others under the control of the medulla succumb. Involvement of this important part of the brain usually brings the case to an early termination. Many years and almost an entire lifetime may be consumed by this slow progressing malady. Fibrillary twitching usually accompanies the advancing atrophy, together with paralysis. The electrical excitability shows a correspond- ing decrease. On account of the absence or presence of a slight rigidity and stiffness in the muscles two forms of the disease have been referred to — namely, the tonic and the atonic. These, as I have intimated before, are confusing and artificial distinctions. All that is necessary to note in regard to this is that in some cases the pyramidal tracts may be suffi- ciently implicated to provoke a mild degree of spasticity and give the case a resemblance to that still more pronounced, though arbitrarily separated clinical typo, amyotrophic lateral sclerosis. There are no objective sensory symptoms in progressive muscular atrophy and the subjective phenomena, like numbness and other mild paresthesia?, are infrequent and inconsequential. Some authors refer to other symptoms which are either of minor significance or indicate extension of the disease to the medulla. Such are sweating, local congestion and some other vasomotor troubles: THE NEURONIC DISEASES 399 inequality of the pupils from irritation of the ciliospinal center ; weak- ened sexual power ; urinary abnormalities. There is a form of the disease, the Charcot-Marie peroneal type, in which heredity plays a striking role and the atrophy begins in the leg. It appears usually under twenty years of age. The trouble ascends, involving successively the peronei muscles, the extensors of the foot, the calf and ultimately the thigh. After many years even the upper extremity may be involved. The foot and hand frequently escape. Pains are complained of by many patients. This disease for the most part resembles an ordinary progressive atrophy. In other respects the type simulates an ascending neuritis. Hoffmann and others believed it was such and named it the neural type of progressive muscular atrophy. There are many good reasons for not regarding it as primarily neuritic in origin, but rather as poliomyelitic, like all the other spinal atrophies. A polyneuritis in which the foot and hand so uniformly escape would certainly be anomalous, and moreover heredity is not an influential factor in the production of peripheral nerve degen- erations. On the other hand, it is a fact that in all forms of progressive spinal atrophy more or less degeneration does occur in the peripheral nerves or outer ends of the diseased lower neurones. This would account for the diseased nerves observed in this type of the trouble by Yirchow, Friedreich and others. The other symptoms upon which the neural theory of the disease is based, as, for instance, the pains, are too insignificant to have much weight when placed in contrast with the many other manifestations which seem so strongly to ally it to the ordinary progressive muscular atrophy. The work of Dana, Marinesco and others has confirmed the belief that the primary cause of the peroneal type, as in all other forms of progressive atrophy, is a lesion of the anterior horns of the cord. De\gnosis. — In typical cases progressive muscular atrophy is easy of diagnosis if close attention be given to the onset, distribution and slow progress of the atrophy. Muscle groups rather than individual muscles or the whole limb is the predominant area of distribution. The atrophy precedes and, as it were, controls the paralysis. Sensory symp- toms are wanting except to a slight extent in the so-called neural, or Charcot-Marie peroneal type of the disease. A slight muscular stiff- ness and fibrillary twitching are -frequently present. The progress of the trouble is slow, skipping from muscle-group to muscle-group, often with temporary remissions, but always steadily onward. Clinically it is always wise to attempt a differential diagnosis be- tween progressive muscular atrophy, amyotrophic lateral sclerosis and chronic anterior poliomyelitis, though many authors insist upon refer- ring to all three as practically the one disease. In amyotrophic lateral sclerosis the marked spasticity with the com- paratively slight atrophy, the commencement of the trouble in the arms, and the bulbar manifestations, are all highly indicative-. In chronic anterior poliomyelitis the symptoms appear more sud- denly, reach a climax, and then recede, leaving a permanent paralysis in individual muscles. The paralysis always precedes the atrophy and is accompanied early, as a rule, by changes in the electrical reaction-. 400 THE NEURONIC DISEASES At times it must be admitted the differential diagnosis is all but im- possible. In such cases time will show the slow extension of the atrophy to other muscles and parts of the body. When bulbar symptoms come on late to complicate the clinical picture the diagnosis of progressive muscular atrophy is assured. Other diseases from which a differential diagnosis may sometimes have to be made are the muscular dystrophies, peripheral neuritis, anthritic neuritis, cervical pachymeningitis hypertrophica, syringo- myelia, general gliosis, spinal caries and the occupation neuroses. From these progressive atrophy is best differentiated by exclusion. Each one has some characteristic symptom or symptoms that do not be- long to progressive atrophy and by this symptom the separation is made even though the atrophy, the paralysis and the course of the malady are the same. The heredity, the age of the patient, the prominent distribu- tion of the wasting and paralysis in the lower truncal and pelvic muscles, the leg and face, the existence of some hypertrophy or pseudo-hyper- trophy and the absence of stiffness and bulbar manifestations, indicate a muscular dystrophy. Multiple neuritis begins abruptly, spreads upward on the limbs and from the legs to the arms, exhibits ataxia, late atrophy, loss of the deep reflexes, electrical changes and marked local sensory phenomena, such as pain and hyp?esthesia Arthritic muscular atrophy is always in and around the diseased joint and does not extend to the entire limb or pick out special muscles or groups of muscles. The paralytic, electrical and reflex phenomena that accompany the classical form of muscular atrophy do not appear with the arthritic wasting. Moreover, the latter recovers rapidly after the joint trouble has disappeared. In the cervical hypertrophic type of pachymeningitis the symptoms seem to select the median and ulnar nerve areas for their manifesta- tion and there are the usual darting, radiating pains of posterior root irritation. The dissociation of the pain and touch sense, and the other trophic characteristics, besides the muscular atrophy, will help to determine the syringomyelia. Spinal caries is differentiated by the pressure symptoms, the meningitic pains and local tenderness, the spinal deformity and the use of skiagraphy. The history of overuse of a special set of muscles, the limitation of the trouble sharply to those muscles and its tendency to get well under simple rest, or at least its. indisposition to spread, points very clearly to an occupation neurosis. Prognosis. — Being a slow, progressive disease, the prognosis of muscular atrophy is bad so far as a cure is concerned. The lost neural elements, upon which the disease depends, cannot be restored by human power. The cases run on, however, for many years, sometimes remit and become stationary, or in a small percentage undergo what may be termed a partial recovery. The possibility of such nn event can be held out to the patient, but it dare not be promised or anticipated. The more symmetrical in distribution the atrophy is the better the hope for a partial recovery. The more marked the type of the disease is, THE NEURONIC DISEASES 4GI as, for instance, the hand, the leg or the amyotrophic, the more apt is it to be permanent. The condition met with at the end of six months or a year cannot be bettered in any way. Cessation at that point is the most than can be hoped for. The chances are that it will progress and extend. Death usually results from bulbar involvement, general ex- haustion and marasmus, or some intercurrent malady. Treatment. — It must be evident from the description of the nature of this disease that treatment is of little avail. Hygienic and symp- tomatic measures are about all that can be recommended. Over-exer- tion should be carefully guarded against and it is always wise to rec- ommend frequent and prolonged rest. All stimulants, general as well as local, alcohol, tobacco, sexual indulgence, cold baths, vigorous mas- sage, active or passive exercise and electricity had better be refrained from, or used very cautiously. Arsenic and thyroid extract may be tested, but they do not promise very much. Gowers. Sanger Brown and others have tried, with some degree of success, the subcutaneous daily injection of the nitrate of strychnia in doses of about 1-25 grain. The treatment may be kept up for six weeks, then stopped for two weeks, then renewed again, and so on until several courses are taken. It is a waste of time and money for a patient to seek new climates, hot springs and highly advertised sanataria in the hope of a cure. Rest, quiet and good nourishment at home, under the careful observation of his medical adviser, is the safest regime for his adoption. AMYOTROPHIC LATERAL SCLEROSIS. Our knowledge of this disease dates from Charcot's descriptions of it in 1869 and later. Other investigators have added much to this knowledge and cleared up some doubtful points in Charcot's original definition. Clinically the affection is revealed by a spastic paralysis with a distinct muscular atrophy and absence of sensory phenomena. Patho- logically it is a degenerative sclerosis of the upper and lower motor neurones. It is usually classed among the progressive muscular atrophies of spinal origin, because it exhibits all of the symptoms and post-mortem findings of these affections, in which the disease proi is located in the anterior horns, anterior roots, and their extensions out into the peripheral nerves. In addition to this, however, amyotrophic lateral sclerosis is a disease process in the lateral columns of the cord, especially the pyramidal tracts. Charcot believed that the degeneration of the pyramidal tracts was primary, causing a degeneration in the cells of the anterior horns and associated anterior root fibres secondarily. It is more probable, from the latest investigations, that the two pi esses, that in the pyramidal tract and that in the anterior horns, are merely coincident and are dependent upon the same cause. As the degeneration has been traced all the way up to the cortex, it is now the accepted opinion that amyotrophic lateral sclerosis is a degenerative disease of the entire motor path. Etiology.— We know very little about this. Heredity does not seem to enter as a factor, though it i> more than probable that a 1 402 THE NEURONIC DISEASES genital neuropathic condition is responsible to a certain extent for its occurrence in certain individuals. It appears with about the same fre- quency in both sexes, though Charcot and Marie were of the opinion that females were more prone to it than males. It is a disease of mid- dle adult life. Most cases show themselves between the ages of thirty- five and forty-five, though they have been seen to occur at any time between the ages of sixty and thirty. Many exciting causes for the disease have been enumerated by the authorities, chief among which are exposure to wet and cold, mental strain, physical exhaustion, dissi- pation, sexual excesses, traumatism, etc. Syphilis does not seem to particularly predispose to the disease, but it has been observed in syphilitics. The etiology may well be summed up by saying that the predisposing cause, a neuropathic constitution, being present, almost any exciting cause in the way of a shock or intoxication may start the degenerative process in the motor path. Pathology and Pathogenesis. — The latest and most carefully made post-mortem examinations show that this disease is pathologically a degeneration of the entire cortico-muscular conducting path. The degeneration of the peripheral motor neurone is identical with that of the myelopathic form of progressive muscular atrophy. The anterior horns are the only part of the spinal gray matter that is degenerated. The pyramidal tracts, both crossed and uncrossed, reveal degeneration and the disease process has been traced up through the medullary motor paths, the peduncles of the brain, the internal capsule and even into the motor areas of the cortex. In the cord other structures have been found degenerated by Spiller, Mott, Probst and others, though these cases have been exceptional. The dorsomedian columns of Goll were wasted, likewise Clarke's columns and the direct cerebellar tracts. In the medulla the degeneration attacks the motor paths and the motor nuclei and the roots of the motor cranial nerves — namely, the hypoglossal, the facial, the vago-accessorius and the motor fifth. In one or two instances the nuclei of the ocular nerves have been im- plicated. In the brain the degeneration was observed in almost the entire motor cortex, the corpus callosum, the middle region of the posterior limb of the internal capsule, and the tangential fibres and the parts of the centrum ovale anatomically and physiologically connected with the cortical motor areas. The large pyramidal cells were diminished in number and the remainder were pigmented and atrophied. Spiller has detected a degeneration in the peripheral nerves even, side by side with undegenerated fibres. No degeneration was regularly found in any of the sensory paths, in the intramuscular nerve fibres or the muscle spindles. Symptoms. — The typical form of amyotrophic lateral sclerosis exhibits certain cardinal symptoms. These are spastic paralysis and atrophy in the upper extremities, and weakness and rigidity with slight atrophy in the lower; fibrillary tremor in some of the wasting muscles : exaggeration of the deep reflexes; more or less alteration of the elec- trical reactions; certain bulbar symptoms showing involvement of the THE NEURONIC DISEASES 403 cranial nerves from the seventh to the twelfth ; and complete absence of all sensory and mental disturbances. There are types of the disease that vary slightly from this general clinical picture, according to the location and predominence of the lesion and the resulting preeminence of one or more groups of symp- toms. For example, in the majority of cases the trouble first shows itself in the upper extremities and later on in the lower, but there are rases in which the reverse order of events takes place. Occasionally a case will first announce itself by slight bulbar symptoms, a glosso- labio-laryngo-pharyngeal paralysis, the extremities not being implicated until later. In most of the cases spasticity and an exaggerated niyotatic re- sponse precede for a time the muscular wasting and even continue along with it pari passu. There are cases, however, where the disease in the lower motor neurones antedates for some time the disease in the pyramidal tracts, and having, for the time being, all the usual signs of a chronic poliomyelitis or even progressive muscuiar atrophy of the Aran-Duchenne type, cannot be distinguished from the latter diseases. It was for this reason, in a large measure, that Gowers, Leyden and even Charcot believed amyotrophic lateral sclerosis to be a mere form of spinal progressive atrophy, though the last author did subdivide the muscular atrophies of spinal origin into the primary or protopathic variety, in which the disease is limited to the lower motor segment, and the deuteropathic, or amyotrophic lateral sclerosis type, in which the disease is in both the lower and upper segments. In this latter type Charcot taught that the degeneration of the anterior cornua was a sec- ondary result of the degeneration of the pyramidal tracts. A cardinal symptom of amyotrophic lateral sclerosis is spasticity of the muscles. This appears before the atrophy, usually in the arms first, except in those very rare cases where the disease in the lower motor neurones has gone so far in advance of that in the pyramidal tracts that they cannot be differentiated clinically at all from Charcot's protopathic type. Even in those cases where the symptoms show them- selves first in the lower extremities there will be found upon careful examination a slight degree of rigidity, with weakness in the arms and hands. In all typical cases, therefore, of amyotrophic lateral sclerosis the arms will be observed to be strongly adducted, the forearm flexed, the hand pronated and the fingers more or less fixed in the claw position. In the legs the stiffness is shown in the walk. The patient steps slowly. more or less rigidly, and with short strides. Later on he seems only to be able to push himself along on the tips of his toes. This spasticity is the result of muscular tension, active contraction and atrophic paralysis. Equally important as tin- spasticity in the symptomatology amyotrophic lateral sclerosis, though not synchronous with it, is the progressive atrophy of the muscles. Individual muscles and groups muscles may be attacked, simultaneously or one after the other, accord- ing to the location and extent of the lesions, ft would be useless attempt to describe all of the types of atrophy that can appear in the 404 THE NEURONIC DISEASES course of this affection. Suffice it to say that the wasting of the mus- cles, which usually is observed from a few months to many years even after the appearance of the spasticity, is never quite as marked as it is in the ordinary forms of anterior poliomyelitis or progressive muscular atrophy, and that furthermore it is more pronounced in the upper than in the lower extremities. In these it begins most frequently in the hand, gradually involving the shoulder and radial regions later. The muscles of the lips, the tongue, the jaw and the face may all become FIGURE 71 Attitude of the hand in amyotrophic lateral sclerosis. (After Charcot.) stiff and atrophied. The trunk muscles, as a rule, escape or are not so profoundly implicated as are those of the extremities. In the final stages of the disease the diaphragm, the internal muscles of respira- tion, and even the visceral muscles may all become more or less affected. At an early period the deep reflexes are exaggerated, as, for exam- ple, those of the quadriceps, gastrocnemius, patella, Achilles, biceps, tri- ceps, scapular. A masseter clonus, elicited by gently tapping the edge of the lower jaw, was first studied by Beevor and Morris Lewis. The Babinski sign may be present, and later on contractures with threat- ening cardiac and respiratory phenomena. Early in the disease the electrical reactions are not far from the normal. As the atrophy ad- vances, however, the reflexes diminish correspondingly and the elec- trical phenomena assume irregularly the characteristics of the reaction of degeneration. At times a characteristic fibrillary tremor or twitching may appear in advance of the atrophy or it may even form the first objective symp- tom of the disease. The motor weakness that is present is not the result of the atrophy and contractures, but is an independent phenomenon. That amyotrophic lateral sclerosis is a disease of the efferent or motor path is clinically shown by the complete absence of all sensory Symptoms. In a few cases a slight paresthesia has been noticed. A THE NEURONIC DISEASES 405 primary degeneration of the columns of Goll, with slight sensory phe- nomena, has been recorded by Hektoen ; and Mills has reported a case of associated tabes and amyotrophic lateral sclerosis. Such cases, while constituting the link between this disease and progressive muscular degeneration on the one hand, and locomotor ataxia on the other, should probably be looked upon as instances of combined sclerosis, rather than as examples of amyotrophic lateral sclerosis with a mere complication. Likewise there are no mental symptoms in this disease, though Spiller, Probst and Sarbo have recorded instances in which some mental disturbance was present. These were probably mixed cases and may well be accounted for by the wide extension, in a few individuals, of the degenerative process. Amyotrophic lateral sclerosis as a typical disease is therefore clinically a motor degenerative paralysis, and is characterized only by the symptoms spasticity, atrophy and paresis. Late in the disease, and usually as its terminal phenomena, bulbar paralysis occurs. Dysarthria proceeds to anarthria. Deglutition be- comes difficult and even impossible. The lips, the tongue, the palate and the cheeks are paralyzed, atrophied and electrically altered. Drool- ing from the mouth occurs, the speech is blurred and 'Tailing" in character, and aphonia supervenes. Finally after the disease has run its course in from two to four years, the end comes as the result of inanition, asphyxia or aspiration pneumonia. Diagnosis. — A typical case of amyotrophic lateral sclerosis is not hard to diagnose, for it presents a strikingly characteristic group of symptoms — namely, spasticity, paresis, atrophy and absence of all sen- sory and mental phenomena. When the disease process in the peripheral motor spino-muscular system precedes for some time the disease process in the central cortico-pyramidal system, it may be impossible to differentiate it clinically from the myelopathic type of progressive mus- cular atrophy. In such cases, however, some slight degree of spasticity can be detected upon careful examination in some muscles later on that have escaped the first onset of the malady. Cases in which other de- generations are associated with those of the amyotrophic may give much trouble in the diagnosis. A most careful weighing and compar- ing of the various symptoms of the associated affection with those oi Charcot's disease can alone render a differentiation at all possible. Bearing in mind the characteristic traits referred to elsewhere, amyo- trophic lateral sclerosis, with its peculiar features, ought not to cause much trouble in differentiating it from progressive muscular atrophy, bulbar and spinal chronic poliomyelitis, syringomyelia, focal spinal pachymeningitis or cervical hypertrophic pachymeningitis, multiple neuritis, the cortical encephalitis of Strumpell, disseminated sclerosis. the muscular dystrophies or diffuse myositis. Prognosis. — This is bad, as the disease invariably terminates fatally in a few years. Treatment. — The treatment is limited and unsatisfactory. Tonics. fresh air and good nutrition should, of course, be given. Though Gowers recommends injections of strychnine, and others have suggi electricity, a trial of the je measures has not seemed to materially modify 406 THE NEURONIC DISEASES the course or termination of this progressive and profound degenera- tion. PROGRESSIVE BULBAR PARALYSIS. This disease was first described by Duchenne in i860, and ten years later its lesion was accurately located by Charcot and Leyden. It is sometimes called progressive glosso-labio-laryngeal paralysis. It is a primary, degenerative disease of the bulbar cranial nerves of motion. These nerves are the analogues of the spinal motor nerves, and their nuclei in the floor of the fourth ventricle are the homologues of the anterior horns of the cord. This form of progres- sive, primary degeneration in them, therefore ; is nothing more nor less than another view of progressive muscular atrophy. That this is so is further proved by the fact that on the one hand bulbar paralysis almost constantly appears sooner or later in the poliomyelitic type of progressive muscular atrophy and the amyotrophic type, and on the other hand degeneration in the pyramidal tracts and in the spinal an- terior horns is almost invariably found in the late cases of this dis- ease. In other words glosso-labio-laryngeal paralysis of the progres- sive type is progressive muscular atrophy prominently appearing in the medulla oblongata. For this reason I classify and describe it here rather than under the head of diseases of the medulla. It is not a dis- ease of the medulla in the true sense of the word. It is merely a phase, a unique manifestation, of progressive muscular atrophy. The symp- tomatology of it may suggest an independent classification, but its pathology binds it down as one of the unique localization types merely of the general disease of the entire motor tract known as progressive muscular atrophy. Only in symptomatology therefore does bulbar paralysis of the progressive type stand forth independently. Etiology. — What has been said of the etiology of the two pre- ceding affections is strictly applicable here, for all three diseases are one and the same. It is rare and occurs mostly in late adult life. It is all but un- known before the fortieth year of age. In a few instances it has been reported as having occurred in children, though there is some doubt about the exact pathological similarity of these cases with the typical disease. I do not regard the infantile hereditary and familial types of progressive bulbar paralysis as corresponding exactly with the type now under consideration. They are more strictly bulbar diseases, con- genital nuclear atrophies, and not a mere part of a general progres- sive motor atrophy. They will be referred to again under the head of diseases of the lower motor segment more particularly. As in the other manifestations of the disease, a neuropathic tend- ency is here present, responding to such exciting causes as cold, exces- sive emotion, shock, traumata and over-exertion of the muscles, such as those of the lips, tongue or larynx, that are innervated by the me- dulla. Pathology and Pathogenesis.— To describe this I would but have to repeal what 1 have said under the head of progressive muscu- THE NEURONIC DISEASE! 407 lar atrophy. The changes are the same, but in this instance they have started first in the nuclei of the motor cranial nerves, namely, the facial, the motor branches of the glosso-pharyngeal and vagus and the hypoglossal. The spinal accesssory, of course, is a spinal nerve in reality so that it escapes in bulbar paralysis, and the limited nosol- ogy does not include implication of the ocular motor nuclei. The motor branch of the fifth nerve is occasionally included in the disease process. The pyramidal tracts may atrophy in some cases before the gray nu- clei, or accompany it or be entirely absent. The same is to be said of the degeneration in the anterior horns of the cord. Symptoms. — It is due almost entirely to its symptomatology that progressive bulbar paralysis owes its distinction as a separate sort of a FIGURE 72. Normal hypoglossal nucleus. Atrophic hypoglossal nucleus in bulbar paralysis, g, blood-vessel. (After Oppenheim.) disease. A progressive, atrophic paralysis involving both sides oi the face and destroying the speech, the deglutition, the phonation and the mastication constitutes the sum total of the clinical manifestations of the disease. Usually the speech is the first function to be affected. Words and sentences with many dentals and Unguals and linguo-palatal letters in them arc- hard to utter and soon tire the patient. He cannot close the lips as tightly as he should to utter the explosives p and 1). ( In account of the added nasal tone, he sounds these letters as it" they were nip or nib. To say smoothly the sentence. "Peter Piper Picked a Peck of Pickled Peppers/' or "Round the Ru{ ck the Ragged Ras cal Ran," is almost impossible. Dysarthria with a nasal twang is thus an early and characteristic manifestation. There is a suggestion in the speech as if something were in the month, so much so thai a mother in a certain case constantly asked her daughter for a long time to take what she had in her mouth out before she addressed her, much to the annoyance of the daughter, who of course, had to regularly deny the allegation. Exhaustion increases the speech difficulty. 408 THE NEURONIC DISEASES Next the tongue is seen to be paralyzed. It rests limp, shriveled and immovable on the floor of the mouth. The mucous membrane on it is thrown into folds. At first there are marked fibrillary twitch- ings and tremors in its muscle. The lingual muscles are markedly atrophied. On account of all this the food bolus cannot be pushed back into the pharynx and deglutition thus becomes positively im- paired. There is regurgitation of fluids up into the nostrils and down into the larynx. This of course provokes choking and coughing. The saliva accumulates and drools out of the mouth over the edges of the paralyzed lips. Vomiting not infrequently occurs as a result of the disturbances of and efforts in swallowing. At length there is com- plete aphagia, or inability to swallow any solid or fluid. Later on in the disease mastication is difficult and sometimes im- possible. The involvement of the laryngeal muscles eventuates in first dysphonia and finally aphonia, the respiration suffering at the same time. There is in the early stages a weakening, a softening, a monot- ony of our 4T2 THE NEURONIC DISEASES FIGURE 73- Juvenile 'y]' e T /f e Muscular atrophy. THE NEURONIC DISEASES 413 FIGURE 74. /Arwyolrovh J rp ' '/r e T yr c Muscular atrophy. 414 THE NEURONIC DISEASES bo THE NEURONIC DISEASES 415 knowledge we must be content to regard them as primary, develop- mental defects in the muscular elements. And yet when the sym- metrical distribution, which not infrequently obtains, is taken into ac- count, when it is remembered that in many cases neural changes are observed which cannot positively be asserted to be always secondary to the muscular changes, and when finally the close biological and physiological relation between the nervous and muscular apparatuses is considered, it is not so unreasonable a suspicion to hold that even the muscular dystrophies may be due to some minute physio-chemical nutritive disturbance in the spinal nerve cells, which our present means FIGURE 78. FIGURE 79. Pseudo-hypertrophic paralysis. (After Duchennc. ) of research cannot detect, but which may be the immediate cause of the muscular deterioration. This of course is only a conjecture and must not yet be assumed as true. It is so much more desirable a hypothesis in accounting for the many similarities between the inns cular dystrophies and the progressive muscular atrophies than is the hypothesis of a primary muscular degeneration in accounting for their few differences, that it is worthy of much consideration and if pos- sible of future establishment by positive proof and demonstration. Symptoms. — Instead of symptoms, one might truthfully say that the symptom of this disease is hypertrophy or atrophy of the muscles. All muscles of the body may be affected, though the change appears 4i6 THE NEURONIC DISEASES more frequently in some than in others. Both hypertrophy and atrophy may appear simultaneously in different, and even in distant, muscles, or in different parts of the same muscle. There is no rule about the matter and the pathology cannot be made a basis for the separation of the malady into types. On account of these muscular alterations certain forms of paralyses occur, deformities appear, and alterations in the bodily movements take place. There are no distinctive sensory symptoms. With the atrophy a parallel paralysis, loss of reflex action and slight electrical changes take place, but none of these are as pronounced FIGURE 80. Attitudes assumed in pseudohypertrophic paralysis during attempts to rise. (Gowers.) or characteristic as they are in the true neural atrophies. Coldness, discoloration and other general vasomotor disturbances are liable to appear in the affected limbs. The general nutrition remains normal. The sphincters continue active. The sexual function is diminished or lost. The mind remains clear. I will now briefly consider the varieties of the disease that have been artificially established on the basis of the particular distribution of the predominant symptom. In doing so I will at the same time be giv- ing a more or less complete clinical picture of the entire affection. Pseudohypertrophic Muscular Paralysis (Muscular Pseudo-hyper- trophy; Lipomatous Muscular Atrophy). In this form of the disease there appears in the child usually a weakness, followed by enlargement of the calves of the legs. A peculiar waddling gait is thus noticed and there is some difficulty experienced in climbing the stairs. Some- times the child falls unexpectedly, and has considerable difficulty in THE NEURONIC DISEASES 417 getting up. It is noticed that when laid upon his back on the floor he first rolls over on his face, draws himself together, keeping his hands and knees upon the floor, and then with a hand on each of his legs climbs up upon himself, sliding his hands, and grasping his legs one after the other at successive levels, until he has attained the erect position. This manceuvre in getting up is so characteristic that it is almost pathognomonic. It is well illustrated in the accompanying pic- tures from Gowers. As the disease progresses the extensors of the leg and the flexors of the thigh become so weak that the attitude grows characteristic. The spine sags forward with a marked concavity open- ing backwards. This lordosis is always seen when the patient is stand- FIGURE 81. Progressive myopathy. Advanced case with marked Lordosis in >ittin.u. Souques-Brissaud.) (After ing and sometimes when seated. In certain mild cases it may disappear in the sitting posture. It is a feature of tin- lumbar part of the spine and causes a marked protrusion of the abdomen forwards and <>t" the shoulders backwards At this Stage of the disease the legs are usually enlarged, ofttimes remarkably so, and are barrel-shaped. The muscles about the hips and in the lumbar region exhibit considerable atroph) with here and there a curious lipomatous-like swelling. The from of the thigh has bulged in this way and one hi]) has been more prominent than tile Other. After tile teg and lumbar muscles, perhaps the shi der and upper arm muscles, are the mOS( frequently implicated, infraspinatus the deltoid, the lower part of the trapezius, the pert. • major, the serratus anticus major, the rhomboids, the biceps, the brachi- 418 THE NEURONIC DISEASES alis internus and the supinator longus may all or any one of them be weak and paralytic. Alternations of hypertrophy and atrophy may be observed in them. The attitude and movements of the shoulders, the elevation of the arms, and the stationary position of the scapulae are all distinctive. In the lower part of the body the glutei, the latissimus dorsi, the quadriceps femoris, the adductors, the calf and peroneal mus- cles exhibit the characteristic change and debility. The tongue in rare instances is hypertrophied. The distal parts of the extremities, par- ticularly the forearms, the neck and face, are rarely included in the disease process. Thus we note that pseudo-hypertrophic paralysis is a widespread affection, though the changes in the leg muscles and the gait are the most constant and obtrusive symptoms. In the Juvenile type of Erb, the scapula-humeral form of the dis- ease, the dystrophy, as its latter name indicates, is most prominent at first in the muscles that make up the shoulder girdle. The same gen- eral process takes place that has already been described for all forms of the disease. Gradually it extends down the arm and very late in the course implicates the forearm and the legs. The beginning of the case is the only feature in which it differs from the other types. As it is slow and progressive it ultimately comes to have all the general quali- ties in regard to the muscular hypertrophy and atrophy, the gait, and the curvature of the spine that they have. The Facio-Scapulo-Humeral type of Landouzy and Dejerine is often spoken of as the infantile form because it so often appears earlier than the others. It may, however, show itself at the same age. The implication of the face with the shoulder and arm is its dominant trait. The weakness and wasting are particularly observable in the orbicularis palpebrarum and the orbicularis oris, so that the eyes cannot be closed completely and the lips protrude tapir-like. The involvement of the buccinators causes difficulty in eating and chewing, and a puffing of the cheeks in talking. The disease of the risorius and zygomaticus major renders smiling all but impossible. As the face in these cases is usu- ally where the trouble manifests itself first, the patients not infrequently declare that from early childhood they have been told by their friends that they never smiled or closed their eyes as they should. So slow and insidious is the extension of the disease down into the shoulder girdle that many of its victims fatuously imagine' they are merely constitu- tionally thin. Ultimately these cases, like those of the other types, develop the same general appearances in other parts of the body. The myopathic face with non-implication of the ocular muscles or the muscles of mastication and deglutition, and the prominence of the atrophy in the shoulder and arm, is the sign-manual of this infantile form of muscular dystrophy. There is a markedly hereditary form in which the symptoms begin in the lower part of the back and legs. It is not sufficiently unique, however, clinically or pathologically, though it is slightly so etiologi- cally, to warrant its further detailed description. Diagnosis. — As our knowledge will increase, it is not improbable that these muscular dystrophies will be seen to be closely related to, I THE NEURONIC DISEASES 419 FIGURE 82. Muscular atrophy. 420 THE NEURONIC DISEASE9 if not identical with, the progressive muscular atrophies. For the present, however, it is safe and convenient to recognize a distinction between the two, for in a general way the dystrophies possess certain preeminent traits apart from those of the progressive spinal atrophies. They may be roughly enumerated as pronounced heredity, early onset of the symptoms, a peculiar and characteristic distribution of the mus- cular changes, the simultaneous association of atrophy, hypertrophy and pseudo-hypertrophy , an absence of fibrillary twitchings and of the electrical reactions of degeneration. Many of the dystrophies do not conform absolutely to all of these requirements, while, on the other hand, occasionally a case of pro- gressive muscular atrophy assumes a remarkable likeness to a dystrophy. These enumerated traits, nevertheless, are important in aiding in the drawing of the distinction. There are a number of diseases in which muscular atrophy occurs which must not be confused with the trouble now under consideration. Chief among them are multiple neuritis, anterior poliomyelitis, pro- gressive muscular atrophy and cerebral atrophy. In multiple neuritis the abrupt onset of the malady, its rise to a climax and its tendency to subside, the marked sensory phenomena, the ataxia, the disproportion between the paralysis and the atrophy, the absence of heredity with the presence of infection or intoxication, will readily enable one to make the correct differential diagnosis. Anterior poliomyelitis exhibits more or less febrile phenomena, is acute or subacute, is not progressive, but rather regressive up to a certain point, and is devoid of practically all of the distinguishing traits, except perhaps the atrophy of the dystrophies. Moreover the electrical reactions are marked in poliomyelitis. The cerebral atrophies are not typical as are the spinal or the dys- trophic. They are monoplegic or hemiplegic in distribution, are pre- ceded by the paralysis, possess all the usual signs of hemiplegic de- generation of the upper motor tract, and are notably wanting in the characteristic traits and mode of development of the dystrophies. Hysteria, epilepsy, idiocy, tabes and poliomyelitis may complicate a dystrophy and so render the diagnosis difficult, especially if the appear- ance of the muscles is not very different from the normal, on account of the nice balance between the atrophy and the hypertrophy. Prognosis. — The course of the disease is apt to be a prolonged one, with periods of quiescence and even of remission. In the late cases, such as occur among the juvenile types, there may even be partial re- covery. Once started, the disease never disappears. Most of the patients die young from implication of the muscles concerned directly in the maintenance of the vital processes, as, for instance, the dia- phragm ; also from general marasmus and intercurrent affections. Treatment. — The treatment is of no avail. General hygienic and symptomatic measures exhaust the entire theme. Exercises must be moderate and the mechanical treatment with baths, electricity and massage must be undertaken very cautiously and tentatively. Tenotomy and other orthopaedic measures may lighten the existence of some of these patients. THE NEURONIC DISEASES 42 1 b. DISEASES OF THE UPPER MOTOR SEGMENT. SPASTIC PARAPLEGIA OF ADULTS. For our knowledge of this affection we are indebted chiefly to Seguin, Erb and Charcot. They named it respectively tetanoid para- plegia, spasmodic spinal paralysis and spasmodic dorsal tabes. It is better identified clinically than it is pathologically. While much differ- ence of opinion still exists, it will nevertheless be well to recognize it as a clinical entity and to await further data in regard to its exact pathology. There is an infantile and an adult form of the disease. Most of the infantile cases, commonly referred to as Little's disease, are really not paraplegic, but hemiplegic. They are cerebral double hemiplegias, or cerebral diplegias due to vascular disease and will be found discussed under the . head of extra-neuronic brain diseases. A pure, primary spastic paralysis of spinal origin in childhood is a great rarity, if it oc- curs at all. Van Gehuchten, Souques, Raymond, Erb and Dejerine all believe that it does exist. Questionable as are the adult and infantile forms of pure primary spinal spastic paralysis, still more questionable are the hereditary forms. The assumption is made in regard to all forms that there is a degen- erative sclerosis limited entirely to the pyramidal Irwts. In a large number of cases both the clinical and pathological evidence is not- wanting, some time or other, to show that the lesion is not so well de- fined. The elasticity of the name lateral sclerosis, which is occasionally employed to designate the disease, gives it an advantage over spastic spinal paralysis, which represents only one symptom and presupposes a narrow and doubtful pathology. Etiology. — The cause of this disease is obscure. This applies to the hereditary as well as to the acquired forms. In the hereditary form several members of a family may be attacked and the symptoms first appear at about the age of five. Nothing is known as to the condition of the ancestry in these cases. It is not improbable that in the acquired forms there may be a slight neuropathic heredity, whereby certain neurones are rendered less able to resist active influences of a deleteri- ous sort. These cases are first observed between the ages of twenty and forty, and in the majority of instances follow sonic sort of intoxi- cation. Syphilis has been credited. I have considered thi> syphilitic form of the disease under the head of spinal syphilis and have there stated my doubt as to its existence. Trauma was thought to have- been the cause in some cases. From the etiological as well as from the pathological side, the more extensive character of the disease than what is assumed in the mere sclerosis of the pyramidal tracts is hinted at; for while most cases reveal upon post-mortem examination ap- pearances that suggest secondary degeneration from transverse myelitis, multiple sclerosis, or cerebral disease, thes< conditions are just the ones to be initiated by the etiological factors to which spinal spastic paralysis is frequently attributed— namely, trauma, the puer- perium, the infection.-, lead intoxication, poisoning from different 422 THE NEURONIC DISEASES forms of lathy rus, etc. Westphal was of the opinion that only in dementia paralytica did such a strictly limited primary sclerosis of the pyramidal tracts ever occur as is assumed in this disease. From the little that we know as yet in regard to its etiology, spastic spinal paralysis in its pure form is probably a result of a general agenesis and therefore is more or less hereditary; or it is the residual secondary degeneration from other and more diffuse diseases of the brain and spinal cord. In a word, the etiology such as we know it to-day does not favor the view that spastic spinal sclerosis is a limit- ed primary degenerative sclerosis of the pyramidal tracts. Pathology and Pathogenesis. — Not much need be said on this score, since we know so little positively. Elsewhere I have shown that the degeneration of the pyramidal tracts noted in Little's type of spastic paralysis is a double hemiplegia and is therefore a cerebral trouble pri- marily. In these as well as in the cases due to a general hereditary agenesis, the cord lesion may be sharply limited to the pyramidal tracts, but it is a secondary manifestation dependent upon brain defects. In spinal spastic paralysis it is presumed that there is a primary degenerative sclerosis, limited solely to the pyramidal, and especially to the crossed pyramidal, tracts. Most of the cases of this slow and long running disease that come to autopsy, show lesions elsewhere than those of these tracts. There is always an open question, therefore, as to the priority, importance and inter-relationship of these various lesions, in their bearing upon the pathogenesis of the particular dis- ease under consideration. It is this that casts a doubt upon the exist- ence of the pure type of spastic spinal paraplegia. It is but just to say, however, that in a few cases examined by Dejerme and Sottas, Striim- pell and others a degenerative sclerosis was found, confined absolutely to the pyramidal tracts. Whether this was merely an advanced lesion in a more general disease process, and whether, if the patients had lived longer, they would have developed other areas of sclerosis as a part of the same general disease process, no one can yet affirm or deny. Symptoms. — The symptoms of the hereditary and acquired forms of the disease are the same, assuming the above limited pathology. In most of the so-called hereditary forms the secovdary nature of the spinal trouble is intimated in the frequent presence of cerebral symp- toms and other indications. In the classic type of the disease the only symptom is the spastic paralysis with its usual accompaniments of muscular rigidity, rctlc.v exaggerations and contractures. This symptom appears gradually, passing through an early stage of weakness; then progressing steadily, it continues to be the sole manifestation to the end. It appears first and advances farther in the legs than in the arms. Often it remains in the lower limbs. Sometimes it implicates one leg more than the other. The extremity feels tense and stiff at first, as if the tendons were con- tracted or were too short. Rapid movements become difficult. Grad- ually the gait assumes a characteristic manner. Rigidity is observed in all passive as well as active movements. The reflexes now manifest a high degree of exaggeration/ The slightest tap on the patella tendon evokes a violent jerk. 'Hie cutaneous reflexes are also greatly height- HE NEURONIC DISEASES FIGURES 83 AND 84. 423 FIGURE 83. Spastic paraplegia of an adult. Striimpell'j medulla. Fig. 84, cfhrical section. Fig. 85, mid section. ( After Jak< b. I 424 THE NEURONIC DISEASES ened. The muscles are undiminished in size and the electrical responses remain normal. Gradually a distinct spastic paresis comes on until walking is practically impossible, or only possible with short, laborious sliding steps on the balls of the toes. The legs incline toward strong adduction and the progression becomes "cross-legged." With all this serious motor trouble in the legs, and sometimes even to a less degree in the arms, there are absolutely no sensory symp- toms, no trophic manifestations, no involvement of the sphincters. In fine, there is nothing abnormal about the patient but this one manifesta- tion, spastic paralysis, though this is. so protean that it represents a most profound sort of disability. Late in the disease, even after many years, the sphincters sometimes become involved, sensory symptoms appear and even ocular troubles supervene. It is a question then whether such manifestations do not remove the case at once from the classical type of primary spastic paralysis. They certainly would if they showed themselves earlier. The diagnosis of primary spastic paralysis is always, therefore, a provisional one, and at best is always questionable. The disease may unmask itself at any time and reveal the symptoms of another trouble, such, for instance, as multiple sclerosis, myelitis, amyotrophic lateral sclerosis, vertebral tumor or hydrocephalus. As a clinical entity the spastic-paretic-symptom-complex may last very many years and progress so slowly as to interfere for a long time but slightly with the patient's usual avocation. It may even come to a complete standstill. The mind is never affected. The infantile cases in which epilepsy, mental deterioration and other cerebral symptoms occur do not belong here. They fall into the group of the cerebral palsies of childhood. Diagnosis. — The diagnosis of spastic spinal paralysis is essentially the diagnosis of a mere symptom-complex. It is easy in typical cases. In atypical cases it must include the larger disease of which the spastic paraplegia is but one indication. Elsewhere under the heads of myelitis and other cord diseases, and the various cerebral troubles in which secondary spastic paraplegia may occur as a residual condition, the diagnosis will be touched upon. Spastic paraplegia sometimes is witnessed in hysteria. It is rarely, however, the only symptom. As a hysterical concomitant it appears abruptly and is subject to psychical influences. Care must be exer- cised not to mistake a spastic paraplegia for the hysteroid manifesta- tions that may occur sometimes in incipient disseminated sclerosis. There is a treacherous similarity at times between multiple sclerosis and hysteria. Spastic paraplegia, organic in the one case, psychic in the other, is not an uncommon phenomenon in both. Further details in regard to the differential diagnosis may be gathered from the discus- sion elsewhere of these two affections. Treatment. — The treatment of the disease is an ungrateful task. Care as to the nutrition, avoidance of exhaustion, massage and gym- nastic exercises constitute about all that we can do. Contractures and deformities are to be managed on general orthopaedic principles. The younger the patient, so much the more promising are the* results to THE NEURONIC DISEASES 425 be expected from these general measures. These patients often enjoy excellent general health and live many years. They must be taught to exercise patience and to make the best of a very chronic state of invalidism. Medicines are of no avail, and as there is little real suf- fering beyond the inconvenience of locomotion, symptomatic treatment is rarely called for. SPASTIC PARAPLEGIA OF CHILDHOOD. LITTLE'S DIS- EASE. The infantile form of spastic paraplegia, as usually described in the books, is a double hemiplegia. It is a question whether such a name as paraplegia cerebralis spastica should not be dropped entirely. A paraplegia has come to mean technically a spinal cord trouble, there- fore to speak of a cerebral paraplegia is not only misleading, but is a contradiction of terms. Xeither symptomatica! ly nor pathologically are these diplegias of childhood spinal cord affections. Including Little's disease, they are, so far as the spinal changes are concerned, merely secondary degenerations of cortical origin. They bear a symptomatic resemblance, to be sure, to the primary spastic paraplegia of adults. The reflexes are highly exaggerated, but the spasmodic jerk is not so vigorous as it is in the adult disease. Foot clonus may sometimes, but very rarely, be obtained. In some cases the muscular power is not much diminished, but walking is quite difficult on account of the contvacturcd condition of the calf muscles, the strong adduction of the legs and the tendency in the gait to the "crossed-legged progression." There is no true muscular atrophy, though the legs do not develop and grow as they should. Some of the cases may resemble Charcot's amyotrophic lateral scler in its early appearance, and then exhibit a certain amount of true mus- cular atrophy. Amyotrophic lateral sclerosis is an adult disease, how- ever, rarely, if ever, occurring before ten years of age ; it involves the arms most extensively; and is distinguishable from infantile spastic paraplegia by other signs. It was the more or less limitation of the paralysis to the lower limbs, and the absence of all cranial symptoms, that led the earlier authorities to attribute the symptoms and the entire disease to the spinal cord. In the arms, however, there may be observed choreic phenomena and usually a considerable degree of athetosis. These arm symptoms are slight in the cases thai may fairl\ be denom- inated "spastic paraplegia." The entire question as to the classification of these cases depends upon the primary or secondary nature of the spinal cord changes. If the cord changes are primary, a condition which would be ver\ ceptional and in my own opinion extremely doubtful, there is no need of creating a new disease i ul of what would then be an ordinar) pri- mary spastic paraplegia, merely on account of the age of the victim. Granting that Van Gehuchten, Souques, Raymond and Erb arc justi- fied in their belief that a primary infantile spastic paraph cord origin does exist, and that, as Dejerine has found, there is evidence of cervical degeneration, with secondary degeneration of the pyramidal 426 THE NEURONIC DISEASES tracts, occurring during fetal life, the point is still more strongly emphasized that there is no need of writing a new section, and describ- ing these infantile cases apart from the general description of the ordi- nary or adult form of the trouble. On the other hand, if the cord changes in these cases are secondary to the cortical lesion, as shown by the frequent epilepsy, cranial nerve palsies and psychical disorders in addition to the pathological findings, there is again no need of elevat- ing the condition into one of a special and unique form of disease. It is then but a particular manifestation of double hemiplegia, a diple- gia, and falls naturally into the discussion of brain diseases. SECONDARY SPASTIC PARALYSES. The many hemiplegic, quadriplegic, diplegic and paraplegic condi- tions that follow intracranial and intraspinal lesions arising in the non- neuronic tissues are not strictly to be considered here, for the degenera- tion is of the secondary or rather Waller ian type, and the primary disease which is really of extra-neuronic origin is the prominent feature. Thus- inflammatory conditions, neoplasms, compressions, vascular lesions of all sorts are commonly followed by degeneration of the descending type in the neurones caught in the damaging processes. This degeneration Is, as it were, a sort of a sequel of the disease. It has its own prognosis and treatment, but they are not the prognosis and treatment of the disease in which they occur. These secondary neuronic degenerations will be considered in detail elsewhere in connection with the diseases out of which they spring. Sometimes it is very difficult to distinguish the primary spastic paralyses from the secondary. A sharp distinction between the two. thus made in the classifications, will, it seems to me, help toward the making of the differentiation in the diagnosis. At all events it will emphasize the differences between the prognosis and the treatment of the two sets of diseases. Primary neuronic degenerations are apparently diseases sni generis and are hopeless in their outlook and unsatisfactory in their treat- ment. Secondary neuronic degenerations are the sequels of diseases which are hopeful and satisfactory to treat in spite of the hopelessness of the secondary results. C DISEASES OF THE LOWER .MOTOR SEGMENT. The degenerations involving the lower motor neurones are in- numerable, but they are both primary and secondary in origin. The former we know less .about than we do of the latter. All severe lesions that damage the cranial nuclei, the anterior horns of the cord, and the peripheral nerves may cause the neurones in their vicinity to undergo a progressive degeneration. In this way the polioencephalitic, polio- myelitic and peripheral neuritic diseases are all more or less followed by degenerative paralysis. This paralysis, however, is virtually a THE NEURONIC DISEASES 427 sort of a sequel of the disease. It may be very prominent and demand its own consideration, as in acute anterior poliomyelitis, for instance. but it is not the disease itself. Its prognosis should not be confounded for a moment with the prognosis of the affection out of which it has sprung. It, of course, like all neuronic degenerations, is systemic in its distribution. I see no reason, however, for employing it as a basis for the classification of the disease. It is a false classification, it seem- to me, that includes among the system diseases chronic poliomyelitis and not cerebral embolism. It would be a still more unreliable classifi- cation that would consider either as a systemic neuronic disease. Poliomyelitis is an inflammation with certain secondary neuronic de- teriorations, even as cerebral embolism is a vascular trouble with like secondary neuronic wasting. Of these secondary atrophies, the one is below in the spinal anterior cornual neurones, the other is above in the cerebral cortical neurones. All such degenerations should be considered merely as one of the results of the primary disease and should in no way be made the basis of their classification. It is frankly admitted that there are many degenerative conditions in the lower motor segment that we cannot yet affirm positively to be either primary or secondary. Most of them are undoubtedly secondary. The few that seem to be primary will be briefly referred to here. OPHTHALMOPLEGIA. The nuclei of the motor nerves of the eye, the third, the fourth and the sixth, lying along the aqueduct of Sylvius are the homologues of the anterior horns of the cord. They contain the cell-bodies of the neurones that innervate the ocular muscles, just as the horns contain the cell-bodies of the neurones that pass out of the anterior roots and innervate the muscles of the body. These nuclei undergo the same sort of degenerative and inflammatory processes that the horns do and, like the latter, they can give rise to an atrophic paralysis that may pick out individual ocular muscles. This is termed Ophthalmoplegia, which for clinical convenience is divided into internal ophthalmoplegia, when the internal ciliary muscles and iris are involved, and external ophthalmoplegia when the external recti, the oblique and palpepral muscles are implicated. A bilateral external ophthalmoplegia, with intact ciliary, iris and lid muscles, is pathognomonic of a nuclear lesion, for no peripheral nerve trouble could hardly present such ;< clinical picture. The nuclear disease max be aeute and vascular. It would be described then ;is .1 particular form of polioencephalitis, often as ;i part of Wernicke's type of acute hemorrhagic polioencephalitis superior, mentioned elsewl under inflammations of the brain. Infection and intoxication are die chief causes of acute ophthal- moplegia. This form of the disease max or ma) noi have a discover able pathological change as its anatomical basis, according to the virulence of the toxin and the duration of its action. The prognosis is noi very favorable in tin alcoh 428 THE NEURONIC DISEASES recoveries often occur in the influenza and other toxic cases. This,, of course, is a secondary degenerative process. Chronic ophthalmoplegia is a slow, progressive degenerative dis- ease which aligns itself easily with the progressive muscular atrophies. In fact, it is not infrequently the precursor of various degenerative system diseases, such as tabes, progressive bulbar paralysis, progres- sive muscular atrophy, etc. It occurs also as a part of the combined diseases of the cord, of multiple sclerosis and of progressive paralysis. It is associated with psychic disorders. It has been seen as a con- genital trouble, as a part of an inherited familial degeneration. Op- penheim is convinced that syphilis can provoke the degenerative process. A slow hemorrhagic cause may be possible. Pathologically the disease is a primary degeneration of the neu- rones of the nuclei of the ocular motor nerves. Symptoms. — These come on gradually and often are not remarked until the disease is well advanced. The vision of the eyes individually is not affected, and as the trouble is so slow and insidious, when the lines of vision are disarranged on account of the palsy, the patient becomes habituated to a monocular vision. Gradually the loss in the mobility of the eyeball is noticed. There is a slight strabismus, more frequently divergent. Examination with the moving finger reveals the limited excursion of the eyeball in attempting to follow it. The eyelids droop and what is termed the "Hutchinson face" is presented. Sometimes amblyopia is complained of. The pupil as a rule is intact and reacts well to both accommodation and light. Prognosis. — The disease is a very chronic one and the prognosis- is unfavorable. Sometimes it becomes stationary. Optic atrophy often combines with it, especially when it is a part of some general systemic degenerative trouble. Treatment. — For the acute cases the treatment is along general antiphlogistic lines and includes purgation, baths to provoke sweating, ice bags and temporal bloodletting. A history of syphilis points out the proper therapy to resort to, while in all cases the causative factor, if such can be found, must receive the brunt of the attack. Galvanism has been recommended ; upon what ground it is hard to conceive. Reme- dies that have been used and are still recommended, but are generally ineffectual, are strychnia, arsenic, potassium iodide, phosphorus and general tonics. Primary Degeneration of the Other Cranial Nerves is exceedingiv rare as an isolated process. A few congenital bilateral facial palsies have been seen, and unilateral nuclear disease of the hypoglossal has been reported. Such cases are always unique and we have no knowl- edge of their true anatomical basis. Most of the primary degenera- tions of these nerves belong to the bulbar palsies and will be described under that head for the sake of convenience, or to the bulbar manifesta- tions merely of other more general troubles, such as progressive mus- cular atrophy, general intoxications and specific infections, where they will receive their appropriate consideration. ( )f primary degeneration of the spinal motor nerves we know- nothing, if indeed it ever occurs. Some forms of toxic peripheral THE NEURONIC DISEASES 429 neuritis may be primarily degenerative and for aught we know be limited to the motor neurones. It is doubtful, however, as in these cases inflammatory and sensory symptoms are often present. MYASTHENIA GRAMS. This is believed to be a bulbar disease, as is indicated in the names asthenic bulbar paralysis and bulbar paralysis without discoverable anatomical lesion; but its functional neuronic character depending, as most authorities think, upon a dynamic disturbance in the lower motor neurones, it may for the present be classified here. A number of cases of the disease have now been reported. I can add to them one that I was recently consulted about, which has since died after presenting for several months a very typical clinical picture, and which seemed to follow an aborted attack of scarlet fever. Etiology. — Middle-aged and young persons are usually selected as victims. Chlorosis and a hereditary taint were noted in one case by Oppenheim and Bruns. Toxaemia was strongly suggested in my case. The man was a teacher in a college where there had been a slight epidemic of scarlet fever. He had a faint, evanescent rash and signs of an aborted attack. Later on he assisted in the formaldehyde fumigations and shortly after this showed the first signs of weakness. Pathology and Pathogenesis. — There is nothing known of this, as no anatomical lesions have been found. Symptoms. — As the name myasthenia gravis indicates, the prom- inent symptom, of this disease is the profound muscular weakness, with remarkable remissions in the weakness. The disease lasts often many months and in that time the patient has spells of such weakness that he will keep his bed ; and yet he will recover so much that hopes will be awakened that he has fully gotten well again. The hopes are soon seen to be fallacious, for on comes the weakness, almost paralysis, again. And thus he goes, up a few weeks, then down again, the weakness disappearing from this group of muscles, to reappear later on in another group. The muscles supplied from the medulla are so frequently affected, and so often are the cause of the patient's death h\ asphyxia, that the name asthenic bulbar paralysis has been given to the ■disease. Other muscles of the body, however, are usually implicated. Ptosis is particularly common. It was almost the first symptom in my case. The muscles of the trunk and extremities are affected. All acts and all movements are restricted. The patient feels as though he could walk as well as any one, but the moment he gets up he begins to weaken and he is chagrined to find that the feeblest efforts, the shortest walks, completely exhaust him. Talking becomes a tremen- dous task. Sometimes he can't masticate and swallow Ins food. A remittent, chronic, progressive glosso-labio-laryngeal paralysis, with weakness and palsy in other parts of the body, constitute tin- motor side of this singular malady. Sometimes the bladder and rectal functions are also implicated. The muscles are norma] m size and do not reveal any abnormal electrical reactions. Noticeable variation of the weakness, from a moribund state altnosl to complete health, and 430 THE NEURONIC DISEASES then back again to a moribund state, and marked, almost dangerous exhaustion are the striking features in the course of the disease. There are no sensory symptoms and the mind continues clear. Diagnosis. — This must be made from the character of the weak- ness and palsy, the remissions during the progress of the disease, the absence of organic muscular changes, the absence of sensory and men- tal symptoms and the probable etiology. Hysteria can be differentiated by the presence of sensory and mental phenomena. Poliomyelitis, or rather polioencephalomyelitis and muscular dystrophy, do not remit as this disease does, and they exhibit organic muscular signs such as the muscular atrophy and electrical changes. There are some things about this disease that lead one to compare it with Landry's syndrome. The latter has even appeared first with the bulbar complexus of symptoms. Prognosis. — The malady is almost universally fatal, though a few cases have gotten well. The trouble appears acutely or insidiously, but it generally runs a remittent, chronic course. It is a dangerous dis- ease, as one can never tell when death may come. The latter usually results from asphyxia or exhaustion. Treatment. — Rest and quietude in bed is about the best we can do. There is a strong temptation to use electricity, but it is a danger- ous procedure. Moreover, it could hardly be expected to do any good, from the nature of the trouble. When the patient cannot swallow a stomach tube may be used, but it involves a great risk. Oppenheim lost a case during the manipulation. If there is the slightest degree of spasm from the introduction of the tube, the treatment had better be desisted. Simple tonics and a mild diaphoretic treatment might prove available, for it is probable that the basis of the trouble is an intoxica- tion or infection of some sort. OCCUPATION MUSCULAR ATROPHY. CRAFT PALSY. PROFESSIONAL PALSIES. This is a borderline degeneration which in some cases is undoubt- edly primary, while in others it is secondary and is associated with a neuritis. The constant use of certain nerves and muscles in one monotonous manoeuvre, demanded by certain occupations, sometimes leads the lat- ter to undergo an atrophic paralysis. Oppenheim mentions a weaver who had to extend his forearm about twenty thousand times a day and who thereupon suffefed from an atrophic paralysis in the right triceps muscle. In this way locksmiths, paper-glazers, blacksmiths, cabinet-makers, gold-polishers, oarsmen, cigar rollers, drummers have been afflicted. Of course, the trouble is mostly seen in the upper ex- tremity. Potato-pickers, seed-sowers and those who kneel a great deal may have the trouble in their lower limbs. Ballet dancers have thus had their calf muscles affected. These cises must not be identified with the occupation neuroses, which are due to a disturbance in the central eoodinating apparatus, rhese cases are to be attributed to over-exertion, pressure, even lacera- tion of the nerves in which run the lower motor neurones. There is an THE NEURONIC DISEASES 43 1 atrophic condition started in this way in these neurones which in most instances is quickly recovered from if the member is placed at rest. This wasting, however, has in a number of instances, in spite of the use of all preventive measures, gone on to the production of a pro- gressive muscular atrophy. General intoxication* with alcohol, lead, infection from influenza, general marasmus and a previous history of acute anterior poliomyelitis seem to favor this unfortunate turn to the paralysis. In some cases there have been slight paresthesia?, and pains and other sensory manifestations that have suggested an interstitial neuritis. In still other cases the trouble is probably due entirely to the muscle and not at all to the nerve, in this way intimating what we have long been suspecting — namely that there is no sharp line of division to be drawn between the atrophies and the dystrophies. Most of these craft palsies get well under favoring conditions, for the atrophy, whether primary or secondary, does not go on to the stage of incurability. \\ 'hen, however, it passes into the progressive form, the prognosis is hopeless. The treatment consists of rest, the careful use of galvanism, hypodermics of strychnia and a general tonic and health-producing regimen. ACUTE ASCENDING PARALYSIS. LANDRY'S PARALYSIS. Landry described in 1859 a form of motor paralysis in which there were no sensory symptoms, no muscular wasting, no electrical reac- tions, no bedsores or other trophic manifestations, no involvement of the bladder or rectal functions. The paralysis was flaccid in character, ascended rapidly up the body and usually terminated in death in a few days or a week, from respiratory involvement and other bulbar symp- toms. There were no post-mortem findings and so Landry. Westphal and others attributed the trouble to some form of acute poisoning. Later on when it was found that some cases of acute ascending paralysis exhibited slight sensory symptoms, and a mild degree of muscular atrophy, with a loss of the deep reflexes and with certain electrical changes, it was believed that Landry had described merely a form of multiple neuritis. Comparisons were drawn between it and the symp- toms produced often by such forms of intoxication as alcohol, curare, lead and the infections. It soon became clear that Landry had not described a new disease, but a symptom-complex often observed in the early stage of virulent forms of intoxication. In one sense, therefore, the term acute ascending paralysis covers more than the term Landry's paralysis, while the latter, in its original, narrow definition, is but one manifestation of a profound intoxication of which there may be other and later manifestations according to the susceptibility of the nervous system involved, the virulence of the poison or the duration ^i its action. Etiology. — Some form of profound intoxication of bacterial origin seems to be at the bottom of Landry's paralysis. Neither the particu- lar toxin nor the micro-organism has ben isolated yet. The disease seems most frequently to follow, or to be associated with, the infectious 432 THE NEURONIC DISEASES fevers, notably typhus, typhoid, pneumonia, variola, pleurisy, diphtheria, anthrax, influenza, pertussis, gonorrhoea (?), syphilis, septicaemia and rabies — a most suggestive list ! Other contributing causes have been named by various writers — namely, exposure to cold, the puerperium, suppressed menstruation, coitus in the standing posture and mental excitement. It is probable that these were mere coincident circum- stances, or at most assisted only in provoking the intoxication or auto- intoxication underlying the disease. Men are more subject to the trouble than women are, the proportion being put by some as high as twelve to four. The middle period of life, twenty to forty, is when the disease occurs most frequently. It will be noted, as Gowers well remarks, that the remote etiology of acute ascending paralysis re- sembles in general that of acute myelitis. Pathology and Pathogenesis. — According to the earlier obser- vations of Landry, Westphal and others, a remarkable peculiarity of this disease was the absence of all post-mortem findings. Landry therefore concluded that the trouble was due to some form of poison- ing, a view which Westphal was the first to establish upon a scientific basis. Even to-day, with our more improved methods of investiga- tion, there are cases in which no pathological changes can be discovered at the autopsy. Such cases, however, are becoming less and less fre- quent every year. In other words, the greater number of the cases nowadays reveal changes of an inflammatory and degenerative type in the nervous system, and more or less the appearances of a septic condition in the other organs. These changes are indefinite, varied and scattered, just as we would expect in a general toxaemia. Dissem- inated foci of inflammation with exudation and capillary hemorrhages, have been observed in the medulla, particularly the pyramidal tracts. Similar changes and other myelitic processes have been found in the spinal cord. Anterolateral tracts have been affected. In some cases there were findings of a typical poliomyelitis, but of a slight degree. Oppenheim says that in one case a degeneration of the anterior roots was the only alteration ; in another a small myelitic focus in addition to a peripheral neuritis. The lesions of the peripheral nerves have been observed frequently enough to incline some authors to regard the dis- ease as a peripheral neuritis. Outside of the nervous system the changes most commonly found included enlargement of the spleen, pleuritic adhesions, hemorrhagic foci in the lungs and bowels, inflam- mation of the lymph glands, especially in the intestines and mesentery, and albuminuria. All of this indicates plainly the toxic origin of the trouble. The bacterial source of the toxin is based upon strong evidence, though as yet no specific micro-organism has been isolated. The researches of Baumgarten, Eisenlohr, Centanni, Curschmann, Remmlinger, Oettinger and Marinesco, Marie and Marinesco, Bailey and Ewing, Seitz, Thoi- not and Maselli, and Buzzard are almost conclusive. By these investi- gators various bacteria have been found in the nervous tissues of pa- tients dying of acute ascending paralysis. By some of them the germs have been cultivated, injected into animals and thus a set of symptoms provoked identical in all respects with those of the disease in man. THE NEURONIC DISEASES 433 In the modern sense, Landry's paralysis cannot be regarded as a distinct pathologico-anatomical entity, in spite of the fact that clinically it affords a fairly definite symptom-complex. It is the expression of an intoxication of bacterial origin, some cases running their course so rapidly as to leave no discoverable lesions, while others of a slower or less virulent type exhibit various, diffuse, infectious lesions. Why this intoxication should seem to select the peripheral motor neurone in most cases whereon to expend its force primarily it is hard to say, but that it is not limited solely in all cases to that neurone is shown by the sensory and other symptoms. This may be explained when we know more about the relative susceptibilities of the different parts of the nervous system, or when, perchance, we discover, if we ever do, a specific germ as the cause of the intoxication. The diffuse character of this poison and the irregularly scattered location of the lesions which it provokes may well cause Landry's paralysis to closely simulate a diffuse myelitis, an anterior poliomyelitis or a multiple neuritis. They are not the same, however, as will be pointed out under the head of diagnosis. The cases that succumb rapidly are apparently primary, profound intoxications of the neurones. For this reason I place the disease pro- visionally here. "When it is shown to be always a pure inflammatory trouble, it will then be removed from the class of neuronic affections of primary origin. Symptoms. — Usually there are premonitory symptoms, such as a feeling of general malaise, numbness of the extremities, slight pains in the back and elsewhere, weakness of the legs with tingling and other symptoms not unlike those found in neurasthenia. These prodro- mata may last several days and gradually pass into the typical symp- tomatology. Many cases, however, begin abruptly with a decided weakness in one or both legs, which in a few hours become completely paralyzed. The paralysis then rapidly travels up the body, involving successively the trunk, the arms and finally the muscles of respiration and deglutition. The patient cannot sit up on account of the paralysis of the back muscles. Coughing, sneezing, defecation and urination are all difficult when the abdominal muscles are involved. Swallowing and breathing become embarrassed and in the end impossible. In a case reported by Westphal the medulla oblongata alone was implicated, the disease beginning and terminating with bulbar symp- toms. The paralysis is not always paraplegic in its distribution or ascend- ing in its course. Sometimes it skips about from the arm to the leg or vice versa. Fagge declares that the great naturalist Cuvier died of a rapidly descending Landry's paralysis. As a rule the deep reflexes arc lost, but the muscles are not atro- phied and the electrical irritability remains normal. There is no f except in a very few cases, in which there has been observed a slight temporary elevation of the temperature. The sensations arc unaffected. Trophic abnormalities are absent. The rectal and bladder functi are undisturbed. Finally the speech grows heavy and indistinct, swal- lowing becomes impossible, respiration grows rapid or assumes the 434 THE NEURONIC DISEASES Cheyne-Stokes characteristics, and death ensues in a few days, usually less than a week, under asphyxia. The mind remains clear to the last. Such is the acute ascending paralysis described by Landry. Even to-day many authors limit the name Landry's paralysis to the above described symptom-complex. The more modern conception of the disease, however, presents a broader clinical picture. For ex- ample, with the paralysis in some cases are associated a moderate de- gree of muscular atrophy, sensory and trophic symptoms, and involve- ment of the bladder and rectal sphincters. Moreover, the distribution of the paralysis is less regular and the progress of it less rapid than as Landry originally described it. And instead of the disease being so universally fatal, not a few cases have gotten entirely well after several weeks' duration. Many of these later cases bear a close resemblance to acute ascending meningo-myelitis, disease of the spinal roots and multiple neuritis. The sensory symptoms when present are very variable. At first there is numbness, formication and other paraesthetic manifestations. A slight loss of sensation is common ; a complete anaesthesia is rare. Hyperesthesia has been observed. Analgesia is sometimes present, and even the muscular sense has been found to be altered. Thus every variety of sensory disturbance may be expected. These symptoms are doubtless due to a secondary involvement of the sensory apparatus, as Landry's paralysis would seem to be primarily a disease of the motor apparatus. As a rule the slight diminution that occurs in the muscle-masses, when the patient lives long enough, is not the same as the typical atrophy that is observed in acute anterior poliomyelitis. Even after many weeks there is still no atrophy nor any alterations of the electrical reaction. Exceptionally, however, there is distinct,, typical muscular atrophy and a characteristic reaction of degeneration. Remak errone- ously thought that this absence of muscular atrophy and normal elec- trical irritability were pathognomic of Landry's disease. ' In exceptional cases the bladder and rectal functions have been affected. According to Oppenheim, even complete paralysis of the sphincters has been observed. In many of the cases there may be for a short time retention of the urine, but as this soon passes off, there is seldom need for the employment of the catheter. The cranial nerves, other than those spoken of in connection with the respiration and deglutition, escape. In rare cases the facial and motor-oculi have been implicated. As a rule there is no fever, no delirium, no clouding of the mental faculties. In a few cases there have been recorded high fever, signs of general septic infection and more or less obliteration of conscious- ness. It may be well questioned whether such manifestations should be included under the head of acute ascending paralysis rather than that of a general septic state of which the paralysis is a concomitant symptom. In these cases hyperidrosis has often been described. Diagnosis. — Multiple neuritis of infectious origin has been identi- fied with Landry's disease (Ross, Dejerine and Goetz, Nauwerck and Barth, Putnam, Klumpke. Krewer, Pitres and Vaillard, Mobius, Kiril- THE NEURONIC DISEASES 435 zew and Mamurowsky, Spiller and others), and yet. as Oppenheim well says, it is not proper to regard them as perfectly identical. Lan- dry's paralysis of the pure type differs from multiple neuritis in the absence of distinct pain and other sensory disturbances, in the non- atrophic condition of the muscles and the preservation of the normal electrical irritability. Meningeal hemorrhage may be distinguished by the pain in the back and the spasm. Acute anterior poliomyelitis does not exhibit the peculiarly rapid, ascending, mere or less paraplegic form of paralysis characteristic of Landry's symptom-complex. The former is notable for the distribution of the paralysis in particular muscles or groups of muscles, which quickly undergo atrophy and manifest well-known abnormal electrical reactions. Aente myelitis or menin go-myelitis of the ascending type is to be differentiated by the presence of fever, sensory troubles, bedsores and other trophic mani- festations, and vesical and rectal phenomena. After all, however, it must be admitted that there are some cases of Landry's syndrome that cannot clinically be distinguished from any one of these, especially from multiple neuritis and acute anterior poliomyelitis. And this is not to be wondered at when one recalls the nature and pathogenesis of Landry's group of phenomena. The same observation will explain the futility of arbitrarily dividing the disease into three distinct types, the bulbar, spinal and peripheral ; or of mak- ing such a separate variety as the descending form, in contradistinction to the ascending. Prognosis. — The course of the disease is variable and the prog- nosis always grave. It is not always fatal, however, as it was at one time considered. Sometimes death takes place in a tew hours, or in two or three days, and sometimes it is postponed for three or four weeks. The rapidity, duration or completeness of the paralysis does not seem in any way to measure the gravity of the disease. The char- acter of the bulbar symptoms and the degree of involvement of the functions of respiration, deglutition, etc., are the guides to be closely watched in forming a prognosis. Steadily progressing cases, even though slow, are unfavorable. Cases have recovered, a fact which justifies a favorable outlook, if there is the slightest retrocession in the progress of the malady. The fatal cases usually terminate inside of a week. Treatment. — Recognizing the toxic origin of Landry's paralysis, it is obvious that the ideal treatment would be the use of an antitoxine. There is none known, however, and so we must still resort to all m ures calculated to support the patient's vitality and to eliminate the poison as rapidly as possible. Unfortunately the rapidity of the dis- too often forestalls all such commendable efforts. Nevertheli it is wise to resort to mild stimulation with alcohol, ammonia and other diffusible stimulants, to give an abundance of easily digested and easily assimilated nourishment, and to increi Far as possible, the func- tions of the emunctory organ-. Warm baths and hot wet packs stimu- late the skin into renewed activity. Counter-irritation over tin- spine, and especially over the region of the kidneys, should be resorted to. The action of the kidneys should lie favored bv tin- n^\ diuretics with 436 THE NEURONIC DISEASES the drinking of an abundance of wafer. Free catharsis is advisable with calomel or elaterium, followed by salines. When swallowing be- comes difficult nourishment and medicines should be administered by the rectal or nasal tube. As for medicines, there are none known upon which we can rely with certainty. In one case following exposure to wet and. cold, and almost at the point of death, ergotin in two-grain doses every hour was followed by a speedy recovery. Salicylate and benzoate of sodium have been recommended. Iodide of potassium and mercury have had their advocates, irrespective of the possible cause of syphilis in some cases. Belladonna has been tried with doubtful results. As for the use of strychnia, one would feel some hesitancy about employing so vigorous a spinal stimulant in a disease in which there are inflammatory and degenerative changes going on. In a word, the nature of the disease, and the results so far ob- tained, indicate a revulsive line of treatment, w T ith the use of anti- phlogistic and diaphoretic measures. Perhaps some day blood-serum therapy will make all such crude measures unnecessary. PERIODICAL PARALYSIS. This is perhaps the place to refer briefly to a remarkable form of paralysis that comes and goes periodicallv. 'The phenomenon has been seen particularly in connection with the movements of the"eye and of the extremities. Periodical Paralysis of the Extremities. With or without an an- aesthesia an intermittent paraplegia of the arms and legs occurs, com- ing on with great regularity, without apparent cause, when the pa- tient is in the full flush of health, awake or asleep. Weakness of the limbs, weariness and drowsiness, and occasionally paraesthesiae precede it. Twenty-four hours usually sees the paralysis complete. The arms, the legs, the neck and occasionally the tongue and pharynx are in- volved. The cranial nerves are unaffected as a rule. There is no fever, but the temperature is often subnormal and the pulse slow. The heredity in the disease is quite marked, so much so that it assumes the form of a "family" disease. Goldflam, for instance, saw a family with twelve afflicted members, the heredity being through the mother. Taylor saw eleven cases in five generations of a family. This with other features of these periodical paralyses makes one think strong- ly of a hysterical affection, but there is a remarkable stumbling block in the way of regarding it as hysterical. Functional as the disease appears to be, there is a reduction, sometimes abolition, of the reflexes ; and stranger yet than all, a reduction or complete abolition of faradic excitability of both the muscles and the nerves. This looks very much like a toxic neurosis or temporary infection involving especially the lower motor neurones. Nothing but toxaemia would produce such a localized, short-lived paralysis. It is not impossible that the toxin, whatever it is, may be periodically evolved in the patient's own body, as the result of his inherited neurotic defect. This is the only sort of a hypothesis that would harmonize all the features of these strange par- THE NEURONIC DISEASES 437 alyses. The evolution of the toxin results periodically from an inher- ited neurosis. This expends itself upon the lower motor neurones and we have the incipiency of an organic neuronic disease, which, however, does not last long enough to provoke permanent changes. COMBINED DISEASES OF THE AFFERENT AXD EFFER- ENT SYSTEMS. It is well known that degeneration and sclerosis in the lateral col- umns of the cord occur late in a few cases of locomotor ataxia. This is looked upon as a mere complication or sequel and not as an integral part of the disease. There are a number of diseases, however, in which the hardening of the lateral and posterior columns proceed simultane- ously, seem to be dependent upon the same causative influence, and exhibit a more or less uniform clinical picture. Heredity plays a sig- nificant role as a predisposing cause, while infection and nutritive dis- turbances a're prominent among the exciting causes. The cord changes observed in pernicious anaemia afford a good illustration of the domi- nance of an exciting cause, while Friedreich's ataxia shows well the power of a hereditary cause. When the disease occurs in childhood, as it does in most of the hereditary types, the undeveloped condition of the nervous system nat- urally causes a modification in the clinical picture different from that which it would have in an adult after the nervous apparatus has ac- quired its full growth and has long been subjected to disciplined and fixed habits. Again, a variation in the clinical exhibition would be wrought, as Westphal long ago pointed out, by the rehtive intensity and extent of the lesion in the lateral or the posterior columns. If the disease process in the posterior columns is in advance of that in the lateral columns, the symptoms bear a resemblance in some respects to those of tabes. If the disease is more pronounced in the lateral than in the posterior columns, the clinical portraiture simulates somewhat spastic paraplegia. Clinically, therefore, we find in these combined scleroses two prominent features that justify their consideration as distinct and inde- pendent affections. The fir^t is the uniformity of the clinical mani- festations due to the combined posterior and lateral lesion. The other is the particular modification of symptoms evoked by the age of the patient and the relative prominence of the lesion in one or the other location. Unless these facts are kept well in mind there will be an un- necessary refinement of classification and the elevation of mere symp- tom groups into distinct and separate diseas From what has alread) been said, it is obvious that the combined sclerosis of the two columns of the cord, so different in their genera] functions as are the lateral and posterior columns, must necessarily pro- voke some marked differences between tin- clinical manifestations of different cases. Theoretically and pathologically there may be no good reason for separating from one another the spinal sclerosis of pernicious anaemia, ataxic paraplegia, hereditary ataxic paraplegia, Friedreich's disease any 438 THE NEURONIC DISEASES FIGURES 87, 88 AND The combined scleroses or system diseases. Fig. 87, cervical section. Fig. 88, dorsal section. Fig. 89, lumbar section (After Jakob.) THE NEURONIC DISEASES 439 more than there is for separating the hemiplegias, diplegias and con- genital spastic "paraplegias" among the cerebral palsies of childhood. Practically, however, it is advantageous to make the distinction, not to emphasize them as distinct diseases, but to point out the individual etiology and minor symptomatology in each particular class. The highest aim of science is to generalize and not to individual- ize. The reduction of all exceptions and their inclusion under a general law, as rapidly as knowledge m regard to them will warrant, should ever be the purpose of scientific endeavor. Unfortunately in this re- spect art and science are often in gentle conflict, and as a result we make for practical convenience distinctions and classifications where scientifically there are no broad lines of difference. For practical purposes then we will do well to carry about in our memory the following types of the combined scleroses enumerated by Dana: i. Combined scleroses of profoundly anaemic and toxic states (Putnam's type). 2. Hereditary spinal ataxia (Friedreich's ataxia and hereditary ataxic paraplegia). 3. Combined scleroses complicating general paresis. 4. Accidental forms (Gowers', ataxic paraplegia). Myelitis, meningomyelitis, posterior leptomeningitis of syphilitic and other infectious origin with secondary degeneration in particular tracts, will explain a large number of cases reported to belong to one or other of these classes. The anaemic and toxic scleroses are clearly not distinct disease entities and in many cases they are merely diffuse condi- tions such as one observes in all forms of general constitutional poison- ing. In pellagra, in ergotism, in leukemia and in carcinoma a combined sclerosis of the cord has been observed. Hereditary spinal ataxia I have discussed in the next section. Under dementia paralytica these complications and sequelae will be referred to. ATAXIC PARAPLEGIA. This is not a disease. It is merely a characteristic syndrome which has many congeners and which is the result of a combination oi two diseases, or rather sets of symptoms dependent upon a lesion in both the lateral and posterior columns of the cord. It may beneficially be adopted as a model for the description of the general symptomatology of these combined scleroses and for pointing out the possible variations that may occur in the clinical picture of some other forms of the com- bination. For tile nonce then I will discuss it as though it were a unique and distinct entity. Etiology. — Heredity of some sort probably plays a role in all of these cases. In most of them it may amount to a mere neuropathic predisposition, a congenital weakness, lack of resistive power to inju- rious influences in certain tracts or neurones. It may be so decisive, as in the six cases observed by Dana, as to almost warrant Its con- sideration as a separate disease under the head of hereditary or familial ata.vie paraplegia. Dana's cases ware all women and began between 440 THE NEURONIC DISEASES the ages of twelve and sixteen. Most cases begin between thirty and forty years of age and appear in the male sex. Absence of a history of syphilis or alcoholism is a striking feature. No exciting cause is known, though it has followed exposure to cold, sleeping in clothes saturated with perspiration, sexual excess, severe concussion of the spine and certain cachexias. I have seen the symptoms follow a severe anaemia and a chronic diarrhoea of twenty years' standing. Whether pernicious anaemia produces a systemic sclerosis in the lateral and pos- terior columns or merely a diffuse myelitic condition is not yet posi- tively settled. Lichtheim, Norine, Minnich, v. Xoorden, Taylor, Water- man and Billings believe that it is a diffuse process. Oppenheim still believes that it is a true combined systemic neurone disease. Taylor and Waterman, Bastianelli, Russell, Batten and Collier, Billings and others consider that the arteriosclerosis, the anaemia, and the other concomitant conditions observed in these cases of combined degenera- tion in the cord are the result of the same general toxaemia that gives rise to the spinal lesion and are not themselves the direct cause of the latter. Little as we know about the etiology of these combined degenera- tions, be they diffuse or systemic, two factors seem to stand out with suspicious prominence, namely, an inherited predisposition and a toxcemic excitation. Pathology and Pathogenesis. — The essential lesion is a degen- erative sclerosis of the posterior and lateral columns of the cord. It may extend throughout the entire length of the cord, though the pro- cess is apt to be more pronounced in the thoracic region. In some in- stances, as for example Friedreich's disease, the degeneration is sharply limited to certain tracts, notably the postero-median, postero- lateral and crossed pyramidal, and the disease is shown to be a purely systemic one. In other instances, as for example the anaemic cases (Bastianelli, Russell, Batten and Collier, Spiller and others) the lesion is less sharply defined and the disease process may be regarded more as a diffuse one. It may be that secondary degenerations of a systemic character follow and are associated with the diffuse lesions in many of the cases, thus accounting for their general systemic appearance upon post mortem examination. Other tracts besides Goll's, Burdach's and the crossed pyramidal that sometimes participate in the degeneration are the direct cerebellar, the anterior or direct pyramidal and the antero-lateral. The gray mat- ter of the cord, the spinal roots and the anterior root zones are not implicated. The membranes have only rarely been diseased. The sclerosis is of the ordinary type though its distribution and intensity are subject to considerable variation in the respective columns where it appears. In the laterial columns it is always less systemic in its limitations than it is in the posterior columns : and usually there is a narrow strip of healthy tissue immediately adjoining either side of the posterior horns. Tn the severe anaemic and toxic cases there may be such a rapid destruction of the neurones that a peculiar perfor- ated appearance is given to the cross section of the cord. Tn such cases THE NEURONIC DISEASES 44I an oedematous condition with destruction of the septa and the forma- tion of granule cells may be observed (Dana). To sum up then, the pathology of the combined scleroses indicates two great groups of cases, the systemic and the diffuse, with the chief foci of disease in the posterior and crossed pyramidal tracts. Outside of Friedreich's disease and the spinal sclerosis of general paresis, which are purely systemic, no sharp differentiation can be made. Some cases are doubtless systemic from the start, others are diffuse but be- come later on systemic on account of the secondary degenerations. Symptoms. — In analyzing the clinical presentation of these cases of combined degeneration as seen in the cord, it must be remembered that we are dealing with two somewhat diametrically opposed sets of symptoms. In the one a spastic spinal paralysis due to degeneration of the pyramidal tracts is combined with a true ataxia, lancinating pains, pupillary rigidity, bladder trouble and other tabetic symptoms; in the other the symptom-complex of tabes is accompanied from the beginning by motor weakness. Studied separately the clinical manifestations of lateral degener- ation would, a priori, seem to neutralize those of posterior degenera- tion. And yet such is not strictly the case. There is never a com- plete annihilation of one set of symptoms by the other. One may be more prominent than the other and thus determine the leaning of the case toward spastic paralysis or locomotor ataxia. Whichever the dis- ease seems to tend toward, always search for indications of the other. Like the lesion, therefore, the symptomatology of these cases is always a combination. This, however, does not express the whole truth, for there is more than a mere combination of symptoms, there is a more or less characteristic modification wrought in the clinical pic- ture by the simple combination. It is the relative constancy of these characteristic modifications of the symptomatology in toto that has led to not a little confusion in the past and betrayed writers into estab- lishing new diseases out of the various clinical presentations. Inter- mediate types are here so numerous, and the gradations from one to the other are so gradual, that no description will stand rigidly for all types. The ataxic paraplegia of Gowers, while not a fixed type, will answer well enough as a basis for a general description of the symptomatology of the combined sclerosis, the description to be always modified to suit the etiology, age of patient and prominence of the loca- tion of the lesion in the particular case in hand. Usually the symptoms appear slowly. There is weakness in the legs for several months or years and then the walking becomes grad- ually impaired. The weakness and incoordination may be confined to the legs, though it is not infrequent that they extend to the arms. Ex- ertion, turning, walking in the dark become arduous. Examination reveals even at this time a real condition of paresis in the muscles. < tae leg, one set of muscles may be implicated more than another. The gait resembles that of tabes but the high elevation of the leg and tin- stamping of the heel on the floor are not so marked as in tin- latter disease. The ataxia is both static and motor. In the great majority of cases the knee-jerk is markedly exagger- 442 1HE NEURONIC DISEASES ated and a distinct rectus clonus can be elicited. Foot clonus is also generally present. This exaggeration of the patella reflex contrasts these cases strongly from locomotor ataxia. It continues mostly throughout the entire course of the disease. In the cases in which the knee-jerk is at first increased, and later annulled, the degeneration in the posterior columns has descended into the lumbar region. The case then resembles mOre nearly tabes but differs from it in manifesting a true progressive motor weakness or paresis. The marked absence of sensory symptoms also distinguishes these cases from tabes. Lightning pains are scarcely ever observed. Some- times dull pains occur in the legs, in the spine and around the body. As a rule cutaneous sensation is unaffected. Hyperesthesia has been rarely noted. I have seen the cutaneous reflex of the sole of the foot highly exaggerated, especially in the anaemic cases. The tickling was not distressing to the patient though the limb was violently drawn up. The cremasteric and abdominal cutaneous reflexes may be lost at times. In exceptional instances the sensory phenomena may be as positive as they are in tabes. These are the cases in which the knee- jerks are lost and the tabetic resemblance is marked. According to Gowers these intermediate cases between ataxic paraplegia and true tabes do not constitute more than five per cent, of all the cases with ataxia and motor weakness. The muscles are not wasted to any extent and the electrical re- sponses are normal. Sexual vigor may be lost early. The sphincters are usually im- paired but only moderately. The eye symptoms when present are the same as those met with in tabes. They are less frequent, however, than they are in the lat- ter disease. Nystagmus does not occur generally ; though with other bulbar symptoms it is a positive feature in Friedreich's hereditary tvpe of combined sclerosis. Articulation is slightly impaired in some cases. Gowers says it is not uncommon. A slight amnesia as a rule is all that character- izes the mental state in the way of deterioration. As the disease continues to advance the paresis becomes more and more marked until it quite overwhelms the incoordination. Finally after a period of going: about on crutches, the patient takes to his bed never to leave it. Stiffness and rigidity in the muscles increase and even before becoming bedridden the case develops into one of spastic paraplegia. The arms are now involved, if they have been fortunate in escaping so far. At length complete paralysis comes on and the case terminates as spinal spastic paralysis usually does. To the end sensation usually remains unimpaired, crises are absent, and the re- flex action of the iris, unless lost early, remains normal. Among the complications are dementia paralytica with its array of mental symptoms, occasional slight muscular atrophy and arthritis. Such in the main is the clinical picture of ataxic paraplegia, or combined sclerosis of the posterior and lateral columns of the cord. The differences to be observed in the symptomatology of. and the prob- able reasons for those differences in. the combined sclerosis of Fried- THE NEURONIC DISEASES 443 reich's ataxia and dementia paralytica will be pointed out when these diseases are discussed. Some other modifications, not sufficient to constitute the basis for the establishment of new diseases, may perhaps be profitably referred to here. In hereditary ataxic paraplegia there is stiffness and weakness in the legs and the ataxia is more cerebellar in type than spinal. Re- flexes are exaggerated. There is some paresthesia. The arms are only slightly involved. Optic neuritis was present in one case. The sphincters are intact, the mind is clear and the prognosis is more fav- orable than in Friedreich's disease. Xo autopsy having been made the true nature of these cases is not determined. They may be re- lated to Friedreich's ataxia or to Marie's hereditary cerebellar ataxia. In the anaemic and toxic cases of combined sclerosis there are apt to be early sensory symptoms chiefly of a paresthetic character. Numbness, pain and anaesthesia may appear in ail four extremities. Gradually the motor weakness comes on and finally the paraplegia. These are associated with the ataxia. Spasticity, exaggerated knee- jerks, ankle-clonus are all present. The mind is weakened toward the end. Severe anaemia, diarrhoea, emaciation are all concomitant mani- festations suggestive of a profound toxemic condition. Diagnosis. — Enough has been said under the head of symptoms to indicate the differential diagnosis of a combined sclerosis from sim- ulated diseases. Locomotor ataxia on the one hand, and spinal spas- tic paralysis on the other, are the affections most likely to cause con- fusion. In tabes the patella reflex is always and earl}' lost ; it is usu- ally exaggerated in the combined disease. The sensory and pupillary phenomena are as a rule sufficiently different in the two diseases to g< i a long way in establishing the differential diagnosis. In primary spas- tic paraplegia there is no ataxia. As Gowers says epigrammatically, ataxic paraplegia is spastic paraplegia plus incoordination. The hered- itary and familial types of combined sclerosis are distinguished by their history, the age of the patient and the repetition of the disease in the same family. Myelitis is sometimes hard to distinguish, and in fact Oppenheim declares that the toxemic ca*ses, while svmtomatically allied to the combined scleroses, are pathologically related to myelitis. Mye- litis, however, is regressive, though of course it may set up a ondary progressive defeneration in the lateral and posterior tracts. Such cases cannot be differentiated. Cerebellar tumor may cause symp- toms resembling ataxic paraplegia, but the headache, vertigo, optic neuritis and other well known signs of an intra-cranial neoplasm are present to aid in the diagnosis. Prognosis. — These degenerations are incurable. They are chronic and progressive in their course. Sometimes they remit symptomatically for a time but the onward course of the disease is again resumed. The danger to life is not great. Kidney disease and bedsores are the ac- cidents most to be dreaded as these, or general exhaustion, usually close the scene. Improvement has occurred and life been prolonged in a few cases. Very exceptionally a cure has been observed. The pr nosis therefore as to life is fair; as t<» recovery had. 444 THE NEURONIC DISEASES Treatment. — The treatment is the same as that for tabes and spastic paraplegia, the two diseases out of which, as it were, ataxic paraplegia is framed. It is no less and no more satisfactory than it is in them. The etiology, especially in the anaemic and toxic cases, and the symptoms, are the chief points of attack in the therapeutic man- agement of ataxic paraplegia. HEREDITARY ATAXIA. Under the broad term hereditary ataxia are included several forms of degenerative disease of the central nervous system, particularly of the cerebellum and spinal cord, in which etiologically heredity plays a dominant role and symptomatically ataxia stands forth as a- leading phenomenon. Three types of hereditary ataxia are recognized, namely, Fried- reich's ataxia, hereditary ataxic paraplegia and hereditary cerebellar ataxia. There will doubtless come others. The exact relationship of these troubles to one another is not clear, though there is such, since they overlap, to a certain extent, both. symptomatically and pathologically. The last is primarily an atrophic disease of the cerebellum, with certain secondary changes in the cord. I will refer to it more in detail elsewhere. The other two are often regarded as diseases of the spinal cord. Their principal lesion seems- to be spinal though there are frequent intra-cranial manifestations which link them together and give them both a sort of connection with the cerebellar type of hereditary ataxia. In one thing they all agree, namely, in being dependent primarily upon an inherited developmental defect, on account of which the sys- temic degeneration occurs in certain encephalo-spinal tracts. Before a more accurate classification of these various types of hereditary ataxia can be made, more definite data will have to be forth- coming in regard to their pathogenesis. For the further description of hereditary cerebellar ataxia the reader is referred to the section on diseases of the cerebellum. FRIEDREICH'S DISEASE. It was in 1861 that Friedreich first described the disease that now bears his name. It is a rare affection, though upwards of two hun- dred cases have been so far reported. Our knowledge of it has been increased by the labors of Fere, Mobius, Cuche, Ormerod, Bury, Sep- pilli, Vizioli, Ladame, Griffith, Burr and others. Heredity and ataxia are the cardinal features of the malady, though there are many other characteristics that serve to identify the particular form of hereditary ataxia which Friedreich described. Of the various names that have been given to the disease from time to- time all have proved to be defective and misleading; hence, objection- able as it is to name an affection after a man, in this particular in- THE NEURONIC DISEASES 445 stance, it is almost necessary to do so until the pathology and noso- logical position of the trouble is more definitely established. Etiology. — Heredity is the predominant factor in the etiology of this disease. It is both direct and indirect. In the broadest sense, therefore. Friedreich's disease is a family disease, a characteristic which stamps it at once. In a goodly number of cases one or more ancestors of the patient have been afflicted with the same malady. Sometimes it can be traced through four generations, increasing in •extent and intensity as it passes on down through the family. Often several members of the same family and generation are attacked. The heredity is not always direct. In the remaining cases it is indirect and seems to follow some neurosis or profound nervous strain in the fore- bears. This indirect heredity is much more frequently traceable than is the direct. The disease therefore is in all likelihood the result of an inherited defect of a developmental and neuropathic character. In the parents we find the following influences at work : neuropathies, such as epilepsy, psychoses, great nervous irritability ; alcoholism ; tu- berculosis ; syphilis ; consanguinity, and other unclassified cachexias and constitutional abnormalities. The hereditary influence need not necessarily be obvious in the parents, for in some cases they have been healthy and sound. In such instances somewhere among the blood relations will the taint be found lurking. In some cases the disease has been precipitated by an infectious fever, notably typhoid, pertussis, variola, meningitis, periostitis, measles, scarlatina, rheumatism and probably diphtheria. This form of ataxia never follows tabes dorsalis either in the parent or in the patient himself. If it does it must be exceedingly rare. Though the affection is hereditary and familial, it is rarely seen before the seventh or after the forty-fifth year of age. A large number of cases develop about the age of puberty. After twenty it rapidly becomes more and more rare. The majority of the cases make their appearance between the ages of six and ten. In fact, roughly speaking the age of ten is the time to expect its onset. It has been seen in a number of instances as early as two years of age. Most authors de- clare that it manifests no marked preference for either sex and yet in Griffith's T43 collected cases the percentage of males was almost double that of females. Dana says that in America, where more cases have been observed than in any other country, the females outnumbei the males in the ratio of three to two. The laboring and agricultural classes, rather than the denizens of the crowded cities, include chiefly the parents of these children. In some instances the outbreak of the disease has been attributed to falls and other traumata, child-bearing lactation and similar depressing factors. Pathology and Pathogenesis. — Much discussion has been ex- pended upon the pathology of the disease and this is not to be won- dered at. It is the most interesting feature about it and necessarih underlies its correct nomenclature and nosological position. Clinicalh the disease seems to be intermediate between tabes dorsalis and dis- seminated sclerosis, having several of the leading symptoms of both. Pathologically, however, it is related to Growers 1 ataxic paraplegia or 446 THE NEURONIC DISEASES the combined scleroses of the lateral and posterior columns of the cord. Seppilli, Brousse, Charcot and others regard it entirely as a distinct and independent malady. Constant lesions are found only in the spinal cord and medulla. The brain and nerves remain intact or are so very rarely implicated as to be excluded from consideration. The cord is generally smaller than normal throughout, and some- what flattened laterally and posteriorly. Along the posterior surface there are indications of a chronic leptomeningitis. The characteristic and constant finding in the cord is a systemic degeneration, involving the posterior columns, the postero-lateral cerebellar tracts and, to a lesser degree perhaps, the crossed pyramidal tracts. Gowers' tract is sometimes included. The change extends the entire length of the cord, though it is as a rule most pronounced in the lumbar region. Goll's columns are completely hardened ; Burdach's almost entirely so^- FIGURE 90. Cross-section of the spinal cord in Friedreich's disease. Pal's stain. (Alter Marinesco and Oppenheim.) A trace of healthy tissue sometimes lies between Burdach's column and the posterior horn. The crossed pyramidal tracts are always, and quite completely, sclerosed. The process seems to be more diffuse in the lateral columns, leaving a slip of healthy tissue alongside of the posterior horn, and rarely or never extending in front of a line drawn transversely through the middle of the cord. In a large number of cases the disease implicates the direct cerebellar tract, and the ascend- ing antero-lateral tract. Rarely are the anterior median columns in- volved. The anterior root-zones and the gray matter are normal. All the nerve-roots may be hardened, the anterior much less frequently than the posterior. The peripheral nerves are not regularly involved. The central canal of the cord has been seen to be inflamed in some instances and there have been present distinct but small cavities. The cells of Clarke's columns are often shrunken and without pro- cesses. Degeneration ^\ the posterior horns has been observed. In the medulla are Found traces of the upward extension of this sclerosis, the hypoglossal nucleus portraying the most significant change. The interpretation that is to be given all these findings is still largely hypothetical. If we exclude rigidly those cases in which some THE NEURONIC DISEASES 447 change in the cerebellum has been observed we find ourselves confronted by a spinal cord disease apparently. In a sense it is a systemic affec- tion but the lesion does not confine itself, especially in the lateral col- umns, within sharp outlines in any one tract. There is a certain de- gree of diffuseness that nullifies all idea of the degeneration being sec- ondary to some brain lesion in the larger number of cases at least, and the view is strengthened that Friedreich's disease is a primary sclerosis of the cord tracts. The similarity of hereditary syphilis and Friedreich's ataxia lends support to this conception. When we attempt to follow the many explanations ottered by various investigators at different times to determine the real pathogenesis of the disease, the original focus of the disease process, and the connection of the many pathological findings with one another, we find ourselves tossed about on a sea of theories and hypotheses. The only theory worth considering is the one which refers the whole trouble to an inherited defect in the cerebellum. This theory promulgated by Hammond has been lately revived and vigorously sup- ported by Menzer, Nonne, Auscher, Senator and others. The post mor- tem findings, however, in the cerebellar tissues are far from being con- clusive. Marie's hereditary cerebellar ataxia is probably an entirely differ- ent trouble and is not to be confused with the cerebellar theory of Friedreich's ataxia just referred to. The insignificance of the changes in the cerebellar cortex, their great rarity, as well as the constancy and character of the spinal cord changes, all incline me to believe that in Friedreich's disease we have a combined degenerative sclerosis of the posterior and lateral columns, with their related structures. It is probably developmental in origin in most cases, certain congenitally weak neurones succumbing under various exciting influences. In other words the disease in my judgment approaches very closely to being a congenital or hereditary ataxic paraplegia. The pathological process being in advance in the posterior col- umns, the symptoms arc largely ataxic in character, to such an extent even as to cause the disease to closely resemble locomotor ataxia. To put it in another way, Friedreich's disease, in its present form, is the hereditary or congenital analogue of those cases of ataxic paraplegia or combined sclerosis in which the ataxia, absent knee-jerks and other manifestations, ally it, symptomatically at least, with tabes dorsalis. The cases which have been reported as cases of hereditary ataxia and connected with Friedreich's disease, such as those of Sanger Brown, Dana and others, in which the reflexes have been exagger- ated, and which have been called hereditary ataxic paraplegia, are also inherited <>r congenital types of combined sclerosis of the lateral and posterior columns in which the disease i>n>ee>s i n the lateral col- umns is far in advance of that in the posterior. If this conception of Friedreich's disease and of hereditary ataxic paraplegia should ultimately prove to be the correct one, both will fall under the general classification of ataxic paraplegia or com- bined sclerosis of the lateral and posterior columns. The difference 448 THE NEURONIC DISEASES between these and the ordinary form of the trouble is dependent upon the hereditary origin of the former, while the difference between each other is the result of the relatively greater severity of the dis- ease process in the lateral or the posterior tracts. Symptoms. — Ataxia is not only the earliest, but it is the domi- nant symptom throughout the entire course of the disease. It usually attacks the lower limbs first and the upper limbs later. The reverse occasionally takes place. Before the ataxia is fully established there are generally preceding symptoms, such as weakness, unsteadiness, oscillation and staggering in the gait, even frequent falling. The ataxia is not exactly like that of tabes, jerky, irregular, with stamping of the heel first. It more nearly resembles a disturbance of equilibrium, a cerebellar ataxia, for which reason Charcot used to speak of it as a demarche tabeto-cerebelleuse. It is marked even when the patient is standing still. It is not increased, however, by closing the eyes, prob- ably because the early age of the patient is such that he has not ac- quired the habit of using his eyes yet in maintaining his equilibrium. As I have intimated the characteristic differences between the symptoms of this disease and tabes are due to the associated degenera- tion in the lateral columns and the occurrence of the disease in young, undeveloped individuals. Eclampsia and hysterical attacks have preceded and accompanied the ataxia in a few cases. In many there are painful, cramp-like spasms in some of the muscles. The face, neck, hands and feet have thus been involuntarily contracted. Tremor, not of the intentional type, but rather simple oscillations, is not infrequent in various parts of the body. Choreiform movements are almost constant in accom- panying the ataxia and may appear in any group of muscles. In ad- vanced cases decided weakness and more or less paralysis are com- mon features. The gait, which has been difficult from the first on account of the incoordination, later on becomes so affected that walk- ing is an absolute impossibility, and the patient keeps to his bed. The paralysis and subsequent contractures add to this unfortunate condi- tion. In many cases curvature of the spine takes place and marked dor- sal flexion of the toes. Talipes, chiefly of the equino-varus type is frequent. The knee-jerks are lost in pure cases of Friedreich's ataxia, which is a most important indication of the disease. In the few cases in which they have been exaggerated, the trouble was probably of cerebellar origin. Exceptionally ankle-clonus has been observed. The cutaneous reflexes are all intact or only very rarely altered. As there are scarcely any sensory manifestations the ataxia and the altered ten- don reflexes cannot be attributed to them. The muscles are not typ- ically atrophied, though there may be some slight wasting late in the disease. Their electrical contractibility is not in the least modified. Coldness of the surface of the body, cyanosis, oedema, chilblains, thick- ening of the skin, development of adipose tissue and even bedsores have been reported as among the rare vasomotor and trophic symptoms, and as particularly late in the disease. Sensory symptoms are not entirely wanting though they are so rare as to justify the general statement that sensation is normal in THE NEURONIC DISEASES 449 Friedreich's disease. The absence of the characteristic pains of tabes distinguishes it. And yet headache has preceded and accompanied the ataxia. When the disease is well established, occasional dull pains are noticed elsewhere. There may be tenderness over the spine. The muscles may be subjected to painful cramps. Pains in the articulations have been complained of. The cutaneous sensibility is not generally involved. Formication, tingling and numbness may be present and even anaesthesia. The muscle-sense is usually intact. Disturbance of the speech is a frequent trait and is one of the most prominent of the bulbar symptoms. It is generally a late symptom and appears about the same time as nystagmus, another most impor- tant symptom. It is a true dysarthria. The speech is slow and labor- ed, irregular and mangled. Sometimes it is rapid and slurring, typic- ally scanning, jerky, or confluent. The trouble may at times be very FIGURE 91. Position of the great toe and condition of the foot in Friedreich's disease. (After Tlriccn.nH \ Brissaud.) marked. At other times it is scarcely noticeable. On account of the rushing, tumbling character of it Seeligmuller spoke of it in one of his cases as an "ataxia of thought," as the patient seemed to begin a new sentence before finishing the old one. The tongue frequently ex- hibits a tremor, an oscillatory movement and a peculiar curling and uncurling. Mastication and deglutition are not generally affected. The chief eye symptom is nystagmus. Though this is a late symp- tom it is a most significant one. It is less common than the speech disorders, though the two usually appear together. Static nystagmus has been observed in a few cases. Strabismus has been mentioned and also a slight degree of ptosis. The pupillary reactions and the vision are undisturbed in the vast majority of cases, which again distinguishes Friedreich's ataxia from tabes dorsalis. The changes in the color fields and the blindness that have been noted have been SO rare that they probably represented some complication. The mental faculties are but little affected, a feature of Fried- reich's disease which helps to differentiate it from disseminated sclero- sis. There may be some amnesia and in rare instances an arrest of 45^ THE NEURONIC DISEASES mental development. Vertigo is rather a frequent symptom, occurring both early and late in the disorder. It may be associated with mere giddiness, fainting or actual loss of consciousness. It may come on while the patient is standing or reclining. Visceral, secretory and sexual disorders are rare and are probably mere accidents. Diagnosis. — The essential points to be noted in the diagnosis of Friedreich's form of hereditary ataxia are the hereditary and familial character of the disease, the early age at which the symptoms first appear, the peculiar ataxia starting in the legs and extending to the arms, the speech disturbances, nystagmus, curvature of the spine, talipes and late paralysis. With these symptoms it is also to be noted that usually the patella reflexes are lost, sensory symptoms are absent or very slight, trophic phenomena are not marked, vision and the eyes generally are intact, the intellect is clear, and the visceral and circu- latory systems show no disorder. The two diseases with which Friedreich's ataxia is liable to be confused are tabes dorsalis and disseminated sclerosis. Locomotor ataxia follows a history of syphilitic infection and appears later in life. It does not reveal any direct heredity and only slightly an indirect. It is very exceptional for the offspring of tabetic parents to suffer from Friedreich's disease. Tabes is to be distinguished by the more char- acteristic ataxic gait, the increase of Romberg's phenomenon when the eyes are shut, the marked pains and other sensory symptoms, the crises, the constant loss of the knee-jerk, the. Argyll-Robertson pupil, optic atrophy and the more prominent late arthritic and other trophic symptoms. Ataxic paraplegia is an adult disease, occurring usually between the ages of thirty and forty. It does not appear like Friedreich's dis- ease in several members of the same family. The patella reflexes are exaggerated, nystagmus is wanting and there is no marked speech trouble. It is to be remembered, however, that Friedreich's malady is, like ataxic paraplegia, a mere combined sclerosis of the posterior and lateral columns. The differences between the two are the result of the differences in the ages between the two sets of patients, the heredi- tary influence, and the greater intensity of the disease process in the one or the other column of the cord. Some cases of Friedreich's disease reveal cerebral and cerebellar symptoms. Indeed it has been thought by some, that the primary trou- ble originates in the cerebellum. They are not typical cases, however, though at times it is extremely difficult to differentiate them. Simple developmental inhibition of the cerebellum, an agenesis, such as Marie has described, can but rarely be separated. And yet in these rare cases the reflexes may be exaggerated, the ocular muscles may be paralyzed, the pupils may be rigid, the vision affected and the deglutition markedly disturbed. Tumor of the cerebellum produces headache, optic neuritis, ver- tigo, vomiting and epileptiform attacks. The gait is "drunken" and is distinctly cerebellar. There is no spinal sclerosis and other deformities such as are observed in Friedreich's trouble. THE NEURONIC DISEASES 45 1 Disseminated sclerosis sometimes can scarcely be differentiated, especially when the nystagmus, tremor and speech disorders are pres- ent. Multiple sclerosis is an adult's disease, however, developing usu- ally after twenty, it is not under hereditary influence, and it progresses remittently. The nystagmus is marked and the speech is more scan- ning in character than it is in hereditary ataxia. Other distinguishing symptoms of multiple sclerosis that aid in the differential diagnosis are exaggeration of the knee-jerks, the ankle-clonus, the intentional tre- mor, the rhythmic oscillations, the rigidity and spasticity of the limbs, the disturbances of the intellect, and the epileptiform and apoplecti- form attacks. Prognosis and Course. — Friedreich's disease is a chronic and progressive affection. It may last twenty, thirty or forty years. It never remits, though at times it seems to cease progressing for a while. The symptoms may abate and thus raise false hopes of a cure. The patient, as a rule, ultimately becomes bedridden and dies of nephritis, decubitus or some intercurrent malady. In regard to the duration of life the prognosis is favorable ; in regard to cure it is absolutely bad. Treatment. — There is no direct treatment known that will mod- ify the progress of the malady. Tonics are always in order, of course, as well as good regimen and proper hygiene. Nitrate of silver, arsenic, phosphorus, iodine, zinc, have all been tried and found wanting. In one case the constant current applied to the spine produced some bene- fit. It is a measure to be used, however, very guardedly. Sympto- matic treatment includes electricity and massage for the weakening muscles, a jacket for the curvature of the spine, and the closest atten- tion to all the functions of the viscera. AMAUROTIC FAMILY IDIOCY. In 1896 Sachs gave this name to a hereditary disease in which idiocy, spastic paralysis and blindness constitute the cardinal symp- toms. On account of the eye trouble, Tay, in 1881, first called atten- tion to the cases. The first autopsy on a case was made by Sachs, in 1887, who at that time attributed the manifestations to a simple agen- esis corticalis. Later on Kingdon reported an autopsy upon a case and was the first to attract attention to the similarity between the cases noted early by the ophthalmologists and those reported by Sachs. Cases and studies of the disease have since been reported by Claiborne, Patrick, Kuh, Jacobi, Hirsch, Peterson, Koplik, Cotton, Hirschberg and others. About thirty or more cases have now been put upon record. Etiology. — The affection seems to be distinctly hereditary and of the familial type of troubles. Peterson reported three members of one family afflicted with it. This would seem to declare most posi- tively for an inherited, rather than a mere congenital, cause, as would also the absence of all birth difficulties and the presence of a prog sive degeneration of the cellular elements of the entire nervous tern. The children are of the Jewish race generally, though not ex- clusively so. They are born normally, and at birth, and for the first few months thereafter, seem in every way to be healthy. The (lis- 452 THE NEURONIC DISEASES order makes its appearance, however, in the first six months of life as a rule. Pathology and Pathogenesis. — The autopsies have not been nu- merous enough to enable us to form a definite pathology. In some cases the findings have suggested a simple arrest of development ; in others an infective process, possibly of a meningo-encephalitic type ; and in still others a meningitis. No vascular or inflammatory changes have actually been discovered and if they were at all present before birth they must have completely disappeared. There are no signs of inherited syphilis or other diathesis. Hydrocephalus of a pronounced character has been noted. The entire nervous system reveals a con- dition of progressive degeneracy. In this connection it is suggestive that many of these children exhibit external degenerative stigmata. A dispute wages around the question as to the primary or sec- ondary nature of the neuronic degeneration. Many pathologists feel that simple agenesis will not wholly account for it. There is, as Kuh puts it, "a distinctly retrogressive and destructive change." Hirsch and Jacobi suspect that an infection, possibly derived from the moth- er's milk, is to be blamed for the trouble. Sachs reported two cases, in opposition to this theory, that were not breast fed. All such ex- planations have to combat the apparently hereditary and frequently familial features of the disease. In Cotton's case, the father's cousin's only child died in convulsions at twenty months, blind and idiotic. I have mentioned Peterson's three cases in one family. Symptoms. — Three great symptoms stand out with special prom- inence in amaurotic idiocy, namely, the mental deterioration, the spastic paralysis of all four limbs and the blindness from optic atrophy, with a peculiar appearance of the macula lutca. During the first five or six months of life the child seems normal in every respect. Then it is noticed that it gradually begins to fail in strength, to be disinclined to move, and to show a falling off of the intelligence. It grows apathetic, is at times restless and silly. In some cases convulsions and epileptiform seizures have occurred. Gradually it becomes stupid with such a hyperacuity of the reflexes that a sudden noise or a gentle slap will cause a great start, even a convulsion. During these mental changes there is an increasing spastic paraly- sis creeping over the muscles of the body. The head and trunk can- not be held erect, and the voluntary movements are distinctly athctoid in character. The associated movements and the reflexes are all mark- edly exaggerated. Tonic contractions occur in the whole musculature upon the slightest stimulation. The general spasticity and reflex ex- aggerations point to profound degenerative changes in the upper mo- tor segment. Towards the end there is complete paralysis. The paralytic symptoms involve the eyes also. Nystagmus, strab- ismus and unequal pupils may all be expected. Amblyopia is thus present at first but as the optic atrophy proceeds it disappears before the growing amaurosis. The appearance of the optic disc is pathog- nomonic in this disease and has been graphically described by Tay, Wadsworth, Carter. Beard, Wescott and others. It is so characteris- tic that, as a symptom of a general disorder, it stands alone, "grand, PLATJO II. Fundus oculi in amaurotic family idiocy. Drawn from life. ( After Beard. ) THE NEURONIC DISEASES 453 gloomy and peculiar." The atrophy of the optic nerve and retina does not appear to be as pronounced as one would expect. The change in and around the macula lutea is the absolutely diagnostic manifesta- tion. Beard describes it thus : Surrounding the fovea centralis, con- centric with it, and two or three times its size, is a liver-colored disc. This disc is the center of a zone of grayish-white which gradually fades away into the normal red-orange of the eye ground. As clear cut as a coin is this livid disc. It is not irregular in outline as is the case in acute inflammatory conditions, where the surrounding retina is infil- trated, nor is it cherry-red or carmine, as in those cases, but is dis- tinctly brownish. It is larger than the fovea ; that is to say, instead of marking the area which is occupied by the cones alone, it marks that which is devoid of the ganglion cells. The whitish zone that sur- rounds the center is also highly characteristic. It is nebulous rather than cloudy. If is almost white at the circumference of the liver- colored disc, thence gradually thins away to nothing, but is translucent and exhibits some color throughout. It does not obscure the retinal vessels which enter it. It only serves to make them more distinct by contrast, so that one is able to trace the tiniest of them right up to the central spot. In addition to those that have just been described, other symp- toms that have occasionally been noted in the disease are muscular flaccidity, tremor, normal and even subnormal reflexes, dysphagia, vomiting, hyperacusis and hyperesthesia. Some of the children have been badly nourished and rachitic. The head has been enlarged and the fontanelles still open. In some cases atrophy of the muscles has been observed in one part of the body while marked rigidity was noted in another part. Occasionally the tendency toward a localization of the symptoms, such as in a hemiplegic condition, has been strongly sug- gestive of a gross, focal lesion. The pulse rate is often rapid ; the temperature is practically normal. Diagnosis. — Given a case of idiocy with a general spastic paraly- sis and the typical appearance of the fundus oculi, the diagnosis is posi- tive and easy. Unless an ophthalmoscopic examination be made, the condition might well be mistaken for a diplcgic one due to some form of prenatal gross vascular lesion or Strumpell's type of meningo- encephalitis or polioencephalitis. In dealing with all of these various forms of infantile neuronic degenerations we are on the borderline between those that are due t<> mere developmental incapacity and those that owe their deterioration to some more gross, external influence, like hemorrhage, inflammation or vascular obstructions. Hence the difficulty in always determining the pathogenesis of these cases and the still more discouraging difti- culty of knowing how always to make an accurate diagnos On account of the heredity, and above all. on account of the unique ocular presentation, I am inclined to lean towards the view that this disease is a fundamental developmental defect, cue in which the newly-coined word of Gowers, abiotrophy, could be applied. All cases diagnosed as amaurotic family idiocy in which the typical eye grounds were not present, and especially all cases in 454 THE NEURONIC DISEASES which focal symptoms were pronounced and not typical eye grounds present, would be open to very great criticism. Infantile hemiplegia and diplegia would be more of a probability for such cases. Without the typical eye ground, amaurotic family idiocy may be mistaken for rachitic pseudo-paralysis, cerebellar tumor, tubercular marasmus and congenital blindness in an idiot. Prognosis. — This is decidedly bad. The children all die sooner or later, usually in a few months or a year or two, from convulsions and general marasmus. Herein this disease differs from the diplegic cases. The early death has even been spoken of as its fourth car- dinal symptom, so uniformly certain is it. This again suggests to my mind the profound biological origin of the degeneration here, as dis- tinguished from the secondary degeneration that accompanies encepha- litic and vascular processes. It is in harmony with what I have several times pointed out before, namely, that the prognosis of the primary neuronic degenerations are almost uniformly bad ; whereas the prog- nosis of the vascular troubles is, comparatively speaking, good. Treatment. — There is no treatment for this, any more than there is for any of the other forms of primary, progressive neuronic degen- eration. MULTIPLE NEURITIS AND NEUROMYELITIS. In this place would naturally fall some of the cases of toxic and infectious polyneuritis and ascending neuromyelitis. The latter are exceedingly rare and their existence doubted by some pathologists. It is believed that a toxic poliomyelitic primary degeneration, rather than an ascending degeneration, is the pathological basis. In many of the cases of peripheral neuritis, the lesion is believed to be a primary de- generation, without any interstitial inflammation, the result of the pro- found toxaemia. Lesions are found of a degenerative sort in the cord and even in the brain in these cases, so that the view is fast gaining ground that these cases represent a diffuse and chronic constitutional or general intoxication with a wide neuronic degenerative process tak- ing place throughout the entire nervous apparatus. The special changes in the peripheral nerve filaments are .believed to be due to the dis- turbed nutrition in the anterior horn cells and posterior ganglia, just as we believe is the case in other forms of infectious trouble. We are not ready, however, to relinquish the idea entirely that the toxin may act also directly upon the nerve terminals and so provoke a local de- generation there. All of this of course does not preclude the idea that some of the polyneuritides are toxic interstitial inflammations with secondary neuronic degeneration. This will be referred to again un- der the head of multiple neuritis discussed in the section devoted to diseases of the peripheral nerves. PROGRESSIVE INTERSTITIAL HYPERTROPHIC NEURITIS OP INFANTS. This is an interesting and rare affection, described recently by Dejerine and Sottas, which lies between the progressive muscular atrophies on the one hand and the frank, interstitial neuritides on the THE NEURONIC DISEASES 455 other. It seems to be a family disease and it always begins in child- hood. The clinical picture resembles a combination of locomotor ataxia with progressive muscular atrophy, so much so that Strumpeil believed the cases were nothing more than combinations of Fried- reich's disease with the hereditary peroneal type of progressive muscu- lar atrophy. The name indicates Dejerine's own idea, however, that it is a form of interstitial neuritis. The post mortem findings were a chronic interstitial neuritis decreasing in severity from the periphery to the center, and involving the posterior roots. There was also a sclerosis of both Goll's and Burdach's columns. Nerves were hyper- trophied and indurated. Symptomatically there were lightning pains, various sensory dis- turbances, an ataxic gait, sluggish pupils, myosis, nystagmus, static ataxia and a kypho-scoliosis. There were no electrical reactions of degeneration though there was a quantitative decrease in the electrical excitability. SECTION C. THE NON-NEURON1C DISEASES. Interstitial, Inflammatory Troubles with Secondary Neu ronic Degenerations. THE NATURE OF THE NON-NEURONIC DISEASES. In our earlier studies upon the histology of the Nervous System, we found that its wonderful network of neurones, making up the innumerable pathways along which the afferent and efferent impulses travel, was supported by a scaffolding, so to speak, of connective tissue, neuroglia, bloodvessels, lymphatics and epithe- lium. The neuroglia we remarked was a peculiar epiblastic tissue that belonged solely to the nervous system. Its function is purely susten- tacular and it is capable of undergoing hyperplasia and degeneration. An illustration of the former is the gliomata that are seen in the gray and white substance of the brain, the cranial nerves and the retina. These unique neurotic growths break down easily sometimes and leave huge cavities. Gliosis therefore is a degenerative process, affecting a special variety of extra-neuronic elements and causing them to press upon and infiltrate among the nerve-cells to their great detriment. Excluding the neuroglia, the extra-neuronic tissue of the nervous system is exactly the same as it is in every other part of the body. The connective-tissue elements and the changes they undergo, the bloodvessels and the disturbances which they are subjected to, differ in no respect here from what they do elsewhere. The inflammations, hemorrhages, neoplastic over-growths and infective processes are in themselves the same in the brain as they are in the lungs. A neuritis, as an inflammatory process, differs in no resped from an interstitial nephritis. An intra-cranial sarcoma is the same soli of a sarcoma that appears in the foot. The only thing, therefore, that makes these dis- eases, of the extra-neuronic type, especially interesting t<> the neurolo- gist is, that when they occur in the nervous system, they necessarily damage the neighboring neurones. The- damage is usually <>!" a me chanical sort and represents compression, laceration and distortion. This is seen in the inflammatory and hemorrhagic conditions. So times, however, they shut off the nourishment of the neurones. Hiis is observed in the embolic and thrombotic conditions. Sometimes they 458 THE NON-NEURONIC DISEASES even twist, stretch and displace the neurones in such a way that they cannot functionate properly. This is illustrated in the infiltrations of the gliomata and other neoplasms. Whatever the nature of the trouble may be in these extra-neu- ronic structures, the damage of the neurones themselves is strictly a secondary affair. The degeneration is therefore often spoken of as secondary. The disease which primarily starts it, and which is a dis- ease entirely outside of the neurones, is very properly therefore spoken of as a non-neuronic disease. As inflammation is really a regressive process, an effort of nature to rid and protect herself from some noxious influence, it tends always to disappear as soon as the harmful agent has been banished. It is thus more or less of an acute and temporary process and when prop- erly assisted it tends always toward recovery. In this way the prog- nosis of the inflammatory diseases of the nervous system is favorable. It must not be confused with the very unfavorable prognosis of the neuronic degenerations, whether these be primary or secondary. Hemorrhages, neoplasms, etc., each have their own special prog- nosis, which may be good, bad, or indifferent, but which should be care- fully distinguished from the prognosis of the mere resulting degenera- tion in the damaged neurones. In a broad sense then, we may say that the prognosis and treat- ment of the non-neuronic diseases are most favorable. The outlook in regard to the secondary neuronic changes which these diseases pro- duce, will depend entirely upon how severe and destructive the pri- mary disease was in its action upon the neurones. There are all grades of disturbance that may be produced in the neuronic elements ; and unless one keeps this fact well in mind, he will find himself treating hopeless and hopeful cases all alike with the ob- vious consequences. If an arterio-sclerosis, for instance, is amenable to treatment at all, the neurasthenic manifestations that often accom- pany it may be made to disappear by the treatment. A neurasthenia that is due to a defective heredity, however, and which really repre- sents an actual nervous incapacity from birth, cannot be expected to be removed by any sort of treatment. The trouble in the former case was a mere secondary result of mild degree from a non-neuronic dis- ease. Its prognosis, therefore, was good, and its treatment satisfac- tory. In the latter case the trouble in the neurones is essential and primary ; it is a positive neuronic defect or degenerative condition ; and hence it is irremediable by any human agency. It is too common an error, and one that oft leads to pessimistic views in regard to neurol- ogy, to say nothing of treatment, to confound the prognosis of these non-neuronic diseases with the prognosis of their secondary results, the neuronic secondary degenerations. For the sake of convenience, the non-neuronic diseases are usu- ally divided into those of the Spina! Cord, the Peripheral Nerves and the Brain. That this is an artificial division is shown by the fact that many of these diseases implicate the whole cerebrospinal axis at the same time, as, for instance, leptomeningitis and disseminated sclerosis. As the lesion, however, is more or less focal, and as the brain and THE NON-NEURONIC DISEASES 459 spinal cord have each their own localization symptoms, it is conve- nient to divide these diseases in accordance with these gross anatomical divisions. The order that will be followed in discussing them will begin with the bony encasement, taking up next the membranes, and finally the connective tissues and bloodvessels. At this point the neurones them- selves will have been reached and there will be nothing left but the neuronic degenerative troubles which were treated of first in the book, because they constitute, in their last analysis, the only true specific nervous diseases. PART I Non-Neuronic Diseases of the Spinal Cord. ANATOMY AND PHYSIOLOGY OF THE SPINAL CORD. SPINAL LOCALIZATION AND TOPOGRAPHY. SYMP- TOMS OF DISEASE OF THE SPINAL CORD. LUMBAR PUNCTURE. Anatomy and Physiology. — It is unfortunate, from the neurolog- ical point of view, that the artificial division of the nervous system into Brain, Spinal Cord and Nerves is so firmly engrafted upon the minds of the profession. These divisions are not made upon a neurological basis, but upon the bony encasements and other gross factors. Essen- tially, neither in anatomy nor in physiology, does the brain differ from the spinal cord. The cells and their inter-connections are arranged and functionate in practically the same way. Their processes inter- lace and run back and forth in such a manner between the brain and cord that the cerebrospinal axis is practically a unit. The nerves are merely the processes that connect this unit with the outlying parts of the body. They are not separate structures but merely extensions from and into the cerebrospinal organ. This cerebrospinal organ is made up of a series of bunches of neurone bodies and in this way carry out the dictum of Owen when he declares that a vertebrate animal, such as man is, is nothing but a clothed sum of .segments. The segmental character of the bony spine is obvious enough and indicates an evolution out of a lower or articulate form of life. The collections of neurone bodies, the ganglionic masses, that lie side by side serially all along the cerebrospinal axis, and which make up the so-called gray matter of the brain and cord, indicate also tin- mental character of the central nervous system. In the cord this is almost as obvious as it is in the bony spine. It Is suggested in the longitudinal series of spinal nerve-roots which have their origin from a series of super-imposed discs. In the brain the same serial disc-like arrangement obtains, but there it has been elaborately broken up and 40o the non-neuronic diseases the ganglionic masses have been, in the course of evolutionary devel- opment, scattered about in order to accommodate the enormously devel- oped condition of these frontal ganglia within the confined space to which they are limited by the more slowly developing skull. In its essential architecture the brain is only an enormous develop- ment of the frontal portion of the spinal cord, and the spinal cord is but a series of little, primitive brains lying side by side all the way along the spinal canal. To bring about harmony of action in this series of ganglionic de- posits of neurone-cell bodies, the latter must necessarily be joined, at least physiologically, together. They are. The processes from one ganglionic set of cells run up and down and here and there to arborize around the neurone cell-bodies in the neighboring and distant gangli- onic deposits. Some of these processes run through the nearby ganglia to reach the more distant ones, and in this way constitute the so-called gray commissures. Some of them run along just outside of the neighboring ganglia, and thus constitute the white associating, commissural and projection tracts. The serial deposits of cell-bodies,, so-called gray ganglia, gray matter, gray columns, and the long and short processes or branches from these cell-bodies that run here and there to connect them with one another, the ganglionic deposits with one another, and the ganglionic centers with the outlying parts of the body, all making up the so-called white matter of the commissures and columns, is the essential completed structure of the central nervous system. When we employ the gross divisions of the anatomists in our terminology, we merely mean to say that the brain is that part of the series of gray deposits, and that part of the white processes, that hap- pen to lie within the skull above the foramen magnum ; the cord is that part which happens to be found within the bony vertebral column ; and the nerves are that part of the processes that lie between the cerebro-spinal axis and the outlying organs and tissues of the body. This is a most important conception of the nervous system to grasp in order to understand the symptomatology of its diseases. For purposes of rough localization we may conveniently speak of brain, cord and nerve diseases, but as a matter of fact there are no dis- tinctive brain, cord and nerve diseases. There are no distinctive brain, cord or nerve functions. All the functions, all the abnormal symptoms of the nervous system, are neuronic functions and neuronic symptoms. Tf they happen to be the functions and the symptoms of those particu- lar neurones that lie within the skull we call them encephalic; if of (hose within the cord, spinal; if of those in the outlying nerves, ueu- ritic. Remember, however, that there is no sharp line of demarkation between these. Encephalic diseases produce spinal lesions and symp- toms and vice versa; spinal diseases produce nenritic lesions and symp- toms and vice versa. The greatest convenience of this artificial di- vision of the nervous system into brain, cord and nerves is in localizing the extra-new ronic lesions that constitute the inflammations, the vascu- lar abnormalities, the neoplasms, etc. THE X0X-XEUR0X1C DISEASES 461 I will now discuss that part of the nervous system commonly called the spinal cord. The spinal cord, spinal marrow > medulla spinalis, is that part of the cerebro-spinal organ that extends from the edge of the foramen magnum in the occipital bone to the lower part of the body of the first lumbar vertebra. The section just above it is called the medulla --Cervical por- -Dorsal por- .—Inferior por- tion of the spinal cord tion of the spinal tion of the spinal cord. (Hirschfeld). cord (Hirschfeld), and cauda equina (Hirschfeld). 1 anteroinferior wall of the fourth ventricle ; 2, superior peduncle of the cerebellum ; 8. middle peduncle of the cerebellum ; 4, inferior peduncle of the cerebellum ; 5, inferior portion of the posterior median columns of the cord; 6, glosso-pharynereal nerve: 7, pneumogastric ; 8, spinal accessory nerve ; 9, 9, 9, 9, dentated ligament : 10, 10, 10, 10, posterior roots of the spinal nerves; 11, 11, 11, 11, posterior lateral ,,v.- ; 12, 1:2, 12, 12. ganglia of the posterior roots of the nerves ; 13, 13, anterior roots of the nerves ; 14, division of the nerves into tiro branches ; 15, lower ex- tremity of the cord : 16, 16, coccvgeal ligament ; 17. 17. cauda equina ; I-VIII. cervical nerves ; I, II, III, IV-XII, dorsal nerves ; I, Il-Y, lumbar- nerves ; I-V, sacral nerves. oblongata. At the other end it terminates in a slender filament, tlu- filuin terminate or central ligament. It is about eighteen inches long and does not quite extend to the full length of the bony canal. It- relation to this canal varies slightly in accordance with the flexion* the body. It is inclosed in a theca, winch is longer and larger than itself and which constitutes the dura mater. Two other membranes en< 462 THE NON-NEURONIC DISEASES the cord also within the dura mater and are known as the arachnoid and the pia mater. Between the dura mater and the bony canal is a space containing much fat and loose areolar tissue, and large venous plexuses. The arachnoid is a double membrane, the outer layer of which hugs closely the under surface of the dura, while the inner layer unites somewhat with the pia. Many delicate fibres pass from one layer to the other. The dural layer is regarded by many histolo- FIGURE 94- 17 —Horizontal section of the cord and its envelopes, at the middle of a vertebral body (Schematic). 1, Spinal cord with 2, its anterior median fissure; 3. its posterior median fissure; 4, anterior roots; 5, posterior roots; 6. pia mater 7, ligamentum dentatum; 8, connect- ing fibres passing from the pia to dura mater; 9, visceral layer and 9', parietal layer of the arachnoid 10, subarachnoid space; 11, arachnoid cavity; 12, dura mater 13, periosteum; 13', external periosteum; 14, cellular tissue situated between the dura mater and the* wall of the vertebral canal; 15, common posterior vertebral ligament; 16, intra-spinal veins; 17> vertebra in section. (Testui.) gists as the arachnoid. The space beneath it then becomes the sub- arachnoid space and contains the cerebro-spinal fluid and is in direct connection with the lymph paths of the peripheral nerves and with the subarachnoid space of the brain. The pia mater is a highly vascular, lace-like membrane which closely invests the spinal cord and sends a thick, fibrous septa into the anterior fissure. It is important to note this, as certain differences between the diseases of the anterior and of the posterior parts of the cord are probably due in part to this arrange- ment of the pia and the corresponding difference of blood supply. With its investing pia mater, the cord swings within the subarachnoid space and cerebro-spinal fluid, being suspended from above and kept in position by the tooth-like ligamentum dentation that fixes it at THE NON-NEURONIC DISEASES 463 various points to its sheath and by the spinal nerve-roots that cross to reach the various intervertebral foramina. The cord is not of the same thickness throughout. In the upper part is the cervical enlargement where the great nerves of the arms come off, extending from the upper limit of the cord to about the first or second dorsal vertebra, while in the lower part is the lesser lumbar enlargement, where the nerves of the lower extremities orig- inate and extending from the tenth dorsal vertebra down to its greatest width opposite the twelfth dorsal vertebra on to its final tapering into the thin conns medullar is. Above the nerve roots pass almost horizontally across the sub- arachnoid space ; in the mid-dorsal region they incline strongly down- ward and thus run for a short distance obliquely across the space ; in the lumbar and lower regions they become almost vertical and run quite a long distance beside the cord before they pass out through their respective foramina. This is to be carefully noted as having a most important bearing upon clinical localization of the lesions within the cord. This mass of almost vertical nerve-roots hanging round about and concealing the terminal, central ligament of the cord con- stitutes the cauda equina, or horsetail. The Alum terminate is a pro- longation of the pia mater, inclosing for about a half of its length the lower end of the central canal of the cord. In the portion below the canal there are connective tissue, blood vessels and three or four minute bundles of medullated nerve fibres, some of which have a few ganglion cells attached. According to Rauber these represent rudimentary coccygeal nerve-roots, but they have no real connection with the proper coccygeal nerves. Superficially the cord is seen to be longitudinally divided by fissure-like depressions, along which emerge the nerve-roots. As the nerve-roots form themselves on each side into an anterior and a posterior set, the cord is thus grandly divided into anterior, lateral and posterior columns. The anterior column is again divided into two 1>\ the anterior median fissure into which dips the pia mater. The pos- terior column is also divided into two by a so-called posterior median fissure, which is not a true fissure, but merely a septum of connective tissue and blood vessels projected down into the cord from the pia mater on its surface. Thus each half of the cord has an anterior column in front of the anterior nerve roots, a later column between the anterior and posterior nerve-roots and a posterior column behind the posterior nerve-roots. We now begin to see thai the cord is a bilateral organ or represents two sides of the nervous system, identical in all respects. This is further demonstrated most beautifully when a transverse section of the cord is made and the ends of the section are examined. If we were to unite the- anterior and posterior median fissures with one cut of the knife longitudinally we would have the two halves of the cord separated, but presenting exactly the same picture. Before taking up the description of the inner appearance of the cord, it may be well to devote a paragraph to its blond supply. The upper part of the cord is nourished by branches from the vertebral. 464 THE NON-NEURONIC DISEASES ascending cervical and superior intercostal arteries. The lower part receives its supply of blood from the dorsal intercostal, lumbar and sacral arteries. These all send off branches which penetrate the inter- vertebral foramina and are finally spread out on the pia mater and the cord. The anterior and posterior spinal arteries run along the cor- FIGURE 95 (A). Format ret /'cut \ First Cervical. Tntermed^ cells Fasc ct/neat* Faifc. grac ///j N. Second Cervical. Clark's Coh Su6st. gelatin etttr. Subst. gefatm. \ r, j- s Col. post. '^termed. Cells ~Pott Toot* Cent, canal 1 Mnz.rvot Fissum at%t . Comml3.ee/ts Csmm,ss. cetl^ Med. motor cells -JKotor tells Zat. motor cells Lateral horn Sixth Cervical. First Dorsal. —Showing Arrangement op Gray and White Matter at Different Levels op the Cord (After Merkel). responding surfaces of the cord and send off everywhere small branches. The branches from the intercostal, lumbar and sacral arteries communicate laterally with the branches from the anterior and posterior medians. Thus are effected the arterial chains. From the anterior spinal artery at the bottom of the anterior fissure minute THE XOX-XEUROXIC DISEASES 4 6; branches enter and supply the greater part of the central gray matter of the cord. They do not supply the posterior horns. From the periphery of the cord other branches from the arterial chains dip into the white matter and the posterior horns. The artericr sulci come from the an- terior tract, pass into the cord and reach the anterior commissure. Here as arterial sulco-commissurales they enter the anterior horns and supply them and a small area of surrounding white matter. FIGURE 95 (B). Clark s col. Post, column- CfarKs Col. Ant. Co L Eighth DorsaL oot Tost, root Ant. root First Lumbar. Claries col. Coajmiss.ce/ls Ant root "Zaterol eel' 9 Third Sacral. Median cells First SacraL — Showing Arrangement of Gray and White Matter at Different Levels of the Cord (After Merk«l). Clarke's column is supplied by branches from these anterior arteries. Most of the white matter and the posterior horns are supplied by the vasocorona or minute arteries which dip directly into the cord from the pia mater. The practical points about all these arteries of the cord arc that the anterior and peripheral set do not anastomose, that they arc ter- minal arteries, that the anterior arc larger than the peripheral and are so much larger than the corresponding venous system that their blood pressure is probably very high. 466 THE NON-NEURONIC DISEASES The first sight to catch the eye, after making a transverse section of the cord, is the arrangement of the gray and white matter. The gray matter is in the center and by means of the cross bar of the H-shape connects the two halves of the cord. Outside of the gray matter and constituting the edge of the cord spreads the white matter. As the ends of the H-like gray matter correspond with the anterior FIGURE 96. TRANSVER8E SECTION OP HALF THE SPINAL MARROW IN THE LUMBAR - ENLARGEMENT (Allen Thomson), f This is a seinidiagram- matic representation taken from a specimen, and founded in part on the statements of Lockhart Clarke and of Kolliker. 1, anterior median fis- sure \ 2, posterior median fissure ; 3, central canal lined with epithelium ; 4, posterior commissure ; 5 % anterior commissure ; 6, posterior column ; 7, la- teral column ; 8, anterior column ; 9, fasciculus of posterior nerve-root enter- ing in one bundle, a part of which passes into the posterior cornu, and a part into the posterior column ; 10, fasciculi of anterior roots ; a, a, caput cornu posterioris with the gelati- nous substance of Rolando ; b, the cervix cornu ; c, pos- terior vesicular column of Clarke ; t$ the right of d t the group of cells in the intermedio - lateral tract ; r, e, fibres of the anterior roots, entering the anterior cornu, and passing through among the cells ; «', fibres from the anterior roots which decussate in the anterior commissure ; <", external fibres from the roots running round the outside of the anterior grey cornu towards the lateral columns ; /, fibres from the posterior commissure and from the pos- terior cornu running towards the anterior. Three groups of cells are seen in the anterior cornu in this region of the cord ; and from thfir position may be described as external, internal, and posterior. (From Quain's Anatomy.) and posterior nerve-roots respectively, we see that the white matter is naturally divided, as the nerve-roots cross it and enter the gray matter, into the anterior, lateral and posterior columns that made up the superficial arrangement of the cord. If we make other sections of the cord we find that the general appearance remains the same, b'-t that the relative amounts of gray and white matter differ at different levels. In the cervical and lumbar enlargements we note especially THE XOX-XEUROXIC DISEASES 467 the relative increase of gray matter ; and high up in the cervical region it is beginning to lose its regular H-like outline. Beyond a cellular-like appearance in some parts of the gray matter and perhaps the greater vascular supply of this over the neighboring white matter, the appearance of the central canal in the middle and some other unimportant features, we cannot learn much more about the cord from a mere macroscopical examination. The moment we turn the microscope upon the picture, however, and especially if we avail ourselves of the marvelous staining methods FIGURE 97. Part of the gray matter of the anterior horn, with the neighboring portion of the lateral tract, showing the fine medullated fibres which pass from the lateral tract into the gray matter, sur- rounded by (pigmented) nerve-cells. (Stained by Weigert- Pal's method.) (After Kolliker.) of modern histological research, we are confronted with a most beauti- ful and suggestive view. The gray matter is at once seen to be made- up of a great mass of neuronic cells with their interlacing proces all supported by a matrix of rich neuroglia, connective-tissue and vas- cular elements. The cells are variable in size, seem to be specially grouped together here and there and send their processes into almost every imaginable direction. The processes that run horizontally a< the cord will, of course, be seen in their full length like fine hairs. Those that run up and down in the cord will, being cut across, appear as minute spots with a delicate ring of white matter around them. Those that pass here and there obliquely will, of course, present a more or less oval appearance with the spot in the middle. 4 68 THE NON-NEURONIC DISEASES It is clear then that we have in the gray matter of the cord a repre- sentative ganglion or bunch of neurone-cell bodies. A full description of a neurone will be found in an earlier part of the book in a section headed the Neurone and the Neurone Doctrine. FIGURE q8. Diagram of the Relations op the Cells and Fibers op the Spinal Cord. (Baker, after Lenhossek.) The right side shows the cells of different classes found in the cord, and their processes. The left side gives the processes of cells whose bodies are either beyond the cord or at other levels, with the distribution of their collaterals. a, a. Motor cells of the anterior horn. c. Commissural cells. d. Golgi commissural cell. e. e. Columnar cells of anterolateral column. f. f. Columnar cells of posterior column. g. Golgi cell of posterior horn. 1. Fibers of posterior root forming the ant*ro-posterior reflex, tract. 2. Fibers passing to the column of, Clarke. 3. Commissural fibers of posterior root* 4. Fibers that enter the posterior horn, k, k. Collaterals of antero-posterior column. 1, 1. Collaterals from the pyramidal tracts. The cells in the anterior part of the gray matter are larger and more angular than they are in the middle or posterior part. Moreover, they arrange themselves in little groups that can almost be observed with the naked eye. These groups are seen to lie anteriorly and antero-laterally and from them run processes that finally pass out through the anterior nerve-roots. Another group is seen near the THE NON-NEURONIC DISEASES 469 outer and mid-lateral part of the gray matter. The course of their processes is not quite so distinct. In the middie of the gray matter just a little back of the end of the cross-bar of the H is another group of small cells which make up the column of Clarke. And finally, in the posterior parts are scattered groups of quite small and roundish cells, intermingled with processes that come in from the posterior nerve-roots. On account of the gross and minute differences thus observable in the gray matter of each half of the spinal cord, it will be convenient for us to use the names of the old anatomists and speak of the anterior part as the anterior horn, the middle-lateral part as the middle horn, and the posterior part as the posterior horn. If we look upon the various groups of cells, just described, m their longitudinal distribu- tion up and down the whole cord, we may justly speak of them as vesicular columns. In the anterior horns, therefore, are two vesicular columns, an anterior and a mesial. In the lumbar region there is a third or posterior vesicular column of the anterior cornu. Laterally between the two horns is the inter -me dio-lateral vesicular column, and at the base of the posterior horn, internally, is the posterior or vesicular column of Clarke. In the posterior horns the cells are not so well grouped as to be contemplated in the light of vesicular columns. When we turn the microscope upon the white matter of the cord we note a marked change in the appearance of the field. There is a complete absence of ganglionic cells and instead a well-packed mass of transversely cut fibres. They appear as little rings with a stained dot, the axis cylinder, in the middle of them. The position of the dot in the ring and the shape of the ring will depend, of course, upon whether the fibre happened to be cut transversely or obliquely across. These fibres vary in size, the largest of them appearing in the posterior part of the lateral columns, and the smallest in the neighborhood of the processus reticularis. It is clear that they are the medullated processes from some neurone bodies embedded somewhere in the ganglionic sub- stance of the cerebro-spinal axis. Around the outer edge of the sectional field of the cord we notice a layer of neuroglia)- tissue. It sends septa transversely into the cord and thus subdivides the white columns into a number of smaller columns, which have a relatively fixed position. The neuroglia is relatively abundant in a ring that surrounds the central canal. Along the line of the posterior roots the peripheral neuroglia enters into the posterior horns and there expands to form the substantia gelatinosa of Rolando. The minute description of the histological elements that we have just been observing, including the neurones, the neuroglia and the rest of the substentacular tissue, will be found in the chapter headed the Neurone and the Neuronic Architecture. We have now to analyze further, if possible, these columns and vesicular masses of gray matter. The methods that enable us to «!-< this are experimental and pathological. The degeneration, r< tion and development of the neurone, together with tin- happy dis- covery that certain parts of the neurone stain different!) with different 470 THE N&N-NEURONIC DISEASES clinical reagents, enable us to trace out with an exquisite beauty of detail the paths of the various impulses through the cord. This has been referred to partly elsewhere. The neurone body being the center of nutrition for the entire neurone, if it is destroyed the neuraxone immediately begins to break down and in a short time is entirely gone. Sometimes we can accom- FIGURE 99- —Transverse section through half the spinal cord, showing the ganglia : A, anterior cornual cells; B, axis-cylinder process of one of these going to posterior root; c, anterior (motor) root ; D,, ^posterior (sensory) root; E, spinal ganglion on posterior root ; F, sympathetic ganglion ; G, ramus communicans ; H, posterior branch of spinal nerve ; 7, anterior branch of spinal nerve ; a, long collaterals from posterior root-fibers reaching to anterior horn ; 6, short collaterals passing toClarke's column ; c, cell in Clarke's column sending an axis-cylinder (d) process to the direct cerebellar tract: e, fiber of the anterior root; /, axis-cylinder from sympathetic ganglion cell, dividing into two branches, one to the periphery, the other toward the cord ; g, fiber of the anterior root terminating by an arborization in the sympa- thetic ganglion ; h, sympathetic fiber passing to periphery (Ramon y Cajal). plish this result by direct experiment. More often and better, dis- ease accomplishes it for us. It is easy to be seen that if by an ex- perimental or pathological insult, a minute focal lesion is established in a mass of neuronic gray matter, the processes from the damaged neurones can be traced throughout their long course by the degenera- tion they undergo. Both primary and secondary neuronic diseases have Uvii most invaluable aids to histology and physiology in this way. When a neuraxone is cut across artificially or severed from its cell-body by a pathological process, that part of the neuraxone that has been cut off undergoes complete and rapid degeneration. The THE NON-NEURON IC DISEASES 471 proximal' part undergoes a much slower and partial degenerative process. If a great bundle of neuraxones be thus treated, the appear- ance in the variously lengthened severed ends will be that of a de- generation that seems to begin at the place of damage and travel towards the periphery. This is known as secondary, or Wallerian, degeneration, after the man who first called attention to it. The degeneration, it is to be again repeated, is always in the direction away from the related cell body or center of nutrition. When the white matter of the cord is cut across it is noticed that in some parts of the columns the degeneration travels upward, in other parts down- FIGURE 100. A SMALL PORTION OF A TRANSVERSE 8E0TION OF THE HUMAN SPINAL CORD. Highly magnified. (B. A. S.) a, a, superficial neuroglia ; b, b, transverse section of part of the lateral column of the cord in which the dark points are the axis-cylinders, and the clear areas the medullary substance of the nerve-fibres : the superficial neuroglia is seen to exhibit the appearance of a fine network in which numerous nuclei and one or two corpora amylacea (c. a) are embedded, and to extend inwards among the nerve-fibres. (From Qnain's Anatomy.) ward. Obviously, then, we have to look for the cell-bodies of the former below the cut and of the latter above the cut. In this way the afferent and efferent tracts of the cord have been most accurately traced out and their cellular origin definitely located. After a large number of these tracts were thus mapped out and demonstrated by experimentation upon the lower animals, and by disease in man, it was discovered that they did not all develop simul- taneously and to the same degree in the embryo. Some appeared and reached maturity earlier than others. The acquisition of the myelin sheath was taken as a fair evidence of the full development <>\ a neurone and its neuraxone. This process of inclination then not occurring at the same time in all tracts of the embryo, it was only necessary to get a sufficiently large series of embryos representing dif- ferent ages and stages of development, and to study them, in order to follow out the lines of location of these particular tracts. Tin's was the embryonic method of Flechsicr to which we are indebted for much 472 THE NON-NEURONIC DISEASES of our knowledge of the minute pathways in the nervous system, es- pecially in the brain. Comparative zoology and embryology, coupled with the observa- tions of evolution and biology, have lent their aid and given much valuable information. Finally, the marvelous staining methods, for which we are hi debt to Golgi, Ramon y Cajal, Nissl, Marchi, Van Gehuchten and a host of enthusiastic and untiring investigators, have laid bare for us the architecture of the very citadel of thought and life. Certain reagents, as bichromate of potash, osmic acid and nitrate of silver, methylene blue, hematoxylin, when properly used, cause changes of coloration in certain parts of the neurones. It would be out of place to consider in detail here these various methods, but their great im- portance in giving us a proper conception of the structure of the nervous system must be acknowledged. It was out of the results of the employment of these methods that the neurone doctrine was formulated, one of the grandest and most useful scientific generaliza- tions ever made in the realm of medicine. As a lesult of all these methods of investigation we have learned that the white matter of the cord consists of three kinds of tracts, projection tracts, commissural and associating. The anterior commissure, just at the bottom of the anterior fis- sure, transmits neuraxones that run from one side of the cord to the other. The association or short tracts make up all of the anterior, lateral and posterior columns that are not included in the projection tracts. They connect one level of gray matter with another and thus, like long and short stitches, sew, as it were, the various ganglionic segments of the cord together. The parts of the columns where these association fibres exist reveal, when cut across, both upward and downward de- generation. The projection tracts are several in number and need special de- scription. Those that connect the brain with the cord are motor in function and degenerate downwards. Those that connect the peripheral nerves of sense with the cord, and certain parts of the cord with the brain, are sensory and degenerate upwards. These tracts consist of the following : 1. Direct or Uncrossed Pyramidal Tract. Turck's Column. This is a small tract that lies immediately on either side of the median fis- sure. It only extends as far as the middle of the dorsal region. Its fibres are the processes of the cells in the Rolandic area of the cerebral cortex. They do not change their direction until just before they terminate, when thev bend somewhat at a right angle possibly, cross to the other side of the cord through the anterior commissure, and finally arborize at various levels around the cells in the anterior horns of the upper part of the cord. 2. Indirect or Crossed Pyramidal Tract. This is rather a large tract, found in the posterior part of the lateral columns. It nowhere approaches the edge of the cord and, except at its postero-internal edge, it is separated by the lateral limiting ZO ne from the posterior THE XOX-XEUROXIC DISEASES 473 r+ & P >~ g rj 5 rt -!_!-"• X <__ fl K-rs c " n p-r<~ w " ° 3 *d «r«< 3 — p •< n -: ~ ~ 5 73 S* P ~ | 73 r» — -1 — ~ » 2- — / * 5" o 474 THE NON-NEURON IC DISEASES horn. It represents the neuraxones of the cells of the Rolandic cerebral cortex. These processes all cross at the decussation of the pyramids, pass down the opposite side of the cord and then arborize around the cells of the anterior horn at various levels all along the cord. FIGURE 103. Diagram of the tracts of the cord. The crossed and uncrossed pyramidal tracts represent respectively ninety and ten per cent, of the entire cerebro-spinal motor paths. In spite of the misleading name of the latter, they both carry inhibitory, controlling and volitional impulses from one side of the brain respective- ly to the opposite vesicular columns of the anterior horn of the cord. Speaking from the neuronic standpoint, they constitute the upper THE NON-NEUROXIC DISEASES 475 motor segment. There are no other projection motor paths in the cord that we know of. 3. Poster o-median Tract. Golfs Column. Funiculus gracilis. This is a narrow, wedge-shaped tract that lies immediately next to the median septum in the posterior column. The point of the wedge does not quite reach to the gray matter anteriorly. The column itself does not extend much below the mid-dorsal region. It degenerates upwards and is thus an afferent or sensory tract. It represents the sensory neuraxones that come into the cord by way of the posterior roots of the same side and that pass up to arborize around the cells of the nucleus gracilis found at the top of the column in the medulla. It -carries special forms of sensation from the muscles, the articulations -and the tendinous sheaths of the same side. 4. Postero-lateral Tract. Burdock's Column. Funiculus cunc- sterior ganglion, on through the posterior root of the column of Burdach, the posterior horn, to the anterior horn of the same side, where it is 478 THE NON-NEURONIC DISEASES transferred to a motor cell and sent on out over its process, through the anterior root, along the motor nerve, to stimulate the correspond- ing muscle into action. Such is the essential story of every form of reflex action. Corresponding sensory and motor areas, as, for instance, the skin and muscles of the same leg, are always the subject of it. There are reflexes a little more complicated than these, however, and illustrate the old physiological laws of Pfiuger in regard to the spread- ing of the reflex action. If the sensory excitation is a strong one in one limb, the opposite limb will respond. If a still stronger stimulus be used, the upper limbs will manifest the action. This, of course, is due to the close association between the special segments in the higher classes of animals. I have already hinted at the fact that the reflex arc, in a normal individual, is not a liberated circuit. Just exactly how it is under the control of the brain we do not know, but we believe that certain reflex-inhibitory processes pass from the cerebral cortex, or rather its cells, down through the pyramidal tracts to arborize around the reflex- motor cells in the anterior horns. In this way every reflex arc is inhibited from above. We are thus saved from being the victims of every stimulus that attacks us and that otherwise would keep us in a state of everlasting movement and muscular rigidity. It is obvious, therefore, that a reflex may be modified by a con- siderable number of, and variously localized, lesions. In the first place, any injury to the arc itself, upon the motor or sensory side (neuritis), or at the center (poliomyelitis), will diminish or abolish it. In deep coma it is lost. Any disease that increases the inhibitory force from above may diminish it, as is seen in some cases of hysteria. On the other hand, how easy it is to comprehend that it may be exaggerated by any disease that suppresses the inhibitory force from the brain. Hemiplegia and pyramidal degenerations generally are characterized by exaggeration of the reflexes. Slight irritation of the posterior roots, when the arc is not broken, also increases it, so does strychnine and tetanus and the neuroses, and certain diffuse diseases above the arc which exalt the excitability of its center. There are many forms of reflex that we could probably trace out, but in clinical practice the skin, or superficial, and the tendon, or deep, reflexes are those we are most interested in. In the former the skin is irritated ; in the latter some muscle tendon is tapped. The termination of the reflex may be in a gland, a blood vessel or a muscle. I have elsewhere described the more common forms of reflex and the methods employed in eliciting them. Closely related to the reflex is the automatic function of the cord. In fact, this function is a form of reflex in which the sympathetic sys- tem relieves the brain and makes the management of the reflex appear all the more independent and automatic. In the cord are found here and there deposits of cells in the gray matter that clearly preside over certain actions of the pupil, micturition, defecation, erection in the genital apparatus and parturition. In the lower cervical and upper dorsal region of the cord, near the THE XOX-XEUROXIC DISEASES 479 origin of the first dorsal root, is the ciliospinal center, or group of cells which regulate automatically the muscle that dilates the pupil and the FIGURE 104. ^[■CaaUXo AtfcuU 1/ the sensation. A lesion of a single pair of posterior nerve roots maj not even be noticed on account of sensory disturbance; and a small lesion in a particular segment may not cause any anaesthesia in the area of distribution of the corresponding nerve. 4 THE NON-NEURONIC DISEASES FIGURE 105. MOTOR. St.-Mastoid Trapezius Diaphragm SENSORY. Neck and scalp Neck and shoulder Shoulder Arm Hand Front of thorax Ensiform area Abdomen y (Umbilicus 10th) r Buttock, upper \ part I Groin and scrotum "i front f outer side ThiRh front inner side Leg, inner side f Buttock, lower part Back of Thigh Leg and Foot r except 1 inner pan Perinaeum and Am from coccv; nub REFLEX. Scapular Epigastric Abdominal Cremasteric \ Knee rejiex Gluteal Foot clonus Plantar A Diagram Designed to Show thb Relations op the Vertebrae to the Spinal Segments, and of the Spinal Nerves to the Motor, Sensory, and Reflex Functions of the Spinal Cord. (Gowkrs ) THE XOX-XEUROXIC DISEASES 487 For our knowledge of these segmental and peripheral correspond- ences we are indebted to Starr, Mills, Ross, Thorburn, Sherrington, Bruns, Sachs and others. Following their teachings, I will give the muscles, areas of sensation and the reflexes, and the corresponding FIGURE 106. LOCATION OF THE SEGMENT FOR Sensibility. Motility. Smell Thalajyn Muscles Sight Head Eyes Face Tongue I s 3 'Hearing \ £ Pharynx J * Larynx (Esophagus Viscera of Chest and Abdomen Occiput. Shoulder g> C Musculo spiral nerve i Median nerve. & I Ulnar nerve ... II Pulv .Sphincter of the iris ciliary -Internal rectus, levator palpebrtt- rectus, inferior and superior 'Inferior oblique .Superior oblique Masseter, temporal, pterygoids [(upper facial) External rectus, frontalis, orbicularis of Mimetic face muscles (lower facial) Mouth and throat muscles Laryngeal muscles Tongue muscles Sterno-cleido mastoid Deep neck muscles Trapezius, Serratus anticus Diaphragm Deltoid, Diceps, pectoralis (clavicular part) rachialis anticus, supinator longus triceps, latissimus dorsi, pectoralis (cos Extensors of the h' ngers and phalanges j Flexors of the fingers and phalanges Interossei and lumbricales , Thenar, hypothenar \ Fore- arm. Thorax Epigastrium Gluteal reglou Inguinal region Thigh Leg Hip anterior external posterior internal anterior Foot rotuin, Penis, etc. Rectum, Bladder Intercostal muscles Back muscles AlKloniin.il muscles — _ Ueopsoas Snrtoriiis ...... Adductors Abductors Quadriceps ' Flexors Extensors Thigh Leg r„nrl , xtensors of foot and boaa rthMtl __, rlneal ) Bladder V Musculature Rectum ) (After Jakob.) segments from which they arc innervated. The accompanying e* quisite diagrams of Head will assist in showing the segmental sensory innervations. It is to be remembered, however, that there arc no sharp lines in nature between the various areas as the picture indie Ope area blends into its neighbors, hence the sensor) localizations 488 THE NON-NEURONIC DISEASES must be made guardedly. The reflex and muscular localization mani- festations are somewhat more distinctly defined. Second and Third Cervical Segments. The sterno-mastoid, trapezius, scaleni and deep neck muscles, together with the diaphragm, are innervated from these segments. Sensation is perceived through them in the back of the head up to the vertex, and in the neck. The sensory nerves involved are the occipitalis major and minor, auricularis magnus, superficialis colli and the supraclavicular. The so-called hypochrondrium reflex is possibly dependent upon them. This con- sists of a sudden inspiration produced by a quick pressure beneath the lower border of the ribs. This is not an important reflex. It is prob- ably due to diaphragmatic excitability. Fourth Cervical Segment. The diaphragm, deltoid, biceps, coraco- brachialis, supinator longus, rhomboid, supra- and infra-spinatus are all partially innervated from this area. The . sensory distribution in- cludes the neck, anterior surface of the shoulder and outer surface of the arm ; and the following nerves, supra-clavicular, circumflex and musculo-cutaneous externally. An important reflex is the pupillary, involving the fourth cervical to the second dorsal segments, and re- vealed in a dilatation by irritation of the neck. Fifth Cervical Segment. This takes part in the innervation of many muscles, especially those of the arm. They are the deltoid, biceps, coraco-brachialis, brachialis anticus, supinator longus, supinator brevis, deep muscles of the shoulder blade, rhomboid, teres minor, clavicular portion of the pectoralis and the serratus magnus. The sensory area includes the back of the shoulder and arm and the outer side of the arm and forearm to the wrist. The nerves concerned are the supra-clavicular, circumflex, external and internal cutaneous and posterior spinal branches. The scapular reflex, or contraction of the scapular muscles by irritation of the skin over the scapula, implicates the fifth cervical to the first dorsal segments. The supinator longus reflex is the flexion of the forearm by the tapping of the tendon of this muscle. Sixth Cervical Segment. Here again a large number of muscles are innervated — namely, the biceps, brachialis anticus, subscapular, clavicular pectoralis, serratus magnus, triceps, extensors of the wrist and fingers and pronators. The sensory areas are the outer side and front of the forearm, the back of the hand along the radial distribu- tion. The nerves chiefly concerned are the external cutaneous, internal cutaneous and radial. In the fifth to the sixth cervical segments we find the center for the triceps reflex. If the tendon just above the elbow be tapped, the forearm jerks in extension. The sixth to the eighth cervical contain the center for the posterior wrist reflex, ex- tention of the hand occurring when the tendons are struck. Seventh Cervical Segment. Here we find the innervation of the long head of the triceps, the extensors of the wrist and fingers, the pronators of the wrist and its flexors, the subscapular, the costal part <>! the pectoralis, the serratus magnus, latissimus dorsi and teres major. The sensory innervation includes the radial distribution in the hand, the median distribution in the palm, thumb, index and halt the middle THE NON-NEURONIC DISEASES 489 finger. The nerves involved are the external cutaneous, internal cutaneous, radial, median and posterior spinal branches. The anterior wrist reflex depends upon the seventh to the eighth cervical, the wrist undergoing flexion when its anterior tendons are tapped. The seventh cervical to the first dorsal contain the centers for the palmar reflex. The fingers close when the palm is stroked. Eighth Cervical Segment. Here are found the origin of stimula- tion for the long head of the triceps, the flexors of the wrist and fin- gers and the intrinsic muscles of the hand. It receives sensation from the ulnar area of the hand, back and palm and the inner border of the forearm. The nerves that preside over this are the internal cutaneous and ulnar. First Dorsal Segment. This innervates the extensors of the thumb, the intrinsic hand muscles and the thenar and hypothenar mus- cles. The nerves involved are chiefly the internal cutaneous and nerve of Wrisberg, or lesser internal cutaneous. The skin chiefly of the inner side of the forearm and arm up to almost the axilla is the sensory area involved. Second Dorsal Segment. Sensation is supplied through this in the inner side of the arm near and in the axilla and the nerve impli- cated is the intercosto-humeral. Second to the Twelfth Dorsal Segments. These stimulate the muscles of the back and abdomen and the erectores spins. They pre- side over the sensation of the skin of the chest and abdomen, in bands running around the body and downward. These bands correspond to the spinal nerves. As I have said, the lines between them are not sharp. The sensation from the upper gluteal region is also marked by these segments. The nerves that are concerned are the intercostals and dorsal posterior nerves. Two reflexes of importance are here represented. The epigastric, involving the fourth to the seventh dorsal segments, is the retraction of the epigastrium by the tickling of the mammary region; the abdominal, involving the seventh to the eleventh dorsal, is the retraction of the abdomen, produced by the stroking of its sides. First Lumbar Segment. The muscles concerned are the ilio-psoas, the rectus and the sartorius. The innervation of the skin involves that over the groin and in front of the scrotum. The nerves are the ilio-hypogastric and ilio-inguinal. The cremasteric reflex, under the control of the first to the third lumbar segments, is revealed in the retraction of the scrotum by the stroking of the inner side <»t" the thigh. Second Lumbar Segment. The muscles here stimulated are the ilio-psoas, sartorius and quadriceps femoris. The skin on the outer side of the thigh, by way of the genito-crural and external cutaiM nerves, is here innervated. That most important of ail reflexes, the patella, in which the knee jerks as the result of a tap on the patella tendon, is here represented. Third Lumbar Segment. Tin- muscles that receive their n supply thence are the quadriceps femoris, anterior part of the bic inner rotators of the thigh and the abductors of the thigh. The skin area affected is the front of the thigh. The nerves are the middle cutaneous, internal cutaneous, long saphenous and the obturator. 490 THE XOX-XEUROXIC DISEASES FIGURE 107. VIII Sensory arras of the skin and their segmental representation in the spinal cord. (After Head.) THE NON-NEURONIC DISEASES 491 FIGURE 108. C VII Sensory areas of the skin and their segmental representation in the spinal cord. (After Head. ) 492 THE NON-NEURONIC DISEASES Fourth Lumbar Segment. Here the muscles represented are the- abductors of the thigh, the adductors, the flexors of the knee, the tibialis anticus and the peroneus longus. Sensation is received from the inner side of the thigh, leg and foot; and the nerves involved are the internal cutaneous, long saphenous and the obturator. The gluteal is an important reflex represented here, though the fifth lumbar seg- ment also is involved. Stroking the buttock causes a dimpling in its fold. Fifth Lumbar Segment. Here we find the source of the nerve supply of the outward rotators, the flexors of the knee and of the ankle,, the peronei and the extensors of the toes. This segment is in corre- spondence with the back and outer side of the leg and ankle, the sole of the foot and the dorsum. The nerves implicated are the external popliteal, external saphenous, musculo-cutaneous and plantar. The Achilles-tendon reflex, another most important reflex, is here centralized. A tap on the tendon causes the foot to extend on the calf. First and Second Sacral Segments. The muscles represented here are the flexors of the ankle, extensors of the ankle, long flexor of the toes and intrinsic muscles of the foot. The back and outer side of the leg and ankle, the sole of the foot and the dorsum are sensitized from these segments. The nerves involved are the external popliteal,, external saphenous, musculo-cutaneous and plantar. From the fifth lumbar to the second sacral segments is the center for the plantar reflex. When the sole of the foot is tickled the toes are all flexed and the leg is retracted. Third, Fourth and Fifth Sacral Segments. These represent the perineal muscles and the muscles of the bladder, rectum and external genitals. Here are also located the automatic reflex vesical and anal centers ; also the genital and parturition centers. From these same segments the skin of the back of the thigh, the anus, the perineum and external genitals is innervated. The nerves involved are the small sciatic, pudic, inferior hemorrhoidal and inferior pudendal. Fifth Sacral and Coccygeal Segments. From hence the coccygeus muscle is supplied and the skin about the anus and coccyx. The nerve is the coccygeal. Symptoms of a Unilateral Lesion. Brown-Scquard's Syndrome^ As this is a localization manifestation, it may well be referred to here. Brown-Sequard long ago noted that whenever a lesion severs the cord half way across, there is complete motor paralysis on the same side as the lesion, incomplete sensory paralysis, or anaesthesia on the oppo- site side. The reason for this is obvious, as the motor tracts decussate in the pyramids above the lesion and the sensory tracts, immediately after they enter the cord, below the lesion. Some additional phenom- ena, however, accompany the syndrome. The sensory disturbance on the side opposite the lesion is an incomplete one because the muscular sense is unaffected. The muscular sense on the side of the lesion is diminished or lost. The paralyzed side, moreover, is by per. esthetic. hyperalgesic and exhibits a slight elevation of temperature. The re- flexes on this side are exaggerated ; though at first they may be absent,, possibly in accordance with Bastian's hypothesis. If mobility returns THE NON-NEURONIC DISEASES 493 FIGURE 109. Diagram to illustrate Brown- Sequard's syndrome. Modified from Brissaud. 494 THE NON-NEURONIC DISEASES to this side, ataxia, according to Botazzi, Herbold, Oppenheim, sets in. On the anaesthetic side, all forms of sensation are lost, except that of the muscular sense. Just above the anaesthetic area on the side oppo- site the lesion there is a hyperaesthetic zone, just in an area on the same side of the lesion and corresponding with the same root-zone there is a semi-girdle ring of pain, hyperaesthesia or anaesthesia. The accompanying diagram, modified from Brissaud, exhibits in. a schematic way the mechanism of Brown-Sequard's syndrome. Dorsal stab wounds are the most common cause of it, though it may occur in tumors, hemorrhages, syphilitic, sclerotic and even myelitic processes that involve the cord half way transversely. The future of these unilateral lesions is usually a good one. The symptoms largely disap- pear, though rarely completely so. Lumbar Puncture. As a means of diagnosis, and in some in- stances, perhaps, of treatment, lumbar puncture has become an estab- lished procedure. At the beginning of the consideration of spinal cord diseases would seem to be the proper place to consider it. Quincke first suggested the operation in 1891 in a paper entitled Hydrocephalus presented to the Tenth Congress of Internal Medicine. Two years later Von Ziemssen reported his experience with it in reducing brain pressure. It was Lichtheim who, about the same time, called attention to the value of it as a diagnostic procedure. Then followed Furbringer, Jacoby and many others, until now it is a familiar manoeuvre. An ordinary syringe with a hollow needle is employed. In insert- ing it the needle may be used alone or attached to the barrel of the syringe. Too short a needle ought never to be used, as ofttimes the insertion has to be made to a considerable depth. Sometimes a thick needle with a stylet is most desirable. Jacoby used a firm, smooth aspirating needle of a millimetre to a millimetre and a half in diameter and eight centimetres in length. Of course, strict asepsis and sterilization are to be employed, and in restless patients an anaesthetic may have to be administered. The column should be rendered convex by the patient flexing his body forward while sitting or lying, as Quincke recommended, upon his left side. The puncture is to be made between the third and fourth, or fourth and fifth, lumbar vertebrae, in the middle line in children, about one centimetre laterally from it in adults, at the height of the lower border of the spinous process, or at about its lower third. The needle is made to pierce the skin and then gently inserted upward and towards the middle line, until there is no further resistance. Usu- ally the needle has to be pushed to a depth of from two to eight centi- metres. As soon as the subarachnoid cavity has been entered, the ccrebro-spinal fluid begins to appear drop by drop or as a slight stream. The latter is due to pressure which may be measured by attaching the needle to a manometer. In adults the normal pressure is about one hundred and fifty millimetres. Tt varies, however, from one hundred and fifty to six hundred and eighty. Quincke found it to vary in chil- dren from seventy to four hundred and seventy millimetres. The flow, when it has slowed down, rarely exeeeds four drops in a minute. Inspiration and expiration modify the flow. Raising the head THE NON-NEURONIC DISEASES 495 increases it. Sometimes a slight change in the position of the needle will cause a flow that has ceased to start up. There is no absolute rule about the amount of fluid to be with- drawn. The object being two-fold — namely, to reduce pressure and to secure fluid for examination — circumstances must determine the amount. Furbringer has taken off at one sitting one hundred and ten cubic centimetres. There is very little pain experienced, if aspiration is not resorted to. Headache sometimes occurs. Aspiration almost always produces pain, sometimes pain that is intolerable. Rarely do any accidents occur during the puncture, if care be exercised. The operation, however, should not become a routine one, for it is not entirely devoid of dan- ger. For instance, a local meningitis may be transformed into a gen- eral meningitis by it. A root of the cauda may be pierced and corre- sponding pain and neuritis set up. In cases of tumor, aneurism and meningitis even, death has followed early the operation. A vein may be entered and the needle become clogged. Blood must be avoided, as it impairs the diagnostic value of the fluid. The needle should be withdrawn while the fluid is still running. In this way the entrance of air is prevented. Sometimes considerable effort is required in pulling out the needle. This is merely on account of the pressure upon the needle of the crossed fibrillar of the fascia. The after treatment of the wound is simple and consists of the application of a little iodoform collodion and a cotton compress, held in place by a piece of adhesive strip. The patient should remain quiet in a horizontal position for several hours afterwards. As a therapeutic measure, lumbar puncture is of little value. It may be employed as a palliative by reducing intra-cerebro-spinal pres- sure. Some day it may prove useful for the direct medication of the brain and cord. As a means of confirming a diagnosis, under some circumstances, it is available. Even here, however, it has its limitations. The state of the fluid does not always and absolutely indicate the pathological condi- tion present. For instance, there may be an inflammatory separation of the cranial and spinal cavities and the fluid that is drawn off be per- fectly normal, though the cerebral meninges are in a high state of disease. The thickened, gelatinous condition of the fluid, sometimes observed, impairs the value of the criteria. Normal cerebrospinal fluid is colorless and alkaline, and con- tains sodium chloride and other inorganic salts. There are o.i per cent, of proteids, consisting chiefly of proto-albumose with some serum-globulin and rarely peptone. It docs not contain any serum- albumin, fibrinogen or nucleoproteid. There is a non-nitrogenous re- ducing substance in it which Claude Bernard thought was sugar, but which Halliburton regards as pyrocatechin derived from the proteids. Pathologically we note in the fluid an increased quantity and change of pressure, change of physical appearance such as cloudin increase of albumin, coagulability and the presence of abnormal sub- stances and micro-organisms. The pressure is increased in hydrocephalic conditions, whether due 496 THE NON-NEURONIC DISEASES to tumor or meningitis, or other cause. It is not constant nor path- ognomonic in any way. Albumin is increased in the inflammatory conditions. The presence of blood may be accidental from the operation, or pathological. Hemorrhages into the brain and cord are the most fre- quent causes of it. Purulent meningitis often reveals pus cells in the fluid and ren- ders it cloudy. It is often cloudy also in tubercular meningitis. The presence of leucocytes may indicate purulent inflammation somewhere, though no pus cells are found. More important than all, however, are the bacteriological find- ings. These are not constant unfortunately, but when they are pres- ent a positive differential diagnosis can be made. The bacteria found are the bacillus tuberculosis in tubercular meningitis, the pneumococ- cus and meningococcus intracellular is in pneumonia meningitis, and the streptococcus and staphylococcus in purulent meningitis. DISEASES OF THE SPINAL COLUMN. These are practically all surgical disorders but they produce neu- ronic derangements of sufficient significance to warrant their consid- ation in a work upon neurology. DISLOCATIONS AND FRACTURES OF THE VERTEBRAE. The great majority of dislocations occur in the cervical region and are the result of blows and injuries of various sorts. In this way the atlas is displaced from the axis. Bone disease not infrequently causes dislocation between the fifth and sixth cervical vertebrae. Dis- placements are exceedingly rare in the dorsal and lumbar region.,, and when they do occur there do not give rise to marked nervous symp- toms. The displacement of the vertebra? may be in any direction and usually both adjoining bones are out of the proper line. The displace- ment may be unilateral ; as a rule, however, it is bilateral and may be complete or incomplete. Luxations in the cervical region may result from blows, twists, wrenches, falls and sudden turning of the head when there is a heavy weight being borne upon it. The deformity consists chiefly of an in- clination of the head forward, and of the spinous processes of the ver- tebrae below the luxated one backward. The chin thus is brought in apposition with the chest or shoulder and the occiput with the nape of the neck. Sometimes a noticeable prominence is to be detected at the side of the neck. Palpation at the back of the pharynx will some- times reveal the dislocation. The irregularity of the spines is always noticeable. The cord may be compressed, bruised or completely divided. The last is a very rare accident. A temporary paralysis caused by compres- sion may quickly disappear as soon as the dislocation is reduced. In the bruised cases there is some extravasation of blood and ere long myelitis. Quadriplegia, anaesthesia, sphincter paralysis and all the THE NON-NEURONIC DISEASES 497 usual signs of transverse myelitis then appear and run their usual course. If the transverse ligament of the atlas is ruptured, as has occurred in hanging and in the lifting of children by the head, the odon- toid process may compress the spinal cord and produce instantaneous death. Fractures may occur in any part of the spinal column and be due to disease of the bones, associated with blows, falls, violent muscular contractions and sudden forcible flexion of the spine. The middle -cervical, upper dorsal and first lumbar vertebrae seem to be the choice victims of the accident. Dislocation often accompanies the fracture. Crepitation, fixation and distortion are the usual surgical symptoms. The cord is usually pressed upon and may be damaged even when there is no constriction of the canal. Rarely is the dura torn but there is almost always rupture of the veins and abundant hemorrhage in the extradural space. In the pia mater are slight extravasations of blood. The cord is usually bruised and compressed by the lower fragment. It is flattened and may even be completely severed. Rarely it has been lacerated longitudinally. Thus extravasation of blood and cavities have occurred in the cord substance. All of this is more or less localized. Immediately a myelitis lights up which may spread beyond the bruised area. Caries, sometimes of a secondary character, follows the injury. There are three great groups of symptoms in spinal fracture. There is the deformity. Then there is the general nervous shock with its sudden and temporary loss of consciousness, its vomiting and its pains and tetanoid manifestations. Lastly there are the signs of con- tusion, laceration and pressure of the cord. The last include the symp- toms of both epidural and subarachnoidal hemorrhage, central hetnato- myelia and transverse myelitis. The transmission of impulses through the cord is usually stopped by the focal damage. Motion and sensation are lost in those parts whose nerve supply is from the segment of the cord below the one that is injured. There is a horizontal or segmental distribution of the sensory phenomena, on the surface of the body. The upper edge of the sensory disturbance does not reach as high as the level of the af- fected vertebra. For the interpretation of the symptoms in these transverse, mental lesions, the reader is referred to the account of the Localiza- tions in the Spinal Cord. Sometimes the symptoms are not such as to afford an opportunity to make a segmental, localization diagnosis. They are irregular and anomalous. In such cases there is only partial damage of the cord and the confusion arises from the associated central hemorrhage in- juring the gray matter but leaving the white tracts uninvolved. In these transverse lesions, together with the motor and sensor} paralysis of all parts below the level of the lesion, there are 1<>>- of the reflexes in the zone of the lesion, or of the segmenl below it. and increase of the reflexes that have their central seal ^till lower down. Bastian's law, supported by Bruns, must be taken into account here. Bastian's hypothesis is that the lesion is only a partial one when there ■are exaggerated reflexes below it. for he says that in all complete 498 THE NON-NEURONIC DISEASES transverse lesions, the reflexes below are abolished. The functions of ;he sphincters are always impaired. In all complete transverse lesions the paralysis in the limbs is a flaccid one. If the lesion is a partial one, it is spastic. All lesions in the lumbar region produce a flaccid paralysis in the legs. Disturbance of the sensibility with preservation of the general motor system means involvement only of the nerve-roots. Pains and twitchings, spasmodic jerkings, contractures and atrophic paralysis in the muscles innervated by the special nerves involved are then the objective phenomena. The clinical picture varies not only in accordance with whether the cord or roots are implicated and the degree of the damage done, but it varies as the lesion occurs in different parts of the column. Death is usually instantaneous in fracture of the atlas or axis. There may be a very slight displacement and the patient live, but he is in imminent danger from the slightest movement. Death usually occurs in such cases in a few weeks from secondary myelitis or even caries. Dislocation, inclination of the head, rigidity and immobility from pain, dyspnoea and bulbar symptoms are the usual signs of upper cer- vical fractures. In all cervical lesions the penis is erected; ejaculation takes place at the moment of injury; the temperature is elevated, the pulse increased and the general vasomotor apparatus disturbed. In mid-cervical fractures, the involvement of the phrenic nerve is a cause of death. Sometimes here there is only a slight displacement but there is constant danger from the slightest movement. Symptoms of the disease in the cervico-dorsal region are the oculo-pupillary disturbances with myosis and narrowing of the palpe- bral opening ; priapism ; vasomotor changes ; early paralysis of the legs and muscles of the trunk ; diaphragmatic respiration ; later in- volvement of the arms, affecting special muscles; muscular spasm and rigidity in the arms and hands ; and general hyperpyrexia. Dorsal lesions leave the arms intact and are usually accompanied by intense pains about the body and complete paralysis in the legs. Bastian's rule about the reflexes applies here, namely, that they are lost if the lesion is complete, exaggerated if it is incomplete. Lumbar lesions provoke a flaccid, atrophic paralysis in the legs, complete loss of reflexes, paralysis of bladder, rectum, genitals and anaesthesia in the distribution of the third and fourth sacrel nerves. This is the saddle type of anaesthesia. It is practically impossible to differentiate lesions of the Cauda equina from those of the conns mcdullaris, for the latter is surrounded by the roots of the former. There are radiating pains around the areas of distribution of the sciatic and pudendal nerves. The prognosis of all these cases is most serious. Dislocations hold out a better hope than do fractures. The latter often kill in- stantaneously or in a very short time. This is especially true of the cervical fractures. The lower dorsal and lumbar fractures may not at once destroy life but they set up spinal disease that causes bedsores, contracture, nephritis, cystitis and all the sequelae of a profound mye- litis that sooner or later does terminate fatallv. THE NON-NEURONIC DISEASES 499 The management of these diseases is practically a surgical ques- tion. After careful study of statistics, Goldscheider formulates the fol- lowing practical conclusions : 1. Recent cases should not be operated on, except where there is merely a comminutive fracture of the vertebra, fragments of which are pressing upon the cord. 2. When the paralysis does not disappear and a deformity also exists, indicating a fracture of the vertebral body, an operation may be in place. 3. Hemorrhage into the spinal canal is not a sufficient cause for laminectomy. 4. Operation is more justifiable in fracture of the lower lumbar vertebrae than in any other region. CARIES OF THE VERTEBRA. TUBERCULAR SPONDY- LITIS. Pott's disease of the spine first attracted attention on account of the nervous manifestations produced by it. The old English surgeon, Percival Pott, described it as long ago as 1779. as a cause of paralysis. It is strictly a surgical, bone disease of tuberculous origin but which secondarily involves the spinal cord in such a way as to bring it most forcibly under the observation of neurology. Etiology. — A tuberculous cachexia underlies the disease, though in a few instances a non-tuberculous traumatic caries does occur. In many cases the tuberculous trouble lies dormant until an injury to the spine starts it into activity. There is an inherited diathesis often pres- ent and not infrequently tuberculous foci of disease are found in the lungs and other parts of the body. Sex seems to have no influence upon the incidence of the disease. It may appear at any age, though it is most frequently observed in childhood and early adult years. It does not always immediately follow an exciting traumatism for it is so insidious that many years may intervene between the injury that starts it and the first manifestations of its presence. It is now known that syphilis has nothing to do with it. Nearby centers of suppuration, as a retro-pharyngeal abscess may initiate a spinal caries. \ on-septic inflammation very rarely, if ever, causes it. Sometimes no cause can be found for it. Patholooy and Pathogenesis. — Usually only one vertebra is affected. Under the effects of a tuberculous osteitis, the body <>f the vertebra breaks down into a fungous, granulation tissue, eventuating even in the formation of liquefaction products, such as caseous and purulent matter. The bone softens and yields under tin- superincum- bent weight so that the spine is deformed and the cord is distorted or pressed upon. 'Hie inter-vertebral substance participates in the dis- ease process and like the body of the vertebra may go on to comp necrosis. The pus burrows in various directions. Xot infrequently, and especiall) under treatment, the disease pro cess ceases, resolution takes place, new hone replaces that which was lost, and a complete recovery is effected. 5°° THE XOX-XEL'RONIC DISEASES The spinal cord is involved secondarily by compression and by the pns that oozes into the spinal canal. Sometimes there is an enor- mous accumulation of purulent matter between the cord and the ver- tebrae, involving the loose areolar tissue found there and the outer sur- face of the dura mater in a severe septic inflammation. When the FIGURE no. Pott's disease. (Whitman.) pressure becomes considerable the cord undergoes a similar inflam- matory process. A myelomalacia may first be produced by the com- pression and obstruction of the bloodvessels and lymphatics by the externa] pachymeningitis. Usually, however, a focal myelitis or men- ingo-myelltis occurs, which becomes more or less transverse though it does not extend longitudinally but a very short distance. The myelitic symptoms are typical and need not be described in greater detail here than to say that there is oedema, softening, prolifer- THE XOX-XEUROXIC DISEASES 5OI ation of the vessels, minute hemorrhagic spots and local secondary neuronic .degenerations. Occasionally the myelitic foci are dissem- inated. Inflammation always occurs when there is compression but sometimes it may appear when there is slight or no compression. In such cases it probably advances from the spinal nerve roots, a neuro- myelitis, or it may be the result of the extension of the disease along the course of the arteries, in which case the primary process may be a soft- ening from a tuberculous arteritis obliterans. The spinal roots of course are often affected. The dorsal cord seems to be the favorite seat of the trouble. The vertebrae being laterally displaced so that one is moved to one side in relation to its neighbor, or the bodies being collapsed, there is a notice- able deformity of the spinal column which often becomes very marked in the mid-dorsal region. One or two spines stand out beyond the oth- ers giving the typical appearance of an "angular curvature." • Symptoms. — The symptoms group themselves under the four heads of (i) constitutional manifestations, (2) the bone deformity, (3) the signs of involvement of the spinal roots, and (4) the signs of compression of the cord and myelitis. Usually the disease is so insidious that no constitutional or febrile symptoms are present. Signs of infection may appear, how- ever, but they belong to the tuberculosis or the general disease and are very variable. They need not concern us here as they are not a part of the spinal cord phenomena. In most cases there is a distinct spinal deformity. This is of the nature usually of a kyphosis. It is commonly known as Pott's hump. It is an acute angled protrusion or convexity backwards of the spinal vertebrae, due to the softening and yielding of their bodies to the superincumbent weight. It is accompanied by a local tenderness, and by which it has usually been preceded. The pain and soreness can be easily elicited by vertical pressure upon, and lateral movement of, the spines. When the disease is in the cervical region, movements of the head often provoke the pain. This of course produces a peculiar, half- voluntary rigidity of the neck and back that has a remote resem- blance to a torticollis. Deformity is not infrequently absent in cervical spondylitis. The tissues are thickened about the spine and a gravity abscess sometimes appears. These abscesses burrow and break out in all sorts of unexpected places. They often confuse greatl) the clinical picture. A retro-pharyngeal abscess, for instance, may interfere with swallowing so much as to lead one to imagine there is some bulbar paresis; or the abscess may follow the psoas muscle and appear well down upon the anterior part of the thigh. (Esophageal obstruction has been suspected from an abscess of spinal origin. The involvement of the nerve roots is witnessed by the charac- teristically distributed pains and their intensity. Sometimes the} absent. Not infrequently motor and sensory symptoms show the im- plication of both anterior and posterior roots. The pain, the hype thesia, the muscular weakness and the slight atrophy follow the course of the nerve distribution. A girdle-like sensation is thus produced. 502 THE NON-NEURONIC DISEASES Instead of the hyperesthesia, there is occasionally a neuritic hyperaes- thesia, or even anaesthesia. The neuritic symptoms all occur only when there is a distinct pachymeningitis which, however, does not mean the majority of the cases. When the cord is implicated, the symptoms are usually those of a transverse myelitis, most frequently of the mid-dorsal type. There is a paraplegia of the legs with spasticity and exaggeration of the knee- jerks. If the cord is completely severed across by the destructive pro- cess the well-known exception of Bastian will be observed and conse- quently there will be flaccidity and loss of the knee-jerks in the lower extremities. Ancesthesia below the area innervated from the diseased segment of the cord is present. Above this a zone of hyperesthesia is often noticed. In this vicinity also occurs the girdle pain, so dis- tinctive a sign of myelitis. The superficial, like the deep reflexes of the lower limbs and lower part of the body are intact or exaggerated. Sometimes they are lost in accordance with PJastian's hypothesis. The vesical and rectal sphincters are paralyzed. Trophic symptoms occur. In brief, the entire clinical picture is that of a distinct transverse mye- litis. Oftentimes the existence of the caries is long recognized, some- times years, before the myelitic paralysis appears. The spinal deform- ity has been seen in early childhood and yet the paraplegia has not occurred until well into adult life. About a year or two after the kyphosis is discovered the paralysis supervenes. Occasionally both appear together. Rarely the paralysis has appeared without any ob- jective sign of bone disease for a time. The onset of the paraplegia is for the most part gradual though it has followed soon after some strain, injury or other exciting cause. In very rare instances it has been instantaneous, probably on account of some sudden displacement after a considerable period of bone dis- ease. Both legs are usually affected, though in some instances one leg has revealed weakness before the other. As a rarity, the Brown- Sequard syndrome may even be witnessed. The hump and tenderness in the spine, when observable, will help to localize the nervous lesion. But often these objective signs are not marked. Then we can often localize the trouble by the peculiar grouping of the nervous exhibitions. The nerve roots that are involved will be shown by the course and location of the pains, the peculiar distribution of the paralysis and atrophy, the hcrpetiform eruption, the hyperesthesia and the anaesthesia. In other words, the signs of a distinct, and local neuritis must be made out and then the origin of the particular nerves involved be traced back to the cord center. ( )f course these neuritic symptoms will only be observed when a pronounced pachymeningitis is in ex- istence. This is not often the case, however. This, as well as the localization of the lesion from the spinal cord symptoms, can be greatly facilitated by a reference to the table presented under the head of spinal localizations in the preceding chapte* THE NON-NEURONIC DISEASES 503 If the damage is high up in the cervical region, there will be a spastic quadriplegia, without atrophy, with the paralysis more marked in the legs. Bulbar symptoms may be present with nystagmus and interference with respiration. Death under such circumstances may be imminent at any moment. The pains shoot up over the occipital region and there is a stiffness and half-voluntary fixation of the head that closely resembles a torticollis. Occipital anaesthesia may be de- tected. A lesion in the lower cervical area produces the well-known oculo-pupillary changes, sweating on one side of the head and vaso- motor disturbances. The legs remain in a condition of spastic para- plegia. The hands and arms undergo an atrophic paralysis which pre- cedes the trouble in the legs. There are sensory disorders especially in the ulnar distribution. The phrenic and spinal accessory nerves may be implicated. When the mid-dorsal region is the seat of the disease, the case is as a rule a typical manifestation of an ordinary transverse myelitis. The paraplegia with rigidity and increased reflexes in the legs, the sphincter disorders, the anaesthesia, the girdle pains and the decubitus constitute its tout ensemble of clinical presentations. As we approach the lumbar region, the paralysis of the legs be- comes flaccid in character and the knee-jerks are lost. These symptoms are subject to considerable variation in details, though when taken broadly as general groups they maintain a high degree of constancy, much more so than one would expect with such a variable lesion. As the myelitis spreads up or down the cord it in- volves this or that part more than another, symptoms appear that one may almost be inclined to regard as complications. Thus in old cases bedsores and cystitis occur. Poliomyelitic changes occur, causing profound atrophy and paralysis, with the classical electrical degenera- tive reactions. Implication of the posterior columns may lead to ataxic exhibitions. Pyramidal and disseminated lesions have been recorded with all their peculiar clinical manifestations. Diagnosis. — The readiness with which caries of the spine can be diagnosed is very different when the signs of bone disease are ob- trusive from what it is when they are absent. If there is a slight pro- trusion of the spine at one point and this is tender upon pressure or movement; if there are neuritic pains that seem to he in nerves that lead back to that point in the cord ; if the reflexes are slightly exagger ated, both skin and tendon reflexes; and if. especially, symptoms show- ing more or less solution in the continuity of the cord, as the spastic paralysis of the legs, anaesthesia, and irregularity ^\ the sphincters, the diagnosis can be made practically certain. The Rontgen raj may he brought into use to reveal an obscure hone lesion, but the u& tuberculin to establish the diagnosis is liable to be followed by dis trous results. If a focus of tuberculosis can he found elsewhere in the body, the diagnosis of course is strengthened, It must be remem- bered that traumata and other non-tuberculous causes can sometimes set up a simple spondylitis with similar implication of the nen structures. 504 THE NON-NEURONIC DISEASES There are cases of caries in which the diagnosis is rendered very difficult by the absence of objective deformity. These cases can rarely be diagnosed with absolute certainty, though some of them are more than merely suspicious. If the patient is youthful and shows tuber- culosis elsewhere, together with a more or less localized set of slow, progressive nervous manifestations pointing to the cord or some of its nerve-roots, the diagnosis may be made with a fair degree of ac- curacy. If all signs of tuberculosis fail and we have nothing to depend upon but the nervous manifestations, the diagnosis becomes extremely difficult and uncertain. In such cases sometimes only waiting for further appearances will clear away the mystery. The disease under these circumstances is liable to be confused with primary myelitis, pro- gressive muscular atrophy, intercostal neuralgia, primary pachymen- ingitis, tubercular meningo-myelitis, intraspinal tumor, neurasthenia and hysterical paraplegia. Repeated careful examinations, with every effort made to elicit the slightest evidence of tuberculous bone dis- ease alone will enable one to establish the true character of these cases. Remember that in caries there are three great neurological fea- tures to be looked for, namely, a progressive, slowly advancing, fixed lesion, that first irritates and then destroys ; secondly, signs of nerve- root involvement such as the pains and atrophic paralyses ; and thirdly, signs of more or less severance of the spinal cord, with corresponding phenomena in all parts of the body below the lesion. Only in pachy- meningitis, primary myelitis, and tumor of the cord is this clinical array of events liable to be closely duplicated. In pachymeningitis the differential diagnosis is sometimes abso- lutely impossible. The usual causes of meningitis, especially syphilis, and the age of the patient must be taken into account. In pachymen- ingitis the cord symptoms are perhaps less and the root symptoms are more pronounced than they are in caries. Primary myelitis does not have the root symptoms to the same degree that caries has. The neuralgic pains and the local trophic signs are less pronounced. In a word primary myelitis is more distinctively a cord disease and exhibits the paraplegic, anaesthetic, girdle, sphinc- teric and trophic symptoms that go more particularly with cord lesions. In tumor the pains are more intense and excruciating, the pro- gression more steady and definite, and the succession of irritation and destruction more regular, as a rule, than they are in caries. In progressive muscular atrophy there are no pains and the atrophy is more systematically distributed. Moreover the extensive- ness of the symptomatology shows the greater extensiveness of the disease process. Intercostal neuralgia exhibits no atrophic paralysis, no cord symp- toms. Neurasthenic and hysterical patients not infrequently complain of spinal pains that might easily lead to a suspicion of tuberculous spondy- litis. In neurasthenia the spine is not often focally painful. It feels sore and heavy throughout its entire length. There are. moreover, no distinct organic manifestations such as caries almost invariably THE NON-NEURONIC DISEASES 505 shows, to some degree at least. In neurasthenia the symptoms are those of fatigue and weariness and in a changeable sort of a way involve the entire nervous system. "Spinal irritation" as a unique condition, apart from the general neurasthenic state, has no place in modern neuro- logical nosology. In hysteria there are tender spots in the spine so frequently that I have long taught that they were almost pathognomic. They are mov- able, however, and are subject to the patient's mental changes. Hys- terical paraplegia is sometimes remarkably similar to organic paralysis. It is spastic; its reflexes are increased; the sensory symptoms are not as marked as is usually the case in hysteria ; and there may even be some muscular atrophy, probably from non-use. It is easy to com- prehend how this may be mistaken for organic disease. A Ye must de- pend entirely in such cases upon finding other hysterical stigmata and especially the mental status must be given close study. It is too fre- quent an error, however, which we should be warned of, the calling of an obscure case of caries one of hysteria. The treatment for caries would not hurt a hysteric ; the treatment for hysteria might bring irre- parable disaster upon a spondylitic case. I have seen a serious organic disease of the spinal cord several times made to pass beyond the repara- tive stage by its treatment upon the erroneous notion that the trou- ble was a mere neurosis. Caution is most imperative here and from my own observation of some of the sad errors that have been commit- ted, I am prone to feel that too much cannot be said in urging the pro- fession to keep their hands oft" of a case of spinal disease when they are in doubt as to whether it is an organic or a functional trouble. Prognosis. — The course of the disease is most variable and un- certain. The deformity may precede or it may follow the nervous manifestations. Steady progression or remissions may occur. Even sudden spontaneous and complete cessation may not be unlooked for. Abscesses are always liable to appear and in high cervical disease, bul- bar manifestations and sudden death need never cause surprise. w ter the paraplegic condition has declared itself, the myelitis is likely to terminate in about a year with the usual signs such as contracture, decubitus, nephritis, cystitis, and death. Occasionally the fatal issue is more the result of a general septicaemia and tuberculosis than <>f any special local trouble. When resolution and cure take place, either the cord manifestations or the spondylitic may vanish first. Youthful patients, and those in whom the disease has nol yet been of long standing, have a better chance both as to life and cure than aged and decrepit individuals and those in whom the history of the malady can be traced many years back. The existence of a general tuberculosis and the character and location of the abscesses modify, oi course, very seriously the prognosis. Mid-dorsal lesions offer the most favorable prognosis; high cervical lesions perhaps the worst. A case once got well after an attack of erysipelas, and I rowers mentions a complete recoverv in a case in which there- was paralysis of all tour limbs and the diaphragm. Serious as the disease is. therefore, on never justified in withholding hope of recovery from the patient and his familv. 506 THE NON-NEURONIC DISEASES Treatment. — The two great indications for treatment are the bone disease and the secondary myelitis. For the tuberculous spondylitis a supporting treatment and pro- longed rest are the two great desiderata. The former involves rich, nourishing, fatty food, plenty of air and such well-established tonics as cod-liver oil and iron. The latter demands the recumbent position in bed ; best on the back because thereby the greatest amount of im- .mobility is obtained. Many months may have to be passed thus but the importance of the rest is so great that no temporizing should be allowed that in any way limits it. For the detailed treatment of the spondylitis the reader should consult the works upon surgery. Suspension, the wearing of a jacket, and the employment of revulsive measures will all be amply discussed there. Suspension seems to me to have too many disadvantages to cause it to be adopted as a routine measure. The cases that have ab- ruptly had their spinal symptoms relieved by it, have in all likelihood been cases with displaced vertebrae. The greatest disadvantage against it is that the rest, which is so necessary and which is more than any medicine or surgical procedure to the patient, is constantly broken. If the spinal extension could be maintained then the method would be both logical and commendable... A certain degree of permanent ex- tension is obtained by a jacket, a jacket with a jury-mast attachment and, better than all, a weight attached to the feet or to a band about the hips while the shoulders are drawn towards the head of the bed with sheeting passed under the arm-pits and around the bed posts. To be sure, not very great extension can be thus secured and it can- not be long maintained without wearying the patient. Its advantages are, however, that it can be easily accomplished without any disturbance in the recumbency of the patient. Except in a few cases, the plaster jacket had better be avoided. It causes much mobility in getting it on and off, and when once on it tempts the patient to go about more than is wise. An extension plas- ter-of-Paris bed, as suggested by Phelps and Lorenz, or even a suit of plaster-of-Paris proposed by Karewski, may not be objectionable for they at least infer the recumbent position. In my own judgment, however, the bed alone is the best, as necessitating the smallest amount of movement and yet furnishing a means of securing prolonged and absolute rest. The revulsive measures, such as setons, cautery and sinapisms, are not as much employed now as they were formerly. In regard to the more violent forms of counter-irritation I rejoice in this, but in regard to the steady, prolonged use of mild counter-irritation I think we may lose a valuable aid to the treatment, if it be entirely discarded. The continuous application of weak mustard plasters or mustard poul- tices may not do much for tuberculous spondylitis, but I am sure it has in my hands helped very greatly, and more than once, the spinal inflammation. The skin must not be damaged, the sinapism must be shifted about, and the treatment must be prolonged for weeks and months. Cupping, wet or dry, is an abrupt treatment and has more disadvantages than advantages. THE NON-NEURONIC DISEASES 507 Operative procedures, such as laminectomy, forcible redressement, have not gained popular favor because their later results have not been so favorable as they promised to be at first. These are surgical ques- tions, however, and their discussion does not belong here. There is no specific treatment for the disease process. Oleum morrhuse and iron about exhaust the list. The syrup of the iodide of iron is particularly useful. Mercury and the iodides would of course come into play if there is a history of syphilis. I consider the calcium salts as very much over-rated. I have administered them time and again without seeing the results that others have claimed for them. The treatment of the nervous lesions is fostered by the treatment .given for the tuberculous bone trouble. In fact, after the bone disease has disappeared the nervous trouble often vanishes also. If compres- sion merely is present, the entire management of the case resolves itself into the management of the bone disease. If myelitis is present, the treatment, secondary to that of the bone trouble, should follow the .general principles laid down for that of inflammation of the cord. Symptomatic treatment is of the greatest importance in combating the "bedsores, the cystitis, the contractures. In the cases that recover, mas- sage and mild faradic stimulation of the muscles may be of use. Tenot- omy may be called for to counteract the deformities produced by the contractures. In mentioning electricity, I desire again to emphasize the fact that it is a most dangerous agent if improperly used. I have sometimes wished it had never been introduced into general therapeutics. With some medical men, and nearly always with the laity, nervous diseases are always the indication for electrical treatment. If the trouble is spinal and they don't know just exactly what it is, they at once be- gin the use of strong galvanic currents up and down the spinal cord. I would like to see such practices made a crime. I have been the un- willing witness of them several times. Electricity is rarely, if ever, called for in spinal or brain diseases. It is most religiously contra- indicated in organic acute diseases and where there is a possible doubt about the accuracy of the diagnosis. To see a mild, stationary myelitis that interfered but little with the patient's comfort suddenly trans- formed into a complete paraplegia, with a rapid hastening of the pa- tient toward death, by a medical man who gloried in the use of his elec- trical paraphernalia, but who could not distinguish a myelitis from a rheumatism, is a most disheartening and exasperating experience. Patients that have once had caries n\ the spine must lead a care- ful, hygienic life and must be warned of the possibility of relapse from a neglect to do so. TUMORS OF TIM': VERTEBRAE. Carcinoma, sarcoma and myxoma are the principal new growths that attack the spinal column and secondarily affect the spinal cord and its functions. Exostoses growing from tin bodies of the vertebrae into the spinal canal may also produce very slow, progressive cord 508 THE NON-NEURON 1C DISEASES symptoms. Of all these, however, cancer and sarcoma are the most common. The same conditions for all of them are usually found else- where in the body. Carcinoma especially is generally due to the same disease in the stomach, womb, mammary gland or other organ from which it has spread by metastasis. Sarcoma and osteosarcoma spread from the surrounding parts and are found chiefly in those advanced in years. Rarely are any of these growths primary in the vertebrae, though when they are primary they usually start in the bodies and spread from one to the other. The vertebral bodies break down and thus allow the formation of an angular curvature. This kyphosis is- not generally so sharp as it is in caries, as more bones take part in it and the tumor is present to modify it. From the bodies there may be an extension of the growth into the neighboring ribs, muscles, along the lateral processes of the corre- sponding vertebrae, narrowing the inter-vertebral foramina through which the spinal nerves pass. The nerve roots are pressed upon, in- flamed and sometimes infiltrated by the cancer. The disease may even advance into the areolar tissue between the dura and the vertebrae and surround the cord completely. The cord is thus compressed and often inflamed. It is never invaded, however, by the neoplasm. The nervous symptoms of special import are the intense pains from the implication of the posterior nerve roots and the signs of com- pression of the cord. Most cases are diagnosed from the deformity of the spinal column, the presence of the disease elsewhere in the body and these nerve root manifestations. The pains follow the course of the spinal nerves and are distinc- tive for their excruciating intensity. They are far more intense than they are in caries. At first' there are remissions but later on they are constant with paroxysmal exacerbations. They are sharp, lancinating and are increased by every movement. They appear in the arms, trunk, and legs, according to the site of the tumor. They are accom- panied by cutaneous hyperaethesia and spots of anaesthesia. The asso- ciated muscles are thrown into a state of spasm and rigidity, are con- tractured, paralyzed and wasted. Spasms and pains often appear to- gether in the paroxysms. In some cases paraplegia develops, often very rapidly. In this the cord manifestations differ from those of caries, where they progress more slowly. Twelve or twenty-four hours may see all power in the legs lost. The intensely painful character of this paralysis led Cruveilhier to name it paraplegia dolorosa. Some- times the onset of the paralysis is slower. The compression of the cord is the explanation of it. There is nothing distinctive about the paralysis. Inflammation more often accompanies it perhaps than it does the paralysis of caries. The diagnosis should carefully take into account the age of the patient, the presence of the disease in other parts of the body, the spinal deformity, the intense character and anatomical distribution of the pains, and the signs of compression of the spinal cord. Skiagraphy may be invoked to assist the diagnosis. Caries is less painful, is tuber- culous in the vast majority of cases and occurs in younger individuals. THE NON-NEURONIC DISEASES 509 Jn intercostal neuralgia the pains are not bilateral and there are no mo- tor or cord phenomena. Cancer is a growth that often runs its course in a few months. The other tumors last for years and then destroy life only secondarily .by the trophic troubles they set up and the intercurrent maladies that intervene. The prognosis of course is always bad. The operative treatment so far has not accomplished anything. Morphine to relieve the pains and general hygiene is about all that can be done. SYPHILITIC DISEASES OF THE VERTEBRAE. Syphilitic exostoses that press upon the cord are not common. They produce deformity or rather a spondylarthrosis ; damage the nerve-roots ; and compress the cord. There is, therefore, nothing dis- tinctive about them but their etiology. This is usually clear. If it is only suspected it may be cleared up by the administration of the iodides in large dosage for a long period of time. ANEURISMAL EROSION OF THE VERTEBRAE. This cannot be diagnosed unless there are other indications in the body of aneurism. Symptoms of a neoplasm such as I have been relating, involving the nerve-roots and the cord, that are suddenly in- terrupted by a complete paraplegia followed by an ascending paralysis, would justify a strong suspicion of a ruptured aneurism within the spinal canal. The condition is of more pathological then clinical in- terest. SPINAL HYDATID DISEASE. Leyden and others have collected a number of reports of the de- velopment of hydatid cysts in the areola tissue between the cord and the bones, and possibly even in the bones themselves. Double cysts, partly in and partly outside of the canal, have been observed. ( H course the cord is compressed and inflamed, with the usual symptoms. The diagnosis is extremely difficult from other vertebral diseases that provoke a similar clinical picture. We can only depend upon the pres ence of the disease elsewhere. Practically all cases prove fatal, even though puncture has been tried as a means of amelioration. LATERAL CURVATURE OF THE SPINE. This rarely, if ever, produces distinct ennl symptoms, even when there is slight pressure. I have seen it. however, ran.se decided weak -ness in the legs, neuralgic pains and severe general neurasthenic man- ifestations. The accompanying picture is thai of a girl who was a sufferer from such symptoms and supposed she was a nervous wreck. A correction of the spinal deformity resulted in her complete restora tion to health and happin 5io THE N0N-NEUR0NIC DISEASES ARTHRITIS DEFORMANS OF THE SPINAL COLUMN. As a phase of the disease rheumatoid arthritis, the articulations be- tween the vertebrae may be affected and even become anchylosed. The whole spine may be involved but it occurs more frequently in the cerv- ical region. The inter-vertebral cartilages undergo atrophy or become calcified, the heads of the bones become enlarged, new bony formation FIGURE in. Lateral curvature of the spine with marked hystero-neurasthenic symptoms. Orthopaedic treatment for the spinal trouble caused complete disappearance of the neurotic manifestations. takes place on the spinous processes and complete fixation occurs. The Spine is thus made rigid. The neck becomes fixed and the chin is in- clined toward the chest. The inter-vertebral foramina are constricted and the nerves pressed upon. The spinal cord is not usually impli- cated. A descending neuritis is seen in some of the nerves involved. The symptoms of the disease include the rigidity of the spine, which does not yield under chloroform narcosis, local tenderness in the spine, THE NON-NEURONIC DISEASES 5II radiating pains and a partial neuritic atrophic paralysis in the area to which the nerves go that are inflamed. The inflamed nerves are lo- cally tender, as well as being the seat of the shooting, lancinating pains. The sympathetic may be implicated when the trouble is in the cervical region. The nerves of the medulla have been affected in a few in- stances. The diagnosis of the malady must be made largely from the presence of the same disease in other joints of the body. The disease is a chronic one, running its course with remissions and exacerbations. It is incurable. The best we can do is to foster a remission by the use of massage, local applications of iodine, the administration of potassium iodide, the salicylates, quinine, iron and arsenic, and the employment of sulphur and alkaline baths. DISEASES OF THE MEMBRANES OF THE SPINAL CORD. ANJEMIA AND HYPEREMIA. Notwithstanding the fact that many of the older neurological writings contain much about anaemia and hyperemia of the spinal membranes, we know practically nothing about these conditions. ( )n account of the peculiar arrangement of the circulation and its rich anastomoses in and around the spinal cord, it may well be doubted whether anaemia or hyperaemia of the membranes ever occurs apart from a similar condition in the cord. Of the possibility of the latter there is not a little dispute. It is not intended by this to deny abso- lutely that anaemia follows a severe hemorrhage, an exhaustive diar- rhoea or an aortic obstruction, or that a hyperaemia is associated with certain sexual and muscular excesses, uraemic and other convulsions, suppression of menstruation and strychnine poisoning. It is but to reiterate that we do not have any positive knowledge of such an under- lying condition. It must be presumed that in the earliest stage of meningitis, and possibly as a residuum after the inflammation, hyperae- mia of the membranes exists for a time. The clinical description of these conditions is full of obscurity. On the one hand many of the symptoms referred to by authors are too subjective in character to possess any value as indications of a gross pathological disturbance; and on the other hand they are very closely like those that are known to be indicative of actual inflammation in the membranes. The heavy feeling in the legs, the weakness amount- ing to paraplegia, the pains in the back, and the supposed relief afford- ed by lying flat on the back, are too indefinite and certainly too posi- tively assumed, as indications of aiucmia. Such a pathology was ai one time adopted for spinal concussion and the traumatic neuroses; bu1 in addition to what Page and others have done t<> show tin- untena- bility of such a pathology, we now know- that the traumatic neuro including railway spine, railwa\ shock and spinal concussion, are mostly cases of hystero-neurasthenia. The same general critcism is to he made of the clinical descrip- tion of hypercemia. the heaviness and weight in the limbs, the numb- ness, the slight paresthesia, the fugitive pains, the muscular twitch- in gs, the disturbance of the sphincters, and the asserted relief obtained 512 THE NON-NEURONIC DISEASES by lying on the face or side, are too subjective in character to be of real symptomatic value ; or are much like the well-recognized symp- toms of actual inflammation. At all events the clinical picture usually accredited to spinal hyperaemia has not yet been sufficiently identified with such a pathological basis. All of which being so it is useless to discuss further the diagnosis, prognosis or treatment of anaemia or hyperaemia of the membranes. INFLAMMATION OF THE SPINAL MEMBRANES. SPINAL MENINGITIS. Of the three membranes covering the spinal cord, two, the dura mater and pia mater, undergo distinct forms of inflammation. The arachnoid becomes implicated with the other two. Only in very ex- ceptional instances does the inflammation confine itself entirely to the membrane originally attacked. Sooner or later the neighboring struc- tures are involved by contiguity. Thus when the dura is inflamed externally the bone above it has usaully been damaged and the loose, connective tissue between it and the bone is involved. When it is inflamed internally the arachnoid and pia are almost certain to be in- cluded. When the pia is the original seat of the disease, the arach- noid above it, including sometimes the dura, and the outer edge of the cord below it, are as a rule more or less implicated in the same process. This disease process may be primary or secondary, simple or purulent, acute or chronic. The symptomatic manifestations may van- greatly, the variations depending upon the character and location of the inflammation. A number of types of spinal meningitis, therefore, are recognized. Involving the dura mater we distinguish an acute and chronic external, and an acute and chronic internal, pacliyiucn- ingitis. The inflammation of the pia mater is known as acute and chronic leptomeningitis. Chronic syphilitic meningitis is sometimes spoken of as arachnitis gummosa. The forms of the disease deserving of further consideration by reason of their clinical importance are external pachymeningitis, hem- orrhagic and hypertrophic internal pachymeningitis, acute leptomen- ingitis, and chronic syphilitic meningitis or rueningomyclitis. The epidemic form of cerebrospinal meningitis will not be discussed here. EXTERNAL PACHYMENINGITIS. This form of spinal meningitis never occurs de novo. Tt is a secondary inflammation. Etiology. — Tuberculosis and caries of the vertebrae, fractures and dislocation, septic processes and purulent inflammations in other parts of the body, such as purulent pleurisy, psoas abscess, puerperal pyaemia, are the causative factors in external pachymeningitis. Pathology. — A purulent inflammation with exudation and pus THE NON-NEURONIC DISEASES 513 in most cases occupies the space between the bone and outer surface of the dura. The latter is covered with a thick, creamy, fibrinous ex- udation either of pus or caseous matter. The dura itself is thickened. The inner surface of the dura is often implicated, but the underlying pia very rarely so. The inflammatory process is more marked poste- riorly than anteriorly on account of the peculiar arrangement of the bloodvessels. The cord itself is compressed, flattened, and in excep- tional cases may show some degree of peripheral inflammation. The thickened membrane surrounding the nerve-roots constricts them and causes them to undergo inflammation, softening and atrophy. All of this produces what is sometimes called compression myelitis and multi- ple neuritis of the nerve-roots. As a rule the disease extends focally along a certain distance, though at times the whole membrane may be involved. The cervical part is not as frequently attacked as the dorsal and lumbar on account of the close attachment of the dura to the bone in the neck. The cellular tissue between the dura and the bone is sometimes purulent throughout the entire length of the canal. Symptoms. — The symptoms of external pachymeningitis are oft- times inextricably confused with those of the original disease. When they can be clearly distinguished they are the signs of irritation and compression of the cord and- nerve-roots. The spine is tender and painful, especially over the focus of the disease. Every movement causes pain. Pains shoot down the limbs and around the trunk. These radiating pains may assume the form of typical girdle, constricting bands. The pains show a tendency to follow anatomical lines of nerve distribution. Various parccstliesicc are present in the extremities, such as numbness, formication, pins-and-needles sensation and tingling. Hypercesthesia of the skin is common. Among the motor manifesta- tions are twitchings of the limbs, various degrees of paresis, partial paraplegia. The reflexes are generally exaggerated and the sphincters may be incompletely affected. If the disease process is limited in ex- tent, its location will modify the symptomatology somewhat. When the cord is inflamed myelitic symptoms may appear. The tempera- ture, respiration and pulse rate will ail assume their characteristics very largely in accordance with the extent and nature of the original trouble that is the cause of the pachymeningitis. Diagnosis. — When there is a history of injury or other disease of the spine, or of suppurative processes elsewhere, the above general •clinical picture will make the diagnosis easy enough. Myelitis is de- void of the general history of these cases, does not have the same sort of rachialgia and radiating pains, nor the temperature, but on the other hand is the cause of more complete paralyses, trophic and vaso motor disturbances and distinct focal signs. There are no radiating pains, paramnesia and histor) of injury and suppuration in muscular rheumatism. Tetanus is distinguishable by its trismus and the excita- tion of the muscular spasms by peripheral irritation. Prognosis. — This is always grave. It depends upon that of the original disease, as well as upon the character of the meningeal in- flammation. If the original trouble were not serious, and the menin- geal symptoms indicated only irritation, some hope of recovery might 514 THE N0X-XEUR0XIC DISEASES be held out. When the original trouble is serious, as it mostly is, and especially when the meningitis has been of long standing, showing involvement of the cord, the prognosis is absolutely bad. Treatment. — The treatment is practically surgical and concerns itself mostly with the primary disease. This once overcome, the men- ingitis is to be treated upon the same principles as any of the more common forms of the disease. CHRONIC CERVICAL HYPERTROPHIC G1TIS. PACHYMENIN- This is an internal inflammation of the dura mater, chronic in duration, hemorrhagic and hypertrophic in character, and involves sooner or later the adjoining arachnoid, pia and cord. It was first de- scribed by Charcot and Joffroy. Etiology. — Adults are usually affected by this disease, though cases have been reported in children. Males are more prone to it than females. Cold, trauma, over-exertion, exposure and alcoholism have been said to produce it. Syphilis, however, plays so large a role in its etiology, that some have wondered whether the disease were not a manifestation of spinal syphilis. It has been supposed to have been brought on also by syringomyelia. In the hemorrhagic form, analogous almost to the hematoma of the cerebral dura, it is not uncommon in the chronic insane. Pathology. — The disease is most frequent in the cervical re- gion, though rarely it may occur in other parts of the column. The most striking pathological feature is the thickening of the dura, almost five and ten times as thick as the normal membrane. The process is a chronic one, is more or less limited to the internal layer of the dura, and ultimately involves the underlying arachnoid, pia and cord. Pre- ceding the hypertrophic process, in most cases, is the hemorrhagic, which in a way resembles a dural hematoma such as occurs on the brain sometimes. The hemorrhage occurs on the internal surface of the dura and is followed by a chronic inflammation. After a time an- other hemorrhage with inflammatory action and deposition of new mat- ter takes place. By the frequent repetition of this pathological action, layer after layer of fibrinous tissue is deposited, gradually thickening, hardening, almost ossifying the membranes, binding them together and constricting and compressing the spinal cord and nerve roots and thus producing in the latter atrophy and sclerosis. In the syphilitic cases the deposited material is of the gummatous variety. As a result of the steady compression and the extension of the disease into the substance of the cord, the latter becomes involved completely across. Even cavities may form in it. Symptoms. — A moment's consideration of the nature and progress of the lesion in this disease will enable one to quite definitely foretell the character and succession of the symptoms. There are quite distinctly three sets of symptoms which follow one another in a regu- lar progression. The first set of symptoms indicate irritation of the cord and roots. There is pain in the neck and stiffness. Pains are THE XOX-XEUROXIC DISEASES L felt in the back of the head and between the shoulder blades. The vertebrae are tender upon percussion. Hyperesthesia and paresthesia occur, especially down the arms and usually more in one arm than the other. Numbness, tingling, pricking, are complained of, especially along the area of distribution of the ulnar and median nerves. The sensory symptoms and particularly the pains are apt to be worse at night. Muscular rigidity, cramps, tremor indicate the motor irritation in the arms and hands. All of these symptoms continue for many weeks or months before the next stage comes on and are obviously due to the meningitis. There may be some slight disturbance of tempera- ture and pulse, of the irregular type. Xausea and vomiting are not entirely unknown. Following this neuralgic stage is the second or paralytic, which is purely neuritic in character. The arms are chiefly affected. They are weak, rigid, contracted and atrophied. The extensors being unop- posed by the normal action of the flexors, there is over-extension of the wrist. The fingers are over-extended in the basal phalanges, sharply flexed in the middle and end phalanges. This gives an almost pathognomonic appearance to the hand, known as the preacher's hand, or main- en- grift : e. This paralysis is of the degenerative type and is most pronounced in those muscles supplied by the ulnar and median nerves. Other nerves of course may be involved, as, for instance, the radial, causing pronation. The pains, anaesthesia, hyperesthesia and trophic manifestations continue during this stage. In some cases it is in this stage that the oculo-pupillary symptoms become marked. There may be dilatation followed by contraction. The pupillary signs, how- ever, are not distinctive in any way of the disease. This is a very chronic stage, even more so than the first. Its symptoms are plainly due to the severe compression and beginning destructive inflamma- tion in the roots and peripheral border of the cord. It is not sharply defined from the last, or paraplegic stage. In the third stage, the paraplegic, the cord is more or less diseased and damaged completely across. The pains and other sensory symp- toms now gradually decrease as the paralysis of the legs comes on. This paralysis is spastic in character and is associated with exagger- ated knee-jerks, increased myotatic irritability, muscular rigidity and spinal trepidation. Anaesthesia is present, as well as disturbance of the sphincters. Gradually weakness and exhaustion supervene and the patient dies of this or some intercurrent malady. The clinical picture of the disease which I have just presented is of the more common, cervical type. The extension of the inflamma- tion up or down the cord will of course modify the picture somewhat. Involvement of the medulla and pons will add corresponding symp- toms, such as impairment of hearing, tachycardia, and other special bulbar manifestations. In the so-called peripheral type of the di» the symptoms are more distinctly limited and localized in the extrem- ities. Diagnosis. — The diagnosis of cervical hypertrophic pachymen- ingitis is at times extremely difficult. At first it may be mistaken for Pott's disease of the spine. Later on the atrophy of the muscles may 5 J T> THE NON-NEURONIC DISEASES lead to the erroneous opinion that the case is one of progressive mus- cular atrophy. And still later, the syringomyelic lesion added to the pachymeningitis will be sure to lead to confusion. Moreover the dis- ease has to be carefully differentiated from myelitis, spinal tumor, tor- ticollis, etc. When the disease is of the typical, cervical type, the localization of the symptoms, their bilaterality, their chronic, progressive charac- ter, their more or less agreement with the succession of stages out- lined above go far toward making the diagnosis positive. At times tumor cannot be differentiated. The safest way to differentiate this from other typical diseases is to exclude this by a careful considera- tion of all of the symptoms of the others. A myelitis, for instance, is not preceded as a rule by a prolonged meningitic period and a pro- gressive muscular atrophy is practically devoid of all sensory phe- nomena. Widal and Le Sourd have suggested that an examination of the cephalo-rachidian fluid should be made in doubtful cases. In Pott's disease, for instance, this fluid is normal with respect to its cellular contents, whereas in pachymeningitis it contains after centrif ligation mononucleated elements, lymphocytes for the most part, mixed with some large cells having a single nucleus and much protoplasm. Prognosis. — This disease is painful, chronic and progressive. Its progress may cease at any stage. Cure has been occasionally reported. Most of the cases, however, terminate in death and a grave prognosis had better always be given. Treatment. — Syphilitic cases of course should be vigorously combated with mercury and the iodides. Other cases may be helped by the use of local counter-irritation. Iodine, mustard, cups, the act- ual cautery, are all indicated. Hydrotherapy has seemed to have been useful in a few cases, in giving relief if not in producing a cure. The pains, spasms, etc., must be treated symptomatically with anodynes, narcotics, antispasmodics. Electricity has a field of usefulness here apparently and is worthy of a trial. It is not impossible that sur- gery may be of some benefit to these cases some day. The indications are suggestive and if undertaken early enough, before there is serious involvement of the cord, lumbar puncture, laminectomy, or incision of the dura may accomplish something favorable. VCUTE SPINAL LEPTOMENINGITIS. This is an acute inflammation of the pia mater, rarely occurring apart from cerebral meningitis, is secondary, and is due to infection. ETIOLOGY. — Simple, primary leptomeningitis is practically un- known. When due to a traumatism, to rheumatism, exposure and sun- stroke it is nevertheless infective in origin. It occurs in tuberculosis, syphilis, typhoid fever and septicaemia following purulent wounds, otitis and the puerperium. It is the form of meningitis that occurs epidemically as cerebrospinal meningitis. Children are the most fre- quent sufferers, and among adults males predominate. Dana remarks that alcoholism predisposes to it. THE NON-NEURONIC DISEASES $IJ Pathology. — The character of the inflammation and its accom- paniments will naturally depend upon the cause and infective character of the original disease. Syphilitic cases reveal the gummatous, gran- ular exudate. In the tuberculous cases there is not a very abundant exudate and it is of a fibrinous nature, grayish in appearance. Pus of course is absent, but there are miliary tubercles in the meshes of the arachnoid and on the inner surface of the dura. Simple exudative meningitis is so rare as to be of but little clinical interest. In most of the cases an exudate is found that is due to various pyogenic micro- organisms such as the streptococcus pyogenes, to the pneumon- coccus, etc. Thus an initial hyperemia is followed by a purulent exu- dation which spreads through the pia and arachnoid and clouds the cerebrospinal fluid. The membranes are bound together by adhesions and adhere closely to the cord. The spinal nerve-roots and the peri- phery of the cord are constricted and slightly inflamed. All of this is more marked on the posterior than the anterior or lateral aspects of the cord. Sometimes the disease localizes itself focally at various levels. When the inflammation ends there is often left a mass of new connective tissue, or the disease passes into a state of chronicity. This causes sclerotic changes and by the compression, irritation and destruc- tion caused by the thickened, adhering membranes keeps alive a group of subacute, permanent symptoms. Symptoms. — The chief symptom of spinal meningitis is pain, and is the result of pressure. The pains are local and radiating, dull, aching, sharp and shooting. The spine itself is tender on pressure and percussion. The tenderness occurs sometimes in spots, these spots usually overlying the foci of maximum inflammation. Sharp, shoot- ing pains are felt up in the neck and back of the head, down into the arms, around the trunk and down along the legs. They follow the course of the nerves and are due to the compression and constriction of the posterior nerve roots. The same pressure and irritation of the anterior roots cause muscular rigidity. The muscles of the back and neck are stiff. The body is sometimes thus bowed forward in the con- dition of opisthotonos with the head boring backward into the pillow. All this usually starts abruptly with a chill, some deration of temperature and alteration of the pulse rate. There may be a Febrile headache and other indications of cerebral meningitis with nausea and vomiting. The urine is retained and the bowels are constipated. Gradually the pains increase as well as the muscular stiffness. As every movement of the back provokes pain, the patient adds his volition to the maintenance of the muscular rigidity. The skin is exceedingly hyper (Esthetic and painful. The relieves an- increased at first, but diminished later on. On account of the muscular stiffness, Kerui^'s sign is present. The so-called tdche bleudtre of the French writers can be elicited and indicates implication of the vasomotor apparatus. It is not important, however. If the patient is not cut off early by death, paralysis, paraplegia or paraparesis will usually come on. The sensibility becomes sened, the bladder grows weak and the relieves become profoundly affected. The respiration, the heart beats and the pupillary activity 518 THE NON-NEURONIC DISEASES may all be implicated. In the last stages complete palsy, atrophy and anaesthesia may be observed. The most severe and rapid onset of symptoms outlined above occur in the septic cases. The tuberculous forms of the disease are slower in their onset and progress, while the infectious forms range between the septic and tuberculous in this respect. Diagnosis. — The diagnosis of spinal meningitis is not difficult. The febrile symptoms, the local tenderness and radiating pains, the tonic and clonic spasms, the Kernig and reflex signs, when all placed together produce an unmistakable picture. In myelitis there is no spinal tenderness and generally less pain but much more pronounced paralysis. The sphincters are affected in myelitis, the reflexes are characteristically exaggerated and the trophic manifestations are more prominent. In tetanus the history of an injury, the trismus and the absence of the irregular, high fever of meningitis, ought to make a differentiation comparatively easy. Rheumatism exhibits mere mus- cular pain, no cutaneous hyperesthesia, bladder symptoms or trophic manifestations. The examination of the cerebrospinal fluid obtained by lumbar puncture may be resorted to in very doubtful cases. It will show in nearly every case a cloudiness, and a leucocytosis, and pos- sibly a few bacilli. Prognosis. — The onset and course of the disease are generally acute and foudroyant. In a few days it generally proves fatal ; hence the prognosis is, as a rule, bad. Sometimes the patients linger several weeks before death overtakes them. These cases may subside and pass into chronic meningitis. The cases with the worst prognosis are those with high fever, intense pains and rapid appearance of paralysis. Septic cases are bad and the tuberculous cases, though ofttimes slow and prolonged, cannot be expected to recover. Very rarely, however, recovery has occurred ; more particularly among the epidemic and otitic cases. Partial recovery, with a remaining bladder weakness, slight paralysis, hyperesthesia may legitimately be always hoped for. Treatment. — The first thing to do in the treatment of spinal meningitis is to put the patient's mind and body at rest. Perfect quietude of room and environment must be obtained. The pains must be suppressed by opiates, and sleep be induced by bromides and other hypnotics. The skin, bowels and kidneys should all be made as active as possible and with the least amount of disturbance of the patient. As the patient is urged to lie most of the time on the side or abdomen, local treatment of the spine can easily be instituted and carried out. An ice bag, of the Chapman type, may be placed alongside of the spine, or wet packs may be similarly employed. After the pain is lessened somewhat, dry cups, leeches and sinapisms may be used. I do not favor too energetic measures for they are apt to irritate and disturb the patient. In most of my cases I use mustard, even diluted with flaxseed, daily or twice a day on alternate sides of the spinal ver- tebrae. I believe more is accomplished by constant prolonged mild counter-irritation than by too hasty or severe methods. Notwithstanding some opinions to the contrary. T am satisfied that mercury is the best medicament now at our disposal for the internal THE NON-NEURONIC DISEASES 519 treatment of spinal meningitis, syphilitic as well as non-syphilitic. It may be used by inunction or by the mouth. Any preparation of mer- cury may be used, though I prefer unguentum hydrargyri, calomel or blue mass. It must be given rather freely, guarded by opium, and for a long period continuously. I have not found it necessary to ex- periment with small doses of potassium iodide or sulphate of magne- sium, given at short intervals, though both are highly indorsed. In the later stages of the disease more heroic counterirritation by means of cantharides, the hot iron, the Paquelin cautery, or even mere hot douches, may be of much advantage. Lumbar puncture with with- drawal of some of the cerebrospinal fluid has been reported to have brought about a cure. In the treatment of meningitis I am convinced that time and patience, with absolute quiet and the mercurials, will do more than harsh or hasty methods. SYPHILITIC SPINAL MENINGITIS. SPINAL SYPHILIS. Syphilis affects the spinal cord, just as it does the brain, in various ways. Certain late products of syphilitic infection, such as the so- called parasyphilitic troubles, like locomotor ataxia, will not be dis- cussed here. Under the above heading, I will consider rather the true syphilitic diseases, namely, meningitis, meningomyelitis, gumma. Our knowledge of spinal syphilis is less than our knowledge of cerebral syphilis ; nevertheless a sharp distinction should always be made, when possible, between syphilitic disease and other affections of the cord that may resemble syphilis. Etiology. — Constitutional syphilis, acquired or inherited, is of course the etiological factor in the production of spinal syphilis. ( )ther determining influences are the age of the patient, his mode of life, the manner of the primary treatment of the constitutional disease, trau- mata, colds and other infectious disease. Spinal syphilis usually makes its appearance early, when it does occur, after the initial lesion. Nonne detected it as early as three months after the acquisition of the syphilis, in most of the cases it appears in about a year after, though in some eases it may not develop until many years after. In the earlier eases gummatous meningitis is the predominant lesion ; in the later eases slow degenerative processes and sclerotic conditions arc more frequent. Pathology and Pathogenesis. — Huebner enumerates four Min- eral forms of spinal syphilis: First, neoplasms of syphilitic origin, including single tumors and small multiple and disseminated formations on the spinal membranes. Second, syphilitic callus, there being found post-mortem a cir- cumscribed induration of the cellular tissue about the cord, generall) with adhesions of the dura mater. Third, simple softening of the cord. This Steenberg describes, though Huebner doubts whether it .should be considered a distinct syphilitic lesion. He also doubts the existence of a pure syphilitic mvelitis. 520 - THE NON-NEURON IC DISEASES Fourth, cases in which symptoms of acute ascending paralysis (Landry's paralysis) occur without discoverable post-mortem findings. After giving a report of a case which manifested symptoms to classify it with Huebner's fourth series, Wood states that he doubts whether these cases ought to be regarded as syphilitic at all. Cer- tainly his own case, of which it was difficult to obtain a complete autopsy, resembled one of peripheral neuritis as much as anything else. The cases of Huebner and Kussmaul were not examined for lesions of the peripheral nerves, so that the fourth class, as a form of syphilis, has scarcely been established. In the production of the second and third classes of Huebner, syphilitic infiltration and gummatous formations play an important role ; so that the softening of the cord and the development of callus should be regarded in the light of secondary effects rather than as direct syphilitic lesions of the cord. This leaves only the first class to be considered. Syphilitic neoplasms are generally connected with the spinal mem- branes. They may grow inward upon the cord and produce destruc- tion of the medullary elements and even chronic inflammation and softening; or they may extend outward and cause agglutination of all the spinal membranes, pressure of the nerve roots, and even disease of the osseous vertebrae. There are probably, therefore, two forms of spinal syphilis or gummatous disease — that in which the membranes are chiefly the site of the disease (Wood), and that in which there is an infiltration of the cord from its own vessels (Rumpf). The meningitis is usually of the pia mater and arachnoid, though in some instances it has been seen to develop from the inner surface of the dura. The membrane is thickened, opaque, clouded and more or less bound to the cord and the nerve roots. Granulation tissue is formed and over the membranes is spread a gelatinous, fibrous sub- stance. The cord is swollen and the dura is held to it by adhesions. Yellow, gray, soft or hard deposits are seen scattered here and there resembling small, circumscribed tumors. The process is not uniform over the whole membrane for in one place it may be highly vascular with thick cellular deposits, and in another hard and fibrous in char- acter. Usually it is more pronounced in the region of the posterior columns, a fact which by comparison lends some color to the theory of Redlich and Obersteincr that locomotor ataxia is pathogeneticallv primarily a chronic form of posterior spinal syphilitic meningitis. In most of the cases the periphery of the cord itself is involved in the inflammatory process, so that we have a meningo-myelitis rather than a simple meningitis. Sometimes the granulation tissue sends septa-like extensions into the cord substance which cause inflammation, atrophy, destruction and sclerosis in the adjoining tissues. The nerve- roots are usually implicated, though it is possible for them to remain normal where they pass through the inflamed and thickened membranes. They usually show signs of infiltration and atrophy. Spots of softening and old hemorrhagic inflammatory foci are some- times present in the cord, having been caused by the obliteration of the inflamed and thick-walled bloodvessels. Oppenheim is responsible THE NON-NEURONIC DISEASES 52 1 for the statement that a simple disseminated and diffuse myelitis, a poliomyelitis (?) may develop from syphilis. Isolated gummata have been reported a number of times, and Kahler has attempted a descrip- tion of what he calls a multiple gummatous neuritis of the nerve roots. Specific gummatous meningitis, with secondary results, is the primary lesion of true spinal syphilis as it is of true cerebral syphilis. In fact the separation of brain syphilis from spinal syphilis is a mere arbitrary distinction based solely upon a convenient anatomical classi- fication of the parts of the nervous apparatus. In a large percentage of cases both the brain and the cord are affected together, the brain symptoms merely over-shadowing those of the cord. Symptoms. — When examining a case of suspected spinal syphilis, it is necessary to remember that the lesion may be meningeal, producing symptoms similar to those of non-specific spinal meningitis ; and that, on the other hand, it may consist of an infiltration of the me- dullary substance itself, giving rise to symptoms indicative of destruc- tion of the sensory-motor tracts. When the gummatous disease is men- ingeal, the earliest symptoms will be those of pain or paresthesia and spasm or paresis, due to compression and irritation of the anterior and posterior nerve roots. Of course, the sensory symptoms will be re- ferred by the patient to the peripheral terminations of the nerves whose roots are undergoing irritation. The principal difference between gummatous disease of the meninges and simple, non-specific, subacute or chronic meningitis, is that the symptoms of the former are apt to be more sharply defined or localized than those of the latter. The reason for this is obvious, since the former lesion is more in the nature of a tumor with comparatively well-defined limits. According to Erb,. the symptoms of syphilitic disease of the spinal cord closely resemble those of myelitis transversa dorsalis ; but the two are, nevertheless, distinguishable by a number of typical signs. The walk, posture, and motion are quite characteristic, and simulate those of spastic paralysis. The tendon reflexes are apt to be marked, with- out much muscular tension. The pains, when present, are usually sharp and cutting, and when the lesion is located in the dorsal or lumbar region they assume the girdle form. Xot unfrequently they resemble the lightning pains tabes dorsalis. Rarely are they dull, aching, and continuous. Often there is no pain at all. On account of the usual meningitis accom- panying the gummatous deposit, there is more or less local tender- ness of the spine on pressure. Wood States that in several instances this local tenderness was attributable to involvement of the vertebral periosteum and vertebrae. All the various sensations common to local meningitis are characteristic of this form of spinal syphilis, such a> numbness, formication of the extremities, "pins-and-needles" sensa- tion, and other bizarre paresthesia? down to the ultimate condition oi complete anaesthesia. There is always distinguishable impaired sensi- bility, but severe pain is not generally felt, according to the observa- tions of Erb. Atrophy is not present. There is little or no invol ment of the head and cerebral nerves. Rarely are the muscles of tin- eye implicated. There is nothing abnormal psychically. 522 THE NON-NEURONIC DISEASES The motor symptoms are the same as those of localized non- specific irritative meningitis, such as rigidity of the neck and limbs, tremor, exaggeration of the reflexes, severe cramps excited by move- ment. Later on, complete paralysis supervenes. If the palsy increases rapidly after long-continued disturbance of sensation, it is almost pathognomonic of syphilitic disease, according to Wood. The sphinc- ters are generally included in the final symptoms. Bedsores and other trophic troubles occur, often with elevated temperature and general septicaemia. In a case of syphiloma of the cord and cauda equina, reported by Osier, death occurred from diffuse central myelitis. There were pains in the legs, particularly in the left, which underwent rapid wasting and presented vasomotor changes. There were pains in the arms, especially the right, without wasting. There was absence of control of the bladder and rectum for two months before death. There were bedsores and arthritis in the knees and ankles. Toward the close of life high fever with delirium came on. There was a gumma in the antero-lateral columns of the cervical cord, opposite the right fourth anterior nerve root. Gummata also involved the third, fourth and fifth anterior sacral nerve roots, and the second and third posterior sacral roots on the left side. Many of the symptoms in this case were due to the accompanying lesions resulting from the presence of the syphilitic new formations. A differential diagnosis in such cases, though extremely difficult, is important if at all possible. In syphilitic disease of the upper cord there may be diplopia, amblyopia, and pupillary irregularity. Tinnitus aurium occurred in a case reported by Weber. The symptoms of the second form of spinal syphilis, namely, syphilitic infiltration of the cord, are usually slower in their onset and vary according to the location of the lesion. As the latter is more or less diffused, so will the symptoms be more or less indefinite. They will range all the way from hyperesthesia to anaesthesia, from spasm to paralysis. Spastic paraplegia without much pain is highly indica- tive. Sometimes the symptoms closely resemble those of locomotor ataxia. True syphilis of the cord does not follow the course of the sensory and motor tracts as do the various systematic scleroses and subsequent degenerations. The symptoms are, therefore, much more mixed and indefinite. There is a class of cases, probably included in Huebner's fourth class, described by many writers under the title of acute syphilitic myelitis, acute syphilitic paraplegia, acute syphilitic spinal paralysis. They usually attain their maximum degree of severity in a few hours or a few days. They may have prodromal symptoms long before, how- ever. Most of these cases subside and pass into a state of partial re- covery ; a few mild cases make a complete recovery ; some pass to a very rapid fatal termination. The symptoms are all those of a typical myelitis. There are cases that resemble subacute and chronic myelitis and are progressive in character. Erb described a type in which there appears a slowly developed spastic paraplegia, exaggeration of the THE NON-NEURONIC DISEASES 523 Itnee-jerks, and disturbance of the bladder. In its pure form there are no sensory phenomena. Most of the cases prove to be not of the pure type, as sensory and other symptoms appear which link them to the great group of variable cases of spinal syphilis. Erb did not there- fore describe a type of the disease, but rather a particular symptom group. Kuh says that in Erb's spinal paralysis the disease has its origin, in all probability, in syphilis of the vessels of the cord in the dorsal re- gion, the lesions of the cord being secondary and involving mainly the lateral tracts and slightly invading the posterior columns. Kowa- lewsky affirms that the cases are quite common in the male sex be- tween the ages of thirty and forty-five. With Oppenheim I am in- clined to regard these cases of Erb's as merely representing a certain stage of the usual form of syphilitic mcningo-myelitis. As Dercum well says, ''When we recall the fact that the virus of syphilis expends itself mainly upon the membranes and bloodvessels, it is not surprising that most varied clinical pictures should present themselves. Xot a single symptom can be taken as constant for all cases. Even the spastic character of the gait may be lacking. Instead of flaccidity, con- tractures may be present and under certain conditions the knee-jerks may be absent.'' The same remarks apply to the cases which Oppen- heim finds so similar to tabes and which he has denominated syphilitic pseudo- tabes. A simple gliosis, an amyotrophic lateral sclerosis, and other dis- eases have been simulated by syphilis. As in brain syphilis, so here, the great diversity of symptoms, their changeability and inconstancy, are one of their characteristic features and go a long way toward es- tablishing the diagnosis of syphilis in doubtful cases. Diagnosis. — There are certain manifestations of spinal syphilis that so simulate other affections of the cord, and are in them selves so indefinite* and irregular, that a diagnosis is always a matter of ex- treme nicety. In such cases a course of anti-syphilitic treatment has sometimes been resorted to before any diagnosis could be made. The more we can avoid, however, such indirect, empirical methods of making diagnoses, and the more we can learn to depend upon the direct manifestations of the disease, the better it will be for the patient and the more creditable to the science of medicine. The diagnosis of spinal syphilis is best made by the exclusion of other spinal affections and by remembering that any one of these other affections, associated with a history of syphilis and presenting more or less unusual manifestations, is very apt to be syphilitic in origin. If a case of apparent locomotor ataxia, for instance, shows an absence of the usual fulgurating pains, or the presence of the patellar reflex, spinal syphilis rather than posterior spinal sclerosis should at once be thought of. Simple myelitis, compression myelitis, multiple sclerosis and (lis ease of the lateral and posterior tracts, may all be simulated b) syphilis. Hence the differential diagnosis ran be taken up better under th various heads. Prognosis. — The prognosis of spinal syphilis should always guarded, even though great improvement is obtained sometimes by 524 THE NON-NEURONIC DISEASES appropriate medication. Occasionally absolute cures have been made More often, however, the delicate constituents of the cord have been irreparably damaged when the case first comes under observation, and then only a prevention of further injury can be hoped for. The less the cord itself is involved the better the prognosis. When, paralysis has continued several months no improvement can be ex- pected. Spastic paresis, bladder trouble, etc., will continue. Relapses are always liable. Treatment. — The treatment usually resolves itself into the ad- ministration of mercury and the iodides. The object is to neutralize at once the syphilitic poison, to sustain the vitality of the cellular struc- tures, and to remove as quickly as possible any gummatous enlarge- ments that may be exerting a deleterious pressure. Each case is a. law unto itself, but in all cases bold, heroic 'treatment is usually needed to check at once all further damage of the nervous elements. Hot and cold spinal douches, suspension, massage, all have their applicability in appropriate cases. The indications are the same as in any of the various forms of organic disease of the spine. Spinal syphilis should be managed just as any other disease of the cord and its membranes, plus the administration, heroically and for a long period of time, of the requisite antisyphilitic remedies. The administration of these is the same as for brain syphilis, to which section the reader is referred for more detailed discussion. SPINAL MENINGEAL HEMORRHAGE. Hematorrhachis. — This condition is so commonly secondary to- some injury or other disease that some authors do not speak of it at all as a special affection. It does occur, very rarely, however, as a primary trouble. Its symptoms are slightly characteristic' I deem it worthy of special, brief consideration therefore. Etiology. — The hemorrhage may occur outside of the dura mater (extra-dural, extra-meningeal hemorrhage), or it may take place be- tween the dura and the cord (sub-dural, intra-meningeal hemorrhage). The former is the result of traumata, blows, falls, fractures, disloca- tions and disease of the vertebrae. The rupture of an aneurism may be the source of the hemorrhage. The blood springs usually from the great venous plexuses that surround the dura. Sub-dural hemorrhage may result from the same causes as the extra-dural, and from all the causes enumerated under the head of hematomyelia in a following section. The clot may extend only an inch or so and not compress the cord at all. On the other hand it may reach the entire length of the canal and exert very severe pres- sure upon the cord. Most of these hemorrhages occur from the pial" vessels in the cervical region. While blood may pass up even into the ventricles of the brain, or vice versa, one must be on his guard in as- suming this, for the intra-cranial and intra-spinal hemorrhage under such circumstances are more apt to be simultaneous phenomena.. SYMPTOMS. — The symptoms of hematorrhachis are characteris- tically meningitic, that is progressively irritative and paralytic upon THE NON-NEURONIC DISEASES 525 the spinal cord and spinal-root functions. The suddenness of these -symptoms and their appearance at first without fever indicate their hemorrhagic origin. Preceding them there may be slight premonitory manifestations and in a few days after their appearance all the signs of a meningitis exhibit themselves. There is pain in the back and pains shooting along the course of the spinal nerves. The nerve pains are neuralgic in character and remittent or intermitttent. The nerves them- selves are not locally tender as they are with the pains of neuritis. The spine is very tender, however. The cranial nerves and head and mind are all unaffected. The skin is generally hyper&sthetic, and there are usually various paresthesia such as tingling, pins-and-needles sen- sation and numbness. The muscles are spasmodic in the same areas as the sensory phenomena. Soon they become rigid and contracted. The back is arched in opisthotonos. Finally in some cases the condition goes on to almost, but not quite, complete anaesthesia and paralysis, es- pecially in the lower limbs. Later on and toward the close trophic manifestations appear and even signs of a distinct me ningo -myelitis. According to the location of the hemorrhage so will the clinical picture be slightly altered. If it occurs in the lumbar region the pains are felt entirely in the legs. The sphincters are paralysed. Paraplegia ■comes on early and the patella reflexes are lost. Sexual irritation may be a troublesome symptom in the beginning. Dorsal hemorrhage sends the pains shooting around the chest and abdomen. When the seat of the trouble is the cervical enlargement, the pains are felt in the neck and arms. Later on the paralysis is spastic in the lower and flaccid in the upper extremities though this is rare and only in very advanced cases with considerable involvement of the cord. The neck may be rigid and immovable. Swallowing is difficult. Respiration is disturbed. The pupils are dilated. Diagnosis. — The history of an injury or appropriate cause for a hemorrhage, the onset of irritative, non-febrile symptoms of a men- ingeal character, ought to go far towards making the diagnosis easy. Meningitis itself approaches more slowly and insidiously, and is ac- companied by fever, and symptoms that tend rather to continuously progress and not to recede, as they often do in hemorrhage. In mye- litis there is fever, more marked paralysis and anaesthesia and not tin- distinctive irritative signs of meningeal trouble. After all, however, it must be admitted that there is such a dose relationship between all of these troubles, hemorrhage, meningitis and myelitis, that a differential diagnosis is sometimes, and under certain conditions, peculiarly difficult and even impossible. At all time can hardly amount to more than a strong suspicion, so frequently and generally do all three diseases overlap and run into one another. Prognosis. — Most of the severe cases prove fatal in a few hours. •Others recede somewhat symptomatically, then become a meningitis and so run their course. If after a few days, three or four, the cord manifests no signs of implication, recovery may be hoped for. I vical hemorrhage for obvious reasons is more dangerous than hem orrhage elsewhere in the spine. Traumatic and infection- iften recover. What is called typhoid spine is sometimes a slight hemor 526 THE NON-NEURONIC DISEASES rhagic meningitis or meningo-myelitis. I have seen such cases recover after one or two years. The cause of the hemorrhage, its extent, its volume, the course and severity of the secondary meningo-myelitic phenomena will all determine the prognosis. Treatment. — This is about the same as that recommended for hematomyelia. The posture should be prone on the stomach or side, and absolute quiet should be maintained. To obtain this sedatives will have to be rather freely given to ease the pain. The bowels should be freely moved. Leeches may be applied to the anus, or cups to the spine. The ice bag to the spine is a regulation measure. In robust cases, venesection may be employed. Aconite and ergot cannot do much harm if judiciously used, but unfortunately they will do very little good. In purpura Dana recommends the trial of the mineral acids or chloride of calcium. The meningitis and residual phenomena are to be treated on general principles laid down for them elsewhere. Mild persistent counter-irritation to the spine and the steady use of small doses of mercury for a long time have helped me much in the later management of these cases DISEASES OF THE SPINAL CORD. CIRCULATORY DISEASES. The arrangement of the bloodvessels in and about the spinal cord is such that temporary changes in them are not liable to occur, and permanent changes are the result of disease and not of mere func- tional derangement. Hence, as before mentioned, ancemia and hypere- mia of the cord are unknown except under very special circumstances. What is seen in this respect after death is absolutely no criterion as to what is present during life. In fact if all hypotheses were to be discarded, all analogies cast aside, all logical deductions waived, and we were to depend absolutely and entirely upon what the cord reveals post-mortem, we would probably say that anaemia and hyperemia never occur. Such is the position taken by some of the more radical pathol- ogists. They declare that the few changes that are observed in the bloodvessels and related tissues are due to gravitation, lethal changes observed in all fatal diseases, and from various mechanical causes oper- ating upon the corpse ; and that the elaborate symptoms so often noted during life and attributed to the alteration in the amount of blood in the cord, are really due to other factors such as toxaemia, quality of the blood, pressure and velocity of the flow. More conservative au- thorities still hold that anaemia and hyperaemia of the cord may and do occur, but arc rare and are little comprehended by us. The symp- toms that have been so elaborately described by the older authors, cannot for a surety be assigned to conditions about which we know so little and whose very existence is problematical. THE XOX-XEUROXIC DISEASES 52/ ANAEMIA. As a simple, primary condition, temporary or permanent, we know absolutely nothing with certainty about anaemia of the spinal cord. There are some experimental observations and clinical facts that are highly suggestive, but the conclusions that are drawn from them must not be regarded as the incontrovertible actualities. For in- stance it is an interesting fact, and one that leads to the drawing of suggestive comparisons, that the ligation of the aorta was, in a few moments, followed in the experiments of Herter, by complete para- plegia and paralysis of the sphincters. Clinically paraplegia has been seen to follow either immediately or at the end of a few days, pro- fuse hemorrhages from the stomach and uterus. The degenerative changes in the spinal cord in pernicious anaemia are well recognized. As a part of a general anaemia, chlorosis and debilitated blood state, the spinal cord may readily be supposed to suffer implication. As a sequel to profuse hemorrhage, exhausting diarrhoea, and mechanical obstruction of any sort which shuts off its blood supply, the anaemia of the cord may be frankly admitted as a possibility. Even local anaemia must undoubtedly exist in cases where the local circulation is affected by a tumor or other cause of obstruction. When, however, we attribute to anaemia zveakncss of the legs, pains in the back, various parcesthesicc, such as numbness and tingling, we are reasoning beyond the proofs, for these symptoms may be due to special changes in the nervous elements rather than to the alteration in the blood quantity, or they may be toxaemic in origin, or they may be a part of the general symptom-complex of the general or local disease with which the anaemia is associated. HYPEREMIA. Of this some writers think we have a little more definite knowl- edge than we have of anaemia, and they even discuss the condition elaborately under the heads of etiology, pathology, symptoms and treat- ment. It seems to me that in the present state of our knowledge such an elaboration of the subject is unwarrantable. Much of what is said cannot be denied, to be sure, but very little of it can be supported with positive proof. As Gowers well says, "volumes might be filled by the collected descriptions of the varieties and symptoms of congestion ^i the spinal cord — descriptions in which the unrestricted play of 'scien tine' fancy has elaborated a symptomatology for the separate eon. tion of every part of the spinal cord." As a condition in the beginning of myelitis it is undeniable. It is not impossible that it may occur in excessive coitus, in certain forms of violent exercise, in suppressed menstruation, in strychnine poison ing, as a residuum of a meningitis or trauma, as a part of the general symptom-complex of some other disease. \s I say, it IS not impossible that such is the case, but where and what are the proofs? Tin- symp- toms do not confirm it. The reputed heaviness and weight in the limbs, the numbness, the creeping sensations, the neuralgic pains, the 528 THE NON-NEURONIC DISEASES weakness in the legs, the muscular twite kings, the disturbances of the sphincters, the general tendency to paraplegic phenomena, the very doubtful increase of distress when a posture on the back is assumed, may all be due to anaemia, to circulatory disturbances in the meninges, to beginning or transient inflammatory states, to irritative lesions of various sorts, to nutritional disturbances in the nervous cells or even to other conditions in the blood itself, than to the mere excess of its quantity. No, it will not do to assume a hyperaemic pathology on such a diffuse, indefinite, loose basis as this symptomatology appears to be. Pathology and treatment suggest no proof. It is singular how au- thors recommend in one paragraph the use of counter-irritation, cup- ping and the ice bag to the spine, morphine and bromides internally and in the chronic cases ergot, muriate of ammonium, iodide of potassium and the galvanic current, when in the paragraph almost immediately preceding it was admitted that these symptoms may be due to very different conditions from that of congestion and that the simple existence of congestion is open to doubt. If the presence of another disease can be made out, we may be at liberty to suppose that this therapy affects that disease and so modifies the spinal manifes- tations. In some cases the beneficial results may be partly psychic in character. I have often in hystero-neur asthenic cases obtained the happiest kind of psychic effects from the use of vigorous cupping of the spine. In such cases it is a favorite trick to get the patient's thoughts in regard to her spine runing in another direction by the use of cups, galvanism, etc. And I have thus relieved many symptoms similar to those above but I did not fancy I was curing a spinal con- gestion. In fact I am still of the opinion that the treatment and its good results were alike largely psychic. EMBOLISM. THROMBOSIS. ENDARTERITIS. These conditions are comparatively rare in the spinal cord. Thrombosis of the minor vessels, together with endarteritis, undoubt- edly underlies some of the acute and chronic focal diseases of the cord, or may be an associative or complicating feature in the pathology of these diseases. As a part of general senile changes and senile pathology, endarteritis may be seen quite frequently in persons past middle life. It is not, however, a special cord condition but the arteries of the cord are affected like those in the rest of the body. Thus in younger persons there is a special as well as a general syphilitic nodular periarteritis or endarteritis, possibly with gummatous menin- gitis. Arteritis obliterans and miliary aneurism may be seen biit they are far rarer in the cord than they are in the brain. SPINAL HEMORRHAGE. Hematoniyclia. Hemorrhage into the Spinal Cord. — As a primary condition, hemorrhage into the substance of the cord is exceedingly rare, even being denied by some. It is thus in marked contrast to hemorrhage into the brain, the difference being probably due to the longer course THE NON- NEURONIC DISEASES 52<) of the spinal bloodvessels. As a secondary condition, associated with myelitis, tumors and cavities, or capillary hemorrhages generally in se- vere tonic and clonic spasms, hemaiomyelia is of little clinical interest because its symptoms are so masked by those of the primary disease, that the diagnosis is always uncertain. Many descriptions have clearly been based upon the secondary forms of the trouble. I will discuss here only the primary form. Etiology. — Men are more subject to it than women. It occurs most often in middle adult life. It has been seen in infants. Alcohol- ism, degenerated arteries in the aged, the hemorrhagic diathesis, pur- pura and other factors doubtless may predispose to it. The influence of heredity, except through the circulatory apparatus, is nil, and cer- tainly not what it is in cerebral apoplexy. Bv all odds, some form of trauma is the leading cause of spinal apoplexy. Blows upon the spine, falls, strains, wrenches, lifting of heavy weights, excessive exercise produce probably nine-tenths of the cases. Somewhat traumatic in a way are those cases said to have "been due to excessive coitus, exposure, suppressed menstruation, bleed- ing piles, difficult parturition. The cases due to fractures of the spine, dislocations, inflammations, syphilis, neoplasms, cavities, tetanus and convulsions are of course merely secondary and of very little practical interest. Pathology and Pathogenesis. — The central vessels of the cord, supplying especially the gray matter are the chief sources of the hem- orrhage. Miliary aneurisms are exceedingly rare upon them. Their coats of course may be degenerated as the result of disease or age. Generally, however, in the primary form of the trouble, there is no very great change observable in them. After the rupture takes place the blood pours out into the surrounding gray substance extending up and down the cord and into the horns, especially the posterior. The white matter is not usually implicated, certainly not to any very great extent. It is more generally compressed than directly damaged. Mye- litis of course soon lights up in and around the hemorrhagic focus, the tissues become softened, disappear and leave a cavity in a few cases. This process Van Giessen calls heniatomyelo porosis, to dis- tinguish it from hematomyelia. Sometimes the clot may be so 1 arm- that the white substance of the cord forms a mere thin, ragged wall around it. It may lacerate its way through the white matter and ex- tend beneath the meninges. The cord is usually enlarged, softened and ruddier in hue near the extravasation. In small hemorrhages there may be no noticeable external changes. Symptoms. — The first great characteristics to be always noticed about the symptoms are that they appear suddenly and are accom- panied by febrile phenomena. As time passes, however, fever, pos sibly some slight recession of the symptoms or the appearance others indicates that the secondary myelitis has begun. As the erup tion of blood produces a sudden break in the continuity of the cord, all parts of the body below it are functionally paralyzed. The patient falls, therefore, with a complete paraplegia. This may involve only his arms or legs or all four extremities. . liucthesia accompanies it an 1 53Q THE NON-NEURONIC DISEASES the sphincters lose their control. In some cases there are slight pre- monitory weakness, tingling and numbness for a few moments, then the shock comes as just described and is profound all at once or in- creases still more than it was at first. At first the reflexes are abol- ished but they soon return and in a little while become highly exag- gerated. The patient's consciousness and sensorium are unaffected and he often complains of pain in the back. The site and size of the clot of course may vary and thus greatly modify this clinical picture in different cases. The paraplegia indi- cates a dorsal or lumbar lesion when it is limited to the legs. In such a case the reflexes may remain lost, the sexual functions be damaged and the bladder and bowels be involuntarily evacuated. There may be a girdle pain, especially when inflammation lights up. A quadri- FIGURE 112. Cross-section of the spinal cord in heniatomyelia. Hemorrhage into the left anterior and posterior horns. (After Minor and Oppenheim.) plegia indicates a cervical hemorrhage. Here an atrophic paralysis occurs in the arms and a spastic condition in the legs. Unlike lumbar hemorrhage, the patella reflexes are now exaggerated. Oculo-pupil- lary symptoms occur if the lower cervical region is implicated. A unilateral hemorrhage may cause the Brown-Sequard syndrome, typical in this case of limitation of the lesion to the gray matter. ( )n one side, the side of the lesion, the leg exhibits a spastic paralysis, while the arm manifests a partial, flaccid, atrophic paralysis. On the opposite side appear anaesthesia, analgesia and thermansesthesia. There may be some hvp.esthesia on the side affected with the motor paraly- sis. In a few days the case assumes all the characteristics of a typical myelitis and the symptomatology of the hemorrhage sinks into that of this disease. Diagnosis. — The abruptness and paraplegic character of the on- set of the trouble should be carefully noticed. There is no fever and the symptoms tend to recede a little for a few days when a change takes place and the case assumes a myelitic complexion entirely. It is THE NON-NEURONIC DISEASES 53 1 difficult, however, in some cases to distinguish the hemorrhagic from the myelitic manifestations. Fortunately the differentiation is rarely a matter of any consequence as hemorrhage almost always sooner or later develops inflammation in the cord and in myelitis hemorrhage may or may not occur without the progress of the disease being very materially affected. Poliomyelitis is to be distinguished by the febrile symptoms and the symptoms that indicate especially damage to the anterior horns. Hemorrhage into the spinal membranes is different from hema- tomyelia clinically in that there is pain in the back, pains of a radiat- ing character along the course of the spinal nerves, rigidity, spinal- root signs and other indications of meningeal irritation. Some have employed and recommended spinal puncture to positively diagnose hematorrhachis. One would fear an increase or renewal of the hem- orrhage in some cases from the disturbance of the cerebrospinal pres- sure in such a manoeuvre. Prognosis. — This is always serious for if death does not occur early, it will in all likelihood later, from the myelitis. Only large hem- orrhages destroy life early. Improvement often occurs and occasionally complete recovery has happened. A small hemorrhage in the dorsal region holds out the best chances for recovery. When myelitis de- velops, the prognosis depends upon that. If sensation returns early the outlook is hopeful. Early trophic manifestations are discouraging. Some cases recover with permanent muscular atrophy and degenera- tive reactions. In a word, the size of the hemorrhage, the extent and location of the clot and the onset and severity of the subsequent myelitis must all be carefully taken into account in affirming the prognosis. Treatment. — Absolute rest in bed for the first few days and weeks must be enjoined. The patient should lie on his side or abdo- men. Ice should be applied over the seat of the hemorrhage, or as near it as possible. Aconite in small doses may be given to restrain the heart action. Ergot by the mouth in a few large doses, or ergotin hypodermically have been recommended. Their efficacy is doubtful, however. Blood letting is a more powerful measure. The later treatment is the same as that for myelitis. MYELITIS. Myelitis is inflammation of the spinal cord. It may be acute. subacute or chronic. It may occur in any part of the cord. As the clinical picture of the disease undergoes some variations in accordance with the character and location of the lesion, subvarieties of myelitis are described, some of which are of sufficient importance to be given a special chapter. When the inflammation surrounds the edge of the cord it is known as peripheral myelitis, or meningomyelitis, since it is usually associated with meningitis. When it is confined to the gray matter immediately encompassing the central canal it i a periependymal myelitis. When it is confined to the gray matter of the cord it is 532 THE NON-NEURONIC DISEASES known as poliomyelitis. Some authors regard acute anterior poliomyeli- tis as a systemic disease because it is limited to the column of gray matter that makes up the anterior cornua. "When the disease extends completely across the cord it is a complete transverse myelitis; when only part way across it is an incomplete transverse mvelitis. These forms may all be more or less limited to the cervical, dorsal or lumbar regions. Disseminated myelitis shows the inflammation scattered in foci irregularly throughout the cord. All of these forms, except the anterior poliomyelitis, will be dis- cussed in detail under the head of symptomatology. Etiology. — Nearly all inflammations of the spinal cord are caused by traumatism or infection. Blows upon the back, fractures or dis- locations of the vertebrae, stab wounds and wrenchings of the spine are among the former ; acute infectious diseases like varicella, sepsis, hydrophobia, tuberculosis, syphilis, malaria, etc., are among the latter. Gonorrhoea produces it and it may occur in pregnancy and in the puer- perium. It has followed tonsillitis, dysentery, erysipelas, typhoid fever, pneumonia, cystitis, influenza, tetanus and phlegmonous parony- chia. Primary acute myelitis is exceedingly rare. The disease may oc- casionally occur, however, as a primary infection. The micro-organ- isms are numerous, therefore, that enter into the etiology. Among them are the streptococci, staphylococci, pncitmococci, diplococci and many others. Whether the germs themselves or their toxines are the direct cause of the inflammation is still unsettled. The germs are not often found in the cord, but this fact does not argue against their being the direct cause, for it is a well known fact that they disintegrate and vanish as soon as they have accomplished their deleterious work. Op- penheim and Grasset believe that myelitis really results from a mixed or secondary infection. An ascending neuritis has been charged as a cause of myelitis. It certainly has followed certain diseases of the urinary apparatus, caisson disease and arteriosclerosis. Some of the so-called re-Hex paralyses fall in here. Among the intoxicants found to have caused myelitis may be noted carbon dioxide, carbon disulphide, illuminating gas, chloroform and nitrobenzine. Adults of early middle life and of the male sex are chiefly affected. Dana affirms that over one-half of the non-traumatic and non-tuber- culous cases in adult life are due to syphilis. We no longer lay any stress upon cold, exposure, over-exertion, sexual excesses or violent emotions in the etiology of the disease, except as general predisposing factors. Pathology and Pathogenesis. — As myelitis is primarily a pure inflammation there is at first hypenemia, congestion, enlargement and proliferation of the bloodvessels, extravasation of the corpuscles, swelling of the perivascular spaces with fluid and a general softening and obliteration of the normal appearances. Sometimes the cord is so softened that it flows out when the pia is cut. This extreme fluidity is partly a post-mortem change. In the early stage the softened area THE XOX-XEUROXIC DISEASES 533 FIGURE 113. -- FIGURE 114. FIGURE 115 Forms of myelitis. Figure 113. Toxic acute column-form. Figure 114. Chronic myelitis of the dorsal cord. Figure 115. "Compression myelitis," du< cinoma of the third dorsal vertebra. (After Jakob. > 534 THE NON-NEURONIC DISEASES is reddish ; then as the coloring matter of the extravasated corpuscles breaks down the softened area becomes mottled, brownish or grayish- yellow in appearance; and finally with the gradual absorption of these coloring matters it becomes paler and whiter. The term softening applied to these three stages of the process should not be confounded with the softening, or myelomalacia from embolic or thrombotic obstruction. Under the microscope the nervous elements are seen to be most seriously damaged. The axis-cylinders and cellular elements are swollen, which at first causes a puffing up of the cord and ah obliteration of the distinction and line of demarkation between the gray and White substance. As degeneration proceeds in these elements the cord appears yellowish, smaller and even cystic at times. Granular cells are found in abundance, and with the fat drops resulting from the breaking down of the myelin are here and there heaped up. The neuroglia is proliferated and the spider-like cells of Deiters are numer- ous and prominent. In a transverse myelitis this condition of the cord extends about an inch or two, shading off above and below. Usually the pia mater is somewhat thickened over the diseased area and often the whole cord. In severe and secondary cases the cord is often reduced in size, rather hard to the touch and under the microscope shows little but an excess of connective tissue. In the cases less severe, or the parts of the cord less seriously affected, congestion and vascular irri- tation are noticeable, some nerve fibres are intact or partially disinte- grated, and cells in various stages of degeneration are seen. A good deal of amorphous material is present studded with nuclei. Symptoms. — In describing the symptoms of myelitis, the trans- verse dorsal type is taken as the model and the variations from that noted in other types. There are no prodromata in the true sense of the word. When the first symptoms make their appearance, the dis- ease has already begun its regular course. These early symptoms may be of a very fugitive and indefinite character and may come on prompt- ly after an injury or they may be delayed and announce themselves later in a severe and definite manner. For some time after a strain, I have seen the pains diagnosed as rheumatism and the attending physi- cian chagrined to observe all the signs of a transverse myelitis, with a fatal issue, follow. I have seen in consultation a case in which a mere disturbance of the bladder with slight, fugitive pains as the only trouble that sent the patient to his doctor. It is well to be alert when such indefinite, slight, changeable sensory phenomena, with motor weakness, are complained of in the history of syphilis or after an injury, for in many cases they will be seen to be the early manifestations of a genuine myelitis. In the larger number of cases the symptoms appear rather abruptly, and in a few hours or a few days rapidly rise to their height. There is an initial numbness in the legs, beginning in the feet. Pains of a dull, aching character are complained of in the back, around the lower part of the body and even down the legs. The legs feel heavy and weak. The patient notices that his walking is growing stiff and labored. The numbness graduall) increases in area and passes into a condition of anaesthesia. The upper limit of the anaesthesia, usually near the THE NON-NEURON1C DISEASES 535 level of the umbilicus, is fairly well marked. Xear this point is felt a cincture pain or constriction girdle sensation. Sometimes for an inch or two above this the skin is hypercesthctic. The bowels are constipated and there is some incontinence of urine. The temperature may be elevated a degree or two but never as much as in meningitis. By this time the disease has assumed its typical clinical appearance, the symp- toms of which are so characteristic that they call for a little more detail of description. The most obvious symptom of a fully developed attack of myelitis is paraplegia. This paralysis is a true paralysis, spastic in character, and affects all of the muscles of both legs. Both the immobility and spasticity are of course due to the break in the continuity of the cord and the separation of the brain from the lower motor neurones of the lumbar area. The myotatic irritability of the muscles of the legs, as well as the tendon reflexes, are greatly exaggerated. A slight tap causes them to contract violently and when this is observed in conjunction with the profound ancesthesia, the symptom is most impressive. The limbs at first lie extended but as the rigidity of the muscles increases, contractures come on and they become strongly flexed at all of the joints. On this account the patient lies on his back or side with his legs drawn tightly up towards his belly. Often there are twitchings and jerkings and other spontaneous movements in the legs, of which the patient is perfectly unconscious except as he happens to see them. As a rule all forms of sensation are lost in the legs and lower part of the body. This ancesthesia is complete and its upper limit is clearly defined by a ring around the body. At a point above this upper border of the anaesthesia there is a girdle or constriction band which at first may be painful but which later on becomes a feeling of pressure or of a vise-like band. For some little distance above the girdle-pain and around the body the skin is hyperaesthetic. As this anccsthetic border with its hxpercesthctic area above moves up or down with the exten- sion of the lesion, it enables one to make a fairly accurate localization diagnosis of the site and probable extent of the lesion. When the anaesthesia is not complete it is most marked in the sense of touch, less so in that of temperature, and least of all in that oipain The anaesthesia involves the bladder and rectum. On account of this and the paralysis of the sphincters, the functions of the bladder and rectum cannot be controlled by the patient. Incontinence and reten- tion of urine and faces are most significant symptoms of this form of myelitis. Priapism occurs as well as sexual impotence. Bedsores in most cases begin to develop early. These are genuine trophic symptoms, though of course they are favored, by weight and pressure, loss of sensation and consequent uncleanliness. M<>- - these sores occur over the trochanters, the sacrum or in the toes, and sometimes they burrow so deep as to expose tin- underlying b Pyogenic infection plays a role also in their production. Pemphigus and other trophic skin troubles sometimes occur. Among the vasomotor and secretory disturbances are tin- arthro- pathies, cedema and anidrosis. The -kin is rough and cold and con gested. Sometimes excessive perspiration occurs. 536 THE NON-NEURONIC DISEASES There is no muscular atrophy nor electrical change. In a word the cardinal symptoms of the typical form of myelitis, namely, the transverse dorsal, are spastic paraplegia, anaesthesia, exag- geration of the superficial and deep reflexes, cincture-pain, disturb- ance of the vesical and rectal sphincters, bedsores and other trophic manifestations, and certain vasomotor and secretory symptoms. A few words must now be devoted to the changes in the above clinical picture caused by a change in the location of the lesion. When the latter does not extend completely across the cord, all of the usual symp- toms are less complete. For instance, the paraplegia is more of a paresis and is irregularly distributed. The same is true of the anaesthesia and the trophic symptoms. The symptoms may even be unilateral or they may be more pronounced on one side than on the other. In some rare cases Brown-Sequard's phenomenon is present, namely, paralysis on the side of the lesion with anaesthesia on the opposite side. The lesion, moreover, may extend up and down the whole dorsal, or in- volve a part of the dorsal with the lumbar region. It may be completely or incompletely transverse. The symptoms under these circumstances will be slightly modified but in a way that can be easily imagined. In the main they will represent the general picture, but the reflexes will be abolished, the paralysis will be more profound and the atrophy will be more distinct. According to Bastian, when the lesion is such that there is a total destruction of the conducting tracts above the reflex- arc, that reflex will be decreased or annulled, whereas in a partial or incomplete destruction it will be exaggerated. This is not entirely proved, however. When the disease is of a slowly progressive charac- ter, the reflex functions of the cord may be retained and the muscular tone may remain unimpaired, thus modifying Bastian's hypothesis to a certain extent. In compression of the cord, where the anatomical lesion is slight or incomplete, there may be a physiological loss of function, and the same may be true of the functions of the cord below a transverse lesion. At all events, as Warrington has pointed out, no case of incomplete division of the cord has been recorded in which all the symptoms described by Bastian were present, and in which the lumbar enlargement was intact. It was Van Gehuchten who taught that the reflexes depend upon the tone of the motor ganglion cells, as well as upon the integrity of the reflex-arc, and that therefore the reflex may be annulled by a complete destruction of conduction in the spinal cord, and exaggerated by a degeneration of the pyramidal tracts. This may explain in part the exaggeration of the knee-jerks in some cases of transverse myelitis, particularly the slowly progressing or high dorsal cases, and its abolition in some other cases, such as the severe and complete cases of the lower dorsal or dorso^-lumbar type. When the disease is entirely of the lumbar or lumbosacral type, the paralysis and anaesthesia are profound, the reflexes are lost, and the implication of the bladder and rectum is decided. The paralysis is of the flaccid type with atrophy and the reaction of degeneration. There are shooting pains in the legs but no girdle sensation about the body. Priapism followed by loss of the sexual function occurs. A cervical myelitis involves all four extremities, quadriplegia or THE NON-NEURONIC DISEASES 537 paraplegia, and trunk in the paralysis and anaesthesia. When the lesion is in the lower part of the cervical cord the paraplegia is spastic in the legs and atrophic in the arms. Moreover there are oculo-pupillary symptoms from implication of the ciliospinal center. When it is in the upper cervical region the arms as well as the legs are spastic and paraplegic. Encroachment of the lesion upon the phrenic center causes a disturbance in the action of the diaphragm. Sometimes in these high forms of myelitis, there are medullary symptoms such as cardiac and respiratory disturbances. In a central or periependymal myelitis the symptoms bear a re- mote resemblance to those of syringomyelia, namely, muscular localized atrophy, disturbance of pain and temperature sense with preservation of tactile sense, and symptoms referable to the viscera especially and vasomotor and secretory apparatus. There may be some pain and irritation but they are distinctly less than in the other forms of mye- litis. Compression myelitis of the periphery of the cord, is characterized particularly by irritative symptoms involving the spinal nerve roots. It is slow in onset and usually being but a part of a meningomyelitis it reveals meningitic symptoms in addition to the myelitic. Therefore it is accompanied by muscular twitchings, spasms and contractures, sharp pains along the course of the nerves, incomplete anaesthesia and paralysis, late involvement only of the sphincters and slight trophic manifestations. The reflexes, however, are exaggerated and sooner or later there is a spastic paraplegia. A disseminated myelitis involving the cord, medulla, pons and brain is the type that follows as a rule the infectious fevers. These cases are not entirely unlike multiple sclerosis in their symptomatol The motor, sensory and trophic symptoms bear the same general char- acteristics noted under the head of the latter disease, and depend upon the extent and location of the foci of disease. Diagnosis. — The diagnosis of myelitis is not difficult in the typical cases. It is the modified and complicated forms that give the most trouble in this respect. In making a diagnosis special attention must be given to the history of the case, its mode of onset and progT the distribution of the individual symptoms and the total clinical pic- ture that is presented. It is well, as a rule, to keep in mind the cardinal symptoms of a typical transverse dorsal myelitis, and with this general clinical picture as a sort of schema, to assign the aberrant symptoms that may appear with, or in place of, the symptoms of this schema to the possible changes in location of the inflammator) Focus. Most of the cases of myelitis begin abruptly and attain their climax rapidly. Paraplegia, anaesthesia, altered reflexes, disturbed function of the bladder and rectum, decubitus, and other trophic, vasi motor and secretory manifestations soon announce themselves in all their com- pleteness. There may be a chill with considerable elevation of tem- perature. Pain is comparatively slight in these acute cases. I frequently the cases are subacute and rarely they are chronic. M of the chronic cases turn out to be multiple sclerosis with predominat- ing spinal symptoms. 538 THE NON-NEURONIC DISEASES From progressive muscular atrophy and amyotrophic lateral sclerosis, this disease can be readily distinguished by its febrile symp- toms, its rapid onset and course, its more prominent sensory mani- festations, its involvement of the sphincters and its lesser degree of muscular atrophy. Spinal tumor is sometimes accompanied by a focal myelitis. It is characterized by slow onset, unilateral symptoms not unfrequemtly of the Brown-Sequard type, and signs of spinal nerve root irritation and compression. The metastatic origin of the neoplasm can sometimes be made out and in some cases it can be definitely localized. In locomotor ataxia there is no true paralysis, but on the other hand a history of syphilis, ataxia, Argyll-Robertson pupil and severe sensory disturbances of a progressive character. Brain palsies are hemiplegic in distribution, spastic, painless, without muscular atrophy or involvement of the sphincters. Multiple sclerosis often resembles and sometimes even develops on top of a disseminated form of myelitis. The differentiation is extremely difficult when the spinal symptoms of multiple sclerosis are so prominent as to obscure the cerebral symptoms. In all cases the head symptoms should therefore be most earnestly sought for and if the case is one of multiple sclerosis there will be found some degree of optic atrophy, diplopia, dysarthria and tremor of the so-called intentional type. Cervical pachymeningitis is slow and progressive as a rule, but whether acute or chronic is char- acterized especially by the irritative and compression symptoms of a typical meningitis with the symptoms of the peripheral myelitis coming on later. Spinal meningitis is to be differentiated by the irritative root symp- toms, the tenderness of the spine upon pressure and the somewhat more marked febrile symptoms. The intense shooting pains along the course of the spinal nerves are characteristic. Moreover the paralysis and atrophy are not so segmental as neuritic in distribution. Hysterical paraplegia may have slight sensory symptoms, some atrophy and a spurious ankle clonus. The special stigmata of hysteria, the psychoneurotic character of the symptoms can scarcely be mistaken if care in the examination is exercised. Other forms of paraplegia are the senile from arteriosclerosis ; traumatic, from operations on the bladder, etc., and neuritic. Prognosis. — The outlook of every case of myelitis is problemati- cal. Unfavorable as it is in many of the cases, I am convinced that generally too pessimistic a view is taken in regard to its cure. Some cases, small in number to be sure, do get well, or at least recede and pass into a mildly chronic or latent condition. Some of these cases improve, then relapse or remit, then improve again only to relapse once more and so on until in the end they die in the usual way. The most hopeful cases are those that follow the acute infectious diseases, especially when the myelitis is of the disseminated type. Intermittent and malarial cases often recover. Syphilitic cases sometimes do. Gonorrhceal cases always get well under proper management. Tbe subacute, chronic, in- sidious, slow-progressing complete types are perhaps the most unfavor- able. Patients sometimes live many years with this type of disease. THE NON-NEUROXIC DISEASES 539 The rapid cases, as a rule, soon cease to progress and even when asso- ciated with meningeal and neuritic symptoms are not unfavorable. Much depends upon the constitution of the patient, the amenability to cure of the underlying etiological disease like caries, tumor, etc., and the willingness to take treatment and the patience on the part of the individual afflicted. I have seen simple myelitis hurried to a fatal issue by injudicious electrical and "osteopathic" treatment. Dorsal myelitis is the most hopeful form of the disease from the ■standpoint of the location of the lesion. Bedsores, cystitis and long- continued, complete paralysis are bad signs. Recession or disappear- ance of sensory symptoms is a particularly favorable sign and usually prophesies complete recovery. Death, when it comes on, usually is the result of exhaustion, cystitis and bedsores. Treatment. — The treatment of a case of myelitis calls for a high degree of patience and skill. The value of a masterly inactivity is sometimes most beautifully exhibited in the management of this trouble. On the other hand when there is a clear realization of the origin, nature and extent of the disease process a persistent, energetic treatment of the right sort will sometimes almost do wonders. Therefore unless the practitioner knows exactly what he is attempting to do, and is familiar with the best means of doing it, he had much better leave the case alone and do nothing. In this way he will at least escape the charge of having brought on a complete paraplegia and of having hastened the end. Under the best conditions the treatment of myelitis is not a grate- ful task; for it is slow and tedious and is punctuated by most surprising and at times thoroughly discouraging relapses. The patient had better be well forewarned of this before the treatment is undertaken and also be frankly told that he may have to lie in bed many months. The prime element in the treatment is rest, absolute rest, mental and physical. If we could but write a prescription for rest as we do for a drug, so as to bring it home to the patient's realization that in this disease rest, rest, rest, is of even greater therapeutic value than am drug, what better results might we not obtain! But t<> tell a patient to keep quiet and to do little but lie down for months and months seems so trite and inert that it is hardly to be wondered at that he rebels and demands some positive form of activity. Here is where a large part of the failure to successfully cope with myelitis is to he charged. I have had under my own care eases that under the a': to remain quiet were after several months progressing most satisfac torily. Trie symptoms were receding and the outlook was becoming decidedly hopeful. Too hopeful, unfortunately, for the patient fondl) imagining that he was about well, got up, started on a long W alk or some other prohibited exercise, only t<» hasten back to bed again in a few hours with the return of pains, numbness and muscular weakn I have had such experiences several times in the course en a single case. Under such circumstances the discouragement is that one is almost tempted to abandon the case and leave the patient to his fate. ( )n the other hand 1 have seen mosl happy results in c that were submissive and that maintained tin- prone p >siti *i for a 54Q THE NON-NEURONIC DISEASES pericKi of time. Given such a willing patient, I do not relinquish all hope of cure, or at least amelioration, until some eighteen months have rolled around without any evidence of improvement. Cures have taken place even after such a long and discouraging experience as that. The second fact to bear always in mind in the early treatment of myelitis is that the disease process is an infectious inflammation. Elimination, therefore, is more important than counter-irritation. A diaphoretic treatment is preeminently called for in this progressing period of the disease. The bowels should be kept well open with calo- mel, salines and other forms of catharsis. The kidneys ma}- be stimu- lated by diuretin or other mild diuretic. The activity of the skin can be provoked by tepid spongings. Full baths had better be avoided until the disease has ceased to progress, and, even then they must be used cautiously and not too frequently. Only lukewarm baths are advisable, and generally they had better be half-baths given in bed and followed by gentle frictions. Hot and cold baths, or other form of hot and cold applications, are positively contra-indicated. Sea-bathing must be prohibited and treatment at the various hot springs and sana- taria is of very doubtful utility. They necessitate too much movement and are apt to be too stimulating. Counter-irritation of the spine is, in my opinion, of little benefit unless it is so mild that it can be used for a long period almost con- tinuously. Iodine, fly-blisters, vesicants of various sorts, the actual cautery and the seton have not been sufficiently beneficial in my obser- vation to encourage me in the use of them. Moreover, they have ad- ded too greatly to the general distress of the patient. I depend more upon the constant application of sinapisms, diluting the mustard some- times with ground flaxseed and changing them about on the back so> that the patient can wear them almost continuously. In this way they soothe the patient and relieve the pain ; above all they do not materially add to his discomfort. Among the remedies that may be employed, iodide of potassium still holds the first place. It must be used steadily and in moderate doses. In syphilitic cases the iodide in large doses and mercury are of course indicated. In the non-syphilitic cases of myelitis I am just as- urgent to contra-indicate the use of mercury as I am to indicate it in the cases of meningitis. If there are symptoms of meningomyelitis I use mercury steadily for a long period of time, guarding it with a small amount of opium. The use of opium or its alkaloid is sometimes advisable to relieve the pain and induce quietude for a time in the early management of the case. I have had no experience with ergot and cannot understand upon what principle it is recommended. Silver in the form of the nitrate has seemed to help some cases; so have the arsenite of sodium,. the salicylates, chloride of gold, and phosphorus. Later on small doses of strychnia, or the hypodermic injection of strychnia, may be employed to combat the paralysis. Cystitis must be carefully guarded against by clean catheterization and flushings and the use of buchu, boric acid, sandalwood and similar drugs. The skin around the scrotal region must lie carefully protected by a urinal or an antiseptic pad. THE NON-NEURONIC DISEASES 54I For bedsores as well as for the atrophic changes aggravated by am- moniacal urine, Palmer recommends the use of a soft linen bag large enough to extend down the thighs and filled with bran moistened with -dilute sulphuric acid. Two ounces of the dilute acid may be used to a quart of bran and it should be renewed every second day. Care in avoiding points of pressure, and the use of a water mat- tress or water pillows, will obviate the development of bedsores. Contractures cannot be forestalled, even morphine and hyoscin usually failing. A permanent bath is sometimes good for .them Tenotomy is of course to be considered in connection with severe forms of contracture. Special forms of myelitis sometimes call for special lines of treatment and such etiological factors as syphilis, malaria, etc., indicate the proper mode of medication. In chronic meningomyelitis much benefit is sometimes obtained from suspension and the spinal jacket. Stefano Personali especially claims good results from this treatment in this form of the disease. He employs the usual apparatus for sus- pension daily for one to four minutes for four days, then for four minutes for twelve days, after which the jacket is applied and worn in bed for three months. W 'hen the patient can walk a little about the house, this jacket is removed and another one applied. This is worn for two months, the patient not being kept so strictly in bed as before. A third jacket is then put on for three months, being made of strong muslin. In some cases the suspension may be made on an inclined plane and then the jacket be applied. Surgery may have a field of usefulness in myelitis aside from the mere removal of tumors, carious bone, etc. ; for in such operations the associated myelitis has been cured. In a case of typhoid myelitis in a young man, rest in bed with carefully arranged coordinating and resisting exercises, the exercises being continued and elaborated after he began to get out of bed, pro- duced happy results in a case of J. K. Mitchell. After recovery all cases must be urged to avoid that which may tend to bring on a relapse. Mountain climbing is therefore dangerous. A sea-voyage is excellent. A quiet, wholesome life, without strain and with plenty of fresh air and nourishing food must be sought after. In this way a recovered case of myelitis may live a normal age without airy- reappearance of the old symptoms. ACUTE ANTERIOR POLIOMYELITIS OF INFANTS. This is a common disease and is sometimes known as infantile palsy, acute atrophic spinal paralysis, or cornual myelitis. As it is primarily an inflammation it should he treated of under the general head of inflammations of the spinal cord. By some writers it is consid- ered among the systemic diseases of the cord, because the primary dis- ease process is limited to the column of gray matter that makes up the anterior horn, with mere secondary processes in the peripheral m neurones. In my own opinion, logical sequence demands that it should be distinctly ranked under the head of a localized inflammation of the 542 THE NON-XEURONIC DISEASES cord, for reasons which will appear when I discuss its pathology and pathogenesis. The disease is characterized especially by a flaccid paralysis and atrophy with certain consequent manifestations, the paralysis and atrophy being limited to individual muscles or groups of muscles, because of the damage to ' their representative cells in the anterior horns. Etiology. — The most striking feature of the etiology of infantile palsy is the age at which it occurs. Though it may appear at any period of life it is preeminently a disease of early childhood. The great majority of the cases occur in the second and third years, when the child is beginning to walk and is undergoing the process of denti- tion. The disease is very rare before the fifth month and is not often seen after the fourth year. According to Sinkler it has been known to start before birth, giving rise to a congenital type of the disease. It is slightly more frequent in boys than in girls. Heredity shows its influence, probably neuropathically, in a very small percentage of cases. It has occurred in several members of the same family. The great majority of the cases take place in the hot months of the summer. It would seem chat certain, time-honored alleged causes, such as sudden chilling of the body, over- exertion, traumata, etc., would be more operative at this time when the child is more out of doors and at greater liberty than in the winter when he is restrained and closely protected. And yet it is now pretty generally agreed that these supposed causes are subsidiary or at least especially operative in only a limited number of cases. The cause of infantile palsy is without doubt some form of in- fection, This is shown by the course of the disease, the circumstances under which it usually occurs, its appearance with other forms of in- fectious nervous diseases like cerebrospinal meningitis and neuritis, and by the fact that it has been not infrequently observed to occur epidemically. Moreover, micro-organisms have been introduced ex- perimentally into the bodies of animals and alterations have occurred in the cord that simulated perfectly those of, the disease under considera- tion. In addition to all this, the disease has followed other forms of infection such as scarlet fever, measles, whooping cough, etc. Most remarkable and suggestive, however, are the epidemics of anterior poliomyelitis that have been reported. In the outbreak in Vermont in 1894, reported by Dana, some 160 cases appeared from June to September in the country along the Otto Creek. In the same year Putnam reported an irruption that occurred in and about Boston. Medin, of Stockholm, described an epidemic in which there were 44 cases in the same town within one month. That the disease is endemic rather than epidemic is suggested by the fact that Oppenheim voices when in his own particular experience he says that most of his cases have seemed to come from Weissensee, near Berlin. The Gloucester, Mass., epidemic that occurred in the summer of 1900, and was reported by Painter, occurred within a radius of four miles and included some 38 cases. The influence of sudden changes of temperature as a contributive cause along with the infection is strikingly shown in Painter's report. THE XOX-XEUROXIC DISEASES 543 The majority of the cases occurred in, or more frequently twenty-four or thirty-six hours after, periods of extreme heat ; and in some in- stances immediately after a sharp drop from an abnormally high tem- perature, or a bath in the ocean. The disease has even attacked domes- tic animals, such as fowls, dogs and horses. In the Vermont epidemic a typical case occurred in a man seventy years of age. Pathology axd Pathogexesis. — The essential lesion in this dis- ease is an inflammation of vascular origin in the anterior gray horns of the spinal cord, with subsequent degeneration and disappearance of the ganglion cells and their processes. The work of Marie and Gold- scheider, Redlich and Siemerling seems to intimate that the process is primarily a general inflammation of the cord and that while the entire cross-section is involved the maximum point of the trouble is located in the anterior horns. An infection is the origin of the inflam- mation, the infectious material (an infectious embolus possibly) reaches the anterior horns by way of the anterior spinal artery. The peculiarity of the blood supply and the relative abundance of the ganglion cells in the lumbar and cervical portions of the cord, explain satisfactorily the preference of the disease for these localities, both in its pathology and symptomatology. The macroscopic and microscopic appearance of the cord and lesion will vary according to the period of the disease in which the examina- tion is made. Most of the earlier examinations of Charcot and others were made in the later stages when the inflammation had long sub- sided and only the atrophic condition was in evidence, and explain the earlier teaching, now known to be erroneous, that the disease is primarily a parenchymatous inflammation of the ganglion cells of the anterior gray horns. Since the investigations made by (ioldscheider, Siemerling, Dauber, Marie, Redlich, Von Leyden, Schultze and others we now know that the disease process is primarily a vascular inflamma- tion with the atrophic changes occurring as secondary results. In the first stage, therefore, we find hyperemia, proliferation of the terminal branches of the anterior spinal artery, dilatation and hypertrophy of the vessels, perivascular effusion and exudation, thrombotic obstruction, extravasations of blood, and leucocytosis. There is a heaping up of the round cells about the vessels and a clouding of the elementary structures. Xo suppuration occurs. The outlines of the horn are ob- scured ; it looks swollen, and in many cases the adjacent parts of the cord and meninges are softened and congested. As the process ad- vances, the ganglion cells become hazy, swollen and involved in an incipient chromatolytic change. Their processes are swollen and exhibit a beginning degeneration. Later on the cells and fibres arc distinctly atrophied. Some of them disappear entirely and their places taken in part by an overgrowth of connective tissue. The involvement of the neighboring white substance decreases. The anterior horn diminishes and shrivels and the cord seems in the vicinity smaller and harder. Sometimes there is an associated sclerosis in the lateral columns. The anterior roots and motor nerves, of course, are atrophia 1 and the re- lated muscular tissues arc wasted and show an overgrowth of con- nective tissue. Alterations of this same character have been observed 544 THE NON-NEURONIC DISEASES in the motor nuclei of the medulla oblongata, since these nuclei are but the upward extension of the anterior gray matter of the cord. When the disease is among the lower medullary nuclei it is known as inferior polioencephalitis ; when among the upper or nuclei of the motoroculi, it is spoken of as superior polioencephalitis (ophthalmo- plegia). It is to be noted that the branches of the anterior spinal artery are, in Cohnheim's sense, terminal arteries and that, as Schaefer has shown, no one set of arterioles is limited in its distribution to any one group of cells. The supply of the posterior parts of the cord by the posterior spinal arteries is comparatively scanty. These facts help to explain the peculiar limitation of the maximum focus of the disease to the anterior or motor part of the cord and the symptoms of paralysis and atrophy in particular muscles or groups of muscles. Symptoms. — The symptomatology of acute anterior poliomyelitis is marvelously definite and uniform. The clinical picture develops and progresses through stages that are singularly distinct and logical. A typical case, except in the very earliest onset of the disease, presents no great difficulties in diagnosis. Remembering the pathology, one can readily understand why every case should show progressively the fol- lowing four symptomatic stages, namely, (i) infectious fever, (2) widespread, sudden, flaccid paralysis, (3) recession of the widespread paralysis with permanent paralysis and atrophy in certain individual muscles, and (4) deformities resulting from wasting of the muscles and contractures. Such is the regular clinical picture and progress of infantile palsy. I will now discuss the symptoms of these four stages of the dis- ease a little more in detail. The beginning of the disease is usually abrupt and unexpected. After a happy day with its playmates, romping in the open air, the child comes in and complains of extreme weariness and distress. Its head begins to ache. It is nauseated and may vomit. It is listless and clings to its mother's arms. Perhaps it has a violent chill. The face becomes hot, dry and flushed. It may soon become stuporous and even slightly delirious. There may be a general convulsion. The child re- fuses all food and in every way shows that it is decidedly sick and feverish. With or without the advice of the family physician, the mother regards and treats the condition as probably a cold, an acute attack of indigestion, distress from the teeth or the possible beginning of an attack of measles. It is put to bed and passes a feverish and restless night. The temperature has been anywhere from 102 degrees to 104 degrees F. In the morning the child wakes with perhaps a slight diminution of the febrile symptoms but completely paralyzed in the legs or arms. This is the frequent way that the disease starts. The febrile stage lasts from a few hours to a few days. Sometimes it is so brief and the paralysis comes on so suddenly, that one involun- tarily thinks of a hemorrhage. At other times it is so prolonged that for many days no diagnosis is possible. For his own credit's sake, the physician will always, in such doubtful cases, keep in mind the possibility of acute poliomyelitis and inform the family of that pos- THE NON-NEURONIC DISEASES 543 sibility. To the average layman paralysis is a most awful disaster, and though, when the paralysis comes on, the physician may assure the family that it will in a large measure disappear or recede, it will not FIGURE 116. Anterior poliomyelitis, showing atropine condition of the righl leg. \)v. Fitch's ease. restore' the confidence shaken by the shock of seeing their child sud- denly paralyzed and by their not having been forewarned of such a possibility. When the paralysis is discovered the general febrile disturbance 546 THE NON-NEURONIC DISEASES has usually subsided somewhat. There may be some rheumatoid pains complained of in the back and extremities for a few days, or there FIGURE 117. Anterior poliomyelitis, showing the atrophic condition of the right Kg. may be retention of urine or some slight gastrointestinal trouble. The paralysis, however, is the dominant symptom. This is flaccid and THE XOX-XEURONIC DISEASES 547 complete from the beginning. Usually it is paraplegic in distribution, involving both legs entirely. Sometimes it involves only one leg, or one leg and one arm, or one arm alone, or both arms. Paralysis of one leg or of one arm is by far the most frequent way it appears. Paralysis of both legs is perhaps the third most common distribution. Paralysis of the four limbs, quadriplegia, or of the two arms alone, crossed paralysis, or hemiplegic paralysis, is very rare. Usually the paralysis includes the whole limb and reaches its height in a few hours or a few days. In a short time it is noticed that some of the muscles of the limb are regaining their mobility while others continue immoble. In other words the volition of the patient can exercise itself upon some of the muscles or muscle-groups but not on others. The extreme paralysis in the latter may last from six to eight weeks, when there is a little recession in their paralytic state. These muscles now begin to degene- rate, waste and undergo a progressive and rather rapid atrophy. Their electrical examination reveals the phenomena of the reaction of de- generation. The reflexes in which the atrophied muscles are concerned are lost. These four symptoms, flaccid motor paralysis, loss of the tendon reflex, atrophy and the reaction of degeneration are pathognomonic of disease of the lower or peripheral motor neurones. Hence the pre- eminent characteristic of the paralysis of infantile palsy is that it is a degenerative one, and that it picks out particular muscles and muscle groups. The muscles of the leg that are mostly affected are those of the anterior tibial group. Sometimes only the extensors of the leg are involved. The sartorius usually escapes. In the arm the deltoid and shoulder muscles are chiefly inplicated. The deltoid may be paralyzed alone, or in combination with the biceps, internal brachial and supinator longus. All kinds of combinations are possible. In a few cases the motor cranial nerves have been included. A slow improvement, may take place in some of these muscles up to the end of a year. After that no further improvement is to be expected. Gowers says that we should never forget that after six months the lesion in the cord has practically become a cicatrix. There are no sensory symptoms that belong to this disease. Some- times in the beginning there are dull pains of a rheumatoid character in the muscles. If these pains become severe or the muscles arc unusually tender on pressure, there is probably some degree of peri- pheral neuritis with the poliomyelitis. In a word the sensations are absolutely normal in uncomplicated infantile palsy. The slight de- crease in sensibility in the paralyzed limb is undoubtedly due to the vasomotor disturbance and lowered temperature of the skin. Some- times this decrease of surface temperature amounts to several degrees and is associated with a bluish, mottled appearance of the skin. There are no psychic troubles excepl the earl) febrile stupor and delirium and the sphincters arc never involved. The whole limb ceases to grow as rapidly as the well limb; the bones do not continue to develop: and as a result the whole extremity appears stunted, shortened and awkward in movement. ( )n account of the atrophy and contractures in certain of the muscles, antagonistic 548 TIIK N ON -NEURONIC DISEASES groups do not work together as they should and deformities and curva- tures result. Talipes equinus, talipes varus and valgus are thus pro- duced, as well as deformities about the knees and curvatures in the spine. The most common form of foot deformity is pes equino-varus on account of the paralysis of the extensors of the foot and toes. When FIGURE 118. Anterior poliomyelitis, showing the wasting of the right leg and foot-drop. the tibialis anticus alone is affected, there is a condition of talipes valgus. When the calf muscles are stricken, pes calcaneus obtains. The claw- hand is a far less frequent form of distortion than are any of those of the feet. In the spinal column there may occur lordosis or scoliosis. Diagnosis. — In the early or febrile stage the diagnosis of acute anterior poliomyelitis is always uncertain and sometimes quite impos- THE NON-NEURONIC DISEASES 549 sible. Even after the discovery of the paralysis, there may be doubt as to the exact cause of it. Very rarely, therefore, is the diagnosis of infantile palsy ever made early. The symptoms at this period of the disease are made light of. They are attributed to dentition, gastro- intestinal trouble, over-exertion and heat, intestinal parasites or some other trifling ailment. The parents are assured that the little one will soon be all right. One can easily imagine the feelings of the parents and the chagrin of the physician under these circumstances when the paralysis is recognized at last. The early diagnosis of the disease is most desirable therefore for more reasons than one. The physician will do well to remember that the paralysis is the special, significant symptom and after forewarning the family, be on the alert for the first indication of it. It usually comes on quickly, even sometimes with the beginning of the fever, rarely later than a few hours or days. An appearance of weakness, listless- ness, exhaustion, disinclination to move the limb should be prompt to awaken suspicion. A close examination will sometimes show very early a little more voluntary mobility on one side than on the other. Such indications, faint as they may be, had better be regarded as the forerunner of a paralysis than be erroneously attributed to the mere malaise of the febrile state. When the paralysis does frankly declare itself, certain characteris- tics about it render the diagnosis certain. Its suddcimess of onset, its completeness from the beginning, its involvement of one of the limbs with later limitation to particular muscles, its gradual improvement, and its association with atrophy, loss of reflex action and the reaction of degeneration render its nature perfectly clear. In the later stages of the disease, the history of the case, the local- ization of the paralysis, the muscular atrophy, the vasomotor disturb- ances, the retardation of growth and the contractures and deformities in the limb make the diagnosis positively easy. A few atypical forms of the di sense may cause some confusion in differentiating them from hematomyelia, acute rachitis, coxitis, osteo- myelitis, syphilitic pseudo-paralysis, acute myelitis, acute disseminated myeloencephalitis, birth palsies, cerebral pals; \e muscular atrophy, lumbar spinal gliosis, congenital muscular defects, and multiple neuritis or neuritis of special ner- In hemorrhage and acute myelitis there are prominent sens symptoms, exaggerated reflexes, involvement of tin- sphincters and bedsores. Hemorrhage, of course, is sudden but does not occur often without trauma. Moreover the paralysis is not preceded by or im- mediately associated with a fever. Myelitis is slower in its onset; its fever is not so high usually and is longer in duration ; its paralysis d<>es not recede and remain permanent in particular muscles as it poliomyelitis. It should be remembered in this connection, however, that the earliest symptoms of infantile palsy are so indicative of a trans- verse myelitis in many cases that some authorities believe that tl of anterior poliomyelitis begins as a mor< complete transv myelitis of a mild degree, with the infiammati receding, except from the anterior horns where its maximum foci were located. I his 55° THE NON-NEURONIC DISEASES is doubtless the explanation in part of the early sensory symptoms like the rheumatoid pains often complained of in poliomyelitis and of the initial paraplegic-like distribution of the paralysis. The hyperaesthetic condition of the involved limb during the first few days of the disease is of much assistance in the making of the diagnosis. Of course as the inflammation subsides these sensory manifestations disappear and as I have said above the further course of the disease is notable for the complete absence of sensory phenomena. As Oppenheim has well pointed out acute rachitis, coxitis, and osteomyelitis are distinguished from the early stage of poliomyelitis by the resistance offered by the child when passive movement is attempted. He contracts his muscles and holds them in a state of rigidity to avoid the pain of movement. Syphilitic pseudo-paralysis is to be established by the history of infection, other signs of specific disease, and the character and distribu- tion of the paralysis. Cerebral palsy comes on abruptly, is hemiplegic in character and is accompanied by rigidity and exaggeration of the reflexes. There is mental depression of a profound sort and stupor, while a series of local or Jackson i an convulsions may occur. Birth palsies are flaccid and degenerative and involve usually only the arm. Progressive muscular atrophy rarely occurs in children. It is gradual in its onset, slow and steady in its progress, without febrile or sensory phenomena. Peripheral neuritis, and especially multiple neuritis, are rare though they do occur in children alone or as complications of poliomyelitis. The similarity of- many of the symptoms sometimes leads to much difficulty in the making of a differential diagnosis. They are more gradual in onset than infantile palsy, are marked by local pain and tenderness on pressure and have slight or no febrile phenomena. When there is fever it lasts longer than the fever of poliomyelitis. The paralysis assumes the bilateral type in multiple neuritis and the mono- plegic type in simple neuritis. It is tardy in its development as com- pared with the paralysis of poliomyelitis. The atrophy is not quite as pronounced and is slower in appearing, while vasomotor disturbance such as oedema may be present. In neuritis there is a greater tendency of involvement of the cranial nerves. It is to be remembered that peripheral neuritis and poliomyelitis may be associated in the same patient. Prognosis. — The prognosis of acute anterior poliomyelitis in re- gard to life is absolutely favorable. In regard to the recession of the paralysis in part and improvement within the first six months or a year it is favorable. In regard to any further improvement after the first year it is absolutely bad. Death has been reported in the first stage, possibly by involve- ment of the bulbar nuclei or other complication. Neither the disease nor its sequelae cause a fatal issue except in the rarest instances. A few complete recoveries have been observed. Improvement — and some- times a great deal of improvement — is what may be usually looked for. THE NON-NEURONIC DISEASES 55 £ The amount and character of the improvement, and the degree and nature of the sequelae, can be influenced by the early treatment. Elec- trical examination helps much in forecasting the probable improvement. Muscles that still respond to the faradic current after two or three weeks will resume their normal functions. When they show the re- action of degeneration they will remain paralyzed and later on become atrophied. Treatment. — The early treatment of infantile palsy is antipyretic and constitutional; the middle treatment \sneurological and orthopaedic ; the later treatment is orthopedic and surgical. In the beginning of the disease the clinical picture is that of an infectious fever. Every effort at this time should be made to check the infective inflammation. The child should be put to bed and kept absolutely at rest. The bowels should be opened freely with calomel ; the kidneys stimulated by some simple diuretic ; and the skin kept active by the use of hot drinks and warm coverings. A few leeches may be used on the spine, or mustard or iodine applied. It is useless and in- advisable in an infectious inflammation of this sort to apply extreme heat or cold to the spine, as is done is meningitis. The spinal ice bag should never be used. Full baths, hot or cold, produce too much dis- turbance and excitement ; do very little good ; and may do a great deal of harm. The fever can best be controlled by aconite and sweet spirits of nitre. The salicylates and belladonna in full dosage have been recom- mended in this initial stage of the disease. I have found rest and quiet, gentle but steady elimination, with mild local counter-irritation with mustard the most advantageous way of managing the first or febrile stage. Usually the paralysis is complete, and has existed for some little time when the patient first comes under medical observation. The treat- ment now becomes almost entirely mechanical. It consists of the steady use of massage, aided by electricity. Of course the patient's general health and nutrition must be kept up, but except for assisting in doing this the further use of drugs is uncalled for. As the result of my own personal observation, I consider the steady use of massage the prime factor in the management of this stage of the disease. Every day, sometimes twice a day, I have the paralyzed limb rubbed and stroked up towards the body. The hand of the operator may be well oiled with olive or cocoanut oil. Ten minutes should be occupied in each seance. The muscles later on may be kneaded as well as stroked. Passive movements of the limb should be instituted early and gymnastic exercises with slight resistance to the movements should he persistently carried out to avoid as much as possible the late contractures and de- formities. It may even he necessary to use stiff bandages, splints, rub- ber muscles and other forms of apparatus t<> assist in this. The early and persistent treatment of these cases in this way cannot be too stren- uously insisted upon. Many of the later deformities and 1<eripheral neuritis. The further progress of the disease is the same as it is in acute anterior poliomyelitis of children. In the chronic type of the disease there is merely an initial weak- ness in the legs or tlu* arms, which progresses slowly and gradually towards paralysis. Usually one <>r two limbs, and more particularly certain groups of muscles in them, only are paralyzed. In other ca all four extremities may be involved. The paralysis is flaccid, dec! atrophic and associated with electrical reaction of degeneration and loss of reflex action if the muscles concerned in the reflex arc arc implicated. Almost any muscle or muscle group in the arm or leg ma} be aftli and it is therefore needless to specify. Fibrillary twitching is often 554 THE XOX -NEURONIC DISEASES present and annoying. There are no other symptoms in typical cases though sometimes dull aching pains are complained of. The disease is so varied in its manifestations that an effort has been made to establish particular types. This it seems to me is un- necessary though it is well to remember perhaps that some cases ad- vance rapidly to a climax, then recede and ultimately return to almost complete health. Other cases advance slowly and with sharply limited atrophy and paralysis, causing them to bear a striking resemblance to progressive muscular atrophy. In still other cases there is a less rapid but more steady advance of the trouble until death occurs in a year or two. In. all of these forms of the disease there are resemblances of a striking sort to multiple neuritis, progressive muscular atrophy, acute ascending myelitis, Landry's paralysis, etc. Indeed it is beginning to be strongly suspected that some of these latter affections, either in part or whole, are poliomyelitic. Diagnosis. — The symptomatic diagnosis of poliomyelitis is readily made even though the case is not strictly typical ; the pathological diag- nosis, however, is often a matter of the greatest difficulty. The etiolo- gical precedents, the initial fever and paralysis, the distribution, char- acter and progress of the latter, the muscular atrophy, the electrical reactions and the absence of sensory symptoms are distinctive if studied en masse in the clinical presentation of the case. The underlying cause of these symptoms, however, may be confused with polyneuritis, pro- gressive muscular atrophy, amyotrophic lateral sclerosis and gliosis. In polyneuritis there are peripheral and local pains in the nerves and muscles of the limbs. There is ataxia. The paralysis is not so sharply elective but involves the ends of the lower limbs first, gradually ascends and ultimately attacks the arms in the same way. Other nerves besides those of the extremities, as for example the cranial nerves, may be involved in neuritis. There are some cases, however, in which a differential diagnosis is quite impossible. This is probably due to the fact that in toxic cases there may be both neuritic and poliomyelitic processes going on at the same time. Not a few authorities believe that in some of these cases, if not all of them, the peripheral, neuritic de- generative process is a mere secondary result of a profound toxic dis- turbance in the anterior cornual cells and their nutritive function. Progressive muscular atrophy is especially liable to be confused with chronic poliomyelitis. It is to be remembered, however, that a low grade poliomyelitic process possibly underlies many of the spinal atrophies. In typical progressive muscular atrophy the atrophy runs ahead of the paralysis and not vice versa, as in poliomyelitis. Moreover there is no real initial stage as in the latter disease and the atrophy usually begins in the small muscles of the hands. Amyotrophic lateral sclerosis is characterized by increased reflexes, a less degree of atrophy, selection of the arms with merely early weak- ness in the legs and marked bulbar symptoms. In general gliosis sensory symptoms are prominent and there are trophic manifestations in the skin as well as the muscles. Prognosis. — The prognosis is good in pure cases so far as life is THE NON-NEURONIC DISEASES 555 concerned. In regard to the paralysis, the hope of a considerable de- gree of restitution may be held out up to a month or two after the onset of acute symptoms. Some cases may even partially recover after that or until a year or two has rolled around. Complete recovery is so exceptional as to almost warrant the assertion that it is never to be expected. As the outlook for the paralysis in multiple neuritis is peculiarly favorable, the more nearly these cases of poliomyelitis ap- proach a multiple neuritis the more hopeful is their prognosis. Death is not a common result of this disease. Treatment. — The management of these cases is practically the same as it is in the infantile form. ABSCESS OF THE CORD. Abscesses rarely form in the spinal cord as they do in the brain. Only a very few cases have been put upon record, and they followed purulent spinal meningitis of both the cord and brain, or were the re- sult of a metastatic transference of the disease from some near or remote focus of suppuration. Myelitis scarcely ever goes on to the production of an abscess. Putrid bronchitis, gonorrhoea, abscess of the prostate gland and traumatism have seemed to be responsible for it in some cases. Minute collections of pus have been noted in a few- instances of poliomyelitis. The gray matter is usually the seat of the abscess, especially when associated with purulent meningitis. In such cases the brain is very often also the seat of the suppurative process. The majority of the abscesses appear in the upper part of the cord though they may locate anywhere. Paraplegia, anesthesia, sphincter paralysis, girdle pains and all the usual signs of a severe meningoniyeiilis are present. Weakness, tremor and incoordination in the arms have been seen in other case- along with the symptoms in the lower extremities. The usual signs of infection are commonly present, such as the irregular fever, the chills, the foul tongue, the subnormal temperature, the debility and general typhoid state. The diagnosis is easy as a rule but the localization of the .ib- is extremely difficult. As the condition is associated usually with a diffuse, or general septicaemia, surgical intervention would be of little avail. Death usu ally takes place- in a few da; TUMORS I IF Till-: SPINAL C< >RD. By the term tiunor i do not mean all forms of excrescences and protuberances that may compress the o>nl <>r damage it anatomically and functionally within a limited ana. Tin symptoms of these may at times be identical with those of tumor. The ultimate results upon die cord may even Ik- tin- same. Vertebral disease, displacements, caries and exostoses of syphilitic or traumatic origin may affect the cord and its membranes very much as a tumor would. Focal hem orrhage. thrombosis, embolism, may in their after effects exhibil an 55& THE NON-tfEURONIC DISEASES obscure resemblance to a spinal neoplasm. This is because at times all of these troubles may be limited and unique, though obscure in their symptomatology, while obscurity and total absence of symptoms obtain sometimes even in tumor. These conditions must therefore always be carefully excluded in the diagnosis. By tumor of the spinal cord I mean those circumscribed morbid growths that appear in the spinal canal and spring from the membranes or develop within the cord substance itself. The interest in these for a long time was merely pathological and post mortem. Erb suggested, in 1878, that they might be operated upon. It was the successful case of Gowers and Horsley, in 1887, however, that renewed the interest in these conditions and caused them to be studied clinically so thor- oughly that now a fairly definite symptomatology attaches to them. They certainly offer a more tempting field for the surgeon than do the brain tumors. Disheartening as are the total results of operative pro- cedures, and especially of medical treatment in both, the results are better in the tumors of the cord than they are in those of the brain. This is significant in view of the fact that the literature would seem to indicate that the latter are much more common than the former. Etiology. — This is not the place to go extensively into the etiol- ogy of tumors. Outside of certain diathetic conditions, like syphilis and tuberculosis, congenital influences as observed in certain glioma- tous and fatty tumors, parasites like echinococci, and a few obscure traumatic incidents, we know very little about their causation. Wet and cold have been followed by the sudden appearance of their symp- toms but it is probable that these agencies merely provoked the men- ingitic, myelitic and other secondary accompaniments of them. Mul- tiple tumors exhibit a faint degree of heredity. Most all spinal tu- mors appear between the ages of thirty and fifty. Tuberculous tu- mors appear earlier. Men give a history of them more often than women. The etiological incidence that belongs to the tumor per se is not materially altered by its being located in the spinal canal. Pathology and Pathogenesis. — A tumor may arise from the vertebrae, the spinal membranes, or the cord itself. All kinds of neo- plasms occur, though there are differences in regard to the frequency of certain types and their location. The great majority of them are meningeal in origin ; next in frequency arc the intraspinal ; least in frequency are the vertebral. Glioma and sarco)na are the commonest, and next after them fibroma, myxoma, gumma and tubercle. Some of the tumors manifest a choice of loca- tion and it is always well to try and differentiate those that are be- tween the bony column and dura (extra-dural), those between the dura and cord (intra-dural), and those that are within the cord itself (intra-medullary). There is a slight difference in their symptoma- tologies. Extra-dural growths may originate in the membrane, the tissue between the membrane and bone, or outside and grow in through the inter-vertebral foramina. The parasitic tumors, echinococcus and cys- ticercus, occur here. Lipomata usually select this site. The intra-dural tumors are sarcoma, myxoma, psammoma, fibro- THE XOX-XEUROXIC DISEASES 55! ma. syphiloma and tubercle. A sarcoma may be single and extended or multiple. Rarely tubercular and parasitic tumors have been seen within the meshes of the arachnoid. Myolipomata have been found here also. Multiple neuromata sometimes occur on the nerve roots. The more, common intra-medullary growths are the gliomata, syph- ilomata. sarcomata and tubercle. Every variety of neoplasm has de- veloped within the cord. These tumors are single or multiple and spring from the pia mater or the wall of the central canal. FIGURE 119. FIGURE 1 jo. Tumor of the medulla spinalis. • r Braubach. ) Tumor in the dura of the upper part <>f the lumbar enlargement. ( After < iowers The size- of these growths i> necessarily small, ranging from that of a pea to an inch or two in length and an inch in thickness. Some spread longitudinally along the cord, notably the gliomata, which ma) tch the entire spinal length. The sarcomata also diffuse them selves btu not quite so much as the gliomata. Sometimes the tumor ensheaths tin- cord as in a tube. The iifpcr and lower dorsal regions of tin cord are the favorite location of tumor. The morbid growths that attack the Cauda i'<]ui>i^ «>f the kne< and paralysis of the blad- der may be seen. From this description it will be readil) son that no absolutely uniform clinical picture can be drawn to fit all cases alike. < >nlv a t: OUgh knowledge of the anatomy and physiology of the 11 enable the physician t«. appreciat< mosl clearly the pn I a tumor. The symptoms must be studied alwa; her: the entire picture | sented by tin- case must be looked at in toto; and while under ob 560 THE NON-NEURONIC DISEASES tion every change in the symptomatology must be carefully noted, cor- related carefully with what precedes and follows it, and compared with the normal functions of the cord. As the lesion is a changing one, the cause of the symptoms will undergo variations. In regard to the symptomatic diagnosis of the location of the tumor the reader is referred to the chapter in the earlier part of the book devoted to spinal localization. The careful determination of the limits of the anaesthesia affords the best guide, and next to this -the muscles that may be paralyzed. These should then be compared with the table of spinal functions reproduced in an earlier chapter. As a rule the tumor is located from two to four inches above the upper- most border of the area of anaesthesia. Spinal sensitiveness may also help slightly to localize the tumor. The peripheral neuralgic pain, if sufficiently constant and localized in the area of a nerve distribution, may assist by leading back to the corresponding nerve-root. Unfor- tunately, however, pain is far too diffuse a symptom to be of much aid. Diagnosis. — This is much more difficult than in brain tumor, for the reason that there is a wider variability in the symptomatology. Note should be carefully taken of the signs indicating gradual, pro- gressive compression of the cord and nerve-roots. The pain and its special character, the progressive paralysis, the rigidity of the spine, the muscular contractions in the limbs, the early and marked exaggera- tion of the reflexes of the leg, the extension of the symptoms from one side to the other, and the possible existence of metastatic conditions of similar import in other parts of the body, such as syphilis, tubercu- losis, multiple sarcomata and neuromata, are all suggestive of a spinal neoplasm. Caries of the spine is to be differentiated from tumor by the ab- sence, or less severity, of the root signs, by the lesser degree of paraly- sis, by the presence of bone disease with external tumor or kyphosis, by pain upon movement, the age of the patient and the existence of the tubercular diathesis. The symptoms of hypertrophic pachymeningitis are bilateral from the beginning, and represent a much more extensive lesion longitu- dinally in the cord. The sensory symptoms in the arms are more marked and there is more often early and limited anaesthesia. The distinction between this form of meningitis and cervical tumor, how- ever, is at times extremely difficult and for obvious reasons. Menin- gitis is often a part of the symptom-complex of tumor. In transverse myelitis the pains are not so severe and radiating, but there is more of a girdle phenomenon. The trouble does not pro- gress and exhibit, like tumor, the intense initial pains, followed by motor paralysis and anaesthesia and the signs of more or less definite localization. Here again, however, a differentiation is often difficult because myelitis is one of the secondary effects of tumor. Occasion- ally the cause of such an obtrusive myelitis can be discovered to be a tumor, by the manifestations and history of the case, before the appear- ance of the myelitic symptoms. Neuralgia of special nerves ought not to give much trouble in the differential diagnosis for it is distinctly unilateral, limited to the THE NON-NEURONIC DISEASES 561 nerve involved, and is devoid of the motor and spinal symptoms of tumor. Skiagraphy should always be kept in mind as a possible means of diagnosing and locating some of the spinal tumors. Prognosis. — Except in syphilitic cases the prognosis of spinal tumor is bad. The course and duration of the trouble depend very largely upon the character, size, and location of the neoplasm. Most cases run from three to five years, two or three years being the aver- age. Tubercles have been known to cease growing. All other forms of tumor continue, as a rule, to steadily progress. Rapid increase of symptoms and stationary periods often alternate in the course of the disease. Even transient improvement may awaken false hopes. Syphilomata are the only growths that are amenable to medicinal in- fluence, and even these, if long neglected, offer an insurmountable re- sistance. In a few other types of tumor lives have been saved bv surgical intervention. Death is caused by exhaustion, bedsores and all the usual sequelae of a severe meningomyelitis of a high degree. Treatment. — There is only one form of spinal tumor that is directly amenable to medicinal treatment, and that is syphiloma ; and in this form the results are usually in inverse ratio to the length of time the trouble has existed, and the destructive damage already done to the cord. Therefore the earlier the treatment is instituted the bet- ter will be the results. It is a good rule, unless the indications point absolutely away from syphilis, to put every case of spinal tumor upon a trial antisyphilitic treatment. This is especially advisable if there has been exposure and a possibility of infection. The frequently evanes- cent character of the primary and secondary manifestations in cases of tertiary syphilis, may give ground for the denial honestly on the part of the patient that he has the disease. If he admits circumstances by which the disease may have been acquired, a most thorough course of antisyphilitic medication should at once be begun and tried for a while. It cannot do the slightest harm, should the tumor be of any other nature than syphilitic, and it may. in case it is the latter, save the patient's life. Of course when there is a clear history of infection. the treatment should not be delayed a moment. A day or two delay may eventuate in irreparable damage to the cord. The treatment should be both prompt and vigorous. Increasing doses of the Iodide <»f potassium, from sixty grains a day on up. should be started, just as I have recommended in the chapter on syphilis of the nervous sys- tem. If in a few days there is no marked change in the symptoms mercurial enunctions had better be added to the treatment. In some old cases, known to be syphilitic, the treatment just suggested may fail to modify the symptoms. In these cases tin- changes in the cord are beyond all human means of repair. Most of the other forms of tumor suggest the possible interven tion of surgery but their medicinal treatment does not extend beyond the administration of tonics and remedies for the symptoms. \ tuber- culous condition may call for tonics, iodine, oleum morrhuae, hut not much direct effect will he thus exerted upon the spinal tubercle. The same mav be said of the use of arsenic and nitrate of silver in glioma 56: THE NON-NEURONIC DISEASES and sarcoma. The iodides are often despairingly tried in all cases, with the vague idea of causing absorption of the growth. The idea is as poorly founded as the results will undoubtedly be. Surgery should always be considered, not merely as a last resort but as offering in some cases a positively hopeful outlook. While the general results are not brilliant, in individual cases some splendid re- turns have been secured. In capable hands the operation is not now so dangerous as it used to be. Even an exploratory operation is to be commended where so desperate a disease is under consideration and there is doubt, leaning, however, towards the diagnosis of tumor. The extra-dural tumors are of course the most favorable for operation, though even the unfavorable intra-medullary growths may be wisely enucleated if they are single and of recent development. According to Gowers and Horsley, who led the way to the surgical treatment of these tumors with their first brilliant case, almost all intra-dural tu- mors are operable. Multiple, metastatic and advanced medullary tumors are beyond all aid, and therefore should not be subjected to the knife. The diagnosis of the location of the tumor must be very definite before an operation is undertaken. In most cases the tendency is to locate the tumor too low in relation to the guiding symptoms. In Gow- ers and Horsley's first case, the tumor was higher than the symptoms suggested, four inches above the level of the pains and anaesthesia. According to Bruns, generally only the segmental height of the upper border of the tumor can be determined. The highest point of the sensory, motor and painful manifestations should be carefully estab- lished and this compared with the known physiological functions of the various segments of the cord. The chapter on spinal localization and topical anatomy of the cord can be consulted for further details. The Rontgen ray is a modern means of great value in some cases for locating the tumor and should always be resorted to to add if possible further confirmation to what may be nevertheless an almost positive topical diagnosis. In all cases, operable or inoperable, the pains will have to be controlled, bedsores will have to be guarded against and properly treated when present, disease of the bladder will need attention and other symptoms, complications and sequela? will have to be continu- ally looked to. The management of a case of spinal tumor is never a very grateful task. CAVITIES OF THE SPINAL CORD. The formation of cavities within the spinal cord is intimately re- lated to the presence of gliomatous tumors. They are the result of congenital defects. The cavity may appear as a primary defect and be the only abnormality present. This is hydromyelia and is homolo- gous with hydrocephalus. Gliomatous tumors may form within the spinal cord, become absorbed and thus leave a cavity, or gliosis may take place in the walls of a congenital cavity, enlarge into a tumor distending and deforming the original cavity and then undergo partial or complete absorption, thus leaving a cyst but one somewhat differ- THE NON-NEUBON1C DISEASES 563 ent in shape from the original. This is known as syringomyelia. Finally, the remains of old hemorrhages and foci of inflammation may undergo partial resorption and leave small cavities. These may or mav not be congenital. These myelitic and hemorrhagic remains may be traumatic in origin or may accompany hydromyelia and gliosis. For the sake of convenience then, the cavities of the cord may be discussed under the three heads, (a) Hydromyelia, (b) gliosis and syringomyelia and (c) myelitic and hemorrhagic excavations. Hydromyelia is primarily a dilatation of the cord, especially of the central canal. It is lined therefore with epithelium. It is of the nature of a developmental defect and may be associated with hydro- cephalus and spina bifida or exist alone. It is an anatomical curiosity without clinical importance. As a rule it only presents symptoms when gliosis or gliomatosis takes place within its walls and it becomes occu- pied by a growing tumor. Then it becomes clinically interesting. Gliosis and Syringomyelia. — Many believe that this and hydro- myelia are identical conditions primarily. The hydromeylia of child- hood evolves in the course of time and from various factors into the syringomyelia of adults. As syringomyelia develops behind the cen- tral canal, and sometimes around it, it is not always a mere modified hydromyelia. It is a posterior spinal defect of development. In most cases it is a persistent patulency of the anterior portion of the posterior limb of the primitive developmental sulcus. Etiology. — Typical syringomyelia, as I have intimated, is a con- genital trouble associated with or following a gliomatous neoplasm. Dana says it is more frequent than amyotrophic lateral sclerosis but less frequent than multiple sclerosis. It is far more common in men than in women. In Schlesinger's 190 cases, 133 were men, 57 women. Most of the cases begin prior to the thirtieth year ; usually about the age of twenty or twenty-five. It is commonly affirmed that heredity does not constitute an etiological factor in this disease, and yet three members in the same family have fallen victims to it. In the non- gliomatous, traumatic syringomyelias there is believed to exist an in- herited or congenital predisposition on account of which the trauma operates in the way that it does. The disease cannot be connected with alcoholism, syphilis or dissipation. It has followed pregnancy and certain infectious diseases. Dana observes that it seems to occur to a large extent among those who follow manual occupations, such as butchers, tailors, etc. I observed it in a man who after recovery from influenza went to work in the fields and daily plunged into cold water while he was hot and perspiring. Oppenheim says that spinal cavities have often been observed in syphilitic individuals and accompanying Other disease of the cord. These cases are not typical, however, as are the non-syphilitic gliomatous cases. Pathology and Pathogenesis. — Upon taking tin- cord out of the spinal canal nothing abnormal may appear about it. Tt may seem a little larger in some places, softer than normal, and fluctuating. The essential finding of the disease is only observed when the cord is cut across transversely. At once a carcmous formation is seen in the gray matter. Tt usually occupies the center of the cord, obliterating and 564 THE NON-NEURONIC DISEASES FIGURE 122. FIGURE 123. FIGURE 124. Figure 122, Hydromyclia of upper dorsal cord. Figures 123 and 124, Syringo- myelia. Sections made in upper and middle cervical cord. (Striimpell — Jakob.) THE NON-NEURONIC DISEASES 565 including the central canal. It may extend in the lateral gray mat- ter or into the horns, especially the posterior horns. In the latter it has been seen to reach almost to the edge of the cord. The white matter is not touched by it. The shape of the cavity is irregular and various. It may be angular, round, oval, stellate, diamond or any imaginable shape. It is most frequently seen in the cervical part of the cord, though it may be in any part, or even extend throughout its entire length. The bore of the cavity when thus extended is never perfectly uniform. There are apt to be here and there wide lacuna with narrow connecting channels. Lining the cavity is a well-formed membrane and around it a layer of gliomatous tissue. In some places this gliomatous tissue is proliferated and extends into the lumen of the cavity. Occasionally it may be seen to occupy the whole cavity and even be distending it. The primitive ciliated epithelium of the original central canal may be noted here and there on the cavity wall. The cells of the gliomatous tumor are seen to be in various stages of formation and decay. Hemorrhagic and inflammatory foci may be observed in certain cases. In some cases other developmental defects have been observed outside of the cord. Thus the brain has revealed an internal hydrocephalus and the cerebellum has been absent. Tumors, probably gliomatous, have also been seen in the pons, the cauda equina and elsewhere, showing that the gliomatous growths of the cord proper constitute but a part of a widespread pathological tendency. The original cause of these cavities is a congenital, developmental defect in the posterior part of the spinal cord. There is a defective closure of the central canal and posterior septum, with the remains of epiblastic tissue in the walls of the imperfect closure. Just to what extent the entire syringomyelic formation is congenital in its origin is not easy to determine ; nor can it be stated positively just how much it has been enlarged and altered by the over-growth and sub- sequent degeneration of the gliomatous tissue. The process of cavity formation is thus a complicated one, always primarily congenital or hydromyelic, but secondarily enlarged and distorted by the intra- cavernous growth and degeneration of a neuroglia tumor. The primitive furrow in the embryo deepens, its sides rise up, arch inward, coalesce, and thus transform it into a tube. At about the sixth week this central canal is comparatively very large, is dia- mond shaped and reaches from the anterior to the posterior surface of the cord, its walls are thinner in front and behind than they are at the sides. As the posterior columns develop, the posterior part of this canal becomes narrow and elongated, almost to the point of being a mere fissure. The surrounding structure consists of embryonal cells which afterward are transformed into the proper nerve elements. Finally the canal becomes divided into an anterior and a posterior por- tion. The former ultimately constitutes the permanent central canal. the sides of the posterior coalesce and form the posterior septum. When either of these divisions of the primitive central canal fail to close properly, there is a surplus of the embryonal epiblastic tissue present which is very liable later on to undergo hyperplasia and tumor formation. The cells are small, round or irregular in shape, with large 566 THE NON-NEURONIC DISEASES nuclei and numerous fine fibrillary prolongations. Bloodvessels pene- trate these gliomatous growths and being diseased rupture and leave hemorrhagic debris. The tumors themselves are very prone to degen- eration and disintegration, transforming an originally limited cavity into a much more extensive one. The disintegration and disappearance of the gliomatous tumor are due, in a measure, to the liquefactive de- generation of the glia cells. The oedema of the cord may be partly re- sponsible for and partly the result of this. Symptoms. — Some cases of syringomyelia do not seem to produce any symptoms and they are only discovered upon post mortem examina- tion. It is probable they are cases of hydromyelia in which no gliosis has taken place. The latter process in the majority of cases probably begins spontaneously, though in some cases it would not occur at all if there had not been some exciting initiative cause, like trauma, hem- orrhage, meningitis, etc. The symptoms of syringomyelia are the result of the 'pressure and destruction of gray matter caused by the tumor. This explains why the infantile form of the trouble is without clinical manifestation generally, whereas the adult form almost always exhibits outward signs. The clinical picture of the disease is subject to wide variations. This would naturally be expected from so variable and extensive a lesion. The establishment of types of the disease, such as Schlesinger and others have made, seems to me to be arbitrary, burdensome and dangerously suggestive of the types being different diseases dependent upon different lesions. The only anatomical basis for the separation of these types is the accidental location of the pressure and destructive influences within the cord. Hence while the clinical features are in a way complex, it is not due to the complexity of the disease but to the complexity of the physiological functions of the cord and their dis- turbance by the particular location of the disease process. As the cavity usually appears first, and is most extensive in the cervical part of the cord the symptoms appear most prominently in the upper part of the chest, the lower part of the neck, in the shoulders, arms, and hands. Perhaps the most striking symptom, due doubtless to the destruc- tion of the gray matter of the central cord, is the progressive muscu- lar atrophy. It usually begins in the hands, sometimes in the shoul- ders and upper arms. Sometimes it develops in the legs. It is a true atrophy and is liable to be mistaken for the ordinary form of spinal progressive muscular atrophy. The smaller muscles of the hand waste first and the main-en- griff e, or claw-hand, develops. The muscles on the back of the scapulae, the deltoid, the biceps and triceps, diminish in size. The wasting is usually bilateral and is accompanied by a fibrillary tremor. There is a partial reaction of degeneration and dis- turbance of the reflexes. Weakness and paresis proceed, pari passu. with muscular wasting. In the legs', which are usually affected later, there is atrophy with more or less spastic paraplegia; in other words a condition resembling amyotrophic lateral sclerosis, and probably pro- duced by the same sort of a disease process. The face and throat mus- cles are not often affected and the sphincters remain intact. On ac- THE NON-NEUBONIC DISEASES 56? count of the unequal wasting of the muscles of the back spinal curva- tures occur, especially scoliosis. The drooping of the scapula and the lessening of the shoulder joint cause unilateral deformities often about the shoulder. The sensory disturbances are unique and almost pathognomonic. There is a dissociation between the tactile and pain senses, a simple analgesia without anccsthesia or hyperesthesia. The temperature sense, like the pain sense, is also lost. A hot iron and a piece of ice placed against the skin awaken only the sense of contact. With the iron hot enough to burn the skin, it still does not cause any distress. This explains the many scars and marks of traumatism so often carried by the patients about on their bodies. The exact anatomical basis for this remarkable symptom is not fully known. It is thought to be due to the destruction of the gray matter of the posterior commissure, through which course the fibres of Gowers' tract that carry the pain and heat impulses. In some instances the conducting functions of the cord for sensory impulses of all sorts are so profoundly damaged that wide areas of complete anaesthesia are observed. The Brown- Sequard type of hemianaesthesia is accordingly noted. Paresthesia is not infre- quent and in rare instances actual pain may be present. The special senses do not participate in the trouble. This dissociation of the tactile and pain sense, with the progres- sive muscular atrophy above and the amyotrophic sclerotic manifesta- tions below, constitute the pathognomonic picture of the disease. Other symptoms of great variety may also be looked for. Among these the vasomotor and trophic signs stand prominently forth. They must be distinguished of course from the mere injuries which these patients so often cause themselves on account of the absence of the pain and heat senses. CEdema, redness, sweating, slow-healing sores appear on the hands and arms. Eczema, bullae, herpetiform eruptions break out on the skin of the body. The latter is sometimes dry and leathery and unhealthy looking. Whitlows occur on the fingers, and the nails become brittle and fall out. In about ten per cent, of the cases arthropathies occur. The bones become fragile and necrosed, and ankyloses take place in the joints. Spontaneous fractures are not to be forgotten as unfortunate possibilities. The mutilations, scars, deformities often render these poor creatures a sorry sight. Inequality of the pupils, and iater on distinct bulbar symptoms with implication also of the rectal, vesical and sexual functions show the gradual and progressive extension of the lesion. Many changes may be rung upon the above more or less typical clinical picture, and some novel combinations in the symptomatology make their appearance. These are not types of the disease, but mere atypical presentations of the symptoms. Morvan, for instance, de- scribed what he supposed was a new disease, but which is now acknowl- edged to be hut a particular presentation Of syringomyelia. In the eases that Morvan described, analgesia, thermo-anaesthesia, tactile anaesthesia, muscular atrophy and felons on the fingers all occurred. A rare group of symptoms thai have been separately seen in differ- ent cases are facial hemiatrophy, anaesthesia of the trigeminal region, 5 68 THE XOX-XEURONIC DISEASES early bulbar phenomena, cranial nerve palsy, nystagmus and mellituria. A sharp limitation of the symptoms may be due to the confinement of the gliosis to the posterior or anterior horns of one side. In three cases Oppenheim observed the disease to be restricted to the lower limbs in the beginning. I saw a case in which in the beginning there was pain in the left hip, passing down the leg and followed by a very slight degree of atrophy, oedema and changes in the joints. Later on the pain ceased in the leg and marked atrophy appeared in both shoulders, in the back muscles and muscles of the scapulae, and part way down the muscles of the arm. Arthropathies were marked in the knee of the left side and shoulder joints. The other symptoms, trophic and sensory, were not unusual. Optic neuritis and choked disc have been seen, and some still maintain that in part, at least, the symptom-complex of Morvaris dis- ease is due to a complicating peripheral neuritis. If there is involvement especially of the posterior tracts, the disease may asume the complexion of tabes dorsalis. Diagnosis. — Progressive muscular atrophy, dissociation of the sensory symptoms, general vasomotor and trophic symptoms, the amyo- trophic lateral sclerosis symptomatology in the lower limbs and spinal curvature constitute the decisive indications of syringomyelia. The numerous atypical combinations and presentations of its symptoms may easily delude one into mistaking it for some other disease. This was much more common in the past than it is to-day, and we now recognize that there were named and described niany separate affections that were really instances of syringomyelia. Both ordinary progressive muscular atrophy and amyotrophic lat- eral sclerosis are very liable to be mistaken for it. The former is de- void of sensory symptoms entirely, and the latter is not characterized by flaccid atrophic paralysis with preservation or diminution of the knee-jerks. The sensory phenomenon when present will indicate con- clusively syringomyelia. Hypertrophic cervical pachymeningitis causes muscular wasting, sensory loss and other resemblances to syringomyelia. It may be dif- ferentiated, however, by the local spinal tenderness, the spinal root symptoms, the lesser extent of the anaesthesia in relation to the wasting and the absence of the characteristic indications of the cord disease. Chronic transverse myelitis should give no very great trouble in the diagnosis. Caries of the spine, with atrophy, sensory disturbances and pupillary changes, is nevertheless separated by the history of tubercu- losis and disease of the vertebrae. Gliosis and glioma of the cord are two separate conditions, accord- ing to Hoffmann. The former is a rapid, the latter a slow process. They are not necessarily syringomyelic. Various peripheral nerve diseases may be confused with syringo- myelia. Morvan's disease does not call for a differential diagnosis, as it is syringomyelia with possibly a complicating neuritis. It is the form of syringomyelia that is most likely to be confused with aincsthctic lep- rosy. The detection in the skin of the lepra bacillus is, of course, final. THE XOX-XEUROXIC DISEASES 5O9 In leprosy the cutaneous evidences are in plaques, including the face and lower limbs as much as the arms. There is no dissociation of the sensory symptoms as in syringomyelia, but the sensory phenomena fol- low the innervation particularly of the peripheral nerves. In leprosy the amyotrophic paraplegia of the lower extremities, the bulbar symp- toms, the nystagmus sometimes seen in syringomyelia are wanting. Neuritis of the brachial plexus has all the earmarks of peripheral neuritis and very few of those of the spinal cord cavity. Hematomyelia has its own clinical manifestation, though it may later on develop into a syringomyelia. Hysteria, Raynaud's disease, acroparesthesia are easily distin- guishable from the affection under consideration if emphasis is laid upon the characteristic symptoms of each respectively. Prognosis. — In regard to recovery this is bad. The disease is a progressive and congenital one. Periods of temporary cessation and even remissions occur. It ma}^ continue to advance for many years. It is doubtful if the case with lues, reported as cured under the use of potassium iodide, was typical. Death occurs from exhaustion, blood- poisoning and bedsores. Treatment. — This is of no avail and for reasons that are quite obvious. Warn the patient against injuring himself; maintain the nutrition of the muscles as far as possible by massage and electricity; administer general tonics ; and try, if you are in a hopeful state of mind, arsenic or silver nitrate. Lumbar puncture entertains the sur- geons without in the least helping the patient, and the finding of some drug or serum that will check the gliomatosis is a dream the realiza- tion of which is more to be hoped for than actually anticipated. Myelitic and Hemorrhagic Cavities. — The names of these suffi- ciently indicate their nature. They may occur alone or with typical syringomyelia. They are not large, are apt to be multiple and are gen- erally lined with connective tissue. SPINA BIFIDA AND OTHER CONGENITAL MALFORMA- TIONS OF THE CORD AND ITS MEMBRANES. Spina Bifida or rachischisis is an embryonal defect depending upon a failure of the spinal canal to close. It belongs to the same class of troubles as hare-lip, cleft palate and exstrophy of the bladder. The bony canal being open behind, the contents protrude under the skin and pro- duce a tumor, varying j n size from a nut to a child's head. The lum- bar region is its most frequent site. In some cases only the membranes, filled with cerebrospinal fluid, protrude. This is a meningocele. Some- times the cord protrudes with the membranes, giving rise to a myelocele or meningomyelocele. When the cord is syringomyelic, as it occasion- ally is, the cavities inside and outside of the cord form one and thus is established a syringomyelocele. The simple meningoceles and meningo- myeloceles are the most common and are sometimes called hydrorrhachis externa. When the cord and nerves are in the sac. they lie on the posterior, median surface. The lining of the sac is, of course, the dura and the subdural arachnoid. Outside of it the skin is usually normal. 57o THE NON-NEURONIC DISEASES thin and covered somewhat with hair. The tumor itself is elastic and fluctuating. Pressure upon it has caused unconsciousness and other cranial disturbances. Other developmental anomalies and signs of degeneracy are often seen in these patients. They are usually mentally defective and are feeble, badly nourished and badly developed individuals. In simple meningocele, the cord being intact, there are often no special neurological symptoms. In the myeloceles, however, there is usually complete paraplegia, with muscular atrophy, more or less an- aesthesia, bladder and rectal trouble, diminution of the knee-jerks and the paralytic type of talipes, especially pes varus. Very few cases live to old age. The prognosis is always grave, with or without treatment. The treatment is entirely surgical. Unless hydrocephalus is pres- ent, extirpation of the sac may be attempted. The results are not very encouraging. Morton's fluid, consisting of iodine gr. x, potassium iodide gr. xxx, glycerin §j, has been injected into the cyst in oj quantities. The injection should be made at the side of the sac and the child be kept still. There is nothing to be gained by simple puncture and with- drawal of the fluid. It is a dangerous procedure. Only several months after birth should surgical intervention be thought of. MALFORMATIONS. There are certain malformations of the spinal cord that are only of anatomical and pathological interest. Such is amyelia or absence of the cord. It is morphologically and biologically interesting that when- ever the cord is thus wanting the brain also is wanting, but that oc- casionally the brain may be absent without the absence of the cord. In these cases of amyelia the spinal nerves are present and their inner roots swing loosely in the spinal cavity. These monsters, who, of course, never live, demonstrate most clearly that the nerves are not intrinsically developments of, or parts of, the cord, and that the brain is practically a hyper-development of certain anterior segments whose homologues are the segments of the so-called spinal cord. An atelo- myclia is the failure of development of a certain segment. When the cord is bifurcated, the condition is spoken of as diastcmatomydia. Rarely is the whole cord involved in this condition. Sometimes the cord is asymmetrical. A double cord, with two central canals, lying side by side, is known as a diplomyelia. None of these are of any clinical interest, but as indications of development and biological evo- lution of this part of the nervous system they are of entrancing interest. Micromyelia is an abnormally small cord. Van Giesen's heterotopia is an abnormally situated mass of gray matter. In most cases it is a post-mortem artefact. CAISSON DISEASE. This is sometimes called diver's paralysis and compressed air dis- ease. It is probably an affection of the entire nervous system or cerebro- spinal axis, though it is usually discussed as a spinal cord trouble be- cause its symptoms are largely paraplegic in character, and what few THE NON-NEURONIC DISEASES 5/1 lesions have been found after death were seen in the cord. As diver's palsy the disease has doubtless long been known. In these modem davs of industrial activity and great engineering projects, with their mining, bridge-building and harbor construction, in which caissoiis filled with compressed air under water are made use of, the trouble has become more frequent and awakened a corresponding interest. Much study has been made of it. both clinically and experimentally, so that the literature is of no mean proportions. Etiology. — The essential cause of the disease is the high atmos- pheric pressure which the victims must perforce undergo in their occu- pation. It is therefore an acute disease with one specific cause, and all such factors as age. sex. race, etc., are of no significance, except so far as they are determined by the occupation. Those who work in caissons, bells and diver's apparatuses are usually subjected to a com- pression of from one to four atmospheres, which means from fifteen to sixty pounds to the square inch. When they emerge from the apparatus and the compression is withdrawn the symptoms of the disease make their first appearance. The differences in individual susceptibility may explain why, under the same conditions, some are affected while others escape. Every indication points to the mechanical pressure, rather than to any chemical or other change in the air, as the primary and essential cause of the disease. Pathology and Pathogenesis. — Xot many post-mortem exam- inations after diver's palsy have been made and our knowledge of its pathological anatomy, if it has any, is meagre. Most of the changes have been found in the thoracic part of the cord. They have consisted of numerous minute foci of hemorrhage and small patches of necro- biosis. Sometimes signs of an acute myelitis, disseminated myelitis, were noted. There were also slight fissures and ruptures. Changes in the brain have not been reported, though in a few cases the symp- tomatology was clearly that of cerebral apoplexy. On account of the scarcity of these post-mortem evidences, all ex- planations of the disease are to be taken somewhat tentatively. Perhaps the grossest explanation is that the increased pressure upon the surface of the body drives the blood inward and that whereas the other viscera are able to accommodate themselves to the congestion thus caused, the brain and spinal cord being encased in a firm, bony b ix, are not able to accommodate themselves quickly enough to the new state of affairs. A. II. Smith is the leading exponent of this view. There are many objections to it. however. The first is that the symp- toms appear not when the C '.need, but when it is re- lieved : not when the man is under the increased pressure, but when he comes out from it. It is a little contradictor} to declare that con- tion and minute hemorrhages, presumably produced while the man is being subjected to the exalted pressure, should produce no symptoms until he emerges from the caisson and the congestion and other sup- posed circulatory disturbances are relieved. Symptoms and k - must be synchronous or we must adopt the idea that the evi- dences of congestion are only manifested when the congestion is undergoing reduction. The illogicality of this, it seems to me. is an LI II 572 THE NON-NEURONIC DISEASES argument against the congestion theory. The theory, moreover, upsets the long-taught physiological fact that on account of their rich anas- tomotic blood supply, both the brain and cord, above all the viscera, are protected from sudden and external circulatory disturbances of a purely mechanical nature. And finally, it does not harmonize with the localization of the chief center of the trouble in the dorsal region of the cord. Gowers suggests that the trouble is primarily due to the revulsive anaemia following the congestion, and that a nutritional dis- turbance, especially in that part of the cord which is so liable to nutri- tional disturbances, the dorsal region, is the immediate source of the paraplegia. Snell's hypothesis that it is due to the bad ventilation of the caisson, and the escape of carbon dioxide and oxygen into the blood-vessels and into the tissues, needs yet to be confirmed. The explanation that at present seems to have the strongest ex- perimental and pathological support is one in which it is affirmed that there is a sudden effervescence of gas in the blood and tissue juices. In this way the peculiar character and course of the symptoms, and the few pathological findings that have been noted, such as the spinal fissures and lacerations, the minute hemorrhages and spots of necrobio- sis from air emboli, are thought to be caused. The inflammatory changes are secondary results. Paul Bert was the first to suggest this theory, though Hoppe, Francois, Rameau and Baequay had previously surmised it. After recently experimenting upon animals, Hill and Macleod believe they have confirmed Bert's conclusions. In their sum- ming up, these investigators say that the circulation is not affected mechanically by compressed air. The cause of the disease is the escape of gas bubbles in the blood-vessels and tissue fluids upon the withdrawal of the compression. All of the fluids of the body generally effervesce somewhat after the manner of an opened bottle of soda water. The symptoms vary because the seats of the air emboli vary. Symptoms. — After a pressure of three or more atmospheres, the symptoms appear in those affected usually after the change is made from the high to the low pressure, especially if the change is made quickly. The individual must have been subjected to the pressure for at least an hour, and it usually is noted that the first signs of the dis- ease show themselves from, half an hour to an hour after he returns to the lower pressure. He may not have reached the normal atmosphere even before their appearance. There is first sadden pain in the limbs and joints, quickly followed by powerlessness and complete paraplegia. Not often are the arms involved. The pain, sharp and neuralgic in character, may appear in the epigastrium. These pains are often paroxysmal and are accom- panied by nausea and vomiting. Sometimes the legs are tender to the touch and the gait, when not completely paraplegic, is stiff anil awk- ward. Cephalalgia and dizziness are frequent. In the severest cases there is complete paralysis of both motion and sensation, involving legs, arms and trunk. In a few rare instances there have been seen hemi- plegia, monoplegia and other symptoms suggestive of cerebral apoplexy. Rapid coma and death have taken place in these apoplectic cases. THE XOX-XEUROXIC DISEASES 573 The sphincters are usually involved, and there may be retention or involuntary emission of urine, constipation and erections of the penis. Clonic contractions upon movement have occurred in the abdominal muscles. In the paraplegic cases that do not get well bedsores, cystitis and all the later signs of a transverse myelitis appear. Progxosis. — Mild cases usually get well ; severe cases usually die. The former may run a course of only a few hours, or one of many weeks and months. In the mildest cases the recovery is generally com- plete. In other cases the paraplegia becomes permanent. Most of these cases run a course and terminate as any case of transverse myelitis would. Cases with cerebral symptoms are usually fatal. Treatment. — Prophylaxis is obviously most important. A care- ful selection should be made as to the general age and physique of the men employed in caisson work. Only strong, young men should be accepted and they should accustom themselves to the conditions before fully entering upon their work. It is imperative that they be total abstainers and temperate in all their habits. It is well to test the men with low pressures first before engaging them. Hill and Macleod sug- gest that the choice men for the work are those who are small, spare and wiry, and from twenty to twenty-five years of age. Emergence from the atmosphere should be gradual and always slower than en- trance. The longer the shift the greater will be the saturation of the body fluids with gas and the slower should be therefore the decom- pressing process. The higher the pressure is, the shorter should be the shift and the slower and more gradual the emergence. Both the caisson and the decompression chamber should be thoroughly ventilated and maintained at a temperature of about 60 degrees F. After decompres- sion the men should remain quiet for an hour or so and be recompressed upon the slightest sign of sickness. By observing these precautions Hill and Macleod believe that work of this character can be carried on without loss of life at a depth even of two hundred feet or about seven atmospheres or +100 pounds' pressure. When the disease has appeared its treatment is entirely symp- tomatic. The intense pains will demand the use of morphine. A. H. Smith suggests the use of ergot. Oxygen inhalations and the use of compressed air have been recommended. The treatment in other re- spects differs in nowise from that of myelitis. DISEASES OF THE CAUDA EQUINA AND FILUM TER- MINALS. Some authors devote a special section to these diseases, because of the multiplicity of the nerve roots here closely grouped together. It seems to me that this is unnecessary, as any disease that attacks the cord above is liable to attack the cauda and iilum. Hemorrhages, syphilitic and other neoplasms, inflammations and traumatisms are among the lesions found in this lowermost part of the spinal canal. In the absence of cord segments, the symptomatology will correspond with that of spinal root disease. The extent and character of the lesion 574 THE NON-NEURONIC DISEASES will determine the extent and character of the symptoms. All of this is to be determined by a rigid comparison of the symptoms with what we know of spinal localization. In a word, the diagnosis of diseases in this part of the cord is a localization diagnosis, plus the special feature that the symptoms will be of the root variety, but of very wide extent because involving so many roots. Bilaterality is a strong characteristic FIGURE 125. Semidiagrammatic illustration to show the topographical relationship between the vertebrae, the canda and the conus. (After Raymond.) of the symptomatology, though not absolutely so. There are small,, focal lesions here as elsewhere. The accompanying diagrams from Raymond and Schultze will assist better than any long description in making a localization diag- nosis. The prognosis and results of operative treatment in this part of the spine are rather encouraging. Spinal Concussion is a term used by some writers for a class of spinal symptoms caused by a fall, a blow or a general concussion, such THE XOX-XEUROXIC DISEASES 575 as occurs in railway accidents, and supposed to be without any ana- tomical foundation. In my opinion, the statement "without any ana- tomical foundation" used by these authors in this connection is a logical self-contradiction and at once wipes out spinal concussion as a special and unique disease. The term is misleading and had better be dropped. FIGURE 126. LTvI A schematic representation of the vertebral column with the lower part of the cord and the ^cauda. A lesion at A and one at B will produce pretty nearly the same set of symptoms. (After Schultze.) What is meant by it is a traumatic neurosis, for a consideration of which the reader is referred to another chapter. Under it the cases that are not of the nature of traumatic neurosis are hemorrhages and other well-known organic troubles that need no further discussion than what I have alreadv devoted to them. 576 THE NON-NEURONIC DISEASES PART Non- Neuronic Diseases of the Peripheral Nerves. Anatomy and Physiology of the Nerves. — We have already seen in our studies of the neurone that the peripheral nerves are nothing but the processes of cells, collected together into bundles and supported and protected by certain non-neuronic elements. Therefore literally there are no such things as peripheral nerves in the sense of being a special structure. They are mere outgrowths, extensions, branches, arms, as it were, from the central cells. We must get rid entirely of the idea that the peripheral nervous system is, as it used to be taught in the older anatomy, a network of conducting fibres, or wires, so to speak, to con- duct impulses from one point to another. It is more than this. It is the outlying part of the central nervous system itself, its own branches and integral structure. This newer conception of the nature of the peripheral nerves is absolutely necessary to grasp, if one is to under- stand their diseases. Many of the changes in the peripheral nerves, as in many of the degenerative forms of peripheral neuritis, so-called, are but the expression of trouble in the cells of the anterior horns of the cord and of the posterior spinal ganglia. A nerve therefore should always be regarded as a mere part of the central apparatus and not in the least as an independent structure. As the nerves are intimately related to, or are parts of, the spinal cord elements, if we are to divide up the great, uniform and harmonious nervous apparatus into such artificial divisions as the brain, spinal cord and nerves, it will be appro- priate to discuss the diseases of the nerves immediately after the dis- cussion of the diseases of the spinal cord. Anatomy speaks of motor and sensory nerves. W T e have already noted that this is somewhat of an artificial distinction so far as the con- stituent neurones are concerned ; for the motility and sensation depend not upon the neurones per se, but upon the end-organs with which they are attached. The motor nerves are but the neuraxones of the neurones whose bodies are located in the anterior horns of the cord and motor cranial nuclei. They are motor because they stimulate muscles. The sensory nerves are merely the peripheral ends of the neurones whose bodies lie in the posterior spinal root-ganglia, or in the ganglia (petrosal, jugular, geniculate, Gasserian, etc.) found on the roots of the sensory cranial nerves, and whose central ends project into the cord and medulla. They carry impulses from the peripheral parts of the body to the central ganglia and are thus sensory in function. When these motor and sensory peripheral processes are bound together by con- nective tissue, in which course the minute nutrient blood-vessels and lymphatics, we have what is termed in gross anatomy a nerve. A transverse section of a nerve then presents an easily understood and typical picture. It is a bundle of smaller bundles, which smaller THE XOX-XEUROXIC DISEASES 577 bundles are made up of the central cell processes. The smaller bundles, called funiculi, are embedded in the perineurium which forms itself into concentric rings or laminae immediately around each funiculus, but whose fibres run in all directions between them. Around the outer edge of the nerve, where it is named the epineurium, it again assumes the ring-like arrangement. The perineurium is a connective tissue structure and contains the arteries, veins and lymphatics. It is therefore that part of the nerve that is capable of undergoing inflammation, hyperplastic new growths and other changes common to the vascular and connective-tissue ele- ments. The perineurium immediately around each funiculus sends septa, endoneurium. in various directions into it, which separate and FIGURE 127. — Transverse section of the sciatic nerve of a cat about x 100.— It consists of bundles {funiculi) of nerve-fibres ensheathed in a fibrous supporting capsule, epi- neurium, A , each bundle has a special sheath (not sufficiently marked out from the epmeunum in the figure) or perineurium B ; the nerve-fibres N/are separated from one another by endoneurium ; L, lymph spaces ; Ar, artery ; V, vein ; F, fat. Somewhat diagrammatic. (V. D. Harris.) support the nerve-fibrils, whose cut ends we can recognize in the little white circles, with a dot in the middle of each circle. Blood-vessels do not seem to penetrate the funiculi, but the endoneurium as well as the perineurium contains lymphatic tracts by which the fibrils are nour- ished. When a nerve sends off branches to unite with other nerves it is a funiculus that leaves the main trunk. The funiculi do not anasto- mose with each other in the same nerve trunk, nor do the branching fibrils emerge from the funiculi until they have reached their destina- tion. In its last analysis, a nerve fibril is seen to consist of an axis cylin- der surrounded by the medullary white substance of Schwann and the neurilemma. The axis cylinder consists of numerous minute strands running along side by side and representing the extension of the intra- cellular fibrous elements of the neurone. The medullary substance is seen to be absent or almost absent at various regular intervals along the course of the fibrils, so that the neurilemma is constricted and sinks 578 THE XOX-XEL T ROXIC DISEASES down close to the axis-cylinder. These constrictions in the fibril are known as the nodes of Ranvier. It is supposed that the nutrient sub- FIGURE 129. 1 "A FIGURE 128. — A node of Ran- vier in a medullated nerve - fibre, viewed from above. The me- dullary sheath is inter- rupted, and the primi- tive sheath thickened. Copied from Axel Key and Retzius. x 750. (Kleinfc Noble Smith ) -Nerve- fibre stained •with osmic acid. A, node; B, nucleus. (Key and Ret- riiw.) stance enters the fibril here. At all events it is always at the nodes that the branches are given off by the fibril. In the white medullary sub- THE X0X-XLUR0X1C DISEASES 579 stance are to be seen numerous minute oblique lines. These are called. Lantermann's incisures. They probably represent breaks caused by the handling of the nerve. About midway in each inter-nodal space, and just beneath the neurilemma, is a nucleus. Non-medullated fibrils are .found in the olfactory and sympathetic nerves. The neurilemma is not found on the neuraxones making up the white substance of the brain and cord. There is nothing to be said of the physiology of the nerves beyond what has been said of the physiology of the neurones. General Pathology of the Peripheral Nerves. — It is clear from their anatomical structure that the nerves, like all parts of the nervous FIGURE 130. FIGURE 131. —Two nerve-fibres of sciatic nerve, a. Node of Ranvier. b. Axis-cylinder, c. Sheath of Schwann, with nuclei. Medullary sheath is not stained, x 300. (Klein and Noble Smith ) -Axis cylinder, high- ly magnified, showing its component fibrils. (M. Schultee.) system, may undergo both the degenerative and the inflammatory process. The former used to be called parenchymatous inflammation. In our previous study of the degeneration and regeneration of the neurone we noted that it was not an inflammatory process at all. The latter is still correctly referred to as an interstitial inflammation, for it is a true inflammatory process and involves the interstitial connective tissue and its vascular contents. When this interstitial tissue becomes. overgrown the nerve fibrillar are compressed and more or less degener- ated ; the nerve becomes hard ; and tin- whole condition takes the name <>f sclerosis. The degeneration thus caused in the true nerve-fibrils is a secondary phenomenon. It is still an open question whether a primary degeneration ever takes place in these peripheral nerve fibrils that is not dependent upon trouble in the cell-bodies and their nutrition. In certain toxic states 5 8o THE NON-NEURONIC DISEASES we often find the peripheral nerve fibrils wasted, but no discoverable signs of inflammation to enable us to say the degeneration was sec- ondary, nor discoverable changes of a positive character in the related cell-bodies. The degeneration under such circumstances appears to be purely primary. Some investigators hold that the toxin has directly damaged the peripheral nerve-endings. They are few, however. Oth- ers maintain that it has affected the nutritive function of the central cells and so caused the degeneration in these remote parts of the neu- rones. When the damage to the central cells is obvious and mechanical, trie resulting deterioration in the peripheral nerve filaments is not a secondary degeneration. It is but a part of the degenerative process in the whole neurone. In primary degeneration I assume then that the entire neurone is affected ; that through hereditary or other influences the cell-body has lost its power of keeping up the nutrition of the processes, and as a result the latter waste. The peripheral degenerations observed in many hereditary diseases and in many toxic and infectious states are un- doubtedly of this character. Certain cachectic states in which there is especially disturbed nutrition, thus exhibit peripheral nerve degenera- tion. Diabetes is an example. Defective metabolism associated with lithaemic, anaemic and autointoxic conditions may be accompanied by the parenchymatous wasting in the nerves. In all of these conditions it seems to me very essential that we should regard the peripheral degeneration as a mere expression of a disturbance in the nutritive function of the central cell-bodies, and that this disturbance is the result of a much larger and more generalize;! disease process. In this sense primary degenerative processes, pure neuronic disease, in the peripheral nerves, is never any thing more than a collateral exhibition in another and more distinct disease. I empha- size this fact because upon it hang the prognosis and treatment of these peripheral degenerative manifestations that seem to be primary. I emphasize it also because it will serve to stimulate the practitioner to always look further than the nerves, when these are primarily de- generated, for a more profound and widespread disease involving the entire organism. The majority of the cases of toxic polyneuritis serve as apt illustrations of my point. The secondary degenerations, similar to the Wallerian degenera- tion that sometimes occurs in the peripheral nerves, result from trau- matic causes that compress, lacerate or sever the neurones, so that parts of them are separated from their nutritive cell-bodies. In this condition the distal or severed end of the neurone undergoes complete disappearance; the proximal end passes through a much slower, in- complete and at times scarcely noticeable retroactive degenerative process. It is presumed that here the whole neurone has been put out of service by the injury and that even the cellular end misses the normal stimulus ncessary to keep it up to its normal nutritive tone. As in all of these traumatic or secondary degenerations, the inflammatory phe- nomenon sooner or later makes its presence known, I will refer to them under the general head of neuritis. THE NON-NEURONIC DISEASES 58 1 NEURITIS. An inflammation in a nerve begins, of course, in the interstitial or non-neuronic tissues. The nerve fibrillar are damaged secondarily. It may well be that the cause, compression for instance, may be just sufficient to initiate the degenerative process in the fibrillae before the inflammatory process in the interstitial tissue has gotten well under way. Under such circumstances the degeneration would be regarded as secondary, but not due to an associated inflammatory process. The distinction is not an important one, however, since it merely means that degeneration is quicker to occur than inflammation in the presence of the same active cause. As sooner or later in all such cases inflamma- tion does appear, it is convenient and not illogical to consider the two processes, inflammation and secondary degeneration, together. When the inflammatory process originates in and remains con- fined to the perineurium, the disease is called perineuritis. If the in- terstitial tissue is the seat of the trouble, it is known as interstitial neuritis. The parenchymatous neuritis of the older writers is a misno- mer, for, as I have shown, it is not inflammatory at all. It is a neural degeneration and exhibits all the changes seen in a secondary or Wal- lerian degeneration. It must be frankly admitted that these refinements of classification are of more pathological than clinical interest. We very rarely have a pure interstitial neuritis without secondary degenera- tion, or a secondary traumatic degeneration without an accompanying inflammation. Practically all cases exhibit the same symptoms. It is unfortunate that we cannot better distinguish the degenerative from the inflammatory trouble by the respective symptoms, for the prognosis of the case could be much more positively affirmed then. According to the relative amount and degree of the degeneration and inflammation, >ive electric shocks, violent massage, direct blows near the nerve may easily cause it to become inflamed, especially in those pre- disposed by general debility. Inflamed swollen joints, neighboring tumor>. exostoses, dislocated and fractured bones are not infrequent causes. Tight bandages, excessive pressure in reducing a luxation, ' 582 THE NOX-N EURO NIC DISEASES wrenches, twists and nerve stretching of all sorts are to be reckoned with etiologically. Extremes of temperature are a prolific source, es- pecially when there is an underlying diathesis. In this way cold is a common cause of facial neuritis. Sometimes two or more causes act together. Sciatic neuritis may be due to the cold and pressure experi- enced in going to stool under special conditions. Corroding substances, acids, burns, scars and cicatricial compression are of the nature of traumata. Strains from overuse are often given credit for lighting up a neuritis, but they are hardly efficient as causes without the presence of an infection or vitiated state of the constitution. They are more of the nature of exciting causes. I have seen a neuritis start from lifting a child suddenly by one arm. Birth palsies, especially those that occur from instrumental delivery and abnormal presentations, are usually neuritic. Non-purulent ascending neuritis used to be much talked of by the older pathologists. Nowadays it is scarcely believed to occur. Only the purulent form really ascends a nerve. Neuritis may be caused by puncture of the nerve with a hypodermic needle, or the introduction of irritants like ether, perosmic acid, chloroform, etc. This explains undoubtedly the results sometimes obtained by the injection of osmic acid in neuralgic nerves and especially in tic douloureux. These traumatic causes are especially prolific of the localized forms of neuritis. Ofttimes they are never anvthing more than mere excit- ing causes. The intoxicants are very significant factors in the production of neuritis. These and the infections give rise to generalized rather more frequently than localized neuritis. In fact, without a determining local insult of some sort they may almost be said to never produce a neuritis in a single nerve. An infectious process, like an abscess or a tuber- culous gland, a cancer or a syphilitic patch, may light up the inflamma- tion in a neighboring nerve. Alcohol, lead, arsenic and other metallic poisons, carbonic acid, carbon disulphide, ptomaines, nicotine, are all to be blamed sometimes for neuritis. The infections that have preceded the malady are diphtheria, vari- ola, typhoid, tuberculosis, influenza, scarlet fever, etc. I have seen syphilitic peripheral neuritis of the multiple type, though many doubt its existence and attribute it to the use of mercury. Syphilitic neuritis of the spinal and cranial nerve-roots is by no means a great rarity. Puerperal neuritis is infectious in origin. Leprous neuritis and the endemic forms known as beri-beri, akatama, etc., are to be mentioned here. In many cachectic and constitutional states, like rheumatism, gout, diabetes, leukemia, arteriosclerosis, malnutrition and autointoxication undoubtedly are the immediate, noxious factors. Arteritis obliterans acts largely in the same way, but more locally. It is hardly necessary, after what I have said in regard to the infections and their importance both as predisposing and exciting causes of neuritis, to mention suppurative processes, nearby or remote from the nerve. Pathology and Pathogenesis. — The whole subject of the pathol- ogy of neuritis has been, up to the present time, in a state of chaos. THE NON-NEURONIC DISEASES 583 This was the natural result of the clinical study of these diseases pre- ceding the pathological. It must be frankly granted that clinically all forms of nerve inflammation and degeneration are practically about the same. The two conditions are not only almost always found to- gether, but symptomatically they cannot be well distinguished apart. When, however, we leave the clinical side of the question and take up the pathological, the distinctions are so sharp and so clean-cut that I believe much of the confusion can be banished by attempting a positive classification of the disease on a purely pathological basis. Neuronic and non-neuronic processes underlie the pathology of neuritis. The former are degenerative, the latter are inflammatory. In some conditions the former may appear alone. It is then a primary degeneration and has been unfortunately and erroneously called paren- chymatous or degenerative neuritis. As I have shown, it is not a neu- ritis at all and should be banished entirely from consideration under this head. It is a rare condition and is merely another manifestation of a much more extensive condition. It may be seen in locomotor ataxia. The much more common form of neuritic degeneration is the secondary. A consideration of this is strictly in place here. It is a local trouble, is due to extra-neuronic factors, and is always a companion of the inflammatory process, though it may sometimes precede, some- times follow the latter. These two, inflammation and secondary degeneration, go hand in hand ; sometimes one, sometimes the other leading. At times the de- generation is so slight or the cause of it is so evanscent that the neu- rones recover before the interstitial inflammatory phenomena have got- ten a fair start. Under such circumstances recovery takes place and the disease has all the appearance of having been a temporary form of primary degeneration. Such are some of the degenerative poly- neuritides, some of the types of Landry's paralysis, some of the severe infective troubles that quickly end in death or recovery before either degeneration or inflammation has gotten sufficient of a start to produce gross, discoverable changes. Doubtless the character of the infection and the neuropathic state of the patient determine to a large extent the predominence of the degenerative or inflammatory process in this twin-like pathological development. In many cases the inflammatory process is so violent, the disease is most emphatically a neuritis with all the symptoms of an inflammation, the degeneratinn coming along as a later and slower process. Thus there are not many types of neuritis as authors declare. There is only one neuritis in which there is a variable, double pro going on. It is simply confusing to talk about perineuritis, interstitial neuritis, parenchymatous neuritis, periaxile segmentary neuritis, dis- seminated nodal neuritis. One might as well talk about motor neuritis, sensory neuritis, neuritis with fever, neuritis without fever, cranial neuritis and podalic neuritis. Medicine is too full of such artificial and useless refinements of classification. As I have urged before, it is the aim and goal of science to combine and generalize a multiplicity of facts, not to break what is already plainly combined, into a multiplicity of fragments. Primary degeneration being banished entirely from the ' 5^4 THE NON-NEURONIC DISEASES neuritides, all of the other forms are but phases of the one combined inflammatory degenerative pathological process which for want of a better name we call neuritis. The inflammatory side of the neuritic picture does not require any lengthy description. There is hyperemia, proliferation of the blood- vessels, extravasation of blood, leucocytosis, swelling, cedema and all the usual signs of an inflammation anywhere. The swelling does not FIGURE 132. t t — Degeneration and regeneration of nerve-fibres, a, nerve-fibre, fifty hours after operation, m y, medullary sheath breaking up into myelin drops, p, granular proto- plasm replacing myelin, n, nucleus, g, primitive sheath, b, nerve-fibre after four days, cy, axis cylinder partly broken up and enclosed in portions of myelin, c, a more advanced stage in which the medullary sheath has almost disappeared. Numerous nuclei, n" are seen, d, commencing.regeneration ; several fibres (t\ t") have sprouted from the somewhat bulbous cut end (6) of the nerve, a, an ajris cylinder which has not yet acquired its medullary Bheath. s, s'. primitive sheath Qf the original fibre. I'Ranvier.) appear uniformly along the whole course, or even a long extent, of the nerve. It appears more prominently in foci or spots. These are prob- ably the explanation of the tender points of Valleix seen in many cases of so-called neuralgia. Sometimes in these spots an exudate appears in abundance and later on a proliferation of the connective tissue, af- fording ground for the name disseminated nodal neuritis. As neurologists we are more interested in the degenerative side of this neuritic process than we are in the inflammatory. This is always of the secondary or Wallerian type of degeneration. It means that the nerve fibrils have been more or less severed from their nutrient centers. THE XOX-XEUROXIC DISEASES 585 I have described this process elsewhere. The medullary white substance of Schwann on the distal side of the lesion, breaks down into fat globules, is reabsorbed and leaves nothing but the axis-cylinder and the neurilemma. The neuclei of the latter proliferate. The axis-cylin- der swells up, liquifies and breaks down into fragments. Gradually these are absorbed so that nothing is left but the neurilemma in the shape of a hollow tube filled with liquid matter and nuclei. Ranvier regards the nuclear proliferation as the primary part of the process, and the breaking up of the myelin sheath as the result of it. Alterations take place simultaneously in the epineurium and perineurium, so that the nerve becomes smaller and harder, in fact sclerosed. The termina- tions of the nerve in the muscles and the end-plates ail participate in the degeneration. Forty-eight hours witnesses these changes and at the end of a couple of weeks they are complete. The cut end of the nerve shows the absence of the fibres and the hyperplasia of the con- nective tissue. The muscles innervated by the damaged nerve also degenerate. The fibres diminish, lose their striations, become granular or waxy. There is proliferation of the sarcolemma and the internal perimysium. Finally the whole muscle atrophies and becomes fibrous or cirrhotic. The nerve on the proximal side of the point of severance or lesion undergoes the change known as the reaction a distance. This is a de- generation of a slower and less complete type, probably due to the ab- sence of normal stimuli. The course of the degeneration follows the well-known Wallerian laws. Thus if the posterior spinal nerve root be cut between the ganglion and the cord, the degeneration occurs in the end attached to the cord and passes even into the cord. The end attached to the ganglion remains intact. Motor nerves and the anterior nerve roots, when cut, degenerate downward in their distal parts. The spinal at- tachment does not degenerate. The regeneration of a nerve is a most interesting and important process. It can only take place when the degeneration has not gone on to completion. In some compression neuritis degeneration of a partial character and regeneration undoubtedly take place with great rapidity, so that profound symptoms are often seen to quickly appear and disappear. In a severed nerve that has been sutured the process of neurolization takes place, though it is denied by some. It consists of a growing forward of the axis cylinder from the central stump into the red end. If the nerve fibrillar are given a bridge of decalcified osseous matter or a fine bundle of catgnt over the lesion, they will ad- vance all the more rapidly and completely. In this they resemble the advancing branches of a growing vine. Months and years arc some- times required for the regenerative proc Symptoms. — When a nerve is damaged there will be motor, sensory, trophic, reflex, electrical, vasomotor and secretory disturbances. The character. of these and the degree of their severity will vary in accordance with the complete or partial character of the damage done. There are early signs of irritation, followed by ti less complete paralysis. It cannot be too strongly impressed upon the mem- 586 THE NON-NEURONIC DISEASES ory that nerve lesions show signs of irritation and paralysis, in all functions, in the areas particularly innervated by the nerve involved. The motor symptoms are spasms and twite kings in particular mus- cles or muscle groups, followed by a flaccid paralysis more or less pro- found. It is soon followed by the loss of reflex response, the electrical reaction, of degeneration and atrophy. . The early symptoms in the sensory sphere are parcesthesice of vari- ous sorts. There are numbness, tingling, pins-and-needles sensation, sense of heat, ants crawling on the skin or formication. Soon pains occur both along the course of the nerve, especially upon pressure, and in its peripheral distribution. On account of the pressure on the nervi nervorum the pains seem to shoot here and there along the course of the nerve. The skin becomes hy paresthetic. Late in the disease it be- comes hyp aesthetic, rarely anaesthetic. A curious and almost path- ognomic feature about the sensations is that they are in spots and that hypaesthetic areas will be intermingled with hypersesthetic areas. The sensory and motor disturbances attack corresponding parts, showing thus the limitation of the disease process to particular nerves. In neu- ralgias, so-called, often the neuritic character of the disease is shown by the local tenderness, the painful swellings which can be felt by palpation and which are probably identical with Valleix's tender points, and by the special tenderness of the nerve where it passes over bony prominences. The trophic disorders of neuritis include a glistening of the skin, herpetiform eruptions, pemphigus, gangrene, formation of slow healing sores, changes in the bone, subcutaneous tissues, hair, nails, etc. The vasomotor and secretory troubles are local hyperemia or cyanosis, with corresponding change of temperature, oedema, hypert- drosis. Fever and chills sometimes occur early. Diagnosis. — The chief signs of neuritis, whether it be prominently degenerative or inflammatory, is the gradual break in all functions in the parts supplied by the particular nerves. Especially significant, how- ever, are the early irritative signs, such as spasms, tonic contractions, sharp pains and paresthesia, followed by flaccid, atropine paralysis, with loss of reflexes and reaction of degeneration. One can hardly make an error of diagnosis if he is careful to get all of these symptoms and view the picture formed by them in toto. It points distinctly to a lesion of a mixed nerve. The differential diagnosis of neuritis from neuralgia and from con- ditions in which it occurs as a side issue, will be taken up more con- veniently in the discussion of these other diseases. Prognosis. — Except in the mildest cases of compression, when recovery may take place in a few days or weeks, the course of neuritis is usually a prolonged one, six months or a year at least being its aver- age duration. The prognosis depends upon many factors. When the disease occurs in an alcoholic or a victim of lead intoxication, infection or other general cachexia, it is apt to be severe and last long, though the exciting cause may seem to have been insignificant. The virulence and character of the toxin in the toxico-infectious cases determines the THE NON-NEURONIC DISEASES 587 prognosis. Some toxins seem to light up a violent inflammatory condi- tion, which soon subsides upon the removal of the cause ; other toxins provoke a more gradual degenerative state with a mild degree of asso- ciated inflammation, which becomes chronic and even leaves a perma- nent defect. When the nerve is completely severed the restitution does not occur for months and sometimes not at all unless the stumps are sutured together. Cases in which it used to be thought that the neuritis ascended into the cord are very doubtful. Most of them have been toxic cases and the degeneration has been rather of the primary type with the nutritive functions of the central cells damaged by the general toxaemia. The prognosis can usually be based upon the electrical phenomena. If the reaction of degeneration is slight, recovery may be expected early. If the reaction of degeneration is complete, the course of the disease will undoubtedly be prolonged, and perhaps there will be some perma- nent defect left in the function of the nerve. Death does not occur from neuritis, unless the nerves involving the heart and respiration are af- fected. Permanent annoying paresthesias, pareses, trophic manifesta- tions need not cause surprise, though they cannot be said to be common. Rheumatic and acute infectious cases offer, as a rule, the most hopeful outlook. Treatment. — The cause, if it can be discovered, must be removed promptly and thoroughly before any treatment is instituted. In the majority of the cases practically this, and putting the limb at rest, are all that will be necessary. In removing the cause both medicinal and surgical resources may have to be called upon. Diatheses, such as rheumatism, tuberculosis, malaria, syphilis, must be combated in the usual way. Abscesses must be drained ; cancerous foci must be re- moved ; diseased glands must be cut away. It hardly needs to be said that alcohol, lead, arsenic and other poisons must be stopped and rapid- ly eliminated from the system. Luxations and subluxations, fractures, exostoses, cicatrices, compressing tumors mav all have to be removed before anything can be done with the inflamed nerve. If the nerve has been severed through an open wound, the wound sh< -uld be enlarged, if necessary, and the ends of the nerve approximated promptly by sutures. Primary suturing has given some excellent re- sults and prevented paralyses and other troubles. For the details of nerve suturing works upon surgery should be consulted. The cause having been attacked as completely as ]>■ ssiWe, an anti- phlogistic treatment may be in order. ( )ften. however, simple rest and quiet is all that is necessary. In the violently inflamed cases cathartics, aconite, general diaphoresis with wet packs, and hot baths may all be thought of. Locally leeches, hot sand bath, hot compresses and later on inunctions of gray ointment, the use of vesicants and light touches with the Paquelin cautery are valuable. In the degenerative cases the anti- phlogistic methods need not be so vigorous. Re>t and antispecific medi- cation is about all they require. There is probably no class of troubles more than the neuritides in which the doctrine of laissez-faire produce- its happiest results. I am convinced that had electricity never been introduced into therapeutics, 588 THE NON-NEUROXIC DISEASES more cases of neuritis would have gotten well. It is a curious fact how electrical treatment is at once thought of the moment the diagnosis of neuritis is made. It would seem that some have the idea that elec- tricity is a sort of a beneficent angel for all diseased nerves. Only let it once get at the nerve and recovery will be sure to take place. As an actual fact electricity has been the bane of diseased nerves, for in being- used too early and in all cases alike, it has simply rasped and irritated them and increased the trouble tenfold. I have frequently seen a case of neuritis slowly but surely recovering suddenly sent into a severe relapse because the attendant labored under the delusion that a diseased nerve was the golden opportunity for him to flourish forth his electrical paraphernalia. The more my observation enlarges the more I am convinced that few and far between are the occasions when the electrical treatment of neuritis is called for. If it can be absolutely determined that the disease is prominently of the degenerative type, and that there is no inflammatory action going on — a condition which it is almost im- possible to determine — electrical treatment with galvanism may and does prove of much avail. Such a condition does occur very late in the violently inflamed cases, and earlier in the mildly inflamed. Hence it is absolutely imperative that electrical treatment should be postponed until all signs of inflammation are gone. In this stage of the disease elec- tricity and massage are of invaluable service. The kind of electricity to be employed will depend upon the site of its application and the degree of degenerative reaction present. Galvanism is to be used upon the nerves, faradism upon the muscles. If there is much degeneration pres- ent, the reaction of it may be so pronounced that neither the galvanic nor faradic current will awaken a response. This is, however, rarely the case. More frequently a partial reaction is present. Under such circumstances the faradic response may be wanting and only the gal- vanic be present. In using the galvanic current the anode may be placed upon the plexus or sternum, and the cathode upon the points indicated in Erb's diagrams in the chapter on electrotherapeutics. A stabile current is to be used and its strength should average about five,, ten or fifteen milliamperes. Labile galvanic stimulation of the muscles may be added to the treatment. Faradic stimulation is to be used as soon as the faradic irritability returns. It is never to be used if there is the slightest sign of inflammation present. It is positively dangerous in the early stages of the disease. Later on it is more beneficial. Evert static electrization is good in the later stages. Massage must not be employed too early either. As a restorative massage is second only to electricity. It is not well to rub directly over the nerve, but along side of it. The manipulations must be gentle and towards the body, and the seances should be daily. Invaluable as electricity and massage are in the treatment of the degenerative results of neuritis, I must repeat the caution not to use either in the early stage of acute inflammation. Atrophy, contractures and shortening can be controlled by them as well as by systematic active and passive exercise. Recently surgery has been accomplishing some brilliant feats in old neuritic cases. A number of operations in the way of secondary THE NON-NEURONIC DISEASES 589 suturing, transplanting and grafting of nerves have been practiced with .great success. The distal end of a bisected nerve may be united with the prox- imal end of another that has been uninjured. The facial and hypo- glossal have thus been united. Where there has-been a break in a nerve transplantation of nerves from animals has been successfully performed. If the break measures more than about four centimetres, the results are not so promising. Nerve tumors have been extirpated and the ends of the stumps thus approximated. Nerves have been freed from tumors, cicatrices, callus and exostoses, even after sixteen years' standing, and restoration of movement and sensation obtained. Ofttimes splints and bandages have to be worn for some time after these operations. Elec- trical treatment is also later on called for and should be continued for a year. MULTIPLE NEURITIS. Paralysis and sensory disturbance was observed long ago to fol- low the ingestion of alcohol, certain febrile exanthemata, constitutional cachexia and poisons of various sorts. Such paralyses were spoken of as alcoholic, lead, febrile, diabetic palsy, etc. Most excellent descrip- tions were given of it by Jackson, Huss, Lancereaux, Duchenne and others. Most of the theories propounded to explain it attributed it to disease of the brain or spinal cord. In 1864 Dumenil made the first careful post-mortem examination of a case dead from alcoholic paralysis and announced that the peripheral nerves were diseased while the cord and brain seemed to be perfectly normal. Though at first neglected, Dumenil's observation soon began to be confirmed by the observations of others, notably Eichhorst, Joffroy and Leyden. The last observer finally established the disease, in 1880, as a distinct entity. Since the work of Leyden, our knowledge of the malady has been steadily ad- vanced by the investigations of Eisenlohr, Striimpell, Ross, Buzzard, Hun, Mills, Cole and many others. That there is such a disease as a pure, uncomplicated polyneuritis is undeniable ; that it is rare is being more and more recognized. As an associated trouble with general con- stitutional intoxication and disease of the central nervous system it is very frequent. Many problems still await a solution in regard to its pathogenesis, its extent and distribution, its complications and accidents and associated troubles, the essential nature of its disease-process, the differential diagnosis and pathology of its various forms, its course and prognosis and its proper management. Etiology. — The causes of multiple neuritis are numerous. Under the head of pathology will be discussed the various types of the disease with mention of the etiological factors that play the principal role in each type. In the vast majority of cases intoxication and infection are the causes. This is so overwhelmingly the case that it is beginning to be believed that all other causes than intoxication and infection serve merely as excitants and not as essential etiological factors. Alcohol is the supreme toxic cause, especially when taken steadily, though mod- erately, for a long period of time. It matters but little whether it is imbibed in the form of whisky, beer or wine, so long as it sets up a 59o THE NON-NEURONIC DISEASES state of chronic alcoholism. After alcohol we find that the disease is caused by ether intoxication, bisulphide of carbon, carbon dioxide, naphtha, lead, arsenic, coffee, mercury, anilin, ptomaine and leucomaine poisoning. It has been attributed to sulphonal and other drugs and medicinal preparations. Among the infectious antecedents we note leprosy, typhoid fever, variola, scarlatina, influenza, diphtheria, erysipe- las, purulent pleuritis, acute articular rheumatism, mumps, gonorrhoea and dysentery. To these must be added tuberculosis, syphilis, malaria, diabetes mellitus, severe anaemia, carcinoma and marasmus from any cause. Septicaemia and puerperal fever have preceded its outbreak. It has been charged to alimentary autointoxication, constipation and putrid bronchitis, icterus and hepatic cirrhosis. Akatama, kakke and beriberi are endemic forms of the disease doubtless due to an infection. The senile form of the disease has been attributed by Oppenheim, Joffroy, Gombault, Schlesinger and others to arterio-sclerosis and arteritis obliterans. Exposure to cold, overexertion, mental and nervous strain are provocative only in the presence of other conditions, such as those mentioned above, and hence are to be regarded more in the light of mere exciting or initiatory causes. The middle period of life, when the above causes are most in evidence, is the time when multiple neuritis is most frequently met with. In children the diphtheritic form is the most common as well as those forms that are due to scarlatina and other infantile infections. In old age the cachectic forms of the disease predominate. Women are the victims of the alcoholic type far oftener than men. Other etiological factors of the disease depend upon the etiology of the underlying con- stitutional intoxication and infection. Pathology and Pathogenesis. — The characteristic pathological finding of multiple neuritis is a parenchymatous degeneration of the peripheral nerve ends. By the older writers this was supposed to be a secondary result of an interstitial inflammation of the nerve trunk or of a primary parenchymatous inflammation. Hence the name neuritis, and its division into interstitial and parenchymatous neuritis. No pathologist of repute nowadays speaks of the latter as an inflammation. As a result of the direct action of the alcohol and ether poisons upon the nerve fibre or its cell, a degenerative process occurs. Therefore we speak at present of two great classes of neuritides, namely, those of inflammatory (interstitial or periaxial) and those of degenerative (parenchymatous, axial) origin. They are the same disease, however. As I have explained under the head of neuritis, this is a degenerative trouble, not of the purely primary type, but of the combined degenera- tive and inflammatory type. Both processes are dependent upon the same cause and are mutually to a certain extent dependent upon each other. Sometimes one, sometimes the other is the more prominent feature of the disease, but both go together. Under this conception we find that polyneuritis can be conveniently subdivided into three general classes, all of which differ slightly from one another in regard to etiology, symptomatology, pathology, prognosis and treatment. THE NON-NEURONIC DISEASES 59 1 In the first class, perhaps the most typical, certainly the largest class, we include the neuritides of the degenerative type, whether the primary cause be toxic (alcohol, arsenic, mercury, etc.) or infections (typhoid, variola, puerperal fever, tuberculosis, syphilis, etc.) or cachectic (diabetes, carcinoma, marasmus). Some of these are primary degenerations and, as 1 have noted elsewhere, are but side issues, as it were, of other diseases. They are not special any more than tubercu- lous glands would be special diseases apart from general tuberculosis. In the second class may be conveniently grouped the neuritides, neuritis of the inflammatory type, in which the parenchymatous degen- eration is entirely secondary. These cases include the endemic, infec- tious types such as leprosy, beriberi and certain forms of unknown origin. In the third class both inflammation and degeneration play a role in their origin. They are the neuritis of diphtheria and some primary forms of unknown origin. The second and third class are the true types of neuritis. In all forms of multiple neuritis the inflammatory signs are never as prominent as are the degenerative. There may be in some of them redness and swelling of the nerve trunk and hyperemia of the sheath. Even minute hemorrhagic extravasations with all the usual manifesta- tions of a severe local inflammation may be seen here and there along the course of the nerve. This is not common, however. More fre- quently there are no discoverable interstitial changes ; only more or less atrophy of the individual nerve-fibres. The myelin substance is broken into clumps; the contour of the fibre is irregular in outline; and the fibres are reduced in size and diminished in number. Among this mass of wasted and diminished fibres some are seen to be regenerating, while others retain their normal, healthy appearance. The disease process in many cases seems to grow less, both in severity and extent, as we ascend the nerve trunk. The point of transition from diseased to healthy nerve tissue varies in different cases and in different nerves even in the same case. In some of the cases the lesion is limited to the peripheral nerve- fibres. This is especially true of the diphtheritic cases, wherein the poison acts directly upon the nerves in the immediate neighborhood of the infected focus. Nevertheless, in the diphtheritic cases and much more so in the other toxic and infectious cases, foci of disease are found in the spinal nerve roots, the spinal cord and its membranes. In alco- holic, lead and other cases, the myelitic and encephalic evidences of dis- ease are being discovered post-mortem more and more frequently. These partake of the nature of poliomyelitic and encephalitic processes with atrophy of the cells. On account of this the view is growing that in these cases the toxin affects the entire nervous system, of which the polyneuritis is> but one of many local expressions. In this way its great variety of symptoms is explained. Some attribute the neuritis even to the functional damage done to the cells in the cord by the poison. Cole and others among the most recent investigators frankly affirm that multiple neuritis, especially of the alcoholic type, is a widespread affec- tion of the nervous system ; that the central changes are not attributable 592 THE NON-NEURONIC DISEASES to the peripheral trouble ; that the lesion of the peripheral neurones is only one of many manifestations of the disease and is accompanied by lesions identical in nature, affecting many groups of neurones situated entirely within the central nervous system ; that though in some cases' the peripheral neurones are mainly affected, in others the morbid process implicates chiefly the central neurones, two groups of cases which do not appear, however, to be sharply divided. The same is true, though not to the same marked degree, in regard to the other forms of polyneuritis, especially those due to lead and arsenic and in which mild delirium occurs with febrile phenomena. As the bacterial products and not the bacteria themselves are credited with the production of the disease, it is not surprising that the infectious cases, like the toxic, should show the same general characteristics in regard to the entire nervous apparatus. Symptomatology. — Charcot used to contend that peripheral neu- ritis is not a distinct disease, but a substratum underlying the symptoms of several very different affections. His contention was supported by the dissimilarity in the clinical pictures presented by the different forms of multiple neuritis. Even to-day we are obliged to discuss the symp- tomatology of alcoholic, lead, arsenic, diphtheritic paralysis, etc., under their own respective heads. Unlike as they are in many respects, these various forms of multiple neuritis present a sufficiently constant and uniform set of symptoms to base a clinical description upon. As the alcoholic type of the disease is the most common and uniform, it will be described first as a sort of symptomatic model. I. Toxic Forms; (a) Alcoholic Type. — The first symptom noticed by the patient is a paresthesia in the feet and hands. It comes on more or less suddenly and consists of numbness, "pins-and-needles"' sensation and a feeling of pricking. There may be actual pain in the limbs, sharp and shooting or dull and rheumatoid in character. These early sensations may occur in the course of chronic alcoholism or after an attack of delirium tremens. Occasionally the temperature may be ele- vated, but as a rule there are no febrile manifestations. The paresthe- sia is bilateral in distribution. It attacks all four limbs about in the same way and gradually ascends toward the hips and shoulders. The pains are irritating, may be sharp or dull, may come and go in the course of particular nerves, and gradually lessen in severity as the disease advances. A feeling of weakness appears in the limbs in a few days and the muscles and tendons begin to feel like stiff parchment to the patient, or as if they were tense and just a little too short. Between the numbness, weakness and slight rigidity, the patient cannot coordi- nate his movements and so finds difficulty in walking and using the hands. In a little while the weakness passes into an actual paralysis. This seems to involve the extensor more than the flexor muscles, caus- ing wrist-drop and ankle-drop. All the nerves of the extremities may- be involved, but the musculo-spiral and the anterior tibial seem to be especially affected. The muscles of the trunk and head as a rule escape and the mind is affected in a particular way only in certain cases. The reflexes are lost, both deep and superficial. The muscles are noticeably wasted and if tested electrically exhibit more or less completely the THE NON-NFURONIC DISEASES 593 reaction of degeneration. There is some anccsthesia, distributed gener- ally or in spots over the limbs. Hyperalgesia is often a valuable symp- tom and the local tenderness in the muscles and nerve trunks is so characteristic a sign that it helps to differentiate the disease from other affections with similar disturbances of sensation and motion. Hyper- esthesia may be discovered in areas of the skin alongside of areas of hypaesthesia. As a result of the sensory disturbances ataxia may be present. Hyperidrosis, oedema, szvelling in and about the joints and even certain skin eruptions and ulcers may be expected among the later vasomotor, secretory and trophic manifestations. The rectal and vesical functions as a rule are unaffected. Such in outline is the clinical pic- ture of a typical multiple neuritis. Such a pure type, however, is rare and it is therefore necessary to consider some of the symptoms a little more in detail. The more prominent of the sensory symptoms, being subjective in character, are less valuable as symptoms than some other signs on that account. They are indicative only of the disease when studied as a part of the clinical picture in toto. If taken by themselves they may be mistaken for hysterical and other psychoneurotic manifestations. Though the sensory phenomena are rarely absent, they assume the most varied and bizarre characteristics. For instance, in the soles of the feet all qualities of sensation may be decreased or lost ; or there may be anaesthesia with hyperalgesia or hyperesthesia with analgesia. A very common combination which I have seen often is anaesthesia with hyperesthesia in adjoining areas. The touch and muscular senses may be diminished, while the pain sense is highly exaggerated, or vice versa. Instead of being diminished the sensations may only be delayed. In the trunk a rare girdle sensation is sometimes found, but it belongs to the other symptoms of central disease usually accompanying alcoholic neuritis. It is to be noted carefully that these sensory manifestations are symmetrical in distribution, that they are more extensive than the corresponding paralysis, that they are partly subjective and objective in character and that they exhibit the variability and irregularity that one would expect from a variable lesion involving a large number of separate and unrelated peripheral nerve fibres. Such an incongruous set of phenomena could only be imitated by a pure psychosis, but a psycho- sis can easily be distinguished from a polyneuritis by other symptoms. Much more striking than the sensory symptoms is the motor paralysis. In some forms of multiple neuritis this is almost the only symptom. It is of course of the flaccid, degenerative type of paralysis, since the lesion involves the lower or peripheral motor neurones. It is always accompanied by distinct muscular atrophy and alterations in the electrical reactions. It is usually more profound in the legs than in the arms, though great variations are possible as to the areas of its distribu- tion. In some respects it resembles marvdously the paralysis of an- terior acute poliomyelitis, for it may involve one leg or certain muscles only in that leg, or only certain muscles in all four limbs or even all the muscles of all the limbs. Curiously where a nerve innervates more than one muscle, it sometimes happens that one muscle will be paralyzed while the others escape. The nerves may show the electrical reactions 594 THE XOX-XEUROXJC DISEASES of degeneration and yet their corresponding muscles not be paralyzed. The peculiar character of this paralysis has even led some to think that the disease is primarily a toxic injury of the cells of the anterior horns of the cord with a mere secondary degeneration in the peripheral axones. Though this is not entirely acceptable, it is undoubtedly true that in some cases, at least, the pathological process and its resulting paralysis is partly poliomyelitic as well as neuritic. In some cases ataxia rather than paralysis is the chief motor mani- festation. It is rare, however, and is the symptomatic basis of the peripheral pseudo-tabes of Dejerine. It is often associated with anaes- thesia, and implicates the legs much more often than the arms. Of course, it changes the gait and active movements and may lead to an erroneous diagnosis of locomotor ataxia. Involuntary muscular twitch- ings sometimes accompany it. The atrophy and tenderness of the muscles are two characteristic symptoms of this disease. They are most always present and when found together in the same patient are pathognomonic. The wasting of the limbs is marked and rapid. It is not as severe and rapid perhaps, nor does it pick out individual muscles so strikingly, as does the atrophy of infantile palsy. It may for a time be obscured by oedema or fatty deposits. The muscles feel soft and flabby, are cold and somewhat pale in color. Pressure and passive movement oiten cause such intense pain that it is impossible to make a complete examination. Abolition of the deep reflexes is the characteristic of polyneuritis. Usually this is complete and early in appearing. Sometimes, however, it is for a brief time preceded by an exaggeration. Occasionally it is incomplete and capable of being partially elicited by reinforcement. The paralysis in such cases is a partial one. The cranial nerves are frequently involved, though their implica- tion is in no way pathognomonic. The same may be said of the brain. And yet it is not to be wondered at in view of the frequency with which disturbances of the brain and cranial nerves are met with that many are beginning to look upon them as essential manifestations of the dis- ease and not as mere complications and accidents. In other words, the view is spreading that the polyneuritis is but one manifestation of a much more extensive disease process, namely, a toxic degeneration of the entire nervous system (Buzzard, Cole and others). Among the cranial nerves the third is most often affected ; next the sixth. Reflex pupillary rigidity is rare, but nystagmus, diplopia and ptosis may all be looked for. Very seldom is the optic nerve involved, though optic neuritis and even partial optic atrophy have been observed several times. There may be a central scotoma, especially for colors. Among the other cranial nerves that may be involved are the facial, the vagus and phrenic and the nerves of mastication and deglutition. Strumpell saw the auditory nerve involved. A polyneuritis limited to the cranial nerves would be a curiosity, and yet such has been referred to by Hosslin and. Mannaberg. The mental symptoms of alcoholic neuritis are deserving of special consideration. They consist of failure of memory, confusion and loss of the proper conceptions of time and space. Korsakoff was the first THE NON -NEURONIC DISEASES 595 to name and systematize this polyneuritic psychosis, though others had observed and described it before him. Amnesia, disorientation, pseudo- reminiscence and confabulation are its chief characteristics. Events of the past are spoken of as about to occur in the future and vice versa. Hallucinations and mild delusions are sometimes present. They are easily repressed. Occasionally they are determined by the sensory symptoms. One of my cases spoke of having just been out in the gar- den picking flowers and getting covered with burrs, when, as a matter of fact, it was a cold, midwinter day and she had not been out of bed for ten days. By many this confusional type of psychosis is regarded merely as an alcoholic complication of the polyneuritis. Others attribute it to a mild toxic encephalitis, which, like the peripheral neuritis, is one of the outward expressions of the underlying chronic toxaemia or alco- holism. The hypothesis has even been set up (Turner) that it is the result of the isolation of the patient's mind by the shutting off of his normal afferent impulses through the widespread neuritis. If alcoholic polyneuritis is a distinct disease in itself, then these mental manifesta- tions are of the nature of pure complications or accidents. If, however, the real disease is an alcoholic degeneration of the entire nervous sys- tem, with peripheral neuritis as one of its outward expressions, the central symptoms will then have to be regarded in the light of another set of outward manifestations. Alcoholic polyneuritis in the majority of instances runs an acute of subacute course and its prognosis is in the main favorable. It ap- pears rather abruptly, rises to its climax in a few weeks or months, remains stationary for about the same time and then gradually recedes. There are violent cases that run a rapid course and terminate in death in a few days. In such cases the cranial nerves, especially the vagus and phrenic, are likely to be involved. The general condition of the patient, the implication of the cranial nerves, the extent of the paralysis, and the degree of muscular atrophy are all factors that influence the prognosis. Rarely the disease runs a progressive or even remitteni chronic course. Occasionally recovery is complete; more frequently it occurs with some more or less permanent defect. This is especially true of the cases with mental symptoms. Convalescence is always slow and tedious. Irritative symptoms, like pain, disappear first; then the anaesthesia gradually clears up or is replaced by paresthesia ; and finally after a long time the hyperesthesia diminishes to normal. Some- times two years have to elapse before all signs of paralysis have van- ished. Relapses are not uncommon. Permanent paralysis in particular muscles, and contractures and deformities from overaction of antag- onistic muscles, are unfortunately possible. (b) Lead Type. — Lead intoxication produces a polyneuritis strik- ingly unlike the other forms of the disease in some respects. It was this fact that led Charcot to contend that multiple neuritis is not a dis- tinct, uniform disease. Motor paralysis confined to a certain area., without distinct sensory disturbances, constitutes the predominant fea- ture in its clinical exhibition. That the disease, however, is an intoxica- tion of the entire nervous system is shown by the brain symptoms and! the arthritic pains. The ingestion of lead is the cause of the disease ; 596 THE NON-NEURON IC DISEASES though it has been seen as an inheritance in children whose parents have been lead workers. There are many sources from which the lead enters the system. Painters, type-setters, pipe-layers and other workers in lead ; potters using lead glazes ; weavers handling leaden weights ; users of cosmetics containing lead ; washers of bottles employing lead shot ; and drinkers of water drawn through lead pipes are all exposed to the disease. So extensive is the use of the metal in modern industry and so insidious at times is the onset of the paralysis that only a most painstaking search will reveal the source of the intoxication. In many cases the motor paralysis is the only symptom ; in all cases it is the characteristic symptom. When it is preceded or accom- panied, as it is usually, by lead colic with its griping pains in the abdo- men, by arthralgia with its pains and soreness in and about the joints and muscles and especially by encephalopathia saturnina with its mental manifestations, the diagnosis cannot be mistaken. The blackish streak or blue-black coloration of the gums along the edge of the teeth, the anaemia, the cachectic state, the disease of the kidneys and the arterio- sclerosis all indicate the wide extent of the intoxication and help to confirm the true origin of the palsy. As I have said, this palsy is striking and pathognomonic almost, for it is never, or rarely, accompanied by sensory phenomena, is limited practically to the arms and preferably to certain muscles. It is a degenerative paralysis as shown by the loss of reflex action, the marked atrophy of the muscles and the electrical reaction of degeneration. It involves the peripheral muscles of the limbs symmetrically, showing a remarkable selective action. Occasionally certain muscles individually are picked out as in infantile palsy. At other times the disease is limited to one arm. This is usually determined by the patient's occupation, the arm or muscles most employed being the first to succumb. The ex- tensors of the hand and fingers are particularly liable to the paralysis, resulting in the easily recognized "wrist-drop" and flexion, of the fin- gers. If the feet are involved, as rarely they are, the peroneal nerve distribution is the one mostly diseased. The tibialis anticus muscle usually escapes under such circumstances. There is no uniformity with which the muscles of the arms are involved, but in typical cases the supinators, especially the supinator longus and the triceps, are spared. The extensor carpi ulnaris and the abductor longus pollicis are also un- affected as a rule. In other words, the radial nerve and its distribution is in the more typical cases the focus of the disease. In other cases the smaller muscles of the hand and thumb under the control of the ulnar and median nerves are also paralyzed. Still more rarely the interossei muscles and the deltoid are paralyzed along with the extensors. In Remak's upper-arm type of the disease, which, however, is atypical, the biceps brachialis interims and deltoid are implicated. Associated with the paralysis there is generally a certain degree of fibrillary tremor, swelling a1x>ut the wrist-joint and rarely thickening of the metacarpal bones. A generalized form of the disease beginning acutely with fever and following a saturnine encephalopathy involves all the muscles of the arms, legs and trunk. THE NON- NEURONIC DISEASES 597 The cerebral disturbances that often accompany saturnine poly- neuritis are numerous and important. They may be general or focal, organic in origin or functional. In this way hemiplegia and hemi- anaesthesia, temporary or permanent, may he occasionally observed. Convulsions, delirium and coma may be preceded by vertigo, tinnitus aurium, restlessness and insomnia. There may be distinct hallucina- tions of sight and hearing and all the manifestations of acute delirium. The cranial nerves may all be affected, optic neuritis and implication of the pneumogastric and phrenic being particularly ominous. The oculomotorius involvement is shown in mild mydriasis, as I recently saw in a very obscure chronic case of general lead intoxication. In chronic encephalopathia melancholia with delusions is the more common manifestation. The mental power fails or there are exalted delusions as there are in dementia paralytica. A degenerative meningoencephal- itis is the pathology in these cases. Hemorrhages, local encphalomala- cia with arteritis may occur here as in anv form of severe intoxication or infection. The prognosis of this form of multiple neuritis is favorable in the earlier stages of the disease and before grave paralyses have occurred or such serious complications as saturnine cachexia, nephritis, optic neuritis and other organic destruction of the tissues of the brain and cranial nerves. Recovery may be hoped for even after the appearance of the reaction of degeneration. Several attacks in succession of the paralysis add to the gravity of the outlook. (c) Arsenical Type. — Intoxication may result from the use of arsenical coloring matters in pigments, wall papers, book covers, etc. Attempted suicide with arsenic or Paris green may terminate in a multi- ple neuritis. Most of the cases of arsenical paralysis are due, however, to the excessive medicinal use of the metal in the form of arsenious acid or Fowler's solution. Symptomatically arsenical paralysis resembles most strikingly the alcoholic type and both forms of the disease differ from the saturnine and diphtheritic types in the greater prominence of the sensory mani- festations. There seem to be two classes of arsenical palsy. In one the muscles are decidedly paralyzed, especially the extensors of the hands and feet. In the other the sensibility is especially affected giving rise to a condition of ataxia or pseudo-tabes. These symptoms follow either acute or chronic poisoning and make their appearance in from a few days to a few weeks. The paralysis in the arms, flaccid and degenerative in character, involves usually the same muscles and causes the same characteristic attitude as lead palsy. The same is true in the legs. The deep reflexes are diminished or abolished and the usual electrical reaction of defen- eration is present. Muscular atrophy occurs very rapidly. Unlike lead, arsenic poisoning provokes a greater degree of sensory disturbance. The sensitiveness of the nerves and muscles to pressure is exceedingly acute. There are initial paresthesia of various sorts on the back of the forearms and front of the legs. Severe darting pains are complained of. Anaesthesia or hyperesthesia may be general or scat- tered about in patches. Sensation in general is almost always decreased, 59^ THE NON-NEURON IC DISEASES especially in the feet, hands and fingers. In these pronounced sensory cases there is incoordination of such a character that they closely re- semble ordinary tabes. The differentiation between the two diseases is made largely upon the presence of a true palsy in the arsenical neuritis. Though we speak of a motor and ataxic form of arsenical polyneuritis, the motor symptoms are more or less present always in both. In these ataxic cases the knee reflexes are practically always absent, pains are present, and the sensations are diminished in regard to touch, increased in regard to pain. Dana, to whom we owe much of our knowledge of this pseudo-tabes, saw optic neuritis in one of his cases. In Seelig- miiller's case the patella reflex was not lost. Psychic disturbances are present in cases of arsenical polyneuritis, though they are not so prominent as in some other forms of intoxication. The weakness of the memory, the mental confusion, and the dis- orientation bear a resemblance to the mental manifestations of alcoholic neuritis. Epilepsy and amaurosis have been recorded. Among the trophic symptoms, especially in the prolonged and se- vere cases, are to be noted herpetiform eruptions on the skin, true herpes zoster, pemphigus, erythematous and bullous outbreaks, glossi- ness of the skin, alopecia, falling off of the nails and other indications of a profound state of cachexia. The initial conjunctivitis and gastric irritation are not unfre- quently seen in cases in which medicinal arsenic has been administered to the limit of tolerance. In many cases the temperature is slightly increased and in nearly all of them the pulse is more or less accelerated. Recovery is the rule in this form of polyneuritis, though the con- valescence may last for months or years. As the bulbar nerves are scarcely ever implicated life is rarely jeopardized. Fatalities usually result from respiratory or cardiac failure when they do occur. (d) Argentic Type. Argyria. — This is an exceedingly rare form of polyneuritis. Gowers has reported a remarkable case in which the symptoms corresponded almost identically with those of lead poisoning, namely, paralysis in both arms of the long extensor of the fingers and of the extensors of the phalanges of the thumb without involvement of the extensor of the metacarpal bone of the thumb, atrophy of the muscles and reaction of degeneration. The legs were not affected and there seem to have been no sensory phenomena. The well-known bronz- ing of the skin and blue-black line on the gums were present. The source of the silver, in this case as well as in the non-neuritic cases of argyria generally, was its prolonged medical administration. As sil- ver is not employed now as it used to be in the treatment of such chronic diseases as epilepsy and spinal sclerosis, argentic intoxication is less frequently heard of. Scarcely any cases of multiple neuritis from this cause have been reported. (e) Mercurial Type. — It has been asserted that there is a mercu- rial multiple neuritis. If so we know very little about it. In most of the cases so reported there has been a history of syphilis treated with mercury. It is an open question whether the polyneuritis was of syphilo-toxic or mercurial origin. Schultze, Buzzard and Oppenheim have described syphilitic polyneuritis ; others doubt its existence. There THE NON-NEUROXIC DISEASES 599 is no question but that a polyneuritis in a syphilitic may be made worse bv the administration of mercury. I have lately seen this most forcibly exemplified and it has been confirmed by Minkowski, Engel and Op- penheim. The symptoms of hydrargyria point to lesions of the brain and cord rather than of the peripheral nerves. They consist of a peculiar tremor not wholly unlike that of paralysis agitans, slight loss of muscular power in one limb more than the other, pains in the region of the fifth nerve and around the joints, formication in the limbs and local analgesia, mental irritability, loss of the power of attention, dis- tress, insomnia, hallucinations and even maniacal outbreaks. There may even be considerable hemipiegic weakness, aphasia and deafness. With all this there is no typical paralysis of neurotic origin though these cases are ofttimes of great severity and acquire their mercury from gross sources as manufactures, mines and the industrial use of the metal. The stomatitis, ulceration of the mucous membrane of the mouth, fetor, bone necrosis, gastric and intestinal catarrh and other symptoms of profound mercurial intoxication are never accompanied by the typical signs of a peripheral multiple neuritis. It seems to me therefore that the existence of mercurial polyneuritis may be legiti- mately doubted and that the reported cases in which there has been a history of syphilis are to be attributed to the specific infection. II. Infectious Forms. — The infectious forms of polyneuritis do not differ symptomatically from the toxic forms though they are de- pendent upon such a multiplicity of infective processes. In the main they are all alike, differing from one another, as in the toxic cases, in some minor details. As the infection is general the lesions of the nervous system are of wide extent with a predominance of parenchy- matous degeneration. The symptoms are therefore cerebral, spinal and neuritic in origin. In some, as in typhoid and scarlatina, they are largely cerebral ; in others, as in variola, dysentery and measles, they are apt to be spinal ; in still others, as in diphtheria, acute rheumatism, erysipelas and puerperal sepsis, they are mostly neuritic. There are no fixed rules in this respect, but as the peripheral neuritis is of a varia- ble degree and extent common to all of these infective conditions, I refer to them under this head, using one or two of the more striking ones, like diphtheria, rheumatism and puerperal sepsis, as more or less illus- trative of all of them. The universal character and wide extent of the intoxication is shown by the multiplicity and variety of the symptoms and especially by the uncertainty among pathologists as to whether the peripheral neuritic degenerations are due to the primary damage of the central cells or to a mere simultaneous degeneration in both central cells and peripheral processes. Perhaps the larger number of investigators are ready to accept both explanations. None of the more recent patholo- gists believe, however, but what the interstitial changes sometimes found in these cases, and suggestive of true inflammation of the nerves, are but late and secondary results of the degeneration. (a) Diphtheritic Type. — In about one-quarter of the eases that recover from diphtheria a remarkable form of motor paralysis occurs, usually appearing from two to three weeks after the subsidence of the 600 THE NON-NEURONIC DISEASES throat trouble, rising to its maximum in two or three weeks more and finally disappearing in from two to three months to a year later. The lesions of the nervous system seem to be the result of both a local and general infection. For this reason some authorities speak of two forms of diphtheritic neuritis, the local and the generalized. A true inflam- mation of the nerves near the site of the primary infection is believed to characterize the local form, while a general specific toxic degenera- tion is thought to represent the latter. This may in part be true, but I am inclined to think that in the main the process is a generalized intoxication which in some cases affects only the nerves round about the original focus of infection where the poison would naturally be the most abundant and virulent, but not sufficiently so to involve the more distant nervous tissues, and in other cases affects both the local and more distant nerves. This is shown in the accidental and experimental infection of other parts of the body with diphtheria wherein the local nerves are again the ones first to be affected, and in the fact that in nearly all the cases of generalized and distant neuritis there is or has been more or less local neuritis near the primary disease. Motor paralysis, flaccid and degenerative, is the predominant symptom of this type of polyneuritis. All the usual characteristics of disease of the lower -motor neurone, such as loss of reflex action, mus- cular atrophy and the electrical reaction of degeneration accompany this paralysis. Sensory manifestations are insignificant or entirely absent. They are always more or less present when the paralysis is severe, and consist of paraesthesiae, such as numbness, tingling, formication or anaesthesia, sometimes hyperesthesia or, what is more common, hypaes- thesia. All forms of sensation may be altered so that incoordination obtains. Aubertin doubts, however, the occurrence of true ataxia. Rarely the bladder and rectum are affected and there is a remarkable absence of vasomotor, secretory and trophic symptoms. Albuminuria is common and in fatal cases almost constant. It is to the distribution of the motor paralysis that diphtheritic polyneuritis owes its distinction. In the larger number of cases it is more or less confined to the muscles of the soft palate. By reason of this fluids regurgitate through the nose and the speech is embarrassed with a nasal twang. Deglutition is therefore difficult and gargling is quite impossible. Puffing of the cheeks and the blowing out of a candle can only be accomplished when the nares are compressed. Upon inspection the palate is seen to be lower than normal, perhaps on one side a little more than on the other, and it does not draw up as it should when the patient utters the sound "ah." Its sensibility is dimin- ished or lost and no reflex response can be obtained through it. If the disease is prolonged the palatal muscles will be seen to be distinctly atrophied. The majority of these cases get well within a few weeks. Next to the paralysis of the palate, both in the time of its appear- ance and the frequency of its occurrence, is the paralysis of the mus- cles of accommodation. Tn many cases, with or without the implica- tion of the palate, the patient complains of diminution of vision for near objects while it may be normal or even increased for far objects. In the fact that this cycloplegia often precedes the palsy of the palate THE NON-NEURONIC DISEASES 6oi and is in all probability due to nuclear degeneration, the generalized nature of the intoxication receives support. The reactions of the pu- pil are generally normal. It may be sluggish to light, but is scarcely ever lost. It may contract when an effort is made to accommodate even though the accommodation is absent. Sometimes the external ocular muscles are involved. Any of the remaining cranial nerves may be affected. Immobility of the epiglottis from implication of the su- perior laryngeal nerves makes swallowing a difficult and dangerous task for fear of food getting into the trachea. Paralysis of the vocal cords through the involvement of the inferior laryngeal nerve causes hoarseness of the voice and even aphonia. Disturbances of the respira- tory and cardiac functions are particularly to be dreaded. These are not rare and often are the cause of death. Though the ophthalmoscope reveals no morbid changes, a concentric contraction of the fields of vision, marked, of course, by the effect of the cycloplegia, has been occa- sionally seen. Hearing seems never to have been affected, but taste and smell have. The tongue and face are rarely ever paralyzed. In many patients paralysis in the limbs follows the above manifes- tations. It may come on while the cranial palsies are still existent or after they have begun to clear up. Weakness, paresthesias and pains are first complained of in all four extremities. This quadriplegia is the indication of the polyneuritic character of the trouble. The legs more often suffer before the arms. The muscles are soft and flabby. The reflexes are diminished or lost. The electrical reaction of degen- eration is present and there is some atrophy. In a word, the paralysis is of the typical flaccid, progressive, degenerative type. As it involves the extensors and flexors of the limbs about equally, there are no de- formities. Walking is impaired or rendered impossible. The sensory manifestations with an ataxic gait may lead to a mistaken diagnosis of locomotor ataxia if the history and entire course of the disease are not given careful consideration. Not often are the trunk muscles involved, and yet they may be so weak that the patient cannot turn over in bed, or the respirations may be embarrassed, or it may be impossible to hold up the head. The diaphragm may cease to act. The prognosis of diphtheritic paralysis is particularly favorable if deglutition is not too much interfered with and the respiratory and cardiac functions continue intact. The local forms of the disease usu- ally get well in a few weeks; the generalized forms attain their maxi- mum in about two or three months and then rapidly decline, the aver- age length of time being three months. Recovery, as a rule, is com- plete, though in exceptional cases certain muscles may remain par- tially paralyzed. Dangerous symptoms arc paralysis of the vagus nerve and of the respiratory muscles, particularly the phrenic. Myocarditis and cardiac paralysis arc not unfrequent and fatal occurrences. Death by inanition has occurred in deglutitory paralysis. Severe cases when fatal die early; others run an irregular course with what seems like a series of relapses and then die of cardiac or respira- tory paralysis. In every case the prognosis is uncertain and especially so if there is involvement of the trunkal musculature. 602 THE NOX-NEUROXIC DISEASES Antitoxin treatment of diphtheria does not seem to have lessened the relative frequency and severity of the subsequent paralysis ; though it is claimed by Lublinski and some others that the generalized form is seen more frequently without the preceding localized form. (b) Rheumatic Type. — Polyneuritis sometimes occurs in con- nection with rheumatism. It is rare, but such cases have been reported by Putnam, Dana, Gowers, Remak and others. I have under observa- tion now an ataxic case due solely to rheumatic polyneuritis. Paralysis of neuritic origin not uncommonly occurs in the joints that are in- flamed and swollen, especially the ankle joint. The exact relationship of the arthritis to the neuritis is not clear. Some hold that it is infec- tious, others that it is purely mechanical and irritative. The symptoms of the neuritis are typical. Thus in a patient reported by Gowers and seen by Remak there was subacute articular rheumatism of both ankle joints with considerable pain and later on incomplete palsy of all four limbs, partial atrophy of the muscles with the reaction of degenera- tion, absence of knee-jerk, tremors and involuntary twitchings and disturbances of sensibility. The generalized nature of the trouble was still further emphasized by the implication of the face and facial nerves and the existence of some optic neuritis. This picture is dupli- cated by my case. The modern view that acute articular rheumatism is primarily a general infection would seem to receive some support from the occasional occurrences thus of polyneuritis with it. (c) Puerperal Type. — This has only been recognized the last few years. It may occur during pregnancy, especially in those who suffer from excessive vomiting. It is due to septic infection and ap- pears in both the localized and generalized forms. In the former the ulnar and median nerves are the affected nerves of choice. Some- times particular nerves in the lower limbs are similarly affected. In the generalized form of the trouble all of the peripheral nerves of the limbs, and any of the cranial nerves, are affected. Optic neuritis has several times been seen. In some cases the clinical picture corresponds quite a little to Landry's paralysis. (d) Other Types. — Almost any form of general infection seems capable of setting up a general neuritis. As the bacilli themselves do not provoke the lesion, but rather their poisonous products, these post- febrile cases correspond with those that result from alcohol and the metallic poisons. The lesion is toxic in origin, of wide distribution and degenerative in character. Owing to some obscure influence, the differ- ent infections give rise to some slight differences in symptomatic de- tails, justifying somewhat the use of the term types of polyneu- ritis in connection with them. For example, typhoid infection of the nervous system shows itself more prominently in brain symptoms and paraplegic weakness.. The same is true of typhus fever, though the paraplegic weakness and the more pronounced signs of poliomyelitis are less common. Variola is characterized more particularly by spinal manifestations along with the neuritic. Spinal symptoms are rare after scarlet fever, whereas cerebral trouble with non-nephritic neuro- retinitis is not uncommon. Many of the symptoms of influenza are undoubtedly due to a mild generalized polyneuritis which in some re- THE NON-NEUKONIC DISEASES 603 sj>ccts resembles post-diphtheritic neuritis. Mumps, dysentery, erysip- elas, pneumonia, malaria, gonorrhoea and purulent pleuritis have ail been followed by neuritis of a more or less disseminated and degener- ative type. Pure peripheral neuritis, especially of the lower limbs, has been observed symptomatically during life and microscopically after death in tuberculous patients. The rarity of true syphilitic polyneu- ritis has caused some to doubt its existence. I have seen it, however, though I am inclined to question whether it ever occurs without some implication of the brain and cord. If it be true, as Savage and others contend, that syphilis never attacks the neurones themselves, then we must look to the nutritive disturbances of the cells in the cord through the specific arteritis or to the compression of the spinal roots by the specific meningitis to explain the degeneration of the peripheral nerves in tabes, in pseudo-tabes and specific polyneuritis generally. Diabetic polyneuritis is characterized by an atrophic paralysis, but especially by the severity of its sensory symptoms. The weakness complained of in the lower extremities is mostly due to implication of the crural, obturator and peroneal nerves. The eye symptoms and visual dis- turbances in diabetics have long been recognized. I have a diabetic under observation in whom paresis of the lower extremities, optic neuritis, unilateral facial numbness, pains and paraesthesiae in various parts of the body, especially about the lips and herpetic eruptions upon the skin of the forehead, have all appeared in a remittent sort of a way. On account of the prominence of the sensory disturbances in these cases a pseudo-tabes may appear and be confused with locomotor ataxia. The prognosis of this form of neuritis is usually favorable. Of great interest, especially to tropical practitioners, are the en- demic forms of polyneuritis. We do not know much about these though it is generally accepted that they are due to an infective agent belonging to certain localities. They occur endemically and epidem- ically. Beriberi, sometimes called kakke and found especially in Japan and along the east coast of Africa, is a form of infectious polyneuritis in which the heart and blood-vessels seem to be particularly involved. The cranial nerves usually escape, except the pneumogastric. The in- tellectual and emotional centers remain untouched. Trophic skin symp- toms are exceedingly rare or entirely absent. Dropsy is a constant manifestation. In the early stages of the disease the specific gravity of the urine is always low, then it rises, and finally ends by going above tin- normal. There are no particularly abnormal elements in the mine. Tin- mortality rate under certain conditions is very high. The general infections character of the trouble is shown by the indication of spinal cord lesions in the zone-like distribution of the anaesthesia along with the polyneuritis symptoms. The old view that the eating of rice con- stituted the most important etiological factor in the disease has been seriously shaken by some recent careful observations and experiments. Some authorities believe that a form of malarial poisoning is the real agent in the production of the disease. Glogner and others claim that they have found in the blood of these patients malarial plasmodia nr similar organisms. . \katama is a form of endemic peripheral neuritis found in West 604 THE NON-NEURONIC DISEASES Central Africa, of obscure origin and characterized by numbness, prick- ling and burning sensations in the presence of cold or damp. It is found in all ages and both sexes, but more often in men than in women, in the young and middle aged than in the old, and never in children or Europeans. The symptoms are all indicative of a mild generalized neuritis. Prognosis is good as to life, but uncertain as to relief. After years of suffering the trouble sometimes ceases spontaneously. Leprous polyneuritis is an endemic, specific form of the disease. The cause of the disease is the bacillus of leprosy and the pathogenesis of the nervous manifestations is a toxic peripheral neuritis. The bacil- lus is abundantly found, as I have seen, in the diseased nerves. The neuritis seems to be of the primary interstitial type, though it is an open question whether the parenchymatous degeneration is due sec- ondarily to mechanical compression by the inflamed interstitial tissue or primarily to the poisonous products of the bacilli. The symptoms,, motor, sensory and trophic, with the sensory predominating, are all typical of a peripheral multiple neuritis. DiAGNOSis.-^-Generally the diagnosis of multiple neuritis is not difficult. A careful consideration of the etiological factors in the case, the mode of origin and general course of the disease, the character and distribution of the symptoms goes far in establishing it. The entire clinical picture exhibited by the patient and not one or two symptoms alone must be studied if mistakes are to be avoided. There are two features in the clinical picture which, when taken in conjunction, will make the diagnosis absolutely positive. In the first place, one must discover the cardinal indications of a peripheral neuritis or degenera- tion, disease of the lower neurones. These consist of flaccid motor paralysis, loss of deep reflexes, muscular atrophy, certain trophic mani- festations, electrical reaction of degeneration and sensory phenomena of a variable character. The details in regard to these symptoms have been considered in the symptomatology. In the second place, the wide distribution of these symptoms must be noted and their relatively irreg- ular severity within this wide area of distribution. A progressive quad- riplegia, involving more or less symmetrically first the ends of the lower limbs and then the upper, with cutaneous patches of hyperesthesia, anaesthesia, hyperalgesia and analgesia, is pathognomonic. If the above facts are kept well in mind, the differential diagnosis between polyneuritis and acute anterior poliomyelitis, ascending myelitis, tabes dorsalis, spinal meningitis and hemorrhage is not diffi- cult. In acute anterior poliomyelitis the age of the patient, the initial feb- rile disturbance and the course of the disease are quite different. In infantile palsy there is apt to be a recession of the symptoms from an initial wide extent of them; in polyneuritis, the trouble usually begins in a limited area and rapidly extends. In the former, especially after the symptoms have begun to recede, there is a remarkable limitation of the paralysis to individual muscles and muscle groups, whereas in the latter much larger groups or even the whole limb is gradually involved in the paralysis. In poliomyelitis the atrophy is somewhat more rapid and severe, especially in particular muscles. Moreover, the damage is THE NON-NEURONIC DISEASES 605 more permanent than it is in multiple neuritis. There are no sensory phenomena, as in the latter disease, nor are the cranial nerves ever im- plicated. The symmetrical distribution of the symptoms, their pro- gressive character, the presence of sensory phenomena, and the impli- cation of the cranial nerves establish the diagnosis of neuritis posi- tively in contradistinction to poliomyelitis. It does not follow, however, that there may not be some poliomyelitic manifestations along with the neuritic. As I have pointed out in connection with the toxic and in- fectious cases, the disease process is an extensive one and it is probable that some of the peripheral degeneration is due to damage to the cells in the gray matter of the cord. In these cases, however, there is always some primary disease of the peripheral nerves also, and on account of this the local tenderness of the nerves and muscles proves the presence of the polyneuritis. If inflammatory alterations of the optic nerve can be detected, the case is positively one of peripheral neuritis. In myelitis the symptoms are more distinctly paraplegic in char- acter, with involvement of the rectal and vesical sphincters. There is present a girdle-pain and the sensory phenomena generally tend to- wards anaesthesia. In myelitis high up in the cord the reflexes are exaggerated, the muscles more or less rigid and later on contractured. The muscular atrophy is not so distinct, whereas the cutaneous trophic symptoms are more pronounced. The electrical responses are not much altered. In spinal hemorrhage and meningitis the character of the pains and their distribution about the trunk, as well as the limbs, the local tenderness in the spine, the inclination towards opisthotonos, the in- volvement of the sphincters and the absence of cranial symptoms, make the diagnosis clear. The sensory types of multiple neuritis may easily be mistaken for tabes dorsal is, for the ataxia, the pains, the absence of the reflexes and even some of the trophic symptoms are the same in both. Pseudo-tabes is an excellent name for these neuritic cases on account of this close similarity. The differential diagnosis in a few exceptional cases is absolutely impossible. The following points, if carefully noted, will help to distinguish one disease from the other in most of the cases: A history of syphilis is obtained in most of the cases of locomotor ataxia. The existence of a true syphilitic polyneuritis is open to question. In the history of multiple neuritis toxins play the greatest role, and after them the infections of a severe and acute character. In tabes dorsalis the symptoms approach gradually and several years after the primary infection ; in pseudo-tabes the onset of the symptoms is rather abrupt, their extension is rapid, and their distribution includes generally all four extremities early and equally. The ataxia in polyneuritis is not so distinctly sensory as it is in tabes; it is a little more paralytic in character. The Argyll-Robertson pupil is not a symptom of multiple neuritis and the early bladder troubles of tabes are rarely complained of in polyneurites. Local tenderness upon pressure is not found in tabes, whereas it is all but pathognomonic in multiple neuritis. The crises, especially the gastric crises, are not symptoms of the latter. The trunkal anaesthesia of tabes is practically unknown in polyneuritis. 6o6 THE NON-NEURONIC DISEASES The progressive and incurable character of tabes is to be contrasted with the curability of polyneuritis and its tendency after a short time to get well. Treatment. — The results of the proper treatment of multiple neuritis are among the happiest that are to be attained in neurology.. A clear recognition of the origin, nature and stage of the disease is a. sine qua non, however, of its successful management. The diagnosis- having been positively established, the first thing to do is to secure the patient's entire confidence and co-operation and to impress upon him the favorable character of the prognosis if he is hopeful and submissive. It is well to explain very clearly to him that the greatest danger of a fatal issue lies in the possible implication of the cardiac and respiratory nerves, and that this is to be avoided by perfect serenity of mind and quietude of body. It is also well to prepare his mind by telling him that the disease usually increases in severity, though not necessarily in ex- tent, for the first four or five weeks, then remains stationary with now and then a possible slight relapse for a month or two longer, and finally disappears in the course of several months more or a year. The avoidance of surprises, disappointments and rebellious opposition as a result of this forewarning is to me a desideratum of much more con- sideration than is the momentary chagrin and depression on the part of the patient when he learns of the prolonged course of his disease. Most patients when thus reasonably appealed to, respond heartily to all that is about to be done for their restoration to< health. If, however, they revolt against the physician's advice, decline his further ministra- tions and undertake a line of treatment and a mode of life which may hasten a fatal termination, the physician will escape all blame in the matter. The two cardinal factors^in the management of multiple neuritis are to remove the cause and to put the patient at absolute rest. No compromise can be allowed in regard to these. In the removal of the cause both passive and active therapy may be necessary. Unless the heart is too weak, all alcoholics should be at once discontinued. In some cases the alcohol can only be diminished. The amount of the diminution can only be determined and regulated by a careful observation of the pulse in each individual case. It is not well therefore ever to suddenly deprive a patient with alcoholic poly- neuritis of all stimulus. A much safer way would be to gradually re- duce the alcohol, rapidly in some cases and more slowly in others, but always as promptly and completely as possible, and to gradually in- crease the administration, pari passu, of some other form of stimulant, like strychnia, ammonium salts, etc. If the disease is due to lead, arsenic, copper, mercury or other toxic agent, of course the source of the poisoning must be sought for and eliminated at once. The iodide of potassium will help to remove the lead from the body. Whatever the toxic substance may be its elimination from the organism should be hastened by free purgation, diuresis and diaphoresis. Free bathing is excellent for this purpose, but it must not cause too much exertion on the part of the patient. The infection that may lurk behind the multiple neuritis calls for THE NON-NEURONIC DISEASES 6oj its own appropriate therapy. Quinine must be given when there is malaria present. Antistreptococcic serum may be tried in scarlatinal, puerperal and other forms of septic polyneuritis. The salicylates are indicated in rheumatic neuritis. Autointoxication with treatment di- rected to the alimentary canal must be thought of. Endemic cases of the disease, like beriberi, must be removed from the infected locality and the latter cleaned up as far as possible with bactericides. Severe anaemia, cachexia and senility with arterio-sclerosis, with which poly- neuritis is sometimes associated, call for their own special treatment. If there is a history of syphilis preceding the neuritis, one natural- ly thinks of the administration of mercury. It is well to try the remedy, but it must be done very cautionsly. It is not positively set- tled yet whether the polyneuritis that sometimes occurs in syphilitics is due to the infection or to the heroic use of mercury, which in most of these cases will be found to have just preceded the onset of the paralysis. In my own opinion the polyneuritis is due to the syphilis and not to the mercury, reasons for which belief I will not stop here to elaborate. Nevertheless I am convinced from personal observation that the administration of mercury may render the multiple neuritis decidedly worse. Therefore, if there is no improvement, and especially if the symptoms are increased by the cautious use of mercury- in poly- neuritis in a syphilitic patient, it had better be promptly discontinued and the iodide of potash used instead. If the case is a severe one, absolute quiet, both of mind and body, is imperative. The paralysis will usually effect the physical rest; the mental calmness must be secured by seclusion and appropriate diver- sion. Hence in the early stage of the disease these patients had better be placed in a hospital where they can be kept apart from all domestic and business intrusion, or if they must remain at home, they should be given a firm and tactful nurse and isolated in some remote apartment. It is more difficult to manage in this respect the milder cases, for they will insist upon moving about some in their partially paralyzed condi- tion. They must be enjoined to keep their bed and only move for the sake of change from the bed to the couch or rolling chair. In a word, all active functioning of the nervous system must be prohibited during the first two or three weeks of the disease. Nor must there be any passive activity, such as massage, hauling of the patient about, etc., during this same period. If immobility and mental rest are accom- plished in the beginning of the disease and the cause promptly removed, it will cease that much sooner to extend and the stationary and con- valescent period will be that much earlier secured. It must never for a moment be forgotten that the cardiac and respiratory functions are liable to be affected in any case and to avoid this danger the rest must be almost absolute. The patient must not move about in bed hastily, or sit up, until all acute symptoius show signs of abating. Even then caution must be observed. In the first stage diaphoretic measures . if used carefully, may be highly advantageous. Hot baths, hot packs fostering perspiration for an hour or two daily or every other day, may be employed. The heart action must be closely watched. Hot drinks may be of some benefit in 6o8 THE NON-NEURONIC DISEASES provoking diaphoresis and diuresis, with or without the hot baths. In one or two instances I have seen the disease increased and extended by the too vigorous use of heat in this way. Cold is always contra- indicated, hence the room must be kept warm and free from draughts. The weakness and pains are two of the most prominent symptoms calling for special attention. The former is to be combated by a strengthening and nutritious diet and by the appropriate use of stimu- lants. Milk in abundance, egg-nog, meat juices, tender beef, fats and ood-liver oil are to be given as freely as the digestive organs will dis- pose of them. Diphtheritic patients may have to be fed through a tube. For the weakness it may be necessary to resort to wine, strychnia, camphor, aromatic spirits of ammonia, etc. The shooting pains and muscular tenderness are often so marked that relief, however slight, must be attempted. For this purpose the salicylate of soda, in twenty- grain doses every two or three hours, is sometimes useful. Dana says that a dram or two of the fluid extract of ergot repeated in three hours sometimes relieves the pain. The coal-tar preparations have not been very effective in my hands and I am loath to push them on account of their depressing action upon the heart. I prefer the use of warmth, such as swathing the limbs in hot flannels and in cloths wrung out in hot water. Painting the limbs with menthol and enveloping them in cotton batting is sometimes soothing. As a last resort morphine may be used. When the diminution of the pains indicates that the disease has entered upon its stationary or convalescent stage, massage, electricity and measures to counteract late contractures and deformity are to be instituted. If there are any signs of irritation remaining, galvanism in the form of a stabile current is the only form of electricity permissible. Later on the labile current, in strength of five to eight milliamperes, may be administered daily for about five minutes. The faradic current to stimulate the muscles should only be employed when convalescence has gotten well under way. Much more effective than electricity in my experience has been massage. The muscles should be gently rubbed and kneaded and stroked once or twice a day for a short time. The use of olive oil or cocoanut oil in the hand of the masseur is agreeable to the patient. The stroking should be gentle, towards the body and in the vicinity of the principal nerves affected. It may be increased in vigor as the case continues to improve. Passive exercises and mild gymnastics may later on take the place of the massage. Exhaustion* must be carefully guarded against in all these mechanical measures and the patient should be urged to lie down and rest frequently. Tonics, such as strychnia, arsenic, iron, quinine and the hvpophosphites, are to be administered. In obstinate paralysis Oppenheim recommends the subcutaneous administration of strychnia, one-sixtieth to one-twentieth grain several times daily. Iodide of potassium, two per cent, carbolic acid and carbolmorphine, the latter two hypodermically in the neighbor- hood of the affected nerves, have received high endorsement. In ad- vanced convalescence general tonic and hygienic measures, including baths, change of climate, abundance of fresh air, good food and judi- ciously guarded exercise are to be recommended. THE NON-NEURONIC DISEASES 609 Deformities, particularly in young persons, are to be prevented bv watching the attitude of the limbs during the course of the disease. When late contractures begin to manifest themselves they are to be combated by massage, passive motion, forcible replacement with the appropriate use of splints and other apparatus. Tenotomy is not de- manded here, as it often is in poliomyelitis, for the disease being more generalized opposing sets of muscles are apt to be more equally af- fected. Relapses are to be warned against and prevented by a strict ad- herence to the directions of the medical adviser. TUMORS OP THE NERVES. NEUROMATA. A neuroma in the modern sense is a nerve tumor, but not all nerve tumors are neuromata. Since the discovery of Virchow that there are specific nerve newgrowths, and newgrowths in the nervous system that are not specifically neural, neuromata have been divided into the true and the false. It is but fair to say that some pathologists do not accept the distinction thus made by Virchow. The true neuroma is very rare and consists of true nervous ele- ments. Virchow speaks of the "myelinic" and "amyelinic'' forms, those that contain respectively medullated and non-medullated fibres. Ziegler is among those who doubt the propriety of separating the neuromata into a special class and believe that the nerve-fibres are not implicated in the neoplasm. He thinks the growths are merely neurofibromata, neu- rosarcomata, neurolipomata, etc., arising in the epineurium and peri- neurium. The false neuromata are heterologous growths and are given the same names that they would have were they in other parts of the body. They include the fibromata, sarcomata, myxomata, syphilomata, etc. The first are the most common and are the basis probably of the majority of the true neuromata. There is nothing distinctive about these false neuromata that need special notice because they happen to occur in the nervous system. Neurone cell-bodies have scarcely ever been discovered in the true neuromata. Between the nerve fibres there is connective tissue which renders the growth hard and firm and which is ofttimes so abundant that the neoplasm stands midway between the true and false neuro- mata. These tumors are multiple or single. They occur mostly on tin- spinal nerves and are usually small, varying in size from a pea to a pigeon's tgg ( i cm. to 6 cm.). They are prone to appear as multiple growths in particular nerve areas. For example, the brachial plexus, the cauda equina or the nerves of the pelvis may be the seat of them. Sometimes they are strung along the nerve trunks like beads. Multiple neuromata are of the neuro- fibroma-tons variety. Tlie\ are exceedingly numerous sometimes, as many as a thousand or more having been counted in one individual. Fibroma molluscum multiplex, a general neurofibromatosis, is the condition in which multiple neuromata are found upon the trunks of the 6io THE NON-NEURONIC DISEASES subcutaneous nerves. Nsevi are generally associated with it, thus indicating the congenital nature of the trouble. Plexiform neuroma, or congenital elephantiasis, is where there is a great interlacing of cords, nodular and tortuous. The fibres of the nerves, as for example, the trigeminal, the lumbar, the brachial and solar plexuses, the nerves of the penis and mammae, thicken, break up and recombine in a twisted sort of a way. They can be felt plainly FIGURE 133. General ueurofi- bromata. Rosary-like arrangement of the neuroma along the peripheral nerves of the right arm; the small knots areplainly visible beneath the skin. (After Robert Smith, reproduced by Marie.) under the skin. The nerve-fibres are united by the hyperplastic con- nective tissue. The disease begins in foetal life and is related to pachydermatocele and elephantiasis mollis. The growth of this condi- tion is extremely slow. It exerts pressure upon the neighboring struc- tures. Sometimes multiple neuromata are malignant. Trauma and hered- ity are behind them and the greater nerve trunks are the seat of them, especially the perineurium. They are spindle-shape and sometimes are very large in size. Sarcomatous cells are often found in them. THE NON-NEURONIC DISEASES 6ll Tubercula dolorosa are small multiple tumors, mostly of the fibro- matous variety, that occur in great profusion on the subcutaneous sensory nerves. They are painful and can easily be felt. Gliomata are not frequently seen in the peripheral nerves. They occur in the auditory nerve. Carcinoma usually occurs as a secondary condition in the form of a diffuse or nodular infiltration of the nerve from a neighboring growth. Rarely it is seen as an isolated neoplasm. Syphilomata occur mostly on the cranial and spinal nerve roots ; not so often on the peripheral nerves. In lepra ancesthctica the nerves are often infiltrated, to a very great degree, causing neuro-fibromatous swellings upon them. The condi- tion is of the nature of a cirrhotic inflammation rather than a nodular development. Nerve tumors are practically always within the nerve sheath. If they are on one side, the nerve itself may pass intact around them. Frequently, however, the nerve is involved and infiltrated, with its fibres spread out over the surface of the growth. Even under these circumstances the nerve may not be much damaged, which explains the frequent absence of symptoms. False neuromata are more damaging to the nerve fibres and are therefore accompanied by more pronounced symptoms than are the true neuromata. Etiology. — The etiology of tumors has not been brought to com- pletion yet. Heredity and a neuropathic constitution are undoubtedly responsible for certain types of them, and to some degree for all of them. This is shown in their familial characteristics and their frequent association, especially the multiple neuromata, with idiocy and cretin- ism. The phthisical and scrofulous condition were thought by Virchow to be blameable for them. Curiously the multiple variety of the disease - eems to belong to men, the isolated to women. Age exercises no in- fluence upon their appearance. Even the hereditary form of the dis- ease may not appear till middle age. The cachexia: give rise to, and determine, the character of the neoplasms. Whatever underlies in the patient's constitution the forma- tion of the fibrous growths, sarcoma, carcinoma, myxoma, lipoma, tuber- cle, is responsible for the same conditions in the nervous system. Traumata and chronic neuritis may initiate the neuromata. Amputa- tion neuromata, or so-called bulbous nerves, belong here. As the nerve fibrillar, when cut, grow out from the proximal end like the twigs of .1 vine, it i> easy to understand how by mechanical obstruction and other- wise they may become twisted and agglutinated together and held h\ the connective tissue in a ma>>. Symptoms. — I have already remarked that as the nerve often courses intact around the tumor, there arc- n<> neural symptoms of its presence. ( )n the other hand, the symptoms may be most severe and even dangerous, the latter when the growth are in such nerves as the pneumogastric, phrenic or other splanchnic nerve. The symptoms are of an irritative or paralytic character and involve all neural functions, motor, sensory, trophic, vasomotor and secretory. In a word, they are the symptoms of neuralgia and neuritis. I * 6l2 THE NON-NEURONIC DISEASES Pain is a particularly common symptom. It may be a local tender- ness or a peripheral sharp, shooting pain. Often it is acute, burning, boring and darting. It follows the course of the nerve or appears in the area of its distribution. In the same area paresthesia occur and more or less hypcusthesia. The topographical correspondence of these sensory phenomena with the motor manifestations is a particularly characteristic feature. The mot or phenomena include twitchings, jerkings, spasms and tonic contractions in the muscles innervated by the diseased nerve. Paralysis and atrophy are very rare. General convulsions and reflex epilepsy have been seen ; also hysteria, neurasthenia and hypochondria- sis. The pain is the most disturbing feature, often completely wearing the patient out and reducing him physically. In general neurofibromatosis Marie has noted the occurrence of psychosis, sensory disorders and convulsions. Paraplegia and vesical paralysis sometimes are the result of neuro- mata of the cauda. Plexiform neuroma rarely produces any other symptom than a hypaesthesia. Prognosis. — Neuromata are progresssive, though they may be- come stationary or regress. Multiple neuromata offer a better general prognosis than do the isolated neoplasms. The reason is obvious. Multiple neuromata are usually fibrous. Among the isolated tumors occur the heterologous growths, such as sarcoma, cancer, etc. The presence and the severity of the symptoms add to the gravity of the prognosis. Treatment. — Except in syphilomata, when, of course, mercury and the iodides are called for, medicines are useless in tumor of the nerves. The condition is essentially a surgical one. Of course, this applies to isolated tumors that can be reached and that reveal symptoms. After extirpation of a neuroma, the possibility of complete paralysis is great. This can be obviated sometimes by an immediate suturing, transplanting or other form of operation upon the nerve stumps. Some tumors can be peeled off; as it were, from the nerve. and when thus re- moved there will be a restoration of the neural functions. This has happily occurred several times. In cases of multiple tumors some of the growths with marked symp- toms can be operated upon. Return of them is the rule, however. Electrolysis and caustics are inadvisable. There is danger of setting up neuritis and making what is bad worse. Strong galvanic currents applied percutaneously, mercury and large doses of iodides have been recommended' for true neuromata and neurofibromata. The results of the treatment, however, are not very encouraging. The terminal neuromata of tubercula dolorosa max he cut out without danger; otherwise these and all painful tumors must needs be quieted with analgesics. There is no treatment for plexiform neuroma. Excision is inad ■ visable. THE NON-NEURONIC DISEASES 613 DISEASES OF THE SPINAL NERVES. PHRENIC NERVE. Diaphragmatic paralysis is usually clue to spinal cord disease. Dis- ease that involves the third and fourth cervical roots implicates the phrenic nerve. It need hardly be mentioned that such diseases include spinal meningitis, particularly pachymeningitis, caries of the spine, fractures, dislocations, exostoses, tumors, hemorrhage and especially gummatous meningitis. Direct traumata do not often reach the nerve, because it is very deep in the neck. Wounds in the neck and tumors and aneurisms in the thorax may implicate it. Duchenne has met with disease of the nerve in lead poisoning. It has been affected bilaterally in alcoholic intoxication. Diphtheria has infected it. Trie influence of cold in setting up a phrenic neuritis should be regarded with skepticism. Paralysis of the diaphragm is, of course, the symptom of phrenic nerve disease. If one nerve is implicated, one side of the diaphragm only is paralyzed. The continued activity of the other side, however, causes the whole muscle to move so that it is often difficult to discover the unilateral paresis. In a state of quietude on the part of the patient, the loss of diaphragmatic stimulation does not materially lower the breathing ca- pacity. Exertion, however, causes quick breathing and a weakening of the voice. This dyspnoea may become very pronounced and dis- tressing. Both inspiration and expiration are affected. The movements of the thorax are usually increased, the upper part of the abdominal wall being so much drawn forward as to simulate the effect of the descent of the diaphragm. Paralysis of the diaphragm produces trouble in diagnosis only when it is unilateral. In most forms of phrenic neuritis it is unilateral. It is bilateral generally in spinal cord diseases dM(\ diseases of the spine. Careful examination will enable one to detect a unilateral paralysis. The breathing sounds at the base of the lung upon that side will be diminished. The electrical responses will be diminished. The objective diaphragmatic phenomenon will be absent. And there may be a painful spot to pressure on the scaleni muscles near the sterno-mastoid muscle. Sometimes the diaphragm is paralyzed from myositis. This may lead to an erroneous diagnosis of phrenic neuritis. It is usually a secondary trouble from peritonitis or pleuritis, on account of which it can usually be readily differentiated. The- characteristic pains of these latter diseases arc- also distinguishing. It is very doubtful if there is a true hysterical diaphragmatic paralysis. Superior COStal breathing is more common in women than in men and it is the type of respiration in nervous people and hysterics. It is easy, therefore, for a hysteric to simulate diaphragmatic paralysis. Mental distraction will cause the epigastrium to advance and thus show the absence, when a deep inspiration is unguardedly taken, of a true organic paralysis. Dyspnoea docs not occur in hysterics. Ether nar- 614 THE NON-NEURONIC DISEASES cosis would confirm the diagnosis. According- to Wernicke, there is a hysterical insufficiency of the phrenic nerve which is associated with fear and inspiratory dyspnoea. Callender has described a primary and isolated degeneration of the diaphragm after death. Whether it ever produces inaction of the muscle during life we do not know. In spinal cord disease that causes diaphragmatic paralysis, other muscles are svmptomatically affected also. The prognosis is generally favorable, though it is dependent upon the cause. Multiple neuritis as a cause offers a rather unfavorable prognosis. I have seen dyspnoea and death occur in this disease in this way. The treatment involves first the removal of the cause. Counter- irritation over the anterior triangle of the neck and electrical stimula- tion of the nerve when inflammation has subsided, about sums up the therapy. Strychnia has been especially commended in the diphtheritic paralysis. THE BRACHIAL PLEXUS. Anatomy. — The brachial plexus is formed by the union of the an- terior trunks of the four lower cervical and the greater part of the first dorsal nerves. It reaches from the lower part of the neck to the axil- lary space. Opposite the coracoid process it ends in the large nerves that go to supply the upper extremity. The fifth and sixth cervical nerves join together at the outer bor- der of the scalenus medius to form an upper trunk. The eighth cervical and first dorsal form in the same way a lower trunk. The seventh forms the middle trunk between these two. Just outside of the scaleni muscles, each primary trunk divides into an anterior and a posterior branch. The anterior branches of the upper and middle trunks unite and form the upper or outer cord of the plexus. The anterior branch of the lower trunk alone forms the lower or inner cord of the plexus. The posterior branches of all the three trunks unite to form the middle or posterior cord. The branches that come off from the brachial plexus are very numerous. Those that originate above the clavicle are the posterior thoracic and the suprascapular nerves, a nerve for the rhomboid mus- cle, another for the subclavius, branches for the scaleni and longus colli muscles and sometimes a branch to the phrenic nerve. Those that originate from the plexus below the clavicle may be arranged as fol- lows, according to Quain, whom I follow in these anatomical facts : From the upper or outer cord — the external of the two anterior thoracic nerves, the muscido-cutaneous, and the outer head of the median. From the lower or inner cord — the inner of the two anterior thoracics, the nerve of Wrisberg or small internal cutaneous, the in- ternal cutaneous, the ulnar and the inner head of the median. From the posterior cord — the subscapular nerves, the circumflex and the musculo-spiral. THE N0X-XEUR0X1C DISEASES 615 For the spinal segmental origin and the ultimate distribution of the branches of the brachial plexus, the reader is referred to the admira- ble tables compiled from Starr, Mills. Sachs, Dana and Thorburn at FIGURE 134. Diagrammatic outline of the cervical and brachial plexuses. (A. T.) 4 The nerves are separated froin the spinal cord at their origin and are supposed to be viewed from before : CI, the first cervical or sub- occipital nerve, and the It oman numbers in succes- sion from II, to VII i, the cor- responding cervical nerves ; DI, the first, and II, and III, the second and third dorsal nerves ; the origin of the posterior primary branch is shown in all the nerves ; of these p 2, indicates the great occipital from the second, and p 3, the smallest occipital nerve from the third. Cervical plexus : 1, anterior primary branch of the first cervical nerve and loop of union with the second nerve ; 2, small occipital nerve ; 3, great auricular nerve ; 3', superficial cervical nerve ; 3 n, communicating branches to the descenclcns noni from the second and third ; 3 s, communicating to the spinal accessory from the third and fourth nerves ; 4, supraclavicular nerves ; the loops or arches of com- munication between the four upper cervical nerves, and between the fourth and fifth, are shown; 4', phrenic nerve. Brachial plexus-: V, to VIII', and D\ the five roots of the brachial plexus ; 5, the rhomboid nerve ; 5', suprascapular ; 5", posterior thoracic ; 6, nerve to the subclavius muscle ; 7, 7, inner and outer anterior thoracic nerves ; 8, 8', 8", sub- scapular nerves. In the larger nerves proceeding to the shoulder and arm from the plexus, those of the anterior divisions are represented of a lighter shade, those belong- ing to the posterior division darker ; ec, external cutaneous or musculo- cutaneous ; m y median ; u, ulnar ; t'c, internal cutaneous ; w, nerve of Wrisberg ; r, musculo-spiral ; c, circumflex ; i,i, intercostal nerves ; i' } lateral branch of the same ; ih, intercosto-humeral nerve. (From Quain.) nerve the end of the chapter. In localizing the lesion in the spinal palsies, the table should be constantly consulted. Individual trunks, as well as the whole plexus, may be attacked by disease. It is practically impossible to make a clinical differentia- 6i6 THE NON-NEUROXIC DISEASES tion between diseases of the trunks of the plexus and the roots out of which they are formed. Traumatic, toxic and infectious causes are here as usual the source of the trouble. The first far outnumber the other two. Dislocations, fractures, contusions, blows about the shoulder are especially impor- tant as causes. Compressing tumors, exostoses, aneurism, arteriosclerotic dilata- tions are of the nature of mechanical traumata. An arthritis of the joint may set up a neuritis in the neighboring nerves. Tight bandages, handcuffs, lying on the arm may produce local trouble in the nerves. I will now take up in detail some of the special paralyses of the brachial plexus and its branches. Erb's Palsy. — This is a combined palsy of the shoulder and arm and is due to a lesion of the trunk of the brachial plexus formed by the fifth and sixth cervical roots. The muscles that are paralyzed are the deltoid, the biceps, the brachialis interims, supinator longus and some- times the supinator brevis, infraspinatus and subscapular. It is a fifth and sixth cervical root or primary trunk palsy. The central parts and upper cords of the plexus are damaged. It is often spoken of as the upper arm palsy. Traumata are the chief cause of it, though it has been seen as an isolated paralysis in toxic and infectious maladies. The position of the arm in which the plexus is made to compress for a long time the clavicle may cause it. Thus it occurs as an obstetrical palsy, as a result of prolonged narcosis with the arm forcibly elevated out- ward and backward. Carrying of heavy burdens on the shoulder has produced it. It has been seen to occur bilaterally. The arm hangs by the side of the body and the forearm cannot be flexed. As an obstetrical palsy Erb's type is slow to recover and when dim to injury in adults it is apt to be severe. It has all the characteristics of a peripheral palsy, such as the flaccidity of the paralysis, the atrophy. the loss of reflex and the reaction of degeneration. Klumpke's Palsy. — This is a brachial plexus paralysis due to dis- ease of the eighth cervical and first dorsal branches. As it involves the triceps, the wrist flexors and pronators, and the flexors and extensors of the hand, it is often called the lozcer arm palsy. Elevation of the arm can take place and the forearm can be flexed and supinated, but extension of the forearm is impossible and the hand is entirely paralyzed. Tumors, exostoses on the first rib, cervical rib, operative traumata, syphilitic meningitis and simple neuritis may cause this lower brachial paralysis. A total brachial paralysis may recede and leave this form as a residuum. In some cases ulnar sensory disturbances are present and even median nerve anaesthesia. If the lesion is close to the cord, involv- ing the rami communicantes to the sympathetic nerve, vasomotor and oculo pupillary phenomena may be observed. The usual treatment and nerve suture apply to these palsies. Total Plexus Palsy. — This is usually of traumatic, rarely of infectious neuritie origin. It occurs sometimes in obstetrical operations. All or parts of the nerves may he involved. Sensory as well as motor phenomena are in evidence. As the intercosto-humeral nerve often THE NON-NEURONIC DISEASES 617 innervates the inner side of the upper arm. sensation is sometimes pre- served here. Regeneration in these cases takes place, but it is slow and prolonged and usually is incomplete, thus leaving some paralysis and atrophy. The treatment is based upon general principles and along the lines indicated under the head of neuritis. The disease cannot be confounded with progressive muscular atrophy, because the sensory phenomena and reaction of degeneration are so prominent. Arthritic palsy is accompanied by the joint manifestations. Obstetrical Palsy. — This form of paralysis is of the brachial plexus type. It is due to unusual presentations and the use of the fin- ger and instruments in the axilla, directly pressing the nerves or forc- ing the shoulder and clavicle upward and backward against them. Vari- ous manoeuvres have been the cause of it and even the wrapping of the cord around the child's neck has resulted in the palsy. Duchenne was the first to describe typical obstetrical palsy. It rarely if ever occurs in normal deliveries. The whole brachial plexus, or a part of it, may be involved. Usu- ally the muscles implicated are the deltoid, biceps, brachial is in tenuis, supinator longus and brevis and infraspinatus. The paralysis is of the usual peripheral, neuritic type with flaccidity, atrophy, loss of reflex and reaction of degeneration. Sensory disturbances are prominent, though they are, of course, hard to discover in infants. The reflex response is the means that we must depend upon to indicate their presence or absence. Many of the obstetrical palsies belong to the Erb type previously described. Rarely the trouble is bilateral, though practically it is a local and unilateral palsy. The disease usually disappears slowly after many weeks or months. Permanent damage of some sort usually remains, though complete re- coveries have been seen. If there is no inflammation, early electrical treatment along lines already indicated under the head of neuritis should be employed and persisted in. SPECIAL NERVE PALSIES OF THE UPPER LIMB. POSTERIOR OR LONG THORACIC NERVE. This is not a common trouble and it occurs mostly in young male adults. The nerve may be damaged in the suprascapular region or in the axilla. Blows, heavy weights, wounds in the axilla may injure the nerve. As a rule strains and over-exertion with the arms raised above the head are the most prolific cause of it. Young gymnasts, trapeze performers and those who use their arms in this way and swing the heavy weight of the body from them are the sufferers. The right nerve is mere frequently affected than the left. It has been seen to follow infection ( diphtheria, typhoid and influenza), rheumatism and the puerperium. Verhaagen thinks it may occur as a hysterical phe- nomenon. The serratus ma gnus is the muscle that is paralyzed. The arm cannot be raised above the horizontal and the shoulder is weakened. I 618 THE NON-NEURONIC DISEASES If the arm is held out horizontally, the lower angle of the scapula stands outward from the thoracic wall. If the arm is elevated and carried for- ward the scapula swings away from the thoracic wall in such a way as to produce a deep groove between the latter and its posterior or inner edge, thus giving a wing-like appearance to it. When the arm hangs limp beside the body, the lower point of the scapula is a little clo be com- pressed. In compression paralysis the electrical reactions are not marked, if noticeable at all. In severer lesions, such as wounds, lacerations, crushes, the reaction of degeneration is generally well developed. Later in the disease the tendon sheaths are swollen and there is often hyperostosis of the metacarpal bones. Prognosis. — Most cases recover. Mild cases run a few weeks. Severe cases last many months or a year or more. The degree of the reaction of degeneration will foretell somewhat whether the case will be a long or short one. Most compression cases recover in four or six weeks ; rarely in a few days. Recently I saw a compression case (hanging the arm over the back of a chair) in a rheumatic with peripheral neuritis, recover almost completely in three or four days. Cases due to knife wounds, punctures and dislocations of the head of the humerus are not so promising. They usually exhibit signs of severe degeneration and therefore require many months to recover. A completely severed nerve offers a bad outlook unless it is immediately sutured, and then it requires many months for the restoration of its function. Ether paralysis runs several months. THE NON-NEURONIC DISEASES 62 1 Treatment. — The general lines of this have already been laid down under the head of Neuritis. Remove and avoid the cause. Nerve suturing has accomplished some very encouraging results upon the musculo-spiral nerve. In freeing the nerve from a tumor, the danger of paralysis must always be thought of. Transplantation has been suc- cessfully performed upon this nerve. After irritative symptoms have subsided there is no treatment so available as that with electricity and massage. Rubber muscles and the fixation of the hand in hyper-extension with splints and bandages may be necessary in old and severe cases. The only medicinal treatment that will be called for will be that to combat the underlying intoxication or infection. Salicylates may be needed for rheumatism, iodides for saturnism, quinine for malaria. Hypodermics of strychnine are at times efficacious. MEDIAN NERVE. An isolated paralysis of this nerve is very rare and is practically always due to trauma. The nerve supplies cutaneous branches to the entire palm and to three and a half fingers. It stimulates the pronator muscles, the flexors of the carpus and the long flexors of the fingers (except the ulnar flexor of the carpus and part of the deep flexor of the fingers). The outer set of the short thumb muscles and two lumbricales are also excited by it. The median is frequently involved with other nerves and it is then due to luxations, strangulation, as with tight bandage, fractures of the bones of the forearm and callus. Forcible muscular contraction of the pronator teres, a severe distortion of the wrist, compression of the cervical ribs and embolism of the axillary artery have been charged with median palsy. The median is usually involved in the craft-palsies, which must not be confounded with the occupation neuroses. Milkers, drummers, cigar-makers, carpet-beaters, dentists, locksmiths and all those who have to use monotonously and for prolonged periods certain muscles of the arms. The median and ulnar nerves are the most frequent seat of puerperal toxic neuritis. Disease of this nerve is revealed in a weakened grip of the hand and impairment of flexion and abduction of the thumb and flexion of the first and second fingers. There is not much change in the attitude of the hand except a slight deviation toward the ulnar side. The flexion of the basal phalanges ; s not involved. Pronation of the hand is lost. Median injuries are usually severe ones with the typical atrophy and reaction of degeneration manifestations. The sensory symptoms are particularly prominent. They consist of pains, hyperesthesia and particularly hypcesthesia in the area of distribution of the nerve. The entire area may lx* involved, including thus the volar part of the palm as Ear as the fourth metacarpal bone, the volar surface of the first three, and the radial side of the fourth finger and the dorsal surface of the index and middle fingers. < )c- 622 THE NON-NEURONIC DISEASES casionally anaesthesia is absent. It has even not been pronounced in- complete section of the nerve. Anomalous anastomoses, or rather anomalous innervations from the ulnar, have upset both the motor and sensory appearances of median disease. The trophic and vasomotor symptoms are prominent in disease of this nerve. Herpes, pemphigus-vesicles, sluggish ulcers, glossing of the skin, falling of the hair o>f the skin, striation of the nails, may be expected. The skin is bluish and cool. There is often infiltration of the subcutaneous tissue. Sometimes hyperidrosis is especially noticeable. Amidrosis may occur. The lesion determines the prognosis, and the treatment calls for no special comment. ULNAR NERVE. This nerve is frequently paralyzed, more so than the median. It stands second to the musculo-spiral in the frequency of its disease. It FIGURE 135. Showing Ark a of Anesthesia in Ulnar-Nerve Palsy CBowlby). is often affected with other nerves by traumata, such as fractures with callus, growths, and luxations. Cuts, bruises, blows all figure in the etiology. It is subject to pressure paralysis and it enters into the pathology of the professional palsies. It is not often affected in lead intoxication. It is an accompaniment of polyneuritis, syphilitic neuritis and other infectious troubles. Tumors develop upon the ulnar and it has exhibited an ascending form of neuritis. The nerve supplies cutaneous filaments to the lower part of the forearm to a small extent and the palmar and dorsal aspects of the hand in the inner half, to the little finger and to the inner or ulnar side of the ring finger. The muscles stimulated by it are the ulnar flexor of the carpus. the deep flexor of the lingers (its inner half), the short muscles of the little finger with the pal maris brevis, the inter ossci of the hand, the in- ner two lumbricales, the adductor pollicis and the inner half of the flexor brevis pollicis. It also furnishes the nerve supply to the joints of the elbow, wrist and hand. Tight closure of the hand is impossible, the weakness being of THE NON-NEURONIC DISEASES 623 course in the little and ring ringers. The terminal phalanges of the last three ringers cannot be flexed, nor can the thumb be adducted. On account of the paralysis of the interossei and lumbricales, the claw hand or the main- en- griff e appears. The fingers cannot be abducted or adducted. Atrophy, loss of reflex and the reaction of degeneration accompany this paralysis as in all similar peripheral palsies. The sensory symptoms are rarely absent in ulnar disease. There are pains, hyperesthesia and anaesthesia, sometimes all existing simul- taneously. Hypaesthesia usually occurs on the volar surface of the hand to a line which passes through the longitudinal center of the ring finger and in a corresponding area on the dorsal surface ; in the little finger and over the ulnar half of the ring finger. Sometimes the sensory disturbance has been limited entirely to the little finger. Trophic, vasomotor and secretory changes, such as are mentioned in connection with median nerve palsy, are rather common. Pressure palsies recover in a few weeks ; severer ones require months and years. FIGURE 136. Complete " claw-hand" in an old ulnar paralysis (cica- trix at A). (After Duchenne ) The treatment may involve nerve suture, neurolysis (freeing of the nerve from tumors, etc.), electricity, massage and the usual measures described at length under the head of neuritis. A primary spontaneous neuritis of symmetrical distribution is a rare condition, occurring in the ulnar nerves of persons of neuropathic constitution and supposed to be due to infection. It is very chronic and scarcely ever ends in recovery. It has followed the acute infec- tious diseases, particularly typhoid fever. There is at first pain and paresthesia in the ulnar distribution. The muscles become weak and atrophy and the characteristic claw-hand appears. Anaesthesia de- velops also. This is probably a true neuritis in which the degenerative side of the process takes precedence because the infection is not virulent enough to provoke gross inflammation, but with the aid of the neu- ropathic heredity is virulent enough to cause damage in the neurones. It is a local, not a true, primary disease, as I have explained at the head of this chapter. Morgan's Disease, or Analgesic Paralysis with Whitlow. There is neuritis of the upper extremities in this disease, but it is a complication of syringomyelia, to which the reader is referred for its more detailed description. 624 THE NON-NEURONIC DISEASES DORSAL NERVES. There are twelve pairs of dorsal nerves, the first pair entering into the formation of the brachial plexus. Except this, and the second and twelfth, which send cutaneous offsets respectively to the arm and hip, the dorsal nerves are almost entirely distributed to the walls of the thorax and abdomen. In certain respiratory troubles and neuralgias alone are they of any special clinical interest. THE LUMBAR PLEXUS. Anatomy. — The lumbar plexus is formed by connections between the anterior primary divisions of the four upper lumbar nerves. It is in the substance of the psoas muscle. It is joined to the sacral plexus by a branch from the fourth lumbar nerve to the fifth. From the first nerve spring the ilio-hypogastric and ilio-inguinal nerves and a communicating branch to the second nerve. The second nerve supplies the greater part of the genito-crural and external cutane- ous nerves and also transmits a branch to the third, from which some of the fibres of the anterior crural and obturator nerves proceed. There are two branches from the third nerve — namely, a large one forming a part of the anterior crural nerve, and a smaller forming a part of the obturator. The fourth nerve sends forth three branches. Two of these complete the obturator and anterior crural nerves, while the third con- nects with the fifth nerve and thus becomes a part of the sacral plexus. This plexus thus innervates the lower part of the abdominal wall and the fore part and inner side of the lower limb. Disease of the lumbar or sacral plexus is not as frequent as of the brachial plexus. The lumbar plexus has been damaged by neoplasms in the abdomen, psoas abscess and vertebral disease. A spontaneous crural neuritis has been seen. Very rarely is the obturator nerve alone affected, though it has been damaged in parturition. The same is to be said of the anterior crural nerve. The gouty diathesis, alcoholic intoxi- cation and diabetes mellitus have caused disease sometimes in the crural. Sometimes it has been a total bilateral paralysis. Fractures have damaged the nerves in a few instances ; but they are well pro- tected. Symptoms. — Sensory symptoms only occur when the two upper lumbar nerves are involved. These involve the abdominal wall. An- esthesia or hypccsthesia may appear in the area of distribution of the anterior and internal cutaneous femoris and of the saphenus major. This includes the anterior and inner surface of the thigh, ihe inner sur- face of the leg and the inner border of the foot nearly to the great toe. This, of course, represents disease in the two lower lumbar nerves and their branches. The motor paralysis is seen in the inability of the patient to extend the leg and flex the hip. This Is a total paralysis. If it is bilateral the gait is markedly disturbed. The pectineus and sartorius do not es- pecially cause any trouble. When the palsy is unilateral, the patient has to step carefully and not Ilex the knee. THE XOX-XLUROXIC DISEASES 62: If the lesion is outside of the pelvis or beyond the point of the giving off of the branches to the iliopsoas muscle, the extensor cruris quadriceps, the sartorius and the pectineus, the paralysis is not a total FIGURE 137. dxh -Diagrammatic out- line OP THE LUMBAR AND SACRAL PLEXUSES WITH THK PRINCIPAL NERVES AIUSINQ FROM THEM. (A. T. ) h DXII, the divided roots c. the last dorsal nerve ; LI, to V, the roots of the five lumbar »erves ; the loops uniting the -interior primary divisions of these nerves together, and the first with the twelfth dorsal, arc shown ; SI, to V, and CI, the sacral and coccygeal nerves ; p, placed on some of the nerves marks the posterior primary divisions cut short ; p, p\ the plexus formed by the union of the posterior branches of the third, fourth, and fifth sacral and the coccygeal nerves ; d, anterior division of the last dorsal nerve, from which d! the lateral cutaneous branch arises ; I, ilio-hypogastric nerve ; 1', ilio-inguinal ; 2, genito-crural ; 2', external cutaneous of the thigh ; ps, branches to the psoas muscle ; cr, anterior crural nerve; U, branches to the iliacus ; ob, obturator nerve ; of, accessory obturator ; IV, V, loop from the fourth and fifth lumbar, forming the lumbo- sacral cord ; 3, superior gluteal nerve ; sc, great sciatic nerve, continued from the sacral plexus ; 4, small sciatic nerve, rising from the plexus poste- riorly ; 4', inferior gluteal nerve ; 5, inferior pudendal ; 5', poste- rior cutaneous of the thigh and leg ; 6, 6, branch to the obtu- rator internus and gemellus su- perior ; 6', 6', branch to the gemellus inferior, quadratus femoris and hip-joint ; 7, twigs to the pyriformis ; 8, pudic nerve ; 9, visceral branches ; 9', twig to the levator ani ; 10, perforating cutaneous nerve ; II, coccygeal branches. (From Quain.) plexus one and hence is incomplete and more limited. In such c severe pain is caused in the region supplied l>v the nerve that is irritated. This occurs sometimes early and is often due to a neoplasm near the 626 THE NON-NEURONIC DISEASES spine. The pains follow the course of the crural and saphenus nerves. The paralysis is of the ordinary neuritic type with atrophy, reaction of degeneration, loss of reflexes and is entirely flaccid. OBTURATOR NERVE. This nerve rarely suffers alone. It has, however, been so affected in tumors and difficult labors. Adduction of the thigh is impossible. The patient cannot throw one leg across the other. There is also some impairment in both external and internal rotation. There are sensory disturbances on the median upper third of the thigh. ANTERIOR CRURAL NERVE. Here there is weakness in the muscles on the front of the thigh. The leg cannot be normally extended. Anaesthesia and pain occur in the crural area. External Cutaneous Nerve. — Only lately has an isolated paralysis of this nerve been noted. There were pains, anaesthesia, parcesthesia and other sensory disorders in the area supplied by this nerve. Walk- ing and standing seemed to bring on the symptoms. Superior Gluteal Nerve. — Gowers mentions that this nerve is very rarely diseased in the isolated form. The gluteus medius and minimus are paralyzed. There is loss of abduction and circumduction of the thigh. THE SACRAL PLEXUS. Anatomy. — It should be remembered that the anterior divisions of the first four sacral nerves make their exit from the spinal canal through the anterior sacral foramina, while the fifth passes out between the sacrum and coccyx. The first three and a part of the fourth enter into the composition of the sacral plexus. The fifth does not form a part of the plexus, but ends on the back of the coccyx. That part of the fourth that does not enter as a constituent of the plexus supplies branches to the viscera and muscles of the pelvis and a connecting branch to the fifth nerve. Sometimes the sixth sacral nerve, or anterior branch of the coccygeal nerve, in its union by connecting filaments with the fifth and fourth sacral nerves, is said to constitute a coccygeal plexus. The first three and part of the fourth sacral nerves then form the sacral plexus. Without much interlacement these unite to form a large upper and a small lower cord or band. The upper represents the union of the lumbosacral cord with the first and second and the greater part of the third sacral nerves. It is continued into the great sciatic nerve. The lower band has a plexiform arrangement and represents the union of the smaller part of the third sacral nerve with the plexus portion of the fourth. It is prolonged into the pudic nerve. The sacral plexus lies on the anterior surface of the pyriformis muscle opposite the side of the sacrum. It escapes through the great sacro-sciatic foramen and ends at the lower border of the pyriformis. In addition to the great sciatic and pudic nerves, it gives off a THE NON-NEURONIC DISEASES 627 number of collateral branches, such as the superior gluteal, which oc- cupies an intermediate position between the two plexuses, lumbar and sacral, the inferior gluteal, the small sciatic, the perforating cutaneous and branches to the pyrifonnis, obturator interims, ge nielli and quad- rat us f em or is muscles. Diseases of the sacral plexus usually mean the sciatic nerve and its branches. Pelvic fractures during parturition, either natural or artifi- cial, fractures of the lumbar vertebrae, are a source of disease in this nerve. The motor manifestations most commonly implicate the per- oneal branch. This is especially the case where the pelvis is contracted and the pressure of the head is severe and prolonged. The peraneal nerve arises from the lumbar sacral cord mostly, which lies on the in- nominate crest and not the pyriform muscle, as the rest of the plexus does. Its fibrillar are thought to be especially sensitive and susceptible to injury, as shown by its early degeneration in disease and the early functionless condition of its muscles after death. Among other causes of sacral disease are to be mentioned pelvic tumors, parametritis and intrapelvic septic processes, subcutaneous injections of ether, mercury, etc., nerve-stretching, fractures and dislocations of the femur. SCIATICA. This is sometimes called neuralgia ischiadic a or malum cotunnii. it is a painful condition of the sciatic nerve and its branches, due in my opinion, to neuritis, though many authors regard it as a pure neuralgia. As stated by the authorities, there are two forms of sciatica, the primary and the secondary. By secondary sciatica is meant pain in the sciatic nerve provoked by some extra-neural cause, as for instance a tumor pressing upon the nerve. It would be well if we could drop the term secondary sciatica in this sense entirely, as the name of a dis- ease of the sciatic nerve ; for it represents no more an affection of that nerve than would the pain caused by a corn on the foot represent an affection of the nerve involved. The very symptom-complex of so- called secondary sciatica is different from that of the true or primary form. In the former the pain is a referred pain, usually peripheral in location, and is not elicited by direct pressure or by the usual manoeu- vres which elicit the pain in the latter. Strictly speaking, there is only one form of sciatica, disease of the nerve itself. This disease, it is sometimes said, may be functional <>r organic, and hence true sciatica has been divided into idiopathic neuralgia of the nerve, and neuritis. Idiopathic functional sciatic neu- ralgia is the term employed to cover all the so-called secondary neural- gias and all painful states of the sciatic nerve unaccompanied by any known or observable changes in the nerve itself or its central connec- tions. Such names as gastralgia. cardialgia. cephalalgia, myalgia, neu- ralgia, and a host of others that have become engrafted upon the science of medicine, are the contributions of practical medicine ; and while they may be very comforting to employ in certain obscure cases, their care- less and indiscriminate application to well recognized symptom-groups and pathological conditions is an unwarrantable looseness in the use 628 THE NON-NEURONIC DISEASES of language which awakens a suspicion of ignorance or of indolence. Idiopathic neuralgia and simple neuritis are constantly being con- founded. Though many cases of so-called neuralgia are clearly in- stances of neuritis, it is much more convenient in the hurry of gen- eral practice to call cases of painful nerves by the undeniable but meaningless name of neuralgia than it is to study the electrical reac- tions and other symptoms characteristic of neuritis. Dana found, after a close study of the subject, that true idiopathic neuralgia is a rare disease, constituting not more than two or three per cent, of all the different forms of nervous disorders. Symptomatic and reflex neu- ralgias are of course far more common. These statistics confirm those of Classin, of Kiel, who collected 434 cases. On account of the generally favorable termination of sciatica, the opportunity for the direct examination of the diseased nerve rarely presents itself. In those cases in which it has been examined, how- ever, the findings have almost invariably been those of a neuritis or perineuritis ; redness with swelling of the nerve-sheath, small hemor- rhages in recent cases, spindle-shaped intumescence, dilated arteries, serous transudations, migration of the leucocytes, proliferation of the connective tissue, adhesions and secondary damage to the nerve-fibres. In so great a majority of the cases have these signs of inflammation been seen that one is easily justified in suspecting that all cases of pri- mary sciatica are cases of sciatic neuritis. This suspicion is moreover confirmed by a careful consideration of the etiology, symptoms, and therapeutics of sciatica. Among the considerable number of cases that have come under my observation, I do not recall one in which I was not satisfied that the nerve itself was more or less inflamed. Etiology. — The etiology of sciatica, as given in the text-books, is remarkably like that of simple neuritis. In one of the recent works sciatica is said to be the result of traumatism, exposure, gout, rheuma- tism, syphilis, or malaria. Lawson declares that nine-tenths of the cases of sciatica are due to exposure to cold, while Erb insists upon the preeminence of this above all other causes. Many authors, as, for instance, Gowers, Tyson, Osier, Anstie, and Weir Mitchell, speak of syphilis as an infrequent cause of sciatica. They doubtless refer to sciatic neuritis; for just like intracranial syphilis, which so frequently affects the nerves of the ocular muscles without causing any inflam- mation in these nerves, so syphilis may cause a secondary sciatica or pain in the nerve by a neoplastic pressure, without giving rise to a primary sciatic neuritis except in rare cases. Infectious diseases, such as typhoid fever, variola, diphtheria, tuberculosis, intoxicants such as alcohol and nicotine, diabetes, and other toxaemic conditions, may all predispose to an attack of sciatica, as well as to a general neuritis ; but exposure to cold and damp, and direct injury of the nerve, are the chief factors that start the attack. Among the traumatic causes may be mentioned contusions and wounds in the perineum, buttocks, pelvis, by falls, pressure, slow, difficult parturition and hard fecal masses. Perimetritis may cause it. The character and location of these causes will determine the unilaterality or bilaterality of the sciatica. It is a noteworthy fact, when considered in this connection, that THE NON-NEURONIC DISEASES 629 heredity and predisposition are of less importance in the etiology of sciatica than they are in the neuralgia of other nerves. In persons suffering from varicose veins, Quenu found the veins in the interior of the sciatic nerves greatly dilated. Of sixty-seven individuals with varicose veins eleven were the victims of sciatica. A case of my own was a German woman, fifty-two years of age, with varicose veins of the right leg, who suffered at rare intervals with slight attacks of pain along the course of the corresponding sciatic nerve. An elastic stocking and the internal administration of hamame- lis afforded her prompt and marked relief. Men are more frequently afflicted w T ith sciatica than women, though this is probably due to the difference between the sexes in re- gard to the incidence of the many other causes of the disease. This is shown by the fact that it is a disease of middle life, being rare in ex- treme old age and practically unknown in childhood. Pathology and Pathogenesis. — This is the same as any form of peripheral neuritis. It is true that in some cases there are no find- ings. It is probable that in such cases the changes have been very slight and evanescent, or the central representation of the nerve may have been functionally at fault. These constitute a small minority of cases and I am of the opinion that if in every case we could examine microscopically the entire nerve we would find more often than we now do positive signs of neuritis. Symptoms. — These begin gradually and steadily increase to a cli- max in the course of a few days or weeks. It is oftener the rule than the exception for the inflammatory process in neuritis to be more in- tense at particular points in the course of the nerve. Indeed, a large number of these cases might well be called focal neuritis, on account of the higher degree of the inflammation at these points or foci. The pain is usually sharper there on account of the greater damage and severer pressure upon the nervi nervorum. And yet what are these tender points of neuritis but the classical puncta dolorosa of neuralgia! These puncta are described as being usually situated in the neighbor- hood of bony prominences. Yalleix, who was the first to draw atten- tion to them, said they would be found to correspond with the poste- rior superior spine of the ilium, the spine of the ischium where the nerve makes its escape from the pelvic cavity, the great trochanter of the femur behind which the nerve runs near the border of the gluteus maximus, the head of the fibula, the internal malleolus, and the dorsum of the foot. In sciatic neuritis it is easy enough to explain the tenderness of the nerve-trunk by means of the inflammatory irritation and pressure upon the nervi nervorum; but in so-called idiopathic neuralgia, in which there is supposed to be no perceptible lesion of the nerve-trunk, it is difficult to comprehend how such exquisite local pain is to be accounted for. Idiopathic neuralgic pain is not localized in the nerve- trunk, but rather in the peripheral distribution of the nerve and its branches. At all events it would not be so keen in the nerve-trunk as it is in neuritis. Every one knows, however, that the local pain in 630 THE NON-NEURONIC DISEASES sciatica is usually so severe that the patient dreads to be touched, and finds sitting down often a most distressing act. Pathology has not yet discovered any change in the trunk of a nerve attacked with so-called idiopathic neuralgia. The change, whatever it may be, is either at the peripheral or central end of the nerve. There are no sensory cells in the course of the nerve itself or its constituent fibers whereby painful impressions are received and transmitted to the brain. And yet neuralgia is one of the most painful of all affections, and the pain, as in sciatica, is even more intolerable in the nerve-trunk than it is at the periphery. Those who deny the inflammatory char- acter of these painful sciaticas have no easy task before them to ex- plain the immediate cause of the pain. In opposition to all the teach- ings of physiology, they must show how a relatively inert nerve-fiber, capable merely of transmitting different impulses, can, when unaf- fected by any perceptible pressure or irritation within or outside of its own substance, give rise to the most excruciating agony. To say that this pain is functional in character, and therefore does not nee 'I any perceptible change in the nerve structure to account for it, only enhances the difficulty, for functional pains are always referred to the extremity of the nerve and its branches and never directly to the nerve-trunk. And again, to say that the nervi nervorum found in the course of the nerve-trunk are the peripheral sources of this so-called idiopathic neuralgia as functional pain, needs a further explanation as to how these nervi nervorum come to be the sources of this pain in the absence of any pressure or damage to them. Does it not seem singu- lar that this mysterious, imperceptible agent of idiopathic neuralgia can thus arouse to the highest degree the function of a sensory nerve and yet not at the same time affect the motor strands? The motor nerves along the course of their trunks are like the sensory nerves ; yet motor spasm, pure and simple, is not a symptom of idiopathic neuralgia. In typical neuritis there are associated with the pain along the course of the nerve-trunk peculiar sensations, paresthesia, and various degrees of motor paresis in the parts supplied by the nerve and its branches. In fact, the combination of pain in the course of the nerve- trunk with various spontaneous irritative motor and sensory phenomena at its distal extremity, is almost pathognomonic of an inflammation of the nerve. The pressure and irritation of the nervi nervorum and re- current fibers sufficiently explain the former, while interference with the power of conduction by the same pressure readily accounts for the latter. And yet some of the authorities still assert that in simple neuralgia of the sciatic nerve there occur various paresthesia?, tonic and clonic spasms in the leg, and even at times a considerable degree of paralysis. In other words, when the nerve is apparently quite un- changed histologically, it is affected functionally in such a mysterious way as to give rise to a paralysis and symptoms of irritation at the same time. The argument itself is a reductio ad absurd inn. The nerve, it is maintained, is so sensitive that the condition is deemed worthy of the dignity of a special name, neuralgia ; while at the same time the nerve is so dead in function as to fail in the proper conduc- THE X0X-XEUR0X1C DISEASES 63 I tion of received impressions. Usually, irritation is the cause of mus- cular spasm ; here, however, irritation is made more frequently to ac- company paralysis. There is a conflict here between physiology and pathology which those who insist upon the non-inflammatory char- acter of idiopathic sciatica will do well to explain. The pain of sciatica is a localized pain, rarely diffuse, and is bor- ing, tearing, and burning in character. It is often so localized that the patient can almost feel the entire nerve like a cord passing down the back of his leg. Like all inflammatory pains, it varies in intensity and is increased by movement and pressure. Walking, straining, coughing, prolonged sitting increase it. It may be excited by the finger in the rectum or vagina. Placing the patient on his back and attempting extreme flexion of the legs, on the body is an excellent diagnostic manoeuvre, as it often cannot be done on account of the ex- treme pain produced. Sometimes the skin is red and slightly oedema- tous, and the local temperature may be somewhat elevated. The earli- est indications of involvement of the nerve-fibers are the peripheral par&sthesia and hyperesthesia for the sensory, and fibrillary twitch- ings and tonic contractions of the muscles for the motor. The reflexes are somewhat increased at first, but diminished in old cases. The electric excitability is at first enhanced, and then more or less lost. In subacute and chronic cases there will be some reaction of degenera- tion. Trophic disturbances sometimes observed include muscular atrophy, herpes, excessive perspiration, and even joint affections. In simple neuritis, posture is at times almost as pathognomonic as is pain upon movement. See illustration where the patient shows in her comparative attitude, the painful and weakened condition of the left leg. The faulty attitude of the body, so characteristic in sciatica, is an old and well recognized symptom. Sometimes the patient leans toward the painful side, sometimes away from it ; but always in a direction to modify the severity of the pain. By the manoeuver the nerve is probably lifted away from certain prominences in the pelvis against which it usually rests. When there is scoliosis of the lumbar column, the concavity is usually towards the sound side. Various ex- planations of this have been given, none of which are entirely satis- factory. Many think it is due to the effort to relieve pressure on the sensory nerve and its branches ; others think it is due to a muscular weakness: still others hold that it is dependent upon the muscular spasm and contracture. Occasionally kyphosis may be seen. Neuralgia of other nerves generally occurs under verv different conditions from those under which neuralgia of the sciatic usually takes place. The victims of such neuralgias often suffer from migraine. pains in some of the special nerves of the head and of the viscera, which pains are remarkably disposed to become periodic. Headaches an 1 visceral pains are quite infrequent among patients suffering from pri- mary simple sciatica. DIAGNOSIS. — Sciatica is more often diagnosed in practice than it actually occurs. Either from the obscurity of the • the lack o\ care in the examination, various lesions of the pelvis, the hip-joint, the 632 THE NON-NEURONIC DISEASES spine and elsewhere, involving the sciatic nerve and causing it to be painful, pass under the name of sciatica. No disease demands a more earnest effort at differential diagno- sis. Its clinical picture is characteristic enough and there is no ex- FIGURE 138. FIGURE 139. Subacute sciatic neuritis. Dr. Fitch's case. The left side is the arfected one, as shown by the difference in the attitude, when resting upon one or the other leg. cuse, except in the rarest instances, for sonic of the gross blunders that are sometimes made. The onset and location of the pain, its variation under certain characteristic manceuvers ; the puncta dolorosa and general tenderness along the course of the nerve, the bodily atti- THE NON-NEURONIC DISEASES 633 tude and disorders of motility, the etiology and course of the entire affection are distinctive. As I am convinced that all forms of genuine peripheral sciatica are neuritic in nature, it is unnecessary to attempt a differential diag- nosis between what is called sciatic neuralgia and sciatic neuritis. The sensory phenomena, the atrophy, the reaction of degeneration, the altered reflexes are the same in both but differ very widely in degree in different cases. In some instances they appear only slightly and then not until late in the disease. These are the chronic cases. Coxalgia from tuberculosis or other disease of the hip- joint is characterized by the peculiar attitude of the leg and the referred pains in the knee-joint. The sciatic signs are wanting. Hysteria, hysterical sciatica, hemorrhoids, arteriosclerosis, arteritis obliterans, varicose veins, various vasomotor spasmodic conditions, need but to be mentioned as causes of pains in the legs that may simu- late sciatic neuritis. None but a tyro in medicine, or one who is superficial in his examinations, would mistake these conditions with their distinctive subjective and objective exhibitions for a true dis- ease of the sciatic nerve. A bursitis, following gonorrhoea, malaria or trauma may cause an achillodynia, or pain at the insertion of the tendo Achilles, upon walking and standing. Inflammation of the calf-muscles, a myositis, may be the cause of the pain in some cases. The other distinctive signs and history of sciatica will be absent. The gouty diathesis, faulty footwear, excessive use of the feet in standing, neurasthenia, may provoke pain in and about the fourth metatarso-phalangeal articulation. This is Morton's metatarsal gia and is usually relieved when the cause is discovered. Prognosis. — The underlying diathesis and predisposition may de- termine the course and prognosis of sciatica. Acute cases due to acute causes usually get well in a few weeks. Chronic cases sometimes run their course with more or less periodic exacerbations. Remissions and even intermissions occur. Rheumatic cases are usually favorable. Very old cases, senile cases and those due to unchangeable causes can- not hope for more than occasional amelioration, with or without treat- ment. Permanent lameness occurs in some. Treatment. — In the management of sciatica the methods that afford the highest degree of success are not only different from those employed in the treatment of other neuralgias, but they are more or less identical with those that prove to be most efficacious in the man- agement of neuritis. Counter-irritation and the actual cautery even, the internal administration of vasomotor sedatives, the free use of cathartics, diuretics, and diaphoretics, are the chief measures depended upon in the subacute and acute stage. Analgesics, narcotics, and sen- sory depressants produce less permanent results in sciatica than they do in other forms of neuralgia, doubtless because they do not affect the organic changes underlying the trouble. According to Pliny, the victims of sciatica in his day had their pains assuaged by the soft tones of the flute. From this ancient musical 634 THE NON-NEURON IC DISEASES method to the modern hypnotic it is not such a far leap. Pain may become a habit even after the organic cause of the pain has disap- peared, a habit which often needs only moral and mental treatment for its relief. In such cases, and they are usually long-standing ones, electricity, baths, moderate exercise, and general hygienic treatment will do much. Most of these cases were undoubtedly neuralgias. Drugs are useful, though secondary in the treatment of sciatica. In the acute stage, general and absolute rest, even to the point of strap- ping the limb if necessary, is a sine qua non. To paralyze the sensi- bility and depress the excited circulation, opium and its alkaloids, acon- ite, belladonna, antipyrin, phenacetin, acetanilid, are all most useful. So many cases are associated with the rheumatic and gouty diatheses that I find myself making large use of the salicylates and colchicum, and with general good results. The deep injection of ether and chloroform, though useful sometimes, has not proved uniformly so in my hands. In old cases, nerve-stretching may be done surgically, or less radically by placing the patient on his back and flexing the legs on the body as far as possible. Sometimes this has broken up a long- standing sciatica, possibly doing it by breaking up small, old adhesions and restoring the circulation. After the trouble has become chronic and all acute signs of in- flammation have disappeared, I resort to the steady use of strychnine, preferably by the subcutaneous method as recommended by Leyden, in doses of from 1-70 to 1-20 of a grain twice a day. Massage, baths, and the steady use of mild currents of electricity should be made every day. For the general principles of treatment see the section on neuritis. Peroneal or External Popliteal Nerve. — It is a curious fact that in all sciatic diseases, the symptoms of the peroneal are more prominent than of other branches. As an isolated paralysis the peroneal is far more frequently affected than the tibialis posticus. In my own opinion there is a developmental, biological reason behind this, though as yet we cannot determine just what it is. It is one of the curious facts of nature that goes with such exhibitions as the localization of syphilitic damage in special neurones in tabes, and the causation of a thyroid-like disease, acromegaly, out of a hypophyseal lesion. Note the frequency of its disease in acute anterior poliomyelitis in this connection. The nerve may be injured by fracture of the fibula and callus. by lacerations in running and making missteps, and by forcible exten- sion of the leg. It has been badly compressed by a tourniquet, by sleeping cross-legged, by wearing of stilts and by prolonged stooping in certain occupations, like potato-picking. The nerve supplies the tibialis anticus, the long extensor of the toes, the short extensor and the peronei. Consequently, the foot drops; it cannot be flexed at the ankle. Ultimately talipes equinus develops. The first phalanges of the toes cannot be extended. The unopposed interossei cause persistent flexion of the first phalanges. This leads to the pied-en- griife appearance. Walking, of course, is difficult and the toes drag on and scrape the floor. The dorsum of the foot and outer half of the leg is apt to be ancesthetic. The paralysis is of the degenerative type with atrophy, loss of re- THE N0N -NEURONIC DISEASES 635 flexes, reaction of degeneration. It is therefore flaccid. Anomalies occur in both the motor and sensory phenomena. Thus the tibialis amicus may escape the palsy and the anaesthesia may not limit itself strictly to the peroneal area. Trophic and vasomotor symptoms occur. Tibialis Posticus, or Internal Pop! ileal. Here the popliteus, calf muscles, tibialis posticus and long flexors of the toes are involved. The plantar muscles are implicated. The ankle joint cannot be extended and the flexed leg cannot be rotated inward. Talipes calcaneus is the deformity that ultimately results. The patient cannot raise himself on his toes. The sole of the foot and the lower haif of the outer part of the back of the leg is anesthetic. Plantar Nerves. — These rarely are diseased alone. Disease of the internal causes anaesthesia on the inner part of the sole and the plantar surface of the three inner toes and half of the fourth. The short flexor ■of the toes is paralyzed, the plantar muscles of the great toe except the adductor and the two inner lumbricales. Disease of the external reveals itself in anaesthesia in the outer half of the sole, the little toe and outer half of the fourth ; paralysis of the flexor accessorius, of the muscles of the little toe, all the interossei, the two outer lumbricales and the adductor of the hallux. As the toes gradually become flexed persistently at the last two joints, and extended at the others, walking is badly interfered with and the appearance is that of the pied-en- griffe. The prognosis and treatment of all of these neuritic troubles of the lower limbs follow the same line that they do in the similar troubles in the upper limb. For the general principles, the reader is referred to the section headed neuritis. 6 3 6 THE NON-NEURONIC DISEASES The following table is taken from Dana's work and is a compilation from Starr, Mills, Sachs, Thorburn, Dana himself and others. Muscles of Tongue, Palate, and Pharynx. Diseases in Name of Normal Symptoms of Defi- Innervated Represented which Muscle is Muscle. Function. cient Action. by in commonly In- volved. Genio- Pushes tongue Tongue when pro- The twelfth Medulla. t glossus. to opposite truded deviates to nerve (hy- side. paralyzed side. poglossal). S t yloglos- Raises tongue Tongue cannot be The twelfth Medulla. Bulbar pal- .sus. backward and moved backward nerve. sies (acute upward. or hollowed out (action deficient in many healthy subjects). and chron- ic) ; in spe- cific and tu- • b e r c ulous. L i ngual All movements When lying in The twelfth Medulla. diseases o f muscle of the tongue mouth deviation nerve. base ; dys- proper. itself. to healthy side ; when protruded deviates to para- lyzed side; if one troph ies. (rare). or both halves are atrophied tongue looks shrivelled. A zy go s Shortening of Uvula deviates to- Probably Medulla. As above. uvulae uvula. ward sound side ; if both sides are paralyzed there are nasal tone and regurgitation through nose pharyngeal plexus; seventh nerve (?). Name of Normal Symptoms of Defi- Innervated Represented Diseases in which Muscle is Muscle. Function. cient Action. by in commonly In- volved. Levator Raises the ve- Arch cannot b e As above. Medulla. As above ; see palati. lum palati. raised in the in- tonation of 4t ah;" if paralysis is bi- lateral flapping of arch and regurgi- tation of food also seventh- nerve affec- tions. through nose. Palato- Prevent food Regurgitation o f The fi f t h ]P Basilar affec- pharyn- from passing food ; nasal nerve tions. geal mus- toward up- speech. cles. per part of pharynx and post er i o r nares. Stylo- pha- Helps to draw Imperfect degluti- Glosso - pha- Medulla. Bulbar affec- ryngeus. larynx up- tion ; food gets ryngeal. tions and dis- ward so as to into windpipe. eases of the be closed by base. epiglottis and overtopped by tongue. Cons tric- Help to push Food is swallowed P h a ryngeal Medulla Diseases of the tors of food into gul- very imperfectly ; plexus. base (bulbar). pharynx. let. sticks in throat. Laryngeal muscles. Movements of Hoarseness and Recurrent Medulla B ul bar trou- vocal cords in difficulty in la ryngeal bles (similar respiration breathing; laryn- nerve ex- symptoms and in articu- goscopy examina- cepting the may be caused lation. tion reveals false crico - 1 h y- by tumors and position of vocal roid m u s- foreign bodies cords (see special cle. in larynx). text-books) THE NON-NEURON F<: DISEASES r W Muscles of Head and Neck. | Diseases in Name of Normal Symptoms of Defi- Innervated Represented which Muscle is Muscle. Function. cient Action. by » commonly In- volved. Sterno- Raises and Inability to raise 1 Spinal ac- Medulla and In bulbar and cleido- turns face to head from bed. or cessory. second and cervical - co r d mastoid. opposite side; other horizoutal third cer- affections ; in head inclines position, if both vical seg- later stages of to same side; muscles are affect- ments. progressive if both mus- ed; if one muscle muscular cles act Con- is affected, no atrophies; jointly head marked change of occasionally is brought position, unless in neuritis. forward.' opposite muscle is contractured ; spasm of muscle frequent; head in- clined to one side. "Rectus To flex head. Cannot flex bead ' L i Upper cervi- I cal. Upper c e r- capitis so as to bring vical seg- a n t icus chin on chest. ments, major. Rectus To flex head. capitis amicus minor. Diseases o f the cervical Rectus Slight rota- Deficient rota- region (niy- capitis tion. tion scarcely ■ elitis, men- lateralis. noticeable, un- less sterno-clei- i n g i t i s , tumor; pro- gressive do-mastoids are diseased. wasting o f Scaleni Elevate ribs- Deficient inspi- Lower cervi- Lower cervi- muscles) anterior when verte- ratory m o v e- cal nerves. c a 1 seg- medi- bral column ments. ments. u s, e t is fixed ; aid poste- in inspira- rior. tion ; slight lateral flex- ion. Flexion of ver- Imperfect flexion Lower cervi- Lon gus tebral col- of upper spine. J l cal nerves. J colli. umn. 638 THE NON-NEURONIC DISEASES Muscles of Shoulders and Upper Extremity. Name of Muscle. T rapezius 1. Cla- vicular portion (respi- ratory; o-u t e r third of c 1 a v i c le to occipi- tal bone) 2.Middle portion (from acromion and outer spine o f scapula to ligament. n u c h m and up- per dorsal spines v. 3. Lower portion and ad- ductor R h o m boids. Normal Function. Levator a n g ul i scapulae. Serratus magnus. Pulls head backward; ro- tates slightly toward side of muscle, so that chin is turned to op- posite side; contraction of both clavicu- lar portions bends head backward ; slight eleva- tion of shoul- ders; aids in deep inspira- tion. Raises shoul- der-blad e ; elevation o f acromion (clavicle goes along). Adduction o f scapula to- ward median line. Oblique move- ment of scap- ula from be- low, upward and inward, so that infe- rior angle is brought near- er the medi- an line; hold spinal margin of scapula down to tho- rax. Draws superi- or inner angle of scapula up- ward; aids in shrugging of shoulders. Rotation of shoulder- blade out- ward, and slight eleva- tion of acro- mion; holds inner margin of scapula to thorax ;brings arm from hor- izontal to ver- tical position. Symptoms of Defi- cient Action. Innervated by Deficient back- ward movement of head; not maiked as a rule because deep muscles perform this function ; shoulder does not move during in- spiration. Acromion depress- ed by weight of upper extremity ; inner upper an- gle may be pulled upward by levator anguli scapulae; internal lower an- gle is nearer to median line. Margin of scapula is about ten cm. distant, instead of being five or six cm. distant from median line ; loss of adductor may be covered up by action o f rhom- boids ; rounding of back. Deep groove be- tween inner mar- gin of scapula and thorax; if serra- tus is normal, this groove disap- pears if arm is ex- tended forward ; shoulder blade cannot be approx iinated to median line. (According to Duchenue this can be effected by upper portion of latissimus dorsi.) Isolated paralysis rare. Scapula pulle<.r\ip- ward; lower inner angle nearer the median line; arm cannot be raised above horizontal position; if arm is stretched forward scapula is re- moved from tho- rax (''winged scapula 1 '); dur- ing abduction of arm, scapula i? moved nearer to median line, and crowds trapezius and rhomboids forward. Spinal acces- sory Spinal acces- sory nerve. Represented Medulla and second and third cervi- c al seg- ments. As above. Diseases in which Muscle is^ commonly In- volved. Progressive- m u sc u 1 a r wasting ; dis- eases of me- dulla and up- per cervical cord ; clavic- ular portion least frequent- ly involved. As above. Spinal acces- sory nerve. Fifth cervi- cal. Third and fifth cervi- cal nerves. Posterior thoracic nerve. Medulla and second and third cervi- c a 1 seg- ments. Fourth and fifth cervi- cal seg- ments. Second and fourth (?) cervical segments. Fifth and sixth cervi- c a 1 seg- ments. As above. Dystrophies and cervical Progressive muscular atro- phies ( d y s- trophies); neu- ritis of part of the brachial plexus ; after traumatic in- juries to shoul- der : in cervi- cal-cord affec- tions. THE XOX-XEUROXTC DISEASES 639 Muscles of Shoulders and Upper Extremity. — Continued. Name of Muscle. Deltoid (three di- visions). lufraspi-") natus. ! Teres f minor, j Subscap- u'aris. Supras p 1 natus. Latissimus dorsi. Teres ma- jor. Pectoral is major. Normal Function. To raise arm to horizontal position, and forward, out- ward, or back- ward ; move- ments possi- ble only i f scapula is fixed by ac- tion of serra- tus and tra- pezius. Rotator hu- meri posticus ( Duchenne ) ; rotate arm outward. Rotator hume- ri a n/t i c u s (Duchenne) ; rotates arm inward Helps to steady shoul- der-joint and to elevate arm forward and outward ; outer antdeof scapula is de- pressed. Pulls the arm when raised, downward and back- ward; if arm is at rest up- p e r portion brings scap- ula nearer the median line ; united action of up- per third of both muscles causes exten- sion of dorsal trunk ; single action causes lateral move- mentof trunk. Rotates raised humerus in- ward; adduc- tion of arm to thorax; slight elevation o f shoulder. Clavicular portion d e - presses hu- merus from raised posi- tion to hori- zontal ; ad- duction of arm, as in giving a hless- ing: sternal portion de- presses arm com pletely, and if arm is at rest draws acromion for- wsrd and backward. Symptoms of Defi- cient Action. Innervated by Represented Can raise shoulder but not arm; shoulder flattened (atrophy) ; groove between acromion and head of hu- merus ; each di- vision of deltoid may be paralyzed singly. Arm cannot be moved outward. Difficulty in writ- ing (Duchenne). Arm cannot be moved inward ; scapula is rubbed against ribs. According to Du- chenne, humerus is separated still farther from acro- mion, if supra- spinatus is affect- ed in addition to deltoid. Arm cannot b e moved backward : insufficient exten- sion of dorsal spine; trunk can- not be moved lat- erally. Circumflex. Very few symp- toms; action sup- plied b y other muscles. Imperfect adduc- tion of arm ; pa- ralysis can be dis- covered best by extending arms and trying to press volar sur- faces against each other. Suprascapu- Fourth cer- As above, lar vical. Supra-] scapular. | Circum- flex. Subscapu- lar nerve. Fourth, fifth, and sixth cervical segments. Diseases in which Muscle is commonly In- volved. As above ; also in Erb's form of obstetrical paralysis. Fourth, fifth, and sixth ce r v i c a 1 segments. As in case of deltoid. Subscapu- lar, also branches of dorsal and 1 u m b a r I nerves pass- ing through muscle. Sixth and As in progres- seventh cer- vical. Subscapular. Anterior thoracic. sive atrophies and dystro- phies; in cer- vico- dorsal lesions; in neuritis. Seventh cer- vical. Fifth, sixth, aud seventh cervical. As above. Amyotrophies and dystro- phies, chiefly; also in lesions of brachial plexus. 640 THE NON-NEURON I C DISEASES Muscles of Arm, Forearm, and Hand. Diseases in Name of Normal Symptoms of Defi- Innervated Represented which Muscle is Muscle. Function. cient Action. by in commonly In- volved. Triceps.... Exteuds fore- Arm cannot be ex- Musculo- Sixth, sev- 1 Poliomyelitis arm; long tended except by its own weignt; if spiral. enth, eighth cervical seg- and other head of tri- affections of ceps, and cor- long head of tri- ments. cervical aco-brachialis ceps is affected subluxation f cord ; trau- help to keep matic inju- head f hu- head of humerus ries ; amyo- merus in posi- occurs easily. y t r op h i es tion. and dystro- Biceps Flexion and Flexion deficient, Musculo -cu- Fourth, fifth, phies (tri- supination of but can be carried taneous. sixth cervi- ceps es- forearm. out in part by other muscles. cal. c a p es in many pe r i phe r al . palsies.) Supin a 1 r Flexes fore- Flexion and prona- Muscu lo - Fourth, fifth As above ; in- longus. arm and aids tion deficient; spiral. cervical. volved in pe- in pronation. muscle does not stand out promi- nently if arm is flexed and a t- ripheral neu ■ ritis (traumat- ic), nob in lead palsy. tempt is made by another to extend it forcibly ; if mus- cle is atrophied arm i s spinule- shaped. Supinator Supinates Deficient supina- Muse ulo- Fifth cervi- Diseases as brevis. hand when forearm is ex- tended. tion of hand. spiral. cal. above ; also i n peripheral palsies. Extensor Extension Wrist cannot be Muse u 1 - Seventh cer- As before ; es- carpi ra- and abduc- flexed dorsally spiral. vical. peciall y in dialis tion of wrist; (extended) or ab- neuritis. longus et che shorter ducted ; flattening brevis. muscle has pure exten- s i n action only. Extension and of forearm. Extensor Wrist cannot be As above. Seventh cer- As above. carpi ul- abduction of flexed dorsally or vical. naris. wrist. adducted; "drop- wrist " is charac- teristic of paraly- sis of extensors. Extensor 1 First phalanges Musculo- Seventh cer- As above. d i g i t - cannot be ex- spiral. vical. rum com- Extension of tended nor fingers munis. | first pha- abducted ; grasp Extensor y langesofall is weak because indicis. fingers and flexor muscles are E x t e n sor abduction. shortened and minimi cannot contract digiti. Flexor 1 Flexion of forcibly. Deficient flexion. Median Eighth cer- As above. carpi wrist and pro- vical. radial is. nation. Flexor Flexion of Flexion and supi- nation impaired. Ulnar Eighth cer- As above. carpi wrist and vical. ulnaris. Palmaris supination. Flexion of Flexion impaired; Median Eighth cer- As above. longus. wrist only. no a n m a 1 ous position of hand from paralysis of vical. wrist ashand falls by its own weight; the flexors f fingers may act as substitutes. THE NON-NEURONIC DISEASES Muscles of Arm, Forearm, and Hand. — Continued. 641 Diseases in Name of Normal Symptoms of De- Innervated Represented which Muscle is Muscle. Function. ficient Action. by in commonly In- vol /ed . Flexor Flexes second Second phalanx cannot be flexed. Median Eighth cer- As above. d i k i to- phalanx to- vical. rum sub- ward first. lines. Flexor Flexes last Last two phalanges Ulnar and Eighth cer- As above; d i g i to- two phalanges cannot be flexed. Median. vical. muscle should rum pro- toward first. be tested with fundus. special care i n cases f traumatic in- juries. Interos s e i Abduction and Fingers cannot be Ulnar, which Eighth cer- As above ; oft- and lum- adduction of abducted, or ad- also sup- vical, first en the first bricales. fingers if first ducted; inter- plies third dorsal. muscles to be phalanges are osseous spaces and fourth affected in extended; are very marked ; 1 u m b r i- progressive flexion of first "Main en griffe " cales; medi- spinal atro- phalanges due to extension an supplies phies. and s i m u 1- of first phalanges first two and taneous e x - and flexion f so m e times tension of sec- Second and third third lum- ond and third phalanges. bricales. phalanges. Thenar Extends first Impairment of ex- Musculo- First dorsal. As before; muscles : phalanx and tension and ad- spiral. more espe- E x t e n sor abducts meta- duction ; flatten- cially in amyo- trophies and pol lici s brevis. carpal bone ; ing of ball of acts with ad- thumb. neuritis. ductor polli- cis longus. Extensor Extends both Deficient extension Musculo- First dorsal. As above. poll icis longus. phalanges of and adduction ; spiral. thumb ; also second phalanx is adduction of flexed toward metacarpal first. bone and backward movement of thumb. A b d uctor Abduction of Deficient abduc- M u scu lo- Fir6t dorsal. As above. poll icis longus. metacarpal tion of metacar- spiral. bone ; aids in pal bone ; if this flexion of muscle and ex- hand. tensor pollicis brevis are par- alyzed adduction results. Abductor Musculo- pollicis brevis. spiral. Opponens pollicis and outer Opposition of thumb. No opposition movement. Median. First dorsal. As above. portion of the flexor brevis. Abductor Flex first pha- No flexion; if mus- Median and As above. pol 1 icis b r e vi 8 ; lanx and ex- cles are paralyzed ulnar. tend second and atrophied, flexor phalanx (like ape hand is brevisand i n terossei), formed. adductor. also have an abduction and adduc- tion action. Flexor Flexes end No flexion of end Median. As above. pollicis phalanx. phalanx. longus. 642 THE NON-NEURONIC DISEASES Muscles of Back and Lower Extremities. Name of Muscle. Erector spinse; sa- cro-lumbalis; lon- gissimus dorsi. Abdominal muscles. Quadratus lumbo- rum. Adductor muscles. Sartorius. Quadriceps femoris. Ilio-psoas. Tensor fasciae lata?. External rotators: Pyriformis . Gemelli. Quadratus femoris. Internal obturator External obturator ■Gluteal muscles. Biceps ; semitendi- nosus and semi- membranosus. Gastrocnemius (al- so plantarius and soleus). Anterior tibial mus- cles (tibialis anti- cus, extensor digi- torum, and exten- sor pollicis lon- gus). Peroneus longus. Posterior tibial mus- cle. Peroneus brevis. I n terossei pedis et lumbricales Adductor; flexor brevis and abduc- tor hallucis. Innervated by Dorsal nerves. Second to twelfth dorsal segments, Dorsal nerves. Second to twelfth dorsal. Lumbar nerves. Obturator nerve, great sciatic and crural. Crural. Third lumbar segment. Crural. Third lumbar. Crural (lumbar plexus). Fourth lumbar. Superior gluteal. Fourth lumbar. Sacral plexus (muscular branches). Fifth lum- bar. Obturator nerve (lum- bar plexus). Inferior gluteal (sacral plexus). First and sec- ond sacral. I Gluteal superior. First [ and second sacral. Sciatic. Fifth lumbar segment. Symptoms of Deficient Action. Internal popliteal, lumbar. Fifth Anterior tibial. Fifth lumbar and first sacral. Peroneal. First and sec- ond sacral segments. Posterior tibial nerve. First and second seg- ments. Peroneal. First and sec- ond segments. Posterior tibial. First and second segments. Posterior tibial. First and second segments. Lordosis of iower spine; perpendicular line from shoulder falls behind os sa- crum : unilateral palsy causes deflection of spine toward sound side. Lordosis with protrusion of nates and abdomen; other actions deficient; can- not straighten up from recumbent position without assistance of hands. Lateral movements of lower vertebra imperfect. No adduction; thigh rolls outward. Flexion impaired; acts imperfectly. Leg cannot be extended; to test it ask patient, who is lying down with hip bent, to stretch out the leg, when pa- tient is sitting down to extend leg. ] Flexion difficult: in bed thigh cannot be J- flexed: difficulty rising from horizon- tal position. Deficient outward rotation inward. leg turned No extension of thigh: great difficulty in climbing; no abduction of thigh; wad- dling gait, exaggerated movement of pelvis. Deficient flexion; action of quadriceps may cause excessive extension ; in standing thigh is flexed to excess ; trunk moved backward. Deficient flexion of foot ; heel cannot be raised ; cannot stand on tiptoes. Deficient extension; " dropfoot," toes scrape floor ; to clear this, excessive flexion at knee and hip ; contracture of flexors and pes equinus or equinovarus. Deficient abduction ; plantar arch les- sened; increased by contracture. Flat- foot ; walking tiresome. I Deficient abduction or adduction , de- f fortuities result from deficiencies. Abduction and adduction of toes defi- cient ; paralysis of interossei ; hyp^rex- tension of first phalanges ; second and third flexed (clawed foot). Deficient flexion of toes ; foot cannot be pushed off ground easily. THE NON-NEURONIC DISEASES 643 DISEASES OF THE CRANIAL NERVES. The olfactory and optic nerves, so-called, are parts of the brain and will be discussed in another part of the book. They are not nerves in the sense of being mere bundles of neuraxones remote from their ganglionic centers. They are part and parcel of the great intra- cranial ganglionic centers and tracts. The olfactory bulb is actually a cerebral lobe, and the optic nerves are pure cerebral tracts. The other cranial nerves are identical in structure with the spinal nerves. They spring from motor and sensory roots. The former have their cells bunched together in nuclei which are the mere forward ex- tension or homologous representation of the anterior horns of the cord. The latter have their cells collected in the ganglia (Gasserian, jugular, petrosal, geniculate, etc.) found on the roots of the sensory nerves. Like the spinal sensory nerves, the processes from these root- ganglion cells divide T-like, the peripheral end going out to the skin and special end-organs and the central end penetrating the medulla to terminate in arborizations in nuclei which are the homologues of the gelatinous substance of Rolando of the posterior horns of the cord. In one respect, however, the cranial nerves differ from the spinal nerves and thus obtain all their confusing arrangement. It is a mere non-essential anatomical difference, which is the result of the working of the great biological law of adaptation and in no way changes the physiology or physiological anatomy of the cranial nerves. The motor and cranial strands do not keep so rigidly apart and regularly arranged serially among the cranial nerves as they do among the spinal. The third, fourth, motor branch of the fifth, sixth, seventh, and twelfth are pure motor nerves. Their sensory correspondents are wrapped up together in the great trigeminal sensory nerve. This is shown by the study of the nuclei of origin of all these nerves. The ninth, tenth and accssory part of the eleventh belong to the visceral system and spring from nuclei on their roots while the motor parts originate in central gray matter that is homologous with the vesi- cular column of Clarke. The spinal part of the eleventh nerve is a pure spinal nerve. In some lower animals the twelfth, or hypoglossal nerve, is outside of the cranium entirely and in every respect simu- lates a spinal motor nerve. The important point in these biological observations to the prac- tical clinician is that diseases of these cranial nerves differ in no re- spect whatever from diseases of the spinal nerves. The essential differences in their semeiology depend entirely upon the organs and structures which they innervate. Disease of those thai go to the heart muscle will naturally awaken more significant manifestations than those that go to the face muscles. The symptoms that arise from dis- ease- of those that terminate in the ear will naturally be different from the symptoms of disease in those that end in the taste bulbs of the tongue. But the same holds true with the spinal nerves. From all points of view, therefore, the cranial nerves must be regarded in the same light as the spinal nerv 644 THE XOX-XEUROXIC DISEASES FIGURE 140. -A. Semibiagrammatic view of a deep dissection of the cranial nerves on the left side of the head (from various authors and from nature). B. Explan- atory outline of the same. (A.T.) \ The Roman numerals from I to XII indicate the roots of the several cranial nerves as they lie in or near their foramina of exit ; V, is upon the large root of the fifth with the Gasserian ganglion in front ; C I, the suboccipital or first cervical nerve ; C VIII, the eighth. The branches of the nerves are marked as follows, viz. : — 1, supraorbital branch of the fifth ; 2, lachrymal passing into the gland ; 3, nasal pausing towards the anterior internal orbital canal, and giving the long root to the ciliary ganglion, 4' ; 3', termination of the nasal nerve ; 4, lower branch of the third nerve ; 5, superior maxillary division of the fifth passing into the infraorbital canal ; 5', the same issuing at the infraorbital foramen and being distributed as inferior palpebral, lateral nasal, and superior labial nerves, 5" ; 6, ganglion of Meckel and Vidian nerve passing back from it ; 6', palatine and other nerves descending from it ; 6", large superficial petrosal nerve ; 7, posterior dental nerves ; 7', placed in the antrum, which has been opened, points to the anterior dental nerve ; 8, inferior maxillary division of the fifth immediately below the foramen ovale ; 8', some of the muscular branches coming from it ; 8-f , the auriculo-temporal branch cut short, and above it the small superficial petrosal nerve connected with the facial ; 9, buccal and ex- ternal pterygoid ; 10, lingual or gustatory nerve ; 10', its distribution to the side and front of the tongue and to the sublingual gland ; 10", submaxillary ganglion ; below 10, the chorda tympani passing forwards from the facial above 12, to join the lingual nerve ; 11, ( From Quain. ) THE NON-NEURON1C DISEASES 645 FIGURE 140. inferior dental nerve ; 11', the same nerve and part of its dental distribution, exposed by - removal of the jaw ; 11", its termination as the mental nerve ; 11"', its raylo-hyoid branch ; 12, twigs of the facial nerve immediately after its exit from the stylo-mastoid foramen. B. 19" to the posterior belly of the digastric and to the stylo-hyoid muscle ; 1ST, temporo-facial division of the facial ; 12", c # ervico- facial division ; 13, trunk of the glosso-pharyngeal passing round the stylo-pharyngeus muscle after giving pharyngeal and muscular branches ; 1H', its distribution on the side and back part of the tongue ; 14, spinal accessory nerve ; 14', the same after having passed through the sterno-mastoid muscle uniting with branches from the cervical nerves ; 15, hypoglossal nerve ; 15', its twig 10 the tbyro-hyoid muscle ; 1")", its distribution to the muscles of the tongue ; 16, its descending branch giving a direct offset to the anterior belly of the omo-hyoid muscle, and receiving the communi- cating branches 16 + , from the cervical nerves ; 1 7, pneumo-gastric nerve ; 17', its superior laryngeal branch ; 17", external laryngeal twig ; 18, superior cervical ganglion of the sym- pathetic nerve, uniting with the upper cervical nerves, and giving at 18' the superficial cardiac nerve ; 19, the trunk of the sympathetic ; 19', the middle cervical ganglion, uniting with some of the cervical nerves, and giving 19", the large or middle cardiac nerve ; 20, continuation of the sympathetic nerve down the neck ; 21, great occipital nerve ; 22, third occipital. (From Quain.) I 646 THE XOX-XEUROXIC DISEASES I will now discuss some of the more prominent diseases of them, thus continuing the discussion of the peripheral nerve diseases, part of which I have taken up in the previous section upon the spinal nerves. The diseases that involve the cranial nuclei and which produce ofttimes clinical pictures that cannot be sharply differentiated from the clinical pictures presented by disease in the respective nerve-trunks, will be considered among the affections of the medulla. In this I am following the same general plan as evidenced in the separation of the spinal nerve diseases from those of the gray matter of the cord. There is no logical congruity in separating, for instance, focal poliomyelitis from peripheral neuritis and throwing together ophthalmoplegia and peripheral ocular nerve palsies. OCULOMOTOR OR THIRD CRANIAL NERVE. This nerve, like the fourth and sixth nerves, arises from the nucleus in the floor of the aqueduct of Sylvius. It appears in the inter- peduncular space, passes forward to enter the orbit by the inner end of the sphenoidal fissure, and supplies all the muscles of the eyeball, ex- cept the superior oblique and the external rectus. Through the lenticu- lar ganglion it also supplies the sphincter muscle of the iris and the ciliary muscle. The elevator of the eyelid is also furnished with a branch. Etiology. — A general diathesis often underlies ocular palsy and the trouble is thus but a side manifestation of another, more extensive disease. With such an underlying diathesis, as, for instance, rheuma- tism, cold can play a very important part in initiating the local trouble. Sometimes the rheumatic, unilateral ophthalmoplegia has merely re- sulted from the extension of the disease from a focus somewhere in the neighborhood. Infection, especially diphtheritic infection, is responsible in some cases. The resulting neuritis may affect the whole or part of the nerve. The muscles of accommodation are particularly liable to be paralyzed. The sixth nerve often, the fourth rarely, is included in this form of palsy. It is not possible at times to distinguish this neuritis from nu- clear disease due to the same cause. Influenza, acute rheumatism, scar- latina, pneumonia, are some other forms of infection that have given rise to ophthalmoplegia. Tumors, thrombosis of the cavernous sinus and neighboring exostoses are in the etiology. Gummatous tumors and other syphilitic conditions at the base of the brain are all well known causes of ocular palsy. There is also a primary gummatous neuritis of the nerve itself. Toxccmia, such as occurs in alcoholism, lead-poisoning, and eating of tainted food (oysters, fish, meat, bologna) has been responsible in a few instances. In diabetes paralysis of the ciliary muscles and disturbances of accommodation are of common observation. The latter has been attributed to intestinal auto-intoxica- tion (Panas). Mydriasis was also present. Most of these cases, if not all of them, are better attributed to nuclear disease. Intracranial traumata need merely to be mentioned to be recog- nized as possible causes. Bone lesions in the orbit must not be over- THE XOX-XEUROXIC DISEASES 647 looked. Fractures, aneurisms, tumors, exostoses, hemorrhages, ex- udates, are all liable to injure the nerve. Obstetrical (forceps) palsy, through pressure, has been seen in the oculomotor. Pressure of a damaging sort may be exercised other- wise also upon the nerve. In tabes, multiple sclerosis, and other general and intracranial diseases, oculomotor neuritis is uncommon. Symptoms. — Remembering the muscles supplied by the third cranial nerve, it is an easy matter to correlate the disease with the abnormal movements of the lid, eyeball and iris. Ptosis is a prominent symptom. The lid hangs down almost to the lower lid. The eye is not absolutely closed and the lid can be slightly raised because the fibres of the occipito-frontalis muscle ex- ercise some effect upon it. Place the hand upon the brow or get the patient to fix the occipito-frontalis and it will be very noticeable that he cannot move the lid a particle. As the internal rectus is innervated by the third nerve and the external by the sixth the ball of the eye cannot be rolled inward or made to follow the finger towards the nose. Only outward can the ball be moved. Contracture of the external rectus ultimately causes a downward, outward strabismus. Other movements of the ball are impossible. The iris remains a trifle larger than normal. It cannot contract. It dilates more rapidly than usual under atropine. Neither light nor convergence contracts it. Diplopia, slight exophthalmos and consequent disturbance of vis- ion result from the displacement of the line of fixation and relaxation of the affected muscles. Pains may accompany the rheumatic and compression form of the trouble. The above picture represents a total paralysis of the nerve. This usually is the case in peripheral disease, though not always so. In nuclear disease there is more frequently a separation of the internal from the external ophthalmoplegia. A bilateral palsy of the sphincter iridis and ciliary muscle, or a bilateral external palsy with retention of accommodation is almost pathognomonic of central disease. Per- ipheral disease may be inferred when neighboring cranial nerves are affected. Though it is impossible of actual demonstration we assume that the paralysis is atrophic and flaccid, as in all peripheral palsies. There is a recurrent or periodical ocular palsy that should re- ceive some mention here. Cases have been seen by Saundby, Snell, Gubler, Mobius, Senator, Buzzard, Pfliiger, Charcot, Manz, ( )ppen- heim and others. Every few days, weeks or months the eyelid falls and remains down for a variable time. The trouble is mostly unilateral. With the paralysis there has been headache, pain in the eyeballs, nausea and vomiting. The disease is supposed to be related to migraine, though it is not strictly identical with it. It may last a week. The whole nerve is usually involved. Albuminuria has accompanied it. Both 648 THE NON-NEURONIC DISEASES sexes are subject to it and it has been seen in children. Female adults are more frequently its victims. It is a mysterious trouble and noth- ing is known of its cause. The most plausible hypothesis is that of Charcot who likens the disease to hemicrania. Prognosis. — This is entirely dependent upon the cause. Infec- tious cases as a rule recover. Properly treated syphilitic cases offer most favorable prognoses. Less favorable, but still not absolutely bad, are the traumatic cases. If the nerve is not too severely damaged and the cause can be removed the outlook is good. Inoperable brain tumors of course render the accompanying ocular palsy hopeless. Tabetic ocular palsies usually recede, though not always. The prognosis of an isolated iridoplegia is grave because it may be the advance sign of tabes or general paresis. It occurs, however, in alcoholism and syph- ilitic cerebral disease and under treatment may recover. Treatment. — First, remove the cause as promptly and completely as possible. If rheumatism, syphilis, influenza, diphtheria or other infection is present it must be attacked with the usual medicines. Sweating must be encouraged. General antiphlogistic treatment, with local vesication and bloodletting, must be instituted. Later on electrical treatment may be tried, though it is not very effective. Galvanism is the best current to use, with the cathode on the back of the neck and the anode directly over the eye. The electrodes may be placed up on the opposite temples. The seance should be only three or four minutes and the current just strong enough to contract the facial muscles. Faradism should never be used. Hypodermics of strychnine are often of much benefit. Prisms and protectors over the affected eye may give relief from the double vision. Ophthalmic surgery has been resorted to for overcoming the ptosis. TROCHLEAR, PATHETICUS, OR FOURTH CRANIAL NERVE. This is the smallest of the cranial nerves and yet has a long course. It springs from the nucleus in the floor of the aqueduct of Sylvius. It emerges from just beneath the corpora quadrigemina, curves outward around the superior peduncle of the cerebellum and the outer side of the crus cerebri, passes forward in the outer wall of the cavernous sinus and into the orbit by way of the sphenoidal fissure, and ultimately termi- nates in the end-plates of the superior oblique muscle. Paralysis of this muscle causes the eye to turn slightly inward when looking down. There may be diplopia in the lower half of the visual field. It is noticeable when the patient descends a flight of steps, all of which seem to be double. THE ABDUCENS, OR SIXTH CRANIAL NERVE. This nerve also originates in the nucleus in the floor of the aque- duct of Sylvius. It supplies the external rectus of the eyeball and thus is appropriately considered in connection with the previous two nerves. THE NON-NEURONIC DISEASES 649 It is, of all the cranial nerves, the one most frequently diseased. This is largely on account of its long, exposed course at the base of the brain. Emerging just behind the pons Varolii above the pyramids, it passes forward close to the floor of the cavernous sinus, enters the orbit through the sphenoidal fissure and terminates in the external rectus.. Its paralysis results in internal strabismus and limitation of ex- ternal movements of the eyeball. Diplopia is present and may be homogeneous. The head is usually turned towards the side of the paralyzed muscle. Gummatous disease particularly attacks the abdu- cens nerve. TRIFACIAL, TRIGEMINAL, OR FIFTH CRANIAL NERVE. This is a combined motor and sensory nerve. The motor root springs from a nucleus in the floor of the fourth ventricle and the Sylvan aqueduct. The sensory root, which represents the central processes of the neurones whose cell-bodies are in the Gasserian ganglion lying on the petrous part of the temporal bone, is traceable under the superior cerebellar peduncle in the pons to the nucleus of Rolando in the medulla. Starting from the sides of the pons Varolii, nearer its upper than its lower border, the small or motor root lies at first above the large or sensory one. Both branches then pass forward to the middle fossa of the skull, enter a recess of the dura over the summit of the petrous bone, where the ganglion in the sensory portion of the nerve is found. The motor trunk passes beneath the ganglion to join the lowest of the three branches of the ganglion just below the foramen ovale. The three branches that project from the Gasserian ganglion are the ophthalmic, which enters the orbit and emerges in the terminal supra-orbital nerve that runs up on the forehead ; the superior maxillary, which is continued foiward to emerge from the supra-maxillary foramen between the orbit and the mouth ; and the inferior maxillary which is distributed to the external ear, the tongue, the lower teeth and by the branch that emerges from the infra-maxillary foramen in the lower jaw\ The motor branch terminates in the muscles of mastication, namely, the temporal, the massetcr and the two pteragoids. This is the great sensory nerve of the face, probably the most important sensory nerve in the body. It imparts common sensibility to the face, the fore part of the head, the eye, the nose, the ear and the mouth, including the greater portion of the tongue. To the ante- rior two-thirds of the latter it is believed to confer the power of taste, the taste fibres coming really from the glossopharyngeal by way of the facial and the chorda tympani. In the orbital cavity, fibres from the sympathetic nerve run along beside the ramus ophthalmicus of the fifth nerve and innervate the dilator muscle of the pupil, after emerging from the ciliary ganglion. To the second branch of the fifth is attached the spheno-palatine gan- glion which is connected with the geniculate ganglion of the facial nerve by the Vidian, or large superficial petrosal nerve. To the third branch is attached the otic ganglion, which also is 650 THE N0N-NEUR0NIC DISEASES connected with the geniculate ganglion of the facial by the small super- ficial petrosal nerve. This last nerve sends a branch, Jacobson's tym- panic nerve, to the glosso-pharyngeal. The innervations of the trigeminal nerve have lately been seen to be very variable. Not often does isolated disease of this nerve occur. The nature of its neuralgic manifestations have been described under the head of neuralgia elsewhere. Etiology. — Neighboring bone disease, caries, exostoses, tumors, chronic meningitis and syphilitic disease have damaged the nerve. I have lately seen a case of gummatous neuritis in it. Traumata and infection, especially rheumatism, have been responsible for its disease in FIGURE 141. Course of the facial nerve and its anastojnoses with the trigeminal and glossopharyngeal nerve* (After Leube.) some cases. Surgical operatiojis in and about the orbit and maxillary bones are to be included sometimes in the etiology. I have seen the nerve manifest paralytic s) mptoms in cases of ear disease. As a col- lateral trouble in brain tumor, hemorrhage, pontile encephalomalacia, tabes and syringomyelia, disease of the trifacial is not an uncommon event. Symptoms. — The symptoms depend upon the character and loca- tion of the lesion. If one remembers the physiological anatomy of the nerve, the symptoms may easily be inferred. Motor, sensory and trophic manifestations occur in total paralysis of the nerve. The jaw cannot be closed, nor moved laterally as it is slightly, in chewing food. The anaesthesia involves the face in front of a line passing transversely across the top of the head, down through the external auditory meatuses and along the lower edge of the lower jaw. The accompanying diagram, with figures corresponding with the branches of the nerve, will show its cutaneous distribution better THE XOX-XEUROXIC DISEASES 6;i than a long description. In this area should be included the mucous membrane of the nose, mouth, tongue, gums and inside of the cheek ; also the conjunctiva and the cornea. On account of the insensitive- ness of the cornea, winking is not performed as systematically as it should be and it becomes irritated from minute particles of matter. The secretion of tears being impaired adds to this misfortune. Vision is blurred slightly. Smell and taste are blunted on account of the dry ■condition of the mucous membrane of the nose and mouth. Often the cheek is bitten and lacerated internally in the process of chewing. We do not know yet just exactly the nature of the role played by the trigeminal nerve in the matter of taste. Many believe that the fibres of the third branch which subserve the function of taste in the anterior two-thirds of the tongue are wholly from the glosso-pharyn- FIGURE 142. Cutaneous area of the head supplied by the trigeminal nerve. geal. albeit they have come by way of the facial and chorda tympani nerves. This being so, the glosso-pharyngeal nerve is the nerve of taste for the whole tongue. However, it is true that taste depends largely upon the ordinary sensory and trophic condition of the mu- cous membrane, and in maintaining this in a normal state, the essential fibre> of the trifacial nerve, those that come from the Gasserian gan- glion, subserve in part the function of taste. At all events gustatory disturbances occur in the anterior two-thirds of the tongue when the fifth nerve or its inferior maxillary or lingual branches are injured. Herpes is a common trigeminal symptom. Indeed the trophic symptoms of disease of this nerve or its branches are among the most important. In neuroparalytic keratitis, the vision is badly blurred: the cornea ulcerates; perforation takes place; the whole eye inflames (panophthalmitis). This is a pure trophic trouble and not due solely to anaesthesia as was formerly thought, ft is not neuroparalytic lint 652 THE NON-NEURONIC DISEASES neuritic in origin. Ulceration also occurs, as a trophic symptom, in the cheek and nose. The teeth loosen and fall out, though this is due more to central disease. Some authorities believe that progressive facial hemiatrophy is a trophic disease of the trigeminal nucleus. As in all neuritic palsies these are flaccid in type, the temporal, masseter and pteragoid muscles atrophy and when they can be exam- ined the electrical reaction of degeneration is found. The jaw re- flex, of course, is absent. In unilateral palsy the immobility of the jaw is not so noticeable. If the patient is asked to press the teeth- to- gether and then still more compress the jaws, it will be noticed that the muscles do not contract on the paralyzed side. Instead of signs of paralysis there may be signs of irritation. Parcesthesice then are in the lead and pains and excessive secretion. There may be a facial spasm and, through reflex irritation, coughing, sneezing, vertigo, etc. The prognosis of course depends upon the nature and location of the lesion. If the cause of the inflammation or irritation can be re- moved, recovery may be expected. Treatment. — The cause must be removed and to a large extent this exhausts the treatment. Syphilis is so prolific a cause that antispe- cific treatment should be persevered in, even if immediate results are not forthcoming. I have had good results from such a mode of pro- cedure. The eyes should always be protected. For pains, analgesics and electrotherapy are our only resources. Surgery may accomplish much if the indications are clear enough. It is questionable whether the risk of producing trifacial neuritis, as is possible in the injection of perosmic acid, will be warranted by the suffering sometimes caused in pure trifacial neuralgia. The latter is fearful at times, but the former has destroyed vision and caused facial atrophy, paralysis and other severe manifestations. THE FACIAL, OR SEVENTH CRANIAL NERVE. This is a pure motor nerve, whose nucleus is in the floor of the fourth ventricle beneath the superior fovea. It appears at the upper part of the medulla in the groove between the olivary and restiform bodies, close to the lower edge of the pons. Outside of it is the audi- tory nerve with a strand, the pars intermedia of Wrisberg, arising in a nucleus beneath the inferior fovea and connecting with the auditory. The facial, auditory, and pars intermedia all enter the internal auditory meatus together. At the bottom of the meatus, however, the facial parts company from the others and enters the aqueduct of Fallopius, following the windings of the canal through the temporal bone to the stylo-mastoid foramen. In the canal it gives off two important branch- es, the chorda tympani, which seems to be a taste nerve, and unites with the lingual branch of the fifth to innervate the anterior two-thirds of the tongue, and the branch to the stapedius muscle. After its emergence from the skull, the main trunk of the nerve passes down- wards and forwards through the parotid gland and terminates by dividing just behind the ramus of the jaw into the temporo-faciat THE NON-NEURONIC DISEASES 653 above and the temporo-ccrvical below, each of which sends numerous branches to the side of the head, the face and the upper part of the neck. As these branches inter-communicate freely, they form a sort of a plexus which is often called the pes anserinus. As the muscles to which these filaments go, namely, the buccinator and all those of the face except the muscles of mastication, play so prominent a role in •expression, the facial is sometimes called the "artist's nerve." To briefly recapitulate, the facial nerve is distributed to the mus- cles of the seal p. of the external ear, nose, mouth, eyelids (excepting the levator palpebral superioris) and to the platysma. It also supplies the muscles of the tympanum, the levator palati and azygos uvulae FIGURE 143. — The facial nerve ii its canal, with its con- NECTING branches, &c (from Sappey, after Hirschfeld and Leveille). 3 The mastoid and a part of the petrous bone have been divided nearly vertically, and the canal of the facial nerve opened in its whole extent from the internal meatus to the stylo-mastoid fora- men ; the Vidian canal has also been opened from the outside : 1, facial nerve in the first horizontal part of its course ; 2, its second part turning backwards ; 3, its vertical portion ; 4, the nerve at its exit from the stylo-mastoid foramen ; 5, geniculate ganglion ; 6, large superficial petrosal nerve ; 7, sphenopalatine ganglion ; 8, small superficial petrosal nerve ; 9, chorda tympani ; 10, posterior auricular branch cut short ; 11, branch to the digastric muscle ; 12, branch to the stylo-hyoid muscle ; 13, twig uniting with the glosso-pharyngeal nerve (Hand 15). < through the large superficial petrosal) and the stylo-hyoid and the posterior belly of the digastric. It is well to remember its wide inter-connection with other nerves. It is thus associated with the fifth nerve, with the spheno-maxillan , submaxillary and otic ganglia, with the auditory, the glosso-pharvn- geal and pneumogastric nerves (through the auricular branch of the latter ) and with parts of the sympathetic and spinal nerve-. Prosopoplegia. or facial palsy, is the most common of the nerve paralyses. We are here only concerned with the peripheral type. though the disease may be of central and cortical origin. ETIOLOGY. — Exposure to cold is the time-honored cause of Bell's palsy. It is thus sometimes spoken of as rheumatic or refrigeratory palsy. It often follows sleeping beside- an open window, working in winter on a derrick with the head, all but the face, completely covered. Car-drivers, expressmen, and persons so exposed are liable. It is ac- knowledged that cold alone can produce the disease in healthy individ- uals. However, it must not be forgotten that in a very large number of the cases, cold only acts conjointly with some underlying infection. 654 THE NO N- NEURONIC DISEASES Lately I have treated a syphilitic sewing-girl whose face became par- alyzed on one side after sitting in a cold draught of air blowing in through an open door. It follows local infectious processes in and about the temporal bone. Caries and otitis media and tonsillar angina have started it. It is not improbable that both diseases may be simul- taneous products of the same general infection. It has followed thus, typhoid, mumps, and influenza. Syphilis has been a prolific source of the trouble in my observation, notwithstanding the fact that some au- thors say that of all the nerves of the body, syphilis respects most the facial. It occurs in diphtheria and the puerperium. Diabetic conditions are often responsible for it ; such as diabetes, gout, leukemia. Compression paralysis often attacks the nerve in basal gumma- tous meningitis. This is a common cause and must be distinguished,, by other signs of the basal disease, from the syphilitic neuritis re- ferred to above. Other forms of meningitis, as well as intracranial tumors, aneurisms, etc., may compress the nerve deleteriously. Trau- mata must not be omitted from its etiology. Not infrequently oper- ations about the ear are responsible for the paralysis. Bilateral dis- ease may occur in the polyneuritides and a diplegic facial paralysis may be the symptom of pontile and medullar disease, such as tumor,, hemorrhage, embolism, aneurism. It has occurred in tetanus. Pre- natal or natal damage may result from the use of the forceps. I once saw a severe facial palsy of this sort. This is called congenital facial paralysis and is usually bilateral. Some of these congenital palsies are undoubtedly nuclear in origin, as also are some of the diphtheritic cases. In many instances a pronounced neuropathic state of the con- stitution lurks behind it. Symptoms. — The cardinal symptom, paralysis of the face, is ob- vious enough. In fact, it is so obvious that the diagnosis of facial palsy does not confer a spark of credit upon the practitioner. Every newsboy can diagnose facial palsy when he sees it. To the physi- cian falls the duty of localizing the lesion, and therein lies the cred- itable diagnosis. The appearance of the face is typical and striking. On the paralyzed side it is smooth, dead and mask-like. The creases of the forehead on that side are gone ; so also are the naso-labial fold and the wrinkles of the cheek and chin. The corner of the mouth droops, the eye is almost completely open. If the patient attempts to whistle or laugh or show his teeth, or draw the mouth over toward the paralyzed side, the paralysis of the orbicularis oris is especially notice- able. If he is asked to close his eyes, the well side closes promptly but the affected side remains open or the upper lid moves slowly and only slightly towards the lower lid. On account of the failure of the buccinator to hold the bolus or food between the teeth, chewing is difficult and in bilateral paralysis all but impossible. Sometimes a slight swelling o\ the face accom- panies these motor manifestations. Occasionally for several days be- fore their onset there will be fugitive pains in the car, face or neck. THE NON-NEUBON1C DISEASES 655 The asymmetry of the face in this disease is the pathognomonic feature. The eyes water, the conjunctiva bulges, the nose inclines toward the well side and saliva tends to drool from the mouth. Upon attempted movement this is rendered more marked. The muscles of the ear are implicated, also the platysma. There is some doubt as to the implication of the uvula in pure peripheral palsy. Occasionally the arch of the palate is lower on one side. As the disease involves usu- ally that part of the nerve in the aqueduct of Fallopius, the paralysis of the stapedius causes hyperacuity of the hearing; and of the chorda tympani, disturbance of the taste in the anterior two-thirds of the tongue. The latter is a form of hemiageusia. Rarely it may be total. Anosmia may occur from lessened dilatation of the nostril. Salivary secretion is diminished on the diseased side. This is one of the vasomotor or secretory effects of the nerve. The tongue is not implicated as that is under the control of the twelfth nerve. Among the trophic manifestations of facial disease are cedema- tous swelling previously mentioned, the herpetiform eruptions and the degenerative muscular changes. The former are rare ; the latter com- mon. The reaction of degeneration is an important symptom as it helps to determine the prognosis. The lid reflexes and skin reflexes are lost, of course, where the arc involves, on the motor side, the paralyzed muscles. Diagnosis. — The most important point of the diagnosis is the location of the lesion. The disease may be central or peripheral. If it is peripheral the flaccidity of the palsy, the muscular atrophy, the reaction of degeneration and the loss of the reflexes will go far to de- termine it. In many cases, however, the paralysis is only partial and these signs are not pronounced enough to form a positive opinion upon. The distribution of the paralysis is an important and valuable means in differentiating central from peripheral facial disease. In peripheral disease the whole nerve is implicated and hence the upper and lower part of the face are equally affected. In central disease the upper part of the face is very much less affected than the lower. This is very noticeable in hemorrhagic apoplexy. The path from the facial center in the cerebral cortex to the facial nuclei in the medulla is not very well known, though we believe it joins that of the arm and leg and passes down through the internal capsule of the pyramidal tracts in the crura cerebri. It decussates. This is the volitional facial tract. When the lesion is anywhere in this part of the tract, the upper facial is intact (probably because oi the bilateral cerebral representation) ; the reflexes and electrical ex- citability are normal. The muscles tend to spasticity and there is no atrophy. Usually the extremities are paralyzed at the same time. If the lesion should be a small one and attack this upper facial path- way between its point of decussation in tin- pons and its termination in the nucleus, the paralysis in relation to the face and limbs will obviously be a crossed paralysis. The localization of the lesion in peripheral disease of the nerve is often important and usually not difficult. Traumatic causes often g 656 THE NON-NEURONIC DISEASES rise to disease of particular branches. A knowledge of the anatomy of the nerve is all that is necessary tinder such circumstances to settle which branch is affected. It is difficult at times to differentiate between pontile facial palsy and palsy due to the peripheral trunk. A close examination will usu- ally elicit other manifestations of the disease of the pons under such circumstances. Other nerves may be involved, especially the sixth and sometimes the seventh. In meningitis and other basal disease implicating the facial nerve, other nerves will also very likely be included, especially the auditor}-. FIGURE 144. Showing the facial trunk from the base Of the skull to the pes ansennus. N.f., facial nerve , F.st., stylomastoid foramen N.a.p., posterior auricular nerve N.p.s., greater sup. petrosal nerve , N.e.c.p.t., communicating nerve, with plex tymp. , N.sl , sta- pedius nerve, Ch.t., chorda tympani , N.n., acoustic nerve. G.g., gangl. geniculi. (After Erb.) Of course, general cerebral symptoms would be expected also, such as pressure headache, vertigo, vomiting, possibly papillitis. If the lesion is peripheral to the point where the chorda tympani is given off, and central to that where the temporo-facial and temporo- cervical separate, only the facial muscles will be paralyzed. If the lesion is in the aqueduct of Fallopius the chorda tympani and stapedius branches will be implicated and there will be loss of the sense of taste in the anterior two-thirds of the tongue and a hypersensi- tiveness of the hearing. Erb's diagram helps to make this clear. Vari- ations are possible, however. Prognosis. — Mild cases usually get well in three or four weeks. Three to six months are required for the average case. Severe cases do not get well uncbr a year and sometimes they remain stationary. THE XOX-XEURONIC DISEASES 657 Of course, the cause and nature of the lesion regulate the prog- nosis. If the cause is removable early, the disease will disappear. Syphilitic cases are very hopeful. Simple refrigerator)- cases are favor- able. Severe infectious cases, and those due to intracranial neoplasms, are often hopeless. The degree of reaction of degeneration is a most valuable indi- cator of the prognosis and probable duration of the disease. If this is partial, the trouble may disappear in a few weeks. If this is complete, the end may not come for many months or years even. Acute cases are better than the slow, chronic and progressive cases. A secondary trouble of much moment are the contractures that oc- cur late in the previously paralyzed muscles. These are not due to electrical treatment, as was once imagined. They are due to the irrita- tion of the nucleus in the forcible attempts at the use of the nerve, or to slight secondary nuclear alterations. Sometimes recovery is incomplete, some muscles regaining their power and others remaining palsied. Treatment. — The first indication of course is to remove the noxious agent. Infections, like rheumatism, syphilis, influenza, must be combated along known lines. Abscesses must be drained. Ear disease should be treated. A general and local antiphlogistic treatment is usually in order. This includes purgation, blood-letting, warm ap- plications beneath the ear, vesication, diaphoresis. When active inflammation has subsided, electrical treatment is called for and gives most happy results. The galvanic current usually has to be employed first. The anode is placed on the sternum or other indifferent spot, while the cathode is placed on the nerve trunk where it emerges from the stylo-mastoid foramen. For two or three minutes daily, three or four milliamperes may be employed in a slow, alternating current. Never use strong currents, also be careful about usin'g the faradic current in severe cases. Secondary contractures cannot be combated by any known means. Massage may help some. THE AUDITORY, OR EIGHTH CRANIAL NERVE. This is a nerve with a double function, both sensory. It subserves hearing and the space-sense. It arises from three nuclei in the floor of the fourth ventricle and emerges from the groove between the pons and the medulla. The nerve passes with the facial to the internal audi- tory meatus. In the meatus it separates into an upper, or small, and a lower, or larger, part. The upper goes to the utricle and the ampullae of the superior and external semicircular canals. The lower innervates the organ of corti in the cochlea, sending off small twigs to the saccule and the posterior semicircular canal. It is in connection with the pars intermedia and the geniculate ganglion of the facial. Disease of this nerve does not occur often in the isolated form. Middle ear disease often extends to the terminal branches of the nerve in the labyrinth. Meniere's symptoms are thus explained. In my own opinion, some of the so-called cases of Meniere's disease are central 658 THE NON-NEURONIC DISEASES troubles, as I have shown elsewhere. Caries, periostitis, tumors, menin- gitis, infections, cachexia? and all the usual causes cited in connection with facial nerve palsy may be operative here. General degenerative troubles, like tabes, multiple sclerosis, alcoholic neuritis, may include the auditory nerve. Syphilis I have seen cause trouble in it many times. This was probably due to gummatous meningitis. Labyrinthine and auditory disease cannot always be differentiated, which is one of the grounds of my contention that Meniere's syndrome is far from be- ing a definite disease, hemorrhagic or otherwise. Basal disease usually involves the facial along with, or more often apart from, the auditory nerve. In every case of aural vertigo, of tinnitus and of disturbance of hearing, the ear should be thoroughly examined. Subjective tinnitus and subjective deafness may occur in so many other functional and organic diseases that the implication of the auditory nerve had better be made by exclusion. The prognosis and treatment of auditory paralysis follow the usual principles. THE GLOSSOPHARYNGEAL, OR NINTH CRANIAL NERVE. The roots of this nerve are traceable back to a nucleus which is closely associated with that of the vagus nerve, and which lies in the floor of the fourth ventricle in the posterior triangle beneath the in- ferior fovea. It appears at the upper part of the medulla, in the groove between the olivary and restiform bodies. It is directed outwards to the jugular foramen. Passing through this in company with the pneu- mogastric and spinal accessory, it presents two ganglia, the jugular and the petrous. The nerve lies between the internal carotid artery and the jugular vein. It courses down over the artery and beneath the styloid process and the muscles connected with it, to the hinder border of the stylo-pharyngeus. Crossing the outer surface of this muscle and pass- ing beneath the hypoglossus, it ends in a number of branches to the posterior part of the tongue. It supplies the mucous membranes of the tongue, pharynx and middle ear, as well as one muscle, the stylo- pharyngeus. It is connected with the inferior maxillary division of the fifth nerve (through the otic ganglion), the facial, the pneumogastric (its trunk and branches) and the sympathetic. This is regarded as preeminently a nerve of taste. We know so little about it, however, that nothing can be said positively in regard to its physiology or pathology. It has motor fibres, as we have seen, and it certainly conducts sensations of simple touch. The nerve may be implicated, like any other cranial nerve, with tumors, traumata, aneu- rism, neighboring inflammation. Thrombosis of the jugular vein ami periphlebitis may affect it. It is subject to degeneration as a part of a most extensive process. Its paralysis causes ancesthesia, ageusia in the posterior third of the tongue and palate and dysphagia. Diphtheria causes some of these symptoms. THE NON-NEURONIC DISEASES 659 THE PNEUMOGASTRIC, OR TENTH CRANIAL NERVE. The roots of this nerve can be followed back to the trigonum vagi in the floor of the fourth ventricle. The nucleus ambiguus is believed to be the origin of the motor strands. The sensory roots represent the cen- tral processes of the neurones whose cell-bodies lie in the ganglion of the root and of the trunk. These processes enter the medulla and de- scend with those of the glosso-pharyngeal root, arborizing around cells which make up the so-called vagus, sensory nucleus. It is very probable that these cells of the terminal nuclei of this and the ninth nerve are but homologues of those making up the vesicular column of Clarke. Both the motor and sensory fibres of this nerve are distributed to viscera chiefly. The roots appear in the groove between the olivary and restiform bodies. This nerve has the largest course of any of the cranial nerves, reaching from the neck to the upper part of the abdomen. It passes outward from the medulla, attains the jugular foramen,, presents two ganglia, is joined by the accessory part of the spinal accessory nerve and finally passes on down the neck and thorax and abdomen dis- tributing branches to the lungs, the heart and the other viscera. In the jugular foramen, a branch is given to the dura mater and one to the ear. In the neck the pharnyx, larynx and the heart are sup- plied. In the thorax, branches are furnished to the heart, the lungs and the oesophagus. In the abdomen the terminal branches are given to the stomach, the liver and other organs. Motion, sensation, secretion and vasomotor action are all under the influence of the vagus. A primary neuritis of this nerve is very rare, if it occurs at all. Disease of any other structure along its course may damage it in that neighborhood, however, and in this way the nerve is not in- frequently affected. Primary inflammation of the nerve has been ob- served in rheumatism, alcoholism, diphtheria, typhoid, pneumonia, scarlatina, malaria, cholera, influenza. Under these circumstances it has usually constituted a part of a polyneuritis. I recently saw it affected in chronic lead-poisoning to a dangerous degree. Arsenic will injure it. Morphine and atropine may intoxicate it. It is usually implicated in bulbar disease. Disease at the base of the skull constitutes an important factor in etiology. This includes caries, exostoses, tumors, aneurisms, hemorrhages, meningitis with exudates. Numerous are the vascular troubles that disturb it, such as arterio- sclerosis, thrombosis of the transverse sinus, jugular thrombosis and phlebitis. Mediastinal tumors, suppuration, tuberculous glands, peri- carditis, and wounds and operations in the neck and thorax must all be sometimes reckoned with. Not only are vagus symptoms associated with tabes and multiple sclerosis but they also appear in hysteria and other neuroses. Symptoms. — A total vagus paralysis may occur from basal dis- ease, but it is not common. ( Hlier cranial nerves are usually implicated at the same time. The eleventh, ninth and twelfth are thus usually included. 660 THE NON-NEURONIC DISEASES The symptoms of vagus palsy usually assume characteristics in accordance with the particular location of the lesion. Phonation is affected and the palate, larynx and fauces are seen to be paralyzed. On account of the condition of the palate the speech, of course, is nasal. Dysphagia is present. The respiration is disturbed. Rarely there has been anaesthesia of the pharynx and larynx. Acceleration of the heart action is an old and well-known symp- tom. The inhibitory control of the nerve over the heart is one of the ancient teachings of physiology. In unilateral lesions the heart has been seen to be retarded. Irritative lesions may exaggerate the in- hibition and so retard the cardiac phenomena. Respiratory disturbance, bulimia, vomiting, absence of hunger and thirst, pain in the stomach and diabetic manifestations are all to be attributed to vagus trouble sometimes. The respiratory implication results usually in what is called aspiration pneumonia, or hypostatic congestion of the lungs. Laryngeal paralysis is especially significant. It may occur alone or as a part of a general vagus palsy. These cases belong to laryngol- ogy. I have under my care a syphilitic with complete paralysis of the right vocal cord. Spasmodic coughing, embarrassed breathing and alteration of the voice are all among the symptoms. In bilateral palsy, there is complete aphonia, inspiratory stridor and dyspnoea. This oc- cus in diseases of the medulla usually and not infrequently in hysteria. I saw it lately as a neurosis following a lightning stroke. As a neurosis phonation is much more affected than respiration, whereas in organic disease respiration is dangerously involved along with the loss of voice. The reason is obvious for respiration is a function much farther removed from the volitional cerebral functions than is phonation. Paroxysmal tachycardia, asthma nervosum and partial paralysis of phonation may all be observed. Treatment. — Seach for and remove the cause. Syphilis especially must be attended to. Alcohol, tobacco and other toxins must be re- moved. Lead should be eliminated in the usual way with baths, purga- tives and the iodides. Supporting diet must be urged. Electrical treatment has not proved of much benefit in my hands, though it is well always to try it. External applications are better than direct applications in the larynx. There is a massage of the larynx. Tracheotomy may have to be resorted to in some dangerous cir- cumstances. THE ACCESSORIUS, OF ELEVENTH CRANIAL NERVE. A part of this nerve, the accessory, belongs practically to the vagus system and physiologically cannot be separated from it. The spinal part of the nerve supplies the sterno-mastoid and trapezius muscles. Some of the cervical nerves assist in partially innervating these muscles so that paralysis of the eleventh nerve does not eventuate always in the complete destruction of their function. The control of the sterno- mastoid is almost completely a function of the eleventh nerve. The THE NON-NEURONIC DISEASES 66l trapezius is in its clavicular portion quite a little subject to the acces- sorius nerve. Disease of the cervical cord and spinal roots may affect the nerve. Also is it liable to damage from carious vertebra?, here, from exostoses, abscesses, tuberculous and syphilitic conditions, tumors and menin- gitic exudations. Compression may occur from trouble in the fora- men magnum. The nerve may be primarily inflamed and this neuritis may be unilateral or bilateral. The chief symptoms of disease in this nerve are complete paraly- sis of the sterno-mastoid muscle, partial paralysis in the trapezius. The latter does not produce much of a deformity. The former causes one form of torticollis or wryneck. The chin cannot be moved back towards the side of the diseased muscle. When both sterno-mastoids are affected, the head falls back upon the shoulders. Prognosis and treatment need no special comment as they follow the ordinary principles for peripheral neuritic disease. THE HYPOGLOSSAL, OR TWELFTH CRANIAL NERVE. This is a pure motor nerve ; springs from a nucleus in the medulla that corresponds with the upward extension of the anterior horn of the cord ; and in some of the lower animals is a pure spinal nerve. It ap- pears at the side of the medulla between the pyramid and olivary body. It supplies, alone or in conjunction with branches from some spinal nerves, all the muscles connected with the hyoid bone, including the tongue, but excepting the digastric, stylo-hyoid, mylo-hyoid and the middle constrictor of the pharynx. It stimulates also the sterno-thyroid muscle. It is in connection with the pneumogastric, lingual, upper three cervical nerves, and the sympathetic. The nerve is more often damaged within its cerebral and medullary course than outside. It occurs in association with cerebral hemiplegia, the tract connecting the cortical center for the tongue with the hypo- glossal nucleus in the medulla running along through the internal cap- sule and crus with the tract for the extremities. In this hemiplegic form of the parlysis, of couse, there is no muscular atrophy. Bilateral or unilateral atrophic paralysis of the tongue is usually a medulla disease. Peripheral damage to the nerve may be caused by favorably located tumors, meningitic exudations, 'hone disease, basal hemorrhage, aneurisms of the verebral artery and dislocations of the upper cervical vertebrae. It has accompanied hydatid cysts. Cervical traumata and tumors may injuriously compress the nerve. Rarely there may be an isolated neuritis, following an infection of some sort, such as scarlatina. It may be associated with tabes, disseminated sclerosis, syringo- myelia, facial hemiatrophy and disease of other cranial nerves. If the palsy is bilateral the tongue lies dead and limp Otl the floor of the mouth. If it is unilateral the tip <>| the- tongue inclines toward the normal side but upon protrusion of it, the whole tongue moves toward the paralyzed side. The root of the tongue, according to Gowers, is a little higher on the paralyzed than on the sound side. The 662 THE NON-NEURONIC DISEASES raphe is arched, with the concavity toward the paralyzed side. The deviation is one of the results of the atrophy in the genioglossus muscle. This paralysis, like all neuritic peripheral palsies, is accompanied by muscular atrophy, flaccidity, fibrillary tremor, incomplete reaction of degeneration and reflex alteration. The speech, mastication, deglutition are not very much embar- rassed when the palsy is unilateral. When it is bilateral they may be very markedly disturbed, such trouble being usually of medullary origin. The prognosis is favorable in syphilitic, traumatic and rheumatic cases. Other cases are relatively unfavorable. The treatment should be carried out along the lines indicated un- der the head of neuritis and the other cranial nerves. PART in Non-Neuronic Diseases of the Brain. ANATOMY AND PHYSIOLOGY OF THE BRAIN. CERE- BRAL LOCALIZATION. CRANIO-CEREBRAL TOPOG- RAPHY. SYMPTOMS OF DISEASE OF THE BRAIN. Anatomy. — As I have shown under the head of the anatomy of the spinal cord, it is unfortunate that the gross and artificial division of the cerebrospinal axis into the brain and cord has become so estab- lished in the minds of the profession. It is not a physiological divis- ion and for the proper conception of nervous diseases, the physiology of the nervous system is a matter of infinitely greater importance than is its mere anatomy. I am convinced that much of the commonly ex- pressed difficulty in the understanding of nervous diseases, is due to the fact that anatomy and physiology have not hitherto taught the structure of the nervous system in the same way. The former has used, and unfortunately still uses, coarse, artificial lines of division, a misleading nomenclature and an irrelevant method of description. The student has always to revise his* anatomical lore as soon as he begins to delve into the physiology of the nervous system. If he is lucky in forgetting much of his anatomy and relearning it upon a physiological basis, he will have no trouble in understanding the diseases of the nervous system. If, however, he is a busy practitioner, who acquired his anatomical knowledge long ago and who now has no time to fol- low up the latest physiological discoveries, he will not understand the diseases of the nervous system. lie will constantly find them a puzzle and a mystery. These diseases represent physiological and anatomico- physiological changes and not merely anatomical abnormalities. The THE NON-NEURONIC DISEASES 663 onlv salvation for the old graduate is to forget his old anatomical studies and relearn the structure of the nervous system upon the mod- ern physiological basis. He must learn that cerebrum, medulla and cord, for instance, are not separate organs, having their own duties to perform ; for they all work together and in unison as one great organ. A still better way to put it would be that these names merely indicate a certain general locality in which are found some of the serial deposits of gray matter that make up the whole apparatus and from whose cells processes run back and forth from one to the other. The brain, so-called, consists merely of that particular set of ganglionic masses that happen to be inside of the skull. Neither anatomically nor functionally does the skull limit them, however. They are as much in touch with the cord, anatomically and physiologically, and the cord is as much in touch with them as one lobe of the liver is with another lobe of the liver. Nay, more, they are in closer touch than are the hepatic lobes, for cerebral elements actually reach down (pyramidal tracts) and become a part of the cord, and spinal elements (direct cerebellar tracts, for instance,) extend up and become parts of the brain. In using the old nomenclature and the old anatomical divisions and subdivisions of the nervous system, I do so in deference to the long- grounded conception of the nervous system still held throughout the profession, and because it would be too radical to all at once cease using convenient names simply because they happen to convey erro- neous physiological ideas. Some day we will speak everywhere and under all circumstances of the cerebrospinal axis as a single organ, just as we now do of the liver. There will grow up in our minds the truth that this is a composite organ with one universal function through- out all of its parts, just as is the case with the liver. We will realize that essentially the cells (neurones) of one part of this organ do not functionate per se differently from the cells (neurones) of another part. We will be reconciled to the startling fact that the apparent differences between the functions of these widely separated neurones or constituent cells of the cerebrospinal organ are due merely to the differences between the end-organs to which they respctively happen to be attached. Certain brain cells see, let us say, and the cord cells do not see, because the former are attached to a particularly differentiated end-organ of epiblastic origin, the eye, that is different from any end-organ with which the cord happens to be attached. The cells themselves, both anatomically and physiologically, however, are from all evidence, exactly the same. This fact must be kept well in mind in our studies of the physiology of the brain or any other part of the nervous system. With the understanding, therefore, that old names are used tor mere rough topographical purposes, and not in any sense as convey- ing a correct idea of the anatomico-ph\ siologieal structure of the nerv- ous system, as we now know it, I will take up the anatomy and phys- iology of the brain. The brain, or encephaioii, is all of that part of the nervous sys- tem that lies above the foramen magnum in the occipital bone and 66 4 THE NON-NEURONIC DISEASES which fills the great cranial cavity. Conventionally it is said to be attached to the spinal cord at the foramen magnum. It is a large, ovoid, convoluted mass, smooth and covered with membranes. Turn- ing it upside down we see that we can divide it conveniently, for topographical purposes, into four sub-masses ; the cerebrum, the cere- bellum, the pons Varolii, and the medulla oblongata. The weight of the brain is a matter of some importance. For the majority of males it ranges between 1,300 and 1,450 grammes. The female brain weighs a trifle less. The heaviest brain that has been put upon record was that of Bismarck. It was calculated to have weighed over 1,867 grammes. Brain weight not only varies in ac- cordance with the sex but with the intelligence, the age and the race as well. The question of the relationship between brain weight and intel- ligence has long been debated. Intelligence is an elastic term. There are men of a high degree of intelligence in one direction, but of dense ignorance in another. Both with or without cultivation, intelligence may mean something very different between two men. The only real standard of intelligence that we could adopt would be one that en- compassed all knowledge and all mentality. That is never seen and hence our definitions of intelligence are always partial. It is a fact that in a few instances of special lines of mental cultivation, correspond- ing parts of the brain have shown some special development. Gam- betta was a great orator. His speech center in the brain was said to have been uncommonly developed. The visual centers in the occipital lobe have been found well developed in artists. These suggestive facts taken in conjunction with the truth that men of marked intellectuality and that certain superior races have had heavier brains than others, one is almost inclined to believe that brain weight has a great deal to do with intelligence. Cuvier's brain weighed 1,830 grammes; Byron's, 1,807; Kant's, 1,650; Schiller's, 1,630; and Dantes', 1,420. English, Scotch and German brains are heavier than those of the Russians, French and Italians. Some of the smallest brains found are among the primitive African and Australian races. The negro brain averages about 1,250 grammes. Perls and Edinger have worked at the prob- lem from another side and obtained similarly suggestive results. They noticed that many men of eminence in the intellectual sphere give evidence of having had hydrocephalus which had healed in childhood. Such was probably the case with Rubenstein, Cuvier, Walter Scott and others. It is suggested that this favored brain expansion with possible increase in weight and size. Of course, it is understood in this that mere bigness of brain does not create intelligence by any means. One of the finest and largest brains I ever removed at a post mortem was from a man who scarcely had his equal for ignorance and stupidity. It is inferred merely that largeness of brain furnishes a large capacity for intelligence if it is exercised. All brains that weigh less than 1,130 grammes in man, or 990 in woman, are spoken of as microcephalic ; all that weigh over 1,490 in man, or 1,345 in woman, are referred to as megalocephalic. At birth the brain weighs about 327 grammes. Up to four years THE XOX-XEUROXIC DISEASES 665 of age it grows very rapidly. From four to seven, its growth is slower. From the latter age to twenty its increase in size is very slow. Then it remains stationary. At 45 and 50, in men and women respectively, it begins to lose in weight but very slowly. At eighty it has lost about 120 grammes. The relation of the brain to the body development, of course, must be always taken into count. The relative weight is about 2 per cent. The Membranes of the Brain. — There are three membranes that cover the brain, namely, the dura mater, the arachnoid, and the pia mater. The dura mater is an inelastic and dense structure, composed of FIGURE 145. rUxu-t spinal, iut. The blood-vessels of the dura mater. Lateral view. (After Heitzmann.) white fibrous and yellow elastic tissue and is lined on its under sur- face with flat endothelial cells. It consists of two layers. The outer layer is the nutrient endosteum and is attached more tightly to the bones at the base of the skull than at the vertex. Through the cranial sutures and various foramina it becomes continuous with the perios- teum on the outside of the skull. The inner layer separates from the outer to form the sheaths of the cranial nerves and the great venous sinuses. Moreover it sends down between the main divisions of the brain the great septa called processes. The falx cerebri and the fcil.v ccrcbelli arc vertical curtains that hang in the great longitudinal fissure and separate the two halves of the brain. Another curtain is the tentorium ccrcbelli which passes horizontally forward into the transverse fissure and separates the pos- terior part of the cerebrum from the cerebellum. 666 THE NON-NEURONIC DISEASES The bony attachments of the falx cerebri are the crusta galli in front and the internal occipital protuberance behind. It is also fastened to the upper surface of the tentorium, along the occipital crest and to the posterior border of the foramen magnum. In the upper convex and lower free borders of this falx are the superior and inferior longi- tudinal sinuses, the superior extending from the foramen caecum back to the torcular Herophili at the internal occipital protuberance, and the inferior uniting with the common vein of Galen, or vena cerebri magna, and becoming the sinus rectus that courses through the tentorium to the torcular. The attachments of the falx cerebelli are the inferior surface of FIGURE 146. The blood- vessel* of the dura mater. Bird's-eye view. (After Heitzmann.) the tentorium and the occipital crest to the posterior edge of the fora- men magnum. Through this septum runs the occipital sinus or sinuses from the foramen magnum upwards and backwards to the torcular. The fastenings of the tentorium are the internal occipital pro- tuberance, the petrous bone and the clinoid processes of the sphenoid. Through a large notch the brain stem is transmitted. At its sides are the lateral sinuses. These run from the torcular, along the horizon- tal arms of the occipital crucial ridge to the petrous bone where the corresponding superior petrosal sinuses enter them and finally turn downward through the sigmoid fossa, uniting with the inferior petro- sal sinuses in the jugular foramina. The pituitary fossa is specially covered by a portion of the dura called the diaphragm selhe. THE NON-XEUROXIC DISEASES 667 It will be observed that the various parts of the brain are by these dural septa encased in membranous boxes, as it were. This pro- tects the brain from sudden jars. It also limits and directs the course of hemorrhagic and purulent collections that may form in them or enter from without. In addition to the sinuses that have already been mentioned, notice must be taken of the circular sinus around the sella turcica, the two cavernous sinuses on either side and joined by the circular, and the .transverse or basilar sinus that unites the inferior petrosal sinuses. The dura receives its blood supply from the anterior and poste- rior ethmoidal branches of the ophthalmic, from the anterior menin- geal of the internal carotid, the great and small meningeals, from the internal maxillary, the meningeal branches of the ascending pharyngeal and occipital, and the posterior meningeal branch of the vertebral. The important artery is the middle meningeal from the external carotid. The veins are numerous and connect with those of the cranium. The dura is the only cerebral membrane that has a nerve supply. There are branches from the sympathetic that accompany the arteries. There are also branches from the trochlear, the ophthalmic, the Gasse- rian ganglion, and the tenth and twelfth. The motor branches of the nerves innervate the arteries. The arachnoid is a loose membrane, delicate and lace-like. The inner surface is so attached to the pia mater by trabecular that there are a lot of inter-communicating subarachnoidal spaces filled with cere- brospinal fluid. On the outer surface are the enlarged villi, known as Pacchionian bodies, or granulationes arachnoideales, that project within and along the superior longitudinal sinus and that probably serve as an outlet for the subarachnoid fluid into the sinus. The mem- brane follows the inner surface of the dura and does not dip into the sulci with the pia. The posterior subarachnoid space communicates with the fourth ventricle through the foramen of Magendie and the foramina of Key and Retzius. The arachnoid is without vessels and nerves. The pia mater is a highly vascular membrane consisting of a net- work of arteries and veins held together by fibro-elastic areolar tissue. Outwardly its epithelium lines the subarachnoid trabecular and spaces. These spaces are very large at the base of the brain. The pia hugs closely the surface of the brain and sends septa and bloodvessels vertic- ally into the brain substance. It dips down everywhere into the sulci and it forms investing sheaths to the cranial nerves. Between the fornix and the interbrain it enters the choroid fissure and ultimately becomes the velum interpositum and part of the tela choroidea ven- triculi tertii. Between the medulla and cerebellum it is inserted over the fourth ventricle and helps to form the posterior choroid tela which is perforated by the foramina of Magendie, Key and Retzius. The anterior, middle and posterior cerebral arteries, the anterior and posterior inferior cerebellar and the superior cerebellar all sup- ply the pia. Its venous supply is also abundant. The veins all empty into the sinuses and are the venae Galeni, choroid plexuses of tin- ven- tricles and the cerebral veins. The sympathetic, of course, innervates 668 THE NON-NEURONIC DISEASES it, as well as the third, fifth, sixth, seventh, ninth, tenth and eleventh cranial nerves. General Anatomy of the Brain. — A brief study of the embryo- logical development of the brain makes clear certain facts that it is well to understand before going on with a discussion of its anatomical structure. By the fifteenth day after conception, the neural tube resulting from the infolding of the epiblast, is complete, except for a short dis- tance posteriorly. In that portion which is to become the encephalon, or brain, later on, two constrictions take place which divide it into FIGURE 147. Epithalamus (Corpus pineale) Metathalamus (Corpora geniculate) Thalamus Sl^^NX Corpora quadrigemina efeficephalqr { % Pedunculus cerebri Rhinencephalon Corpus striatum Sulcus hypothalamics [Monroi] , Hypothalamus * Chiasm* opticum Cerebellum Fossa rhomboidea Brain of the human embryo five weeks old. (After His.) Right half viewed from the lelt- three primary brain vesicles. Behind the third vesicle, the tube con- tinues small and constitutes the later spinal cord. The brain vesicles develop rapidly and again break up into subdivisions. A constriction appears in the anterior and posterior vesicles. Thus ultimately there are five vesicles. These with their surrounding elements are named from before backwards, the Prosencephalon, or Telencephalon ; the Thalamencephalon, or Diencephalon ; the Mesencephalon ; the Meten- cephalon ; and the Myelencephalon. As development goes on the various parts of the brain assume their adult appearance in these vesicular areas and the interconnected cavity within them becomes the ventricles. The adult ventricles consist of the two lateral, the third, fourth and fifth; with the foramina of Monroe connecting, y-shape, the two lateral with the third, and the aqueduct of Sylvius the third with the fourth. THE NON-NEURONIC DISEASES 669 Along the sides of these ventricles can be traced the groove or line of Monroe which, according to Minot, is of great significance in FIGURE 148. Embryonal Development of the Brain. A — Brain and Spinal Cord at the Third Month. B — Base of the Brain at the Fourth Month. C — Median surface of the Brain at the Fourth Month. D — Outer surface of a Cerebral Hemisphere at the Fifth Month. Jakob.) (After showing the developmental separation between the dorsal or receiving zone of the cerebrospinal organ and the ventral or emissive zone. In 670 THE NON-NEURONIC DISEASES the former are small nerve cells whose processes never pass out of the central nervous apparatus to form nerves. In the latter are the large cells whose processes do pass out to become nerves. The sensory nerve paths which are received by the dorsal zone are the processes of the cells in the cranial nerve ganglia and posterior spinal ganglia which are developed out of the original neural crests and not directly from that part of the neural tube from which the brain and cord de- velop. FIGURE 149. FRONTAL 10BE (After Gray.) The parts around the vesicles are not all equally elaborated in the adult brain. For instance, the prosencephelon in man becomes enormously developed into the cerebral cortex. The diencephalon is rather poorly developed and the olfactory projections (erroneously called nerves) are actually atrophied. In some lower animals the olfac- tory bulbs are larger than the cerebral hemispheres. The mesen- cephalon is a rudimentary organ. The metencephalon is well elabor- ated, whereas the myelencephalon is again relatively undeveloped. Three great flexures occur in the cephalic part of the neural tube, two ventral and one dorsal, which further help to disguise the primi- THE XOX-XEUROXIC DISEASES 67I tive origin of the brain and cord of the adult. The mesencephalic ventral flexure is a very early one and at about the twenty-eighth day amounts to nearly 180 degrees. The thalamencephalon is thus brought almost into contact with the epencephalon. A cervical, ventral flexure occurs where the last vesicle joins the spinal cord. It represents the bending of the head upon the body of the embryo and at about the fourth week it amounts to 90 degrees. There is finally a late dorsal flexure, sometimes called the metencephalic, at the point where the FIGURE 150. View of the corpus callosum from above (from Sappey after Fo- ville). 4 The upper sur- face of the corpus callosum has been fully exposed by separating the ce- rebral hemispheres and throwing them, to the side ; the gy- rus fornicatus has been partly de- tached, and the transverse fibres of the corpus callo- sum traced for some distance into the cerebral medul- lary substance. l,the upper sur- face of the corpus callosum ; 2, me- dian furrow or raphe ; 3, longitu- dinal striae bound- ing the furrow ; 4, swelling formed by the transverse bands as they pass into the cerebrum, arching over the side of the lateral ventricle ; 5, anterior extremity or knee of the corpus callosum ; 6, posterior extremity ; 7, anterior, and 8, posterior fibres proceeding from the corpus callosum into the frontal and occipital lobes respectively ; 9, margin of the swelling ; 10, anterior part of the gyrus fornicatus ; 11, fissure between the corpus callosum and this convolution opened out ; outside 12, is the termination of the calloso-niarginal fissure, and before 13 is the parieto-occipital fissure; 13, upper surface of the cerebellum fourth and fifth vesicles approximate. B3 the eighth week it amounts to 180 degrees and thus causes the dorsal part of the cerebellum to partially rest upon the medulla oblongata. The cervical and meten- cephalic flexures are temporary ; the mesencephalic is permanent. This is all the result of the working of the laws of evolution and in large part represents the principle of adaptation to environment. The value of this lies in the fact that we can thus unravel the complicated architecture and functions of the adult human brain by the compara- tive study of the lower and less developed brains of the animal world. 6j2 THE NON-NEURONIC DISEASES The marvelous harmony and consistency of nature are thus not only realized in the primitive tracings of the highest expression of vital de- velopment, the human brain, but the value of all morphological and biological studies is enormously enhanced. I will now resume the general anatomical description of the en- cephalon. The following ganglionic deposits and their connecting pro- cesses are to be passed in review: I. Cerebrum (Prosencephalon) consisting of a. Hemispheres (Telencephalon), Their cortex and centrum ovale, Corpus callosum, Fornix, Commissura hippocampi, and Anterior commissure. b. Interbrain (Diencephalon), Lamina cinerea, Tuber cinereum and infundibulum, Pituitary body or hypophysis cerebri, Corpora albicantia, Optic thalami, Middle and posterior commissure, Pineal body or epiphysis cerebri, External geniculate bodies, Optic tracts and commissure. II. Midbrain, Crus Cerebri or Pedunculus Cerebri Crustae ( Mesencephalon ) , Substantia nigra, Tegmentum, Corpora quadrigemina, Internal geniculate bodies. III. Hindbrain (Metencephalon), Pons Varolii, Cerebellum. IV. Afterbrain (Myelencephalon), Medulla oblongata. Most of these parts are shown in the accompanying diagrams. For a further general anatomical description of them the reader is referred to works upon anatomy. Lobes, Convolutions, Fissures and Sulci of tlic Brain. — It is im- portant for topographical purposes that the surface of the brain be mapped out in areas and named. The fissures and sulci serve as most convenient lines of division. The accompanying maps of the brain will assist the reader and save a long description. The surface of the brain is called the cortex. It is seen to be divided into a great many subdivisions, as though it were made up of a mass of worms, by large and small fissures. The fissures are re- ferred to as primary and secondary. They represent infoldings of the cortex, allowing thus of a great area to be packed away in a small THE XOX-XEUROXIC DISEASES 673 space. This is a condition of comparative intelligence and evolution- ary development, for primitive brains are deficient in fissuration. The higher in the scale of life the animal is the greater the amount of convoluting is there of the brain surface. The fissures and sulci corre- spond with the developmental rearrangement, therefore, of various ganglionic deposits in the brain to accommodate them to the narrow capacity of the skull. This is virtually true of all of them, but as some of them seem to be older and more fundamental, more permanent, FIGURE 151. LobU9 irontalis Cavuni sei>ti pellucidi Lamina so^ii pellucidi Corpus callosum (cut through) Foramen interventricular* [Monroi| Columna fornici* Lobus temporalis Corpus fornicis Taenia former us striatum Caput nuclei caudati Capsr.la interna Nucleus leutiformis Stria terminate Hippocampus Pars centralis ventriculi lateralis Comu inferius ventriculi lateralis Cauda nuclei caudati Plexus chorioi- deus ventriculi lateralis Bulbus comu posteriori Cornu posterius ventriculi lateralis Fissura calcarina Lobus occipital! Spleniuni corporis callosi Lateral Ventricle, opened, viewed from above. (After Spalteholz.) than others, we speak of them as the primary fissures, and the others as the secondary. The primary fissures of the brain arc : The Longitudinal, between the two hemispheres. The Transverse, or choroidal, extending in betweeen the cerebrum and cerebellum. The Rolandic, on the upper mid-surface of the cerebrum. This is a most important localizing fissure from the surgical point of view. 674 THE NON-NEURONIC DISEASES The Sylvian, in the mid-lateral area, and important on account of the middle meningeal artery that passes up along it. The Parietooccipital, on the posterior internal surface of the hemisphere chiefly. The Calcarine and Parietal fissures are by some classified as pri- mary. Five great general divisions of the surface of the brain are out- Lobus temporalis Digitationea ippocampi Cornu infer I us ventriculi lateralis. Uncus fgyri bippo<:ampi] yTus hippocampi Eminent ia collaterals Fascia d^ctaja hippocampi Fimbria hippocampi Taenia fimbriae. Hippocampus Crus fornicis Trigonum collaterale Fissura calcari Trigonum habenulae Commissnra habenularuin Lobus occipitalis Third Ventricle, opened, viewed from above. (After Spalteholz.) lined by these fissures. Above the Sylvian and in front of the Rolan- dic fissure is the Frontal Lobe. Above the Sylvian and behind the Rolandic is the Parietal Lobe. Below the horizontal limb of the Syl- vian fissure lies the Temporal or Temporo-sphenoidal Lobe. Behind the Parietooccipital is the Occipital Lobe. If the Sylvian fissure be separated, a cortical area will be found lying at the bottom of it. This is the Central Lobe or Island of AY/7, and contains the great central ganglia. To these are sometimes added the Limbic Lobe, on the mesial surface of the hemisphere just above the corpus callosum, and the Olfactory Lobe or Bulb which is so much atrophied in man. THE XOX- NEURONIC DISEASES 675 These lobes are again subdivided into lobules and convolutions by the secondary fissures and sulci. On the outer surface of the Frontal Lobe are two horizontal and one vertical sulcus, thus forming a superior, middle and inferior con- volution, and behind them the precentral or ascending central convolu- tion. The part of the lobe that is immediately behind the short, ver- FIGURE 153. Schematic representation of the cerebrum viewed from ab tical limb of the Sylvian fissure is known as the operculum. The mesial surface of the lobe includes the paracentral lobule, just in front of the posterior end of the calloso-marginal fissure, and the superior frontal gyrus in front of that. The basal surface is grooved for the olfactory bulb. The Parietal Lobe includes on the outer surface the postcentral 676 THE NON-NEURONIC DISEASES or posterior ascending convolution and the upper and lower lobules, separated by the interparietal sulcus, which is long, arched and fre- quently interrupted. The convolution of the inferior lobule that sur- rounds the posterior end of the horizontal limb of the fissure of Syl- vius is known as the supra-marginal. That which surrounds the pos- terior end in like manner of the superior temporal fissure is called the angular gyrus. The forward end of the interparietal fissure is the inferior retrocentral, and the hinder end the anterior occipital. So im- portant are the ascending convolutions on either side of the fissure of Rolando, that we are beginning to put them together and call them the central lobule. This must not be confounded with the central lobe, or Island of Reil. On the median surface of the hemisphere the parietal FIGURE 154- Schematic representation of the right cerebral hemisphere. lobe runs over into the precuneus just above the parieto-occipital fis- sure. The precuneus is continued forward as the gyrus fornkatus, which lies just above the corpus callosum and curves around the knee in front, to terminate in the subcallossal gyrus. Above the fornicate gyrus is the c alio so -marginal sulcus. On the exterior surface of the Occipital Lobe we find the supe- rior, middle and inferior gyri, the first being separated from the parietal lobe by the anterior occipital sulcus and from the second occip- ital convolution by the lateral occipital sulcus. ( )n the median sur- face the cuncus is an important lobule, wedge-shaped, and lying be- tween the parieto-occipital above and the calcarine fissure below. On the under surface of this lobe is the end of the gyrus hippocampi, separated from the occipito-temporal gyrus by the occipitotemporal fissure. The gyrus hippocampi also appears on the median surface of THE XON-NEUROXIC DISEASES 677 the hemisphere just beneath the dentate gyrus and is continued back- ward into the lingual gyrus. The Temporal Lobe is divided into superior, middle and inferior convolutions by superior and inferior temporal sulci. On the mesial surface of the brain the middle tem- poral gyrus seems to run back into the gyrus fusiformis. The front part of the temporal lobe on the median surface is known as the uncus, or gyrus uncinatus. CRAXIO-CEREBRAL TOPOGRAPHY. For the guidance, of the surgeon, it is obviously necessary that the relationship of the underlying fissures and convolutions of the FIGURE 155. Schematic representation of the right cerebral hemisphere, viewed from the left. brain to the various parts of the outside of the skull shouM be known. l>roca, long ago, attempted to determine this by boring small holes through the skull, inserting pegs, then carefully removing the calva- rium and noting the parts of the brain that were damaged. Turner adopted the method of removing small parts of the bone and observ- ing the fissures and convolutions that appeared below. To Hefftler and Bischoff, Fere, Seguin and others are we indebted for very ac- curate knowledge as to this relationship between the brain and the sutures and other points on the skull. The fissures that it is especially desirable to know about in this respect are the Rolandic. Sylvian and Parieto-occipital. The accom- panying diagram shows the relationship. For surgical purposes, however, the marks on the skull are not always pronounced enough to serve as guides, hence other methods 678 THE XOX-NEURONIC DISEASES have been devised by Thane, Seguin, Horsley, Dana, Reid and Fraser. These methods are based upon exact measures, here and there, from definite and fixed points of prominence. All one needs is a steel, pliable tape measure and a blue pencil or brush with iodine on it. The head, of course, must be shaved. The innumerable instruments like the cyrtometer, encephalometer, may be dispensed with. FIGURE 156. Schematic representation of the base of the cerebrum. To locate the fissure of Rolando, the distance from the glabella to the inion has to be measured. About two centimeters behind the middle point on this line, or more accurately 55./ per cent, of the whole line measured back from the glabella, will be found the upper end of the fissure. In adult men this point generall) measures about 48 mm. behind the bregma, in women 45, and in children from 30 to 42, according to age. A line running downward and forward, at an angle of 67 degrees with the sagittal lino, will correspond with the tipper two-thirds of the fissure. The lower third is a trifle more vertical. THE XOX-XEUROXIC DISEASES 6/9 The end of the fissure below will just about touch a line drawn from the stephanion to the asterion. Bennet and Godlee's method for determining the Rolandic fissure is somewhat different and is based upon the views of Reid, largely. A vertical line is erected from the anterior border of the external audi- tory meatus. Measuring about two inches back of the point where this line cuts the glabella-inion-sagittal line, a parallel line is drawn vertically downward again and beside the posterior border of the FIGURE 157. Outline sketch op the skull, with the position of some of the principal cerebral sulci marked upon it £ Sy, Sylvian fissure; Sy.a., Sy.p., its anterior and posterior limbs ; /.J?., furrow of Rolando ; tr.fr. f., transverse frontal furrow ; i.par.f., intraparietal furrow; p.o., position of the parieto-occipital fissure; par.j. parallel furrow; m.t.s.f., middle temporo- sphenoidal furrow. ( Modified from Thane. ) mastoid process. Where the posterior line touches the sagittal the upper end of the fissure is found. Its lower end corresponds with a point on the anterior vertical line about two inches above the upper border of the external auditory meatus. After the Rolandic fissure has thus been determined, it is an easy matter to establish the relative position of the central convolutions and their particular motor areas. The fissure of Sylvius, running horizontally, lies usually just a little above the parieto-squamous suture. A line following the external orbital process, the parieto-squamous suture and the parietal eminence will pretty nearly outline its course. The fissure is higher up and more oblique in children. The point of union between the ver- 68o THE NON-NEURONIC DISEASES tical and horizontal limbs of this fissure is, according to Merkel, about four or four and a half centimeters above the middle of the zygomatic arch. The parieto-occipital fissure is determined by first finding the lambda, marking a point 3 mm. in front of it, drawing a line at right angles to the sagittal through this point, and then measuring about 2.5 cm. on this line either side of the sagittal point. The frontal lobe lies almost entirely behind the frontal bone. Behind the parietal will be found the central convolutions the parietal lobe and a part of the occipital lobe. The parietal boss con- ceals the supra-marginal gyrus. The greater part of the temporal lobe is behind the temporal bone. Ofttimes it is desired to reach the ventricles. Usually there are several ways of doing this. Keen recommends the lateral route. Draw a base line through the lower border of the orbit and the ex- ternal auditory meatus. At a point on this line Ij4 inches behind the meatus erect a vertical line. A point on this vertical line \]/\ inches above the base line is where the trocar may be introduced. The in- strument should be plunged, after trephining, of course, into the brain in the direction of a point 2^ or 3 inches vertically above the oppo- site external auditory meatus. From 2 to 2V4 inches in the ventricle will be penetrated. The Minute Structure of the Brain. — I have enumerated the main anatomical parts of the brain. It is now necessary to discuss its minute structure. Like all other parts of the nervous system, this consists of a network of neurones, supported by sustentacular tissue consisting of neuroglia, connective tissue, bloodvessels, lymphatics and ependymal epithelium. The brain differs from the cord not in the character of its ele- ments but merely in the arrangement of them and the relative quantity of gray to white matter. The neurones are the same kind of neu- rones ; are bunched together in the same way ; have various sized processes ; and are distributed in relation to one another in contiguous series in the same way. It hardly needs to be said that the susten- tacular elements are exactly the same as those found outside of the cranium. It is obvious, therefore, that little need be said upon the constituent elements of the brain beyond what has already been said in the chapters devoted to the neurone and the neuronic architecture. Some consideration must be given, however, to the arrangement of these constituent elements in the encephalic mass. As in the cord, so here we find gray and white matter. The gray matter is spread out on the surface in the form of the cortex and de- posited here and there in clumps or bilateral masses all through the en- cephalon. The white matter appears in a large area just beneath the cortex and in streaks and radii here and there between the masses of gray matter, and between the brain and the parts of the nervous sys- tem outside of the head. It is very clear then that we have ganglia here, just as we have in the cord, consisting of little accumulations of neurone bodies in special spots and bundles of neuraxones making up the pathways along which impulses of all sorts travel between dif- THE XOX-XEURONIC DISEASES 681 ferent parts of the brain and between the brain and the rest of the nervous system. At a first glance we discover that the brain contains the following" deposits of gray matter or ganglionic collections of cells — viz., cerebral cortex, corpora striata, olfactory lobes, optic thalami, corpora genicu- lata, corpora albicantia, Luy's body, corpora quadrigemina, red nuclei, substantia nigra, cerebellar cortex, cerebellar nuclei, pontile nuclei, olivary bodies, nodal nuclei and the cranial nuclei. The processes of the cell bodies which enter into the composition of these ganglionic masses make up three great groups of tracts which FIGURE 158. Diagram to illustrate the association, the brain. 'OmmtSSUral, and project ion tracts of constitute the white matter. They are the association tracts, the commissural tracts and the projection tracts. It now behooves us to analyze these ganglia and tracts a little more in detail. I would again urge the reader to note the great similarity, so far in our account, of the brain with the spinal cord. Cortex. — If the much infolded cerebral cortex were spread out smooth, as you would smooth out a handkerchief, it would be a thin sheet of gray matter, with an area of about three hundred square inches, and a fairly uniform thickness of about one-tenth or one-twelfth of an inch. Therefore the convolutions, which, by the way, correspond at the seventh month with those of the adult baboon, are seen, when the 682 THE NON-NEURONIC DISEASES brain is sliced transversely, to have a narrow gray border following all of their changes and detours. Running through the middle of this gray border, parallel to its edges, is a fine white line. In the vicinity of FIGURE 159. *■■ ■ m 8! Kit .1 '',-"■, 'k|> i-.',.|. " ■, ■''$•■ f'^ I ■''■■ ■■•".■"•■^v>"-: HUMAN CEREBRAL CORTEX. Central convolution. A, medullary substance; b, layer of swindle cells; c, radial bundles; d, giant pyramidal cells; e, large pyramidal cells; f, stratum of Baillarger; g, small pyramidal cells; h, tangential fibres; i, pia mater. Occipital lobe. A, medullary substance; b, layer of spindle cells; c, inner granular layer; d, layer of large pyramidal cells; e, miter granular layer: f, layer of Vici) d'Azyr; g, small pyramidal c Us; h. tangential film s. Conni ammonis. A, ependyma of lateral ventricle; b, medullary layer of alveus; c. layer of spindle cells; d. large pyramidal cells; e, stratum radiatum; f, layer of lacunae; g, small pyramidal cells; h, tangential fibres. Fascia dentata Tarini. A, spindle cells and nerve-fibres; b, large pyramidal cells; c, stra- tum granulosum (small pyramidal cells). (After llenda.) the calcarine fissure it is so distinct that it can be seen with the naked eye. It is here spoken of as the white line of Vicq d'Azyr. Under the microscope, here and in other convolutions, a second inner and finer THE NON-NEURONIC DISEASES 683 line can be seen. It is known as the inner line of Baillarger. These lines at once demonstrate to us that the cortical gray substance is ar- ranged in layers. With a high power of the microscope it is observed that the gray lines represent the massing of cell-bodies, whereas the white striae are caused by the predominence of their processes. Five or seven different layers of cells have thus been differentiated in the cortex, and the effort is being made to assign to them respectively par- ticular functions. At the edge of the cortex, just beneath the pia mater, is a layer of neuroglia with only a few small neuronic cells in it. A great mass of fibres run through it parallel with the surface, for which reason they are spoken of as the tangential fibres. Just below the tan- gential fibres are the stria of Bechterew and Kaes. Xext below this come the small pyramidal cells, making up the ambiguous layer of Andriezen and the second general layer, counting the layer of tangential fibres as the first. The striae of Bechterew and Kaes make up the upper half of the second or ambiguous layer. Nu- merous small round cells mingle with the small pyramidal cells of this layer. Between the second and the third layers there is a superradiary network. The third layer is a very broad one, presents in its middle the appearance known as the striae of Baillarger, and is commonly called the large pyramidal cell layer. These cells are the largest in the cerebral cortex, are most abundant in the Rolandic area, and being triangular or pyramidal in shape, have their apices pointing in the direction of the tangential layer and send off from their bases great long neuraxones which run to the lowest depths of the cortex and out into the underlying white substance. From the apices project upwards long dendrites and from the sides shorter dendrites. The cells increase in size as we proceed downward in this layer, until we meet with the very largest of them at the bottom. Between this large pyramidal layer and the next, or fourth layer is an interradiary network. In the upper part of the fourth layer the cells suddenly appear small and spindle-shaped and hence the layer has been spoken of as the spindle cell layer, or layer of polymorphous cells. The lower part of this layer assumes a markedly fibrous appearance and has been recog- nized as being made up of Meynert's intra-cortical association fibres. Immediately beneath the last and fourth layer comes the white substance of the brain, made up largely here of the subcortical as ciation fibres. To recapitulate, then, the cerebral cortex from without inward is seen to be made up of the four great layers, the tangential, the small pyramidal, the large pyramidal and the spindle-celled. The other layers mentioned are mere subdivisions of these. Neurones and sustentacular tissue are the constituents. Nothing need be said further of these than what has already been mentioned. The large cells seem to be emissive or motor in function and are most abundant in the motor areas ; the small cells are probably receptive OT sensory and are found more numerous in the sensor) areas. This. of course, produces variations of a secondary sort in the appearance of the cortex. It is well, however, to keep in mind its more or I 68 4 THE NON-NEUROXIC DISEASES FIGURE 160. Tangential fibres. Stria? of Bechterew and de> Kaes. Superradiary network (of the. / second and third layers). Striae of Baillarger. Interradiary network Cof tho third and fourth layers). Meynert's infracortical association fibres. Subcortical association fibres. Ram C o , r y 1 ci < ja;' i ; gram ° f tHe diffCm,t byerS ° f "" nnbni a "'" ^ After THE NON-NEURONIC DISEASES 685 uniform four-layer appearance and not to confuse one's self with too fine a secondary subdivision of it. The directions in which the cells lie, their shapes and the minute course of their processes are morpho- logical facts that as yet have no special clinical significance. The primary fissures in the cerebral cortex average a little less than an inch (20 to 23 mm.) in depth. The total area of the cortex, according to Donaldson's careful estimation, is 2,352 sq. cm. Of this the frontal lobe has 41 per cent.; the parietal 21 per cent.; the tem- poral 27 per cent. ; the occipital 10 per cent. ; and the island of Reil 1 per cent. It is roughly estimated that the total area of gray matter in the sulci is about twice that on the surface. After correcting, by recent data, the errors of judgment of Hammaberg, Meynert, Wagner and Donaldson, and supplying what they had obviously omitted, Thompson has estimated the number of cells in the cortex to be 9200 millions. This is about 0.23 per cent, of the number of cells, by Francke's estimation, in the whole human body. This is undoubtedly the most accurate calculation of these marvelous elements that has ever been made. This enormous number of functional nerve cells makes up only 1.37 per cent, of the total volume of the cortex. These cells vary considerably in size. The small pyramidal and spinal cells measure from 1-1200 to 1-600 of an inch. There are many small round cells no larger than 1-2500 in. In the central and para- central convolutions may be seen the huge giant cells of Betz, which attain the size of 1-500 to 1-200 in. in diameter. These various types of cells are believed to have distinctive functions. In the second and granule layers are the small receiving cells. The spindle cells and cells of Cajal, nestling partly in the first and partly in the fourth layer, are believed to be associative and coordinat- ing in function. The large pyramidal cells in the third layer are almost certainly emissive cells. They resemble the large motor cells in the anterior horns of the cord. They are seen most abundantly in the motor areas of the cortex, whereas the smaller cells predominate in the sensory areas. A curious feature of these large, deeply-seated, pyra- midal cells is that their dendrites extend upwards a long distance and end in the tangential layer in dense, profuse ramifications. From these small, terminal branches a series of minute secondary twigs, each end- ing in a little knob, project laterally. The opportunity is thus afforded for countless possible combinations between the dendrites of the cells, such as a speculative psychologist would have never dared to imagine. The fibres of the cortex I have already indicated as the tangential and the striae of Uaillarger and the striae of Bechterew and kaes. To these should be added the superradiary, the interradiary layers and the intercortical and subcortical association fibres. They of course are all neuraxones from their respective cells. There are transverse or rather vertical fibres that come in from the sensory projection system and carry afferent impulses or pass out and carry efferent impulses. The association fibres are both long and short and represent the proa of the cells reaching over to arlx>rize around the cells in the next con- volution or more remote parts of the cortex. The short fibres pass around under the fissures, while the long run in all imaginable direc- 686 THE NON-NEURONIC DISEASES tions through the white centrum semiovale. The projection processes that pass in and out of the cortex are arranged in little bundles in such a way as to give the latter a columnar appearance. Olfactory Lobes. — These are parts of the brain and in structure FIGURE 161. . — Section across the hippocampus major, dentate fissure, dentate fascia and fimbria (after Henle). Gh, part of the gyms hippocampi or uncinate convolution : Fd, fascia dentata or flentate convolution ; between them is the dentate fissure ; Fi, fimbria, composed of ongitudinal fibres here cut across ; 1, 2, medullary centre of the hippocampal gyrus pro- longed around the hippocampus, H, as the so-called alveus, into the fimbria ; 3, layer of large pyramidal cells ; 4, stratum radiatum ; 5, stratum laciniosnm ; 6, superficial medullary lamina, involuted around the dentate fissure ; **, termination of this lamina, the fibres here running longitudinally; 7, superficial neuroglia, of the fascia dentata; *, stratum granulosum. reveal the typical cortical arrangement of the neurones. They have been termed the rhinencephalon and are developed from a secondary division of the first cerebral vesicle. The olfactory apparatus is a much atrophied affair in man. It includes, when traced from before back- THE XOX-XEUROXIC DISEASES 687 wards, the olfactory bulb with its peripheral processes, the olfactory tracts and their lateral roots, with the gray root or trigonum between them, the hippocampal convolution, cornua ammonis, part of the con- volution of the corpus callosum, the nerves of Lancisi and the anterior commissure. In addition to all this there are connections between the olfactory apparatus and the interbrain. FIGURE 162. Cortex of the olfactory bull) of the rabbit, showing tin- olfactory fibres, the stratum glomerulosum, the bipolar granule-cells, the layer of large' pyramidal eel'-, the externa; granular layer, the radial fibres, the internal granular layer and the medullary layer, from above downwards. (After Benda.) The medulla olfactorius, constituting the olfactory field, arises for the most part from the bulbu s. One tract, the olfactory bundle to the cornua ammonis .has been outlined. Another tract from tin- olfac- tory field runs backward and cm be followed into the region of the corpora albicmtia. It passes through the ventral regions of the Cor- pora striat'i. bnt receives no fibres from them. Some of these fibres 688 THE NON-NEURONIC DISEASES even go so far back as the region of the interpeduncular ganglia, per- haps even into the fillet. A third tract starts from the lateral parts of the medulla olfac- torius of the olfactory field and passes through the anterior part of the thalami. It then reaches the inner surface of the ventricle and as the taeniae thalami ultimately reaches the ganglia habenulae. Thus as a large complex of ganglia and bundles, running through the greater part of the brain, the olfactory fibres are to be traced as far as into the cerebral cortex on the one hand, and as far as the ganglion habenulae, and possibly the corpus mamillare, on the other. Other ganglia of the midbrain and interbrain stand in close connection with these ganglia. What are called the olfactory nerves are the central processes of FIGURE 163. —Nervous mechanism of the olfactory apparatus, a, bipolar cells of the olfactory apparatus (Max Schultze's olfactorial cejte) ; b, olfactory glomeruli ; c, mitral cells ; d, granule of white layer ; e, external root of the olfactory tract ; f, grey matter of the sphenoidal region of the cortex ; a, small cell of the mitral layer ; b, basket of a glomerulus ; c, spiny basket of a granule ; e, collateral of the axis-cylinder process of a mitral cell ; /, collaterals terminating in the molecular layer of the frontal and sphenoidal convolutions ; g, superficial triangular cells of the cortex : h, supporting epithelium cells of the olfactory mucous membrane. (Ramon y Cajal.) the sense cells of the olfactory mucous membrane in the nose. Passing through the cribriform plate, these processes decussate repeatedly after arriving at the ventral surface of the bulb. The arborization of each process intermingles with the thick branches of a dendritic process from a cell above and thus forms the complex roundish olfactory glomerulus. The cell above gives oft other processes of like nature, but only this one enters into relation with the peripheral cell in the glomerulus. Thus the pathway of smell impulses is seen to consist of a peripheral or pri- mary neurone and a central or secondary neurone. Other cells have been seen, however, in the cortex of the olfactory bulb, but their nature is still somewhat doubtful. These types of granular cells, marked a. b, c, are observed in the accompanying figure lying between the cells first described and the layer of medullated fibres. It will be observed that this illustration of the cortex of the bulb bears a most striking resemblance, upside down, to the cortex of the cerebrum. The layer of mitral cells, called so on account of their THE XOX-XEUROXIC DISEASES 689 shape, send up their neuraxones to become part of the olfactory tract. The granular cells have no axis cylinders, resemble the spongioblast cells of the retina and are merely associative in function. The neu- raxones of the olfactory tract have been traced, as described above, into the olfactory field of the brain. Corpora Striata. — These are two important basal ganglia which, with the optic thalami, are second only to the cortex. They not only modify impulses that pass through them, but they probably originate FIGURE 164. Lobus frontali! Commissar* anterior Pars tecta colomnae (omicis Caput nuclei caudati Pars frontalis capsulae internae Putanien Globus pallid Truncus corporis callosi Comu anterius rentriculi lateralii / Caput nuclei caudati Pars libera colomnae forni Fasciculus [Vicq d'Aij-ii, Nucleus hrpothalamicus [Corpus LutsI' Para occipitalis capsulac interna*^ Fasciculus retro^ flexua [Meynerti; Nucleus ruber tegmenli "~ Stratum grLseum centrale — Corpus g*Dl- -coUtuiD mediate Lobus occioiial ■na longltudinalis lateralis Horizontal section through the brain, viewed from above. In the left half of the picture, the plane of section is about 1 cm deeper than it is in the right half. (After Spalteholz.) subconscious impulses that play an important role Each striate body is divided into a caudate nucleus nucleus. Between the two nuclei dorsally run the sensory and motor pathways to and from the cortex ternal capsule, wherein a great mass of fibres from parts of the cortex are closely brought together. To the corpus is the external capsule and beyond that a gray matter known as the claustrum. in mentalization. and a lenticular it projection . This is the in- widcly separated the outer sid long thin strip of 690 THE NON-NEUROXIC DISEASES The internal capsule is a part of the brain that the clinician must keep always clear in his memory. As so many functional systems con- verge here, a small lesion may cause a wide distribution of sympto- matic manifestations. The arrangement of the pathways through it is relatively constant, so that if the lesion be small enough to affect but one of them we could theoretically definitely localize it. Just behind the genu or knee of the internal capsule course the great motor or pyramidal tracts from the cortex to the cord. In front of these, and therefore at just about the knee, are the facial and hypoglossal fibres. Behind the pyramidal tracts, in what is called the tegmental radiation, pass the sensory fibres with the pathway from the occipital to the beginning of the optic nerve most posteriorly. The other special sense FIGURE 165. Stria longitudinalis medial is Stria longitudinalis lateralis Truncus corporis callosi Cauda nuclei caudal Stria terminalis Vena terminalis Corpus fornicis Stratum zonale tbalami Lamina medullaris thalanii Nucleus anterior thalanii Nucleus lateralis thalanii Nucleus medialis thalanii Fasciculus thalamo- mamillaris [Vicq d'Azyri] Massa intermedia Capsula interna Nucleus lentiformis Hypothalamus Pedunculus cerebri Tractus opticus Sulcus corporis callos / Lamina chorioidia epitheliaHs Pars centralis ventricuJi lateralis Plexus chorioideus ventriculi lateralis Ependyma ventriculi Lamina affix a Taenia cborioidea Taenia fornici Tela chorioidea ventriculi tertii Plexua chorioideus ventriculi tertii Taenia thalami 'entriculus terliilS Nuclei corporis mamillaris' Frontal section through the middle of the third ventricle. (After Spalteholz.) paths are shown clinically as probably also passing through this part of the capsule. The caudate nucleus is anatomically divided into the head in front and the tail behind. The tail curves round the brain stem and can be traced almost to the inferior horn of the lateral ventricle. The head of the nucleus is related to the gray matter of the anterior perforated space. The nucleus is in continuation with the amygdalum of the cortex in the temporal lobe, the claustrum and the outer part of the lenticular nucleus. The lenticular nucleus is also in relation with the anterior per- forated space. It is significantly divided into the put amen, or outer portion, and the globus pallidus, or inner part. The putamen and caudate nucleus enter into the formation of the fibre system of the corpus striatum and thus constitute a rudimentary but biologically very ancient part of the central nervous system. By a few association fibres the corpus striatum is connected with the cortex above. By an abundant set of fibres which originate in its own cells it is brought THE XOX-XEUROXIC DISEASES 69I into physiological association with various parts of its own substance and with parts below it. The cortical fibres that pass directly through it have been already referred to in the description of the internal cap- sule. The nucleus is in intimate connection with the corpus sub- thalamicum, optic thalamus, inferior olivary bodies and cerebellum and cranial nerve nuclei in the medulla. The putamen-caudate-nucleus is also in close union, by means of connecting fibres, with the globus palli- dus. The cells in this part of the brain measure from 1-1800 to 1-500 FIGURE 166. 1 rum us lorporis ( alio Septum ik.'11ii Pars centralis ventriculi lateral Caput nuclei t-andal Corpus fom Caaeola i Gyrus frontalis superior Bndiatio corporis eallosi Padialio corporis Capsola Corpus manitllare Foraiuon raeru Nucleus olivari« inferior I'vramis inedullae oblongatae Decussalio pyramid Vertical section through the brain, viewed from in front. In the left half of the cerebrum, a second cut was made parallel to the first and a plane presented to view somewhat farther dorsalward. (After Spalteholz.) in. in diameter. Some of them are very large and are found in the lenticular nucleus. Optic Thalami. — These are two large collections of nuclei that are on either side of the third ventricle. They are connected by the middle gray commissure. They form part of the walls of the lateral ventricles. As constituting the greater part of the interbrain, the thalami lie just behind the cerebrum. Their constituent nuclei are not sharply sep- arated from one another. They are referred to as the mesial, lateral and anterior nuclei. The pulvinar is the entire posterior part of the thalamus and, like a tumor, projects into the ventricle. ( )n the median border is the ganglion habenulce. Lying against the ventral and ex- ternal surface of the pulvinar and projecting far into its substance is 692 THE NON-NEURONIC DISEASES the peculiar grayish lateral geniculate body. It gives origin to many fibres of the optic tract. The thalamus is in connection with the corpus subthalamicum, the red nucleus and the corpora albicautia beneath it and with the geniculate bodies postero-external to it. The corpora quadrigemina are also in connection with it. The cells of the optic thalami are multipolar, rather large, and loosely grouped together. There is also a stellate, small cell. The associative Golgi type of cell is also found. The neuraxones from these cells pass up to the cortex. In it are also the terminal arboriza- tions of neuraxones of cells lying in the cortex above and in the sensory cranial and spinal nuclei below. The associative cells serve as the connecting links between these afferent and efferent paths that meet in the thalamus. Its anatomy indicates that this ganglion is a most important way station between a great mass of inpouring and out- FIGURE 167. — Scheme of the retinal elements. A, cones of the fovea centralis ; B, granules (nuclei) of these cones ; C, synapse between the cones and bipolar cells in external molecular layer ; D, synapse between the bipolar and ganglion cells in the internal molecular layer ; a and b, rods and cones in other regions of the retina ; c, bipolar cell destined for the cones ; d, bipolar cell destined for the rods ; E, e, ganglion cells ; {, spongioblast; g, efferent fibre (? trophic), originating from the cell m, in geniculate ody ; h, optic nerve ; i, terminal arborisations of optic nerve fibres in geniculate body ; /, fibre3 from the cells of geniculate body on the way to cerebral cortex. (B,. y Cajal.) going impulses. It is preeminently and first of all a great "receiving station" for fibres from the cortex and for fibres from the corpus striatum. In proportion to its great mass, it sends an exceeding small number of fibres downward. Optic Tracts. — As these are not nerves, but actual tracts of the brain, it will be appropriate to speak of them in this place. The retinal cells, like the olfactory cells and the spinal sensory cells in the posterior ganglia, send their central processes in to unite with the next set of lieu rones. The retina is a purely nervous structure and is made up of three layers, which are subdivided into six. The three primary layers are those of the visual cells, the bipolar cells and the ganglion cells. Each of these layers is divided into two again, as follows, layer of rods and cones and external granular layer, external molecular and internal granular layer, internal molecular and ganglionic layer. The neu- raxones of this last layer of cells become the optic tracts, each tract containing about 500,000 of them. The rods and cones are merely the THE XOX-XEUROXIC DISEASES 693 highly differentiated peripheral processes of the bipolar cells that make up the external granular layer. The central processes of these remark- able peripheral neurones pass inward to'come into apposition, by means FIGURE 168. ^.^ X. \ l ^ = ~~ V- Y J : (-"MS A_L •' K MP0RA || V = ===3 ^n *E ^=< * '8U*' — Diagram of visual paths. (From Vialet, modified.) OP. N., Optic nerve. OP. C, Optic chiasm. OP. T.. Optic tract. OP. R., Optic radiations. GEN., Genic- ulate body. THO., Optic thalamus. C. QU., Corpora quadrigemina. C. C. Corpus callosum. V. S., Visual speech centre. A. S., Auditory speech centre. M. S„ Motor speech centre. A lesion at 1 causes blindness of that eye; at 2, bi-temporal hemia- nopia; at 3, nasal hemianopia. Symmetrical lesions at 3 and 3' would cause bi-nasal hemianopia; at 4, hemianopia of both eyes, with hemianopic pupillary inaction; at 5 and 6, hemianopia of both eyes, pupillary reflexes normal ; at 7, amblyopia, espe- cially of opposite eye ; at 8, on left side, word-blindness. of their arborizations, with the second bipolar-cell layer, whos es have more or less of a horizontal direction. The ganglionic layer contains large horizontal cells, which are, according to Cajal, spon- 694 THE NON-NEURONIC DISEASES gioblasts. There are in the retina the terminal arborizations of cells embedded in the optic thalami, corpora geniculata and anterior corpora quadrigemina. These neurones, of course, carry the impulses from the primary optic centers to the retina, whereas the previous cells carry the impulses to the optic centers from the retina. The optic tracts partly decussate in the optic chiasm and then curve backwards around the crura to divide into the lateral and mesial root and ultimately terminate in arborizations in the primary optic FIGURE 169. — Section through the superior part cp one op the superior corpora QUADRIGEMINA AND THE ADJACENT PART OP THE OPTIC THALAMUS (after Meynert). 5, aqueduct of Sylvius; gr, grey matter of the aqueduct; c.q.s, quadrigeminal eminence, consisting of : I, stratum le'mnisci ; 0, stratum opticum ; c, stratum cinereum ; Th, thalamus (pulvinar) ; c.g.i, c.g.e, intei-nal and external geniculate bodies ; br.s, br.i, superior and inferior brachia ; /, fillet; p.l, posterior longitudinal bundle; r, raphe ; III, third nerve ; n. Ill, its nucleus ; l.p.p, posterior perforated space ; s.n, substantia nigra ; above this is the tegmentum with its nucleus, the latter being indicated by the circular area ; cr, crusta ; II, optic tract : M, medullary centre of the hemisphere; n.c, nucleus caudatus; st, stria terminalis. centers — namely, the pulvinar, the external geniculate bodies and the anterior corpora quadrigemina. In the latter nuclei these optic fibres are brought into association with the oculomotor nerve, and thus is explained the reflexes of the eye. Moreover, these primary optic cen- ters are in connection with other cranial nerve nuclei in the medulla. The processes of the cells of these primary optic centers pass through the extreme posterior part of the internal capsule and c tinning on as the optic radiations of Gratiolet ultimately terminate in the cortex of the occipital lobe, chiefly the aniens. By reason of the partial decussation of the tract at the chiasm, the temporal half of each retina is connected with the occipital lobe of the same side, the nasal half with the lobe of the opposite side. This is shown in the accom- THE NOX-XEUROXIC DISEASES 695 panying diagram from Yialet. The fibres that connect the two tracts behind, running as a commissure between the primary nuclei, are not shown because they are of no significance clinically so far as we know. Regio Subthalamica. — This is a very complicated region just be- neath the optic thalami and contains a number of small grayish ganglia whose functions we do not know much about. The nucleus tegmenti, or red nucleus of the tegmentum, is highly vascular and rich in cells. External to this lies the lenticular-shaped Luys' body or corpus sub- thalamicum. It has a few cells, but rather abundant plexus of fibres. Somewhat mesial to Luys ? body is the substantia nigra of Somer- ing, an accumulation of grayish pigmented cells of rather large size. Dorsal to the substantia nigra is the tegmentum, or sensory part of the FIGURE 170. "TV o nrt o -T a nt i I e Transverse section through the corpora quadrigemina and cerebral peduncles ; diagrammatically represented, to show the relative positions of the prominent tracts. cerebral peduncles, while ventral to it is the pes or motor part con- taining the great fibre system of the motor cortex and capsule. A.S far as the end of the midbrain can the substantia nigra be demon- strated above the pes. In it terminates the last remnant of the fibre system from the corpus striatum. From the posterior part of the red nucleus a fibre system can be traced to the opposite half of the cerebellum. This is an important tract, the tr actus tegmento-cerebellaris. Corpora Quadrigemina. — These art- four tubercles just behind and l>eneath the optic thalami, two in front and two behind. Beneath them is the third ventricle and the upper end of the aqueduct of Sylvius. On top of them rests the epiphysis cerebri or pineal gland, which in some low forms of life has all the appearance of an atrophied primitive eye. It is sometimes called the conarium. It contains solid epithelial tubules, 696 THE NON-NEURONIC DISEASES an abundant blood-supply and the so-called brain-sand. This remark- able structure in several selachians and in many reptiles passes through a hole in the skull to a sense organ under the skin. In this organ we can see a cornea, a lens, a retina, and below this a pigmental layer. In the higher forms of life it has become useless and withdrawn into the head. Immediately in front of the pineal gland is the ganglion habenulce, which appears to receive fibres from the olfactory field by way of the tcenia thalami. From the anterior corpora quadrigemina arise part of the optic nerves. Thus we see three ganglia in this immediate neighborhood FIGURE 171. SJratum zonalc thalami Pulvinar Stratum griseum colliculi superior corporum quadrigeminorum Stratum zonale Corpus pineale Commissura posterior [cerebri] Stratum griseum tffi i centrale Aquaeductus cerebri [Sylvii] Forniatio reticularis (Nucleus lateralis superior) Nucleus n.oculomotorii- Fasciculus longitudi- nalis medialis Decussatio tegmenti dorsalis Nucleus ruber tegmenti Ganglion interpedunculare Capsula interna Corpus gcniculaturo mediate Lemniscus medialis [sensitivus] . Tractus opticus N. oculoqnotorius Pedunculus cerebri Substantia nigra Section at the level of the root of the oculomotorius nerve. (After Spalteholz.) serving as the origin of the optic nerves — namely, the pulvinar, the anterior corpora quadrigemina and the external geniculate bodies. They all receive fibres from the occipital lobe which come by way of the optic radiations and the posterior part of the internal capsule. These are the fibres of Gratiolet. The posterior quadrigeniiiial bodies receive fibres from the tem- poral lobe. The great development of these posterior bodies in whales leads to the supposition that they have something to do with the audi- tory nerve, by way of the nucleus acusticus. The maintenance of equi- librium, it must be remembered, is one of the functions probably of the semicircular canals. The anterior lobes of the quadrigeminal bodies picsent a micro- scopic appearance resembling layers of the cortical type, according to Spitzka. Outside are optic tract fibres, then a thin layer of small nerve-cells, then again some optic fibres and finally a layer of a few large cells. The posterior loins arc less striking in appearance and THE XOX-XEUROXIC DISEASES 697 merely contain some small cells and one nucleus filled with a network of fine fibres. The two posterior ganglia are connected by fibres that pass over the aqueduct of Sylvius. Beneath the corpora quadngemina are the peduncles of the brain, the aqueduct of Sylvius separating the corpora from the latter. I have already spoken of the substantia nigra lying in the middle and stretching transversely across each peduncle. The part above the substantia is the tegmentum; that below is the pes pedunculi. The glossopharyngeus N. vagoa. Nucleus n. cochlears dorsali [Nucleus tuberculi acustici] Tractus spinalis and nucleus tractus spinalis n. trigeiuini Nucleus n. vestibularis spinalis [Radix descendeo! ' Nucleus alac clnereae, tractus solitarius and nucleus tractus solitarii Ganglion cotnmissurale alaTe cinereat Diagrammatic representation of the nuclei of origin of the motor, and the primary terminal nuclei of the sensory cerebral nerves. (After Held.) substantia seems to be in part the termination posteriorly, in the stratum intermedium, of the fibre-system of the striate body. The real function of the gray matter we know nothing about. The tegmen- tum is the great sensory tract and contains the decussating fibres of the anterior cerebellar peduncle which connect upon Opposite sides the cerebellum and the red nucleus of the tegmentum. The pes pedunculi, below the substantia nigra, contains the great efferent system-fibres from the cortex. According to Dejerine, the fibres in the outer fifth of it arise from the middle part of the temi>oral lobe. The median fifth fibres come from the operculum. The middle three-fifths of the pes contain fibres from the posterior portion of the 698 THE XOX-XEUROXIC DISEASES frontal lobe and the central convolutions. This is the pyramidal tract, or tractus cortico-spinalis. Behind the quadrigeminal bodies, in the central gray matter, ap- pear the first of the cranial nerve nuclei — namely, the collection of cells along side of the aqueduct of Sylvius, that give rise to the oculo- motorius nerve. These nuclei deserve special consideration as local- ization symptoms in connection with them are often very definite. Cranial Nerve Nuclei. — These nuclei or ganglionic masses are in all respects the homologues of the anterior horns of the spinal cord. Anterior median (small- celltd) nucleus Edinger-WestphaV s nucleus [median and lateral groups) Central-median sagittal nucleus Lateral (large-celled) oculomotor nucleus Mixed trochlear oculo- motor nucleus Trochlear nucleus Trochlear nerve- Post, longitudinal fasciculus Trochlear decussation in the velum Grouping of the nuclei of the oculomotor region. Schematichorizont&l section. ( Aiter Siemerliug.) They give origin to motor cranial nerves or to the motor strands of the cranial nerves and, like the spinal motor cells, preside over their nutri- tion. They are to be found along the aqueduct of Sylvius, in the floor of the fourth ventricle and in the deeper parts of the pons and medulla. It may appropriately be mentioned in this connection that the ganglia on the sensory cranial nerves are the homologues of the gan- glia on the posterior spinal nerve roots. In the development of the cephalic portion of the neural crest in the embryo there appear five pairs of ganglia, which gradually shift their position to the ventral side of the brain. They are the jugular, petrosal, geniculate, auditory and Gosserian ganglia. From them originate the sensory parts of the pneumogastric, the glosso-pharyngeal and facial nerves, all of the THE NON-NEURONIC DISEASES 699 auditory, and sensory part of the trifacial. In all of these ganglia, except the auditory, the cells become unipolar. The single process di- vides T-like into peripheral and central fibres, just as they do in the posterior spinal ganglia. The central process assumes all the appear- ances of a neuraxone ; the peripheral that of a modified dendrite. The auditory ganglion moves backward to a point behind the geniculate ganglion. Its dendrites run outward to their termination in the acoustic apparatus of the ear. Its axones penetrate the medulla. The central axones of the other ganglia give of! numerous collaterals, enter the brain and divide T-like. They pass up and down and arborize chiefly in the terminal nuclei of the respective nerves, some of them, the excito-reflex fibres, terminating in the nuclei of the motor nerves. The similarity between the cranial nerves and the spinal nerves is thus made obvious and the contention upheld that the brain in a large measure, at least, is but a highly developed and much rearranged por- tion of the serial ganglia that constitute the entire cerebro-spinal axis. Xucleus of the Motor-Oculi Nerve. — This nucleus lies in the floor of the aqueduct of Sylvius. It consists of a number of subsidiary nuclei that supply the different muscles that rotate the eye ball, that contract the pupil and that elevate the lid. The nucleus is connected with those of the fourth and sixth cranial nerves behind it by means of fibres running in the posterior longitudinal bundle. It is also con- nected with the optic nerve, but just how is still a matter of dispute. Some authors state that the ocular facial nerve strands arise in the distal part of the nuclear area of the third nerve, though this is contra- dicted by Scruff, Cassirer, Siemerling and others. In the third or -oculomotor nucleus the subsidiary nuclei are arranged somewhat thus antero-posteriorly. Median Line. Sphincter iridis. Musculus ciliaris. Levator palpebral. Rectus interims. Rectus superior. Rectus inferior. ( )bliquus inferior. The neuraxones from these little groups of cells enter the third cranial nerve and terminate in the correspondingly named muscles. The same may he said of the nuclei of the fourth and sixth cranial nerves lying still farther posteriori}- and sending their stimulation re- spectively to the superior oblique and external rectus muscles of the eye. Note that the sixth fibres do not decussate; all the others do. The posterior longitudinal fasciculus, that I said a moment ago connected the various subsidiary parts of the third nucleus, also in all probability connect all of the cranial nuclei. It extends much farther f< rward than the third nucleus. In all vertebrates it extends from the interbrain to the region of the anterior columns of the spinal cord, and is thus one of the fundamental features of the brain. In a way it be- longs to the pyramidal system. Trigeminal Xucleus. — The sensory fibres of the fifth cranial nerve arise, as I have stated, from the < rasserian ganglion resting on the petrous part of the temporal bone. The central ends of these neuron,- 700 THE N0N-NEUR0NIC DISEASES ramify in the sensory nucleus of the preoblongata. They divide, just like the central ends of the spinal sensory neurones do in the columns of Burdach, into a descending branch, which forms the spinal or long- root of the trigeminus, and an ascending branch. The collaterals from these up-and-down branches arborize and ramify in the neighboring gray matter, which is continuous with the spinal substantia gelatinosa and surround the cells therein found. It is believed that collaterals also extend to the facial and other cranial nerve nuclei. The pontile por- tion of the nucleus, according to Kolliker, is only the beginning of the gray matter. It is thus made plain how the components of the fifth nerve represent the sensory correspondents of the third, fourth, sixth, FIGURE 174. ™" IV — Sections through the origin of the focrth nerve (Stilling). £ A, transverse section at the place, of emergence of the nerve-fibres. B, oblique section carried along the course of the bundles from the nucleus of origin to the place of emer- gence. Aq, Sylvian aqueduct, with its surrounding grey matter ; IV, the nerve-bundles emerging ; IV\ decussation of the nerves of the two sides ; IV", a round bundle passing downwards by the side of the aqueduct to emerge a little lower down ; ?i, IV, nucleus of the fourth nerve. /, fillet; s. c. p., superior cerebellar peduncle; d.V., descending root of the fifth nerve ; pi, posterior longitudinal bundle ; r, raphe. motor branch of the fifth, the seventh and the twelfth nerves. Instead of remaining separated as they are in the cord below, the sensory paths have here all become amalgamated into the fifth nerve, while the motor correspondents still remain separated. It is believed from recent investigation that the proximal part of the trigeminal root fibres correspond to the third branch of the nerve, while the distal part corresponds to the first. The motor root springs, of course, from the large-cell motor nu- cleus. There is also a cerebral root to this nerve, called the mesen- cephalic, because it has its origin in the pregeminal region just in front of the aqueduct of Sylvius. This is probably a motor root. Facial Nucleus. — This is a large-cell nucleus, homologous to the anterior horn of the cord, and lies mesially to the spinal trigeminal in the lower part of the pons. After joining, the processes from its cells curve in a bundle around the sixth nucleus and then continue obliquely outward and downward between the facial nucleus and the trigeminal root. The two facial nuclei are connected by commissural fibres. The nerve of Wrisberg has its origin apparently from the ganglion THE X0X-XEUR0X1C DISEASES 701 ^eniculi and is thus a part of the sensory glossopharyngeal root. By this nerve the chorda tympani becomes united with the glosso-pharyn- _geal nerve or the solitary fasciculus. Thus is explained the visceral function of the facial nerve as seen in the taste phenomena related to the chorda tympani. The facial nucleus belongs to the series of the motor nuclei of the ninth, tenth and eleventh nerves, which represent the upward prolongation of the lateral and anterior gray matter of the cord. The FIGURE 176. a0& Fare? Cross-section through the medulla at the height of the entrance of the acoustic ( Enlargement .1.) VHP, ventral acoustic nucleus; VIII*, dorsal acoustic nucleus; N. C, cochlear nerve ; Pc, cerebellar peduncle (restiform body) ; R.d. VIII, descending acoustic root ; Slr.ac. , acoustic stria ; co, cerebello-olivary fibres, etc. (After Kolliker.) nucleus receives a cortical stimulus by way of the fibres that course- down through the anterior part of the posterior limb or knee of the internal capsule. These fibres lie in the crusta to the inner side of the pyramidal tract. They decussate before reaching the nucleus. 702 THE N0N-NEUR0NIC DISEASES THE XOX-XEUROXIC DISEASES 703 Auditory Nuclei. — Innervating the cochlea and semicircular canals of the internal ear, the auditory nerve contains fibres that subserve different functions — namely, hearing and space-sense appreciation, and hence has two sets of nuclei. The nerve enters the medulla by the two roots, lateral or posterior and median or anterior. These roots are connected with three nuclei. The cochlear root processes terminate and arborize in the accessorius, or ventral acoustic nucleus and acoustic tubercle that lie in the substance of the lateral root and between it and FIGURE 177. Nucleus n. vesti- bularis superior _ (Fleebsig, Bechterew) Nucleus n. vestibular! inediMU [Schwalbe Format io reticularis- Corpus restiformc Radix n. vestibularis and nucleus n. vesti- bularis lateralis |Deitcrs| Radix n coctalearis Traclus spinali>_-. ~]^H d. irigeuiiDi 1 - - ' n. facialis j . J inKrolivarc^-^^^^A ■■ Hk ^Kk lemnisci - ~^^^^B lA^fl ^JjfX. affi y >■!<= f V r\f Radix vestibularis .Nucleus arcuntus Nucleus oUfMla tnfc Pons [Varuli] rior Pyrarnis [nicdul'ac qbl<>". Section through the middle of the cerebellum and through the medulla oblongata. (After Flechsig and Held.) the median root. They are the pathway of the auditory impressions and arise in the spiral ganglion of the cochlea. They probably connect somewhat also with the other ganglia or nuclei. The vestibular root processes originate in the ganglion searpae <>f the labyrinth and instead of hearing, have the function of sending coordinating impulses to the central organ, particularly the cerebellum. Their central arborizations are in the dorsal central inner nucleus or chief nucleus and the large cell nucleus of Dieter and the continuation of this in the vestibular 704 THE N0N-NEUR0NIC DISEASES nucleus of Bechterew. This vestibular pathway is not only continued up into the cerebellum, but it seems to run down towards the spine in a tract which is a continuation of the funiculus cuneatus (Burdach's column). It is known as the descending acoustic root. The acoustic strict seen running across the floor of the fourth ventricle is a sensory tract from the acoustic tubercle that decussates in the raphe and con- tinues on into the lateral lemniscus. The accessory nucleus sends its cell processes up through the lateral lemniscus to the posterior corpora quadrigemina and so on to FIGURE 178. Posterior view of the medulla oblongata, the fourth ventricle and the severed cerebellum. 1, fourth ventricle; 2, striae acusticse; 3, posterior cerebellar pedun- cle; 4, fasciculus gracilis; 5, anterior cerebellar peduncle; 6, fillet; 7, middle cere- bellar peduncle; 8, corpora quadrigemina. (Affer Hirschfeld and Leveille.) the first and second temporal convolutions. Some fibres pass directly through the lemniscus and tegmentum to the temporal lobe without going to the posterior corpora quadrigemina. This nucleus connects also with the superior olivary bodies, with other cranial nuclei, and by way of the acoustic stria? with the formatio reticularis, sensory tract, posterior corpora quadrigemina and temporal cortical area. The Dieter's and chief nuclei send their processes to the direct sensory cerebellar tract and thence to the red nucleus of the tegmentum and the emboliform and globose nuclei of the cerebellum. Vago-glosso-pharyngeal Nucleus. — The nuclei of the ninth and tenth cranial nerves cannot well be separated. The sensory branches have the cell bodies of their neurones nesting in their respective ganglia on the roots. From thence the central processes enter the brain, divide T-like and send one branch downward and the other upward. The downward brandies constitute what used to be called the ascending root of these nerves. They give off collaterals. This so- called ascending root is the solitary bundle or fasciculus. It receives the central terminations of the neurones in the root ganglia (jugular, THE XOX-XEUROXIC DISEASES 7"5 petrosal, plexiform, etc.) of the vagus and glosso-pharyngeal nerves. It sends its own processes on up into the brain. The motor branches of these nerves arise from one common nucleus, the nucleus ambigaus. The accessory branch of the eleventh or spinal accessory nerve arises from the nucleus ambiguus. The nucleus is probably homo- logous in some respects to* the column of Clarke. The motor branch of the spinal accessory nerve arises from a group of cells in the an- terior horns of the cervical coid reaching from the first nearly to the sixth cervical segment. Its representation in the upper three segments, however, is the most important. Hypoglossal Nucleus. — This nucleus is the homologue of the an FIGURE 179 II. e a|^H|=^ Section of cerebellar cortex, stained by Golgi's method ; 1 . taken across the lamina; n. in the direction of the lamina; a, outer or molecular layer; b, inner or granular layer ; c, white matter, o, Cell of Purkinje ; b, small cells of inner layer ; c, dendrons-of these cells ; d, axis-cylinder process of one of \heae cells becoming longitu- dinal in the outer layer ; e, bifurcation of one of these ; g, a similar cell lying in the -white matter. (Ramon y Cajal.) terior horns of the cord and of the nuclei of the third, fourth and sixth nerves. It lies near the raphe in the lower part of the floor of the fourth ventricle. Jts cells are large and it has a rich network. hi some lower animals the hypoglossal is a pure spinal nerve. There are some minor nodal nuclei and other small deposits of gray matter in addition to these to be found in the after-brain or medulla. The Olivary Bodies are two large ganglia lying on either side of the after-brain. They connect with the cerebellum, hnt of their func- tions we know nothing. Cerebellum. — The cerebellum lies beneath the posterior part of the cerebrum; consists of a middle and two lateral lobes; and is con- nected with the cerebrum above and tin- COrd below. The two sides the cerebellum are united by pontile fibres. In general structure it i^ not very unlike the cerebrum. The vermis, or middle lobe, and the 706 THE NON-NEURONIC DISEASES S k °3 C «S-S . eS^ 3 o 'C OTI <■< «- — «3 >, OC°« 3 © ^ cS T"*- < *-" Q 0> O 60,3 4JOOC 21 ^ — < O t> •-• *l a,Q > -TJ *£ -£a J2.a m _^ •V8 .?5 <* s sa a, g <~ > 0^: Sm M ^3 0> © £a is N . 2-^ -a .0 ** a> bo 3 — ' c IS l£ a I« tr^ *s v. a ce — t-.' .H* 5 - » c <«« 1- ^ s'3 .2 3 *2 ej 3 SI J- aj 3 t - ° a 5) 60 >»8 fe *? » e3 v Bjg a; ^ 2. .3 bo 3 3 &£ V » 1 6 - — =3.5 — 'S 5 3 i- to « 1 .0 (B 0) — a> Stf O S a -J 2 = • .2 > •• c — a 8 ■O 3 .2 8 |5 X a: 8«m »T — fc-g 1 3 ^£ 1 «* 5 © 35 3 t- V >» C.3 SB THE NON-NEURONIC DISEASES 707 hemispheres are divided into lobe? and lobules and fantastically named. The gray matter or cortical layer on its surface is so minutely and peculiarly folded that with its intervening white matter it gives the appearance of an arbor vitas. In the center of the white substance is the convoluted nucleus called the corpus dentatum, or ciliary body. Other nuclei are the cnibolifonn, fastigium and globosus. Cerebellar Cortex. — This is a remarkable arrangement of neu- rones. With the lew power of magnification it can be seen that there are three distinct layers; externally the zona molecularis, internally the zona granulosa, and between the two the layer of the large cells FIGURE 181. Nucleus lcntlformi! Capsula externa Capsula interim N. cochlear)! Nucleus n. cochlearis ventralis Tnbcrculum acuslicinu Illustration to show the course of some of the more prominenl conduction paths in the brain. (After Held.) of Purkinje. These cells of Purkinje arc among the largest in the nervous system, measuring from 1-800 to [-600 1 1" an inch. They pos- also large round nuclei. Each cell sends a neuraxone down into the zona granulosa, on the way giving off collaterals that bend around, pass upward and break up into branches, around the cell-body. The dendritic processes arc enormously profuse and extend up toward and almost to the edge of the cerebellum. The profuseness of the dendritic elaboration is best seen in a section made in the sagittal plane. Among the profuse dendritic branches are mingle. I thick fibres oi 708 THE NON-NEURONIC DISEASES unknown origin, though they apparently have come in from the white substance crossing the zona granulosa. Among them are other, small. stellate cells, which send their processes here and there parallel to the surface of the cortex and down to the bodies of the Purkinje cells, which they surround with their terminal arborizations in such a basket- like way that they have been called basket-cells. These smaller cells that obviously have given the name molecular layer to this outermost FIGURE 182. Illustration to show the course of the pyramidal tract and of the cerebro- pontile paths; the termination of the auditory conduction path and the indirect continuation of the medial lemniscus and the brachium conjunctivum from the lateral nucleus of the thalamus. (After Held.) part of the cortex serve well as inter-communicating elements between the individual Purkinje cells. The granular layer is made up chiefly of small polygonal cells with large nuclei, measuring about 1-5000 inch in diameter. They have short dendrites with clubbed extremities and a neuraxone. The latter passes up to the molecular layer, where it divides into two branches which run transversely to the axis of the body of the cell. There are in the granular layer also cells of the Golgi type, large, with axis THE XOX-XEUROXIC DISEASES 709 cylinders that divide and subdivide and end in a finely ramifying plexus. The fibre systems of the cerebellum, like those of the cerebrum, are tangential and radiating. The complicated arrangement of the ele- ments of the cerebellar cortex and their character suggest the possi- bilitv of an enormous number of combinations. Hence it reveals a FIGURE 183. Illustration to show the position of the pyramidal tract, the secondary optic radiation, the fronto-pontile path, the temporo-pontile path, the auditory con- duction path, the primary optic radiation, the medial lemniscus, the hrachium COnjunctivum, the lateral areas of the formalin reticularis, and the cortical path from the lateral nucleus of the thalamus. (After Meld.) marvelous condition fur bringing together and coordinating and har- monizing the activities of a greal many diverse elements. The large cells being so comparatively less numerous than the small, W e that the cerebellum is a great receiving or way station for impulses traveling between the brain and the periphery. It adjusts nerve im- pulses and coordinates their further distribution. yiO THE NON-NEURONIC DISEASES The Conducting Tracts of the Brain. — In the brain, as in the cord, the same laws in regard to the degeneration of the neurones when separated from their nutritive centers in the cells hold true. By this means the tracts of the brain and the tracts between the brain and cord have been largely traced. In our stud}- of the minute anatomy of the brain most all of these tracts have been mentioned. It may be well to briefly recapitulate them here. The reader is also referred to the chapters on the neuronic architecture and on the anatomy and physiology of the cord. The calculation has been made that only about one-third of the brain cortex is involved in the projection tracts. The remaining two- thirds do not send processes out to or receive processes directly from the periphery. This two-thirds is intimately inter-connected by asso- ciation, commissural and other intercalated tracts. The motor projection tracts start from the cells of the central con- volutions of the cortex and pass down through the internal capsule to the various cranial nuclei and anterior cornua of the spinal cord, as has been previously explained. It is throughout a crossed tract, though the crossing does not occur at the same place for all parts of it. The parts that pass to the cranial nuclei we cannot trace as clearly as some other parts, but we believe they decussate just before they reach the respective nuclei. Of the remainder of the tract, nine-tenths of it decussates in the pyramidal decussation of the medulla, and one-tenth crosses over lower down in the anterior commissure of the cord. The sensory projection tracts are more complicated and less well known than the motor. Those of the posterior columns of the cord terminate in the nuclei of the medulla found at the top of these columns. Thence the tract continues across to the other side in the lemniscus or fillet decussation (internal arciform fibres, midolivary strata, chief lemniscus), and so on up to the cortex directly or after interruption in the optic thalamus, or possibly also in the lenticular nu- cleus. Another sensory pathway is the antero-lateral, Gowers' column, the chief lemniscus of the medulla and cortex directly or after interrup- tion. It terminates in the central convolutions, parietal areas and gyrus fornicatus. The tracts from the sensory cranial nerve nuclei, the nucleo-cor- tical tracts, are very little known. They decussate in the raphe or perhaps pass through the chief lemniscus. Many are the ways in which sensory impulses reach the brain, and for aught we know, there may be more intermediary neurones than have so far been described in each tract. The cerebellar tracts, the visual tracts and others have all been sufficiently noted when describing the cerebellum, optic nerves, ete. CIRCULATION OF THE BRAIN. Before passing on to the consideration of the physiology of the brain, some note must be taken of its circulation. Including the scalp, the skull and the dura mater, the head and its contents are furnished with blood by way of the external and internal carotids and the verte- bral arteries, the latter f: rming the basilar artery. THE NON-NEURONIC DISEASES 711 The external carotid supplies the outside of the head, sending into the interior of the cranium only meningeal arteries. These meningeal branches include the inferior meningeal, the posterior meningeal, the middle and small meningeals. Of all the meningeal arteries the middle meningeal is the most important, because it is the largest, is most direct in its communication between the outside and inside of the skull, and its area of distribution is related to parts of the brain of great functional significance. The blood returning from the meninges empties into the diploic veins and thence passes to the lower occipital and lateral sinuses. The venae comites carry some of it. The internal carotid has no branches in the neck. It supplies the interior of the cranium by means of its anterior meningeal branches, anterior and middle cerebral, posterior communicating and anterior choroid branches. The basilar artery terminates in the posterior cerebral, anterior, superior and inferior cerebellar and posterior meningeal branches. The inter-communication of the branches of the internal carotid in front and the basilar behind forms at the base of the brain a remark- able anastomosis known as the circle of Willis. This circle of Willis is really a heptagon and reaches from a point in the longitudinal fissure in front of the optic chiasm back to the edge of the pons Varolii. It measures about an inch in width and is an inch and a half antero-pos- teriorly. Its front limitation is formed of the anterior cerebral arteries that have just left the internal carotids and that are united by the transverse anterior communicating artery. Its lateral boundaries are made up of the posterior communicating arteries which form an anas- tomosis between the internal carotids and the posterior cerebral arteries which bound the circle behind. This circle of Willis supplies especially the midbrain and the basal structures. The midbrain receives blood also from the superior cerebellar arteries. The cerebral cortex and white substance, together with the pia mater, receive their vascular supply from the large distal branches of the cerebral arteries. The internal ganglia and interbrain are fur- nished by the small proximal branches. It is both convenient and physiological, therefore, to discuss the blood supply of the cerebrum under the heads of the cortical system and the ganglionic system. The choroid system should also be taken into special account. Cortical System. — Entering the front lower part of the longitudinal fissure, after winding around the genu of the corpus callosum, the anterior cerebral arteries pass backward along the mesial surface of the hemispheres as far as the parietooccipital fissure. From each artery Spring the anterior internal frontal, supplying the internal orbital eon volution, olfactory bulb, superior frontal and anterior half of the mid- dle frontal convolutions; the middle internal frontal supplying the Corpus callosum, the gyrus fornicatUS, marginal gyrus and upper end of the ascending frontal convolution : and the posterior internal frontal, supplying the quadrate lobule and part of Hie superior parietal convo- lution. In the Sylvian fissure runs a most important artery, the middle 712 THE XOX-XEUROXIC DISEASES cerebral. This has four branches. Of these the external and inferior frontal furnish blood to the anterior and posterior orbital and third frontal convolutions ; the ascending frontal and ascending parietal carry it to the convolutions of the same name ; and the parietotemporal supplies it to the supra-marginal, angular, superior and middle temporal FIGURE 184. The arteries at the base of the brain. (After Monakow.) Acera, anterior cerebral artery Coma, anterior communicating artery ; A FSy, artery of the Sylvian fissure ; Alcnt, lenticular artery 1-5, the cortical chief branches of the Sylvian artery ; cp, posterior communicating artery Acha anterior choroid artery ; x, lateral branches of the posterior communicating artery'; Acp, posterior cerebral artery ; Abas, basilar artery ; At, temporal artery ; Aocc, occipital artery , A ccrsup, suwricr cerebellar artery ; A cer med, median cerebellar artery ; A cer ivf, inferior cerebellar artery anterior spinal artery. Three cortical branches spring from the posterior cerebral arteries and cover the tentorial area of the brain. The occipital goes to the aniens and cortex of the occipital lobe ; the uncinate supplies the un- cinate convolution (the hippocampal and lingual areas)"; and the tem- poro-sphenoidal carries its blood to the fourth, third and part of the second temporal gyri. THE XOX-XEUROXIC DISEASES 7*3 Ganglionic System. — This system is constituted of the small ar- teries of the circle of Willis and cerebral arteries. Generally six groups of vessels are recognized. Some of these are of immense importance to the pathologist and clinician. The antero-incdian ganglionic arteries originate in the anterior cerebrals and the anterior communicating. They penetrate the lamina cinerea and a few of them supply the head of the caudate nucleus and anterior wall of the third ventricle. FIGURE 185. re Lateral view of the right hemisphere showiDg the area supplied by the middle cerebral artery. (After Monakow.) Art.F.S., artery of the Sylvian fossa; I-V, the five chief branches of this artery; Ft, central fissure; FS, Sylvian fossa; OP., interparietal fissure; I, first temporal fissure; Fi-F 3 , first, second, and third frontal convolutions ; SM, supramarginal gyrus ; Ang, angular gyrus ; 0i-03. first, second, and third occipital convolutions. The dotted line shows the area supplied by the Sylvian artery. The antero-lateral ganglionic arteries, making up the second and third groups, arise from the middle cerebrals just outside of the circle of Willis. They penetrate the anterior perforated space and carry blood to the corpora striata, internal capsules and optic thalami. 'Hie lenticulo-striate artery of this group, tint supplies the entire striate body, is sometimes called, On account of the frequencj <>!' its rupture, the artery of cerebral hemorrhage. The postero-median ganglionic arteries are branches of the ■ terior cerebral and posterior communicating arteries. The inter- peduncular structures and crustae receive their blood fr-nn them and after piercing the posterior perforated lamina they supply the walls the third ventricle and the mesial parts of the optic thalami. 714 THE NON-NEURONIC DISEASES The fifth and sixth groups are made up of the poster o-lateral ganglionic arteries. They spring from the posterior cerebral arteries after the latter have wound around the crura and are distributed to the posterior parts of the optic thalami, the corpora quadrigemina and the corpora geniculata. These ganglionic arteries do not anastomose with one another or with other cortical arteries . They belong to the Cohnheim type of terminal arteries. On account of the relatively poor blood supply of the brain between the cortical and ganglionic systems, this area is par- ticularly liable to encephalomalacia. The choroid arteries are three in number. The anterior choroids spring from the internal carotids, enter the apices of the descending horn of the lateral ventricles and supply the inferior two-thirds of the choroid plexus, part of the velum interpositum, the hippocampus major and corpus fimbriatum. The posiero-lateral choroids originate in the posterior cerebrals and are distributed to the upper third of the choroid plexus of the lateral ventricles and to the velum interpositum. The postero-medial choroids also start from the cerebral arteries and supply the choroid plexuses of the third ventricle and its velum inter- positum. The velum interpositum also receives branches from the superior cerebellar arteries. Veins. — The vena cerebri magna, common vein of Galen, collects the blood from the velum interpositum, the lateral and the third ven- tricles. The superior cerebral, medial cerebral and inferior cerebral veins drain the surface of the brain and empty into the neighboring sinuses. It may be of some clinical importance to note that the in- ferior cerebral veins, on the tentorial surface of the brain, empty into the petrosal sinuses against the current. Lymphatics are not found in the brain or cord, but there are perivascular lymph spaces. The pons Varolii, medulla oblongata and cerebellum are supplied by terminal branches wholly from the vertebral and basilar arteries. They are called nuclear and radicular arteries and need no special de- scription. The hypoglossal nerve root is furnished with blood by the anterior spinal artery ; the other cranial nerve roots get their supply from branches of the vertebrals. The olives and pyramids are supplied from either the vertebrals or the anterior spinals. The rest i form bodies get their supply from the inferior cerebellars, which also send branches to the roots of the vagus, accessorius, spinal root of the trigeminus and the reticular field generally. PHYSIOLOGY OF Till-; BRAIN AND CEREURAL LOCALIZ \- TION. The credit for the earliest suggestion that the brain is a composite rather than a single organ is usually given to the phrenologist. Gall ( 1 758-1828), whose work Sur les Fonctions du Cervcau et sur Celles dc Chacune de ses Parties appeared in 1825. M. Farabeuf, the dis- tinguished anatomist of Paris, has discovered, however, a work by Joseph Baader, a professor at Freiburg-in>Bresgau from 1740 to 1750. THE NON-NEURONIC DISEASES 715 in which it is clearly intimated that the sensory and motor functions of the brain may possibly be separated and localized. The work is entitled Observationes Mediccc Incisionibus Cadaverwm Anatomicis Illustra- tes, was published separately in 1762, and was reprinted in Sandifort's Thesaurus Dissertationuin in 1778. Notwithstanding these early hints at cerebral localization, the scientific world continued to believe with Flourens (1794-1867) that all the faculties occupied the same place, that to perceive and to will constituted essentially the same faculty, and that this faculty resided in a single organ. The mapping out of the brain surface into sensory, motor and other areas is distinctly a recent advance in physiology ; the explanation of the correlation of these areas, and of the dependence of mind upon brain, is still an unsolved problem. The subject of cerebral localization is one of unequal magnitude, according as it is looked at from the standpoint of the surgeon, the physiologist, or the psychologist. In its consideration the practical bearings have been so generally uppermost it has happened that only those parts of the brain have received the largest amount of study which could be reached by the surgeon's knife. Certain superficial sensory and motor areas have been the most completely established, though these areas probably represent the coarsest and most primitive functions of w T hich the brain is capable. It is obvious that other and deeper parts of the encephalon play an important, if not really a more important role in the tout ensemble of brain activity, and that the local- ization of the functions of these parts is quite as much of a desideratum scientifically as is the localization of the sensory and motor areas of the cortex. The physiologist is called upon to explain the function and presence of the following deposits of gray matter in the cranium, the localization phenomena of some of which are already fairly well understood : Cerebral cortex; olfactory lobes; corpora striata (caudate and lenticular nuclei) ; optic thalami (and pulvinar) ; corpora geniculate (external and internal); corpora mamillaria ; corpora subthalamica ; corpora quadrigemina (anterior and posterior) ; epiphysis; hypophysis; red nuclei ; substantia nigra ; cerebellar cortex ; cerebellar nuclei ; pontile nuclei; corpora olivaria ; nodal nuclei; cranial nuclei. These gray deposits have each their special raison d'etre, and all doubtless play their part in the sum total of brain activity. It is a matter of extreme difficult) and of inten.se interest to define accurately their functions. The more exact diagnosis of focal lesions and the more rational explanation of nientalization both call loudly for a solu- tion of this problem. The question involves more than that of making a mere surgical diagnosis; it involves tin- whole subject of normal an 1 abnormal mental and cerebral phenomena. Physiology must here pro- ceed hand in hand with psycholog) and endeavor to localize the encephalic seat of such manifestations as memory, volition, intellection, imagination, etc., or at least the seat of their highest manifestation. For the practical purposes of diagnosis and surgical interference-, the study of the cerebral localizations must include also certain other parts of the encephalic m-'ss, a-, fi r instance, the centrum semiovale, yi6 THE NON-NEURONIC DISEASES the corpus callosum, the internal capsule, the crura cerebri and other association and projection tracts. In discussing, therefore, the cerebral localizations from the widest standpoint, one is obliged to discuss the function, or functions, of the entire encephalon, its psychoses as well as its neuroses. Only by such a wide discussion can one hope to avoid confusion and prevent the splitting up of the theme into disconnected and irrelevant facts. As our knowledge of the functions of each part of the encephalic mass becomes more precise, the so-called schools of loealizationists become more and more amalgamated. Among the investigators of cerebral localization there are at pres- ent three schools of varying degrees of prominence. In a general way, it may be said that the English-speaking physiologists regard the sensory and motor areas of the brain as more or less separate and dis- tinct, and as centers for the direct reception and emission of sensory and motor impulses. The Germans incline to the view that these areas are the centers for the direct reception and emission of nervous im- pulses, but that they coincide or more or less intermingle. The French and Italian investigators study the question more from the psycho- logical side, and place less stress upon the separation or commingling of the sensory-motor areas than they do upon the doctrine that the cortex is a general center for the representation of motor and sensory images. Psychosis, according to the latter, is a sensory-motor phe- nomenon, and mentalization is the product of the combined activities of the sensory and motor areas. They hold that the direct sensory- motor elements are lower down than the cortex ; that impulses of cerebral origin are as much peripheral as are those starting from the skin ; and that voluntary action is as much of a reflex as is an ordinary involuntary spinal reflex. This of course is a mere outline statement of the three views in vogue. Before taking up cerebral localization in detail, I desire to empha- size the fact that many of the higher so-called cortical centers subserve in some prominent, yet unaccountable, way very complicated psycho- physical processes. Such processes, for instance, are speech, writing, reading. And yet these are doubtless very elementary psycho-physical processes in comparison with those that probably underlie what are called imagination, reasoning and general intellection. It has always seemed to me to be a crude conception of psycho- logical processes of all sorts to suppose that they were each individually under the absolute and sole control of certain small areas in the cortex. The sharp differentiation made by some physiologists between the cortical centers, the narrow way in which they limit them and the dis- tinct functions which they assign to them, as though there were hardly any commingling or interconnection between them, has long seemed to me to be puerile, grossly materialistic and absolutely unwarranted by any scientific proof worthy of the name. There is no doubt that the brain, like the spinal cord, is a great compound and complex organ. It has relatively independent functions in its various parts. These parts and functions, however, are never absolutely independent. Their independence is only so far as they THE NON-NEURONIC DISEASE- 717 subserve the particular purposes of motion and sensation. So far as we now know, there is only one type of motor impulse, five of sensory impulses which again are somewhat subdivided. The former are emissive, the latter are receptive. Beyond these we know of no dis- tinct, well-defined impulses passing- in or out of the brain. I have referred to all of this elsewhere. I repeat it here because I desire to emphasize the distinction between these ingoing and outgoing impulses, and what is called psychosis. A psychosis is distinctly not the same as a motor or a sensory impulse. A psychosis is a compound, complicated phenomenon. Its elements are motor and. sensory, but it itself is a combination of elements. A psychosis is, therefore, most emphatically a function. The function is the interplay and inter- mingling of the elementary motor and sensory elements in such a wax- as to eventuate in the psychosis. If the basic elements are normal and the interplay is normal, or according to commonly accepted standards, the psychosis will be normal. If the basic elements, wholly or in part, are disordered, or if their inter-connections and inter-relationships are abnormal or different from the commonly accepted standards (as de- picted in the relative degrees of intelligence, education, etc.), the psy- chosis will actually be, or seem to be, abnormal. This is a psychological truth boldly and briefly stated here because space will not permit of further elaboration of it. The reader who wishes to study this view of the mind for himself is referred to the writings of Wundt, the leading exponent of modern physiological psychology. Speech, reading and writing, as I have pointed out elsewhere, arc not mere motor and sensory phenomena. They are the expression of a high order of psychosis. It follows, therefore, that they are not sub- served alone by fixed small centers in the cortex, but by most compli- cated intra-cerebral mechanisms, of which the so-called cortical centers are but the prominent outposts, as it were. All statements, therefore, such as that the anterior lobes subserve the higher mental powers; that Broca's center regulates motor speech; that the occipital lobe is the memory center for visual images, must be taken only relatively. In every complicated act that is more than a mere emission of a motor impulse, or the reception of a simple sensory impulse, in a word, in every psychic phenomenon, however small or vast it may be, so long as it is psychic and indicates psychic process the whole brain must be conceived as taking a greater or less part in the process. Psychosis is a brain function, not a function of its mere constituents. With this understanding of the nature of what are called the cerebral localization centers, 1 will proceed to discuss them briefly in detail. The Cerebral Cortex. — Local disease of the prefrontal lobes docs not give rise to any very definite localizing symptoms. It has been determined experimentally that at the base of the first and second frontal convolutions is a small area which controls tin- lateral move- ments of the head, tin- elevation of tin- eyelids, and the dilatation of the pupils. This has not been well established in man, however. Aside 7 i8 THE NON-NEURONIC DISEASES from these areas, the prefrontal lobes are usually credited with the ex- pression of the higher intellectual life. It has been observed that lesions of these lobes cause a change in the disposition and temperament, a lack of self-control, undue irritability, loss of the power of attention, loss of memory, general apathy, foolishness, and a tendency to laugh and cry immoderately without adequate cause. According to Flechsig, a delirium of grandeur accompanies irritative lesions, to be followed later on by a loss of judgment and general mental hebetude. Phelps has recently analyzed 295 cases of brain injury and con- cludes that the frontal lobes, and especially the left frontal lobe, is the FIGURE 186. Schematic representation of the left cerebral hemisphere, showing the known cortical centers in man. seat of the higher mentalization phenomena. It has been long recog- nized that the entire left side of the brain is superior to the right, but it is startling to learn that we only think with our left frontal lobes. Phelps observes that in every instance in which a laceration was con- fined to the right lobe, the mental faculties were unaffected. This may explain some of the puzzling cases in which brain injury sometimes profoundly affects the mind and sometimes does not. In calculating the degree of mental deterioration, the observer must give due consideration to the patient's previous mental capacity. No motor or sensory symptoms are awakened by lesions limited to the prefrontal lobes. If the lesion, such as, for instance, a tumor, increases in size so as to produce an irritative, followed by a destructive, change in the neighboring convolutions, the above-mentioned symptoms will be accompanied by "neighborhood symptoms." Anosmia has been observed to follow disease of the basal parts of these lobes, but it may he. a mere "neighborhood symptom" due to their proximity to the olfactorv lobes and tracts. THE NON-NEURONIC DISEASES 7IO, According to Brims, Oppenheim, Bernhardt and others, tumors of the frontal lobes cause a disturbance of equilibrium identical with the so-called cerebellar ataxia. Moeli and Wernicke had already made mention of this frontal ataxia. A tract from the frontal lobe descends in the median part of the foot of the cerebral cms as far as the nuclei of the pons. These nuclei are connected by fibres running through the middle peduncles with the cerebellar hemisphere of the opposite side. Bruns, who was the first to call special attention to the probable cause of this frontal ataxia, argues that the highest volitional centers for the great trunk musculature send impulses to the cerebellum by way of this fronto-ponto-cerebellar tract and thus exercise a certain voli- tional control over the latter's coordinating function. Much uncer- tainty surrounds our knowledge of this tract, however. Mills declares that, in his experience, pure, uncomplicated disturbance of equilibrium resembling cerebellar ataxia has not often been present in tumors of the frontal lobes. In two cases of cerebral tumor reported by Wiener, in which the neoplasm encroached upon the frontal lobe, there was prcnounced ataxia. It may be that in certain cases of internal hydro- cephalus in which there are a peculiar staggering gait resembling that of cerebellar ataxia and an absence of indications of pressure in and around the fourth ventricle, as in a case recently brought to my clinic, the ataxia is due to pressure upon the frontal lobes by the fluid in the lateral ventricles. Ataxia, when a symptom of cerebral disease, ap- pears under three forms, each being caused respectively by lesions in the motor region, in the internal capsule, and in the parietal lobe. The sensorial areas of the cortex are made at the present day to include the gyrus fornicatus (Ferrier, Schafer, Horsley and Flechsig), the hippocampal convolution, the precuneus, portions of the parietal convolutions (von Monakow), and the posterior part of the central convolutions (Flechsig, Hosel). In a general way the sensory area includes the postero-parietal parts of the hemispheres. This seems to be well established b\ pathological observations in man, as well as by experimentation upon animals. It must be remembered that Ferrier's observations in regard to the centers of sensation were made mostly upon monkeys and the lower animals and that focal epilepsies, which finally terminate in a general convulsion, indicate that the human is far higher and more intricate in structure than is the animal brain. It is greatly to be regretted that patients presenting symptoms of a lesion in the motor zone of the cortex are not more frequently and minutely examined with regard to general sensation. In many of the cases of paralysis reported in literature there is no mention whatever sensation; and in others in which the attempt has been made to discover the sensibility of the skin, the methods adopted haw been so crude and unscientific, and the results obtained SO meagre and indefi- nite, that the report, in this respect at least, is almost valueless. In comparison with the motor tests for the localization of a cortical lesion, sensory tests will probably always remain less efficient. The variation of the persona] equation .done is enough to shake one's confidence in sensory symptoms; and to properly perform these tests there must be exercised an unusual amount of patience, shrewdness and insight into 720 THE NON-NEURONIC DISEASES human nature. The instruments of precision must be employed in every conceivable manner and frequently at most unexpected moments. Unlike the testing for paralysis, we are here made to rely almost solely upon the patient and his subjective feelings. Hence we must study closely the patient's idiosyncrasies and peculiar temperament, and make due allowance for his past experiences and present intellectual status. His entire physical condition must at the same time be taken into consideration, for if there be a paresis present, for example, a sensory response may be very materially modified by the inability of the muscles to act and manifest the appropriate motor response. 1 have known of erroneous sensory diagnoses being made just in this way. Notwithstanding all these difficulties and drawbacks, it is ex- tremely desirable that every case of paralysis be carefully and accurately examined for general sensation ; for our knowledge of the location of the sensory centers is at the present day much less definite than that of the motor centers. Moreover, the experiments of the laboratory seem to favor the view that the sensory areas of the cortex are sep- arate and distinct from the motor areas, while clinico-pathological data intimate that they are identical, or at least are partly coincident. As long ago as 1888, and as recently as 1894, Dana contributed two valuable and exhaustive studies upon the relationship of the motor and sensory areas. Basing his arguments and proofs upon laboratory experiments and clinical observations, he concludes that injuries and destruction of the motor cortex are accompanied by disturbance of cutaneous and musculo-articular sensations. He holds that the -motor cortex is essentially motor, not sensory in the way that Bastian holds it to be. The parietal lobe is the great seat of sensation, but the motor cortex is a "sensory-memory-motor organ." On the other hand, Mills reiterated in 1902 what he stated in 1898, that he believed in the separate localization in the cerebrum of the representation of sensation and of movements. In regard to the location of the centers for cutaneous sensations — touch, pain, temperature and even the sensation commonly known as the "muscular sense" — many views have been advanced. The general consensus of opinion holds that the optic thalamus is the basal ganglion for the reception of sensory impulses from the outer world. Luys, Ferrier, von Monakow, Foumier and Crichton Brown so taught, though Flourens, Longet, Tamburini and Scruff attributed motor functions to the ganglion. Above the thalamus the sensory paths radiate through the corona and terminate chiefly in the cortex of the parietal and tem- poro-sphenoidal lobes. Below the thalamus the sensory paths are clear- ly distinguishable from the motor. Do these two paths so unite or intermingle above the ganglion as to terminate in the same cortical areas, or do they still remain sharply and wholly apart, one set (motor) going to the central convolutions, the other set (sensory) turning back to end in the postero-parietal lobes? In his earlier experiments Ferrier found that injury to the hip- pocampal convolution and cornu ammonis produced loss of tactile sensi- bility on the opposite side of the body. He therefore located the center for tactile sensibility in the region of the hippocampus. This was con- THE XOX-XEUROXIC DISEASES / 2 I firmed by Yeo in his experiments upon monkeys. Horsley and S chafer have extended the views of Ferrier, and as a result of their investiga- tions have concluded that the whole of the limbic lobe (including th? callosal and hippocampal regions) preside over the sensations of touch and pain, if not exclusively, at least to a very large extent. In some of their experiments the loss of sensation was accompanied by motor phenomena, which they attributed to unintentional injury to the motor area during the performance of the operation. As opposed to Munk and Scruff, who would possibly argue from this the identity, in part at least, of the motor and sensory areas, they state that in some of their experiments there was paresis of the lower limbs, with anaesthesia of the upper limbs, and in others anaesthesia without paralysis. There seems to be some very intimate connection, at all events, between the limbic lobe and the central convolutions ; for when we recall how fre- quently sensory aurae and other paraesthesiae precede and accompany Jacksonian symptoms produced by a limited lesion in the motor cortex, we are compelled to admit the extreme intimacy, if not the identity, of the motor and sensory areas. Many of these focal lesions do not extend deep enough to involve the radiating fibres from the sensory areas and their mutual influence must, therefore, be entirely cellular and transmitted from one to the other by associating- neurones. The influence may be merely of an inhibitory sort, for clinically the anaes- thesia accompanying such forms of local paralysis is never complete, but is rather of the inhibitory type. Brown-Sequard gave special atten- tion to the extensive inhibitory functions of the nervous system, and in the light of his investigations such an explanation is not entirely un- warrantable. If the sensory and motor areas were identical, it would he hard to comprehend how a limited focal lesion could give rise to a complete paralysis and incomplete anaesthesia in related parts of the body. Both sets of cortical cells would presumably be simultaneously injured and the anaesthesia would run parallel in extent and severity with the amount of paralysis. There is a suggestion in the recent observations of Sherrington and Griinbaum that may explain the conflict of views in regard to sensation as a function of the central convolutions. These experi- menters found in their elaborate examination of all types of anthropoid apes only the pre-central (ascending frontal) convolution to be motor. No motor response was ever obtained by stimulation of the post-central convolution (ascending parietal). Flechsig includes the post-central convolution in the tactile radia- tion. All that can be affirmed positively, then, is that the sensory con- ducting tract passes to a large extent into the central convolutions, though its sphere of radiation is to be found elsewhere, particularly in the parietal lobes (von Monakow). Some hold that only touch and muscular sense are appreciated by the central areas, while other sensa- tions, especially that of pain, are to he located in the gyrus fornicatUS. The stereognostic sense is located by von Monakow in the central areas, by others in the parietal ; but, as I have shown elsewhere, this is such ■a complex sense that it can hardly he justly assigned to any one special 722 THE NON-NEURON IC DISEASES area. Mills locates it in the superior parietal convolution. The parietal lobe is held responsible for the perception of the muscular sense by Nothnagel, Luciani, Mills and others, a view which has been supported from clinical observations by Vetter, Basset and von Monakow. As I have also shown elsewhere, it is not at all improbable that even here in regard to the muscular sense, the motor zone has come into active play and had much to do with the sense of position. It is to be noted that Charcot and Pitres doubted the sensory importance of the motor regions. In regard to the cortical visual area there is still some dispute, though speaking generally it may be said to coincide with the occipital lobe. The cuneus and the parts around the calcarine fissure (Hen- schen) are usually assigned as its limits. Oppenheim teaches that the gyrus fusiformis (lateral occipitotemporal), lingualis (median occipito- temporal), and first occipital convolution should probably be included. As everybody knows, destruction of this area causes bilateral hemi- anopsia of the opposite side. Total blindness follows extirpation of both occipital areas. Further localization of the visual conceptions of space, light and color is quite impossible, though there are many hypotheses proposed. The angular gyrus is no longer regarded as a center for visual sensations. The disturbance of sight which Ferrier and others long ago attributed to injury of the gyrus is now known to have been caused by involvement in the injury of the visual con- ducting paths that run through and near it. It has been recorded that lesions of the left inferior temporal lobe, the supra- marginal gyrus, cause alexia — inability to read — without disturbance of the ability to speak. The cortical area for the sense of smell has been localized in the gyrus uncinatus, as injury here has in some instances produced anos- mia (Hughlings Jackson). In a case of anosmia reported by Schafer and Frey there was atrophy of the olfactory tract and changes in the gyrus uncinatus and horn of ammon. The olfactory apparatus is much atrophied in man as compared with the lower animals. From his com- parative studies Edinger is led to assign the cortical areas for smell to the lobus pyriformis and the cornu ammonis. Professor Oiiodi, of Budapest, notes that in a case of cacosmia there was found a tumor in the right gyrus hippocampi. Hallucinations of smell have been asso- ciated with tumors of the gyrus uncinatus and gyrus fornicatus, though Edinger doubts the power of the gyrus fornicatus to perceive sensa- tions of smell. The cacosmia in this case may well have been a mere "neighborhood symptom." Hemorrhages, emboli and tumors of the temporal lobe have been accompanied by loss of smell, and the same explanation is probably applicable in these cases. The cortical area for the perception of taste has not yet been dis- covered. It is probably near and closely related to the center for the sense of smell. Paget believed that the perception of thirst and hunger was somehow associated with the basal surface of the temporal lobe. The center for hearing is somewhat better known, and is generally accepted as coinciding more or less with the upper convolution of the temporal lobe — that part of the convolution, according to Flechsig, THE XOX-XEUROXIC DISEASES 723 which lies hidden in the fossa of Sylvius. Ferrier at first located this center positively in the superior temporal convolution of monkeys. The experiments of Brown and Schafer, however, showed conclusively that even when both temporal lobes were removed there was no loss of the power of hearing. In reply to Ferrier and Yeo, these experimenters remarked that they had "frequently observed that monkeys which have experienced no cerebral lesion whatever will sometimes fail to start or show any sign of hearing at the report of a pistol, whilst others will react very strongly to such a noise.' 7 Might not this be easily accounted for on the basis of the association of ideas, some monkeys having from their individual experience in some way become fearful of certain kinds of noise, while others, lacking such experience, remain indifferent to them? If such were the case, it would open the way for the considera- tion of the superior temporal gyrus as a mere psychic or memory center for hearing, and thus harmonize the two opposing views of Ferrier and Yeo on the one hand, and of Brown and Schafer on the other. As a matter of fact, it is now pretty generally accepted that the superior temporal convolution is the seat of mind audition and that its destruction causes ziord-dcafness, and not sound-deafness. This sensory aphasia literally consists of a loss of the power of recog- nizing word sounds and their constructive association. The victims of this form of aphasia are in the plight of one who is talking to a for- eigner whose language he is absolutely ignorant of. When such a patient suffers from a bilateral lesion in the superior temporal convolu- tions he is said to have "cortical deafness." In this connection it is well to remember that this psychic word- center is the most important of the group of centers involved in the production of speech. Its proximity to the island of Reil may account, therefore, for the fact that lesions of the island usually cause dis- turbance of the power to correlate the speech centers, and thus pro- duce such symptoms as paraphasia, lapsus linguae, defective word and sentence formation, etc. See further the section on the disturbances of language. Our knowledge of the motor areas of the cerebral cortex is much more definite, so far as their location is concerned, than is our knowl- edge of the sensory areas. The examination of motor manifestations, both in the laboratory experimentally, and at the bedside clinically, is susceptible of a high degree of accuracy as compared with the exam- ination of sensory phenomena. As a result of extensive experimenta- tion upon the lower animals and of clinico-pathological observation in man, it has come to be established that the motor zone correspond-, with the central convolutions on either side of the fissure of Rolando,, the adjoining parts of the frontal and parietal lobes, the paracentral! lobule, and the supramarginal gyrus. There are no known sharply defined boundaries to this zone. Its center or rather centers of maxi- mum energy seem to lie along the central convolution just in front of the fissure of Rolando. In comparison with both anterior and posterior central convolutions, the areas of the frontal and parietal lobes lying contiguous to them play a minor role. The experiments made recently upon the apes by Sherrington and Grunbaum localize the motor center 724 THE NON-NEURONIC DISEASES absolutely in front of the fissure of Rolando. There was no response of a motor character whatever to stimulation of the post-central (as- cending parietal) convolution. The view is fast gaining ground that there are no pure motor centers behind the Rolandic fissure. The so- called motor areas thus indicated control each the musculature of the opposite half of the body. This control is not direct, but is exercised through certain lower deposits of gray matter. They are bilateral areas, therefore, and in both hemispheres subserve the representation of definite, and purposive movements produced by groups of muscles rather than the individual muscles themselves. They are the teachable memory areas for acquired movements. Most authorities regard them as purely motor in function, while others look upon them as sensory- motor. Focal lesions of these areas provoke monospasms and unilateral convulsions, followed by an oncoming paralysis which may vary in type, according to the location, character, and extent of the lesion. The paralysis which succeeds the spasm is more or less localized in extent and degree. It is of the monoplegic type, involving, however, related muscles that functionate in the expression of particular movements rather than the single muscles. If the lesion is unlateral and extensive, the paralysis is of the facio-brachio-crural type, a true hemiplegia. This paralysis is accompanied by an exaltation of the spinal reflexes. It always begins in the same part — or rather the spasm that precedes it — in different attacks, as for instance, the toes, the fingers, the face. The hemiplegia is not crossed, the face and body being affected on the same side. It is, of course, on the side opposite that, of the lesion. There is no direct involvement of the nuclei of the cranial nerves. Muscles that usually work together, as for instance, those of degluti- tion and respiration, and which are equally represented in both cere- bral hemispheres, exhibit but little paresis or else are so quickly com- pensated for in the unaffected hemisphere that their weakness passes unnoticed. The more complex and individualized the muscles are in their normal activity the more will they be paralyzed ; hence the upper extremity shows a higher degree of paralysis than the lower. There is no atrophy of the muscles and the electrical reactions remain normal. Late contractures occur in the parts paralyzed. In the lower third of the central convolutions, just above the fissure of Sylvius and embracing the lower end of the Rolandic fissure, are located the centers for the face, the tongue, and the upper part of the oesophagus. Just anterior to this center, at the posterior extrem- ity of the third frontal convolution, lies Broca's well-known center of speech, while behind it is a small area that controls the movements of the larynx. The larngeal muscles are rarely paralyzed as they work together and in lesions of one side or the other are quickly compen- sated for. The speech center is a bilateral center, but is only active apparently on the left side in right-handed people. The ri^ht speech center is active in left-handed people and can, by training, be made to compensate in some cases for loss of the left center. These centers are called the memory centers for the emission or motor part of speech, hence their disease gives rise to motor aphasia. THE XON-XEUROXIC DISEASES 725 Passing upward along the fissure of Rolando, we meet seriatim the centers for the fingers, the hand, the arm, and the shoulder, the maximum points of the centers seeming to occupy especially the mid- dle third of the precentral convolution. In the upper third of the convolutions are the centers for the leg, the foot, the toes, and the hallux, the last being found close to the mar- gin of the hemisphere and even extending well over into the paracen- tral lobule. On account of the nearness of these marginal centers of both hemispheres to one another, paraplegic, better stated as diple- gic, conditions, are relatively frequent, as Charcot pointed out. Just in front of the upper part of the precentral convolution the great trunk musculature is said to be represented. Horsley and Shafer locate the trunk center in the marginal convolution. Munk believes that it lies in the frontal lobes, while Jackson assigns it entirely to the cerebellum. It must be remembered that all of these centers shade off into one another so that no sharp outline can be assigned to any one of them individually. They are the only cortical motor areas that can be ac- cepted at the present day with anything like positiveness in man. Ex- periments upon the monkey have indicated that these areas may again be subdivided and smaller areas detected for the representation of small and special forms of movement. Within the lower centers, for instance, we find clearly represented the laryngeal, masticatory and deglutitory muscles, though there is some discussion as to the accuracy of all of them in regard to certain details. Lateral movements of the head, ele- vation of the eyelids, and dilatation of the pupils are all represented, according to the observations of Ferrier upon monkeys, in the posterior ends of the first and second frontal convolutions. The visceral muscles and vasomotor system do not seem to have any representation at all in the cerebral cortex, though that hardly seems possible when we remember how the emotions affect the circu- lation of the face and how even the viscera are perceived at times in the realm of consciousness. Their bilaterality and close unity of func- tion, as well as the fact that all the organs of vegetative life are more especially under the control of the sympathetic nervous system, may account for the lack of their higher representation in the cortex. Some authorities declare that a regulating influence upon the heart and bloo 1 vessels, upon the heat production and upon the visceral nervous appa- ratus is exercised in the opposite half of the body by centers located in and near the central convolutions. These, however, together with tin- centers for the bladder and rectum and for the trophic regulation of the musculature in general, though supposed to reside in and about the motor zone, are all entirely hypothetical. Certainly no symptoms in unlateral disease of the cerebral cortex have yet afforded us am basis whereon to locate or even to assume the existence of such centers. Those who maintain that the histological structure of the central convolution affords a clue to their proper function reason beyond the facts. Gowers says, for instance, that "it is instructive to note that in this part are found the largest ganglion cells met with in the cortex, cells comparable to, though exceeding in size, the certainly motor cells 7^6 THE JSON-NEURONIC DISEASES of the anterior cornua of the spinal cord." On the other hand, we find in the hippocampal convolution, which is admitted to be sensory in func- tion, pyramidal cells very similar to those found in the central convo- lutions. They lie just beyond what is known as the stratum radiatum. Furthermore, these large cells have never as yet been absolutely proved to be motor. They may be muscular, in the sense that they are in immediate connection with the musculature of the body and capable of a grosser and more vigorous form of activity than are the more delicate receptive sensory cells. Neurones, whether of the motor or sensory type, are all alike in their intimate structure so far as we know, and their exhibition of neurility is to all intents and purposes the same everywhere. When we remember that sensation itself is but a mode of motion, we can understand that these giant-cells may differ from the smaller ones in degree rather than in kind. The reflex character of most of the sensory-motor phenomena would seem to indicate that both sets of neurones possess the faculty of receiving and emitting nervous impulses and it is hard to escape the conclusion, therefore, that the essential differences in their functions are the result of the nature of the terminal end-organs with which they are respectively connected. As an essential part of the neurone doctrine, it is now taught even more emphatically than heretofore that the terms sensory and motor as applied to the nervous elements have more regard to their peri- pheral end-organs than to their innate structure or even to their cen- tral connections. Physicists are resolving all phenomena into molecular motion, the differences in the phenomena into the different forms of molecular motion. Light, heat, electricity are but the expression of the differ- ent modes of movement among the molecules of matter. Nervous activity has not infrequently been likened to electricity, but whether the comparison be true or not, it is more probable that the former is as much a mode of molecular motion as is the latter. Chemical changes accompany both and chemical changes are the result of atomic trans- formations. Both sensory and motor phenomena, nervous as well as muscular, are alike the result of molecular movements. The simple muscular contractions are recognized as the direct expression of mole- cular vibrations within the muscular elements and the reflex circle, beginning in a peripheral irritation, running through the center from sensory to motor cell and finally passing out again to terminate in gross muscular contractions, is simply the transmission of the same molecu- lar disturbance, differing in degree, but not in kind, throughout the various parts of the arc. This is so universally accepted now by science that its reiteration seems almost like a truism, but for my present pur- pose of showing the universality of the one phenomenon needs to be repeated. If the reader cares to pursue this line of thought further, he may consult Gowers' admirable little brochure entitled The Dynamics of Life, in which the author most elaborately and convincingly eluci- dates the fact that all neuro-muscular phenomena are but the mani- festation of chemical phenomena and that these phenomena are hut the expression of atomic and molecular motion, the origin of which motion is to be attributed primarily, so far as we are capable of knowing, to THE NON-NEURONIC DISEASES 72.7 the solar influence through light and heat. Nay, more. Even the higher, psychic manifestations of nerve force can be reduced by analy- sis to real or representative movement, even the gross muscular move- ments, as has been well pointed out by Romanes in his Mental Evolu- tion in Animals. The most positive knowledge we possess in regard to the functions of the cortex is that the central convolutions somehow preside over the movements of the body. And yet, as Gowers says, "We need 1101 conceive that these parts subserve no other function (we shall pres- ently see reason for believing that they have sensory as well as motor functions)." Injury to these convolutions produces spasm or paraly- sis ; and so far as we know, these symptoms are not produced by cor- tical lesions outside of these convolutions or their immediate neigh- borhood. That these are not the only centers which preside over vol- untary movements is to be inferred from many experiments upon ani- mals. A rabbit whose entire cerebrum has been removed can still run ; and according to Goltz, there was no complete paralysis, but only a slight anaesthesia observed in a dog whose whole brain except one occipito-parietal lobe was excised. It is an old established fact that removal of the cerebral hem- ispheres, olfactory ganglia, optic lobes, corpora striata, and optic thalami does not eventuate in the loss of voluntary motion and gen- eral sensibility. There is loss of intelligence and of rational inhibi- tion, but the animal still executes purposive, instinctive movements which are quite different from the simple reflex acts depending upon the spinal cord. Hence arises the question, what is the nature of these disturbances of voluntary motion caused by the destruction of the cor- tical motor areas? A dog deprived of his two sigmoid gyri does not manifest motor paralysis, if by that term is meant absolute loss of motility. There is not a single physiologist who denies such an ob- servation ; hence the ground for the criticisms of Goltz, the distin- guished opponent of the localization theories. The motor areas must preside therefore in some special way over the muscular apparatus and not directly, as at first seemed to be the case. In 1876 Hitzig spoke of the effects of cortical lesions in the motor area as "the expression of an abnormal representative action;" in other words, as the result of a destruction of the motor images belonging to certain voluntary movements. The muscles of the animal are not paralyzed, but the animal no longer possesses the mental pictures or ideal representations of the movements it should make or desires to make. Nothnagel and Bastian favor the theory of Hitzig, but they differ from him in locating a center for the "muscular sense" outside of the central lobules. The former declared to the Sixth Congress for Internal Medicine, held at Wiesbaden, that he had never known focal lesions of the motor zone to produce loss of the muscular sense, but that such lesions were often accompanied by loss of the tactile sense. In a paper published in Medicine I suggested that tin- SO-cailed "muscular sense" is but a differentiated expression of the tactile sense. Noth- nagel places the sensory area in the parietal lobes, where he also lo- cates, with Seguin, the special center for the muscular sense.. Noth- 728 THE NON-NEURONIC DISEASES nagel further affirms that the parietal lobes bear the same relationship to the central and paracentral lobules that the convolution or area of Broca bears to the cortical hypoglossal and laryngeal motor areas. In- jury to the parietal centers may produce ataxia without true paralysis, and injury to the central areas may cause paralysis without the loss of the muscular sense. This is somewhat of a return to the limitation views of Ferrier as originally propounded ; and, in fact, both Nothnagel and Charcot separate the motor areas from the centers for cutaneous and muscular sensibility. For Charcot, however, the cortical motor centers are the seat of motor representations or conceptions which must necessarily precede the accomplishment of any conscious volun- tary movement, while the muscular sense or kinsesthesic sensibility of Bastian is subserved by the cortical centers for sensation. According to Schiff, the so-called motor areas are entirely sen- sory ; their activity is largely of the nature of a reflex ; and the paraly- sis due to their destruction is really the result of the loss of tactile sen- sibility. Munk does not believe that these centers determine move- ments directly or in any sort of reflex manner, but merely by the awak- ening of the mental images or representations of both general sensi- bility and motility. These ideational centers are not in immediate connection with the individual muscles, nor do they exert their in- fluence directly for their individual activity. As Dr. Theodore W. Fisher tersely puts it, "The will can only control and the mind is only conscious of movement in the mass." The fact that stimulation of certain areas of the cortex produces movement of certain groups of muscles and eventuates in the coordinated movements of the segments of the limbs shows that movements, and not muscles are represented in the cortex. Many of the opponents of Ferrier have lost sight of this fact because this investigator insisted so strenuously upon the ana- tomical distinction and separation of the sensory and motor areas, and upon the sharp limitations of the latter to the central lobes. Munk's theory is popular in Germany and makes the so-called motor areas almost entirely sensory. The activity of these centers results in "conceptions of movements" rather than in movements them- selves. They are the psychical source of the impulses which farther down the tract (possibly in the basal ganglia or anterior cornua of the cord) are transformed into the true motor stimuli that set the mus- cles into action. The cortical motor area is therefore named by Munk the "Sense-sphere" (Fuhlsphare) . Gowers' objection to this theory is that the "sense of innervation," an element of the threefold "con- ception of movement," is a sense of something which is not itself sen- sory and which is commonly termed motor and that destruction of this region causes loss of this "motor" function out of all proportion to any demonstrable sensory loss. The first of these objections seems inade- quate to me; for it is a fact as Strieker and others have shown that movements are preceded by psychical representations, which representa- tions must obviously be of a sensory character. Franck inclines to the theory of a reflex action being the essential function of the motor areas. He believes that the motor, like the sensory, zones are only the points of departure for voluntary motor impulses while the true THE NON-NEURONIC DISEASES /2') motor apparatus or executive centers are to be found in the cellular elements of the medulla oblongata and spinal cord. All of the cortical areas, according to this author, are merely centers of voluntary asso- ciation. We know that the sensory and motor elements of the cortex are united by associating tracts, just as they are lower down in the cord, to complete the reflex arcs. The reflex phenomena, both spinal and cerebral, necessarily presuppose such a functional union of the sensory and motor radicles. This it seems to me is quite adequate to explain the occasional association of the two sets of symptoms when the lesion happens to be limited to one or the other area. . Formerly all reflex functions were supposed to belong exclusively to the spinal cord ; but to-day we consider the cerebral centers as much the centers for reflex action as are the spinal. Many of the Jacksonian epilepsies are undoubtedly of simple reflex origin. Mills reported a case in 1880, clearly Jacksonian in type, in which there had been seiz- ures for eleven years as the result of a fibroma in the hand. When the tumor was excised, the epileptic seizures ultimately disappeared. ''In the normal brain," Mills writes, "no reflex actions can be performed without exciting to action secondary volitional movements, which no longer require the stimulating influence of a reflex action. " The ab- sence of gross cortical lesions in some forms of Jacksonian epilepsy, and Heidenhain's experiments in hypnotism, with other observations and facts, all prove conclusively the reflex function of the cortical areas of the brain. Franck speaks of them as psychomotor centers, because they con- trol by their psychical influence the true, lower motor apparatus. He says that "in examining the movements produced by the excitation of points under the control of the cerebral cortex, they may be regarded as analogous to reflex movements ; but the essential difference between the movements so started and the ordinary reflexes consists in the point of departure of the original impulse. In one case it is cerebral, in the other cutaneous ; but in every case it is peripheral in relation to the center of motion (medullary centers)." Strictly speaking, then, the pyramidal tracts transmit afferent and not efferent impulses to the motor cells of the cord and medulla. Marique adopts, in part, this reflex theory as explanatory of the functions of the cortical motor areas; for he found that simple separation of the motor from the sensory areas, as a result of cutting the associating tracts, produced the same form of paralysis as when the cortical motor areas themselves were removed. He concluded, therefore, that the motor areas could not act of themselves, but that functionally they were dependent upon the impulses transmitted to them from the sensory area- of the parieto- occipital region. This question is far from being settled, however, since Mich varying results have been obtained in the experiment- of Franck and Pitres, Marique, Vareth, Exner and Paneth. According to Soury, Lisso has collected since 1882 some eighty- eight cases in which there was disturbance of sensation when the lesion was confined to the motor area. Hence Tripier and Gilbert Ballet have named the cortical area the "sensitive-motor zone." Exner admits that the different areas for tactile sensibility of the various parts of the 73° THE NON-NEURONIC DISEASES body are closely intermingled with the motor areas. In his study of aphasia, Dejerine says : "These disturbances of cutaneous sensibility accompany the beginning of the paralysis itself in the majority of cases ; sometimes they even precede the latter. These phenomena are not rare in the course of a hemiplegia of cortical origin and they deserve close study." The lesion in Dejerine's case was confined exclusively to the motor cortex, leaving the sensory paths intact. Dupuy notes that Horsley reported to the Congress of Brighton, in 1886, that the removal of a part of the motor cortex in three patients for epileptoid seizures symptomati® of cortical lesions resulted in the partial or complete loss of sensibility. Seguin and Weir reported the case of a man, aged thirty-nine, who complained in the autumn of 1882 of severe head pain and convulsive attacks limited to the right side of the neck and face. From 1885 these attacks occurred very frequently and were associated with a sensation of cold. The spasms involved the right arm and hand as well as the face. Finally paresis supervened in these parts and was accompanied by aphasia and agraphia. The diagnosis was tumor in the facial center of the left motor zone. Operation proved it to be a sarcoma, about an inch in thickness, nesting beneath the foot of the second frontal and the anterior border of the ascending frontal convo- lutions. A report of the case in the Journal of Mental and Nervous Disease, December, 1888, one year after the operation, states that there was unquestionably anaesthesia of the cheek, hand and forearm to contact tests as well as with the aesthesiometer. There was also anaes- thesia of the lower part of the face, lips and inside of the cheek. From the study of the sensory tracts and of a series of American cases of cortical lesion, Starr came to the conclusion that the motor and sensory areas coincided, though the latter extended beyond the former and in- cluded the postero-parietal lobe as well as the Rolandic region. Four of the cases in Starr's list belonged to Mills, and in reviewing his notes the latter came to the conclusion that they at least did not support the inference drawn by Starr. From a careful review of one hundred and thirty-seven cases, Dana decided that the sensory (tactile sensa- tion) and motor areas were coextensive. In the discussion of Dana's paper, Starr and Seguin argued with the author, while Mills favored the teaching of Ferrier — namely, that the center for tactile impressiini> is to be found in the gyrus fornicatus and gyrus hippocampi. Sher- rington and Grunbaum's experiments tend to negative the idea that the gyrus fornicatus is concerned with common sensation. Strieker maintains that motion and sensation are so intimately re- lated that they are almost identical. His views may be portrayed most clearly by means of an illustration. When one represents to himself, for instance, the movements of a cloud, the muscles of the eyes underg > the same sort of a sensation as though they were actually gazing at a moving cloud. By checking this muscular sensation within the eyes, the mental image of the cloud immediately ceases its movement. The cloud seems absolutely stationary. Among die Italians. Lussma and Lemoigne maintain that there are sensory and motor areas in the cerebral hemispheres, but that they do not act upon one another after the manner of a simple reflex. Tarn- THE NON-NEURONIC DISEASES . J$ I "burini, Luciani and Seppilli have elaborated since 1876 the theory thai the "motor zone" consists, en the one hand, of the center for cutane- ous and muscular sensibility and, on the other, of the centers for motor ideations. This, it will be recognized, is a very elaborate, mixed and comprehensive hypothesis. It well illustrates, says Soury, the eclectic tendencies of the Italian school. As a general rule the Italians are as much opposed to the views of Hitzig and Xothnagel as they are to those of Schiff and Goltz. They lean somewhat toward the theories of Ferrier and his English followers. In other words, they believe that the paralysis caused by injury of the cortical motor areas, pathological or experimental, is neither ataxic nor reflex. It is decidedly and en- tirely a paralysis in the truest sense of the word. Such at least seems to be the opinion of Albertoni and Michieli, Lussana and Lemoigne, Tamburini, Luciani, Seppilli, Maragliano, Bianchi, Palmerini, Ton- nini and others. In the language of Maragliano, all motor phenomena should be regarded ''as dependent upon true voluntary motor centers, which are capable, without the intermediation of any other motor cen- ters, of starting into activity the muscular apparatus of the body." Equally concise and clear in their statements are Luciani and Tam- burini. These authors, unlike Ferrier, locate even the centers of vol- untary motion in the basal ganglia, especially the corpora striata. Injury to these ganglia as well as to the corresponding centers of the cortex does not result in ataxia, or in the phenomena of incoordination ; nor is any alteration of general sensation to be detected in animals whose motor zones have been operated upon. The striate body, it will be remembered, is, according to Ferrier, the general center for movements which were once voluntary but which have afterward be- come automatic. Thus a dog can still run and swim if the striate body is left intact, though the corresponding cortical areas may have been removed. Luciani and Tamburini endow the basal ganglia with the same pyschic functions possessed by the cortical areas and thus differ considerably from Ferrier. They speak of them as psychomoter cen- ters, probably meaning thereby that the basal ganglia as well as the cortical areas are in direct connection with the sensory centers. "The basal ganglia," they say, "and especially the corpora striata, poss the same physiological value as the centers for voluntary motion do; or, in other words, these centers can be put into action directly by psycho-sensitive sensorial processes." When these authors promul- gated the above-mentioned ideas, it was supposed that the function of the striate bodies was motor; now, however, it is known that the hemi- plegias which result from hemorrhage in this region are caused by the injury done to the internal capsule. In fact, the striate' bodies have teen transformed completely into cysts without a paralysis occurring so long as the internal capsule remained untouched. Hence these earlier views of Luciani and Tamburini are now regarded as quite untenable. Nevertheless, it must be admitted that the recent study of the striate bodies (caudate nuclei) especially by Baginsky and Lehmann seems to demonstrate that there was a modicum of truth in the earlier views of these Italian authors. The so-called paralysis which results from a destructive lesion of 732 THE NON-NEURONIC DISEASES the excitable zone of the cerebral cortex is believed by some of the Italian investigators to be due entirely to a loss of sensation. Silvio Venturi, of Padua, adopted this ataxic theory as far back as 1878. It was Schiff, the distinguished predecessor of Luciani in the chair o£ physiology at Florence, who became the chief exponent of this sen- sory explanation of the paralysis. In 1871, the year after the experi- ments of Fritsch and Hitzig, the editor of L'Imparziale medico, in- spired by Scruff, wrote that all the immediate effects of injury to the so-called motor centers of Hitzig were really the result of a disturbance of sensibility and were limited entirely to that sphere. In several Flor- entine publications, issued in 1873 and 1876, Schiff recalls these facts. According to this physiologist, a dog with a cerebral lesion is not affected with any loss of energy in the muscles of the opposite half of the body, but merely with an absence of the feeling of surety and power of adjustment in its various movements because of the loss of tactile sensibility. The general movements of leaping and running are all well performed, but there are marked evidences of an uncertainty in regard to the position of the limbs. This anaesthesia is entirely cuta- neous, according to Schiff, while the sensibility to pain and pressure re- mains unaffected. Alterations of the sense of pressure and of tempera- ture are caused by the increased depth of the cerebral mutilations.. While these views, together with the experiments of Schiff, are not accepted by the majority of physiologists, they have had their influence in greatly modifying the original theories of many in regard to the motor character of the so-called motor areas. Hitzig himself, who was so vigorous an upholder of the purely motor theory, has altered his conceptions to such an extent as to refer to these motor phenomena under the name of disorders of the muscular conscience. Schiff's. theory is that of a pure reflex. An impulse from the periphery is sent inward to the brain and there within some hitherto undiscovered sub- cortical centers it is transmuted into an efferent impulse which is sent out again to the muscular apparatus. This cerebral reflex, he insists, is similar to the spinal reflex and the phenomena due to its disturb- ance are similar to the reflex phenomena due to injury of the posterior columns of the cord. It should be observed that Schiff locates the re- flex centers, not in the cortex itself, but somewhere beneath the cor- tex. Von Monakow holds that all bilaterally acting muscles, such as- those of respiration, deglutition, and mastication are innervated from some unknown subcortical centers that vary slightly, if at all, from the cortex. There have been many objections and objectors to these views of Schiff and his following in regard to them is quite small. It must be admitted, however, that, like his opponent, Hitzig, he has done valuable service to the science of cerebral localization ; for if Hitzig discovered the results of injury to the motor areas of the cortex, Schiff revealed those due to injury of the centers for tactile sensation. To judge from the doctrines of Munk, Luciani and Seppilli, the future will have to acknowledge Schiff, as well as Hitzig, as one of its fore- runners. When Schiff asserts that "one thing is positive, there are no cortical centers," we are somehow forcibly reminded of Goitz. Though the former enumerated many objectors, chiefly among the THE N0N-NEUR0N1C DISEASES 733 followers of Ferrier, such as Lussana, Tamburini, Bechterew and Hors- Jey, his teachings have acted as a wholesome check upon the too rapid assumption that all movements of the body originate from certain lim- ited areas of the cerebral cortex. They have induced experimenters to study somewhat more minutely the character of all muscular move- ments, the relation of the centers for this movement to the centers for .sensation, and the influence, if there be any, of the higher mental fac- ulties, the purely psychic functions of the brain. Tamburini has attempted a reconciliation of the two opposing views just described. He admits it is probable that the points of the cortex where the inpouring sensory impulses are transformed into out- going motor impulses correspond identically with the cortical areas upon which so much study has been expended. In his own words, "Each of these centers may be at the same time the focus for the reception and perception of the sensory excitations sent inward from a given part of the body and the point of departure for the voluntary centrifugal impulse going to the muscles of the same part.'' This, he believes, ex- plains the absence of sensibility in the direct production of localized movements ; for in the one instance the electric current takes the place of the nervous current or impulse and in the other the removal of the cortical centers must necessarily abolish the perception of the peripheral impressions. In conjunction with Luciani, Tamburini has extended the same theory in regard to the excito-motor zone of the cortex to the centers for sight and hearing. The movements of the ear and eye- ball, initiated by a sensory impression at the periphery, are not of the nature of a pure reflex, as Ferrier believes, but simply the result of the intermingling of the corresponding sensory and motor elements of the cortex. In other words, corresponding sensory and motor cells lie in close juxtaposition within the various cortical areas. These views of Tamburini seem to be supported by an anatomical as well as by a phys- iological basis ; for, as 1 have already pointed out, the region of the hippocampus major, which Ferrier and his followers assert is sensory in function, contains a row of large pyramidal cells between the medul- lary center and the so-called stratum radiatum. These cells are iden- tical, so far as their microscopical appearances are concerned, with the supposed motor cells of the central convolutions and anterior horns of the cord. In this connection it is interesting and suggestive to note Flechsig thinks that for every sensory path (corticopetal) there is a correspond- ing motor (corticofugal) path. For instance, beside the auditor v myelogenetic area in the first temporal convolution, there is a cortico- fugal area or radiation which he believes is motor, though this has not yet been demonstrated. And so on for all the sensory motor couplets which, taken en masse for the entire brain, make up his primordial zones or regions of early development. In 1880 Luciana, who, like Scppilli. found that the effects of a circumscribed lesion within the motor zone of the cortex were not only paralysis or paresis, but also ;> more or less pronounced alteration of the muscular and cutaneous sensibility, wrote that "the motor centers and the sensory centers, which together perform a very complex func- 734 THE NON-NEURONIC DISEASES tion, are intermingled or lie in the closest proximity (in gran vicinanza) within the cerebral cortex." He verified the phenomena described by Munk, but he did not look upon the paralysis as psychical in character ; that is, as the result of the loss of mere sensation of mental images or psychic representations. It was purely organic and depended directly upon the injury to the motor cells, just as the disturbed sensibility de- pended upon the injury to the neighboring sensory elements. The mo- tor centers, therefore, like the centers for sight and hearing, were of a complex nature and in reality were sensory-motor. Luciani says, "the motor centers are not localized within the cortical area, called up to the present time 'the motor zone,' a name which we must hereafter abandon, since this zone is not exclusively motor. All the various re- gions of the cortex are more or less strewn with special motor centers. In order to be positive of having destroyed all the motor centers, one must remove the entire cerebral cortex." This is a remarkable state- ment of a singularly comprehensive theory, and leads us -back once more to the vigorous contentions of the school of Goltz and somewhat to the teaching of Flechsig. Danillo has demonstrated that when the occipital lobe, which is supposed to be entirely sensory in function, is stimulated, the move- ments of the eyes are not simply reflex, as Ferrier believed, because they are still observed when after the ablation of the entire cortex of the occipital lobe the underlying white substance is electrically excited. In this white substance, therefore, there must be motor fibres springing directly from the cortex of the occipital lobe, unless we imagine that these same fibres go to the central lobules and through them, rather than directly, transmit the motor impulse to the muscles of the eye. Even such an hypothesis, however, would represent the process within the occipital lobe cortex as reflective, but the outgoing impulse in such a case would be transmitted into an ordinary motor one before it finally reached the eye muscles. Bechterew finds that when the so-called motor areas of the cen- tral lobules are separated from the occipital centers, stimulation of the latter still gives rise to the same muscular response. He argues, fur- thermore, that the movements of these muscles cannot possibly be of the nature of a simple reflex, because they are always uniform and localized within the same group. Stimulation of the occipital cortex excites motor points therefore that do not belong to the so-called motor zone whose fibers pass only through the pyramidal fasciculi of the cord. These mixed centers being thus situated outside of the cor- tical areas to which the pyramidal fasciculi run require for their ex- citation a much stronger and longer current ; their destruction does not involve manifest disturbances of motion ; and the movements which they give rise to are not very clearly differentiated from those caused by excitation of the true motor zone. Bechterew reasons that the sen- sory areas do contain motor centers, or at least motor elements, and he accounts for the fact of their stimulation requiring a stronger cur- rent and one longer in duration on the ground that they do not connect with their corresponding muscles through the intermediation of the anterior roots of the spinal cord, but through certain masses of gray THE NON-NEURONIC DISEASES 735 matter situated deeply within the cerebral hemispheres, probably the optic thalami. According to Bechterew, then, there are two sorts of motor centers within the cerebral cortex. One variety is easily ex- citable and is found in the central convolutions ; the other is not so easily excited and is scattered in among the sensory centers. This au- thor bases his views chiefly upon his experiments on new-born animals, in which the movements of the ear and the conjugate movements of the eyes can only be produced by direct stimulation of the correspond- ing areas of the cortex one week and one month respectively after the movements of the limbs have been elicited by excitation of the central convolutions. It is not to be doubted, therefore, that the sensory areas of the cortex contain some motor elements and exercise some special influence over the associated muscular apparatus. From this rapid review of the modern theories current among the English and Con- tinental physiologists, we note clearly that there is a growing tendency to believe that the motor centers of the central convolutions have sen- sory elements more or less intermingled with them. Thus far I have discussed only the localizations, so far as they are known, in the cerebral cortex ; but these do not by any means include all of the localizations in the brain. The functions of the basal ganglia, the arrangement of the projection tracts especially as they course through the internal capsule and the functions of the cerebellum are all sufficiently known to afford some clue as to the location of lesions in these parts. As the white matter of the hemispheres consists of association, commissural and projection tracts, a lesion within them cannot be sharply diagnosticated and distinguished from a lesion of the cortical areas with which they are connected. From the standpoint of surgery, it is extremely desirable to be able to distinguish a cortical from a sub- cortical lesion and even if possible the depth of the subcortical. Not many lesions are entirely and completely cortical, and many of those which are often supposed to be cortical are discovered upon post mor- tem to be subcortical. Tumors of the centrum ovale usually give rise to more extensive symptoms than do those of equal size in the cortex. Tn other words, the nearer the lesion is to the cortex the more likely it is to produce spasm and paralysis or paresthesia and anaesthesia in, for instance, the arm, the leg, or a part of the face, while on the other hand the farther it is away from the cortex, as, for instance, in the internal capsule, the more likely it is to provoke a hemiplegia or a hem- ianesthesia. The spreading out of the tracts above the capsule explains clearly enough the reason why cortical lesions tend to produce mono- plegias and subcortical more extensive paralyses. Though not abso- lutely reliable, this distinction is an important and valuable one. Seguin made a careful study of the differential signs between the cortical and subcortical tumors, and in a general way concluded that cortical lesions produced "localized clonic spasm, epileptic attacks beginning with local spasm, followed by paralysis; early appearance of local cranial pain and tenderness; increased local cranial temperature;" whereas sub- cortical lesions gave rise to "local- or hemi-paresis followed by spasm ; predominance of tonic spasm: absence, small degree or very late ap- 736 THE NON-NEURONIC DISEASES pearance of local headache and of tenderness to percussion ; normal cranial temperature." Among the subcortical lesions those of the internal capsule are of special significance. On account of the course of the middle cerebral artery, hemorrhage into the internal capsule or its immediate neigh- borhood, the corpora striata and optic thalami, occurs more frequently than it does into the centrum semiovale or on to the cortex. Usually the extravasation of blood causes such an extensive destruction of tissue that a localization diagnosis is quite out of the question; and even were the focal lesion in the internal capsule to be so small as ta confine its deleterious influence to only certain of the tracts passing through it, a diagnosis intra vitam between capsular and cortical dis- turbance would be often impossible and always extremely difficult. To attempt a localization diagnosis of a diminutive lesion within the cap- sule, one must know clearly the relative positions of the various tracts that run through it. The anterior two-thirds of the posterior limb of the capsule are occupied by the projection tracts from the motor areas of the cortex. These fibres hold approximately the same relative posi- tion to each other that the areas of the cortex do among themselves ; that is, beginning at the knee of the capsule and passing backward, we meet the face, arm and leg tracts, which, however, are not sharply defined, but blend with each other at the line of separation. Horsley, Beevor, and others have located the bundles of fibres for the glossal, masticatory, and laryngeal muscles in the knee of the capsule. Von Monakow says that the speech path positively passes through the cap- sular knee. When this part of the capsule only is involved in a lesion, there occurs paralysis of the inferior branch of the facial nerve and of the hypoglossal of the opposite side. If the disease is upon the left side disturbance of the speech will also occur. In the posterior third of the posterior limb of the capsule course the sensory projection tracts that terminate in the cortex, those from the visual areas of the occip- ital lobe being the farthest behind. In the anterior limb of the capsule is to be found the fronto-pontile tract of Brissaud, about which, how- ever, there is still much diversity of opinion. Injury to this anterior limb causes no definite localizing symptoms. With this picture of the internal capsule in the mind, the diagnostician might attempt a local- ization of a small lesion, but, as I have indicated above, he could not feel sure of the correctness of his diagnosis. Usually a lesion of the capsule is so large and destructive that the accompanying paralysis assumes the form of a total hemiplegia, the well-known faciohypo- glossal-brachio-crural type, of the opposite half of the body. With this hemiplegia is associated more or less hemianesthesia. The following symptoms are supposed to indicate tumor of the corpus callosum : General symptoms of brain tumor such as head- ache, etc. ; gradual hemiplegia followed by paraplegia ; great mental clulness ; sleepiness ; stupidity ; indifference to the external world and a low, inanimate condition generally ; absence of the cranial nerves ; terminal coma and death. There is much doubt about all this, however, as the symptomatology is so general and includes so much that is in- dicative of tumor of the ventricles. A definite localization of the lesion THE NON-NEURONIC DISEASES 737 is therefore extremely difficult if not quite impossible. The bilaterality of the paralysis and its irregular type, when taken in conjunction with the mental deterioration and general signs of intra-cerebral tumor, are highly suggestive, but hardly much more. In Schuffer's collected twenty-live cases of tumor of the corpus callosum, ten exhibited optic neuritis, while in seven it was absent. Headache was wanting in fifteen. In all of the cases there was a change in the mentality, in- cluding weakness of the intellect and memory, somnolence, etc. Con- vulsions occurred in eleven. Disturbances of sensibility were exceed- ingly rare. Mental changes are more constant with tumors of the cor- pus callosum than with tumors in other parts of the brain. When such mental changes are unaccompanied by any definite localizing symptoms, a tumor of the corpus callosum ought always to be thought of. Complete destruction of and focal lesions within the corpora striata do not give rise to any definite localizing symptoms. The old idea that the caudate nucleus has something to do with the automatic movements of running, walking, etc., and that the lenticular nucleus (globulus pallidus) is associated with the sensory paths is not well established. Lesions of these bodies only reveal themselves when they cause paralysis by pressing upon the internal capsule. There are no symptoms with small, inherent lesions. The optic thalami are clearly in close relationship with the gen- eral and special sensory areas of the cortex. The expression of the emo- tions is somehow connected with these ganglia, for when they are in- volved in cases of cerebral paralysis, the involuntary manifestation of delight, joy, sorrow, disgust, etc., seems to be impossible. The psychic reflexes such as laughing and crying, are disturbed. Lesions of the posterior part oi the thalami, namely, the pulvinar, cause partial blind- ness, though this is not absolutely certain. A tumor of the pulvinar and adjacent parts will produce a hemianopsia which may be distinguished from a hemianopsia of occipito-cortical origin by the hemiopic pupillary reaction, since these primary visual centers control the movements of the pupils through the third nerve. Disturbances of hearing, taste, smell, and the tactile and general muscular senses have all been attrib- uted to disease of the optic thalami, but none of them have been posi- tively established. Modifications of coordination and even general muscular atrophy on the opposite side of the body have also been seen. It is obvious that as the optic thalami are closely connected with the corresponding cortical centers, a differential diagnosis between a thala- mic and a cortical lesion is quite impossible. In a case of a lesion con- fined solely to this ganglion, reported by Hunter, there was as usual a loss of the senses of smell, of sight, of hearing and of touch; the woman sank gradually and remained a stranger to all external im- pressions. The extensive character of these symptoms would of course suggest a basal rather than a cortical lesion, but even in such a case it would be difficult to say just how much of this symptomatology was due to pressure upon the internal capsule rather than to injury to the thalami. , In spite of our fairly accurate knowledge of the anatom- ical connections of the basal ganglia, we know very little about the 738 THE NON-NEURONIC DISEASES functions of this part of the brain. Though Bechterew regards these ganglia as reflex organs controlling the muscular apparatus by means of both centrifugal and centripetal fibers, and though he, with Xoth- nagel and Brissaud, looks upon the optic thalamus as a center for the involuntary automatic movements, the subconscious, non-volitional psycho-reflexes of the body, his views are still largely hypothetical. In- voluntary movements have been observed in disease of the thalamus, and Nothnagel has shown that the same cause will give rise to a crossed paralysis "observed only in laughing while the nerve still obeys the will" (Oppenheim). Still more questionable than all this are the views of Bechterew, Scruff, Lussana, Sinkler, and others that the cardiac, gastric, intestinal, sexual, vasomotor, secretory and trophic centers are located in the optic thalamus. In a word then, the basal ganglia do not at the present day present any definite localizing symp- toms, and granting that they did, it would scarcely be of much practical value to us beyond the making of a diagnosis excluding the cortex, for these ganglia are not amenable to surgical interference. The anterior corpora quadrigemina, together with the external geniculate bodies and puhinar, constitute the , primary centers for sight. The geniculate bodies seem to be the chief terminus of the optic fibers, and in this respect are closely associated with the pulvinar. Bechterew alleges as a result of his experiments that lesions of the anterior quadrigeminal bodies produce blindness, but this has not been observed in man. Their relationship to the pupillary reflex and ciliary muscles (Henschen, von Monakow) is still problematical. Stilling be- lieved that some of the fibres of the optic tract could be traced to the corpora subthalamic a, but in regard to this there is no certainty. No localizing symptoms are known in connection with Lilys' body, though much is said about its role in the maintenance of equilibrium and of coordination. The same is said, however, of the corpora quadri- gemina, the geniculate bodies, and pretty nearly all the basal ganglia, all of which brings to mind the words of Foster, who, when comment- ing upon the interpolation of ganglionic masses in the course of a con- tinuous tract, such, for instance, as the optic tract, says : "All day long and every day, multitudinous afferent impulses from eye and ear and skin and muscle and other tissues and organs are streaming into our nervous system. . . . By the checks and counterchecks of cerebral and spinal activities all these impulses are drilled and mar- shaled and kept in orderly array till a movement is called for ; and thus we are able to execute at will the most complex bodily manoeuvres, knowing only why and unconscious or but dimly conscious how we carry them out." The posterior corpora quadrigemina and internal geniculate bodies are usually given the function of controlling the reflex movements associated with hearing. Von Monakow is not fully satisfied that the internal geniculate bodies have anything to do with audition. Fibres from the cerebellum enter both the posterior (|uadrigeminal tubercles and the internal geniculates, so that one of the signs of lesion in or near them is disturbed equilibrium. Beyond this we know of no symp- THE XOX-XEUROXIC DISEASES 739 toms that are produced by focal lesions limited solely to the corpora quadrigemina and geniculate bodies. The red nucleus of the tegmentum belongs to the cerebellar sys- tem, and therefore subserves in part the maintenance of equilibrium. The nucleus dentatus, the olivary body, the red nucleus, and the gray matter of the pons all bear a general relationship to the cerebellar cor- tex the same as the corpus striatum and the optic thalamus do to the cerebral cortex. They all connect ultimately with the anterior horns of the cord. No special localizing symptom can therefore be assigned to the red nucleus. Lesions of the corpora mamillaria are not known by any localiz- ing signs. Tumors of the hypophysis or pituitary body, especially of the ante- rior part of it, are so often associated with the general symptomatology of acromegaly that they have almost come to be regarded as the cause of this disease. Histological structure and embryological development both demonstrate that the anterior part of the pituitary body is a glan- dular organ rather than a part of the cerebrum, a fact which might explain in part the relationship between it and acromegaly. In the Revista sperimentale di freniatria, 1892, is a report of the results of the destruction of the pituitary gland in thirty experiments upon dogs and cats. Complete destruction proved rapidly fatal ; partial destruction, less rapidly. The animal was profoundly prostrated and exhibited a change of character. There was muscular weakness with clonico-tonic convulsions. The breathing became difficult. Anorexia alternated with bulimia. Alkaline polyuria was present, accompanied by polydipsia, depressed temperature, and emaciation out of all proportion to the anorexia. The inference was drawn that the destruction of the gland eventuated in the formation and accumulation of some special toxic substance within the organism. The localizing symptoms of slow dis- ease in this gland are therefore neighborhood symptoms, acromegaly and some other signs of general emaciation. Among the neighbor- hood symptoms that should be closely studied in suspected tumor are such as are referable to involvement of the optic chiasm, the circle of Willis, and the structures in and about the third ventricle. Lesions of the epiphysis or pineal gland can only be suspected from neighborhood symptoms which point to implication of the optic thalami and the parts about the midbrain. Such symptoms, however, are usu- ally too general to be of any very great value in making a localization diagnosis. Focal disease of the crura cerebri is rare, but when present may be recognized by a hemiplegia and possibly a hemianesthesia. There is no way, however, of distinguishing such a lesion from one involving the corresponding motor and sensory areas of the cortex, except by re- membering that the sensory-motor area is much more confined in the crura than in the cortex, and hence will exhibit a more widespread set of symptoms with the same-sized lesion. In tumor of the crura, fur- thermore, the third nerve will in all probability be implicated, giving rise to a "crossed paralysis," hemiplegia alternans. In this form of hemiplegia the degeneration occurs in the central neurone for the body 7 4 o THE NON-NEURONIC DISEASES and the peripheral neurone for the eye ; therefore the body muscles ex- hibit a spastic form of paralysis while those for the eye are affected with a flaccid paralysis and degenerative atrophy. The localization of lesions in the pons and the medulla is largely a matter of remembering the location and arrangement of the cranial nerve nuclei. Symptoms showing an involvement of the pyramidal tracts are the principal ones in pontile lesions. Tumors of the pons often cause conjugate deviation of the eyes, which, unlike the same symptom when produced by cerebral disease, is in a direction away from the side of the lesion. Especially true is this of tumors lying near the cephalic border of the pons. Lesions lying above a horizontal line drawn roughly through the points of emergence of the trigeminal nerves cause a hemiplegia and facial paralysis on the opposite side of the body, also a probable involvement of the third nerve. Lesions be- low this line produce a hemiplegia of the opposite side of the body and paralysis of the same side of the face. This facial paralysis is central and must be distinguished from that caused by disease of the facial root fibres or nuclei. The electrical reactions will not be those of a peripheral paralysis ; the paralysis itself will be more or less incom- plete, though more pronounced, than a paralysis due to a lesion of the facial tract higher* up in the pyramidal paths or in the cortex. Tumors upon either side of the pons which encroach upon the peduncles give rise to forced movements of the body, the movements being either toward or away from the seat of the lesion. Vasomotor phenomena, such as pallor, flushing, temperature changes, mucous hemorrhages, and epistaxis, have all been attributed to disease of the pons. Disturb- ances of hearing and of taste have been observed by Mills in disease of the lateral aspect of the pons. Vertigo, ataxia, and trismus may even occur as irritative symptoms of pontile lesions. Most authors declare that no known symptoms result from lesions in the lateral lobes of the cerebellum. All localizations in this part of the brain are referred to the middle lobe or vermis. Disease of this lobe is supposed to be indicated by disturbances of equilibrium, forced movements, and a peculiar gait known as cerebellar ataxia. Such is the teaching of Nothnagel. It should be remembered, however, that in the middle lobe decussate all the tracts that course from all parts of the cerebellar cortex, and that it may be these tracts that are especially affected in disease of the vermis. Kuh says he has seen a case in which an angeio-sarcoma had almost totally destroyed the mid- dle lobe, and yet there never was any indication of incoordination. Mills does not regard the cerebellum in any part as a latent region. In tumors of the lateral lobes the general symptoms of an encephalic growth receive a special cerebellar stamp. The vomiting, vertigo, and optic neuritis, which is more frequent in cases of cerebellar tumor than of tumors elsewhere in the encephalon, are more or less increased and are accompanied by signs of compression upon the neighboring parts of the medulla and corpora quadrigemina. Most of the symptoms attributed to cerebellar disease are really pressure symptoms. The ver- tigo, vomiting, and headache can only be considered as valuable local- izing signs by exclusion of other parts o\ the brain. Incoordination, THE NON-NEURONIC DISEASES 741 vertigo, and dysarthria, or scanning speech, are the only symptoms that may be said to more or less definitely indicate a cerebellar lesion. With these nystagmus and occipital headache are highly suspicious. Jackson and Russel have credited the cerebellum with a special influ- ence upon the great trunkal muscles, but this is probably due in part to its general coordinating function. On account of the wide connec- tion of this part of the brain with almost all the other parts of the en- cephalon above and the spinal cord below, the general function of it is believed to be that of securing the . higher automatic and psycho- reflex movements. Even its great function of maintaining the equi- librium is in all probability due to its relationship to the eighth or space-sense nerve. The wonder, therefore, is that the localizing symp- toms in connection with the cerebellum are as definite as they are. According to Bechterew, the olivary bodies subserve the function of coordination, the impulses reaching the cerebellum by way of the decussating cerebello-olivary tract. No known localization symptoms have as yet been attributed directly to the olives. According to the same authority, the superior olives act* as a reflex center for correlating the movements of the head and eyes with auditor)' impressions. A consideration of the localizing symptoms in connection with the pontile, nodal and cranial nuclei would involve a discussion of the cranial nerves. Thus far I have attempted to give a brief resume of the modern views upon the sensory motor functions of the brain, and in doing so I have incidentally pointed out the localization symptoms of focal dis- ease within the encephalic mass. The localization of the mind and the relationship of these sensory motor functions to mentalization is a vastly more difficult problem. Nevertheless 1 will briefly attempt to indicate the probable direction which its future solution will take. Luys long ago remarked that certain convolutions were distended and hypertrophied when certain mental symptoms were present. In cases of delirium with delusions and hallucinations he found a pro- nounced gibbosity of the paracentral lobule to which a strictly motor function is usually assigned. Clinical observation has so far shown that only the motor zone possesses epileptogenous characteristics, though with epilepsy there are associated many sensory phenomena. It is unfortunate that in Lilys' observations no mention was made as to whether the delusions and hallucinations partook of the motor rather than of the sensory type; that is, whether they were mental representa- tions of abnormal kinesis or aesthesis. They seemed to show, at all events, that the cortical areas, both motor and sensory, are ideational and psychic, psychomotor and psycho-sensory rather than simply or- ganic. Mickle showed that hallucinations were due mostly to lesions in the sensory centers of the brain. The cells of the sensory areas an- preeminently receptive, and neither physiological experiment nor clinico-pathological observation has yet proved that they are in any wa\ emissive, except so far as they may send inhibitory impulses into the motor and into other sensory centers whereby there may be such a thing as a tactile paral) sensory aphasia. There can be little doubt that these sensory cells 74 2 THE NON-NEURONIC DISEASES connected, mediately or immediately, with the motor cells of the cortex. These two sets of cells, thus intimately associated, form the summit, or bend, as it were, of the arc about which travel certain sensory motor impulses, and in all likelihood constitute the true physical basis of mind. Von Monakow declared at the Thirteenth International Medical Congress, held in 1900, that he believed that the diverse elements which had to do with the psychical functions were scattered all over the entire cerebral cortex. The gross character of the general sensibility and movements of the body, as compared with the special senses, necessi- tates a larger and grosser development of the centers that subserve the former functions than those that subserve the latter. This may be the reason, too, why they are more readily determined than the latter. In connection with sight and hearing, however, there is pretty conclusive proof that the sensory and motor cells are in close juxtaposition or at least functional association. It is a warrantable inference therefore that the same ideational centers cap, as it were, the arcs about which play the afferent and efferent impulses from the eye and ear. Hence I take it that the cerebral cortex is, after all, only a grand reflex center, the summit of the highest reflex arc. It is far more delicately organ- ized, more richly endowed with elements, and more keenly specialized than the reflex centers of the cord, but in all other respects it is quite comparable to the latter, both functionally and structurally. Midway between the sensory and motor elements and in countless interconnections with them are, of course, countless neurones and path- ways, commissural and associational tracts. Mind rests upon the whole basis, afferent, interpolated, and efferent tracts and centers. It is a product of their activity and not a -thing with a local habitat in this particular spot or ganglionic mass. This does not deny the possible existence of a metaphysical mind, but physiological and physiological psychology is incapable of taking up that question. The morphological and embryological development of the brain throws a flood of light upon the relations existing between the motor and sensory elements. Among the invertebrates there is, strictly speak- ing, no such brain or spinal cord as we find among the higher order of animals. Nevertheless, the spinal cord of the latter is clearly fore- shadowed in the series of sensory-motor ganglia with their respective projecting nerves found in the individual segments of the invertebrate. We know that in the articulates and insects each segment is furnished, as it were, with a little brain of its own, which is nothing more nor less than the summit of the reflex arc for that particular segment. We furthermore know that the ganglia of the more forward segments of the creature are but a hyperdevelopment, caused by an increase of local function, of that part of the primitive cord or elementary chain of ganglia. The explanation of the cranial nerves is only possible upon a Study of them from the evolutional and embryological standpoint; for whereas the visceral components of some of them, as. for instance, trie vagus, are still traceable to their primitive segmental origin, others have disappeared or become incorporated with adjoining sensory and motor nerves as to quite obscure their primitive simple arrangement. In the lower cord the visceral components of the segmental nerve stip- THE NON-NEURONIC DISEASES 743 ply have completely disappeared or been replaced by the sympathetic, leaving only the cutaneous and muscular nerve supply represented in the posterior and anterior roots. All of which proves the primitive segmental structure of the entire cerebro-spinal axis. In some of the higher representatives of the worms and insects the ganglia of the most anterior part of the body become massed together and undergo a somewhat fuller development to subserve the purpose and function of special senses. In this way they assume in a measure the general char- acteristics of the vertebrate brain, and in their further phylogenetic de- velopment become so complex and changed in form and location as to involve their corresponding nerves in the same confused and complicat- ed condition. Hence the absence of that simplicity among the cranial nerves that we see in the arrangement of the spinal nerves. Note the exquisite arrangement — exquisite for simplicity — of the nervous S3 r stem of the white ant (Termes), of the fly (Musca), and of the scorpion spider (Thelyphonus caudatus). In all of these creatures the various segmental ganglia are connected with each other like the cells of an electric battery. Some of them are so far advanced in de- velopment that they simulate, without representing, the brain and spinal cord of the highest order of animals. In the vertebrates and even in their highest representatives, man and the apes, the primitive inverte- brate structure of the whole cerebro-spinal axis is not wholly lost. As Owen says, a vertebrate is a clothed sum of segments, and therefore is nothing but a higher invertebrate. In the lowest vertebrate, the amphioxus, there is only a spinal cord without, comparatively speaking, any brain ; and in all of its habits this humble creature closely resem- bles the invertebrates. Whether it be finally accepted or not that the human skull is composed of a number of metamorphosed vertebrae, the emibryological development of the brain and spinal cord out of the one continuous germ layer and the remarkable similarity of the brain and cord in their more gross structure and functions, are all extremely indicative of the primitive characteristics of the encephalon of man. If there be any truth whatever in the principle of evolution, it seems to me that it is not irrational to look upon the brain of the highest vertebrates as a more highly developed portion of the spinal marrow or ganglia of the cord lying most frontad. Tn speaking of what he calls the neurone-complex in its relation to brain function, von Monakow says that it is the sum of individual neurones, which articulate one from the other, forming progressively coordinate systems (projection systems of Meynert), of which systems the neurones become greater and greater in extenl as they approach the cortex (cells of projection and association) the minimum of winch is necessary to produce in the adult a simple nervous act such as a lumin- ous impression. Romanes, in his Mental Evolution in Animals, adopts a somewhat similar view when he undertakes an explanation of con- sciousness and its dej>endence upon the integrity of the cerebral e rtex ; for he likens cortical activity to the reflex function of the lower parts of the central nervous system, and then says thai consciousness is the result of the increased resistance which the original sensory impulses meet with in their passage through the more highly developed complex 744 THE NON-NEURONIC DISEASES and intricate structure of the cortex. As von Monakow notes, the sensory tracts, upon which consciousness and intellection primarily de- pend, are composed of a great number of these "neurone-complexes," all of a similar nature, the more central of which are myelinized first, while the more peripheral ones are myelinized later. If all this be so, we should expect some similarity between the arrangement of the sensory-motor elements of the brain and those of the vertebral segments of the cord. The motor cells would not be identical with, but might lie in close proximity and intimate relationship with the corresponding sensory cells. The extreme simplicity of the arrangement of the cells in the cord is, of course, quite obliterated in the brain by the overcrowding of so vast a number of nervous elements in so confined a space as the cranium. The rolling about of the en- cephalic ganglia in their embryological and evolutional development, so as to accommodate themselves to the constrained limits of the skull, results in a more or less complete disappearance of that relative and simple arrangement of the sensory and motor elements seen in the vertebral segments of the cord. We can clearly recognize, however, that the ganglia and cerebral cortex are like the centers of the sensory motor reflex arcs found in the cornua of the cord. It is observable, for instance, in the relative arrangement and mutual development and de- pendence of the motor and sensory areas of the brain. General sensa- tion is the least specialized of man's sensory functions, and being co- extensive with the entire surface of the body, proves man's connection with the lower animals. The contemporaneous development, the simi- larity in extent and the relative anteroposterior location of the cortical sensory and motor areas, all reveal their connection with similarly re- lated parts of the body and their homology with the sensory-motor arcs of the cord. Their unusual development in size and functional activity gives them a preponderance over the corresponding segmental elements of the cord, and in their function, especially, makes them to appear as though they were voluntary while the latter are involuntary. But vol- untary and involuntary are relative terms, merely expressing different degrees of the same form of nervous activity. The psychic functions of the cerebral cortex are involuntary quite as much, if not in so glaring a manner, as are the involuntary functions of the various segments of the cord. They are both alike a form of reflex action, but instead of the cerebral reflexes being direct and unconscious, as the spinal reflexes are, the more sensitively and highly organized character of the cortex causes them to assume the nature of consciousness and self-will, while the con- nection of the cortex with the segments of the cord, as these in turn are again connected with one another, causes the cortical reflexes to be transmitted and perceived through the medium of the spinal centers rather than directly from the periphery of the body. In regard to the special senses of sight and hearing, the reflex sensory-motor arc does net pass through the cord or its ganglia, but is limited solely to the encephalic ganglia, while their sensory elements are always posterior to and more or less in close proximity with the motor elements just as they are in the spinal segments. The bilaterality of the cord, both in structure and in function, is THE NON-NEURONIC DISEASES 745 duplicated in that of the encephalon. At the session of the Societe de biologie de Paris, May 25, 1889, M. Dupuy reported a case that showed the identity of the two hemispheres of the brain in regard to function. The patient possessed the power of moving the two eyes in different directions simultaneously. Even the perceptive centers seemed to be distinct, in spite of the opinion of Horsley to the contrary, for the young woman saw objects with one eye, and when she tried to use both eyes together to view the same object she was overcome with dizziness. Magnan has had four cases of this sort, one of which he reported in full. The patient heard one class of statements in one ear and another in the opposite ear. Agreeable ideas only were readily received on one side of the head, disagreeable ones on the other. The independence of the hemispheres has for a long time been a subject of discussion and partial acceptance among neurologists. Dr. C. L. Bruce reported in Brain, 1895, a case of double consciousness in regard to which he con- cluded that there was right hemispheric melancholia or dementia, with left hemispheric mania. Kiernan has also reported two cases which led him to believe in the independent action of the hemispheres. The com- ing possibility of a complete independence of action on the part of the hemispheres in regard to their higher, or psychic, function would seem to be foreshadowed in the fact already well recognized that the more complex and independent movements of the body, such as those of the hand, have less of a bilateral representation in the cortex than do those movements which are less complex, such as the movements of the respiratory and deglutitory muscles. Speech and its mechanism have attained such an exalted degree of development and specialization that they are already subserved by one hemisphere independently of the other. Phelps' recent conclusions from a large clinico-pathological analysis, that the left brain is the preeminent seat of the mental faculties, is indicative of the independence, in part at least, of the hemispheres. Brown-Sequard insisted long ago upon the identity of function in the two hemispheres, and physiold'gists are now beginning to recognize the truth of this fact and to admit the possibility of their independence of action. The whole question is most interestingly discussed by Dr. Ireland in his work The Blot on tJie Brain. I conclude, therefore, that man's mental activity is largely auto- matic and purely reflex; that the cortical centers for ideation arc con- stituted, localized and interrelated much like the reflex centers of the cord, but that the larger and more complex development of the former confers upon them the possibility of a higher and mon- complex form of activity; that the sensory and motor elements of the cortex are not identical, but are separate, are closely intermingled, and are most intimately associated with one another in function; and, finally, that mentalization is somehow subserved by the entire cortex and its more or less independence of action in the two hemispheres. If this progressive differentiation of function, based upon the principle of simple reflex action, continues within the encephalon as it has up to the present time in the evolutional development of the whole nervous system in animals, it may ultimately happen that the mind will acquire the power of thinking upon two or more subjects at one and 746 THE NON-NEURONIC DISEASES the same moment. Its capabilities in this direction will be immensely enhanced, and the old dictum of the psychologists that the association of two or more ideas is always consecutive, never coincident, will be overthrown. In his essay upon the Molecular Dynamics of the En- cephalon, published in the American Journal of Insanity for October, 1889, Dr. Williams hints at this future possibility. "Who knows," he asks, "but that the more fully evolved mind of the future shall learn, after the present mind has come to look with stunned discouragement upon the vast field of knowledge presented to its view by an advanced civilization, gradually to so adjust its fibres and its vessels by isolating them into separate fields momentarily (since the isolation is the acme aimed at by the higher mind in its concentrated workings to-day), that consciousness may, in fact, come to be not a single energy, but a double, triple, quadruple or multiple energy ? Imagine, for example, the school boy of the thirtieth century studying his mathematics visually with concentration, while orally listening to a lecture on a different topic. In all seriousness, there seem to be some reasons for believing that such a seemingly paradoxical mentality may be evolved." The tendency of mental and brain development is certainly in this direction. It is already so striking a characteristic of the human mind as to place man in a position above the lower animals. The separation of the various representative areas of the cortex, so that even now an animal can feel a prick of a pin in the foot, move the paw and listen intently to some unwonted sound, all at the same instant, would seem to give promise of such a future possibility in brain development. A mere glance at the localization of the various centers of the cerebro- spinal apparatus suggests the similarity of the brain and cord in regard to their primitive structure and inclines one to believe in the more ancient and higher development, both anatomically and physi- ologically, of the more forward part of this apparatus. The principle of inhibition, ever since its discovery in connection with the cardiac nerves, has been applied to the highe% cerebral functions, but with variable success until quite recently. When closely studied it appears that inhibition is the highest function of nervous matter. To inhibit an action and thus to control it, presupposes on the part of a nervous cen- ter a finer degree of organization than does the power merely to receive and transmit reflex impulses. Inhibition is the basal phenomenon of the higher mind, and is of such a startling character that it gives us the notion of an absolute independence resulting in free will. An afferent impulse is reflected into the motorial sphere, but is checked or inhibited by some other afferent impulse or impulses before it eventu- ates into actual movement ; and at once we jump to the conclusion that our free and independent will has been the cause of the motor phenom- ena. The inhibitory function of the mind is admitted by all the physi- ologists and psychologists. The former assert that were it not for this magnificent function of all the cerebral centers, or at least of some of the highest among them, our bodies would be kept in a constant state of convulsion through the manifold impulses which our nervous systems are ever receiving and reflecting outward again to the muscles. Mills speaks of the higher psychical centers in the forebrain as con- THE NON-NEUROXIC DISEASES 747 stituting the "inhibiting lobe." When we recollect that all mentalization is based upon sensory and motor phenomena, and that the cortical areas for the psychical representation of these phenomena are quite fairly outlined, we are not surprised that there should be so much mystery surrounding the functions of the unknown areas of the brain. We noted that injuries of the prefrontal lobes always gave rise to mental deterioration with abnormal psychical phenomena, while the best physiologists admit that one function of this terra incognita of the cerebral cortex is at least that of inhibition. Putting all facts and in- ferences together, then, we may conclude that the power of inhibition is the last of the acquired functions and is in all probability the special characteristic of the most highly organized nervous matter; that this power resides in the gray matter of the prefrontal lobes to a very large degree ; and that therefore these lobes are preeminently the psychical centers, though the mind in toto is the result of the combined activities of all the sensory motor elements of the cortex. We may even infer that this anterior, highly specialized portion of the brain differs from the rest of the nervous apparatus in degree rather than in kind. Not only do the higher cerebral centers exercise an inhibitory con- trol over the spinal cord and lower centers, but certain cerebral centers clearly exercise the same function in relation to other cerebral centers. I suggested this fact a long time ago. This intracerebral inhibition was again pointed out by Pick, of Prague, before the Thirteenth Interna- tional Medical Congress in 1900, and illustrated by the inhibitory con- trol which the auditory speech centers exercise over the motor speech center. As is well known, persons suddenly attacked with word- deafness exhibit in addition to paraphasia, a special symptom, log- orrhcea. The importance of this in this connection is that it furnishes the first positive proof that in the sphere of the higher psychical phe- nomena, the mechanism of inhibition enters just the same as it docs in the domain of the lower nervous system. The known sensory and motor cerebral centers which have been described include, it will be remembered, the central convolutions, part of the first and second temporal, part of the occipital, the hippocampus. the uncus, and part of the limbic lobes. These areas are in connection with what Flechsig calls the projection system of fibres. In function they are identified with voluntary movement, general and tactile sense. bearing, sight, smell and taste. They represent approximately about One-third of the whole brain surface. The remaining two-thirds do not seem to connect with parts of the nervous apparatus lying below the brain, but with one another and with the parts already referred to. Flechsig calls these latter areas the "higher centers." or "association centers," and believes that they have to do especially with the com- plex mental acts, a view, however, in which he is not well supported by other authorities. As Sachs argues, projection and association fibres probably arise from every cortical area. Even Flechsig himself has recently admitted that the projection and association areas are not distinctly defined as he originally believed them to be. The generally accepted idea that the frontal lobes preside over the higher psychic phenomena need not preclude the idea that the entire C rt*tex is more 74-8 THE NON-NEURONIC DISEASES or less involved in the act of thinking. As Hitzig remarks, Flechsig's opinions upon the purely intellectual function of the association areas are at the present moment extremely hypothetical. Even the theory which localizes the so-called memory-pictures in certain cell groups is not absolutely proved. About all that can be affirmed positively, as the result of the brilliant myelinization method of investigation em- ployed by Flechsig, is that in the cortex certain areas are primordial, in which areas the projection bundles are so much thicker than they are in other cortical territories ; and that the development of the sensory centers precedes in all likelihood that of the cortical areas which serve as a basis for the intellect. To go further than this and declare that the intellect is the result of special, limited cortical foci, with a par- ticular anatomical structure, as Flechsig is inclined to do, is hardly allowable. The work of Flechsig is admitted by all, however, to mark an important advance in our knowledge of the structure and function of the brain. In the evolution of the nervous system the centers of the medulla oblongata which preside over the vital processes may be the oldest in time. They are first because they are necessary to life itself. They take precedence phylogenetically. Ontogenetically, however, there are centers which precede even these in importance, and if, as the biologists tell us, the more highly organized matter is probably the more ancient (though this is not an established fact by any means), these higher psychical centers may be, after all, the oldest in time, while those of the medulla may be later differentiations of primitive nervous matter for the special regulation of the vital processes. It is not improbable, therefore, that the highly specialized inhibitory centers of the brain are the most ancient portions of the nervous apparatus, so far as their mere evolution is concerned, though from the embryological and physi- ological standpoint the cord is to be regarded as the primitive factor. In its influence upon the next lower centers the forebrain may be compared to the entire brain itself in its influence upon the centers of the medulla and spinal cord. In both cases the influence is chiefly inhibitory. Thus the inhibitory function is graduated from that of the highest psychical centers down to that of the lowest organic cen- ters of the cord where it is practically so overpowered by the supreme inhibitory influence of the highest centers, that it appears to be almost entirely absent, reducing the cord, as it were, to a mere organ of gross and unobstructed reflex action. From its evolution, therefore, the con- clusion may be drawn that the entire cerebro-spinal axis is a series of connected sensory motor ganglia, reflex couplets, or arcs, differing in its several parts more in degree than in kind. The more frontal ganglia subserve the purpose of psychosis, which in its highest manifestation is the expression of an inhibition and which differs from the spinal cord neurosis chiefly in respect to consciousness. Consciousness and its physiological explanation are in need of much elucidation ; its localiza- tion is still an undetermined question. Brain and spinal cord are never- theless shown to be mere arbitrary divisions of a uniform, continuous, composite organ whose functions are more or less the same throughout THE NON-NEURONIC DISEASES 749 ■all of its parts, these functions being simply adaptive modifications of the primitive sensory-motor reflex. SYMPTOMS OF DISEASE OF THE BRAIX. The nervous system, as I have previously pointed out, is a uni- form, similarly arranged network of neurones throughout all of its parts. Every evidence points to the fact that these neurones possess, per se, the same general functions wherever they may be located. In- tracranial neurones are no different, structurally or functionally, from intraspinal neurones. Association and commissural tracts are prac- tically the same as projection tracts and their extension into peripheral nerves. The only dissimilarity between different parts of this vast and beautiful network of irritable cells called neurones is to be found in certain adventitious elements, such as the sustentacular structures and the end-organs. The nervous system is a single organ with a single uniform func- tion. Neural irritability and self-nutrition are its only essential func- tions when it is contemplated apart from all of its mere connections with other organs and its juxtaposition to certain connective-tissue and vascular elements. If it were thus entirely independent and separate from everything else, its symptomatology would be a matter of mar- velous simplicity. The simple function of neurility, or nervous irritability, inherent in the neurones, is, however, made to appear very complex and varied by the fact that different groups of neurones are connected with different structures in different parts of the body. So far as the neurones them- selves are concerned, sensation is the same in the optic as it is in the spinal neurones ; motion is the same in the facial nerve neurones as it is in the cardiac nerve neurones ; secretion is the same in the salivary gland neurones as it is in the renal cell neurones. To the particular end-organs of sense, motion and secretion, the neurones merely fur- nish a peculiar form of irritability ; or, to state it better, they are irri- tated in their own peculiar way by certain sensory organs, which irrita- tion they transfer, as a form of excitability, over to certain motor and secretory organs. The functions of the nervous apparatus may thus be regarded as primary and secondary. Neurility and self-nutrition arc its primary functions; motion, sensation, trophism are its secondary functions. In the diagnosis of a nervous disease the pathological diagnosis involves, and is largely dependent upon, the disturbances of the primary functions. Primary and secondary degenerations, inflammations, etc., all have to do with the manifestation of neurility and self-nutrition in the nervous elements. We have no special names for these primary Symptomatic manifestations of disease in the neurones, because at the time that neurological nomenclature was created the neuronic structure of the nervous apparatus was unknown. We use the terms paralysis, irritation, spasm sometimes in the sense of deficient or exaggerated neurone irritability. It is obvious, though, that tin- utius are used in a metaphorical sense. We are applying, in using them, our ideas 750 THE N0N-NEUR0NIC DISEASES formed from muscular manifestations to the neurone manifestations^ We say a nerve is paralyzed, whereas the word paralysis specifically means loss of motion or sensation. This loss of motion or sensation may indeed be due to the death of the neurones, but it may also be due to damage in the muscular tissue or sensory end-organ. It is ob- vious that we are in need of a nomenclature to distinguish neurone ces- sation of function apart from mere muscular or sensory cessation of function. Until we have such a nomenclature, we must still go on applying terms symptomatic of non-neural disturbances to the symp- tomatic exhibitions of pure neural disturbances. Because of this hiatus in neurological nomenclature, it is all the more imperative that we should most carefully distinguish the neural from the non-neural mani- iestations of disease. We must be careful not to attribute every paralysis to nervous trouble. We must not imagine that every visual disturbance is indicative of disease in the eyeball. We must not fancy that every parsesthesia is a sign of neuronic disorder. Like a great wall around the nervous system is the aggregate of all these peripheral end- organs that are in relation to it. Behind this wall is hiding the nervous network which is uniform in structure and function throughout all of its parts. There are diseases which belong to and damage the end- organs or outer wall. There are diseases that damage the nervous ap- paratus hiding behind it. The two must be carefully differentiated, else most disastrous blunders will be made. Nine-tenths of neurological diagnostic criteria involve the mani- festations in the outer wall around the nervous apparatus, the end- organs. As these end-organs are so varied, we have a varied symp- tomatology. In this way we localize lesions within the nervous system. I repeat with emphasis that these localization symptoms which are based upon the secondary functions of the nervous apparatus must not be imagined as giving us any special information as to the primary or neuronic disturbances. Inferentially they help us to determine whether the disease is neural or non-neural, but only inferentially. The latter must be made out from the etiology and certain special phenomena like the electrical reaction of degeneration. The localization symptoms being the most obtrusive, and those upon which we must largely depend in the making of our diagnosis, it is profitable that we should discuss them along general lines before taking them up in detail under the head of special diseases. The ideal localization of neurological manifestations would, of course, be the neuronic. We have attained this desirable goal in re- gard to a few diseases. We recognize the neuronic localization of the lesion, for instance, in locomotor ataxia, progressive muscular atrophy, certain degenerative neuritides. In time we may determine the neuronic localization of many more, if not all, of the special diseases to which the nervous apparatus is liable. We may be able in time to localize the neurones that are at fault in hysterical and other psychic troubles. At present our knowledge of nervous physiology is so small and coarse that in many of our localizations we cannot get farther than such crude anatomical subdivisions as the brain, spinal cord and nerves. We have refined a little upon these crude localizations without yet attaining to THE NON-NEURONIC DISEASES 75 1 the neuronic refinement of them. Thus, in the brain we have discov- ered certain areas of the cortex as having certain peripheral relation- ships that give them apparently distinct, secondary functions. The outlines of these areas, however, are still most obscure and their inter- relationships with one another are still mysterious. In regard to other parts of the cortex and the great central ganglia at the base of the brain, we know practically nothing, though functionally they are at the basis of mentalization and other cerebral phenomena. In a coarse way, then, we can distinguish what we are pleased to call brain symptoms. A few of these brain symptoms have been dis- cussed in the previous section upon the Physiology of the Brain and Cerebral Localization. Here I purpose to recapitulate a few of the more important manifestations which indicate in a rough way disease 01 that part of the nervous apparatus that happens to constitute the cere- brum. The symptoms of disease of the medulla and cerebellum will be considered later. In the making of a diagnosis it is important, first, to decide that the trouble is a brain disease, and secondly, to localize the lesion in some part of the brain. The former can be decided from general symptoms ; the latter must be made from focal symptoms. General Symptoms of Disease of the Braix. — Of these head- ache is probably one of the most common. Nearly every disease of the brain, whether neuronic or non-neuronic, organic or functional, is more or less accompanied by headache. It must not be inferred from this, however, that all headaches point to brain disease. There are many nerves in and about the head that may be variously affected without the brain being the direct source of the pain. Nevertheless, it is a most important truth that when the brain is affected, headache is sometime or other present. It would lead me too far to discuss cerebral headaches here; es- pecially as I have taken up the subject of cephalalgia in a special chapter. By itself, headache is of very little value as a symptom. In con- junction with other symptoms it is of tHe greatest value. In organic cerebral disease the headache is somewhat definitely localized, is asso- ciated with tenderness upon percussion, is more or less constant, though it may be with periods of exacerbation, and is of a dull, boring char- acter, or sharp, knife-like and penetrating. It is increased by such sensory irritation as loud noises, bright light, sudden jars caused bv coughing, sneezing, etc. Circulatory disturbances may modify it. In the psychoneuroses, such as hysteria and neurasthenia, it is apt to be more diffuse, variable as to intensity and location, and to a certain extent under the influence of the mind. Vomiting of a particular type is characteristic of brain disease. It is spoken of as cerebral or nervous vomiting. The suddenness of it has caused it to be named "projectile" vomiting. It is frequently associated with headache, and comes and disappears with the nervous exacerbations. It is usually not dependent upon the ingestion of food, but may take place at most unexpected times. It is devoid of Strain. In all these respects it is unlike the vomiting of gastric disorder. The 752 THE NON-NEURONIC DISEASES vomiting of alcoholism is a gastric symptom. The vomiting of fever, unemia, migraine, hysteria and some cases of neurasthenia is due to cerebral disturbance. The most characteristic form of projectile emesis is seen in tumor of the cerebellum and medulla. In combination with other symptoms it is all but pathognomonic of disease here. It occurs in meningitis, abscess, hemorrhage and other lesions that increase the intracranial pressure. Irritation of the gastric vagus center in the medulla is the probable explanation of it. Nausea may or may not accompany the vomiting. Sometimes it is very distressing. Occasionally it occurs without the hyperemesis. Vomiting, with or without nausea, associated with cephalalgia and not accountable for on the ground of gastric trouble, should arouse the strongest sort of a suspicion of intracranial disease. An immediate examination of the eyes is then called for to' determine the absence or presence of choked disc. Vertigo is not an important brain symptom. It is not the same as incoordination or mere loss of equilibrium, which is so marked a feature in cerebellar disease. As I have discussed the nature of vertigo in considerable detail in a section by itself, I will omit further consid- eration of it here. Temperature. — Elevation of the temperature accompanies many affections of the brain, but it is due to the general disease process and not to the special fact that the latter is located in the brain. Infectious inflammatory states produce fever when they attack the brain, just the same as when they attack any other organ. It is not always easy to determine how much of the temperature is due to the general infection and how much to the local inflammation constituting part of that general infection. A tubercular meningitis may exhibit an elevated temperature, not on account of the inflamed meninges so much as on account of the general constitutional tuberculosis. Sometimes there is a rise of temperature in disease of the brain when the pathological process is not markedly inflammatory. It is then the expression of a disturbed nervous function. Very high tempera- tures occur thus. Lesions of the pons and medulla, especially, provoke such pure neurotic temperatures. They may even run as high as 10S degrees F. and be very misleading. In hysteria, neurasthenia and other neuroses, neurotic elevation of temperature certainly does occur at times. There are supposed to be heat regulating centers in the brain, and they probably are intimately associated with the vasomotor centers. It is a difficult matter at times to differentiate a mere nervous elevation of temperature from one due to an infectious or inflammatory process. It may be said, however, that pure nervous temperatures are apt to be sudden, changeable and temporary. The pulse does not exhibit any corresponding relationship to them. When they are very high they are usually bad signs. Sometimes there is a serious intracranial disease without any alteration of the temperature. In the initial stage of cerebral hemorrhage the temperature drops below the normal. Pulse. — It is a curious fact that the pulse may be much less fre- THE NON-NEURONIC DISEASES 753 quent in febrile disease of the brain than it would be with the same febrile affection in any other organ. In some diseases of the brain it may be subnormal. This is seen in tumor and abscess. Simple inflam- matory and infectious processes usually have a rapid pulse, though there is less frequently here than in disease elsewhere a correspondence between the pulse and the temperature. A rapid pulse may indicate damage to the vagus center in the medulla. The slow pulse of menin- gitis in children, of apoplexy and of general intracranial pressure is believed to be due to over-action of the inhibitory .center upon the heart. Respiration. — The respiratory phenomena of brain disease are numerous and important. The respirations are usually diminished when the brain functions are lowered. I recently saw a dangerous ex- ample of this in a case of chronic lead encephalopathy. In coma and stupor it is often lessened. In meningitis and apoplectic strokes it assumes the Cheyne-Stokes type and becomes a bad omen. This type of breathing occurs in other diseases, such as pneumonia, scarlet fever, diphtheria, opium poisoning, but it is a less grave symptom in them than it is in brain disease. In the Cheyne-Stokes breathing there is a remarkable cycle of increasing and decreasing depth. There are first a few superficial breaths. These gradually increase in rapidity and depth and become noisy. Then a fall takes place until there is actual cessation. After a pause, however, the slight respiratory movements begin again, grad- itally increase in intensity, until there is a deep dyspnceal breathing once more and the respirations again lessen. The pupils usually con- tract and the heart action diminishes during the pause, which may last from five to forty seconds. The duration of the cycle may be fifteen to seventy-five seconds. Never do the respirations of each cycle exceed thirty in number. Sometimes the respirations begin with great suddenness. Some- times they begin and terminate suddenly. This has been seen in meningitis and is known as Biot's type of breathing. Irregular breathing and cessation of breathing occur in disease of ihe medulla. Under such circumstances the heart has been seen to continue in action and death was prevented by artificial respiration. Renal symptoms may be indicative of disease of the brain, but more often they are concomitant manifestations. Albuminuria and glycosuria, more frequently polyuria, accompany lesions in the medul- la. Cardiac disease and renal trouble are not infrequently associated with cerebral vascular disorders. Mental disorders are the caua urinary and faecal incontinence when there is no paralysis of the vesical and rectal sphincters. The polyuria of the neuroses and psychoneur such as hysteria and certain spasmodic troubles, need only to be men- tioned. Psychic Symptoms. Psychosis. — The symptoms which I have considered up to this point are so general in character that they only point to intracranial disease when they are associated with other more suggestive signs. Many of them occur in other diseases. They are therefore not in the least distinctively encephalic. The symptoms which we are now about to take up point to the 754 THE NON-NEURONIC DISEASES brain, but while often dependent directly upon brain disease, they are not necessarily so in all cases. A psychosis means a mental aberration, an unusual exhibition of mental manifestation. Were we acquainted with the true nature of the mind, there would be less confusion in connection with these symp- toms and their localizing worth would be greater than it is. The seat of the mind is assumed to be the brain ; some say the anterior part of the frontal lobe. It has even been affirmed that in right-handed people it is in the anterior left frontal lobe. As a matter of fact, we know nothing of its localization. It probably has its chief focus of manifestation in the brain cortex, because there the highest neurone cell-bodies are located, but that its existence is not due solely to the cortex in its healthy state is shown by the fact that it can be made to exhibit most extraordinary and even insane manifestations by dis- ease in parts of the body remote from the brain. In other words, mind is more or less coextensive with the nervous mechanism. How are we to explain this apparent contradiction that the mind is located in the brain and yet is so subject to remote neural disorders? It seems to me that no psychology offers so clear an explanation as does the voluntaristic psychology so. ably taught by Wundt, of Ger- many. The mind is not a thing or entity with a local seat. It is but the name which we apply to the internal interplay of the afferent impulses one upon the other. It is a pure function, a secondary product. Even the use of the term product is misleading, for mind is not even pro- duced from something; it is the something itself, and that something is the inner operation of the thousand and one impulses passing up to the brain along the afferent pathways and playing against and in and out among themselves. They are the same impulses, perhaps slightly modified in the cord, that started from the periphery, but now in their aggregate complex intermingling constitute what we call the mind. Mental activity is but the insi'de view of the neurone activities whose outside view is given us by physiology. Physiology is the objective contemplation of elementary nervous functions ; psychology is their subjective contemplation. Physiology and psychology are thus facing each other and are explaining from directly opposite points of view the same set of neural manifestations. They both analyze and combine the phenomena which present themselves in the workings of the nervous system on the outward and the inward sides respectively. Outwardly the physio- logical elements are the neurones and their manifestations under vari- ous stimuli. Inwardly the psychological elements are the sensations and feelings. When the outer elements are collected together and work normally, we have a normal, physiological nervous apparatus; when the inner elements are taken in the aggregate and work together normally, we have the normal manifestation of mind. The psychic elements include the five senses and the feelings associated with them. Out of the innumerable combinations and recombinations of these psychic elements the various faculties of the mind, such as the mem- THE NON-NEURONIC DISEASES 755 on', the imagination, the will, the judgment, are evolved. To show how would be, of course, beyond the province of this work. This physiological psychology and explanation of mind is called voluntaristic because it teaches that all of the faculties, like the volition, are mere processes. It has long been recognized that volition is but a convenient term to express the overflow of certain afferent impulses into efferent impulses. The will is not something apart by itself ; it is a high order of functional manifestation between certain psychic ele- ments ; which psychic elements in turn are but the inner representation of the primary physiological sensations. What is true of the will is true of the mind and all of its so-called faculties, and hence the name voluntaristic for this psychology. I felt it necessary to introduce here this brief statement of the voluntaristic explanation of mind, because it underlies the explanations of the psychoses presented in this work. The mind being thus seen to be dependent upon the physical senses, just as a chord struck upon the piano is dependent upon the individual notes, but not being the same as the physical senses, but rather the result of their aggregate activities, just as the musical chord is not the same as the individual notes, but is something different resulting from their aggregate sound, it can readily be understood how it can be disarranged by disturbance both in the individual senses and in their mere interrelation to each other. Just as an inharmonious chord can be produced out of a harmonious chord upon the pianoforte by one note of the chord being broken on the one hand or by the introduction, abstraction or other disarrangement among the notes themselves ; so a psychosis can be set up by a physical disease in any one or more of the anatomico-physiological elements upon which mind depends, or by a functional disarrangement among the elements which otherwise may be perfectly norma). In this way we can account for mental symptoms that sometimes seem to be due to actual physical disease and that at other times do not seem to have any apparent physical basis at all. For example, maniacal manifestations are sometimes clearly due to gross meningo-encephalitic changes ; at other times, as in certain forms <»f insanity, they seem to occur without any discoverable physical basis. Hysterical exhibitions are clearly enough due to physical diseases in some instances; in other instances no changes of a physical sort have ever been detected. In the former case the psychosis was a physical phenomenon ; in the latter it was purely a functional exhibition. The reader will now understand why the psychic symptoms which I am about to tabulate are not absolutely indicative of organic brain disease, though they are always indicative of the functional disturbance of the mind. He will also note how important it is not to jump to an immediate conclusion that the brain Is diseased or tin- mind hopelessly affected as soon as any of the following mental symptoms show them- selves. The cause of these symptoms must always be sought for first in some physical disease. If this is removed, they may vanish, [n some cases, however, these mental symptoms will appear without the existence of any physical disease. They are probably the result then of disarranged action among the primary psychic elements, such as is 75^ THE NON'-NEURONIC DISEASES sometimes seen in the primary psychoses like the primary hysterias and fundamental insanities. None but psychic treatment can hope to reach these cases, and even that cannot be expected to do much in severe examples. Wundt has shown that there are two sets of psychic elements, the psychic sensations and the feelings, out of which the entire mind is elaborated. The sensations are the psychic counterparts of the physio- logical five senses. They combine and interplay to form the memory, imagination, reason, judgment and intellectual faculties. The feelings are the indefinable appreciation of the psychical sensations ; they are accompaniments of the latter. They are the result of the functional appreciation of the psychic elements by the combined product of those elements, or the beginning mind or ego. The}' are the counterparts, therefore, in a way, of the objectively combined physiological elements that enter into the constitution of a normal nervous mechanism. They combine to form emotions, which emotions determine the motives, and so on up to the elaboration of the will. At the bottom of mind are the psychic sensations and psychic feelings ; at the top, and gradually elaborated out of these along two lines, as it were, are the judgment and the volition. When the mind is affected with disease the top, of course, falls and the amount of the loss is proportional to the extent of the disease. The judgment and the will are the first to topple. The next lower processes are thus left without the inhibition normally exercised upon them by the higher faculties. In this way we see in disease the imag- ination on the one hand running riot in all sorts of delusions, and the emotions on the other hand bursting out into uncontrollable exaltations and depressions. If still more of the mind is overthrown, the imagina- tion and the emotions disappear, amnesia is present and the victim sits in a condition of stupor. The next stage still lower is one of complete dementia, with the patient in a mere vegetative state. The last stage is the total abolition of all mind or mental death. This last condition is probably never seen so long as the lower or vegetative functions are active, for even the lowest idiot has a trace of a con- scious mind still left. Total abolition of mind is only present in death. As a temporary condition, total abolition is seen in coma of a profound type. I will now trace downward this gradual disappearance of the mind as it is seen in the more common psychic manifestations of brain disease. Abnormal Intellectual States. — These, of course, are innumerable and varied, but as they are the outgrowth of psychic sensations, they involve illusions, hallucinations and delusions. The basal elements be- ing disturbed, the composite ideas and all their inter-connections in the most exalted forms of mental acts are also disturbed. Hence conscious- ness, attention, cognition and recognition, memory, imagination and reasoning are all more or less upset. An Musi oil is a misinterpretation of an external impression. By reason of disorder in the transmitting apparatus, afferent special and common sensory nerves and their endings, an impulse is perceived dif- THE XON-NEURONTC DISEASES 757 ferently from what it should be. The essential feature of an illusion is that there is an actual external stimulation of the nerves, but it is not recognized in its true light. Illusions may occur through any of the avenues of sense, but they are most common in the visual and auditory fields. The victim of illusions beholds a brother, say, and mistakes him for a total stranger ; he hears a whistle and declares a cat mewed ; he lifts a feather and it seems like a piece of lead. Mild, temporary illusions are common enough in ordinary life among perfectly normal people. Our senses are not perfect and they are constantly giving us inaccurate information; but we immediately correct the errors and so go on as normal beings about our occupations. The insane individual is not able to correct his illusions and so he acts accordingly in a way that is most extraordinary and unusual. The immediate cause of many illusions can readily be discovered in the afferent peripheral apparatus. It may be wax in the ear that is disturbing the ingoing auditory impulses. A stone in the bladder may be interpreted by the patient's sensorium as a small animal that has gotten in there. Most of the so-called hallucinations in childhood are really illusions. Pavor nocturnus may sometimes be due to them. In the majority of cases the immediate cause of illusions is a dis- order in the perceptive center. This is shown by the fact that the illusions often harmonize with the delusions present and thus partake somewhat of the nature of hallucinations. It is obvious that an illu- sion does not necessarily represent a very profound degree of mental alienation. It becomes a sign of insanity only when it remains un- corrected. A deeper degree of mental disturbance is revealed in a hallu- cination. Here non-existent objects and impressions are perceived; or, in other words, the sensorium projects into the outer world, through the avenues of sense, its own formed sensations, which sensations are the result of a disorder in the sense-apparatus or nerve-center. Unlike illusions, hallucinations are more than mere misinterpreta- tions of veritable stimuli. They are the perception of stimuli that are immediate to the sensorium, but are accepted as being veritably objec- tive and outside of the sense-apparatus. Hallucinations are also common in normal life, but not so com- mon as illusions. They are immediately corrected in the same way as the latter. The continuous feeling of the absent member in an ampu- tated arm or leg is an excellent illustration of an hallucination. Kvery one has heard a noise sometime or other when there was actual silence, or seen the shadow of a figure when no person was near. Illusions and hallucinations play a large role in spiritualistic phenomena and the seances elaborately built up upon them. Hallucinations arc due to a disorder of the sensory nerve centers and are therefore more serious and fundamental than are illusions, which are usually due to disease in the peripheral sensory apparatus. Hallucinations often partake of the nature of the patient's de- lusions and thus represent a most advanced state of alienation. Mis delusions being of a persecutory character, say, he assumes a frightened manner, begs to be protected and shows in every way that he imagines 75& THE NON-NEURONIC DISEASES he sees those who are going to harm him. Some of the attitudes of hystero-epilepsy are undoubtedly due to these delusion hallucinations. They occur in some sane people under high excitement and when they are between sleep and waking. Baillarger calls these psychic hallu- cinations, or pseudo-hallucinations. Hallucinations may be due to irritations in the cortex, but they are not regarded as signs of insanity so long as the subject of them is able to correct them and recognize their nature. Hallucinations under- lie many of the assaults and murders committed by the insane. Delusions are false beliefs. Illusions and hallucinations represent primary disorder in the psychic elements and therefore in the psychic ideas which are the outgrowth of these elements. When the inter-con- nections especially between these psychical ideas or compounds are disordered, delusions make their appearance. Delusions are therefore generally indicative of a most profound sort of mental aberration, be- cause they are not immediately dependent upon disorder in anatomico- physiological elements, but in the functional interplay and inter-con- nection between the psychical elements and compounds which are de- pendent upon them. Delusions are radical and decidedly mental. They may be dependent upon a physical disease in a physical basis, but they are also, and usually, dependent upon a complicated and complex func- tional disturbance in a very high order of mechanism. Delusions may occur in health as well as disease. It is difficult to draw a sharp line between the delusions of sanity and insanity. Estab- lished beliefs and convictions must be taken into account. The whole history of mankind is full of false beliefs. If these are not corrected by later knowledge, those who continue to live under them are not considered insane. If they are corrected, then those who still retain them are regarded as mentally unbalanced. Believers in astrology, witchcraft and mythological gods are deemed mentally diseased if they live in the heart of modern civilization. They would not have been deemed so had they lived in earlier times or did they now live among barbarians. Therefore the ability of the victim to correct his delusions must be considered when the latter are being considered as signs of mental alienation. Because we cannot always determine that ability, from our want of knowledge in regard to the patient's early influences, education and present environment, it is sometimes a difficult question to decide as to whether his delusions are insane or merely eccentric. The laity have a ready rule for determining what they consider de- lusions; for they hold everybody crazy that do not hold the beliefs that they do. To the scientific physician, however, falls the arduous task to compare the delusions with all that makes up the patient's past history and his present environment, and from that comparison deduce his conclusions as to their sane or insane origin. It is the province of psychiatry to lay down the guiding principles whereby the physician is to arrive at a final opinion. Delusions may be sensorial or ideal. The former arc founded upon illusions and hallucinations. If these are persistent and uncorrected, and the patient acts in accordance with them, he is said to be deluded. His entire mentalization is under the control of the illusion or hallu- THE NON-NEURONIC DISEASES 759 cination. Ideal delusions are spontaneous false concepts, unrelated to any sensory disturbances. A patient who believes that he is a million- aire, that he has a thousand children, that he is one of the twelve apostles, is the victim of an ideal delusion. These delusions are prom- inent in the religious sphere because religion, or man's dependence upon a superior, protecting Intelligence, is an ancient and primitive concept. They may occur in any sphere, however, especially the sexual, alimentary and in the higher regions of thought. Many of the obsessions of the psychoneuroses, the imperative con- ceptions, the fixed ideas, the phobias mentioned under the head of neu- rasthenia, are of the nature of ideal delusions. The difficulty in eradi- cating them sometimes is recognized by every clinician and shows the profound depth of their origin. At other times, fortunately, they are so slight as to be scarcely more than the similar delusions held by per- sons in perfect health. Delusions occur in toxic conditions (lead, cannabis indica, alcohol, etc.), in low adynamic states, in starvation and in the delirium of acute general diseases. Abnormal Emotional States. — As I have pointed out, the emo- tional states are the outgrowth of those psychic elements called the feelings. The feelings are the accompaniments of the psychic sensa- tions. Whereas the latter lead up to the intellect, the feelings by com- bination and recombination ultimately lead up to the will. There are mental disturbances that show themselves prominently in the emo- tional sphere, just as there are others that exhibit themselves promi- nently in the intellectual sphere. In most diseases both spheres are more or less implicated together. The abnormal emotional states assume the character of exaltations and depressions. These range all the way from health up to actual maniacal excitation of the most violent sort, or down to a most extra- ordinary depth of melancholic stupor or pure indifference. In normal individuals we often observe a high degree of emotion- ality. Emotions underlie the motives, as the word itself indicates — e-motives. These e-motives combine and overflow into the motor sphere and we have the manifestation of volition. If they are weak and inade- quate, there is no volition and the subject remains inactive. Exaltation and depression are usually dependent upon the relative activity of the mental processes. In exaltation, as its name indicates, there is excess of ideation, of muscular power (apparently?), of circula- tion, of visceral activity and of the appetites. These reactions are stimulating and pleasurable. Hence exaltation is usually accompanied by an ecstatic, satisfied and happy frame of mind. This, however, may easily run over into incoherence, verbal delirium, muscular excitability and physical violence. The condition is seen in mania, mania sine delirio, dementia paralytica, etc. This exaltation may be due to nervous weakness (loss of inhibi- tion), as well as to excess of nervous force. It may alternate with states of depression. Exaltation is sometimes termed psy clamp sia because, like muscular spasm, it is a sort of mental over-action or psychic spasm. 760 THE NON-NEURON IC DISEASES Depression is usually accompanied by corresponding physical con- ditions. There is general debility, lowered muscular power, sluggish visceral functions, reduced appetites, retarded metamorphosis. It is the opposite of stimulation and therefore not pleasurable. For this reason it is often termed psychalgia, or psychic pain. There is a w r ant of voli- tion here, a want of speech, a want of interest. Usually there is intense melancholy. This may pass over into complete stupor and indifference. An appearance of indifference must not mislead the physician, however, into thinking that it is real always. In many cases there is psychic hyperesthesia, but it is so keen and so painful psychically that all other psychic manifestations are completely paralyzed and an attitude of utter, profound indifference is assumed. Depression is seen in melan- cholia, in hysteria, in neurasthenia and in the prodromal stage of mania and dementia paralytica. The consciousness is often disturbed and its disturbance is among some of the most important of the brain symptoms. Upon the basis of the voluntaristic psychology, consciousness is the name for the gen- eral synthesis of psychical processes, in which general synthesis the single compounds are marked off as more intimate combinations. When this inter-connection of compounds (ideas and emotions) is interrupted, there is deep sleep, a faint, or coma. Disturbances of consciousness involve abnormal changes in the combination of psychical compounds, even though the compounds them- selves show no internal changes whatever. This is a most important distinction, for upon it we can explain many of the phenomena of dis- ease which involve the conscious state as apart from the mere intellec- tual and emotional states referred to above. There are probably in- numerable forms of disturbed consciousness, but we recognize in prac- tical semeiology only a few, and these I will now refer to briefly. Delirium is a characteristic disturbance of consciousness in which there is mental confusion and excitement with hallucinations, motor restlessness and illogical and incoherent thinking expressed in words and acts that betray the condition present. The mental processes are here perverted. The delirium may be active or quiet, low and mutter- ing or wild and exhausting. It is not so common a manifestation of organic brain disease as it is of cerebral intoxication, infection and fever. In the delirium grave, or Bell's mania, the symptom is most typically observed. There are alternations of active, loquacious periods, with violent struggling and attempts to leap out of the window, etc. I have often noticed that the hallucinations revolved around some line of thought which deeply concerned the patient before the disease ap- peared. Hallucinations of speech and act, indicating a basis of fear, may thus have some slight foundation. Often, however, there is no accounting for the direction of the hallucinations. They seem to spring up utterly without rhyme or reason. Loss of Consciousness. — Here there is a complete suppression of the mind. The psychic elements themselves have been abolished, or their interplay has been completely annulled. This condition is close to sleep. In fact, it is a comoulsory sleep with certain attendant phe- nomena. It is a very common symptom of brain disease. It occurs THE NON-NEURONIC DISEASES 761 under many varied conditions. It may be partial or complete. The mere vegetative functions of the body, the heart action and respiration, need not necessarily be affected in coma, though often they are. Hebetude, stupor, somnolence, sopor and coma are all degrees of the loss of consciousness. Stimulation can arouse the patient less and less as he passes from the first into the last. In coma the sensations, the reflexes and all avenues to the mind seem to be absolutely closed. The whole brain is usually affected in these conditions. Tumor, abscess, tuberculous meningitis and other forms of intra- cranial pressure and intoxication frequently are accompanied by mental stupor and dullness. The patient is listless and indifferent, but can be aroused into a momentary exhibition of intelligence. He seems to be resting under a cloud, which again closes over his mind as soon as he is left to himself. In somnolence the stupor assumes a deeper shade and resembles profound slumber. In these states the urine and faeces are often involuntarily passed, though the sphincters are normal. Food placed in the mouth may at first be chewed and then is left there with- out further mastication. The patient seems to have suddenly gone to sleep in the very midst of the act. In coma there is no response what- ever and there is no attempt at chewing and swallowing unless the coma is very light. The loss of consciousness may come on gradually or abruptly. It is not the same as syncope exactly, and yet in both phenomena the immediate cause is the anaemia of the cortex. Symptomatically the great feature of coma is the loss of all sensibility. It is seen in epileptic seizures, in surgical cerebral compression or concussion, in uraemic poisoning, in acute alcoholism, in toxic narcosis from opiates, morphia, chloral, etc., and in congestive or malignant malarial fever. Its most typical manifestation is probably observed in cerebral apoplexy, whether hemorrhagic or embolic. The differential diagnosis of the different forms of coma will be found under the heads of the disease in which it occurs. However, it is so important a symptom that a brief recapitulation will not be out of order here. In epileptic coma one must look for signs of a previous attack, such as the lacerated tongue, the ecchymoses on the face, neck and chest, the frothy saliva, the involuntary micturition or seminal dis- charge. The coma is of brief duration and i.s followed by a small rise of temperature. The unconsciousness of cerebral concussion or compression is asso- ciated with injury about the head or body, oozing from tin- cars or nose of blood, meningeal hemorrhage with gradual stupor without pronounced hemiplegia. In urcemic coma there is anasarca and slow pulse. If the urine is drawn by catheter and tested, it is albuminous and contains casts. The ophthalmoscope reveals a ncuro-rctinitis. In alcoholic coma the patient can be aroused, it is more of a condition of stupor. The breath is alcoholic and the temperature may be normal or subnormal. Alcohol can sometimes be found in the urine. 762 THE N0N-NEUR0N1C DISEASES In malignant malarial coma there are no physical or paralytic symptoms, though the rectal temperature is high. The spleen is en- larged. The blood examination for the plasmodium is final. Opium narcosis is characterized by contracted pupils, subnormal temperature and slow respiration. Apoplectic coma is profoundly paralytic. There is hemiplegia with conjugate deviation of the head and eyes, the patient looking to- wards the lesion. The pupils are dilated. The temperature, at first sub- normal, soon rises. The Cheyne-Stokes respiration, pupillary changes, alterations in volume and rate of the pulse beat are all accompaniments, though not important ones, of the comatose state. Double consciousness is a condition of the mind seen in certain hysterical states, such as the hypnotic and somnambulic, and in certain forms of epilepsy and insanity. The patient seems to have both a normal and a morbid mind and she alternates from one to the other. Some remarkable cases of this trouble have been seen. It is purely a mental disorder and seems to be due to a partial abeyance at times of the mind. It occurs in a slight form in hysterics and sometimes it is the cause of the strange wanderings and doings of epileptics. Amnesia. Mental Failure. — Loss of memory is a common sign of brain disease. It occurs in the mildest neuroses. It may be so marked that the condition becomes one of actual dementia. Retention is the first requisite of all mental activity. None of the higher faculties can exist if memory is gone. Chronic and disseminated diseases of the cortex usually have among their earliest and prominent symptoms amnesia. There are special types of amnesia, which, however, are not of sufficient importance to need detailed description here. There are certain psycho-sensory and psycho -motor states that might very appropriately be discussed here under the head of the psychic manifestations of brain disease. They are, for instance, word- deafness, word-blindness or alexia, and the disorders of speech included under the name of aphasia. As I have pointed out under the head of Cerebral Localizations, there are areas in the cortex that in some mysterious way subserve the performance of these complicated acts, such as speech and writing. For clinical purposes we can use these acts as localizing signs, and for that reason I will discuss theni along with the other more distinctive focal signs of brain disease in the fol- lowing sections. I desire here, however, to emphasize the fact that speech, reading, word-hearing and writing are psycho-physical exhibitions, and to pro- test against the too common assumption that they are the result of certain physical activities in certain limited groups of cells called "cortical centers." A speech-center, a writing-center may indeed rep- resent the point of convergence or physical inter-connection for all those anatomico-physiological processes that underlie the correspond- ing psycho-physical manifestations. Ablation of these centers neces- sarily breaks up the psychic phenomena dependent upon them and ex- pressed physically through them, but that these centers are special independent little organs, as it were, for the performance of the func- THE NON-NEUBONIC DISEASES 763 tion of speech, of reading, of writing, etc., none but the crudest tyro in psychological investigation would for a moment believe. Regarding this point, which I have long insisted upon, I can do no better than quote a paragraph from Wundt : "When the destruction of certain cortical regions produces definite disturbances, in voluntary move- ments and sensations, or when such a destruction interferes with the formation of certain classes of ideas, it is perfectly justifiable to con- clude that these regions furnish certain indispensable links in the chain of physical processes which run parallel to the psychical processes in question. The assumptions often made on the basis of these phenom- ena, that there is in the brain a special organ for the faculties of speech and writing, or that visual, tonal and verbal ideas are stored in special cortical cells, are not only the results of the grossest physiological mis- conceptions, but they are irreconcilable with the psychological analysis of these functions. Psychologically regarded, these asumptions are nothing but modern revivals of that most unfortunate form of faculty- psychology known as phrenology." DISTURBANCES OF LANGUAGE. Most authors treat of these symptoms under the general name of speech disturbances. I prefer the use of the word language, because it has a broader meaning than the word speech has come to possess in popular parlance. I believe that much confusion has been thrown around this entire subject by the difficulty one has in thinking of speech in the bread sense that we think of language. Speech has come to be rather synonymous with emissive language and more particularly articulated emissive language. It is not easy to think of written speech or mere pantomimic speech, and it is next to impossible to regard un- worded speech, whether receptive, inherent or emissive in relation to the brain, in the same clear, well-defined light that we regard verbal or articulated speech. The word language, it seems to me, is so broad and yet so accurate that it can well replace the confusing word speech. Language is the power of communicating ideas to another. It makes no difference in what manner or by what agencies the communi- cation is made, whether with vocal sounds, with recorded symbols, with gesture movements, it is all language. A mere touch of the hand may convey an idea to a receptive subject: a simple wink may mean yes or no as much as the spoken or written word. Nay, more, it is not even necessary that the person to whom the ideas are being communi- cated should understand either the spoken, written or gestured sign. So long as a sign of some sort stands for the ideas in the mind of him who is trying to communicate them, it constitutes a language. A Hottentot may try ever so hard to communicate his ideas to me. He has a language, even though I comprehend not an iota of his vocal utterances, his graphic signs or his gesture movements. He has his ideas, that is obvious; and he has his own particular symbols for them, which he has adopted for himself. Those symbols constitute hi> lan- guage. By means of them he attempts to communicate his ideas another individual, whether that individual be a human or an animal. 764 THE N0N-NEURON1C DISEASES Indeed, he communicates the ideas to himself by them, or, in other words, he attaches to those ideas of his own certain adventitious, fixed symbols which even to himself he uses to fix and recall the ideas nec- essary for the continuance of his thinking. In its last analysis, then, language is nothing more or less than the symbolising of ideas. This is a most important fact to grasp before beginning the discussion of the disturbances of language. In the definition of language two things must be clearly empha- sized. The first is that it is a symbol, a sign, a something in place of something else. The symbolizing is not conferred upon living creatures from without, but it springs originally from within. A symbol is first formed in the mind ; is first employed there ; and is then expressed outwardly. In this way language begins in animals and infants. Mere sounds, for instance, like mama or papa, originally without meaning and uttered by the infant in its feeble struggles to make its little ideas known to those about it, have become crystallized into words. Origin- ally, however, they were nothing but sounds and meant, as symbols of ideas, nothing more or less than the crow of the cock or the call of the moose. The so-called roots of civilized speech when traced back to their source reveal the fact that primitive language was imitative as far as possible. The savage who rubs two sticks together to convey an idea to his fellow savage is employing a language as much as the dog who barks and leads his master to the drowning child, or as that child when. it cries and stretches out its hands to the nursing bottle. . All of these movements and noises are for the purpose of com- municating ideas. In time these movements and noises, by repetition, by the unconscious process of mutual agreement, by combination and recombination, become the fixed symbols of higher language. They first represent something in the mind of the animal to his own mind,, and thus they become the basis of his thinking. The capacity to form and retain these symbols is what distinguishes man as a higher think- ing animal than the brute. Babies cannot think like adults and one adult cannot think like another adult, largely because their powers of fixing these linguistic symbols within their psychic mechanism is weak. Language, therefore, as an aggregate of symbols springs out of thinking and in turn assists the processes of thought. I hope the reader has noted that in the evolution of language it is thought that gives it birth. If animals had no thoughts, however primitive they may be,, language would never come into existence. This leads to the second point that should be emphasized in the definition of language — namely, that it is for the communication or representation of ideas. If ideas are wanting, there will be no lan- guage. Now animals make sounds and perform acts that are not based upon any ideas, such, for instance, as the thousand and one reflex acts, the acts of mere vegetative life, that never become language and in themselves are not in the least linguistic. They only become linguistic the moment they are made to take the place of an idea, the moment they are adopted as symbols for a thought. Winking, for instance, is per- THE NON-NEURONIC DISEASES 765 formed as a mere reflex act a thousand times a day ; it is not language until it is made to suggest some idea. The essential point in language then is that it is a symbol for ideas. As I have said before, it is perfectly inconsequential as to what the character of the symbol may be, whether it is known to any other than to the one who adopts it, so long as it is a symbol and so long as it stands for an idea, it is language. This is not the place to go into further detail in regard to this fascinating subject, the development of language, but I felt that the above brief notice of it was necessary to emphasize the fact, which seems to be lost sight of by some physiologists in their discussion of the cortical speech centers, that language is a psychic and not a mere physiological phenomenon. It is the outward panorama, as it were, of ideas. Every discussion of it and its disturbance involves a discussion of the nature of ideas. These, as I have shown elsewhere, are what we call in modern physiological psychology, psychical compounds. They represent combinations between psychical sensations and the feel- ings. The psychical sensations and feelings are the correlates of and riave as their parallel physiological bases, the five senses of the body. Ideas being thus shown to be complex and compound processes, it must follow that language based upon them must have a compound and complex structure. It is therefore useless to look for specific centers in the brain for the elaboration of psychic manifestations, which in their very nature show that they are compounded of many psycho-physical processes. Physiologists are awakening to the fact that speech centers, such as they used to be thought of, do not exist in the brain. There are groups of cells here and there in the cortex that undoubtedly subserve the purpose of bringing together widely divergent processes and so far as these groups of cells subserve the function of symbolizing ideas they may be spoken of as speech or language centers. These so-called speech centers are available for localizing purposes. Their connections with other centers are in a few instances so well known that we can ofttimes localize a lesion very definitely when the intervening association tracts are damaged. Under no circumstances, however, are we justified in attributing the function of speech to any one cortical area or subcortical tract. Only by clearly recognizing the above described nature of language and its relation to all of the cortical, and perhaps subcortical, functions can we explain some of the strange phenomena observed in focal lesions which we had expected from our physiology to produce a defi- nite set of linguistic symptoms. To account for some of these unex- pected manifestations, those who ignore psychology entirely and believe that the evidences of physiology point to special cortical spots as the source of such complicated psycho-physiological phenomena as speak- ing, reading, writing are, have to resort to some very queer and illog- ical reasoning. Thus they talk about individual variations . contradict- ing, as it were, their own position when they found their physiologica] teachings upon such narrow, materialistic bases a- they doj They say one person uses his visual memory more than another in his speech; another his auditory; a third his motor. .Ml of this is true and in 766 THE NON-NEURONIC DISEASES itself reveals that language is a compound, variable process, a func- tional psychosis, and not, as they have just been stating, a physiological manifestation in certain fixed parts of the cortex. In one sentence they show that mind is not an entity, that psychosis is not a thing, but a' function, a process, an interplay between certain lower and more elementary psycho-physiological sensations ; and yet in the next sen- tence they try to fix that mere function, process or interplay in the anatomico-physiological functions of a few nerve cells and fibres grouped in so-called centers. No wonder they say with a half-surprised manner, "the loss of a certain center will cause a hardly noticeable dis- order in one and a severe and persistent disturbance in another." - Elaborate paradigms are constructed based upon the idea that speech is subserved by special cortical centers, and yet immediately after the paradigms it is stated that partial lesions occur very often (a fact not always demonstrable, though too often assumed), and that such cases cannot be grouped in the paradigms. Disturbances of language, then, are psychic symptoms. They are not more definite as localization symptoms than are some of the illu- sions and hallucinations previously referred to. Their definiteness occurs on account of the sphere in which they occur — namely, the sym- bolizing of ideas. In the manufacture of these symbols, animals use movements and the secondary result of a particular form of movement, sound. Behind all language, therefore, is some form of motility. If this motility involves the sound producing apparatus, we have loss of ' articulate speech. This may be purely paralytic. The muscles con- cerned may be directly paralyzed or immobile. This is a disturbance in articular speech. It is called anarthria if it is complete; dysarthria if it is incomplete. The muscles, with their nerve supply, that are involved are those of the lips, tongue, palate, larynx and respiratory apparatus. Sounds may be made if all of the muscles are not paralyzed, but they cannot be articulated into definite forms to subserve the pur- poses of speech. When sounds cannot be produced the condition is known as aphonia. A patient suffering from anarthria need not neces- sarily be aphonic. He can make a noise, but he can't speak, because some of his muscles refuse to work. The muscles by which speech is articulated are innervated from the nuclei of the medulla, hence their paralysis is usually a bulbar symptom. But these nuclei are under the control of the higher centers in the cortex. They may be paralyzed, therefore, from disease in these cortical centers, as, for instance, the facial and hypoglossal near the bottom of the central convolutions. As these cortical areas are repre- sented in the opposite hemisphere and the two sets are intimately con- nected by commissural tracts, cortical paralysis of the muscles of articu- lation is not apt to be permanent. Symmetrical disease in the hemi- spheres, however, may permanently damage the articulation. This is what is known as pseudobulbar paralysis, because it so closely simu- lates disease of the medulla. All degrees of paralysis may occur from disease of the bulb. The speech may be only slightly deranged, or it may be so profoundly affected that nothing but sounds are heard. When the lips are paral- THE NON-NEURONIC DISEASES 767 yzed, p, b and m are sounded like f snd v ; when the tongue is affected, the 1 and t resemble y and th ; implication of the palate turns p and b, the explosive labials, into the continuous labial m, because the posterior nares cannot be shut off. Other slighter forms of dysarthria are the confluent and elisive, when the consonants and syllables are run together; and the staccato or scanning speech, when the syllables are unduly separated as one sep- arates them when he is scanning a line of poetry. These defects are all self-evident and their examination needs no special description. We have just seen that language, spoken as well as other forms of language, is something more than the utterance of noises, etc. It is a symbolic utterance of sound. The sound must be made in a particular way to convey the proper idea. This necessitates the employment of the muscles in a particular, combined manner. The manner must be thought out in the brain before it is put into execution. If the word to be uttered, the symbol, belongs to old and fixed language, the in- dividual's memory will be involved in his thinking as to how he should put the muscles of his vocal apparatus to produce just the sound he wants to. It is obvious that in this process, which after long years of practice has become subconscious in part, a fine, delicate coordinating mechanism is involved. The focus of this mechanism, or rather the foci of these mechanisms, constitute what are called the cortical speech centers. Disease of these centers causes a loss of language. This is known as aphasia. Aphasia, then, does not mean paralysis in the sense that anarthria does. It means merely psychic paralysis for language. It stands for a loss of the memory pictures of speech, the loss of the power of sym- bolizing one's ideas. As symbols are various, but always involving a motor idea, aphasia is of a varied type, but is always related to motility more or less. Complete aphasia would involve all forms of symboliza- tion and as a result would represent a condition in which there is no language whatever. That would probably mean a condition in which there were no ideas, or complete idiocy. Partial aphasia would mean that the power of symbolizing one's thoughts is lost in one way but not in another. Several forms of partial aphasia are now recognized clinically, though there are doubtless innumerable forms that are so fine as to quite escape ordinary detection. Those that are clinically recognized are motor or ataxic aphasia, amnesic aphasia, alexia and agraphia. Pantomimic or gesture aphasia and paraphasia should be included. These are all forms of language defect and represent a disorder in the psycho-sensory-motor mechanism that underlies the whole process of language production. There is, of course, a parallel physiological sensory motor mechanism in the cerebral cortex, certain of whose points we can recognize in the motor speech center of Broca, the sensory speech center of Wernicke and the centers for word-blindness, etc. Of these the first is the most definite, doubtless because the funda- mental purpose of language being the fixation and communication of ideas by symbols and symbolization being fundamentally a motor and emissive process, every form of speech has in it actually, or represented 768 THE NON-NEURONIC DISEASES in it potentially, some form of motility. Even receptive speech and the unuttered language of thinking are essentially emissive for it is a process wherein the mind communicates to itself, as it were, in another form its own ideas. As t«he emissive process can only take place over the motor pathways, we have in every word, every form of language the motor element lurking. We have discovered that the anatomico-physi- ological concomitant of this motor manifestation resides in the group of cells known as Broca's center, and to some extent all of the motor centers. These are in communication with the hearing and visual and touch centers, wherein reside those cellular elements that associate sounds and sights with the muscular movements in such a way as con- stitutes the aggregate network out of which the linguistic function is evolved. The interconnections in this basal sensory motor network is the essential point to note, for it is upon them that what we call language has its foundations. The network may be broken here or there so that there is a partial rupture in the sensory motor complexus, or the group of cells that here and there serve to unite the various parts of the net may be obliterated. In this way we have the partial and the special types of aphasia established. That there are innumerable types of aphasia, indicating countless possible breaks in this wonderful com- plexus of sensory motor inter-connections more than we can now locate, is hinted at in such manifestations as the forgetting of particular names, of particular gestures and of particular forms of print. Language is the product of an associative process; not of mere cell activity per se. It is a psychic phenomenon resulting from inter- connections between sensory and motor elements. The so-called centers of language are merely the points at which some of these fundamental inter-connections are most obviously brought about. Aphasia is the result of a break somewhere in the network of inter-connections. If the break is at the so-called centers, the aphasia may be grossly and wholly motor or sensory in type. If the break is between the centers it may merely sever the motor from the sensory side of the complexus, or it may sever one form of motor from another form of motor element in it, or one sensory from another sensory element in it. As the inter- connections are fine and innumerable, so the ruptures may be fine and innumerable. I will now take up and discuss a few of the gross ruptures which we recognize chiefly. The reader will find some assistance possibly in the section devoted to the consideration of the Cerebral Localizations. Motor Aphasia. — The term ataxic as applied to this form of aphasia should be dropped, because ataxia can be produced by sensory as well as motor disturbances. Motor aphasia is usually produced by a lesion at the base of the third, left frontal convolution, Broca's center, in right-handed people. The corresponding area in the opposite hemi- sphere is the active one in left-handed individuals. Disease of one side is occasionally compensated for by development of the function of the other side, thus causing the aphasia, under certain conditions, to ulti- mately pass away. Disease anywhere in the conducting path from the center also produces motor aphasia. THE NON-NEURONIC DISEASES 769 Motor aphasia is a loss of voluntary speech. There is a distinct want of memory pictures by which the muscular apparatus is put into condition to emit the desired sound. An aphasic patient is not speech- less. He can utter words and often automatically can roll off long- grammatical sentences. Verses that he has memorized can often be correctly recited. Singing, which usually automatically sustains rhe verses, often makes the task exceedingly easy. Even a commonplace automatic conversation can be carried on in mild cases. The moment, however, the conversation becomes voluntary and unusual, it halts and delays. Irrelevant words and sentences are introduced. Wrong words are used. Words having opposite meaning from those intended are uttered. Syllables are misplaced and doubled. Complete motor aphasia is practically always accompanied by right hemiplegia. Bastian saw the exceptional instance in which a left- handed person experienced a lesion of the left hemisphere and had right aphasia with right-sided hemiplegia. Sensory Aphasia, IVord-Dcafness. — Here the patient is in the con- dition of a person listening to an unknown foreign language. He hears the sounds correctly but they mean nothing to him. They are only sounds. There is no actual deafness. This form of aphasia often accompanies the motor. It is easily tested by asking the patient to do or say something. He stands mute. He has heard but he does not comprehend. Voluntary speech, if this amnesic type of aphasia only is present, is ready enough. Reading and writing, except from dictation, are carried on normally. Alexia and agraphia, however, are very often present. The extent and character of the lesion determine this. The seat of the disease is here located in the first, left temporal convolution in right-handed persons. The es- sential trouble is the loss of the memory pictures for sounded words. Some impairment of speech may be caused by this amnesic aphasia from its interference with the processes of thought. Thinking neces- sitates the use of symbols and in higher language-using animals, like man, the vocal symbols are made to serve the processes of ratiocina- tion. When the memory of these vocal symbols is gone it is obvious that the thinking processes are lowered and especially the utterance of those processes in words that must be searched for partly in the affected region. Mechanical or automatic speech is therefore better in amnesic aphasics than spontaneous, voluntary speech; and mere spontaneous, voluntary speech is easier than a conversation, in which the mind is not left so much to its own devices. This inability to awaken word tone pictures in one's self, apart from the mere loss of word-perception, is called amnestic aphasia. The higher psychic cen- ters are disconnected from the ordinary language centers, though audi- tory impulses reach and stimulate- the latter. Agraphia. — This may occur alone hut it more generally accom- panies motor aphasia. There is some doubt as to the nature of this symptom. It is clearly a motor or psycho-motor disturbance. Some have supposed it was due to disease in Broca's center, because as they say, internal motor vocal speech always precedes its outward expres- sion in writing. Motor agraphia, however, does occur apart from ordi- yyO THE NON-NEURONIC DISEASES nary motor aphasia. The trouble, moreover, seems to be more deeply- seated than would be a mere loss of memory pictures for movements in the right arm and hand, for the patients cannot even form the words out of lettered blocks placed before them. It probably represents a much larger disconnection in the linguistic network than is represented in any one center or single tract. No such special center is known to exist. If inner speech precedes written speech, there may be a discon- nection between Broca's center and that for the hand. Most frequently agraphia accompanies verbal sensory aphasia and motor aphasia. As I have shown, language is practically always an emissive sen- sory-motor phenomenon ; hence internal language must somewhat in- volve both sensory and motor processes before it is uttered in writing or any other way. These patients can very often write from printed copy though they cannot write from diction. They do not, of couse, understand or comprehend what they are copying. The visual appara- tus takes in the letters on the page and the hand reproduces them. The patient is like one who is copying an unfamiliar, foreign language. Alexia or Word-blindness. — This may be isolated, though it usu- ally accompanies sensory aphasia. A lesion has often been found in these cases in the angular gyrus in the left lower parietal lobe. Here the tracts course that connect the visual centers in the occipital lobe with the auditory center in the temporal lobe. The reason for the frequent association of alexia and amnesic aphasia is that an internal language must be presupposed before an intelligent comprehension of that which comes in by way of the visual apparatus can be expected. These patients with alexia are like persons reading an unknown, for- eign tongue. They see but they do not comprehend. Of course, writ- ing is impossible to them. This is not the agraphia just described, for spontaneous writing and writing from verbal dictation (in isolated alexia) is easy enough. The alexic patient cannot write because the graphic symbols are absolutely unintelligible to him. In agraphia the disturbance of the comprehension of language precedes and prevents the inditing of symbols ; in alexia the disturbance of the comprehension of language follows the writing instantaneously. The latter is like a man trying to build a brick wall, who, the moment he lays the brick down it ceases to be to him a brick or a wall, but something absolutely different and irrelevant. Curiously, such patients can sometimes read writing by tracing the outlines of the letter with their finger. The sensory pathways of the arm are here made the connecting link be- tween the written language and the intact speech centers. Pure alexia, as I have been describing, is of subcortical origin. Right hemianopsia for obvious anatomical reasons, often accompanies pure alexia. Visual Aphasia. — This is a rare condition in which objects are seen and recognized but cannot be called by name, though they can be named if in any way they can be perceived through the other senses. Let such a patient look at a bell, for instance, and he cannot name it ; let the bell be sounded, however, and he at once states what it is. Lesions in this curious condition have been discovered at the border of the left occipital and temporal lobes ; but they have always THE NON-NEURONIC DISEASES 771 been extensive enough to implicate both tracts from the occipital* visual areas to the sensory speech center in the temporal lobe. Alexia., hemianopsia and sensory aphasia usual!}- accompany this condition. Oppenheim asserts that he finds this trouble not uncommon in otitic cerebral abscess. In tactile aphasia, which is exceedingly rare, objects that are touched cannot be named. This must not be confused with astereog- nosis. Xo discussion of aphasia is complete without a notice of the disorders of pantomime. These occur among aphasics and involve the gesture language. Amimia and paramimia have a similar import to aphasia and paraphasia. A pantomimic action is the product of a psy- chic process. It is therefore something more than a mere emphatic gesture. These patients nod "yes" when they mean "no." The causes of aphasia may be functional or organic. As I have shown elsewhere a pyschosis is the only nervous disease that can be strictly functional unless we include many forms of reflex disturb- ances under the name functional. It is possible to have a disorder in the interplay and interconnection between perfectly normal psycho- physical elements. Among the organic causes of aphasia are to be in- cluded the toxic causes that upset the basic neurones. It is only on the possibility of a psychosis being a true functional manifestation that we can explain the fact that aphasia may follow a severe fright and occur in hysteric states. In migraine it is probably associated with vasomotor disorder. It. of course, can be produced in a reflex way. It has thus been seen in children as the result of worms and minor surgical operations. Exhausted states of the blood and the nervous system may give rise to it. Among the toxic antecedents are to be recorded the acute infectious fevers, such as typhoid, pneumonia, scarlatina, nephritic con- ditions with uraemia, carcinoma, gout and in santonin poisoning. By far the most common organic cause is focal encephalomalacia because it is so frequently a cortical trouble. Softening is more fre- quently a cause than is hemorrhage. The middle cerebral artery, espe- cially the left, that is so frequently the seat of these vascular troubles, is the artery that supplies the most important language-production re- gion of the brain. As a rule lesions in the deeper parts, the central ganglia and internal capsule, do not cause aphasia. They maw how- ever, if they are large enough to compress the cortical areas. In uni- lateral lesions that do not compress these parts, the speech emissive- impulses find their way to the opposite hemisphere by way of the cor- pus callosum, and so on outward. Cerebral abscess may cause amnesic aphasia. Motor aphasia has followed abscesses also of traumatic and metastatic origin. Pressure aphasia may accompany suitably located intracranial neo- plasms. In children tuberculosis and tuberculous meningitis are some- times responsible. Gonorrheal embolism has been blamed for it by Tit res and limns. Direct traumatism is a factor in the etiology. A congenital aphasia has been observed. It is probably due to a prenatal or natal hemorrhage. 772 THE NON-NEURONIC DISEASES Toxic and functional aphasia usually disappear soon and com- pletely. As an indirect focal symptom it may disappear if the original cause changes so as to relieve the pressure (hemorrhage) or obstruc- tion (embolism). Usually it requires several weeks or months, though a week or so may see it vanish. In children, there is always the pos- sibility of the right hemisphere taking up the function when the left is damaged beyond recovery. The nature of the cause and its amena- bility to treatment obviously determine the prognosis. The treatment, further spoken of under the head of cerebral hem- orrhage and softening, involves removal of the cause, combating the underlying disease, and re-educating the patient. A correct diagnosis as to the nature of the loss of speech and a little ingenuity in setting the linguistic tasks for the patient to practice daily is all that need to be suggested here. Motor Symptoms. — Irritative Symptoms. Convulsions. — These may be provoked by direct irritation, as in meningitis or tumor ; or by the alteration of the gray matter in regard to its nutrition. Irritations of the cortical motor zone, of course, affect the opposite side of the body, as all the cortico-spinal motor tracts decussate. As a result tonic and clonic spasms occur which may be limited to certain movements or involve all the movements. When the irritation is bilateral, the convulsion is general. In this way we have monospasms, hemispasms and universal convulsions. The movements may begin with twitchings or they may start in a tonic spasm and pass into the clonic state. Oft- times a twitching will begin in a certain small area and then spread gradually until a large part or even the whole body is convulsed. There is no fixed rule as to the succession of parts involved. Generally the progress of events can be seen in the spasms to be proceeding up or down the central convolutions. Thus if the movement starts in the leg, it attacks next the arm and finally the face ; or if the face is the earliest seat of the manifestation, it passes next to the arm and finally to the leg. A convulsion that starts in the arm is apt to involve the face before it does the leg. In monospasms, consciousness is not disturbed. When, however, the convulsion becomes general, consciousness is usually lost. These localized monospasms are spoken of as partial or cortical epilepsy. As manifestations of Jacksoniau epilepsy, I have discussed them elsewhere. It is a question whether all organic, gross, irritative lesions that provoke such monospasms should be regarded as epileptic. They are epileptiform. They occur in organic and functional troubles. Toxic conditions, such as alcoholism, uraemia, saturninism, seem to be capable of producing them, though it is not improbable that these are merely organic also, being due to hemorrhagic and embolic alterations of a minute character. A temporary paresis usually follows these manifestations. If it remains it means that the lesion is more than an irritative one. It has begun to exercise a destructive influence upon the affected cortical area. Sensation is often implicated with these irritative motor phenom- ena. There may be various paresthesia* in the limb that is subjected to the spasm. It may be only an accompanying symptom or it may be THE NON-NEURONIC DISEASES 7/3 the sole symptom in the form of an equivalent. It is now believed that the sensory elements are most intimately intermingled with the motor, or at least are intimately interconnected, so that it may well be that the true irritative epilepsies in this case are really sensory phe- nomena with secondary motor manifestations, even as occurs in gen- eral epilepsy. Cortical epilepsy occurs from irritative subcortical lesions but disappears absolutely when the cortex is destroyed. Choreic and athetoid manifestations are irritative symptoms but they are not believed to be due to cortical irritation. As they usually occur in hemiplegic conditions they are thought to depend upon the irritation of the thalamus. They occur more frequently in disease of the thalamus and are best discussed after hemiplegia. A tetanoid spasm has been observed in disease of the cerebellum, or other lesion that produces increased pressure beneath the tentorium. The head is bent backward with opisthotonos, the jaws are clenched and the whole appearance is that of traumatic tetanus. Forced move- ments occur. It is not known whether the symptom is indicative of cerebellar or pontile disease. The condition lasts sometimes for hours, though the tonic contraction is broken by clonic jerkings. General convulsions, due to disease of the brain, such as tumor, embolic hemiplegia, and meningitis, may so closely simulate hysteria as to deserve the adjective hysteroid. Paralytic Symptoms. Monoplegia. Hemiplegia. — The first great characteristic of cerebral paralysis is that it is distinctly of the voli- tional power over the musculature. In diseases of the lower motor segment, the paralysis, of course, will have all the appearance of being in the volitional sphere, because this segment lies between the upper seg- ment and the musculature, and therefore cuts off impulses passing from one to the other. In the muscles, however, that are partly vol- untary and partly involuntary, as for instance, the muscles^of respira- tion and deglutition, it is rendered obvious enough when the volun- tary movements are impossible from upper segment disease, while the involuntary movements continue intact. A second great characteristic of brain palsy is that, for evident anatomical reasons, the muscles are not affected with a true atrophy. Their nutrition is dependent primarily upon the ganglionic cells in the anterior horns of the cord. For the same reason there is no reaction of degeneration observed in cerebral palsies. As the upper or cerebral motor segment exercises an inhibitory control over the lower, when it is diseased, the paralysis is accompanied by an increase of all the relieves. This i> a third important charac- teristic of cerebral paralysis. A fourth is that the paralysis affects the movements of the body rather than the mere individual muscles. The areas of the cerebral cortex subserve facial movements, arm movements, leg movements, etc. A cerebral paralysis never picks out special muscles, hut only those muscles and their antagonists thai normally arc employed in the production of higher, purposive psycho-motor activities. This type of paralysis is known as monoplegia. Cortical monoplegias correspond, as pointed out in the section on 774 THE NON-NEURONIC DISEASES Cerebral Localization, with the particular centers involved in the lesion. But these centers have their cell processes bunched together, like the stem of a bouquet, in the internal capsule and so on down to the ter- mination of the pyramidal tracts in the medulla and cord. Obviously the farther down below the cortex the given lesion of the same size is located, the more extensive will be the paralysis on the opposite side of the body. If the lesion is in the internal capsule, for instance, where the bundle is probably as compact as anywhere, the entire oppo- site half of the body will be paralyzed. This is known as hemiplegia. If both hemispheres or their tracts are diseased, the hemiplegia will be bilateral. This condition is termed diplegia. Between a hemiplegia and a monoplegia, it is clear that we can have any extent of palsy, according to the particular location of the lesion. Hemiplegia then is the great, characteristic type of cerebral paraly- sis. It involves the entire half of the body. For this reason it is some- times spoken of as the facio-brachio-crural type of paralysis. The in- ability is most pronounced in those muscles and parts of the body that subserve the higher, complicated voluntary movements. There is a direct ratio between the degree of intelligence involved in the normal movements and the degree of palsy which they have now undergone. For this reason the hand is more affected than the foot, the arm more than the leg, the arm and leg more than the face, the tongue and lower part of the face more than the upper part of the face, and the upper part of the face more than the great truncal muscles. With the evolution of human intelligence and brain power there is a constant tendency to separate the actions of the two sides of the body. This is shown in the remarkable feats accomplished, as the re- sult of practice, by pianists which ordinary mortals cannot do. It has been .intimated that in time we may move each eye without regard to the other. At all events, it is a fact that the great tendency in cere- bral evolution is towards the separation of the functions of the hemi- spheres. In those movements in which this separation has reached the highest degree at the present time, disease of one or other hemisphere will be most marked. Where, however, the movements are bilateral still, as they are in the movements of the eyeballs and the apparatuses of sound, deglutition, and respiration, the paralysis is not so evident because the unaffected hemisphere takes up in part the duties of the diseased hemisphere. In other words, the bilateral representation of these latter movements in the two sides of the brain is more positive than it is of the former movements. In bilateral lesions, however, the laryngeal, deglutitory and masticator)' are affected and we have what is termed pseudo-bidbar paralysis. Of the trunk muscles, the trape- zius is affected as a rule, though the sterno-mastoid aets normally. In a deep respiration, the paralyzed side expands somewhat less than the other side. The cases in which hemiplegia has occurred on the same side as the lesion are open to grave doubt. Most of them were diagnosed be- fore our knowledge of the central nervous system was as exact as it is to-day. Pressure by contrecoup or lesions in the opposite hemisphere, or in the pons and medulla, were not taken into account. However, a THE NON-NEURONIC DISEASES FIGURE 187. tE G 775 — Diagram of motor path from right brain. The upper segment is black, the lower The nuclei of the motor cerebral nerves are shown on the left side : on the right side the cerebral nerves of that side are indicated. A lesion at 1 would cause upper segment paralysis in the arm of the opposite side — cerebral monoplegia ; at 2, upper segment paralysis of the whole opposite side of the body — hemiplegia; at 3, upper segment paralysis of the opposite face, arm, and leg. and lower segment paralysis of the eye muscles on the same side — crossed paralvsis; at 4, upper segment paraly- sis of opposite arm and leg, and lower segment paralysis of the face and the external rectus on the same side — crossed paralysis: at 5, upper segment paralysis of all mus- cles below lesion, and lower segment paralysis of muscles represented at level of lesion — spinal paraplegia; at 6, lower segment paralysis of muscles localized at seat of lesion — anterior poliomyelitis. (Van Gehuchten, modified.) Jj6 THE NON-NEURONIC DISEASES congenital absence of the pyramidal decussation, a developmental anomaly, is assumed in explanation of these homolateral hemiplegias. The gait of a hemiplegic is characteristic. The leg remains weak and is dragged by the patient. The extensors of the foot and toes remaining paralyzed, the equino-varus attitude is assumed and the toes not being lifted off the floor, the foot is scraped outward in a half circle each time it is moved forward. The flexors of the knee are apt to remain paralyzed also. In the arm the palsy remains in the hand and fingers, the movements of the shoulder and elbow being par- tially regained. Though the paralysis is voluntary, the muscles act under certain conditions. Under strong emotion, gesticulation and in associated movements this is often the case. Automatic and reflex functions they still retain. The psychic or mental paralysis of Krafft-Ebing and Bruns is due to the dissociation between the cortical sensory and motor elements. Upon request the patient moves his limbs but not of his own purpose or desire. Contractures, early and late, usually follow the cerebral palsies. The former develop in the first few hours or days after an apoplectic stroke; whereas the latter appear many weeks later, never before two weeks from the onset of the trouble. The former is due to an irrita- tion of the pyramidal tracts ; the latter are not clue to, though they pro- ceed in order with, the corresponding secondary degeneration in the pyramidal tract. Adduction, flexion in the arm, pronation in the hand, and flexion in the fingers occur. The leg is extended and the foot, as I have stated above, is in the attitude of equino-varus. The increase of the contracture by sensory stimulation, especially cold, electricity, etc., its disappearance in sleep, and other factors indicate that it is un- doubtedly due to the slight overaction of the lower motor segment when the inhibitory power of the upper segment is weakened. This explains, of course, the association of an increased action of the deep reflexes with these contractures. In some cases there is a slight wasting of the muscles from non-use but no true atrophy. What atrophy there is, is probably due to the absence of the normal nervous impulses, which in themselves are somewhat stimulating to the nutrition of the muscular tissues. In every hemiplegic there is some weakness in the unparalyzed side. Associated movements not infrequently occur in the paralyzed limbs. If the patient, for instance, grasps vigorously with his well hand or suddenly raises his unaffected arm, the same movement will be partially performed in the paralyzed hand and arm. The changes of a trophic nature that are occasionally observed in and about the joints of hemiplegics are supposed to be due to neuritic or consecutive myelitic changes and not directly to the defeneration in the pyramidal tract. Alterations of local temperature, sweating, (edema, redness, etc., in the paralyzed limbs are sometimes noted and may be due to vaso- motor disturbances in the cortex. Hcmichorea. — As a post-henuplegic phenomenon this is not un- THE XOX -NEURONIC DISEASES JJJ common ; as a pre-hemiplegic one it is rare. According to Charcot it is due to a slow, progressive hemorrhage in the optic thalamus, irritat- ing the pyramidal fibres where they course through the internal cap- sule. The movements are jerky, irregular and incoordinated just as they are in any, chorea. The whole side or extremity is usually in- volved. Athetosis. — In the fingers and toes occur, after hemiplegia, espe- cially infantile hemiplegia, curious, wavy, involuntary movements, that are the result of irritation of the pyramidal tract. The fingers undergo continuous polyp-like slow extension and flexion, adduction and ab- duction. They occur in sleep ; sometimes only upon voluntary move- ment. The fingers are moved separately, causing a most grotesque and bizarre appearance. Opposite movements are made in adjoining fingers simultaneously sometimes. Other movements of irritative origin that are neither choreic nor athetoid are also seen in conditions of hemiplegia. Among these may be cited the associated movements referred to above, unilateral tremor and movements similar to those of paralysis agitans and disseminated sclerosis. Alternate Hemiplegia. — When the pyramidal tract is damaged in the lower part of the pons, just after the central facial tract has decus- sated, the paralysis will be in the face on the same side as the lesion but in the limbs on the opposite side. In an alternate hemiplegia, the hypoglossus may be paralyzed on one side and the extremities on the other. The tongue will then protrude toward the sound side. Conjugate Deviation of the Head and Eyes. — This is often seen as a symptom of brain disease. In cerebral lesions the deviation is toward the unparalyzed side, or towards the side of the lesion. The muscles that draw the head and eyes toward the hemiplegic side arc. of course, weakened as the other muscles on that side are. Spastic conditions naturally cause the deviation to be in the other direction or toward the spastic side. The reason is obvious. Lesions of the pons often deviate the eyes toward the side opposite the lesion, the location of the latter being in the common center for the abducens of the same side, and the internal rectus of the opposite side. Sensory Symptoms. — All qualities of sensation may be impaired in intra-cranial disease, especially the senses of touch and position. Hemianesthesia j rarely complete, occurs with lesions in the cortical motor areas. When the sense of position is disturbed the lesion is thought to be in the parietal lobe, though there arc strong grounds for believing that this disorder originates from disease limited to the cen- tral convolutions. In monoplegias the motor and sensory phenomena are confined to the same parts. Sometimes the cortical motor zone and other parts of the cortex can be destroyed without 1<>^ of sensation. Wernicke and ( )ppenheim claim they have observed a tactile paraly- sis that Is a mental anaesthesia. With the sensibility intact in tin- hand. objects could not be recognized by touch. (See my views upon this subject under the head of psychic .symptoms, localization centers and psychic blindness. ) Hemianesthesia occurs on the side Opposite that of the cerebral 778 THE NON-NEURON1C DISEASES FIGURE 188. Position of the fingers in athetosis. (After Strumpell. THE XOX-XEUROXIC DISEASES 779 lesion. It involves the entire skin and mucous surfaces of one-half of the body when it is complete. Grasset claims that the cornea is in- tact but that is hardly probable. The immediate cause of this symp- tom is, of course, damage to the sensory conducting tracts. Cortical hemianesthesia is rare. The course of the sensory tracts have been traced in the description of the anatomy of the brain. The anaesthesia is not always the same in all parts of the affected side of the body. It will sometimes be more marked in one place than in another. All forms of sensation may not be equally involved. Hemianaesthesia is often accompanied by impairment of the spe- cial senses, the two sets of pathways being contiguous. Hence with it there may be hemianopsia or crossed amblyopia. Crossed anccsthesia occurs in upper pontile lesions, the fifth nerve being affected on one side and the path from the limbs on the other. Pontile disease also .sometimes causes incomplete bilateral anaesthesia. Sensory irritation or pain in the limbs often occurs when the sen- sory tracts are only partially destroyed. Sometimes under these cir- cumstances numbness, tingling and other paraesthesiae are observed. These, cannot be determined as tract or cortical symptoms. Ataxia sometimes occurs m the opposite extremities from disease of the sensory cortex and the conducting tracts. In hemiplegia and hemianaesthesia there are apt to be vasomotor and trophic symptoms. In the affected limbs the cutaneous tempera- ture is lowered, the parts are reddened, or cyanotic and ©edematous. In severe cases a tendency to decubitus is present. Skin eruptions, falling of the hair, and even gangrene have been observed. Slight arthropathy may occur. Olfactory Symptoms. — In examining the sense of smell we are wholly dependent upon the subjective testimony of the patient. When the olfactory nerves are affected by way of the anterior nares we call the sensation an odor ; when by way of the posterior nares, it is termed a flavor. As the nares are innervated by the trigeminal as well as by the olfactory nerves, the examination must be made with substances that affect only the latter nerve, such as musk, asafoetida, oil of cloves, oil of peppermint, oil of lavender, oil of turpentine. The substance is simply held under one nostril, while the dther nostril is closed. Am- monia and acetic acid irritate the filaments of the fifth nerve in the mucous membrane. The sense in the two nostrils should be com- pared and the intelligence of the patient in regard to the subject o: odors must be given due consideration. There is an olfactory reflex. Disgusting odors cause the head to be drawn away and the mouth to be retracted. For a description of the ol factor) apparatus, the section on the anatomy of the brain should be consulted. Anosmia or the loss of the sense of smell may. of course, occur from local disease of the mucous membrane. As a symptom of brain trouble it may be due to various traumata, disease- of the hones, tumors in the anterior fossa of the skull, local meningitis, syphilitic or other- wise. Olfactory neuritis, like optic neuritis, may accompany brain tumor. Anosmia has been seen in tabes and as the result of primary atrophy in old age. It may he bilateral or unilateral. As a eongeni- w 780 THE NON-NEURONIC DISEASES tal condition it is observed in idiots. It resulted once from congeni- tal absence of the olfactory nerves. Hereditary anosmia has been re- ported. Hyperosmia, or olfactory hyperesthesia, occurs in hysteria and insanity. It is largely a psychosis in the olfactory sphere. It is not. of much clinical importance. Olfactory parcesthesice and hallucinations occur. They have been, seen as aurae in focal epilepsy when the lesion was in the temporo- sphenoidal lobe. They are not common at all. Optic Symptoms. — The optic apparatus is a part of the brain,, as I have explained in the section upon anatomy. It extends from the cornea in front to the occipital cortex behind. The neural part includes that from the retina to the cortical area in the cuneus. The symptoms of disease in this long tract are of the greatest neurological importance. They group themselves naturally under the two heads of visual disturbances and ophthalmoscopic alterations. Both of these sets of symptoms may be examined objectively but the latter are more accurate than the former because they are absolutely unrelated to the patient's consciousness. Among the former are various alterations- in the field of vision, chief of which are concentric contraction of the field, scotomata and hemianopsia. Psychic blindness will be included in the discussion of these visual symptoms. Chief among the fundus- changes, seen through the ophthalmoscope, are the so-called "choked' disc" or optic neuritis, and optic atrophy. Visual Disturbances. — In examining a patient's vision for neu- rological purposes, it is incumbent to search for refractive and other errors, though this belongs especially to the province of the ophthal- mologist. In certain nervous conditions such as the unstable neu- roses, ocular defects may, as exciting causes, set up headache and other symptomatic troubles. These having been eliminated, the visual disturbances are to be attributed to an intracranial disorder of some sort. We cannot always distinguish by these visual signs optic nerve from cerebral disease but in regard to some of them we can draw air but conclusive inferences. Pupillary reflex disorders may be due to optic nerve disease and hence the pupils should always be examined also. The discussion of the pupillary phenomena naturally falls under the head of disorders of the third nerve. Concentric Contraction of the Visual Field. — The determination of the outer limits of the field of vision for each eye is accurately made with a perimeter. This is not the place to give a minute description of this apparatus and the manner of using it. For that the reader should consult works upon ophthalmology. Suffice it to say. however, that it consists of a semicircular arc or band which revolves upon a center, so that it can be placed in the direction of any meridian. This arc is divided into degrees from zero at the center to ninety at cither cud. By means of a head rest the patient is enabled, with one eye covered, to place the other eye in such a position as to fix its gaze upon a small object at the center of the arc. While the gaze is kept thus fixed, a small white object is slid from the outer end of the arc towards the THE NON-NEURONIC DISEASES 781 center. The moment the patient declares that he sees it, the degree on the scale is noted and marks the outer limit of the field of vision in that meridian. The process is repeated for all meridians and the de- grees jotted down on a chart upon which the normal or average visual field is indicated. By connecting these several points on the chart with a continuous line, the visual held of the patient is seen at a glance -and can be compared with the normal field. When the white object, moving on the arc, appears blurred or •disappears entirely, there is a defect in the field which is termed a FIGURE 180. Normal field of vision. B, blind spot of Mariotte. scotoma. The head of the optic nerve, known as the blind spot of Mariotte, and situated about 10 degrees to the outer side of the fixa- tion point, furnishes a physiological scotoma. Instead of white, little objects of different color may be em- ployed and thus the color fields be taken and compared. The perimeter shows that normally the field for green is the smallest. Then follow in order, from within outwards, red, yellow and blue. It should also be noted that the field of vision is more extensive -externally than it is internally. The outer part of the retina is less sensitive than the inner, and the bridge of the nose cuts it off some- what. As considerable variations in the field are observed normally, in every case of unilateral alteration where accurate results are desired, the normal eye should also be examined perimetrically at the same time. In the absence of a perimeter, a rough examination of the fields may be made in the following manner. Place the patient with his back to the window. Take a seat directly in front of him at a distance of about three feet. Cover the eye of tin- patient that is not to be ex- amined with a towel. Ask him to lix his gaze steadily upon the oppos- 782 THE NON-NEURONIC DISEASES ing eye of the examiner. The latter then closes his other eve and at the same time brings his ringer along the different horizontal and ver- tical lines into the field of vision, holding it about half way between himself and the patient. For the color fields a small piece of green, red or blue paper on the end of a pen-holder may be substituted for the finger. As the finger or bit of colored paper is gradually moved from above, from below, or from the side towards the mutual visual axis, the patient is told to say when he first sees it. If the patient and examiner see it at the same moment at all points in the edge of the field, the latter is normal. If the examiner sees it in this line or that before the patient does, there is a defect at that point in the pa- tient's visual field. A concentric contraction of the fields of vision may take place in conditions of functional weakness, like hysteria and neurasthenia, from simple repetition of the test or from the attention being directed to them for a short time. This is even more marked when there is a patholog- ical contraction present. This is not a retinal but a central phenome- non, for if the contraction is thus produced in one part of the field of one eye, the corresponding part of the field of the other eye will be seen to be similarly affected. When the white fields are contracted, the color fields are as a rule correspondingly reduced, so that it may be said that a generally con- tracted field is a normal field in miniature merely. Sometimes there is no contraction in the white field while the color fields are markedly reduced. The color perception is generally more easily blurred or destroyed than is the white. As a rule it is only necessary to test the green and red fields as they are the smallest and undergo the most diminution in morbid states. It must not be forgotten that concentric contraction may occur slightly in myopia, myosis, paresis of accommodation and with opaque corneal spots. These being eliminated as possible causes, we find that this phenomenon is to be re- garded as a sign of disease in the retina, optic nerve or brain. Loss of sight anywhere within a visual field is spoken of as a scotoma. Scotomata may be variously situated and of all shapes and sizes. They may be central or peripheral. Concentric ring scotomata occur in hysteria. They are organic or functional in origin and may therefore be due to variously located lesions in the optic apparatus. They may invade the color fields as well as the white. In toxic ambly- opia both eyes are affected and the patient complains of misty vision, which is due to a central color ( relative) scotoma, especially for red and green. The fixed point is a favorite site for scotomata. The peri- meter aids one in detecting scotomata, as I have already pointed out. Definite geometric defects in the visual fields are usually classed under the head of hemianopsia. This is generally a bilateral blurring of the half-fields, though it may be unilateral. It is easily enough ex- amined. Often the patient is all too aware of it himself. All the ex- aminer has to do is to introduce from different sides some small white or colored object into the visual field of each eye. lie may use the perimeter if he wishes to accurately outline the intact part of the field. A most remarkable defect that has been seen several times is that of THE NON-NEURONIC DISEASES 783 hemianopsia for colors without any change in the white field. Usually of course the color fields are affected in correspondence with the white. This will be referred to again. In hemianopsia the dividing line between the dark and light part of the field generally passes through the fixation point in the center. It may be horizontal or vertical. In this way we have horizontal and vertical hemianopsia. The former is almost always due to retinal lesions, such as embolism, injuries, etc. In vertical hemianopsia the central or dividing line generally passes a little to one side or around the fixation point, leaving the central vision very acute. This vertical hemianopsia is spoken of as temporal when the temporal halves of the visual fields are dark, indicating anaesthesia in the nasal halves of the retinae ; as nasal when the inner or nasal halves of the fields are ob- scured, denoting anaesthesia in the outer or temporal halves of the retinae; as lateral or homonymous in which physiologically similar halves of the field are darkened, as for example the temporal half- field of the left eye and the nasal half-field of the right. An incom- plete hemianopsia may be sector-like and involve only a quadrant, or even less, of one or both fields. Of all forms of hemianopsia the vertical are the most frequent. Xasal hemianopsia is exceedingly rare and is of small practical inter- est. There is much doubt overhanging the inferior and superior hemi- anopsias. In addition to the above conditions, the vision should always be tested in regard to its acuity, for which purpose test types are em- ployed, and for the perception of color, for which variously hued wools are employed according to the method devised by Holmgren. For details in regard to these tests works upon ophthalmology should be consulted. Every neurologist should be familiar with the use of the ophthalmoscope. The ophthalmoscopic alterations are among the most important symptoms that we have of intracranial disease. The head of the optic nerve is exposed to direct observation at the fundus of the eye and as this so-called nerve is really a part of the brain, in observ- ing its changes we are observing the brain itself, as it were. I will now discuss the localization phenomena of the optic ap- paratus, taking up the parts of the latter in an orderly way from the optic disc backwards, and note, as far as our present knowledge per- mits, the relationship between the special symptomatology and the site of the lesion. What may be called associated changes in the eye, discoverable with the ophthalmoscope, may at times be most helpful in diagnosing a brain disease. These changes are not due to the brain trouble but are merely collateral manifestations of a much more general trouble, of which the brain is the seat of other manifestations. For instance, syphilitic choroiditis or tubercles of the choroid may confirm the di nosis of brain syphilis or tubercular meningitis. Albuminuric retinitis is associated with an arterial degeneration that not infrequently eventuates in a cerebral hemorrhage or thrombotic encephalomalacia. Miliary aneurisms and vascular lesions of tin brain may legitimately be surmised when the same conditions are observed in the retina. 7«4 THE NON-NEURONIC DISEASES Hemorrhages in the retina and in the pia mater have been seen to- gether in severe anaemia, purura, leucocythaemia, albuminuria, gout, ulcerative endocarditis and septicaemia. Degeneration of the brain sometimes is accompanied by simple atrophy of the disc. Neuro-retinitis and optic atrophy are the chief ophthalmoscopic changes observable in disease of the brain, because they are consecu- tive to and dependent upon the latter. Optic neuritis, papillitis, neuro-retinitis, choked disc are all differ- ent names for the same pathological process so far as the optic disc is concerned. A difference of degree may be represented by the terms FIGURE 190. Normal Fundus Oculi. papillitis and choked disc, but the underlying process of both is es- sentially the same. As everyone knows from his anatomy the optic nerve enters the back of the eyeball through an opening in the sclerotic coat. Its fibres bend at right angles like the rays of the sun and spread out on the retina. That part of the nerve which we behold in the fundus as the blind spot is the papilla. With the ophthalmoscope we can see through the translucent, radiating optic nerve fibres the circular edge oi the disc. The surface of the disc is not uniform. It has usually a "physio- logical cup" in its center which is rather pale from the scarcity or absence of bloodvessels in it. The periphery of the disc is more 1 cular and hence more rosy in tint. Simple congestion of the dis< rare and of little importance. Much more significant is the swelling of the papilla and diminished translucencv which it exhibits when there is an actual pathological change. With such a change, the edge of the THE N0N- NEURONIC DISEASES 7*5 disc becomes blurred and finally entirely obscured. The dis- tinctness of the outline of the disc, as affected by a morbid change, undergoes greater modification under the direct than under the indirect method of examination with the ophthalmoscope, Gradually the change involves the entire circumference of the disc ; the swelling obliterates the central cup with a mere trace of a depression left ; and the vessels, as they course down the sides of the enlarged papilla, diminish or disappear from view because the plane of their reflection has ceased to be at right angles FIGURE 191. Optic neuritis (choked disc) in cerebral tumor. (After Gowers.) to the line of the examiner's vision. The entire papilla assumes a red or grayish red tint, increases to two or three times its normal diameter and exhibits in its surface spots which are the accumulations <»i" tin- products of degeneration. Early in the process the vessels are little altered but soon the veins become swollen, the arteries narrow and the blood extra vasated. The rapidity of the process varies in different cases. In a feu- days the swelling may increase and measure six or more diopters from its blurred base to the apex. We speak of it as choked disc when '1 measures as much as two diopters. The change may reach a very high degree of intensity in a couple of weeks, or it max si ill he chronic 786 T 1 1 E X ON- N E U RO X I C DI SE A SES and moderate after several months. Quick developments of the disease usually mean high intensity of it. When the papillitis subsides the arteries become more contracted, the veins diminish in size, and the swelling after lingering awhile slowly settles back revealing once more the edge of the normal disc. Unless the neuritis has been very moderate, some permanent alterations re- main, consisting of new tissue, with a white and atrophied appearance and associated damage to the neighboring edge of the choroid. This is sometimes called consecutive, or neuritic, atrophy. The retina in severe cases may have its nutrition affected, especially near the macula, and this leads to an appearance in it much like that of albuminuric retinitis. Under the microscope, the papilla presents in its diseased state all of the usual changes observed in neuritic inflammation. The anatomical cause of choked disc, as it is seen for instance FIGURE 192. Section through a "choked' Randall.) disc in brain tumor. (After de Schweinitz and in brain tumor, is obscure. It may be due to one or more conditions. It is without doubt in the beginning a pure oedema of the nerve head as a part of the inflammatory process going on inside of the cranium. Whether this oedema is a mere mechanical product, due to compres- sion or the carrying of the increased subdural and arachnoid fluids along the channels of the nerve to the papilla, or to the irritating toxic qualities of that fluid, or to both causes combined, we do not know positively. The probability is that the neuritis is due to the descent of the irritative influence which, when it reaches the papilla, sets up an intense inflammation. This is aided by the retention of the inflam- matory fluids, carrying irritating material, on account of the distention of the sheath. A chronic intracranial disease may cause an acute optic neuritis but a chronic neuritis never follows an acute brain lesion. There is a parallelism between the chronicity of the neuritis and that of the cerebral disease. Subsidence of the brain trouble is usually ac- companied by lessening of the neuritic trouble. Thus we can follow, by direct observation to a certain extent, the progress of the intra- cranial disease. PLATE Til. Optic neuro-papillitis in a case of known cerebral tumor. Drawn from life. ' After Beard. THE XOX-XEUROXIC DISEASES 787 The vision is often but little affected in the presence of choked disc. When the process is advanced, however, the visual fields are con- tracted and there is a diminution of the central acuity. Complete amaurosis sooner or later occurs. Sometimes the blindness appears abruptly, remains awhile and disappears with equal suddenness. This has been explained on the basis of a periodic increase in the intra- cranial pressure. Among the causes of choked disc are to be noted especially brain tumor (occuring in 70 per cent, of all cases of tumor), internal hydro- cephalus, cerebral abscess and sinus thrombosis. Optic neuritis also occurs occasionally in cerebral hemorrhage, anaemia from loss of blood, diabetes, albuminuria, acute infectious fevers, saturnine encephalo- pathy. More rarely it has been seen in acute non-purulent encephalitis. It is not uncommon in brain syphilis and basal meningitis. Hemorrha- gic pachymeningitis, ulcerative, endocarditis, and certain cranial anomalies such as oxycephalic have been accompanied by it. There is said to be a rheumatic optic neuritis. Optic atrophy usually follows continued optic neuritis. There are two forms of atrophy recognized clinically. Primary optic atrophy, as is seen in dementia paralytica and in disseminated sclerosis, tabes and perhaps rarely in some cases of syphilis, is an associated condition and is not a direct proof of intracranial disease. It is distinguished by marked pallor, the border of the papilla being more sharply de- fined than it is normally. Great variations are possible, however, so that a diagnosis from ophthalmoscopic appearances should be made cautiously and only after repeated examinations. Secondary, or consecutive optic neuritis, follows brain disease such as tumors, wounds, bone diseases, etc. Unlike primary atrophy, it is more frequently unilateral. The visual disturbance then precedes the eye-ground changes. The vision of course is lost in optic atrophy. In disseminated sclerosis it is rarely a complete loss, however, or lasting. Amblyopia accompanies partial atrophy, though there may be amblyopia without atrophy. Such are some of the toxic amblyopias. These are seen as a result of retrobulbar neuritis occurring occasionally in polyneuritis and after acute infectious diseases. Chronic alcoholic and nicotine poisoning are the principal causes of toxic amblyopia. In some cases other causes have been reported, as for instance salicylic preparations, chronic lead poisoning, methyl alcohol ("Columbia spirits," Jamaica ginger, bay rum) quinine, carbolic acid, carbon disulphide, etc. In these cases the visual disorder is bilateral, foggy or misty. There is a relatively central scotoma for red and green which extends out- ward from the fixation point and includes the blind spot. Central visual acuity is diminished. This is all due to an interstitial optic neuritis of the papillomacular fasciculus in the optic trunk. Then nothing to be seen as a rule with the ophthalmoscope, though there- may be a slight pallor of the temporal half of the papilla. A non- toxic amblyopia is seen but its cause is unknown. There is als< hereditarv form with an absolute central scotoma. Though it is not 788 THE NON-NEURONIC DISEASES seen in these cases, it is to be remembered that in chronic lead poison- ing a progressive optic atrophy and ocular paresis do occur. Choked disc, optic neuritis, optic atrophy and toxic amblyopia are not very definite as localizing symptoms. In fact they belong to the general brain symptoms, though they are more definite than such general symptoms as headache, vomiting and vertigo previously dis- cussed. We have now to discuss the visual manifestations by which lesions can more or less be definitely localized in the optic tract. The reader is again urged to refer to the chapter upon the anatomy of the brain where the optic tracts and their decussation are described and diagram- matically illustrated. Behind the optic nerve, of whose diseases the etiojogy and symp- tomatology we have just been studying and found to be not very FIGURE 193. Field of Vision in Chronic Lead Poisomnj (Nimier.) definite as localization signs, we come to the optic chiasm, the optic tracts, the primary optic centers in the inter- and mid-brains, the optic radiations of Gratiolet and the occipital cortex. The symptoms of disease in any of these parts of the optic apparatus are almost en- tirely visual. In other words there need not be with them any oph- thalmoscopic alterations unless the optic nerve trunk is at the same time implicated. A lesion of the chiasm, if of long standing, may. however, set up a descending atrophy and thus present a change in the appearance of the fundus oculi. The chiasm is affected generally by tumors of the hypophysis cerebri. Syphilitic and tuberculous growths are not uncommon and may be inside or outside of the chiasm. Pressure from a neighboring pachymeningitis may affect the commissure. Other sources of dam- age are internal hydrocephalus, distention of the infundibulum of the third ventricle and vascular lesions such as interstitial hemorrhage THE NON-NEURONIC DISEASES 789 within its mass. It has been diseased in tabetic atrophy and has been the seat of an interstitial inflammation. If the reader will carefully note the course of the optic fibres through the chiasm, he will readily understand a bitemporal hemi- anopsia, such as is illustrated in the article on acromegaly in another part of the volume. The center of the chiasm being most involved, the mesial halves of the two retinae are anaesthetic. Nasal hemianopsia is exceedingly rare and of course would be most likely a unilateral phenomenon, whereas temporal hemianopsia is practically always bilateral. Amblyopia occurs at first but total blindness comes on sooner or later from the consecutive atrophy. The optic tract behind the chiasm may be injured by tumors at the base of the brain on the inner part of the temporo-sphenoidal lobe. Softening and hemorrhage occasionally, but rarely, damage the tract. Sometimes spots of disseminated sclerosis occur in it. The crura cerebri may be implicated at the same time. Within the hemisphere the tract is hurt by softening, hemorrhage, tumor and various trau- matic lesions. A lesion that destroys a tract on one side causes homonymous hemianopsia. As I have pointed out elsewhere the line of division in these hemianopsias is vertical and does not pass, as a rule, through the fixation point. It passes around the latter so that the central acuity is not disturbed. There are many variations on this, however. The variations are probably due to individual differences in the de- cussation of the nerve fibres. There is no special localization valua- tion to be attached to these differences in the course of the line of division between the light and dark half-fields through or around the fixation center. Cowers says he doubts whether the dividing line ever passes actually through the fixing point* The hemianopsia is not always complete. A quadrant may repre- sent the blind area or any sector-like form. This is to be attributed to the partial extension of the lesion in the tract or visual centers. It is very rare in diseases of the tract where the fibres all lie so close together. It has been seen in partial softening. Partial hemianopsia is common enough, however, in disease of the occipital lobe. In hemianopsia there is always some central amblyopia, even when the division is a considerable distance from the fixing point. The light half of the fields in hemianopsia usually preserve their normal boundaries. Very often, however, they arc contracted some- what, even when there is no suspicion of a peripheral or ring neuritis. I have already referred to the fact that the color fields are affected in hemianopsia in the same way that the light fields are. / /emiaehrom- atopsia is a remarkable phenomenon that has been occasionally seen. The half fields for color are affected while those for ordinary white light are intact. The division line here seems to pass through the fixation point. The lesion in this case is probably in the occipital lobe. Just where the center that is affected is located in the lobe we do nol know. Except as hemiachromatopsia (lesion in the occipital cortex) and with a contraction of the unaffected half fields (lesion probably in the 790 THE N0N-NEUR0NIC DISEASES optic radiation near the thalamus), there is nothing distinctive about hemianopsia by which we can localize the lesion more definitely than to say it is in the optic tract somewhere between the chiasm and the occipital cortex. As the connection between the optic tract and third nerve occurs in the primary optic centers, Wilbrand and Wernicke have tried to make use of this fact in localizing a lesion in connection with hemianopsia. If light be thrown on the unaffected half-field and the lesion causing the hemianopsia be in front of, or in, the primary optic centers, there will be no pupillary response ; but if the lesion be behind the geniculate and quadrigeminal bodies, there will be a pupil- lary response. This does not work out so well clinically, though theoretically it is strictly correct if we are sure of our knowledge in regard to the reflex centers in the primary optic nuclei. Other symptoms added to the hemianopsia often enable us to localize the lesion very definitely. Hemianopsia due to lesions in the optic tracts immediately behind the chiasm is usually accompanied by other cranial nerve symptoms. If the lesion be in the thalamus or external geniculate bodies, the collateral symptoms will generally show implication of the internal capsule and be a hemiplegia or hemianses- thesia on the same side as the hemianopsia. If the lesion is in the left hemisphere, the visual paths are so located that with the hemi- anopsia will probably be some speech disturbance. If the lesion is in the cortical or subcortical fibres, irritative signs, such as facial hallu- cinations which are apparently the only symptoms of an irritating focus in the visual center, will appear. For obvious reasons, reading is extremely difficult in right-sided hemianopsia. The condition, even when slight, is usually recognized by the patient, and yet a case is recorded where the trouble was only discovered by the nurse noting that the patient never ate the articles of food placed upon one side of the plate. Hemianopsia patients usu- ally carry their heads turned a little toward the blind side. These patients are liable to accidents. Bilateral hemianopsia, if complete, necessarily results in total blindness. In this way extensive lesions in the optic apparatus behind the primary centers cause complete loss of sight. Incomplete bilateral hemianopsia, in which central vision is not lost, may not result in total blindness, but the orientation, or ability to distinguish things will be greatly impaired. Complete color blindness of apoplectiform onset, bilateral hemiachromatopsia, has been seen. The ordinary vision was not disturbed. There are no ophthalmoscopic changes in hemianopsia, except what may be attributed to the causative lesion, such as a tumor or associated hemorrhage. Hemianopsia may be transient or permanent, functional or toxic. It is not at all improbable that in some cases of uremic amaurosis and lead poisoning, there is a toxic, transient, bilateral hemianopsia of cortical origin, for the pupillary responses often remain normal. It these are lost too then the blindness is probably neuritic in origin. A transient functional hemianopsia may associate itself with the scin- THE NON-NEURONIC DISEASES 791 filiating scotomata of migraine. As an indirect focal symptom, a transitory hemianopsia has been seen to follow an apoplectic attack. Psychic Blindness. This peculiar disorder was first observed by Munk in animals. It has been noticed in man also a number of times. Vision is not lost but visual comprehension is gone. Objects, including of course, written word symbols are seen but not recognized. There seems to be a complete failure of memory pictures in the visual sphere. A familiar person, a chair, a street are clearly seen but they seem perfectly strange and unfamiliar to the patient. The patient sees with his eyes but not with his mind. This psychic blindness is not the same as visual aphasia. Munk produced the condition in animals by removing the occipital lobes. In man it has usually been associated with homonymous bilateral hemianopsia of one side. As it does not always occur with hemianopsia, there is doubt about its being a mani- festation of an occipital lesion. This is so frankly recognized that those who have tried to explain the phenomenon, have adopted the notion that there is still a higher visual center, possibly in the anterior part of the occipital lobe or lower part of the parietal lobe, that re- ceives impulses from the occipital cortex and there gives them their higher or psychic interpretation. The existence of this so-called higher visual center is not only hypothetical but it is unsupported by the slightest shadow of anatomical evidence. It is a sorry makeshift from one dilemma into another. Psychic blindness is a psychic manifestation and should be classi- fied under the previously named psychic symptoms. I mention it here because it happens to be a psychosis in the visual sphere. As I have stated at the end of the section on the mental manifestations of brain disease it is a crude conception of psychological phenomena to think that they are subserved by small cortical areas scattered here and there throughout the brain. It belongs to the mode of reasoning adopt- ed in the phrenology of the long ago. Visual recognition is a complicated act. It is the result of a combination among a great many elementary psychic sensations. These psychic sensations have their physiological counterparts in the pure physiological visual sensations that are received by the cortex of the occipital lobe. Simple psychic elementary vision may well be conceived therefore as having its seat in the occipital cortex. But psychic ele- mentary vision is something very much more simple than visual mem- ory and visual recognition. These are the result of inter-connections between psychical compounds which are themselves made up of anatomico-psychic elements. From the very nature of things there cannot be a single center for compound, complex psychic phenomena, though there may be centers where the various constituent elements are brought in touch with one another, or at least in which certain physiological inter-connections are established from which start the various psychic manifestations. The whole doctrine of the cortical centers has up t<> the present time been most crudely taught and only now arc we beginning to realize that these so-called centers which we have heretofore supposed subserved most complicated psychic acts arc merely the starting 79 2 THE NON-NEURONIC DISEASES points, the threshholds, the central clearing houses, as it were, of the vast and intricate workings of the mind which for its physical basis requires the whole brain with all its enormously inter-connected parts. Psychic sensation of the elementary vision may have its seat in the occipital lobe. Psychic visual memory and comprehension is probably coextensive with all of the brain functions. Visual hallucinations are psychic symptoms that happen to be in the visual sphere. Closely related to them, if not exactly the same, are mcgalopsia, or the apparent enlargement of objects, and micropsia, or' the apparent reduction m the size of objects. These may be due to displacement of the retinal rods and cones. More often, however, they are among the psychic phenomena of neurasthenia and hysteria. Monocular diplopia seen in hysteria is a psychosis. True diplopia is a visual disturbance caused by paralysis in the ocular motor nerves and is mentioned elsewhere. Hyperesthesia of the retina, causing photophobia, lachrymation and involuntary closure of the eyelids may be due to optic nerve hyper- sensitiveness. There is reason to believe that the fifth nerve is at the bottom of photophobia. It is a suspicious fact that diseases in which photophobia is most marked involve the structures that are innervated by the trigeminal nerve. There may be a central inter-connection, however, between the optic and the fifth nerves. Gustatory Symptoms. — These are easily examined by placing various substances on the tongue. The four kinds of taste are sweet, sour, salty and bitter. Sugar, vinegar, salt and quinine may be used to test each respectively. Disorders of taste are usually due to nerve diseases and have been referred to under the head of the trigeminal and glosso-pharyngeal nerves. There are hallucinations of taste. Auditory Symptoms. — These are tested by whispering, after closing the ear that is not under examination, by holding at different distances a ticking watch and by sounding a tuning fork. A normal person should be employed as a control test. Bone conduction of sound is determined after closing the ears by placing the watch, or handle of the sounding tuning fork against the mastoid bone or be- tween the teeth. Bone conduction is often preserved when the mechani- cal hearing is lost. When the nerves and their centers are diseased. bone conduction is also lost. Rhine's test is an excellent one to distinguish nerve from mere ear deafness. In a normal person the vibrating fork, after being placed against the bone and no longer heard, is placed in front of the ear. Its sound is again heard. This occurs exactly the same way in nervous impairment of audition, except when it approaches actual deafness. In mechanical impairment, the fork is not heard the second time. Weber's is also a good test. Place the sounding fork on the frontal bone and it will be heard as it from a distance. The sound will be referred to both ears. If one ear is then closed, the sound will be referred to that ear. When the same test is made upon a patient, if the impairment of hearing is nervous the sound will be said to be heard in the well ear ; if it is due to the sound-conducting apparatus, it will be referred to the diseased ear. THE NON-XEURONIC DISEASES 793 According to Schwabach the fork on the head is heard longer than normally in disease of the conducting apparatus, shorter than normally in cerebral impairment of hearing. When there is irritability with audition, the condition is some- times called acoustic hyperesthesia. Paracusis is where a tone is heard deeper or higher than it really is. If the sound ear hears the normal tone at the same time, the condition is called diplacusis. DISEASES OF THE MEMBRANES OF THE BRAIN. The diseases of the intracranial meninges are all essentially in- flammatory in type. The origin, course and ultimate results of this inflammation may be different in different cases but its essential nature is the same in all. It may be so slight, incipient or transient as to merely amount to a circulatory disturbance. Anaemia and hyperemia may express the entire pathology of the case; on the other hand the most violent characteristics of a severe inflammation, simple or puru- lent, may constitute the disease process. Of the three membranes the dura mater and the pia mater are the only ones whose inflammation is of clinical significance. The arachnoid is never diseased by itself. Ancemia. — This has no clinical existence as a disease by itself, so far as we know. In anaemia of the brain, the membranes are also anaemic. It cannot therefore be discussed here. Hypcrccmia. — Congestion is always the first stage of an inflam- mation and consequently has all of its symptoms in a slight or transient form. There may be headache, throbbing and full in character, ver- tigo, delirium, convulsions, and even coma and death. These fulmi- nant cases reveal an intense meningeal hyperaemia, which is perhaps better described as a rapid, overwhelming meningitis. The whole or a part of the membranes may be involved. When the pia is impli- cated, it is a part of congestion of the brain. When the dura is the seat of the congestion it has the same etiology and symptomatology practically as pachymeningitis. PACHYMENINGITIS. As the dura mater consists of two layers, a thin, internal, smooth- ly epitheliated layer and an external, loose, periosteal layer, either of which may be primarily inflamed, we have a pachymeningitis interna and a pachymeningitis externa. External Pachymeningitis. — This is the most common inflam- mation of the dura, (ienerally it is a secondary disease and is asso- ciated with surgical conditions. Etiology. — Fractures of the skull, blows and other injuries to the head, with effusion of blood between the bone and the membrane, are the most glaring causes of it. Diseases of the bones of the head, caries and necrosis of the petrous, mastoid, ethmoid or outer table even of the squamous bones may light up the inflammation. Erysipelas, otitis media, ozaena and other disease^ may cause it by extension. 794 THE NON-NEURONIC DISEASES Syphilis and neoplasms have originated it. As a great rarity it has been seen to occur without any traceable cause. For obvious reasons it is rare in children. Pathology. — The findings of the primary trouble which has caused the pachymeningitis are of course a part of the pathology. The dura itself exhibits all the changes of a purulent inflammation. At first it is congested, red, cedematous and later on infiltrated and bathed in pus which flows and accumulates between it and the bone. Oc- casionally the purulent matter extends between the two layers of the dura and even into the arachnoid beneath it, setting up a purulent dura-arachnitis. The pia may become involved and all the membranes be glued together or bound by adhesions. Sometimes the inflammation goes down without the appearance of pus, in which case it often be- comes firmly attached to the skull and is penetrated by osteophyte growths. In syphilitic cases the bones may be greatly thickened, the pus large in quantity and the membrane itself in a very rough and almost sacculated condition. Symptoms. — These are indefinite because they are obscured by the symptoms of the primary disease and because the lesion is so out- stretched and variable. The discovery of the original cause will often enable the diagnostician to determine which of the mass of symptoms present are to be attributed to the meningitis. Usually there is a gradual onset of headache, fever and delirium. There are compres- sion symptoms, especially of a hemiplegic type if the motor cortex is implicated. Therefore convulsions and paralyses are to be looked for. Focal symptoms are rare. I once was able to follow the extension of the brain compression by the extension of the clot from the hem- orrhage, in the case of a child whom I saw fall several stories and fracture its skull. Trephining failed to save life. The course of these cases is acute or subacute and is largely due to the nature of the primary disease. As a rule the prognosis is serious but somewhat better than it is in most of the other forms of menin- gitis. The treatment is entirely surgical and is largely the treatment of the underlying cause. Pus must be evacuated. If there is reason to believe that there is no pus present, the treatment of the mem- branous trouble is the same as for any meningitis. Pachymeningitis Interna. — Pathologists recognize two forms of this, the purulent and the hemorrhagic. They are both uncommon, especially the former. Some authors, like Osier, speak of a pseudo- membranous internal pachymeningitis, though it is not usually recog- nized. Osier saw it as a secondary process in pneumonia. Purulent internal pachymeningitis has been seen, very rarely, however, as a primary condition. Generally it is secondary to purulent inflamma- tion of the pia or dura. Hemorrhagic internal pachymeningitis is a most interesting and remarkable disease and is still a subject of much debate among pathologists. Some writers classify it among the intra- cranial hemorrhages while others still continue to regard it as a men- ingeal inflammation. Pachymeningitis interna hemorrhagica. Hematoma of the Dura THE NON-NEURONIC DISEASES 795 Mater. Meningeal Blood Tumor. — This is a rare disease of the dura mater, characterized especially by the formation of an inflammatory membrane with hemorrhagic effusion upon its under surface. Whether the hemorrhage or the membranous deposit takes precedence in the disease process is the question that is still being debated. I am in- clined to think that the disease is primarily a meningitis of a particular type and that the hemorrhage is a secondary accident, as it were. According to Gowers, Prescott Hewitt., as far back as 1845, described the trouble as primarily a dural hemorrhage. Virchow studied it anew and in 1854 demonstrated that the origin of it was a particular variety of pachymeningitis. This explanation satisfied the medical world until Huguenin recently revived Hewitt's hypothesis and ad- duced some evidence in support of it. As the question is still sub judice, the disease will continue to be classified by authors in accordance with their individual confidence in the proofs of one view or the other. Etiology. — The disease is very rare in general practice. It is a secondary condition and most of the cases are seen among the insane in the asylums. It seems to be consecutive to some forms of chronic insanity, especially chronic dementia, general paresis, senile dementia, chronic mania, chronic melancholia and chronic epileptic psychosis. It has been seen to follow acute mania and imbecility, chronic alcohol- ism and Huntington's chorea. Most of these diseases, it will be noticed, are chronic inflammations and atrophy of the cerebral tissue. It has occurred secondarily to certain vitiated and cachectic blood states, such as is seen in profound anaemia, in hemorrhagic purpura, certain acute fevers like rheumatism and smallpox, in scurvy, leukemia, and rarely syphilis. It may be observed in a mild form in renal dis- eases, chronic heart troubles and phthisis. Traumatic injuries to the head may cause it. It may occur as a primary trouble also. Males are subject to it more than females. It is so comparatively frequent in old age, especially with mental breakdown, that it is sup- posed to be in part at least the expression of physical deterioration as much as of actual disease. It occurs in infantile scurvy though as a rule it is relatively infrequent in childhood. Pathology and Pathogenesis. — The essential feature of dural hematoma is the formation of a delicate, vascular membrane on the under surface of the dura mater and between it and the arachnoid. In about half of the cases this formation extends bilaterally over both "hemispheres. In many cases, however, it is limited in extent, spread- ing over only one hemisphere or part of a hemisphere. It may occur at the base of the brain, in the middle and posterior fossae likewise. It is at first a soft red tissue which afterwards becomes paler and harder and more compact. It varies in thickness and has a laminated con- struction with hemorrhagic extravasation between its six or seven layers. Its edges may be adherent and the blood-clot be inclosed in a sort of sac arrangement. Sometimes these contain merely a slightly -colored serum with crystals of cholesterine. Adhesions between the layers of the membrane at a number of places cause it to have a cystic appearance. The vascular condition, its rich supply of blood- vessels and the degeneration of the extravasated blood, causes the 79^ THE NON-NEURONIC DISEASES ruddy tint in the earlier stages ; while development of delicate fibrous- tissue and other organization material are responsible for its later firmness and paleness. Not all cases present the same exhibition nor does the same case manifest exactly the same changes in all parts of the membrane at the same time. In mild cases, for instance, the latter may be like a veil or delicate piece of lace, faintly rose-tinted and easily pulled off from the dura. The hemorrhages into this may be small and punctated, causing spots of brown, brownish-red or yellow here and there according to the age of the extravasation. On the other hand the disease process may be so violent and elaborate that a thick, many-layered, intensely red and hard deposit is made. It may cover the brain like a closely-fitting cap and adhere it strongly to the men- inges. In such cases it is difficult to pull it away without tearing off pieces of the brain substance. The brain next to it is usually atrophied. Three typical apearances may be noted in the development of this hematoma. Some cases clearly show that it consists of a deli- cate, inflammatory or organization exudate into which has penetrated numerous bloodvessels and much granulation tissue. In other cases- there seems to have been nothing but a simple subdural hemorrhage, though, as Osier suggests, the membrane may have been in existence but was destroyed by the blood. In a third set of cases both vascular membrane and hemorrhagic extravasation seem to have been developed together. It is owing to the fact that the membane thus sometimes appears without the evidences of hemorrhage, and that at other times the hemorrhagic residua appear without the evidences of the formation of a membrane, that so much controversy has sprung up in regard to the primary nature of the entire process. Virchow adduced strongs proof that the trouble began with internal pachymeningitis, that the membrane was nothing but an inflammatory exudate and that the presence of the blood was a mere secondary phenomenon. The etio- logy and course of the disease, the appearance of the exudate without hemorrhagic extravasation in the light cases, and above all the irregu- lar distribution and cystic and punctated character of the blood de- posits, as though when fluid it found difficult)' in spreading out evenly over the surface of the dura, are strong arguments in favor ofVirchows view. Hence I treat of the disease as a form of pachymeningitis. It must be admitted, however, in all candor that the position taken by Huguenin and others is far from being a weak one, namely, that a dural hemorrhage takes place and that later on the clot undergoes organization and produces all the collateral appearances through irri- tation both of the brain and dura. Symptoms. — The clinical picture of hematoma of the brain is very indefinite, for its symptoms may range from nil all the way to those of a profound apoplectic stroke. They are least marked or absent entirely in the slight eases, whereas in the traumatic and other cases they are both severe and extensive. Ofttimes they are marked by the general symptoms of the disease of which the hemorrhagic effusion is an accompaniment. This is the ease in general paresis par- THE NON-NEURONIC DISEASES 79/ ticularly. The seat and character of the lesion, the nature of the -associated disease or injury, and the stage at which the disease has arrived all determine the general outlines of the symptomatology. When a scries of apoplectiform seizures occur, followed by cerebral pressure symptoms, in a patient affected with any of the diseases known to be causative of hematoma, the latter may be diagnosed. Usually the first symptoms are very gradual and insidious, almost prodromal, in their onset. A feeling of general distress and restless- ness, excitement almost resembling delirium, is followed by an in- creasing headache, vomiting, unilateral epileptiform twit chin gs and coma. The patient emerges in rare cases from this or he may remain in this state for several days or weeks and then die with or without another seizure. At times the beginning of the trouble is a genuine apoplectic stroke. All of the usual signs appear, coma, paralysis, con- jugate deviation of the eyes, contraction of the pupils, retardation of the pulse, labored respiration, etc. Vomiting and elevation of the tem- perature are not infrequent. All of the symptoms referable to unila- teral or bilateral pressure, tollowing a sudden access and showing more or less of a progression, may appear in different cases. The hemiplegia may be one-sided or double. Both it and the monoplegia are not as complete as in ordinary intracranial hemorrhage and are often pre- ceded by local spasms and general convulsions. A hemiparesis of one side may occur with marked contractures on the other. These are all often merely temporary phenomena and may appear with or without coma. It is very exceptional for the sensibility to be disturbed. Aphasia and nystagmus have been seen in some cases. Choked disc is rather a frequent manifestation. The temperature may be subnor- mal but it very often goes very high.. 106 degrees F. just before death. Diagnosis. — This is always extremely difficult. In children it is pretty nearly impossible. The chief traits that must be depended upon are the presence of injury (these cases usually present the most marked clinical presentation) or disease with which hematoma is known to occur, a series of apoplectiform seizures occurring like ex- acerbations with remissions in a disease that manifests headache, ver- tigo, choked disc and other pressure symptoms, temporary or slight monoplegia or hemiplegia followed and preceded by spasms, convul- sions and contractures. The extensive involvement of the body with such paralytic manifestations, not so profound as they are in ordinary hemorrhage, would be strongly suggestive of hematoma. Cerebral glioma sometimes presents itself in the same way as hematoma. The diagnosis is then impossible, though a suspicion may be aroused by the presence of some mental or senile condition in which hematoma occurs. In ordinary meningitis the diagnosis is often very difficult. Haema- toma, however, is devoid of nuchal rigidity and cranial nerve < except the optic) involvement. In meningitis the remittent character of the symptoms, the apoplectic seizures, the choked disc and the intercurrent convulsions do not obtain. Prognosis. — This is bad. Death usually occurs sooner or later. 79^ THE NON-NEURONIC DISEASES Treatment. — This may have in view the checking of the inflamma- tion and the hemorrhages. In neither, however, is it very successful. The former suggests free purgation and diuresis, the application of the ice bag, leeches and bloodletting in robust individuals. Counter-irrita- tion has been tried. To check the hemorrhage has heretofore proved to be an impossible task, hence there are no measures to be recom- mended beyond those commonly resorted to in intracranial hemorrhage. In both traumatic and non-traumatic hematoma, operation has. been resorted to with a very fair degree of success. Localization symptoms of some degree of definiteness must be present. Dangerous- compression of the brain particularly suggests the consideration of surgical relief. LEPTOMENINGITIS. An inflammation of the pia mater includes as a rule the arachnoid and the cortex, so that the terms pia-arachnoiditis and meningo-en- cephalitis are justified. In most cases it is a diffuse condition, in- volving both the vertex and base of the brain and not infrequently even the spinal cord. Excepting the tuberculous and syphilitic types, the vertex is affected in most of the cases chiefly. The symptoms vary in accordance with the location of the disease, but not sufficiently so to establish that as a basis of classification. Usually the disease is acute, though it may become chronic. It is highly doubtful if it is very simple. Most generally it is infective and purulent. Sometimes it is specific and epidemic. Its symptomatology is fairly uniform in all types. No special classification of it is therefore called for, though for convenience sake we may discuss separately Acute (simple and purulent) Leptomeningitis, Epidemic Cerebrospinal Leptomeningitis and Tuberculous Leptomeningitis. Syphilitic Meningitis is considered elsewhere under the head of cerebral syphilis, and serous meningitis is referred to under the head of hydrocephalus. It is misleading and in- correct to speak as some authors do of the brain condition, the so- called "wet-brain," of subacute alcoholism as one of serous meningitis. It is not meningitis at all but a mere serous effusion. Acute (simple and purulent) Leptomeningitis. — It is very questionable whether a simple, non-toxic inflammation of the meninges ever occurs. The nearest approach to it would seem to be in some of the cases of Quincke's hydrocephalus wherein the ependyma of the ven- tricles, and even the cortical meninges, exhibit signs of a low grade of pure inflammation. Older writers used to refer to a simple meningitis that was caused by insolation, mental strain and simple cerebral con- cussion. In some of these cases which succumbed rapidly to death. nothing but intense congestion was discovered. This, however, is not accepted by many modern pathologists as proof positive of the non- purulent character of these cases. They hold that they are merely foudroyant cases that do not reach the purulent stage, or arc not cases of meningitis at all. Simple non-purulent meningitis is therefore de- clared to have no existence. Whether we are yet in a state of knowl- edge to adopt so radical a position or not. we are obliged to recognize that the vast majority of all the cases of leptomeningitis are of septic THE NON-NEURONIC DISEASES 799 origin. The source and nature of the poison are different in different cases. Usually it is an infection carried to the brain from some out- side focus by way of the blood. The meningitis is therefore a second- ary process except perhaps in the epidemic cerebrospinal form. It is convenient to group these various sources of the infective pro- cess under several general heads. Traumata, acute alcoholism and other depressing influences, and in tuberculous meningitis even heredity, exercise an influence as predisposing factors. The infective fevers provoke leptomeningitis though not as fre- quently as the mere fibrile delirium has led many practitioners to sup- pose. It occurs in pneumonia, erysipelas, septicaemia, smallpox, typhoid, influenza, measles, mumps, scarlet fever, pyaemia, acute arti- cular rheumatism, ulcerative endocarditis, empyema, gonorrhoea, etc. The first three give rise to it more frequently than the others. It has been ascribed to actinomycosis and is doubtfully associated with in- testinal catarrh. The meningitis due. to these causes is practically al- ways purulent, though the suppurative process may vary in character and severity according to the particular microorganism involved. It is an error to refer to this form of meningitis as metastatic. The disease is not transferred. The meningitis is simply the expression of a gen- eral infection of the organism and may be the only expression. A pneumococcus meningitis has been seen without any implication of the lungs whatever. Injuries and diseases in the bones of the skull, involving the men- inges are a not uncommon cause of acute suppurative leptomeningitis. The injuries that directly involve the membranes and cause the ordi- narv form of pachymeningitis are not referred to here. Fractures, blows, concussion may set up a local suppuration with transmission of the germs to the pia mater, a sinus thrombosis from the veins of the diploe carrying the infection. Caries of the petrous part of the temporal bone from otitis media, necrosis of the cribriform plate of the ethmoid and disease of the cavities of the skull and their bony walls may light up the meningeal inflammation. Neighboring foci of suppurative procc esses must also be held re- sponsible, such as chronic purulent otitis, mastoiditis, disease of the nasal cavity, the orbit, the antrum, frontal sinuses, etc. Then again the infection may come in the other direction from an abscess, a tumor, an old hemorrhagic or necrotic focus in the brain. An infected embolus from an endocarditis is a common cause of the latter. A puncture with a sound has carried the disease from the nose into the cranial cavity. Vascular sheaths form another path along which the poison passes inward ; also the nerve sheaths. In certain diseases there is a terminal infection that sometimes re- veals itself in a leptomeningitis. It is thus sometimes seen in chronic nephritis, gout, valvular and muscular heart disease, senile states, arteriosclerosis and the wasting diseases of childhood. In some cases the source of the infection cannot be determined and the cause of the disease is hidden in obscurity. Thus it is made clear that acute leptomeningitis is a bacteriological 800 THE N0N-NEUR0NIC DISEASES disease, a secondary infective process in most cases, with a varied etiology. The relationship of the disease to age, sex, climate, occupation, season and station in life is largely a matter of the original source and nature of the primary infection. It may be noted, however, that men are slightly more affected by it than are women, and that it is most common in children between the ages of one and ten, since during these years the infective diseases are most rife and disastrous. Pathology and Pathogenesis. — The great pathogenetic fact in the development of leptomeningitis is always the presence of disease germs or their products. These may be of various types and may be the basis of a general or a local infection. Somehow they get to the pia-arachnoid membrane and there set up a typical infective or puru- lent inflammation. In some cases they have been recovered from the diseased membrane post mortem ; in other cases only the remains of their destructive work have been seen. It is doubtful if all of the germs that are believed to give rise to meningitis will be recovered. We are not sure of the exact influence of those that have been so far recovered. We dare not yet dogmatize in regard to the relative importance of the germs themselves or their biological products. In a word, though we have gotten so far as to recognize acute leptomenin- gitis as a germ disease, we are still in the dark in regard to many of the details. Direct examination of the exudate has been rewarded many times with the finding of numberless streptococci and staphylococci. Of these the former are the more frequently met with. A form of men- ingitis that occurs with pneumonia and as an independent affection seems to be due to the pneumococcus of Fraenkel. It has been recov- ered many times from the membrane. The meningococcus, or diplo- coccus intracellularis of Weichselbaum, is in all probability responsible for some cases, especially of the sporadic and epidemic cases of cere- brospinal meningitis, though in these have been found other bacteria also. The pneumo-bacillus of Friedlaender, the typhoid bacillus, the colon bacillus, the gonococcus, the tubercle bacillus, and the staphylo- coccus have all been isolated from the diseased meninges. The streptococcus is the most important in the pathogenesis of acute purulent leptomeningitis. It is the germ usually found in those cases due to traumata, chronic otitis media, and the septic processes generally ; in those associated with ulcerative endocarditis, and those due to terminal infection. The staphylococci are found in some of the last. Purulent inflammation may characterize all parts of the pia- arachnoid membrane, though as a rule it appears mostly on the ver- tex. It usually covers the entire brain, though it may be unilateral or even focal. It assumes the latter form especially in those eases where the infection comes from some nearby suppurating focus. It is more extensive in the general, infective cases. The pia is at first congested. violently reddened, oedematous and cloudy. Gradually a purulent de- posit spreads over the sulci, collections of pus appear in spots, unite and finally give to the membrane a yellowish, thick, creamy appear- THE NON-NEURONIC DISEASES 8oi ance. The convolutions are completely hidden. Infiltration takes place into the superficies of the brain substance ; small hemorrhages occur ; pus deposits in small foci ; and a general encephalitic process with some atrophy even may appear. Encephalomalacia and abscesses are possible. Very prone is the pus to deposit itself at the base of the brain in the Sylvian fissure around the chiasm, beneath the cerebel- lum and beside the pons. Sometimes the dura is implicated. In the ventricles there pours out a sero-purulent exudate which may so distend them that a distinct condition of hydrocephalus is produced. The distension is rarely, however, as great as it is in the tuberculosis form of the disease. As a very rare condition it is said that a puru- lent meningitis may be limited to the ventricles in childhood. A per- manent collection of fluid may be left in the ventricular cavities. In many cases the spinal meninges are simultaneously affected, so that the disease may be truthfully termed a cerebrospinal diffuse meningitis. In and about the sheaths of the nerve trunks the purulent matter may sometimes be seen. Symptoms. — Pathology and practical diagnosis are at logger- heads in regard to the frequency of meningitis in the infectious fevers. The former finds it very rarely, the latter very often. This discrep- ancy is accounted for by the fact that many symptoms of simple con- gestion as it occurs in these fevers are like those of a frank menin- gitis. Mild delirium, muscular twitchings, spasms and retraction of the neck, seen in typhoid fever, for instance, are largely due to the com- bined effect of the infection, congestion and elevated temperature rather than to an inflammation of the meninges. Post mortem exam- inations show that the latter is a very rare complication of the fever. The clinical picture of an acute purulent leptomeningitis is a very variable one, because it is the result of a general, diffuse lesion of infectious origin. Nevertheless there is a sufficient degree of uni- formity about it to establish a description of the disease which will more or less fit all cases. There are always two sides to it. On the one hand are the symptoms that are always characteristic of a general infection ; on the other are those that indicate implication of the en- tire brain or parts of it. The differences between the various types of infection that cause the meningeal trouble produce differences in many of the details of the general symptomatology of the infection. The differences between the locations of the main foci of inflamma- tion in different cases cause a marked variability in many of the cere- bral manifestations. Some cases appear abruptly while others have distinct prodromata. A feeling of general malaise, head soreness, heavy feeling about the eyes, vertiginous distress, possibly vomiting, anorexia and general irritability and uncompanionableness in a few cases precede for some days the outbreak of the signs of inflammation. The prodromata arc: more characteristic of tuberculous than of the simple acute menin- gitis. The latter as a rule begins somewhat suddenly with a chill, an intense headache, a rise of temperature, a rapid pulse and all the usual signs of an acute infective process. If these occur in the course of the same infective process elsewhere in the body, they will be obscured, 802 THE NON-NEURONIC DISEASES of course, by the symptoms of the general febrile disease. In such a case the involvement of the meninges must be determined largely by the nature and location of the original source of infection, and its probable influence in producing a meningitis, as well as by the more definitely localizing cerebral manifestations. Among the latter headache easily takes the first rank. It is a sharp, excruciating pain that appears with intense exacerbations all over the head, though chiefly in the occipital and frontal regions. It is never entirely absent, though at times it may undergo a temporary recession or remission. It is a persistent, steady pain with sharp, boring, knife-like flashes. Even stupor does' not bring relief to its victim for in the night when asleep, or later in the disease when the comatose condition comes on, he cries out and seizes and grasps his head with his hands in manifest suffering. In certain rare cases of septicemic meningitis, and others due to blood states, the disease has been seen to run its course without, or with only a slight, headache. Vomiting, of the cerebral type, is very often present. It should be distinguished, if possible, always from the vomiting that some- times accompanies the profound alimentary disturbance caused by the fever and general infectious disease. It is sudden, projectile and unin- fluenced by the presence or character of the food in the stomacn. With the headache and vomiting, there is always more or less vertigo, especially when the patient moves. Nausea may or may not coexist. The bowels are generally constipated and there is flattening of the abdomen with a rapid development of an appearance of general emaciation. Most cases are initiated with a fever which in a few days rises to 103 degrees or 104 degrees F., sometimes higher. Just before death it may go to 108 degrees. It may be masked by the. fever of the pri- mary disease. It is an irregular, fluctuating temperature. In some cases it may be subnormal, and in a few instances it is normal through- out the entire course of the disease. The usual febrile accompani- ments such as dry skin, coated tongue, parched lips, all go with it. The pulse is rapid, rising to 160 and i"8o before the lethal issue. Very often it is slow, even as low as 40 and unsteady. The respirations are not often very greatly disturbed. They may. however, be rapid, irregular, feeble and suddenly cease. A Cheyne- Stokes breathing is a bad omen. Meningitis of the base of the brain and particularly in the posterior fossa causes these phenomena most markedly. The urine is scanty, high-colored and usually contains some albu- min. Sometimes sugar is present in it. It is purely a febrile urine unless it partakes of the composition of a urine that would be ex- pected in the primitive disease underlying the meningitis. The men- ingitis of the terminal stages of renal and cardiac affections must not be forgotten in this respect. During stupor both retention and in- continence of urine may be expected. After the headache perhaps the most characteristic symptom of meningitis of this type is the delirium. This comes on early, as a rule is first noisy and excited, then becomes milder and muttering and THE ^OX-NEURONIC DISEASES 803 finally sinks into stupor and late coma. Sometimes it alternates with a drowsy or somnolent condition. The patient is dazed in it, it mani- fests itself in his sleep and with it there are evidences of distinct blunt- ing of his sensorium. It usually accompanies the headache and when once developed is more or less persistent until the advent of the coma. Hyperesthesia of the skin and of all the sensory nerves, particu- larly the optic and auditory, is very often a pronounced symptom. The patient will complain of the touch of the bed-clothing, of a slight draft of air, of the light in the room and of every trivial noise. If stupid he will suddenly start when touched. In quite deep stupor I have seen the patient move when touched, in a slow, reflex, automatic way that was most weird and uncanny. The muscles are also hyper- sensitive so that a slight tap will set up a most vigorous reflex. The so-called spinal phenomenon, which is a sort of temporary opisthotonos, is partly due to muscular hyperexcitability. A light tap on the lum- bar muscles sets up a spastic retraction of the column. Sometimes the muscles are permanently rigid and spastic. This is particularly the case in the neck. The child seems to have its head drawn back- ward in an effort to burrow it into the pillow. Disease in the pos- terior fossa especially provokes this symptom. Another expression of the general muscular rigidity is seen in Kernig's sign. At one time this was supposed to be pathognomonic of meningitis. It has been seen, however, in many other diseases, though it is a valuable one in meningitis. It is easily tested. With the patient sitting or lying on his back, place the thighs at right angles to the body and note how impossible it is to completely extend the legs upon the thighs. The sign may be unilateral, as I have several times seen it, or bilateral as it usually is. The rigidity and retraction of the abdomen gives it the well known scaphoid, or boat-like appear- ance. All of the nerves of the body, especially the cranial nerves, may enter into the make-up of the clinical picture. Irritability, rigidity and paralysis may be among their exhibitions. A certain amount of local- ization of the lesion can be determined by the general and cranial nerve symptoms. The latter, for instance, are more obvious in basal men- ingitis ; hemiplegic and monoplegic phenomena in vertex meningitis. Even unilateral hyperesthesia and hemianesthesia have been seen. Pupillary changes are very frequent and important signs of the disease. As a rule the pupils are bilaterally, very rarely unilaterally, contracted in the earlier stages of the affection ; dilated in the later. Ptosis and strabismus, and even nystagmus, are present at times and easily accounted for. Strabismus is particularly important, especially if it shows a tendency to be transient at first or present only on move- ment. Implication of the facial nerve occurs often. The facial mus- cles are spasmodic at first, then rigid and later on partially paralyzed. Trismus may be present. The paralysis is of the lower motor or peri- pheral type with partial reaction of degeneration. Other motor cranial nerves may be included as shown by the difficulty of mastication, of swallowing, of articulation, of vocalization, and of glossal movement. 804 THE NON-NEURONIC DISEASES The vision is sometimes partially affected. Optic neuritis, and even atrophy is not uncommon in basal meningitis, but it is far less frequent in the purulent meningitis of the vertex, it is very rarely complete enough to eventuate in a choked disc, and as a result the vision is but little decreased. The reflexes, trophic and vasomotor symptoms are not particu- larly significant. The deep reflexes during the irritative stage are in- creased but as stupor comes on they diminish. Among the vasomotor manifestations is the well-known tdche cerebrate, of the French authors. Drawing the thumb nail across the skin, a brilliant and more or less permanent red line is produced. This phenomenon is seen in so many other diseases that it has no special value. In many long running cases there is a strong tendency to the formation of bedsores, vesicles, etc. Hence hot bottles and other irritants must be used cautiously. Early in the disease general convulsions or even unilateral con- vulsions may occur but they are not common. Diagnosis. — The diagnosis of acute leptomeningitis is not dif- ficult if the case is a typical one and not obscured by the presence of another more general infectious process. Mistakes are constantly made, however, as the disease is rarely typical, as it is very often asso- ciated with an over-clouding septic process elsewhere, and as prac- titioners too often lay too much stress upon special symptoms. In recognizing the disease its etiology is a matter of very great importance. Not only the disease itself is rendered probable but the nature of the inflammation and the location are to be quite positively inferred from the existence of a general infection, like pneumonia and septicaemia or of a neighboring nidus of suppuration like chronic otitis or trauma. The onset and character of the symptoms should be carefully noted. They appear somewhat abruptly or rather acutely as signs of an infective process: chill, fever, vomiting, headache, rapid pulse, irreg- ular respiration, constipation, febrile urine. Soon the signs of cere- bral irritation or compression appear: persistent headache, delirium, convulsions, stupor, retraction of the neck, contraction followed by dilatation of the pupils, high temperature with slow pulse and irregu- lar breathing, Kernig's sign, and disturbed vision. Finally focal signs and terminal coma make the case plain. This picture may vary in details but in the main it is so charac- teristic that the presence of meningitis can hardly be overlooked. The greatest danger is that of calling many diseased states and many other affections with a symptomatology which resembles in some of its features the symptomatology of meningitis, the latter disease. Vor this reason the term meningitis appears much more frequently in clin- ical than in autopsy records. There is not a symptom of meningitis that does not belong also to some other disease prominently, and many combinations of the. symptoms are to be seen in other conditions. All of which again emphasizes the fact that the diagnosis of acute menin- gitis must be made upon the symptomatic presentation of the ease in toto, and that its differential diagnosis from other troubles must be attained both by direct and indirect or exclusion methods. THE NON-NEURONIC DISEASES 805 Perhaps the commonest mistake that is made is to diagnose an incipient infectious fever of atypical onset for meningitis. This error is more frequent than the reverse, namely, the diagnosing of a men- ingitis as a simple infectious fever. Typhoid and pneumonia probably are the leading culprits in this respect, though septicaemia, pyaemia or any of the other septic affections may cause the error. If the lungs are affected their examination, as well as the presence of all the usual signs of a pneumonitis, will indicate the nature of the malady. Adults, and especially children, have headache, delirium, vomiting, fever and stupor as a part of the general infective process without meningitis, hence the latter must not be inferred from these symptoms alone. Focal symptoms, signs of cerebral irritation, pupillary irregularities, twitchings, convulsive movements, optic neuritis, progressive advance- ment of all these manifestations from a mere initial transiency to a more or less permanency, are the aids in determining a meningitis. If the trouble be typhoid fever the rose spots, the characteristic stools, the course of the temperature, the enlargement of the spleen and the Widal blood test will be present ; if meningitis, the general and focal signs of this trouble will be in evidence. Jenner pointed out long ago that simple febrile headache ceases with the onset of delirium, whereas in meningitis it continues with the delirium and even with the stupor. Septicaemia is to be suspected from the absence of the more charac- teristic clinical picture of meningitis, and the presence of repeated chills, cutaneous and retinal hemorrhages, swelling of the joints and suppurative processes generally. A history of ear disease and the examination of the ear and mas- toid cells will help to distinguish a cerebral abscess in the posterior fossa from a meningitis in the same locality. This distinction is always difficult, however, particularly as some leptomeningitis may accompany even an extradural abscess. Much more difficult to com- prehend are those cases of purulent otitis with cerebral manifestations much like those of a meningitis, which rapidly get well after opera- tion, followed by drainage of the ear and mastoid bone. A pyaemia may explain some of the symptoms but the occasional optic neuritis, strabismus, aphasia and other focal symptoms cannot be so accounted for ; and the recovery is too rapid and complete to suspect the presence of a meningeal inflammation. Retraction of the head is seen in rheumatism of the muscles of the back of the neck but the other characteristic signs of meningitis, espe- cially the involvement of the cranial nerves, are wanting. Urcemic poisoning ofttimes resembles an irritative meningitis. Unlike the latter disease the urine contains casts with the albumin, and the focal symptoms are wanting. Simple meningeal hemorrhage is abrupt in onset, apoplectic in character and without the febrile and septic manifestations. Intracranial tumor produces headache, vertigo, stupor, but not the septic and febrile symptoms. Choked disc and even optic neuritis are comparatively rare in meningitis. An acute syphilitic meningitis may be determined by the history of the infection and the results of antispecific medication. 8o6 THE NON-NEURON IC DISEASES Pseudo-meningitis is a complexus of symptoms which resembles that of a true meningitis but which upon autopsy reveals no indica- tion of the latter. Congestion, oedema and other changes have been noted. Quincke's hydrocephalus, alcoholic, so-called, serous menin- gitis or "wet brain," and some other forms of developmental and toxsemic anomalies belong here. It is possible that mild auto-intoxi- cation from gastrointestinal catarrh sometimes causes these pseudo- meningitic symptoms in children. In all of these cases whether hydro- cephaloid, encephaloid, toxaemic or congestive, the absence of the char- acteristic febrile phenomena, the peculiar history of the cases, the non- purulent indication of the entire trouble, the absence of the focal symp- toms and the non-meningeal traits of the disease in its general course are to be made note of. Some very awkward errors have been committed between hysteria and meningitis. Oppenheim was called to see a woman who had had her head shaved and antimonial ointment rubbed on it, and yet who was out of bed, well and attending to her duties in a few hours. This mistake is more likely to occur between hysteria and tuberculous men- ingitis. A full appreciation of the real nature of hysteria, a psychosis, and the detection of distinct organic symptoms, such as the fever, the pupillary changes, the optic neuritis, the incontinence of urine, the cutaneous trophic lesions, will help to distinguish one disease from the other. The psychic excitement, the psychic distribution of the sensory phenomena and paralyses, the variability, and rapid change- ability of these under psychic influence are unmistakable in hysteria. The permanency of psychosis or psychic traits in the clinical picture indicate the organic disease. Errors are liable either way, hysteria for meningitis or meningitis for hysteria. During the last few years lumbar puncture has been employed to a considerable extent, in diagnosing meningitis. Beyond indicating the presence of certain inflammatory ingredients in the cerebrospinal fluid, and thereby demonstrating the presence of inflammation somewhere in the cerebrospinal canal, it cannot be said to have proved itself a ver\ definite diagnostic procedure. The risks attending it so far outbalance the smallness and uncertainty of the knowledge obtained by it, that it cannot be recommended as a routine method. In certain cases where the diagnosis is absolutely impossible by the symptoms, and it is im- perative that the nature of the disease should be definitely known, it may be resorted to. The details in regard to it will be found discussed in an earlier section upon diagnosis of diseases of the nervous sys- tem or of the spinal cord. The cerebrospinal fluid is to be examined physically, chemically, microscopically and bacteriologically. The find- ings will be uncertain and on account of adhesions here and there between the inflamed membranes, shutting off parts of the central cavity, nothing definite will be learned as to the location of the inflam- matory process. The chief information obtainable from the proeedure is in regard to the bacteria when they are reeovered from the fluid. and the presence of an actual inflammatory lesion. There are no - cial physical changes except perhaps a slight thickening and clouding of the fluid in some of the cases. The presence of large numbers oi THE NON-NEURONIC DISEASES 8o # 7 leucocytes will indicate a purulent meningitis. The bacteria that may be isolated are especially the streptococcus, the staphylococcus, the pneumococcus, the meningococcus intracellular is, the diplococcus lanceolatus and the tubercle bacillus. The albumin may be increased and some blood may be present. Prognosis. — This is generally bad. Recovery from purulent meningitis never occurs. Some simple forms have become chronic, while a few have recovered. Gowers, however, cites two recoveries from the symptoms of meningitis in post-puerperal septicaemia. The difficulty of the diagnosis always casts a doubt upon the reported re- coveries and yet warns us never to be too positive in holding out an unfavorable prognosis. Coma is practically a fatal sign. The less acute the disease is the better the chance of its becoming chronic or disappearing entirely. If coma does not come on for three or four weeks, the chance of es- caping death is that much improved. Traumatic cases and those sim- ple cases due to adjacent disease are perhaps the least grave. Sinus thrombosis and cerebral abscess are not uncommon complications and enter somewhat into the character of the prognosis. Treatment. — In a disease so invariably fatal one approaches the question of treatment with a degree of hesitancy. Certain measures and certain remedies have been recommended, and prophylaxis is always in order ; but after all has been done, it will be found that the course of the affection has been but little modified. The primary disease or source of infection should always be attended to, of course. An otitis media, a diseased mastoid, a puru- lent rhinitis, any abscess or suppurative focus in or near the cranium, should receive proper surgical treatment. An incipient meningitis may be checked thus. Cranial traumata should be properly dressed and trephining should be thought of in meningo-encephalitis. The patient should be put absolutely at rest in bed and all noise and light excluded from the room. The ventilation should be free ; the diet should be light, nutritious and frequently given. If swallow- ing is difficult, nutrient enemata may be resorted to. The temperature is to be kept within bounds by cool sponging, and the pains are to be assuaged by opiates, phenacetine and antipyretics. The bowels should be opened freely with calomel and salines. Diuretics are als > commendable. The rationale of bloodletting, counter-irritation, cold douching of the head for a suppurative process is not very apparent. These measures all have their advocates, however. Their employment en- tertains the attendants more than it benefits the patient. In robust subjects and where the disease has followed a sunstroke. mental shock, trauma or other cause of congestion, a few leeches may be applied to the mastoid region. Not much blood ought to be ab- stracted, however. Most of these patients are already thin and anaemic and can ill afford to lose any more blood. Cold may be applied to the head with advantage. The headache is sometimes relieved and the temperature noticeably lowered. The cold (louche, the ice bag, the coiled tubing made to fit the head like a cap and to carry a stream of 808 THE NON-NEURONIC DISEASES cold water continuously, or cracked ice in a towel, may be used for this purpose. Counter-irritation with antimonial ointment after shav- ing the head, the application of vesicants to it, the use of fly-blisters or mustard to the neck and similar measures have been all recom- mended but are illogical, discomforting to the patient and without the slightest ultimate benefit. If it is thought best to counter-irritate, a few light touches with the Paquelin cautery is the best way to do it. While this is being done, hot applications should be applied to the lower extremities. A treatment strongly commended abroad is the rubbing of the head with a twenty per cent, iodoform ointment. Internally iodide of potassium, mercury, perchloride of iron in large doses, iodoform, gr. vj to gr. xij, daily, have all their advocates and are always available for trial. The vomiting is controlled usually by dietary measures, absolute quiet and the swallowing of pieces of cracked ice. It is a cerebral vomit, however, and is therefore abso- lutely uncontrollable until the cerebral excitement subsides somewhat. Digitalis may be given to support a weak pulse. Salicin and quinine, sometimes given for the elevated temperature, do not seem on that account to ward off in the least the fatal issue. Surgery has been appealed to in this disease with some slight de- gree of success apparently. Ventricular puncture, incision of the dura and evacuation of the exudate in the meninges has seemed to effect some recoveries. Lumbar puncture also has been successfully em- ployed by Quincke, Furbringer, Lichtheim and others. The spinal column has been trephined, the dura incised, and drainage established. In spite of the few apparent successes by these various measures, they are in need of further trial before a final opinion can be passed as to their actual value in the long run of cases. Epidemic Cerebrospinal Meningitis. — This is sometimes called Spotted Fever, occurs both epidemically and sporadically, and is due to a special microorganism. An acute purulent leptomeningitis is its most prominent lesion and the cause of its leading symptoms. Epi- demics have occurred in nearly all the countries of Europe and in the United States, causing great destruction of life. Four thousand peo- ple died of it in Sweden alone between the years of 1854 and i860. Severe outbreaks took place in the United States from 1805 to ^o. in France between 1837 and 1850, in other parts of Europe between 1854 and 1874, and in a great many regions since 1875. Maryland was visited by it in 1892, New York in 1893, Boston in 1898. Since the first description of it by Vieusseux during the outbreak in Geneva in 1805, the disease has been closely studied by Danielson and Mann. Jackson, Welch, Warren, North, Stille, Hirsch, Councilman, Mallory, Wright and others. Wright's contributions to the literature of the subject are especially valuable. Etiology. — The essential cause of the malady is a specific germ but there are certain other moments that enter into its etiology and must be taken into account. Children constitute the majority of its victims, though it may occur at any age. In civil life children and young adults manifest a particular susceptibility. Of course, adults make up the list of cases when it occurs in military life. In some THE NON-NEURONIC DISEASES 809 epidemics adults have escaped entirely. Males outnumber females somewhat. Cold and temperate climates constitute its favored locali- ties, and the Spring and Winter its choice seasons. Endemic influ- ences are practically nil, as the disease is seen to break out in widely separated and most dissimilar iocahties. It does not seem to be directly contagious nor invited by personal habits or characteristics. Unsani- tary surroundings, crowding together, as in tenement houses or mili- tary barracks, the squalor and wretchedness of the cities, seem to foster it. Babes nursing sick mothers have escaped; attendants upon the sick are rarely attacked; and the handling of the clothing and excretions of the sufferers does not seem to be particularly danger- ous. Over-exertion, all conditions of bodily and mental depression, association with pigs and dogs afflicted with a similar affection, as noted in some of the Irish epidemics, predispose to it. Immunity is not conferred against a second attack. Country districts are not ex- empt, and the disease very often returns in the same locality. Pathology and Pathogenesis. — As a rule the intracranial find- ings are about the same as those described under the previous section headed acute purulent meningitis. The pia-arachnoid is soft, red, con- gested, opaque and filled with a creamy, purulent exudate over the entire brain, but especially at the base. The convolutions of the hem- ispheres, the basal structures and the roots of the cranial nerves are bathed in the pus and hugged by the intensely congested membrane. By the fibrino-purulent exudate the meninges are thickened not only at the base of the brain and vertex but up and down the posterior sur- face of the cord also, especially in the dorsal and lumbar regions. In the more chronic cases patches of scattered yellow indicate the earlier presence of the exudate. The ventricles are dilated, sometimes to an enormous extent. Pus appears in them as well as in the posterior horns of the cord. The neighboring brain substance is softened and damaged, compressed by the thickened meninges on one side and the dilated ventricles on the other, and reveals foci of old hemorrhagic extravasation and even of sharp meningo-encephalitis. Small abscesses may be present also. All of the cranial nerves, but especially the optic, the trigeminus, the facial and the auditory, and all of the spinal nerves, may be found imbedded in and infiltrated by the exudate. The labyrinth as a consequence may become involved. Otitis media with perforation, irido-choroiditis and panophthalmitis may all follow. In other parts of the body signs of bronchopneumonia, pleuritis, enlarge- ment of the spleen, acute nephritis, swelling of the intestinal follicles and disease of the joints may be observed. In some cases death takes place so swiftly that none of these con- ditions have had time to make their appearance, and therefore, there are no post mortem findings. In light cases sometimes only congestion of the meninges and a mere trace of an exudate are to be discovered. There is much variation in this respect and so wide are the differ- ences that the disease has actually been classified in types, such as the malignant, the mild and the aborted, the acute, intermittent and chronic. A microscopic and bacteriological examination of the exudate has lately given us most valuable information as to the probable cause of 8lO THE NON-NEURONIC DISEASES the disease. Polynuclear leucocytes are found closely packed in the fibrinous material, also cells two to eight times the diameter of a leucocyte. In variable number many diplococci also are found, the most frequent one being - the diplococcus intracellular is meningitidis, described by Weichselbaum in 1887. The polynuclear leucocytes are almost constantly the habitat of this microorganism. Many believe that is the cause of spotted fever because it is so constantly observed. Other microorganisms that provoke or at least one associated with acute purulent meningitis have also not infrequently been seen, so that some pathologists still maintain that we are not yet in a position to affirm positively that the diplococcus intracellularis is the cause of cerebrospinal fever. It is not like Fraenkel's pneumococcus, though the latter has often been associated with it in this and other forms of acute meningitis. In tuberculous meningitis, and even in the nasal mucous membrane of perfectly healthy children, the Weichselbaum diplococcus has been observed. Further study of the bacteriology of this form of meningitis would seem to be desirable therefore before its pathogenesis can be dogma- tized upon. Symptoms. — As the clinical picture is such a varied one it will be well perhaps to describe a typical case and discuss its main symp- toms in detail first, and then enumerate and point out the differences in the recognized types afterwards. There are two great groups of clinical signs of the disease in every case. The first are those of the general profound toxcemia and constitutional malady; the second are those that belong to the menin- geal inflammation and some complicating features. The period of incubation of the germ is unknown and the prod- romata are not usually very prominent. Most cases begin abruptly or if there are premonitory indications, they consist of the usual feeling of malaise, slight headache, backache, dullness, nausea, constipation and anorexia. Much more commonly there is a chill, a sudden access of fever, vomiting and a severe headache. The scene opens just as nearly all of the infectious fevers do. Vertigo, restlessness, insomnia. and general convulsions may be added to it. In a day or so the men- ingitic symptoms begin to declare themselves. The headache increases and becomes intense, especially in the occipital region and down the back of the neck. The temperature steadily rises to 100 degrees or 102 degrees, and the pulse becomes rapid and irregular. Both run a varied and irregular course. With the pain in the back of the neck, the mus- cles become rigid ana there is marked nucha! retraction. This has been so extreme that the head hung back between the shoulder blades. The spine gradually becomes arched so that there is opisthotonos. Orthotonos is probably more frequent than opisthotonos. Pains and rigidity, severe in type, appear in the limbs. Every touch to the patient, every effort to move him causes him to cry out with the agony. He writhes, his face becomes contracted and he shows in an unmistakable manner that he is in extreme pain. Trismus, abdominal retraction, strabismus and a general muscular rigidity so that the pa- tient can be lifted like a statue, are all present. The maxillary, spinal THE NON-NEURONIC DISEASES 8ll truncal and leg and arm muscles are all involved and reveal, with the tenderness and shooting pains, without ever passing into paralysis, the .typical picture of cerebral and spinal meningitis with implication of the cranial and spinal nerve roots. The sensorium for a time is clear but soon delirium and a clouding of the faculties add themselves to - the complexus of manifestations. The cephalalgia continues, the fever exacerbates and a wild and maniacal delirium seizes the patient. He raves and strikes out and becomes a perfect fury. The delirium is a gradual and early manifestation. In the beginning, it is associated with violent erotic Or sexual excitement. In a few days it exhausts itself and sinks rapidly into the stupor and comatose condition. Just before death, which occurs in these cases in from ten days to a couple of weeks, the fever, which has been very erratic, suddenly rises to 106 degrees or 108 degrees, the pulse becomes small, feeble and irregular, the respirations grow labored and assume the Cheyne- Stokes type or sighing characteristic, and the coma becomes profound and complete. Among the other symptoms are to be noted the coated tongue, foul breath and constipation. There is retention and incontinence of urine. Some albuminuria is present ; sometimes glycosuria. The urine at first is typically a febrile urine but later it is apt to be exces- sive in amount. The spleen is almost constantly enlarged, though on account of the pain cannot generally be palpated. Later manifestations are of a paralytic and trophic character. The name of the disease, spotted fever, is given on account of the marked cutaneous symptoms. They are not a constant feature of the disease, however. The skin may be merely erythematous or mottled. Rose spots, as in typhoid fever, sometimes are present ; at other times only petechias and scattered purple spots are to be seen. Herpes occurs more frequently than it does in pneumonia or intermittent fever. Other •cutaneous manifestations that have been noted are erythema nodosum, ■ecthyma, urticaria and pemphigus. Gangrene of the skin has been a r are observation. Other complications of the meningitis are conjunc- tivitis, a neuroparalytic keratitis, chemosis, mydriasis, optic neuritis, strabismus, facial paresis and deafness. The implication of the cra- nial nerves, of course, explains these symptoms. Purulent irido- choroiditis is a particularly frequent trouble, and is in all probability the result of metastasis. Amaurosis is its ultimate result. Aphasia has "been seen. General convulsions are not common except in children. Hemi- plegia and monoplegia may be present but hidden by the clouded con- sciousness. Paraplegia, loss of patella reflex ( increased usually at first), paralysis of the sphincter vesicae and girdle pains indicate the disease process in the cord. Bedsores, joint troubles and extreme emaciation occur in the protracted cases. As these symptoms are not always the same in all cases, as not all cases are fatal by any means, and as the disease runs a course any- where from a few hours to several months, clinicians have established the following types : I. The Malignant or Fulminant Form. — This may appear epi- demically or sporadically. The disease seems to burst out all at once. 8l2 THE NON-NEURONIC DISEASES The chills, headache, fever, vomiting, delirium, somnolence, convul- sions, spasms, rigidity and terminal coma appear suddenly and run their course in a few hours. The onset, the development and the termi- nation are swift and terrible. In one case the whole gamut of symp- toms from start to finish was run in five hours. Other cases have died in twenty-four hours or less. Truly may these cases be styled apo- plectic. 2. The Ordinary Form. — This has been described. 3. The Rudimentary or Abortive Form. — These cases are very confusing when they occur sporadically, though during an epidemic they may be correctly recognized. The symptoms are all mild and in- definite. The headache and backache are slight. Possibly there is some restlessness, irritability, insomnia or dullness. There may be nausea. The nuchal muscles may seem a little stiff. Fever is slight or absents Two classes of these cases are recognized. In one the symptoms be- gin abruptly and with considerable severity, but in a few days they die out or subside to almost nothing. In the other they are never more than slight and the cases are entirely ambulatory. 4. Intermittent Form. — These are protracted cases, often run- ning many months with complete intermissions or marked remissions in all of their symptoms. They may even appear as quotidian, tertian and other types. Some cases run a steady, severe course at first, and then pass into convalescence as an intermittent disease. Pyaemia rather than malaria is the suggestive feature about the fever. 5. Chronic Form. — This is frequent according to Heubner. With a very variable symptomatology, these cases run along, now up,, now down, but never intermittent, for three and six months. Intense marasmus usually supervenes. As Osier says, it is not improbable that hydrocephalus or intracranial abscess is at the bottom of these. Diagnosis. — The diagnosis of epidemic cerebrospinal meningitis offers the greatest difficulties only when the case is a sporadic one, or in those cases that now and then continue- to break out in a locality where the epidemic has prevailed. If the complete picture of the dis- ease is kept well in mind, the general septic febrile process, the charac- teristic cerebral and spinal meningitic manifestations, the marked cutaneous exhibitions and the frequent complications, such as pleurisy, broncho-pneumonia, pericarditis, parotitis and implication of the artic- ular joints, I think one will have little trouble in making a diagnosis, even though all of these indications are not present at the same time. The history of the case must be always given due weight. Perchance the patient has had the disease before, or there has been a recent epi- demic in the vicinity. If the epidemic is raging at the time, there is. no doubt about the diagnosis whatever. Sporadic cases are hard to distinguish sometimes, especially when protracted and not very violent, from tuberculous meningitis. Tuber- culosis elsewhere or in the family is suggestive of the latter. More- over the spinal symptoms are not so prominent, whereas the mental are slightly more so in the tuberculous disease of the membranes. The herpes and other skin, manifestations, the enlarged spleen, the com- plications, speak for the epidemic disease, or at least they are not the THE NON-NEURONIC DISEASES 813 same as they are in tuberculous infection. Lumbar puncture and the finding of the specific micrococci or bacilli offers the crucial test of .the true nature of the trouble. Atypical forms of typhoid fever and cerebrospinal meningitis may oe confounded with each other. In the former the temperature runs .a different course, there are loose stools with tympanites and an ab- sence of the herpes, joint troubles and characteristically combined cere- bral and spinal meningitis manifestations. Here again lumbar punc- ture comes to our rescue. Pneumococcus meningitis, which may occur either alone or as an accompaniment of pneumonia, is sometimes very like the epidemic form. According to Leichtenstern, with the former the contraction of the muscles of the neck is often wanting, while in the latter it is inva- riably present. The former quickly leads to delirium and coma, whereas in the latter the sensorium is clear for a long time or even throughout the entire course of the disease. The former is rapidly fatal, the latter Is frequently recovered from. In the former, symptoms pointing to -a wide extension of the meningeal infection of the brain and cord, in- volving the spinal and cranial nerve roots, are less pronounced or even absent as compared with the same symptoms in the latter. Lumbar puncture may settle the question to a large extent by discovering the pneumococcus in the one case or the diplococcus intracellularis in the other. Epidemic cerebrospinal meningitis has some imitations in small- pox, tetanus, uraemic coma, acute articular rheumatism and hysteria. A careful consideration of the history, a thorough examination of the patient and the recollection of the clinical picture, in to to, of the spe- cific meningitis will enable one to avoid error in the differential diag- nosis. Prognosis. — This is always serious but not necessarily bad. In epidemics the mortality ranges from twenty to seventy-five per cent. In children it is more fatal than in adults. Early in the epidemics severe cases predominate, later on the mild cases are in the majority. Very rapid or very protracted cases are not hopeful. Mild cases that run a medium course offer the best outlook. They often get well, though usually leaving some defect or complication, such as blind- ness or deafness, which are apt to be permanent if they show no signs of receding in the first three months after the outbreak of the dis- ease. Deep coma, repeated convulsions and high fever are bad omens. Existing complications such as pneumonia, endocarditis, and pericar- ditis add to the gravity of the prognosis. Fulminant cases destroy t In- patient in a few hours or a day or two. Ordinary cases run an irregu- lar course, as a rule, of two or three weeks. Protracted cases may continue for many weeks. Chronic cases may not be free from signs of the disease for five or six months. Sequelae are unfortunately com- mon. Death is caused by exhaustion, decubitus, marasmus in chronic cases; by the intense intoxication with asphyxia and annihilation of all the vital processes in the acute and fulminant cases. Treatment. — There is no specific remedy for the disease. After placing him in the best hygienic environment, the most we can do for 814 THE NON-NEURONIC DISEASES the patient is to sustain his strength, ameliorate his sufferings and com- bat some of the symptoms. Opium usually has to be given freely. Morphia administered hypodermically is the best. Mercury, iodide of potassium, salicylate of sodium, ergot, belladonna, calabar bean, quinine in large doses, digitalis, benzoate of sodium, chloral, potassium bromide and alcohol are some of the remedies that have been recommended for both the disease in general and to meet special indications. The long and varied list of them, and the continued high mortality of the disease, demonstrate how futile is practically all of our therapy in re- lation to it. In regard to bloodletting, hydrotherapy, counter-irritation and the application of cold to the head and spine, the same principles are ap- plicable here that I suggested under the head of acute purulent menin- gitis. Hot baths have lately been very favorably recommended, as also- has lumbar puncture. Plain, nutritious diet, of milk and strong broths especially, must be given during the fever. The stomach tube may have to be em- ployed if swallowing is difficult. Stimulants are needed regularly in most cases. The residual symptoms call for no special treatment. Tuberculous Meningitis. — This is a form of meningitis that generally runs a subacute course, is due to a specific germ and occurs mostly in children. It used to be spoken of entirely, and is even still sometimes referred to, as acute hydrocephalus, or "water on the brain." Basilar meningitis is another of its more popular synonyms. Though its study really dates from the observations of Robert Wright, of Edinburgh, in 1768, our accurate knowledge of it was laid down by Gerhard, of Philadelphia, in 1832 and 1833. It has a few distinctive features that warrant its discussion in a section by itself, though in the main it is the same in all respects, except etiology, as all the other forms of leptomeningitis. Etiology. — The essential cause of the disease is the tubercle bacillus. This usually enters the circulation from a tuberculous lesion somewhere else in the body, lungs, joints, intestines, and so reaches the base of the brain where it sets up its specific inflammation. It is thus a secondary disease, or infection. There are cases in which both during life and after death no primary focus can be discovered. It is always highly probable that the latter has been overlooked for a com- plete examination grossly and minutely of all the tissues of the body is a very difficult task. Nevertheless it may be true, as believed by some authorities, that in a few instances the disease may be primary in the brain, the bacilli entering the cranium by way of the nose and cribri- form plate of the ethmoid bone. A certain amount of predisposition towards the disease is shown in the fact that it attacks preferably individuals of a certain age. It is frequent in children, rare in adults. It is scarcely ever seen during the first year of life. Most cases occur between two and ten years of age. The majority of those that I have seen, have been about four and six years of age. It occasionally breaks out in early adult years. It is exceedingly infrequent after forty. Every explanation hitherto THE NON-NEURONIC DISEASES 51 5 offered to account for the predominance of the disease in childhood is entirely hypothetical. Some think it is due to the relative rapidity of the vital processes in the growing brain of the child ; others hold that it has relationship to the frequency of alimentary tuberculosis in children. It has frequently been charged to the ingestion of tubercle-infected milk. The eruptive fevers, especially measles, have in a considerable number of my cases immediately preceded its revelation. These may act along with such assigned subsidiary causes, as cranial traumata, ex- cessive emotional excitement, mental strain and alcoholism in dimin- ishing the individual's general power of resistence. The bronchial trou- bles of measles and pertussis may open the gateway for the infected air to scatter the germs into the circulation. Males suffer more than females. Pathology and Pathogenesis. — The disease may appear any- where in the meninges of the brain and spinal cord. In fact many of the cases are cerebrospinal meningitis with the cerebral manifes- tations masking the spinal. The favorite seat of the trouble seems to be the base of the brain, hence the name basilar meningitis. In the vicin- ity of the great vessels found here, the arteries branching off from the anterior middle and posterior cerebrals, the pia is inflamed, granular and covered with an exudate. Around the optic chiasm, in the inter- peduncular space, up along the Sylvian fossae, beneath the medulla oblongata and cerebellum, and even in some cases up on the convexity of the hemispheres and down along the spinal cord, the meninges are thickened, opaque and covered with the gelatinous effusion. There turbidity, matting together and nodular contents are unmistakable. All along the vessels, especially at the points of their bifurcation, or branching, are found the miliary tubercles which caused Guersant in 1827 to use the name granular meningitis. Sometimes these coalesce and form larger tuberculous nodules. In these are found the specific bacilli of tuberculosis. By stripping off the membrane and holding it up before a bright light the tubercles become very evident. They are seen to be imbedded in the exudate and also where there are no signs of inflammation. They are identical in structure with the same sort of tubercles found elsewhere in the body. They may be very numerous or only few and widely scattered. The dura may be invaded by them, especially in the region of the middle meningeal artery. They are sometimes seen in the tunica intima of the arteries in conjunction with an endarteritis and a proliferation of the adventitia. In such cases the calibre of the vessel may be diminished and a thrombosis occur. The choroid plexus is usually included in the infection, and the nodules, with redness and the exudate, are seen scattered along the sheaths of the cranial nerves. The neighboring brain substance is always more or less implicated so that the condition partakes of the nature of a meningo-encephalitis. It is injected, infiltrated with leucocytes, oedenia- tous, softened in spots and punctated with small hemorrhagic foci. Spots of softening from tuberculous arteritis also appear in the basal ganglia, cerebral peduncles and the spinal cord, even in some case- as far as the filum terminale. The ventricles are usually dilated and full of turbid fluid, producing a condition of internal hydrocephalus. The 8l6 THE NON-NEURON JC DISEASES ependyma is softened and diseased. The fornix and septum lucidum are often broken down. As is usually the case in hydrocephalus with ventricular pressure the cerebral convolutions are compressed and flattened and the sulci are partially or completely obliterated. Such is the pathological picture of an ordinary case of tuberculous menin- gitis. There are cases, however, which are swift and fulminant in which not much more than the presence of the tubercles and intense congestion can be discovered. In other cases the disease runs such a prolonged course that a superficial, strata-like exudate with spots of caseation and other evidences of old or chronic inflammation are all that is to be seen. Symptoms. — Clinically tuberculous meningitis assumes a varied guise. It is an infectious disease usually with more or less prominent symptoms of local lesions in other parts of the body as well as in the brain. On this account we usually find the trouble in weak, puny, pale, anaemic, ailing and badly-nourished individuals, who at the same time have a diseased lung, joint, ear or lymph glands. The general debili- tated physique and its functional inadequacies present their own par- ticular symptomatology. The local nidus of the disease adds its quota to the tout ensemble of the clinical picture. In the midst of all this the clinical signs of the meningitis must be traced out ere a diagno- sis of the latter can be assumed. To add still more to the difficulty, the meningitis itself may be a very diffuse or limited lesion, involv- ing large or small areas of functional brain substance ; it may be a rapid and stormy process, or a quiet, progressive inflammation, each with its own variety of symptoms ; and finally it may im- plicate such functionally dissimilar elements, in such a variable way that nothing definite can seem to be made out of the complex, clinical presentation. In' view of all these difficulties it is not surprising that the trouble is so often overlooked or not recog- nized until coma and death are close at hand. The natural hesitancy, born of a desire to avoid, if possible, the final confirmation of so sad and fatal an affliction, clouds the physician's best judgment even when it is struggling in the midst of one pf the most difficult diagnostic prob- lems that medicine sometimes offers. One should approach the diagnosis of tuberculous meningitis therefore carefully, with a clear and unbiased reasoning faculty, and with a perfect mental conception of what a typical case should look- like. For there is a clinical picture of tuberculous meningitis that is sufficiently distinct and uniform to warrant its being described as a disease per se, however surrounded or clouded it may be by other man- ifestations. So clear and distinct is this portraiture that many divide it into three periods, the prodromal, the irritative and the paralytic, and give a special description of each of them. Premonitions of tuberculous meningitis may occur weeks and months before it frankly announces its presence. They are sometimes very marked and in none but a tyro would fail to awaken a strong sus- picion. At other times they are so slight and evanescent that their significance is hardly appreciable until the disease is sufficiently ad- vanced to make a diagnosis a comparatively easy matter. The child's THE NON-NEURONIC DISEASES 817 disposition indicates a change. It is somewhat fretful and irritable and cross. It seems to be subject to spells and paroxysmal outbreaks of crying and worrying. In the intervals it may appear somewhat in- different or unsocial and dull. As these changes often follow an attack of measles or other disease or are associated with some cachectic condition, they are attributed to general ill health. After awhile attacks of headache appear at various intervals and the child complains of the acuteness and darting character of the pain. In one case the parents noted months before an unsteadiness of gait which they attributed to convalescent weakness. In sleep the threatened victim is restless, tosses about, talks, has terrifying dreams and cries out now and then or sud- denly wakens up with a start. As the case advances the headache be- comes excruciating and unnerving. Periodical at first, it is now more or less persistent with intolerable exacerbations. The little patient seizes his head with his two hands and screams with the agony. He instinctively avoids the light and the slightest noise rouses him into a frenzy. A mild delirium now begins to assert itself and with it an un- conquerable insomnia. Unexpected and unaccountable attacks of vom- iting take place. The appetite is lost. Constipation is troublesome. Emaciation shows itself. Occasionally there will be a slight, irregular elevation of the temperature. Many cases seem to glide gradually from the prodromal condi- tion into the easily recognized state of the disease. In other cases, however, perhaps the majority, after a few weeks of these indefinite, transient exhibitions of peevishness, headache, fever and vomiting, the child suddenly becomes violently ill. There is a chill or a convulsion. with violent vomiting, sudden rise of temperature, intense headache and many of the signs that accompany the outbreak of all the infec- tions fevers. Ofttimes over-exertion at play, exposure to the hot sun, or indiscretion in eating seems to precipitate the attack. Unless he is suspicious and guarded, the physician will be very liable to ply reme- dies to correct the supposed indigestion and offer a favorable prognosis. It is a matter of profound chagrin and sorrow to the attendant, as I have several times witnessed, when at the end of ten days or a couple of weeks coma comes on in spite of all his treatment and hopeful prom- ises, and the patient dies while he is forced to admit that if he had only been more studious of the case he might have recognized the pre- monitory signs many weeks before. The temperature rises to 103 degrees. The pulse becomes rapid and then slower. The respirations do not change much. The sen- sorium remains for a while clear and the headache grows more in- tense. A short, sharp, distressing cry, the so-called hydrocephalic cry. is vented now and then. The sleep is broken and fretful, or there may be insomnia. The muscles begin to twitch and start. The pupils arc contracted. Vertigo is complained of. Light and sound are absolutely unbearable. Delirium alternates with somnolence. The teeth are ground together and the head is burrowed into the pillow. This is the so-called stage of irritation. The motor and sensory manifestations of it are striking and characteristic. The hyperesthesia, both of the general and special senses, is all but too obvious. The patient resents 8l8 THE NON-NEURONIC DISEASES every touch upon his skin and muscular tissues. He doesn't want to be moved. He begs simply to be left alone free from contact as much as possible of bedding and clothing. Following the early transitory twitchings, the muscles of the neck, face, back, arms and legs become rigid. Before the contractures become marked the reflexes are notice- ably exaggerated and Kernigs sign is pronounced. Trismus is pres- ent. The back is bowed in opisthotonos. The abdomen is hard and retracted. The arms and legs and hands and feet are all more or less flexed, rigid and contractured. In this stage general or partial con- vulsions may occur or epileptiform attacks, or brief tetanic contrac- tions in the entire musculature of the body. Gradually these symptoms lead into the stage when stupor reveals the exhausted and hydrocephalic conditions of the encephalon. All signs of irritation subside and the patient lies still, dull, apathetic and uncomplaining. He may utter an occasional cry or there may be a slight convulsive wave pass over the body but as a rule all is still. The coma is clearly deepening and the earlier suffering is being replaced bv an appearance of death. The pupils are now dilated and unequal. Vision is obscured, or at least there is no reflex response as in health. The eyeballs are rolled from one side to the other. Strabismus is pres- ent ; sometimes a condition not unlike nystagmus. In a little while signs of optic neuritis or choked disc may appear at the fundus. Oc- casionally a tuberculous choroiditis can be made out. Respirations be- come sighing. The temperature fluctuates widely even from day to day. The pulse usually drops below the normal. The skin is erythe- matous in patches and if rubbed with the finger nail exhibits the un- important tdche cerebrate of the French writers. The bowels are still obstinately constipated and the abdomen is boat-shaped. Gradually from this stage the patient passes into the final or paralytic stage in which paralysis, not complete, however, of the cra- nial nerves and of special groups of muscles, and coma constitute the predominant symptoms. An occasional twitch or convulsion may occur and with a great effort the child may be aroused. As a rule, however, intense coma, immobility with contracture or relaxation and a general collapsed typhoid state is the condition present. After awhile the patient cannot be roused at all. Involuntary evacuations of the bowels and bladder take place. The pulse becomes rapid and feeble. A low delirium may continue. The temperature falls to subnormal, even to 94 or 93 degrees, rising suddenly to 106 or 108 degrees just before death, which occurs usually from two to three weeks after the onset of the malady. In adults the disease may appear abruptly, run a violent course with exaggeration of all the symptoms and terminate in death in a few- days. Children also are occasionally attacked this way. Afore often. however, in children the disease runs a more protracted, irregular course. Even remissions occur that raise false hopes of recovery. In these cases monoplegias, hemiplegias, aphasia, Jacksonian epileptiform phenomena, rigidity followed by temporary relaxation or permanent contractures, tremors, athetoid, cataleptoid and tetanic conditions need not surprise one if they appear. A moment's consideration of the THE NON-NEURONIC DISEASES 819 lesion and its location, the thickenings, the arteritis, the hemorrhages, thromboses and foci of softening in and about the area supplied by the middle cerebral artery will explain them. Diagnosis. — In a frank case, the diagnosis of tuberculous menin- gitis is not a matter of great difficulty. The discovery of a tuberculous focus somewhere else in the body, the prodromal history, the onset, course and character of the symptoms present, if carefully analyzed and properly valued in their various relationships, will afford an outline picture that can easily be recognized. And yet, as I have said before, sometimes the diagnosis is a most difficult matter on account of the irregularity of the symptomatology of the meningitic trouble, and espe- cially on account of the masking of the case by the symptoms of the complications that are often present. Lumbar puncture is usually con- clusive even though the bacilli are not always recovered. The menin- gitic character of the fluid, the evidence of leucocytosis, its ready coagulability, its high pressure, its cloudy, purulent appearance though it may be clear, are all confirmatory when the symptoms point in the least toward tuberculosis. The trismus in some cases may cause them to be mistaken for tetanus. The history of an injury determines the latter, however. Typhoid fever and other infectious diseases, gastrointestinal catarrh, anaemia, hemicrania may be confused with the early stages of menin- gitis. Care in eliciting the minute history of the case and in the personal examination of the patient will forestall error. The same may be said of general miliary tuberculosis which may or may not have men- ingitis associated with it. Sometimes, when meningitis seems to be the only affection present, its tuberculous nature can be suspected when the prodromal stage is prolonged and preceded even by weeks and months of transient premonitions, when the temperature is moderately raised, when its entire course lacks violence and great severity, when tuberculous choroiditis can be discovered and when the disease occurs in a child. Prognosis. — This is decidedly bad. The reported recoveries have probably been cases that were erroneously diagnosed, in spite of the fact that a few such cases have been put upon record by reliable author- ities. I have never seen a recovery in a positive case of the disease. Freyhan, according to Oppenheim, had a case recover even after the tubercle bacilli had been secured from the cerebrospinal fluid. The possible contingencies surrounding such a case are so numerous, that in view of the almost constant fatality of the disease, one may be cused for retaining a doubt in regard to it. The disease runs a more typical course in children than it does in adults. Tt may be latent or be quite over-shadowed by the primary trouble for a long time. \> a local meningitis of the vertex, it may cause only aphasia, hemiplegia, Jacksonian manifestations, merely to become generalized years later. Tt is believed that some of the so-called aborted cases are due to lulu r cular intoxication of the brain rather than a true meningitis. Treatment. — Prophylaxis is important. Tuberculous foci should always be removed from other parts of the body, surgically or other- 320 THE NOX- NEURONIC DISEASES wise, if possible. A tuberculous child should be treated on general principles with fresh air, good food, nutritious diet, careful nursing, cod liver oil, and tonics. The administration of large doses of potassium iodide (600 grains a day in one case that recovered) and symptomatic treatment bounds our therapy in this intractable disease. Surgical measures such as ventricular tapping as a palliative, tre- phining and subarachnoid drainage and lumbar puncture as radical, have been tried with a modicum of success. Afore experience will be needed before final judgment can be passed upon the operative treat- ment of tuberculous meningitis. Chronic Meningitis. — Except as a syphilitic product, chronic meningitis, is rare, much more so clinically than pathologically, though in general practice it is often diagnosed in headache, after isolation and following injuries to the head. It may occur at the vertex. On the autopsy table it is more often seen at the base. It plays a role in the production of intense hydrocephalus by reason of the adhesions it causes and the closure of the interventricular openings. Its symptom- atology is usually slight? or nil. The symptoms often attributed to it are mostly due to compression, congestion and other disturbances of the brain substance, especially the cortex. In alcoholic subjects there is a condition that is sometimes spoken of as serous meningitis. Inflammation is not present, however. The headache, slight delirium, mental failure and tendency to optic neuritis are due to congestion merely. Intoxication of the brain elements is also probably responsible in part for the symptoms. Multiple neuritis and hepatic engorgement often complicate these cases. Chronic syphilitic meningitis has been spoken of under the head of syphilis of the central nervous system. There are many forms of obscure basal meningitis in children as well as in adults, which run an irregular course and are probably due to inherited syphilis. Chronic ossifying meningitis is a pathological curiosity that has been seen in some old cases of persistent headache, epilepsy and in- sanity. There is a chronic infantile meningitis, the non-tuberculous pos- terior meningitis of infants that is of considerable interest. It is some- times associated with syphilis and may cause a severe hydrocephalus. Its chief symptom is retraction of the head, so that it has even been called the "cervical opisthotonos of infants." The posterior fossa of the skull is its choice location. Sometimes its onset is sudden ; at other times it is gradual. Fever, vomiting, rigidity of the limbs and convulsions have all been pointed out in connection with it by Gee and Barlow. The retraction of the head may be intermittent. Palsy is rare. Sometimes there are strabismus and nystagmus. The head be- comes hydrocephalic in size- after a time in some cases. Recovery or death may take place in from a month to a year or a year and a half. Sometimes weeks before death, the child passes into a state n\ stupor or coma. Antisyphilitic treatment should always be given a trial in these cases. THE XOX-XEUROXIC DISEASES 82 1 CIRCULATORY DISEASES OF THE BRAIN. From clinical as well as experimental observation we know that the brain is very susceptible of the slightest change in its circulation. Just how this is we are not always able to determine. Its functional integrity is clearly dependent upon the quality and quantity of blood flowing through it, upon the blood pressure, and upon the velocity of the flow. The relative importance of these various factors is still un- known to us. In the older pathology mere blood pressure and quan- tity of blood constituted a basis whereon were erected elaborate clin- ical descriptions and symptomatic differentiations. It is probable that in an organ so highly vascularized as the brain is and with such a rich anastomosis between all parts of its main circulatory apparatus, the changes of pressure and amount of blood would have to be compara- tively gross to effect marked changes in the cerebral symptomatology. As a sort of reaction to this coarse and largely hypothetical clinico- pathology of the past, it is now denied by many that any changes, capable of being observed at the bedside, take place in the mere pres- sure of the blood or the quantity circulating through the brain. This is perhaps an extreme in the opposite direction, though it is a whole- some protest to the many fine focal and generalized distinctions that used to be made in the symptomatology erected upon the old hypo- thetical pathology. There are symptomatic manifestations, however, that are clearly due to circulatory disturbances. In accounting for these, modern pathology lays the greater stress upon the quality of the blood and the velocity of its flow in the capillaries. The composition of the blood we know is subject to great variations. Its corpuscular richness, its chemical and bacteriological ingredients, its endogenous and exogenous contents, its gases, its specific gravity and physical state are all finer factors in influencing the delicate activities of the cerebral neurones, it would seem, than are the mere pressure and quantity of the fluid. Moreover the rapidity of flow depending largely upon the tension of the vascular walls, and causing a correspondingly swift removal of deleterious products of metabolism and conveyance to the nerve cells of stimulating, nutritious pabulum would also appear to be a more in- fluential factor. These facts are supported by experimental research and dovetail better with what we know nowadays of cerebral physiol- ogy and pathology than the old theories based upon mere hyperemia and anaemia. They are founded upon better proofs than the mere post mortem macroscopic appearances of the brain. The latter, it is now recognized, are to a large extent due to such post mortem influ- ences as the position of the corpse, pressure about the neck, tin* examination, etc. Ante mortem appearances of the face and skin have too long been adopted as arguments for a similar condition in the intracranial tis- sues. There is no assurety that they are the same, and some facts even intimate that they rarely are. Facial pallor during life and cerebral pallor after death, do not by any means prove that the brain was in 822 THE NON-NEURON IC DISEASES like condition while the patient was alive and manifesting the symp- toms supposed to de due to them. Notwithstanding our symptomatology of cerebral anaemia and hyperemia is based upon clinical and experimental observations, fur- ther studies are needed to determine just how much is due to each of the respective factors that always more or less accompany these circu- latory conditions, namely, the quantity of the bipod, its quality, the pressure of its column, and the swiftness of its flow, yntil we learn more about these, we will still have to treat of the clinical manifesta- tions under the two general heads of cerebral anosmia and cerebral- hypercemia. CEREBRAL ANAEMIA. It is perfectly reasonable to suppose that under certain conditions the quantity of blood in the brain may be diminished, even consider- ably, below the normal. What is the normal for any one particular individual and how far below the normal can the quantity of intra- cranial blood of that individual be reduced before the brain manifests it outwardly? Who can tell? Hence when we outline a symptoma- tology for cerebral anaemia, it must not be imagined that we attribute the symptoms entirely to the mere want of blood. Other unknown fac- tors are undoubtedly at work, such as, for instance, the nutritive pov- erty of the blood, its altered composition, the shock caused by the sud- den disturbance in its flow and the diminished pressure in some cases in the blood-vessels. Etiology. — So far as we know, cerebral anaemia is both a local and general condition and may be dependent upon both heal and gen- eral causes. An embolus, for instance, causes a local anaemia, followed by other changes in the brain ; a cachetic state of the constitution may be accompanied by deficient and bad blood supply. The anaemia may therefore be primary or secondary. The immediate and remote causes of the condition are therefore innumerable and I can only mention a few by way of illustration. Severe hemorrhage in some other part of the body, or even in the brain itself, may provoke a symptomatic anaemia. The coma of hemorrhagic apoplexy is believed to be due partly to the sudden anaemia of the cortex caused by the local loss of blood and certainly in sud- den and profound hemorrhages from the lungs, stomach, bowels, uterus, syncope and other signs of severe cerebral anaemia are often observed. The sudden withdrawal of ascitic fluid, paracentesis of the abdomen, precipitate parturition, abrupt disturbance of the heart ac- tion from valvular or other form of disease, violent cupping, nervous collapse as seen in simple swooning, may suddenly diminish the amount of blood flowing to the brain. The same condition as a continuous one follows probably the exhaustive febrile diseases, cardiac weak- ness, partial obstruction by tumor, aneurism and diseased blood vessels, and hydrocephalic conditions. Pressure upon or ligature of the carotid produces signs of transient cerebral anaemia. Vasomotor spasm and anaemia have been blamed for certain forms of migrainous headache and epileptiform phenomena. It is thus seen THE NON-NEURONIC DISEASES 823 that a great variety of causes, mechanical and physiological, sudden and gradual, may be blamed for the intracranial disturbance. Let me repeat, however, that simply the diminution alone in the intracranial quantity of blood is not to be regarded as the whole of the pathology of the trouble, or as the sole cause of its symptomatic exhibitions. Pathology and Pathogenesis. — Macroscopically the brain looks pale and whitish. There is a noticeable deficiency in the number of puncta vasculosa in the white substance. The gray matter looks whiter than it should and its vascularity seems diminished. The membranes are pale and there is an effusion of serum in the pia mater. Both inside and outside of the ventricles the cerebrospinal fluid is slightly in- creased. Paleness and excessive moisture are the chief gross appear- ances. Microscopically the nerve cells are said to be changed, are un- duly translucent or granular ; while the vascular walls are somewhat degenerated and thickened in some cases. These appearances may be general throughout the brain or local, according to the nature of the primary cause. Symptoms. — These are practically the same for all cases, though in detail they will vary widely, according to whether the anaemia is general or local, primary or secondary, slight or severe. " A simple attack of syncope illustrates well the main features of the clinical picture. The patient, suddenly or gradually, experiences a sinking sensation, his head gets light, spots float before his eyes or his vision gets clouded, he feels dizzy and sud- denly weak, his heart seems to flutter, his breathing is somewhat sigh- ing and heavy, his ears buzz, he is nauseated and may even vomit. He falls or is quickly placed upon a chair or couch. His eyes close and his consciousness vanishes. In a few moments he comes to, is dazed or slightly delirious, manifests apathy and a desire for sleep. The pupils are contracted though later on they may be dilated. In severer cases, such as follow sudden loss of blood, the symptoms are more swift in onset and more profound. There is a sudden flash of light before the eyes, followed by darkness, a quick loss of consciousness, vomiting and falling. The pulse is weak and hurried; the respirations are labored, shallow and sighing; the skin is moist and cool, and the pupils are dilated. The reflexes arc all annulled, the pulse grows slower and feebler and in some cases the patient dies in a convulsion. In other cases spasms pass over the body and the patient awakens momentarily delirious and complaining of severe headache. Some- times the consciousness vanishes only when the patient attempts to sit up, being present as long as he keeps the recumbent position. \ had a case of profound neurasthenic anaemia in a woman who while perfectly flat on the back swooned away every few moments. Some of the severe hemorrhagic cases may continue from several minutes only to an hour. They are dangerously near death and one cannot at times foretell the outcome. In general chronic anaemia there is a general sluggishness of the cerebral processes with a tendency to functional vagaries, paresthesia and even hallucinations. Irritable weakness of the nerve elements Is the nearest we can come to an explanation of the pathogenetic phe- 824 THE NON-NEURONIC DISEASES nomena. These patients are drowsy, indifferent, lack initiative force and staying qualities. Their memories are weak, the volition lacks vigor, the imagination and intellect will only work under a vigorous spurring. They are prone to sleep and yawn and are regarded as splen- did illustrations of laziness. Everything but to sit and read, is an effort to them, especially all mental exertion. Sometimes they arouse into tremendous irritability and excitement over trivialities and com- plain of every noise and unusual ray of light. They are decidedly neurasthenic. Heaviness of the head, insomnia at night, occasional spells of syncope, mistiness before the eyes and vertigo upon every sud- den movement, all relieved by lying down, are the lot of these suffer- ers of anaemia from chlorosis, frequent hemorrhages, pernicious anaemia and similar causes. Marshall Hall has applied the adjective hydrocephaloid or hydroen- cephaloid to a type of cerebral anaemia seen in children with prolonged diarrhoea. The patient lies in a semicomatose condition with eyes partially open, pupils contracted, fontanelle depressed, with symptoms later on resembling those of basilar meningitis, such as dilatation of the pupils, convulsions, strabismus, retraction of the head and death in profound coma. Anaemia is probably at the bottom of the cases in which delirium is associated with marasmus and extreme inanition. In the cases which in the past have been spoken of as oedema of the brain, anaemia is in all likelihood the fundamental pathological basis of the symptoms. As a part of the anaemic state, malnutrition and intoxication, as I have already intimated, may play the chief role. Diagnosis. — Is easy when all attendant circumstances are taken into account. The immediate cause, the underlying cachexia, the char- acter and distribution of the general symptoms in general anaemia, and focal symptoms in local anaemia, and the absence of all other signs of organic disease of the brain will form the basis of a correct diagnosis. Congestion of the brain may in a few instances simulate anaemia, and organic disease may have anaemia as a part of its pathological com- plexus. These, however, with care are easily differentiated. Prognosis. — This is generally favorable in mild cases. Cases due to heart lesions, exhaustive hemorrhages and other grave condi- tions, with coma, are dangerous and too often fatal. Serious signs of trouble are convulsions, pupillary dilatation and light iridoplegia. The hydrocephaloid cases of children are usually favorable under prompt and proper treatment. Visual disorders sometimes remain after severe anaemic spells. Treatment. — First and foremost is to force the blood back into the brain as quickly and judiciously as possible. In acute cases the patient should be placed with the head downwards. In chronic cases, I usually always have them occupy a bed with the toot of it slightly elevated on bricks. In the acute cases the limbs may be slightly bound or an Esmarch bandage may be temporarily applied. Transfusion may have to be employed in some cases. The cause of and character of the attack must always be taken into account. If the heart has sud- denly failed, proper stimulants in the way of alcohol, ammonia salts. THE NON-NEURONIC DISEASES 825 aromatic spirits of ammonia, must be administered. If there is a vasomotor spasm, amyl nitrite and nitroglycerin are called for. A few drops of the former may be inhaled from a handkerchief, while the latter is to be given hypodermically. They should not, of course, be given together. Cold and heat to the skin, tickling, faradization, sinapisms,- am- monia inhalations may do in most cases of simple syncope. Artificial respiration may be required in some severe cases. The general management of the cases involves the treatment of the primary disease and the improvement of the general anaemic con- dition. The focal anaemias are referred to under the head of cerebral softening or encephalomalacia. CEREBRAL HYPEREMIA. The statements previously made in regard to the possibility and frequency of circulatory disturbances in the brain apply to hyperemia as well as to anaemia. In the past too much was assumed and too definite a description of the symptomatology of a supposed cerebral congestion was promulgated. Actual facts were wanting and theories ran riot. On the other hand to-day in some quarters there is too strenuous a denial that cerebral congestion ever occurs or presents dis- tinctive symptoms. Both logic and data proclaim that the condition does sometimes occur and that certain special manifestations are the direct outgrowth of the condition. We can therefore reasonably oc- cupy a middle ground and wait for further observations to clear up many things that are still unaccountable. The essential fact in cerebral hyperaemia is, of course, the exces- sive amount of blood in the cerebral vessels. This tells, however, only a part of the story. Much is still within the dark in regard to the mechanism of the production of this congestion and in regard to asso- ciated conditions upon which the symptoms may depend for aught we know, such as the composition of the blood, the speed of its flow, the influence of its altered pressure, etc. Changes of quantity alone are gross and unsatisfactory grounds whereon to explain the delicate changes of nervous activity. It has been declared that there is a permanent form of cerebral congestion caused by continuous mental exertion, by certain constitu- tional plethora and by some other conditions. This is very question- able, however, and even were the possibility and probability of such to be granted, it would still be hard to say what in any particular indi- vidual should be the normal quantity of the blood in the encephalon and at what point it ceases to be normal and becomes a! (normal or true hyperaemia. Clinically we are much more concerned about the tem- porary or transient cerebral congestions, as they at least exhibit lines of demarkation, coarse to be sure at times, whereby we can distinguish the normal from the abnormal. Temporary hyperaemia of the brain may be active and arterial going topsy-turvy. It usually lasts from a few minutes to a few hours. The restoration to consciousness is more or less rapid and complete, though a state of somnolence may follow for some time. The coma is believed to be due to the sudden disturbance of the circulation, 832 THE NON-NEURONIC DISEASES (anaemia) and to the molecular concussion throughout the entire brain, provoked by the abrupt outpouring of the blood. The paralysis, like the coma, varies in different patients according to the extent, location and rapidity of the hemorrhage. It is always of the hemiplegic or facio-brachio-crural type and is on the opposite side of the body from that of the hemorrhage. It involves most the finer complicated movements, hence the hand and arm are more affected than the foot and leg, and both extremities more than the face. Mus- cles that have a bilateral representation in the brain and work on both sides of the body in unison are but little affected ; hence the respiratory muscles, those of the abdomen and of the eyes soon recover from the first shock. Dysarthria may occur from the implication of the finer movements of the muscles of articulation. This must be carefully dis- tinguished from the aphasia that occurs in right-sided hemiplegia. The lower part of the face is always more involved than the upper, a differential indication of facial palsy of central origin. The face is asymmetrical, the corner of the mouth droops, the naso-labial crease is obliterated and the saliva oozes out on the paralyzed side. Deglutition is difficult. The laryngeal muscles are not particularly involved. With the onset of the coma the temperature usually falls one or two degrees. In a few hours it rises to normal or even passes the nor- mal point. On the paralyzed side the temperature is usually higher than on the other side. If the temperature remains subnormal, or, on the other hand, if it takes a rapid rise to 102 degrees or 103 degrees F., the coma continuing, death is near at hand. Just before the fatal issue the temperature may sink again. Vomiting occurs in cerebellar hemorrhage, but very rarely in cerebral hemorrhage. The conjugate deviation of the head and eyes in the majority of cases is toward the side of the hemorrhage. In other words, the patient seems to stare in the direction of the lesion. In exceptional cases, es- pecially when there are unilateral convulsions or spasms, or when early rigidity occurs, the conjugate deviation of the head and eyes may be away from the lesion and toward the paralyzed side, or more accurate- ly, convulsed side. As a rule this symptom disappears with the wear- ing away of the coma. For the first day or so the amount of urine passed may be larger than normal and it may contain albumin and sugar. The sequence of events in and immediately after the stage of seiz- ure varies considerably in different individuals and depends upon the extent and severity of the hemorrhage. In a few rapidly fatal cases, especially when the blood has broken into the ventricles, the patient fails to emerge from the coma, the respiration assumes the Cheyne- Stokes character, the temperature ascends to 103 degrees F. or higher. the pulse beats violently and the patient dies in a day or two with hypostatic pneumonia. In another class of cases, fatal but more tardily so, consciousness returns partially, mild delirium occurs, with head- ache, restlessness and anxiety. With the reaction period setting in in about forty-eight hours and continuing for a week or more, the tem- perature, after remaining normal for a time, rises, and pneumonia THE NON-NEURONIC DISEASES 833 develops and kills the patient in a state of unconsciousness. These cases may last even as long as two months. Early rigidity develops in their paralyzed limbs, serious trophic changes occur, such as sloughing and vesicular formations, and perhaps the congestion at the bases of the lungs. In the favorable cases, which happily constitute the larger number, the initial coma disappears in a few hours, leaving the mind entirely unaffected, or at the worst only weak and confused. The deviation of the eyes and the temporary ptosis pass away with the coma, the sound side of the body resumes its proper activity, the paralyzed side shows a slight tendency toward rigidity with return or even slight increase of the deep reflexes, the temperature, the pupils and respirations return to their normal condition. A slight febrile reaction with some temporary constitutional disturbance accompanying the in- flammatory changes in the apoplectic area, and the absorption of the blood may occur in a week or so and then pass away, leaving as the prominent and almost sole constituent of the clinical picture the hemi- plegia with aphasia when it is on the right side in right-handed individ- uals, or without aphasia when it is on the left side. Complete hemiplegia is of the facio-brachio-crural type and in- volves the arm more than the leg, and the leg more than the face. In partial hemiplegia any one of these members may be the seat of the paralysis. So dominant and all-engrossing is this one symptom of this so-called chronic stage of the disease, that the patient is in common parlance referred to as a "hemiplegic." He enters upon this role usually about a month after the stroke and when all of the other symp- toms have vanished or diminished to such a degree as to be quite un- noticeable. It is usually stated that the ends of the extremities are more dis- tinctly affected with paralysis* than are the segments nearer the trunk. The difference is only apparent, however, because in this as in all cerebral palsies the more the movements are complicated and the more they are related to the higher intelligence normally, the more they are affected when paralyzed. The loss of power moreover involves seg- ments of the body rather than individual muscles ; movements rather than special anatomical structures merely. Movements that normally depend upon muscles that act bilaterally, such as those of respiration, phonation and facial expression, are but slightly, if at all, affected. Each side here is probably better repre- sented bilaterally in the brain than are the movements of the arms and legs. That there is some bilateral representation of the latter in the cortex is shown by their tendency to act together in untrained nor- mal individuals, and by the fact that even in true hemiplegia some weakness is observed on the sound side. The movements of the lower part of the face, as in all central facial palsies, are more involved than those of the upper part. The frontal muscles and the orbiculares palpe- brarum continue to act, while the cheek, the side of the nose and the corner of the mouth are almost or quite immovable. The tongue is pro- truded towards the paralyzed side because the involvement of the mus- cle on that side of it leaves the muscle of the other side without its nor- 834 THE NON-NEURON IC DISEASES mal opposition. The soft palate may be slightly pendant, but the mus- cles of deglutition are not seriously involved. On account of the paralysis of the tongue, lips, cheek, etc., there is a true paralytic dysarthria, which must not be confused with the disturbance of speech known as aphasia. I have seen such an error made. In aphasia wrong words or no words at all are uttered ; the few that may be employed are not affected in any way by articulation ; the trouble is one of psychic origin rather than of mere muscular in- efficiency. On the other hand, in paralytic dysarthria there is no lack of words and speech, no utterance of wrong words, but rather a bad and inefficient articulation of them. The trapezius is liable to be included in the paralysis. Certain automatic movements, like crying and laughing, are usually performed when the face cannot be moved voluntarily. The paralysis in the limbs may be partial or complete. In the for- mer condition a slight degree of activity in certain directions is re- tained. The fingers in a few days or weeks can be slowly and partially flexed ; the arm can be slightly bent at the elbow ; and the whole extremity can be drawn over towards the body. The movements are all feeble and sluggish, however, and performed clearly with a heroic effort. The flexors, as a rule, show more of a tendency to recover than do the extensors. A great number of variations obtain in regard to the distribution of the paralysis. As a rule the leg recovers much more than the arm and nearly always so much that walking after a fashion is possible. In many cases only the face and arm remain paralyzed. In others the face recovers and the arm remains paralyzed more than the leg. It is very rare for the face and arm to escape or recover while the leg alone remains involved. In mild cases many kinds of coarse work can be done with the hemiplegic hand and arm ; usually the finer kinds of work requiring nice adjustment and coordination, such as writing, playing upon musical instruments, etc., are impossible or extremely difficult. It is rare for these finer movements ever to become possible again unless the case has been unusually mild and a complete recovery obtained. The inability to write must be carefully examined in some cases so as not to be confused with agraphia. The distinction is usually easily made and demands only a little ingenuity in applying the proper tests. The gait of the hemiplegic is characteristic and is to be carefully differentiated from that of the ataxic and spastic paralytic. It depends upon the motor paralysis, the slight rigidity or contracture and the slight weakness and wasting of the muscles. In severe cases it is typical. The support of the body is placed well upon the sound limb; the paralyzed member, slightly rigid and even contractured in old cases, is dragged forward in a sort of a half-circle around the sound member as an axis, the pelvis drawn up a trifle on the affected side and the foot planted forward upon the entire sole without the toes having once been clearly lifted from off the floor. The simple manoeuvre of stepping" sidewise will usually discover the hemiplegic side in those very slight cases, as in some instances of senile hemiplegia, in which the gait is not very obviously altered. THE NON-NEUKONIC DISEASES 835 After the first few weeks the paralysis assumes the spastic type. In rare and unaccountable cases it may remain flaccid throughout. From being absent during the comatose stage., the reflexes become normal and ultimately exaggerated. Even ankle-clonus may be present. Sometimes the exaggeration of the reflexes is bilateial, though it is always more exaggerated on the paralyzed than on the well side. The superficial reflexes usually recover in part, though they are always more or less diminished. The electrical reactions are normal ; and there is no true muscular atrophy, only a slight wasting from non-use. An early rigidity appears in the muscles during the reaction period. A late rigidity with contracture and deformity appears as the accom- paniment of the secondary degenerative sclerosis in the pyramidal tracts. These late contractures affect the hand and arm more than the leg, are somewhat painful and remain permanently. As a result the elbow is partially flexed and the fingers bent. I have seen during the development of this late rigidity violent muscular spasm or cramp with most excruciating pains. In a few instances there has been no rigidity or contracture, but tremor, post-paralytic chorea, athetosis, arthropathy and even muscular atrophy have been observed on the hemiplegic side. The sensory phenomena of cerebral hemorrhage and hemiplegia are not in any way pathognomonic. Hemianesthesia may occur with the paralysis if the posterior part of the internal capsule is injured. This, however, is rarely total. Usually it passes off quickly and leaves only a partial anaesthesia or rather hypsesthesia in various parts of the affected side. Much more common are the various paresthesias, show- ing irritation of the sensory tracts or cortex. Pain is often com- plained of, especially of a burning character, about the articulations. Xumbness is the more common form of paresthesia spoken of. The special senses as a rule escape all involvement. Occasionally, how- ever, hemiopia on the side of the lesion has been noted and the taste, smell and hearing -have been blunted. According to Gowers, hom- onymous hemianopsia of the half-fields opposite the lesion is more frequent than reports would seem to indicate. If the examiner sud- denly brings the finger before the eye in the early stage of the trouble, first from one side and then from the other, it will be noticed that the eyelids blink when the finger comes from the unparalyzed side, but not when it approaches from the other side. Trophic manifestations often show themselves early, within the- first five or six weeks, in severe cases. The joints become inflamed and acute decubitus develops on the paralyzed side of the gluteal re- gion. Sloughing in the lower part of the back, which may come on as early as forty-eight hours after the stroke, is a very unfortunate event. It usually means death. A flushing occurs first, according to Charcot, then a violet discoloration, vesicles, ulceration and finally an eschar. The hypostatic pneumonia which sometimes occurs is be- lieved by some to be a trophic phenomenon. The same may be said of the gastric hemorrhages. Hyperidrosis, cutaneous eruptions and increased growth of hair have been observed in rare instances. I 836 THE NON-NEURONIC DISEASES have often noted the glossy appearance and cool character of the skin in the paralyzed limbs. Rare but interesting are the ataxic hemiplegias. Here the hem- orrhage has confined itself to the area of the sensory tract and the ataxia results from the complete hemianesthesia. The hemiplegia in such instances may be absent entirely or only transient. Hemiataxia, of course, is the true character of the trouble and its one-sidedness readily distinguishes it from all other forms of ataxia. It would be extraordinary if so profound a shock as cerebral hem- orrhage induces did not affect the mind somewhat. The aphasia, early stupor, delirium and somnolence are all partly in the psychic sphere. Irritability, emotionalism, failure of memory are some of the more specific mental disturbances that occur in the older victims of the dis- ease. Marked mental deterioration, irritative epilepsv and terminal insanity have been known to follow cerebral hemorrhage, especially one of large degree. It is to be remembered that mentalization is sub- served by language and that when aphasia is of long duration the want of speech may well react upon the mental faculties and cause them to deteriorate. The mere realization of the fact that the paralysis is per- manent and that the apoplectic danger, as shown by the present attack, hangs like the sword of Damocles constantly over the patient's head, has its influence in depressing the mental forces. The discussion of the symptomatology of other than cerebral forms of intracranial hemorrhage will be taken up when the diseases of the meninges, pons, medulla, etc., are considered. A word should be spoken here, however, in regard to ventricular licmorrhage. As a primary condition it is exceedingly rare. Usually the blood enters the ventricles by extravasation from neighboring parts of the brain or bursts into them suddenly in the course of an ordinary apoplexy. The symptoms are always under such circumstances serious and profound. The coma deepens, or if it had begun to clear up, it abruptly returns and is more profound than it was before. Convulsions sweep over all four extremities, followed by complete paralysis. The muscles are rigid on the hemiplegic side, or in all four members. The pulse is- retarded, respiration is extremely embarrassed, the temperature is de- creased and just before death, which usually takes place inside of twenty-four hours, the eyes become fixed and staring, the pupils dilated and the general appearance of cyanosis and asphyxia occurs. Diagnosis. — The physician is rarely confronted with a more im- portant problem and one that makes larger demands upon his knowl- edge and judgment, than when he is called upon to make a diagnosis in an apoplectic stroke. The proper management of the case depends at first almost entirely upon whether the stroke is due to hemorrhage, embolism or thrombotic obstruction. Moreover, the coma and other symptoms of the first stage are simulated by a number of other dis- eases that are not apoplectic. And finally there are different degrees among the cerebral hemorrhages themselves that must be differen- tiated if possible in order to form some opinion in regard to the prob- able ultimate outcome. While all these questions and distinctions are at times hard to THE NON-NEURONIC DISEASES 837 settle, owing to the multiplicity, variety and great differences in the individual symptoms of cerebral hemorrhage, there is a uniformity and consistency generally about the clinical picture that, when studied in toto, help very materially towards the 'establishment of the diagnosis. Coma and paralysis are the two guiding posts that always point unerr- ingly to a serious intracranial lesion. The approach of, the character of and the associated manifestations surrounding the coma and the paralysis are the chief means by which we must determine the nature of the lesion producing them. In hemorrhage the onset is sudden or with very slight prodromes, and the maximum is attained almost immediately. The coma is usually deep and precedes the paralysis. The latter at first seems to involve all the limbs, but soon recedes from one side. The face, arm and leg of the hemiplegic side are implicated. The paralysis is flaccid and complete from the start and is only rarely preceded by convulsive movements. The pupils, contracted at first, soon become dilated, unequal and irresponsive to light. The flushed countenance, puffing cheek, labored stertorous breathing, rapid pulse, early subnormal temperature and occasional involuntary micturition- and evacuation of the bowels are contributing signs to the completing of the clinical picture of cerebral hemorrhage. The cases of cerebral hemorrhage that develop slowly, taking a day or two for the attainment of their maximum degree of coma and paralysis, may be mistaken for thrombosis, especially in the aged with atheromatous arteries. So common is this error in diagnosis that I am confident the relative proportion of cases of hemorrhage has been greatly exaggerated in the reports. A large number of them have been cases of thrombosis. Admitting the occasional difficulty encountered in making the differentiation, a closer attention to the course of the disease and the changes exhibited by the individual symptoms will render such errors less frequent. There is a group of cases, usually seen for the first time when they are comatose, in which the coma comes on rapidly rather than abruptly and the paralysis is not as profound as in many other cases. ( )r the loss of power develops in the course of a few hours and is followed by a gradual loss of consciousness. These so-called ingraves- cent cases cannot be distinguished usually from cases of embolism or thrombosis unless there are some very suggestive circumstances in connection with the age of the patient, his constitutional diseases and the immediate cause of the stroke. Even then the diagnosis falls short of being positive. The tvpical cases of cerebral hemorrhage that come on abruptly and are in a state of profound coma and paralysis must be differentiat- ed from the coma of uraemia, alcoholism, opium poisoning, diabetes and syncope. Epilepsy and hysteria must be excluded also. In urccmic coma there is no hemiplegia and the pupils are net unequal. The coma comes on gradually or less suddenly than it does in hemorrhage and is usually preceded by blindness, convulsions and vomiting. The temperature is below normal always. A chemical and microscopic examination of the urine and the existence of albuminuric retinitis, with the peculiar physiognomy that accompanies nephritis, 838 THE NON-NEURONIC DISEASES would, of course, determine the diagnosis. In diabetic coma the breath may exhale the odor of acetone. Urinalysis here will also establish the nature of the disease. Severe alcoholism is not infrequently confounded with hem- orrhagic apoplexy, as the records of some of the police stations will amply prove. The odor of the breath cannot be taken as a criterion, for the patient may have felt the apoplexy coming on and taken the stimulant to ward off the sudden weakness or vertigo. In alcoholism the coma is incomplete, the pupils are equal, the limbs show no ten- dency to a hemiplegic condition, the temperature is not lowered and there are some manifestations of delirium. The examination of the contents of the stomach, as the patient usually vomits, will strongly suggest the length of time the alcohol has been ingested. In opium poisoning the pupils are equal and strongly contracted, the respirations are slow and there is no true paralysis. Epileptic attacks are preceded by a cry. The pupils are equal and dilated, the tongue is serrated, the teeth are clenched, and instead of paralysis the muscles are in a state of convulsion. Consciousness returns rapidly, to be followed by headache and drowsiness. Hysteria is so purely psychic that one wonders how the mistake can ever be made between it and the coma of cerebral hemorrhage. The stigmata of hysteria, such as the anaesthesias, will always be dis- covered if sought for in cases of hysterical hemiplegia. Syncope is not characterized by complete coma. It depends upon an impaired heart action and therefore leads one to examine care- fully the pulse, the general hue of the patient and the heart. Moreover, syncope is of short duration and is not accompanied by any paralytic symptoms. In general paresis apoplectiform seizures are not uncommon. The history of preceding mental symptoms, headache, elevation of tem- perature and preservation of the reflexes are usually enough to indicate the presence of encephalitis. Perhaps the greatest difficulty in the differential diagnosis of cerebral hemorrhage is when an attempt is made to distinguish it from an embolic or thrombotic apoplexy. In some cases the differen- tiation is absolutely impossible. The history of the case, its mode of onset and its associated conditions are most important. Cerebral embolism occurs earlier in life than hemorrhage. The age of forty is approximately the period before and after which occur respectively embolism and hem&rrhage. Embolism is usually asso- ciated with heart disease, rheumatism, syphilis or the puerperium. Prodromes sometimes, but rarely, precede the apoplectic stroke. The coma comes on gradually, is less deep than it is in hemorrhage and as a rule follows the appearance of the hemiplegia. The pulse and heart action in embolism are weak, in hemorrhage they are strong and vio- lent. The face is not flushed, the temperature is not disturbed, nor is the coma so prolonged as in hemorrhage. The paralysis comes on more gradually, is less complete, is associated with convulsive movements, and is more rigid and less widely distributed in embolic obstruction THE NON-NEURON1C DISEASES 839 than in hemorrhagic apoplexy. Infarcts in remote organs and repeti- tions of the stroke indicate embolus. It is much more difficult to differentiate hemorrhage from throm- bosis, particularly as both occur in old age and are dependent upon similar diseased states of the blood vessels. Prodromata, often for days and weeks, precede the coma, which is apt to be slight in degree and short of duration. The succession of slight spells of semi-con- sciousness, the irritability, the paraesthesise, the general asthenia, the muscular twitchings of thrombosis I have seen diagnosed and treated as hysterical. In thrombosis there is psychic weakness, hardness of the arteries, evidences of a fatty heart, and absence of stertorous breathings, flushed face, temperature phenomena and convulsive move- ments of large amplitude. Prognosis. — The prognosis of cerebral hemorrhage should al- ways be guarded, especially in the comatose stage and until after the period of febrile reaction. The size of the hemorrhage, its location and the duration of the coma all determine the future outlook. If the coma is profound and prolonged three or four days, if it is initiated with a subnormal temperature or accompanied by an excessive rise of tem- perature, if decubitus or pneumonia appears, or if delirium develops and continues, the outlook is bad. Ingravescent apoplexy with its progressive somnolence passing into deep coma is usually fatal. Signs of nephritis, Cheyne-Stokes breathing and symptoms of rupture into the ventricles are all bad indications. A history of alcohol- ism adds to the gravity of the prognosis. Death under these circum- stances may occur anywhere from a few hours to a few days after the stroke. The larger number of cases of cerebral hemorrhage usually re- cover, in part at least, from their first attack. In a few years, three to five, they have another attack, from which they die or recover with difficulty and more serious residua. Rarely do they have a third or fourth stroke. A favorable prognosis may be held out if the coma clears up early and at the end of the first week there has been no fever or only a slight rise of temperature and none of the ill omens referred to above. Complete recovery is rare, though improvement may continue for two years. A hemiplegia that remains the same for one month can hardly le expected to improve very much. After a year no further change can be hoped for in the great majority of cases. If a return of move- ment shows itself within a few days after the stroke, and continues, the outlook for recovery from the paralysis is particularly bright. Hemiplcgic aphasia dependent upon hemorrhage usually disap- pears sooner or later. Partial aphasia, however, may remain perma- nently. Initial hemianopsia as a rule disappears after a few days, [f it does not it may be looked upon as permanent. The power of walking is acquired at various periods in different cases. In some cases only a few weeks or even days intervene between the stroke and the time when the patient begins moving about. In the 84O THE NON-NEURONIC DISEASES majority of cases, however, walking is not commenced for three or four months after the stroke. The gait at the end of a year is apt to be the permanent one thereafter. Treatment. — As the management of apoplexy, and especially of the hemiplegia that follows it, is in the main the same for all forms, I will treat of it here in detail and merely refer to the differences in the treatment of embolic and thrombotic apoplexy in the next section headed cerebral softening. The dissimilar modes of first handling the patient demanded in cerebral hemorrhage and embolic obstruction enhance a hundred-fold the need of care in diagnosing the nature of the lesion. A misjudg- ment in the arrangement of the position of the patient may mean death instead of life. The administration of a heart stimulant in place of a sedative may bring the case promptly to a fatal issue. An unwise attempt to evacuate a full stomach may provoke a renewal of the hemorrhage. A simple venesection may hasten or retard the end. All these points must be quickly and seriously considered, usually in the presence of the patient, who will be found surrounded by terrified and clamorous friends. In considering the general treatment of apoplexy most authors begin by dividing the subject into the prophylactic treatment and the management of the attack and its sequelae. It had been better, it seems to me, if the emphasis had been laid more upon the treatment of the respective conditions of hemorrhage and embolism. In the main the prophylaxis is about the same for both. A quiet life, both physically and mentally, is a desideratum. The digestive and nutritive organs should be relieved of strain and encour- aged to functionate normally. The emunctories should all be kept active to rid the system of deleterious substances. Sleep should be se- cured. Mental and physical activity should be evenly balanced. In a word, the threatened victim should be induced to lead a life of mild and even tenor, devoid of anxiety and brightened by as much happi- ness as possible. Beyond this little can be offered in the way of advice against an attack of hemorrhage or embolism. It is quite otherwise in regard to the attack itself. Xow the advice becomes of definite and distinct importance. The differential diagnosis must be made as sharply as possible. The suggestions to the nurse and attendants must be precise and not open to the least misconstruc- tion. The patient when first seen will be almost invariably found lying tiat on his back. Position is an important factor, and it behooves one to decide quickly how to place the patient to the best advantage. Gravity plays a role, though a slight one, in the force of the circula- tion ; hence it must be remembered in connection with the nature of the lesion. If the case is one of hemorrhage, a half-reclining position on one or the other side of the body is the best. Heidenhain recom- mends that the patient be put in a sitting-erect position and maintained in such an attitude as long as possible. I have seen amelioration of the symptoms by this simple manoeuvre. Often the friends standing about will strenuously object, but a compromise may be made by prop- ping the patient against the overturned back of a chair in a half- THE XOX-XEUROXIC DISEASES 84I reclining position. While he is in this attitude cold in the form of cracked ice should be applied to the head to cause as far as possible contraction of the cerebral vessels, and heat to the lower extremities to dilate the blood vessels there and so lessen the blood-pressure in the brain. The latter may be further accomplished by the cautious ad- ministration of a cardiac sedative, such as tincture of aconite or vera- trum. If the case is one of embolic obstruction the reverse of all this should be done. Now we desire to increase the intracranial blood- pressure ; for the embolism being already there and beyond the hope of immediate removal, it is our object to check its progress and fur- ther development by suddenly making it stationary. To do this the patient's head should be placed as low as possible. The cold and heat may be applied as before, but instead of a cardiac sedative a stimulant now acts more favorably, such as alcohol, ammonia, or even small doses of digitalis and ether. The importance of attending at once to the decubitus of the pa- tient is brought out by the fact that undoubtedly some fatalities occur from suffocation. Kind friends, not knowing what to do, put the patient on his back, with head low, and administer brandy, milk or whisky. Very often the tongue is partially paralyzed, as well as the pharynx, while the sensitiveness of the buccal mucous membrane is lost. Saliva and fluids collect, obstruct and even enter the air-passages, setting up inhalation-pneumonia. The obvious thing to do is of course to raise the head, place the patient on the side, carefully pull forward the tongue, and swab out the mouth with a dry rag or bit of linen moistened with some aromatic, antiseptic mouth-wash. As the respiratory power is usually diminished, it is important that the patient should have all the fresh air possible. Crowding about his bed should be forbidden, and the windows even in cold weather should be more or less open. Tt may even be necessary to perform artificial respiration, this being done with as little disturbance of the patient as possible, and only until the respiratory centers have sufficiently recov- ered from their shock to resume their normal functions. All con- striction about the neck by tight clothing must be removed, so that the return of circulation may not be obstructed. To increase the blood- pressure within the cranium in embolism, it may be well at times to apply the Esmarch elastic bandage to the lower extremities. It would be unwise to do this as a routine measure, but its worth in some cases cannot be doubted. To determine the blood away from the head we have two rapii and powerful methods — namely, bleeding and hydragogue purgatior. The old-time practice of placing two or three drops of croton oil on the back of the patient's tongue is a good one. A brisk enema may be employed in some cases, but usually it is too slow in action. The same may be said of elaterium. Cerebral congestion is certainly modified by quick action upon the circulatory and glandular apparatus of the bowel, as can be clearly demonstrated; hence it is a rational con- clusion to hold that the same means will be effective in lessening the intracranial blood-pressure in hemorrhage. 842 THE NON-NEURONIC DISEASES In regard to venesection, or the abstraction of ten or a dozen ounces of blood, there is a wide divergence of opinion. At one time it was a routine practice and consequently abused. On the other hand, there are those who boast that they never bleed a patient. Of the two extremes the latter is the preferable one. The abstraction of a few ounces of blood is almost immediately followed by a partial return to consciousness, and hence the one-time popularity of the treatment. It should be remembered, however, that the heart action in apoplexy is usually weakened. Therefore, to still more weaken it by depriving it of its natural stimulus, the blood, is hardly a commendable procedure. Only in sthenic cases with flushed countenance, full pulsating vessels, and a vigorous general physique is venesection to be thought of. The thinning of the blood diminishes its tendency to coagulation at the seat of hemorrhage and lessens its stimulating power upon the medullary cardiac center and heart muscle. In jnost cases these disadvantages would so far outweigh the possible little benefit due to the mechanical diminution of the intracranial blood-pressure that I am generally op- posed to the operation. In the few cases in which I have abstracted blood, it seemed to me that the slight benefit and partial restoration to consciousness were so temporary as to be without any special value. In one case I fear it hastened the end, though both the attendant and myself anticipated the best of results. All such slow means of withdrawing blood from the head as leeches to the temples, mustard to the nape of the neck, blisters, etc., may be tried in some cases, perhaps most cases ; for, fortunately, even if they are slow, they are not open to such objections as is venesection. If they irritate the patient they had better be desisted from, for the patient's distress will tend to a return or extension of the hemorrhage. Diuretics are judicious, and in this connection it is well to mention that catheterization will sooner or later be required in almost every case. The bladder is usually paralyzed to a certain extent and fails to void its contents. Unless the catheter is promptly and systematically used the patient will experience distress, if he does not acquire a cystitis. The skin should be attended to, and a gentle rubbing of the surface of the body with alcohol and tepid water cannot fail to be of some benefit. The enormous capillary circulation in the deeper layers of the skin may be made to retain a large amount of blood by the application of warmth to the body surface. Moreover, the cleansing of the skin with warm water awakens the activity of the glandular apparatus, and this will aid in determining the flow of blood away from the head. It will thus be seen that very active treatment immediately after an apoplectic shock is uncalled for. Beyond careful nursing, quietude, arranging of the patient's position, attending to the respiration, nutri- tion and excretions, the physician can give but little advice. If the shock is so severe that death is inevitable, little that art or science can do will be of any value one way or the other. The end will come in a few days or hours, and though the occasional twitching of the muscles of the extremities as a result of the local irritation of the clot may awaken false hopes of a return to consciousness, the physician will THE NON-NEURONIC DISEASES 84/, serve his interests best by carefully guarding the family against any fallacious anticipations. If, however, consciousness partially or com- pletely returns, and the patient is a hemiplegic with or without aphasia, the physician can do a great deal, not only for the victim's comfort, but even to ameliorate to a large extent the distress caused by the various sequelae. In fact, this is the stage of apoplexy in which medical science can play its best hand. The storm has passed ; it now remains to restore order as far as possible out of the wreck, and in doing this the attendant can sometimes do wonders. In the first place, every precaution spoken of under the head of prophylaxis should be adopted to prevent a return of the shock. Absolute quiet, mental and physical, with the head elevated : light, nutritious, non-nitrogenous diet ; gentle sponging of the surface of the body ; abundance of fresh air ; and all those agencies calculated to cheer, invigorate and nourish the patient, so far as he is capable of being so acted upon, should be carefully thought of. While the clot is undergoing organization and surrounding itself Avith a capsule, the paralyzed muscles will become somewhat atrophied. To preserve the muscles in the hope of their resuming their function if the nervous centers should regain part of their power, faradism, massage and gentle passive movements are to be instituted. Just how soon this is to be done is the source of a difference of opinion. In my own experience I have found it advantageous to begin very early — gently, of course, at first — not later than the first or second week after the shock and subsidence of all signs of irritation or possible inflam- mation. Passive movements and the passage through the muscles of a current of faradic electricity, just sufficiently strong to produce easy, regular contractions, should be made systematically for a few minutes each day. I am fond of using olive or cocoanut oil when giving the massage, as it softens ,the skin and, I believe, favors to a slight extent the nutrition of the underlying tissues. At no time should anything cold be placed against the body, and at all times the patient should be clad with warm, non-irritating garments. Later on it is a good thing to encourage the patient to attempt voluntary movement. Of course. this will be impossible in many instances, but he should move the well side, and the attendant may assist him by raising at the same time the paralyzed limb. Ofttimes the patient thinks he is doing this of his own will, and the hopefulness which it inspires cannot but be of benefit : nay, more, such simple manoeuvres have kept the cortical memory or representative centers alive, so that when the motor areas had partially recovered their function the patient was better able to attempt and even succeed in performing voluntary movement than he otherwise would have been. Re-education of paralyzed, or rather partially paralvzed. muscles is an important part of the physician's duty. Many a hemiplegic would have been less hemiplegic if the memories of mus- cular movement had been preserved by the early practice of passive movement in conjunction with attempted voluntary movement. What I have just said applies likewise to the aphasia following the shock. The speech center should be encouraged to functionate, or at least an attempt made early in the trouble to awaken the activity "i" 844 THE NON-NEURONIC DISEASES the corresponding speech center of the opposite side. These centers are memory or representative centers for speech, and hence if a patient is utterly neglected it will happen that when the center recovers from the shock, the power of speech will be much less than it otherwise would have been. Hence, as soon as consciousness is sufficiently re- stored, I have the nurse for a few moments each day teach, as she would a child, the names of persons and things, and if possible get the patient to repeat them after her. It is astonishing in a certain per- centage of cases how strikingly and rapidly the aphasic symptom may in part be recovered from by this prompt and regular re-education of the memory centers for speech. In cerebral as well as muscular physi- ology it is an axiom that exercise increases nutrition and function. Many an aphasic hemiplegic would undoubtedly have been less of an aphasic and less of a hemiplegic by the systematic exercise of his cere- bral structures as soon after the shock as possible, and when all signs of inflammation and irritation had subsided. In all traumatic cases, and in all cases in which the symptoms in- dicate a superficial location of the clot, trephining for the removal of the latter is to be taken into serious consideration. I recall the case of a boy of some five years of age who fell over a balustrade and sus- tained a severe concussion of the head, with the formation of an intra- cranial clot with all its accompanying signs of unconsciousness, local- ization and stertor. The spreading of the clot immediately after the fall could be clearly observed by the spreading and deepening of the muscular paralysis. Trephining was immediately performed, with a partial restoration to consciousness and a slight return of the muscu- lar power. The action of the muscles was a striking phenomenon. At first they were violently contracted, probably because of the irritative action of the clot ; then they became paralyzed completely, with a more or less set rigidity. After trephining they relaxed completely, and remained so, or were very feebly moved as a result of subcon- scious volition. Death occurred in a few hours, however, from ex- haustion and shock. The removal of a clot deep in the centrum ovale is a hazardous and difficult operation. It is attempted, however, but with little genuine success. The paralysis in such a case is not re- lieved, but a source of infection and irritation is removed and makes the operation at least one to be thoughtfully considered. I have very little faith in the so-called sorbefacient powers of such remedies as the iodide of potash, gold, arsenic, etc. As alteratives they may indeed somehow modify the clot or the results of its presence in the cerebral tissues, but I am inclined to chink that much of their effect is due to some kind of stimulant tonic power. At all events, their administration does seem to be of some benefit in some eases, and they are therefore to be commended. THE XOX- NEURONIC DISEASES 845 ENCEPHALOMALACIA. EMBOLISM AND THROMBOSIS. Acute cerebral softening, or encephalomalacia, is a local anaemic necrobiosis caused by the sudden occlusion of an artery, and is char- acterized by an apoplectic stroke and hemiplegia. The immediate cause of the obstruction to the circulation is an embolus or a thrombus. The origin of the latter is an abnormal condition of the blood or vascular walls. Etiology. — The etiology of the circulator}' disease determines practically the etiology of encephalomalacia. Embolism generally oc- curs in the young and middle-aged, before the fortieth year. Throm- bosis is a complication of advanced years. Syphilitic thrombosis, how- ever, occurs in early adult life. Women are said to suffer more from embolism than men, but statistics differ on this point. The predisposing causes of both embolism and thrombosis are many and of the highest degree of importance. They are so clearly bound up with the pathology and pathogenesis of cerebral softening, that it will be well to discuss all together. Given the predisposing causes, certain exciting influences such as fright and other sudden mental shock, unwonted physical exercise. abnormal physiological strains of all sorts, parturition, sudden chilling of the skin, lowered heart action, etc., may provoke an apoplectic at- tack. Pathology and Pathogenesis. — Eighty-nine per cent, of the cases of embolism are associated with heart disease. Vegetations from acute or chronic endocarditis and segments from ulcerative processes within the heart are the source of the emboli. Heart clots in the auricular appendix and dvtring the puerperium have produced them. They may spring from a disintegrating thrombus elsewhere. Atheroma or aneurism of the aorta, as well as ulcerative bronchitis, disease in and about the pulmonary veins, and gangrene of the lung, may all be responsible for them. A history of rheumatism with valvular disease and cardiac weakness is present in the great majority of the cases of embolism. Changes in the condition of the blood itself wrought by such influences as the acute infectious fevers, pulmonary tuberculosis, puerperal sepsis and even anaemia have been credited with the formation of emboli. It is more likely, however, that an endocarditic condition is set Up under these conditions, which, together with the disturbed heart action, is the source of the clots. Such deteriorated blood states are more prone to originate thrombi. These, however, may send off secondary embolic particles, a view which is supported somewhat by the fact that in these infection- states the obstructions in the brain are usually minute and multiple. After reaching the brain an embolus usually lodges at the bifurca- tion of some vessel, or if small enough may pass on into an arteriole and completely occlude it. The middle cerebral artery and its end branches are the favorites. The left side is mostly affected because the 846 THE NON-NEURONIC DISEASES blood current is most direct from the heart on that side. Other arteries may rarely be the seat of the obstruction. Emboli have gotten caught in the internal carotid, vertebral, deep cerebral and posterior cerebral arteries. Once lodged, the embolus becomes more or less closely attached to the vascular wall, at times calcified and the center of a secondary local thrombus. It may thus continue to grow and extend into the neighboring vessels. Sometimes it begins to break up and send off other emboli before necrobiosis has been instituted by the first. The thrombotic process is quite different from all this. Here syphilitic arteritis, senile atheroma, weakened heart action and various blood dyscrasise constitute the great predisposing causes. Alcohol, lead, gout, chlorosis, leukaemia and even heredity are obviously impor- tant elements in the causation of thrombosis in this manner. Multiple thrombi have been seen after extensive burns upon the skin and carbon monoxide poisoning. They may result secondarily, as I have already stated, from a fixed embolus. Oppenheim affirms that in his experi- ence atheroma has been incited by certain diseases of the nervous sys- tem and by continued emotional excitement in which there was a per- manent disturbance in the functions of the vascular apparatus. The most common location of a thrombus is the middle cerebral and basilar arteries, though it may start up anywhere. In a general way the base of the brain and the larger vessels are its favorite lo- calities. The complete occlusion of a vessel by an embolus or thrombus produces an intense local ancemia and if there is no anastomosis with other vessels, softening and destruction of tissue in the affected area. These softened areas may be found anywhere in the brain, most com- monly in the cortex and centrum ovale, where the blood vessels are of the terminal variety. The necrobiosis usually begins about a day and a half after the occlusion of the vessel, though swelling and other changes probably occur immediately. Red, yellow and white encephalomalacia are usually referred to by the pathologists. They are merely different stages of the necrobiotic process, the color being dependent upon the blood. Red softening is the first stage and is most marked in cortical foci. As the coloring matter of the blood changes in the affected area a yellowish hue is given to it. This is seen only after several weeks. Finally the yellow tinge passes into a white or bluish-white and the focus contains the debris of degenerated nerve cells and fibres. The presence of the myeline drops, parenchymatous detritus and granular cells aids in distinguishing this condition from mere post-mortem maceration. Absorption ultimately takes place, a cyst is formed, contracts and leaves a permanent cicatrix. Inflammation, suppuration and sclerosis may all be observed in the affected and adjoining areas. The size of the softened focus may he from that of a pinhead to an entire hemisphere. It may be large and single or small and multiple. It is unnecessary to describe here the contributory pathological findings in encephalomalacia, such as the diseases of the heart, blood vessels and other organs. THE NON-NEURONIC DISEASES 847 Symptoms. — The onset of embolism is usually sudden and without premonitory indications. In rare cases there may be the slightest sort of paresthesia immediately before the occlusion, especially if the embolus should catch at a bifurcation. If the obstructed artery is particularly small there may be no disturbance of the consciousness. Usually, however, after some local or general convulsive twitchings, hemiplegia appears and then temporary loss of consciousness. The coma is lighter and of shorter duration than it is in hemorrhage. The temperature does not alter at first, but in a few days it rises somewhat. In septic embolism it may attain a very high degree and be preceded by distinct chills. A unilateral epiletiform seizure, or even a condition resembling status epilepticus, may take the place of the coma. In some cases merely drowsiness with slight delirium occurs. Vomiting, flushed countenance, stertorous breathing and other symptoms of hem- orrhage are wanting. The pulse is feeble as a rule, the countenance slightly paler than normal and the skin cool. Thrombosis is always preceded by prodromes. These are so in- sidious, varied and prolonged sometimes that mistakes in diagnosis are easily made. In syphilitic cases they consist of headache, queer feel- ings about the body, temporary attacks of double vision, cranial nerve palsies and sudden spells of weakness. In other cases hysteroid mani- festations may be observed ; vertigo, extreme emotionalism and irrita- bility, numbness of the hands and feet, temporary aphasia, transient hemiplegic attacks and periods of drowsiness and stupor. Loss of memory, incoherence of speech, slight dysarthria, temporary attacks of disturbed respiration and heart action add to the confusion. Swal- lowing may be troublesome at times, a slight cough may appear and other temporary symptoms may indicate bulbar difficulty. When the attack begins in thrombosis the loss of consciousness is gradual. If the vessels involved are small, or it is due to syphilis, there may be no disturbance of the mind ^whatever. The hemiplegia is usually slow- in developing and precedes somewhat the development of the coma. Neither the paralysis nor the loss of consciousness is as deep as it is in hemorrhage, nor as abrupt and limited as it is in embolism. The hemiplegia is variable and rarely complete. The temperature may tall slightly in the beginning just as it does in hemorrhage. As the basal arteries, such as the basilar, vertebral and posterior cerebral, are more liable to be the seat of thrombosis than of hemorrhage, basal and bulbar manifestations are to be expected more in the former. The hemiplegia of both embolism and thrombosis tends toward rapid and early improvement, unless a very large vessel has been ob- structed. It may be preceded for some days by a hemiparesis-, but as a rule it appears pretty much in the same way that it appears in hemorrhage. If it is right-sided, aphasia accompanies it. The conditions after the subsidence of the acute stage are tin- same in softening as they are in hemorrhage. Occasionally, however, in cortical softening the limitation of the area affected gives rise to limited paralyses, monoplegias, aphasia alone or monoplegias with hemianopsia. Hemianaesthesia may appear with the hemiplegia or exist alone. 848 THE NON-NEURONIC DISEASES The location of the softened spot cannot always be determined from the clinical picture, for the lesion may be a multiple one. In many cases, however, the vessel occluded and the area involved can be more than suspected from the peculiar character and grouping of the symp- toms. One needs only to recall the functions of the particular part of the cortex supplied by the affected artery. Bulbar and pontile symptoms occur when the vertebrals or basilar artery is obstructed. In the case of the former the symptoms are those of acute bulbar paralysis, as detailed elsewhere. „ In the latter the paralysis is apt to be bilateral, associated with rigidity or spasm, and accompanied by a rapid rise of temperature. The results of obstruction in the internal carotid are very variable on account of the anastomoses in the circle of Willis. If these are abundant, the vessel may be ligated and no paralysis will follow ; if the vessels are too narrow or wanting, the softening may be very extensive with hemiplegia, permanent coma and early death. In such a case the clot usually extends into the branching arteries. The inclusion of the arteria centralis retinae in this same lesion provokes of course most serious visual disturbances. Of all the arteries the middle cerebral is the one most commonly affected. The internal capsule is softened and a permanent hemiplegia is established, with temporary or permanent hemianesthesia. Aphasia occurs with disease of the left artery. If one of the branches of the artery is obstructed, the symptoms will follow the localization of the encephalomalacia in the particular convolutions supplied by those re- spective branches. Hence various monoplegias, hemiplegia and motor aphasia will develop. Facio-lmgual monoplegia follows obliteration of the first branch, and aphasia also, if it is on the left side. The second branch when occluded causes a facio-lingual paralysis. The third and fourth branches supply the lower part of the parietal lobes, including the supra-marginal and angular gyri. Their damage results in alexia, in hemianopsia and zvord-deafness. When the fifth branch is plugged word-deafness is the main symptom. It will be noted that our modern knowledge of cerebral localiza- tions, coupled with the knowledge of the minute circulation of the brain, underlies our appreciation of these various groups of symptoms. The anterior cerebral arteries supply the anterior parts of the brain. Their obstruction is compensated for in part by the middle cerebrals. No symptoms therefore usually occur, though it has been thought that mental inefficiency as well as monoplegia of the leg have been caused by it. Diagnosis. — The most important, and ofttimes the most difficult, differential diagnosis to make is between hemorrhage and acute soft- ening: The subject is discussed under the head of cerebral hem- orrhage, but it will probably be well to enumerate here some of the more significant differential signs. In acute softening early and late period of life point respectively to embolism and thrombosis. Hem- orrhage occurs more particularly in the latter half of adult life and at the beginning of old age, from forty to sixty approximately. In acute softening there is a history of syphilis, rheumatism with recurrent THE NON-NEURONIC DISEASES 849 endocarditis, infection or an atheromatous condition of the arteries ; in hemorrhage heredity and vascular disease are present somewhat. Abrupt onset without premonitory indications, sudden deep coma, gen- eral paralysis that shows a tendency to recede and become more re- stricted in its distribution, unequal pupils, conjugate deviation of the head and eyes, flushed face, full bounding pulse and the temperature phenomenon indicate hemorrhage ; whereas in acute encephalomalacia prodromes are frequent, much more so in thrombosis than in embolism, the paralysis and loss of consciousness approach more gradually, the former slightly preceding the latter, the limbs are convulsed, the heart is weak, the muscles are not completely relaxed, and the temperature phenomenon of hemorrhage is absent. Aphasia is common in embol- ism, as the lesion usually affects the left side ; and mental changes characterize thrombosis on account of the prolonged disturbance of the nutrition of the brain. Prognosis. — The prognosis in regard to life is generally favor- able in softening and as compared with that of hemorrhage during the stage of coma the hope of recovery may be expressed with a greater degree of positiveness. However, it is an unfavorable sign if the coma is deep, the heart action bad and the patient does not come to for several days. The outlook in carotid and basilar thrombosis is bad. . Recovery practically never takes place, though the end may be long deferred, the patient continuing sometimes for months and even years, experi- encing remissions and relapses in thrombosis particularly. I have seen several of these cases variously diagnosed and treated by different practitioners during a period of many months, in one case over a year. Recurrence is less prone to appear in embolism than in thrombosis. Most of the heiniplegics that continue to live many years without a relapse owe their paralysis to a small area of softening. In regard to the future outlook for the hemiplegia, it is about the same in softening as it is in hemorrhage. If after the first month there is no change, the patient had better be informed of the perma- nency of the condition. Speech disturbances are apt to be a little more fixed here than in hemorrhage after a certain period has been passed, and yet in the latter the initial aphasia usually lasts a trifle longer. In arriving at the prognosis of encephalomalacia the extent of the lesion and the location of the obstruction exercise great weight. Treatment. — The differential treatment of the comatose stage of acute softening, and the general management of the sequelae, are dis- cussed in detail under the head of cerebral hemorrhage. I desire heir merely to reiterate the injunction to do as little as possible, in >< •un- cases practically nothing, until it is decided whether tin- case is one of softening or hemorrhage. If it is the former, the patient had l» be placed horizontal or even with the foot of tin- bed slightly elevated. As the clot cannot be removed, it is desirable to urge it on to a smaller vessel if possible and to fix it there as soon as possible. Its furthci enlargement is to be prevented by every means in our power. Tin indications for the accomplishment of these ends are t<> favor the flow of the blood towards the head by gravity and stimulation of the 850 THE NON-NEURONIC DISEASES heart and by avoiding ail measures that have a tendency to deplete the blood. Venesection must be rigidly avoided. The bowels must not be acted upon with cathartics too soon. The patient must be kept at absolute rest with his head low. Alcohol, digitalis, strophanthus, strychnia and nitroglycerin are available for stimulating the circula- tion. With these exceptions, the treatment of apoplexy from arterial obstruction differs in nowise from that of apoplexy from hemorrhage. A history of syphilis calls for the prompt administration of mer- cury and the iodides. These had better be continued for a long time with the heart stimulants and general tonics. The management of the hemiplegia, aphasia and other symp- tomatic treatment are discussed elsewhere. THROMBOSIS OF THE INTRACRANIAL VEINS AND SINUSES. Thrombi are liable to form in any of the intracranial veins and sinuses. They are of serious import both on account of the effects they themselves produce, and on account of the effects produced by the malady of which they are a collateral result. Etiology.— Etiologically, thrombi must be divided into the pri- mary and secondary. A primary thrombus is one that forms merely on account of the inherent state of the blood or the circulation. A secondary thrombus is the outgrowth of some serious disease, usually septic, in the neighborhood. Primary thromboses are called "marantic," because they usually occur in adynamic states. Children and the aged are usually affected, though, of course, all ages are subject to marantic conditions or states of marasmus. In exhausting diarrhoeas, such as infants particularly suffer from, wasting pulmonary affections, prolonged fevers and long- continued suppurative processes, even acute specific maladies, the blood is so altered and the heart is so weakened that coagulation readily supervenes in these veins, where the circulation at best is not an active one. The anatomical structure of a sinus precludes any great elas- ticity in its walls ; hence when the blood is thinned and diminished in amount in these low states, the sinus does not contract and equalize the circulation. This fosters stasis and coagulation. Anaemia less frequently than chlorosis provokes a primary thrombosis. Severe hem- orrhages have been followed by it. It occurs in adults sometimes after a long cachexia, such as cancer and phthisis. The puerperal state must at times be charged with it. The superior longitudinal sinus seems to be the favorite site tor the formation of a primary thrombus. After this come the lateral and cavernous sinuses. There are certain mechanical features about the circulation that go far towards explaining this selective action on the part of the clot. The collateral veins that enter the longitudinal sinus do so at an angle and in a direction the reverse of that of the blood current in it. The sinus and venous currents thus meet in opposing directions. The latter also have to ascend to reach the main flow in the sinus besides themselves receiving blood from ascending arteries. THE NON-NEURONIC DISEASES §5 1 The irregularity of the inner wall of the vessels has some effect also, as is shown by the fact that the clot is attached, as it were, to one side while the blood goes flowing along in the channel beside it. Secondary thrombosis is usually the product of a phlebitis in the sinus wall or of the veins entering it. This phlebitis is most commonly originated by caries of the bone or internal ear disease. It is the result of a septic process generally of infectious origin. Nearly every sinus is the recipient of veins from the exterior of the skull. The transverse sinus receives thus through the mastoid foramen a vein which brings in blood from the occipital and posterior auricular veins on the outside of the head; while the longitudinal sinus receives a communication through the parietal foramen from the external veins of the skull. Other sinuses are similarly supplied. Injuries to the bone with inflammation of the diploe, tubercular meningitis, erysipelas, carbuncle of the face or neck, malignant ulcera- tion, orbital phlegmon, panophthalmitis and suppurating eczema of the scalp are all sources of the infection. A general septicaemia is to blame for it sometimes. Compression of the sinus has been followed by a secondary thrombosis. Of course, these secondary thrombi always occur in the sinus that is nearest to the primary seat of infection. The ear is probably the most common seat and hence these thrombi are most frequently seen in the superior petrosal or lateral sinus. Unlike the primary thrombi, they are rather infrequent in the superior longitudinal sinus. Pathology and Pathogenesis. — All or part of a sinus may be filled with the clot. In rare instances every sinus has been the seat of the thrombotic formation. The clot is dark red, soft and friable if recent, but is lighter in color, harder, more friable, granular and laminated if an old one. Recent clots are not quite as adherent to the sinus wall as are the older ones. Secondary thrombi are usually asso- ciated with phlebitis, are puriform and softened. The veins are not, as a rule, inflamed in the primary or marantic thrombi. It is said that Heubner recovered microorganisms from what seemed to be primary thrombi. In secondary thrombi abscesses are observed in the vessel walls, and masses of pus are discovered in even remote veins, such as the subclavian and superior vena cava. Meningitis is common under these conditions, naturally. Behind the thrombus there is, of course, congestion, oedema. The contributing vessels are engorged and swollen. The capillaries arc often ruptured in the gray substance of the brain and there arc in- numerable, minute hemorrhagic foci. A sinus thrombus may be compensated for by the blood passing around by another channel. This is not possible in venous thromb >sis. The veins not encasing the clot are tortuous and dilated. Softening occurs in the cerebral tissues as a result of the oedema and hemorrhage. Tt may be very extensive or minute or in innumer- able scattered or mulberry-like spots. Meningeal hemorrhage takes place occasionally, but rarely. If recovery should happily take place, the damage is liardh as great as that which is left after arterial obstruction. Secondary 852 THE NON-NEURONIC DISEASES abscesses have been found in the lungs and remoter parts |fcom a soft- ened, scattered intracranial thrombus. Gowers says that in about half of the cases of secondary thrombosis these secondary remote septic abscesses are found. Symptoms. — These are not very distinctive. They are very apt to be masked by the symptoms of the disease out of which the throm- bus originated. In primary thrombosis, for instance, the adynamia, the anaemia, the heart weakness, the chlorosis are so prominent that one loses sight of the symptoms caused by the sinus obstruction. The ear disease, the meningitis, the septic manifestations generally quite over- ride the signs of the secondary thrombus that may be present. The presence of an obstruction to the venous flow in the brain would be expected to produce two sets of symptoms — namely, those of a local character depending upon the location of the clot and the part of the brain implicated, and those dependent upon the disturbed circulation outside of the head. Neither of these, however, are at all characteristic, and the latter, which are the most so, are at the same time the rarest. A clot in the sinus produces obvious enough mani- festations in the external cranial and facial veins that happen to pass through and connect with that sinus ; but unfortunately the phenomena are not often to be seen. Superior Longitudinal Sinus. — Thrombosis of this sinus may be accompanied by an cedematous condition in the cranium and a dilated, twisted and tortuous state of the veins lying over the frontal, parietal and temporal bones. Venous epistaxis may occur from the vessels of the nose that connect with it in front. In children, who have been afflicted with exhausting diarrhoea, the fontanelles are usually de- pressed. When this thrombus appears, however, the fontanelles bulge and become very tense from the associated oedema and congestion. Among the cerebral complications are to be noted symptoms simi- lar to those of increased intracranial pressure, meningitis and soften- ing. There is apathy, stupor, somnolence, even coma. Severe head- ache is indicated, with vomiting and convulsions. Delirium is some- times present. The convulsions may be local or general. Rigidity of the neck and back, as in opisthotonos, more or less obtains. Tremor, incoordination and 'Contracture, strabismus and paralysis of the limbs are possible symptoms. The paralysis may be unilateral if the clot extends into the veins covering one hemisphere. Even monoplegic phenomena have been rarely seen. Aphasia is almost unknown. Hemianaesthesia has been but infrequently observed. The pulse and respiration are variable. They may be normal or subnormal. They are usually elevated near death, which in most patients occurs in coma in a few days or a week or two. The tempera- ture as a rule is normal, though it has risen in leaps at times, even as high as 108 degrees F. These symptoms are so irregular and indefinite that it is clear a diagnosis can only be made upon the etiology and signs of congestion. Often no diagnosis can he made, for a similar clinical picture can occasionally be presented by infantile hydrocephaloid disease, chloro meningitis, encephalitis and cerebral hemorrhage. THE NON-NEURONIC DISEASES 853 Cavernous Sinus. — Here the eyelids and temples are apt to be cedematous, and the veins in and about the orbit to be tortuous and dilated. The eyeball may be protruded and the optic papilla (edematous. It is said that even choked disc has been observed. The retinal veins have certainly been congested. Amblyopia and amaurosis have result- ed in some cases, even with a normal appearance of the eye ground. Neuralgic pains have been felt in the fifth nerve and headache is common. The nerves that run to the orbit in the wall of the sinus, such as the third, fourth and sixth nerves, are liable to be involved. Neuro- paralytic manifestations may be not unexpected. Petrosal Sinus. — There are no known symptoms of thrombosis in either of the petrosal sinuses. Lateral or Transverse Sinus. — There is here apt to be oedema behind and over the mastoid process. Gerhardt's observation is open to question — namely, that the external jugular vein on the affected side is less full of blood than it is on the unaffected side, because on the affected side of the head the blood that should flow into the external jugular flows largely on account of the presence of the clot now into the internal jugular. There are no distinctive cerebral symptoms, but there have been observed disorders of the ninth, tenth, eleventh and twelfth cranial nerves. In secondary, phlebitic thrombosis there is not much direct dam- age done to the brain. The meningitis that is present, however, may provoke marked cerebral manifestations and the septic source of the whole trouble may lead to a cerebral abscess. The mere occlusion, however, does not produce these or other distinctive cerebral phenom- ena. Sometimes the clot itself is the seat of a violent purulent process. It softens and breaks down and lights up a virulent form of septicaemia, with the establishment of secondary distant foci in the lungs and else- where, these being the first indications of the presence of the clot. These secondary septic symptoms are of the usual character and need no special description here. Among the cerebral symptoms that now begin to appear are to be noted apathy, dullness, stupor, headache, motor and sensory irritative meningitic phenomena, such as unequal paralysis, muscular twitchings, rigidity and disordered sensation. These secondary thrombi last from a few days to a couple of weeks and the sufferer dies in coma. Rec ery is so rare as hardly ever to be counted upon. Primary clots offer a better prognosis, sometimes being absorbed entirely or leaving per- manent cerebral damage. Prognosis. — This is very serious in all eases. Secondary phlebitic thrombosis is practically a fatal disease. ( )ccasionallv in children the symptoms of a primary, marantic clot disappear. In adults this is much rarer. If the general signs pass away, there may nevertheless remain some local permanent damage and disorder. I Operative treatment has improved somewhat the prognosis of the secondary thrombi. The secondary results, such as the leptomeningitis, cerebral ab ptic foci in the lungs, have their own prognosis, which, of course, deter mines that of the thrombosis. TREATMENT. — The treatment of sinus thrombosis almost limits 854 THE XOX-XEUROXIC DISEASES itself to the treatment of the cause. In the primary form the vigor and general health must be restored as rapidly as possible. The circulation should be stimulated and tonics administered. Never should blood- letting be practiced. The recumbent attitude with the head slightly elevated should be maintained and flexion of the neck must be care- fully avoided. In the secondary form all accessible foci of pus should be sur- gically drained. The statistics of some surgeons are remarkably favor- able. According to Henschen, of 145 cases, fifty-eight per cent, were cured. The reader is referred to works upon surgery for further details. When septicaemia is general, tincture of the perchloride of iron, quinine and the salicylates should be persistently administered. INFANTILE HEMIPLEGIA. The cerebral palsies of childhood constitute a group of cases that has provoked hitherto a great deal of discussion and much unnecessary refinement of classification. As the name indicates, and as will appear in the study of their pathology and pathogenesis, they are all due pri- marily to some defect or lesion in the brain. They are all hemiplegic in character and distribution. If the lesion extends to bilateral parts of the brain, the hemiplegia is bilateral or double, and the disease is known as cerebral diplegia. No distinction of any practical value can be made between unilateral and bilateral hemiplegia in childhood except upon an etiological and pathological basis. As no such basis is yet known, we must perforce continue to recognize them both as mani- festations of the same general trouble. Under the name of Little's disease, cerebral paraplegia, congenital or juvenile spastic paraparesis, congenital spastic rigidity of the limbs, some writers have endeavored to establish a separate affection, though they admit that it is cerebral in origin, in the vast majority of cases at least, and that the paralysis has all the characteristics of a true hemi- plegia. The term paraplegia, which has become so fixed as a defini- tion of spinal paralysis, is extremely inappropriate. The limitation of the palsy more particularly to the lower limbs, the absence of certain mental manifestations, and the prominence of the lesion in the pyra- midal tracts do not in the least withdraw Little's disease from the double hemiplegias of childhood. On the other hand, the etiology, pathogenesis and symptomatology show very conclusively that it is primarily cerebral in origin with secondary changes in the cord char- acteristic of all hemiplegias. All of the cerebral palsies of childhood now under consideration, including Little's type, are due to a degenerative change in the upper motor or cortico-spinal tract. The unilateral or bilateral distribution of this degeneration is a mere matter of etiology. By all the more recent pathologists it is believed that cortical disease of some sort is to he charged with the beginning of the degeneration. The separation of these palsies into different types therefore merely on account of the distribution of the paralysis, their relationship to the act of parturition, or the prominence of the lesion in the pyramidal tracts is unnecessary, THE NON-NEURONIC DISEASES 855 unwarranted and confusing. The only way to dispose of them, until we have a better foundation for differentiation than we have to-day in their etiology and pathology, is to group them all together in a single class and to discuss them under the head of diseases of the brain or of the upper motor segment. Etiology. — The cerebral palsies of childhood are accidental and are therefore not influenced by such etiological factors as heredity, age, sex, nationality, climate, etc. Their causes are numerous ; for the sake of convenience, therefore, we divide them into the prenatal, natal and postnatal, remembering all the while that the same immediate cause, as, for example, cerebral hemorrhage or embolism, may be the same in all three periods. In about one-third of these palsies the cause is prenatal, and we denominate the disease congenital. The damage to the brain is ac- complished during intrauterine life and the disease is merely congenital and not in the least hereditary, as I have seen it stated. Intrauterine traumata,, injuries to the mother during pregnancy, psychic shocks. any accident in fact that may cause an inflammation to the motor zone of the fetal brain, are among the more common prenatal causes. Hemorrhage and embolism are the lesions in the majority of these cases. Hereditary syphilis has been credited with the disease, though this is open to doubt. The evidences of a meningoencephalitis, which Striimpell believed to be the underlying condition in the diplegic cases, are not forthcoming, though it is still held that syphilis and other toxic influences are at work in some cases. Simple agenesis, or lack of brain development, ab initio, without apparent cause, traumatic or toxic, may account for a few cases. The cases due to injury during birth are more numerous. Among these natal causes should be remembered slow and tedious parturition, compression of the head by tjie mother's birth passage or by the for- ceps, retention of the head high up before delivery, precipitate labor, etc. Most of these accidents occur in first labors and many of them are associated with asphyxia and foot presentations. The possible existence of a prenatal cause should always make one hesitate before positively attributing the accident to one of these obstetrical causes. The most skillful use of the forceps will not save an obstetrician's rep- utation if the child is paralyzed, though from an almost positively es- tablished prenatal accident. The laity have unfortunatel) acquired the notion that all these forms of paralysis arc due to the use of the forceps, and it behooves us to re-educate them. In these eases the lesion is usually thrombosis, embolism, meningeal or intracerebral hemorrhage. They are less frequently diplegic in character than are the prenatal cases, and they are more distinctively hemiplegic. The postnatal cases may be hemorrhagic and due to traumata, bill they are more commonly due to disease, tumor and general c institu- tional influences. It is these cases thai Striimpell believed especially to be dependent upon a polioencephalitis, an affection analogous to and in the same general category as poliomyelitis, [nfection certainly plays here a large role; measles and scarlet fever with nephritis and endo carditis cspeciallv being frequent among its antecedents, h lias h-m 856 THE NON-NEURONIC DISEASES attributed also to pneumonia, pertussis, variola and even vaccination. Vascular disease and probably embolism are the immediate causes of the paralytic condition. Cranial injuries and mental shock in pro- voking hemorrhage are undoubtedly among the later postnatal causes. In one case the child was thought to be dead immediately after birth and allowed to lie a long time exposed upon a marble slab. It is doubtful, however, whether the disease is ever produced in this way, especially in the absence of infection or trauma. Pathology and Pathogenesis. — The pathological findings in the cerebral palsies of infants are numerous and varied. In one thing only are they uniform, and that is in their involvement of the cortex and related subcortical and spinal structures. The varied etiology of the trouble causes the varied pathology. The relationship of the clinical picture to the morbid anatomy is not always as distinct as could be^ desired, but the connection between the primary cortical damage and the secondary spinal degeneration is definitely recognized. Post- mortem testimony affirms very clearly that the pathogenesis of all this class of paralysis involves a simple agenesis, or failure of brain develop- ment, a general diffuse sclerosis of the cortex following, perhaps, a meningo-encephalitis or a very doubtful polioencephalitis, a hemorrhage or an encephalomalacia from embolism or thrombosis. These condi- tions do not differ per se from the same conditions in the adult ; but as they occur in the young and undeveloped brain, their ultimate re- sults are not the same. An inflammation, sclerosis or a compressing clot that prevents the growth of the brain will, of course, produce a different pathological and clinical exhibition from that produced by the same influences in a fully grown brain. The same is to be said of the secondary degenerations in the underlying related tracts. The most obvious of the congenital changes thus brought about is the condition known as porencephaly. In all of these conditions the motor area of the cortex is almost constantly involved. In rare instances it may be the only area implicated. In the larger number of cases other parts of the brain are also affected, though, it may be, not so obtrusively as the motor areas. The general process is a more or less diffuse one, with the initial focus usually in or near the motor elements, but occasionally away from them. Thus porencephaly as well as other changes have been found in the occipital lobe and cerebellum, with corresponding congenital clinical manifestations. On account of the porencephaly, late sclerosis with induration and atrophy of the hemispheres, it is often quite impossible to make a satisfactory guess as to the exact nature of the original lesion or disorder. Many cases are believed to be due to simple failure of development. cerebral agenesis, without any other discoverable cause or pathogenetic factor. The nervous elements in the cortex exhibit microscopic changes that suggest mere defective development. Such cases have been studied especially by Sachs and may in part explain such anomalous observations as that of ( )ppenheim, wherein a mother and a daughter were afflicted with the same type of paralysis. A general, diffuse sclerotic condition of the cortex is found more frequently than that of simple agenesis. This is probably due to a THE NON-NEURONIC DISEASES 857 low grade of chronic inflammation, which may have been chronic from the beginning or have succeeded a meningoencephalitis. If the in- flammation is more or less localized, it may have diffused itself from a focus started by a hemorrhage or other trauma ; or if it is so widely spread out as to involve the greater part of the cortex it is probably toxic or infectious in origin. The few cases that are attribut- ed to syphilis, and the many that seem to follow the infectious fevers, may thus be explained. A whole hemisphere may be affected thus, and on account of the atrophy may be rendered much smaller than it nor- mally should be. This is sometimes called lobar sclerosis. Excrescences in the brain substance, hypertrophic sclerosis and hydrocephalus are accompaniments sometimes of this condition. The effort of Strumpell to align these cases, in part at least, with infantile spinal palsy by at- tributing them to a pure polioencephalitis, has not been very successful ; largely because no evidences of such inflammation, apart from men- ingo-encephalitis, have been found post mortem. The hypothesis, how- ever, is not unworthy of further investigation. By all odds the great majority of these cases, even of the diplegic type, are due to hemorrhage, embolism or thrombosis. The relative frequency of these respectively cannot be definitely determined. In traumatic cases, whether intra- or extra-uterine, intra-cerebral hem- orrhage certainly accounts for the hemiplegia, while meningeal hem- orrhage explains the bilateral or diplegic paralyses. Infective and toxic states, with the associated vascular disease, are the general causes underlying most of the cases of embolism and thrombosis. Areas of focal softening, dried-up cysts and spots of cicatricial atrophy are the evidences usually presented. When the nutrition of a part of the brain is shut off by direct damage or by arterial obstruction, it ceases to grow, a large cavity is formed, which may extendi into the ventricle, funnel-shaped and the condition of porencephaly established. This is usually prenatal in origin, though it may start after birth. It is said to occur in about one- fourth of the cases. It may be observed in one or both hemispheres. A curious condition that has been found in the brains of some of these diplegics is that known as microgyria. It is a developmental anomaly clearly and reveals a great increase in the number (A gyri and intervening small sulci. The whole gray cortex seems to be fold- ed in and out much as it is in the vermis cerebelli. < >tto has illustrated it well. The secondary changes in the projection tracts from the defective cortex are again slightly different from what they are in the adult cases. The development is checked and not fully developed elements merely destroyed. Some have thought that this developmental inhibi- tion might be primary in the cord and s. 1 accounl for the rases of supposed primary congenital spastic paraplegia. This is very doubtful. however. Developmental degeneration in neuraxones withpul primary changes of any sort in the related cell bodies would upset all our mod- ern ideas in regard to neurone histopathology. Symptoms. — In most of the cases of infantile cerebral palsy tlu- disease does not attract attention until the paralysis i- quite noticeable. 858 THE NON-NEURONIC DISEASES Only in the postnatal cases can the initial phenomena be observed, and even in these cases they are frequently overlooked or misinterpreted. According to the nature of the case, they consist of a unilateral or general convulsion, followed by a hemiplegia, or a febrile outbreak with vomiting, delirium, stupor, followed by a more or less diplegic form of paralysis. Instead of one or two general or unilateral con- vulsions, a series of spasms may appear for several days or weeks, and ultimately pass into a state of bilateral or unilateral hemiplegia. The convulsions may occur at or immediately after birth, but the paralysis may not be noticed for weeks or months later. This is not so much due to the fact that the paralytic is far removed from the convulsive stage as that the former is not made obvious until the period has ar- rived at which the finer movements of the limbs, which suffer the most, would normally begin to announce themselves. Therefore most of the cases of cerebral palsy in children occur in the period between six months and three years of age. The infantile peculiarities of these hemiplegias are due obviously to the fact that they occur in the preeminently developmental period of life. The two prominent features that stand out in the clinical picture of these palsies are the characteristic paralysis of the extremities and cer- tain other symptoms that are even more distinctively of cerebral origin, such as epilepsy, strabismus, speech troubles and psychical dis- turbances. The paralysis in the limbs, as I have said, follows soon after the convulsive stage. It is distinctly a hemiplegia or unilateral symptom. It usually involves the lower extremities more than the upper. In some cases its onset is the same as it is in the hemiplegia of adults. The entire body is convulsed and paralyzed during a brief comatose stage. Gradually, however, one side of the body recovers leaving the face, arm and leg of the opposite side limp or immovable. More frequently spasmodic movements occur in the face, eyes, neck, arm or leg. As these quiet down, some slight return of movement appears in the face, then the arm and even the leg. The face as a rule quickly regains its normal appearance and activity ; the arm continues to improve until in some cases it may appear to be quite natural again ; the leg, how- ever, usually recovers only in part and is nearly always permanently affected. When the arm continues to be paralyzed the leg as a rule is still more so. When the age arrives for the child to attempt walk- ing, the paralysis of the limbs becomes extremely obvious and often it is not until this time that the parents fully realize that their child is seriously affected. The attempted steps taken by the little one arc seen to be awkward, difficult, stiff and jerky. In the course of time the gait assumes a. characteristic manner. The strong adduction of the legs, their irregular, stiff, spasmodic movements and their want of equilibrium in the activity of the antagonistic sets of muscles, cause the typical cross-legged progression and attitude shown in the accom- panying photographs. The knees rub against each other, the joints are slightly flexed, and the foot is so extended that it rests entirely on the balls of the toes. Rigidity, motor weakness and marked exagger- ation of the knee-jerks can all be observed. There are no sensory THE NON-NEURONIC DISEASES 859 O ^ ■= 86o THE NON-NEURONIC DISEASES symptoms, no muscular atrophy, or very little, no electrical alterations of importance ; simply a spastic, motor paralysis with contracture in certain groups of muscles resulting in a characteristic gait and attitude. The arms may or may not be affected in company with the legs_ When they are, their movements and attitude are typical. As they are less involved as a rule than the legs, their movements are not so rigid and weak as they are choreic and athetoid or vermicular and waxy-like. The joints are somewhat flexed and the muscles contractured. Ad- duction, flexion and exaggerated tendon reflexes are characteristic- phenomena. I have described the above symptoms as being hemiplegic in order to impress the fact that they are unilateral in origin. As a matter of fact they are bilateral in distribution, and being more frequent and more marked in the lower than in the upper extremities, the entire clinical picture so closely resembles a spinal paraplegia that it affords some slight ground for the suspicion that there may be a primary spastic paraplegia in infants comparable to the adult primary spastic para- plegia of Erb. Until the existence of spinal spastic paraplegia is better established than it is to-day, it seems to me preferable to apply to these cases the name diplegia, or double hemiplegia, and not the misleading term paraplegia. In the cases that are distinctively unilateral the same general de- scription of the paralysis applies, except that here the arm is more affected than the leg. The face usually manifests irritative rather than paralytic symptoms. The tongue is temporarily involved. The leg- recovers earlier and more than the arm. In a word, the clinical pic- ture is identical with that of cerebral hemorrhage, embolism and throm- bosis in adults. The motor weakness, the rigidity, the flexions, the contractures and the exaggerated reflexes differ in nowise from the adult forms, only as they interfere with the further development of the parts. Deformity, shortening, diminution in size and muscular wasting' from non-use are the inevitable results. All degrees of the trouble may be observed, from a scarcely perceptible rigidity up to an extreme paralysis. The tendon reflexes are always increased and the muscular tone heightened. Occasionally an ankle clonus may be evoked. The arm may be paralyzed and the leg merely spastic. In fact, great varia- bility occurs in the grouping of the symptoms of infantile hemiplegia. A curious and noteworthy phenomenon in these early hemiplegias is the associated movements. When a movement is performed on the well side, a lesser but corresponding movement is automatically per- formed on the paralyzed side. Much more common in childhood hemiplegia are the symptoms of cerebral irritation. They consist chiefly of choreiform, athetoid and trembling movements. Involuntary, irregular, incoordinated jactita- tions occur in the limb and resemble exactly a true chorea except in being so localized. They have been more or less present in every case that I have examined. The frequency and distribution of these post- hemiplegic choreas throw considerable light upon the probable patho- genesis of all forms of chorea. The athetoid movements often observed in these cases consist of slow, deliberate, wormy, wax-like flexion and THE NON-NEURONIC DISEASES 86l -extension, especially of the lingers and hand. They give to the move- ments and attitude an appearance of excessive affectation. Athetosis may occur in the leg as well as in the arm. One limb may even be choreic and another athetoid. In some exceptional cases an intentional tremor, such as is observed in disseminated sclerosis, may be detected. The cranial nerves may be implicated, causing strabismus, speech disturbances, difficult deglutition and other signs of bulbar disease. The motor character of all these manifestations is so overwhelming that one is prone to regard the lesion as solely one of the motor area of the cortex. It would upset, or at least greatly confuse, the accepted patho- genesis of these cases if no sensory symptoms were ever to have been found. As it is now believed that the sensory elements of the cortex are largely intermingled with the motor elements, one would think that sensory symptoms ought to be more frequently met with. Two ex- planations are possible to account for their absence in the report of eases. In the first place, it is extremely difficult to examine sensation satisfactorily in children so young as these are and to get reliable re- sponses ; and in the second place, the lesion in a large, number of cases, perhaps the largest number, involves more particularly the motor part of the internal capsule, and only temporarily and indirectly affects the sensory part. Nevertheless in a few carefully examined cases hypaesthesia, Wernicke's mental tactile anaesthesia, hemianopsia and aphasia have been noted. In all of the cases of infantile hemiplegia, whether unilateral or bilateral, prenatal or postnatal, cerebral symptoms of an irritative and developmental character sooner or later announce themselves. Some of these have already been referred to, as, for instance, the cranial nerve disturbances, the aphasia, the hemianopsia, and the choreic and athetoid exhibitions. Most important, however, are the epileptic at- tacks and the mental deterioration. Epilepsy and imbecility are so fre- quent that when found in conjunction with the paralysis become almost pathognomonic. Nearly two-thirds of the cases are subject to epileptic at dicks. Spratling finds that the most potent, immediate and single cause of epilepsy is the cerebral palsies of early life. The attacks may come on soon after the convulsive stage or years after. Usually the paralysis has existed a year or two before the fits began. I have seen a case in which the convulsion was entirely epileptiform and in which the tit- soon ceased in spite of the high degree of hemiplegia. The tit may be of the Jacksonian or partial type, but more commonly it is generalized as in ordinary genuine epilepsy. Unilateral epileptiform convulsions and spasms, without loss of consciousness, are not uncommon. The fit is rarely as protean as it is in ordinary epilepsy, though one may not be surprised to see the severest kind of fits with the cry, the tongue biting, the Unconsciousness and the convulsions of the idiopathic dis ease. Even status epilepticus, followed by early death, ma\ occur. I lately saw a case in which the hemiplegia had left only the merest i in the hand, but the epilepsy was formidable. So severe a lesion as usually causes these infantile palsies could hardlv be expected to leave the mental faculties intact The mental 862 THE NON-NEURONIC DISEASES zveakness, imbecility and even idiocy are usually the direct result of the damage done to the growing brain. On the other hand, care must be taken to discover, if possible, if the mental deterioration may not be a secondary result of the epilepsy, of loss of hearing, or absence of lan- guage from mere aphasia. In many cases the general intelligence is not much affected, but upon close examination it will be noted that there is some degree of irritability of temper, exalted emotionalism and other symptoms that are hy steroid in character. It is important always to note the degree of mental impairment, for the prognosis and possible improvement of the paralytic symptoms depend in large meas- ure upon the degree of mentality in the case. Moreover in those cases in which the psychic disturbances are the least the paralyses that have remained somewhat stationary occasionally undergo a slight change for the better after puberty. Cranial malformations, facial asymmetry, prognathism, defective teeth and other stigmata of degeneration are found in a certain per- centage of these early hemiplegics. The skull may be microcephalic or macrocephalic, flattened on the side, of the lesion or otherwise altered with depressions, fissures, etc. It is w r ell to carefully distinguish always true stigmata of degeneracy from the mere changes wrought in the bones and other tissues by such influences as pressure, disturbed local nutrition and delayed development. Diagnosis. — The diagnosis of a case of cerebral palsy of child- hood is not particularly difficult if the history of the case is we'll studied and the hemiplegic character of the paralysis, with the possible existence of cerebral symptoms, are taken well into consideration. As distinguished from a spinal palsy, these cases involve larger groups of muscles, have exaggerated reflexes, show no true muscular atrophy or electrical reaction of degeneration and are accompanied by cranial symptoms, epilepsy and mental disorders. The real differential diagnosis in these cases involves more par- ticularly the distinction between the unilateral hemiplegias, the diple- gias and the spastic paralysis of Little's type. It must be remembered ■that they are all cerebral in origin and therefore are all hemiplegic. In regard to the character of the paralysis, it is the same in all of them ; being merely unilateral in some cases, bilateral in others. This indi- cates involvement of one or both hemispheres with such lesions as hemorrhage, embolism, thrombosis, meningo-encephalitis, encephalitis or simple agenesis. The pathological diagnosis is not always easy, because the same pathogenetic factors may underlie very different sets of symptoms. In general, however, it may be said that prenatal cases produce largely the Little's type of the disease in which a diple- gia of the lower limbs, with but little involvement of the arms and scarcely any of the mental faculties, constitutes the salient feature in the clinical picture, all depending upon simple cerebral agenesis, hem- orrhage, chiefly meningeal, or meningo-encephalitis. The natal cases or true birth palsies are in most instances due to meningeal hemorrhage. They are diplegic and involve the arms and the mental faculties rather more frequently than the preceding group of cases. In the postnatal cases and those following the infectious fevers hemorrhage, chiefly THE NON-NEURONIC DISEASES S63 intracerebral, embolism and thrombosis are the more common causes of the paralysis. These cases are usually unilateral. They exhibit the pre-hemiplegic and post-hemiplegic choreic phenomena, and athetosis rather more frequently than the cases that are more distinctly diplegic. While epilepsy and mental deterioration are present in these cases, the imbecility and idiocy are rare as compared with the diplegic cases. As the paralysis in all of these cases may occasionally be slight and the epilepsy, chorea or psychic disturbance be very prominent, confusion is likely to arise in the differential diagnosis between these accompani- ments of congenital spastic palsy and ordinary epilepsy, chorea and insanity. A careful regard for the anamnesis, the character and distribution of the special symptoms and the results of treatment will make clear the real nature of the case in hand. Prognosis. — The cerebral palsies of childhood are chronic and incurable. Nervous elements that have been profoundly damaged or prevented from growing cannot be restored by human agencies. The course of the disease, the time and cause of death and other factors in its prognosis are determined largely by the appearance and course of particular symptoms. Hemorrhagic and acute encephalitic cases usually recede somewhat and some improvement in the resulting paralysis may be hoped for up to the end of the first year. After that it remains stationary, except in a few of the diplegic cases in which the mind continues intact, in whom some improvement may be looked for after puberty. Diplegic cases as a rule are more stationary than the simple hemiplegic. If, however, epilepsy and imbecility develop, the prognosis becomes bad and the asylum is usually their final refuge. The absence of mental disorder secures a better prognosis for the paralysis. The diplegia of Little's type therefore can often be mark- edly improved, as cerebral disturbances other than the double hemi- plegia are relatively infrequent. Chorea, athetosis and contractures are unfavorable symptoms. They usually remain in spite of all kinds of treatment. Epilepsy may come on late or early. It may pass at any time into status epilepticus and destroy the patient. The presence of true stigmata of degeneracy renders the prognosis unfavorable. Aphasia usually disappears, though dysarthria may continue permanently. Some hemiplegics reach an old age, others succumb about the time of the change of life or pass into actual insanity. Treatment. — The treatment of the onset must be in accordance with the pathological diagnosis. Hemorrhage, embolism, thrombosis, encephalitis have their own particular lines of treatment. In febrile cases ice bags, antipyretics, cathartics, etc., are all in order. The treatment should be carried out on general principles. Rarely, h<>w ever, is the practitioner called upon to treat the early onset of the trouble. As a rule lie is consulted only when the paralysis lias been some time in existence. Mechanical and pedagogic treatment 1^ about all that can be in stituted. There is no medicine with which to combat the changes in the central nervous system. 'Tonics are all that ran be thought of in the way of medicinal agents. Daily massage, electricity and pass 864 THE NON-NEURONIC DISEASES movements may be employed for the paralysis. Epilepsy is to be treated as usual with the careful administration of the bromides. This symptom, like the chorea and athetosis, is not very amenable to me- dicinal management. Contractures should be overcome by stretching, forcible replacement, splints, tenotomy and other well-known orthor- psedic and surgical procedures. Education, discipline, gymnastics and systematized exercises can sometimes accomplish very gratifying results in cases in which the intelligence is preserved. I would warn against over-exercise in car- rying out this line of treatment. Exhaustion must be guarded against. With this single, proviso, it may be truthfully said that these patients cannot be encouraged too much to use and use systematically as well as they can their paralyzed limbs. Trephining and craniectomy as suggested by Lannelongue have been tried in some of these cases ; clots have been removed and cysts have been evacuated. The ultimate results have not been sufficiently favorable in a large enough number to pronounce definitely upon the utility of these surgical measures. Further experimentation is needed. ENCEPHALITIS. By the term encephalitis is meant an inflammation of any part of the encephalic mass, particularly the gray substance. The inflam- matory process may be acute or chronic, diffuse or focal, suppurative or non-suppurative, simple, toxic, exudative and hemorrhagic. It may be primary and limited to the gray substance, or it may be secondary and associated with a meningitis or other inflammatory process. There are many forms of encephalitis, therefore, some of which will now be dis- cussed, while others will be considered in other chapters. Chronic menin go- en cephalitis, or dementia paralytica, is of suffi- cient dignity to have a chapter by itself. The encephalitis sometimes found in connection with chorea insaniens, acute mania, delirium tremens, the maniacal form of exophthalmic goitre and hydrophobia, will be noticed when these diseases are discussed. The spastic paralysis of childhood is in some instances doubtless due to a foetal meningo- encephalitis, while the hemiplegia of children has been thought by Striimpell, though not by others, to be the result of a polio-encephalitis, a sort of homologue of poliomyelitis. Indeed, after infectious diseases there sometimes is found a diffuse encephalitis, which constitutes a part of a disseminated my elo- encephalitis. Disseminated encephalitis is the name sometimes given to the cerebral form of multiple sclerosis. Syphilitic encephalitis will be noticed under the head of syphilis of the nervous system. When treating of the ocular palsies and of diseases of the medulla oblongata respectively, mention will be made of superior and inferior hemorrhagic polio-encephalitis. It will be observed that these forms of encephalitis are either parts of a larger inflammatory process, or are so focalized as to be dis- cussed to a better advantage elsewhere. In this section two of the principal forms of the disease only will be treated of in detail — namely, the acute non-purulent hemorrhagic THE XOX-XEURONIC DISEASES 865 encephalitis and the acute and chronic purulent encephalitis, or cerebral abscess. ACUTE HEMORRHAGIC ENCEPHALITIS. This is a scattered, focal, non-suppurative inflammation of the brain, characterized especially by spots of intense congestion with capillary hemorrhages. Etiology. — Though many causes have been credited with this disease, it is now pretty well established that some form of infection is the cause in the vast majority of cases. Influenza seems particu- larly blameworthy, many cases always occurring during an epidemic. It has followed measles, scarlet fever, pneumonia, erysipelas, pertussis, mumps and diphtheria. It has been seen in the victims of an ulcerative endocarditis. According to Osier, the most typical encephalitis ac- companies the meningitis of cerebro-spinal fever. Dana adds typhoid and typhus to the causative infections and says that it may occur in connection with the puerperal state. It is an open question whether it can result from acute alcoholism without coincident infection. Sun- stroke, Dana believes, has produced it, while some observations would seem to indicate that it may follow simple traumata. It is not im- probable that the traumatic encephalitis may be different from the infectious, so little is known about it. It is a common observation, however, that a simple contusion or local shock often serves to estab- lish a nidus for the bacteria or other microorganisms of a latent infec- tion to begin their work in. Pathology axd Pathogenesis. — The disease process is seen post mortem as an acute inflammation of the exudative and hemorrhagic type. The capillaries are engorged; the tissues are hyperaemic, swol- len and moist; the vascular sheaths are distended with fluid: there is an infiltration of leucocytes ; and in the older cases there are granu- lated cells and proliferated gliomatous cells. There is irritation and degeneration of the nervous elements. All of which arc seen to be limited to more or less circumscribed areas scattered about in the cere- bral cortex, central ganglia, centrum semiovale and cerebellum. Some- times these extend into the medulla and pons, giving rise to the form of the disease known as acute bulbar myelitis and acute inferior polioencephalitis. The process may even reach as far down as the cord, thus involving the whole cerebro-spinal axis and giving rise to the disease known as disseminated myelo-encephalitis. According i«- Oppenheim, a combination of encephalitis with acute anterior polio myelitis has been observed, and it was Strumpeirs idea that tin- acute encephalitis, which he believed to be the cause of infantile cerebral paralysis, was in some way related by analogy with inflammation <»t' tin- anterior cornua in infantile spinal paralysis. Many observers have noted a sinus thrombosis with this encephalitis, while in severe > Oppenheim has found a marked ventricular exudate, meningeal hyper semia, etc. When the procr mild one with recovery, minute sclerotic foci may be left in the brain. In the severer cases an extensive softening may occur and larger hemorrhages, even terminating in a massive apoplexy, as once seen by Dana. 866 THE NON-NEURONIC DISEASES Symptoms. — This disease is most frequently observed in girls and children of tender years. They rarely complain of any premonitions, for the headache, the malaise, the mental apathy may be the result of the infection rather than of the encephalitis. The latter usually an- nounces itself abruptly by a chill, a sadden rise of temperature and a stuporous, semiconscious condition that closely simulates an apoplectic attack. Unlike apoplexy, however, the pupils react to light, the re- flexes remain unaffected and the coma is not so deep. The pulse be- comes quick and feeble and the respirations are rapid. Unlike menin- gitis, there is no stiffness of the back of the neck and no signs of a generalized cortical irritation. In many cases the coma rapidly deep- ens and in twenty-four or forty-eight hours the patient is dead. Sometimes the disease runs a milder and more protracted course of several weeks, with remissions and exacerbations. In such cases there may be a temporary clearing up of the mental stupor, but there will be left a monoplegia or a hemiplegia. Aphasia is not unlikely to occur and even cortical epilepsy and hemianopsia. In some cases optic neuritis was seen. The location and nature of the lesions are various and hence there is no strict uniformity in the clinical picture. Diagnosis. — Meningitis is the disease with which hemorrhagic encephalitis is most frequently confounded. Sometimes it is absolutely impossible to make a differential diagnosis. In encephalitis, however, the absence of the irritative signs, like the muscular rigidity of the back of the neck, the normal pupillary reaction, the absence of Kernig's sign, the absence of cutaneous hyperesthesia, involuntary cry with the presence of paralytic manifestations, epileptiform attacks and a history of infection, will give much assistance. The differentiation of a tuber- culous meningitis might be made by lumbar puncture and the discovery of a tuberculous process in the lungs or bowels. Prognosis. — The prognosis is grave, but not necessarily bad. Re- covery occasionally occurs with a convalescence lasting for weeks, months and sometimes years. Rarely does recovery take place devoid of all permanent defect. A hemiplegia, multiple sclerosis or mental deterioration may be the after-clap. Treatment. — Rest, both mental and physical, absolute rest, is the best that can be done in the way of treatment. In the beginning active purgation with calomel, croton oil and stimulation of the kidneys are indicated. Cold compresses and an ice-bag to the head, venesection, leeches all constitute good routine measures. The feet may be kept in hot water. Stupor with a high fever would indicate the need of aconite. Strychnia might be used in asthenic cases. In all classes of cases the nourishment should be kept well up and the excessive pain should be controlled with morphine. Chloral and the bromides may be used to combat sleeplessness and irritability. Oppenheim says that in one case that recovered calomel in large doses — even to the point of producing stomatitis — was used. There is nothing to be said particu- larly of the after-treatment and the management of the convalescence. It is about the same as it would be in a case of ordinary cerebral hem- orrhage. THE NON-NEURONIC DISEASES $6/ ACUTE SUPPURATIVE ENCEPHALITIS. (CEREBRAL ABSCESS.) Idiopathic brain abscess is practically unknown, though at rare intervals a case is observed in which no cause can be found for the suppurative process. It is probable that in such cases a direct cause was present sometime, but so long ago or so unobtrusive was it that it had been quite forgotten. Cerebral abscess is then the result of an acute suppurative encephalitis, focal or diffuse, produced by microor- ganisms introduced into the brain from without. Etiology. — Acute suppurative encephalitis is always the result of bacteria that have gained entrance into the brain substance in three general ways. It is etiologically due, therefore, to direct injury of the head, to extension of a suppurative process in the tissues immediately surrounding the brain, or to a metastatic transference of the bacteria by the blood from some 1 emote center of suppuration in the body. It is estimated that about 30 per cent, of the brain abscesses are due to traumatism. A fracture or puncture of the skull is the usual form of the injury. In some cases a foreign body has passed into the brain; in others a mere contusion of the scalp has been sufficient to give entrance to the germs. The majority of the traumatic cases have been complicated fractures in the frontal and parietal parts of the head. Operations within the nasal and orbital cavities have been re- sponsible in a few instances. Bone fracture is not absolutely necessary for the admission of the germs, since infected emboli and implication of the lymphatics are fully adequate. It is clear how some of these slighter traumatic causes may readily be overlooked when it is remem- bered that sometimes the cerebral symptoms do not announce them- selves until many years, ten, twenty and thirty even, after the injury. Somewhat more than a third of the cases are due to extension of the suppurative process or the transmission of the microorganisms from chronic otitis media and disease of the mastoid cells. Of these ear cases, it is found that about 85 per cent, are due to chronic suppuration, and the remaining 15 per cent, to acute suppuration in the ear. The number of cases due to mastoid disease is less than those due to otitis media. Most of these ear troubles follow the infections fevers of childhood. They sometimes continue many years, undergo remissions, or even disappear entirely long before the evidences of brain trouble declare themselves. In a considerable number of cases I have been struck with the statement of the patient that many years before she had had a running from her cars for a time, but her physician had succeeded in stopping the discharge. Sometimes there is perforation of the tympanic membrane in these eases and even earies of the petrous bone, with periodical attacks of otorrhcea. In rare instances the suppurative process has extended from the nasal or orbital cavity; or there has been caries of the neighboring bones, such as the ethmoid, sphenoid, superior maxilla. The distant sources of infection are numerous and varied. [n 868 THE NON-NEURONIC DISEASES some way by metastasis the germs are carried to the brain from the most remote parts of the body. Even a purulent osteitis or arthritis may in rare instances be the origin of the brain abscess. It is stated that about one-fifth of the cases are due to pyemic emboli from the thoracic cavity. Bronchiectasis, empyema, pulmonary gangrene or abscess, purulent pericarditis, ulcerative endocarditis, have been fol- lowed by brain abscess. The latter has been attributed to abscesses in the abdominal viscera, to actinomycosis and to simple thrush. Tubercle bacilli and pneumococci have been recovered from it. In these cases the abscesses are apt to be small and multiple. Cerebral abscess occurs more frequently in males than in females, and oftenest between the ages of ten and thirty. Pathology and Pathogenesis. — Abscesses may occur in all parts of the brain and are usually associated with signs of traumatism, ear trouble or remote suppurative foci. They may be outside of the dura {extradural), inside of the dura (subdural) , in the cortex or in the cen- trum semiovale. Multiple abscesses, as many as a hundred having been counted in one case, may be no larger than a pea; simple abscesses may attain the size of an orange, or even occupy one whole hemi- sphere. Cerebral abscesses are usually single ; cerebellar are more apt to be multiple. As a rule the abscess lies just beneath the seat of injury; occasionally, however, it is found on the opposite side, the result of the contre-coup. Traumatic abscesses are largely extradural and are accompanied by meningitis. They are more frequent in the frontal and parietal lobes, as the front of the head is most exposed to blows. Otitic cerebral abscesses are found usually in and just beneath the gray cortex of the temporo-sphenoidal lobe on the same side as the affected ear. Abscesses due to mastoid disease are generally located in the cerebellum. In the pons and medulla they are rarly seen. Chronic abscesses are round in shape and encapsulated within a fibrous wall, which takes about four weeks to form. There is nothing unique about the formation process of these abscesses. The usual pyogenic microorganisms are found within them, especially the streptococcus pyogenes, the staphylococcus pyogenes aureus, albus and citreus. They undergo the usual transformation of red and yellow softening, with crowding of the leucocytes, infiltration of inflammatory exudate, destruction of nerve cells and processes, ap- pearance of granular corpuscles, etc. Symptoms. — The symptoms of brain abscess arrange themselves into three groups — namely, those of a general character due to the presence of a septic process, those due to intracranial pressure from the presence of the abscess, and those that depend upon the particular location of the abscess within or upon the encephalic mass. In the history of every cerebral abscess, especially if it is clonic. practically all of these three groups of symptoms have been more or less in evidence sometime or other. They may not all be present simultaneously, and the last or localization group is the one most fre- quently absent. Moreover, they do not all exist with the same degree of prominence. In one case the septic symptoms may be the most prominent, as is often the case in the pyaemic metastatic multiple THE NON-NEURONIC DISEASES 869 abscesses ; in another case the pressure symptoms may be the only markedly noticeable ones ; in a third case there may be distinct local- ization symptoms, but the septic and pressure signs may be so slight or evanescent that one hesitates to diagnose the neoplasm as an abscess. It is calculated that in about 8 or 10 per cent, of cases the symptoms are so slight that they are overlooked entirely and the abscess is not discovered until after death. With all this variability and uncertainty about its symptomatology, a brain abscess can pretty nearly always be strongly suspected, if not absolutely diagnosed, if the history and physical examination are gone into minutely and conscientiously. This applies with special force to the early diagnosis of the trouble and is of paramount importance because the earlier the diagnosis is made the brighter is the hope from surgical intervention. Strictly speaking, there are no prodromes, but what are sometimes called prodromes are early symptoms in one case or even late symptoms in a mild or latent abscess. A slight headache, a feeling of malaise, oc- casional chilliness, attacks of cardiac palpitation, sudden and unac- countable slight elevations of temperature, certain fugitive vasomotor manifestations, occasional attacks of insomnia, an annoying degree of slight mental hebetude with a feeling of depression and anxiety — in a word, all the signs of a mild form of toxcenne neurasthenia — may con- tinue for months and years before more definite indications declare themselves. I saw a case that misled, by reason of the indefiniteness its clinical manifestations, many diagnosticians for seventeen years, during which period the patient had been a number of times suspected of being insane, being sent each time to an asylum and discharged as cured after several weeks. When T saw her the clinical picture was clear enough to warrant a positive diagnosis of abscess, from which the patient finally died in coma several days later. It is difficult to say usually how much of these early symptoms are due to the sepsis, especially if there are other septic foci elsewhere in the body, and how much to the cerebral neoplasm alone and its non- septic disturbances. The headache, for instance, in these circumstances cannot always be assigned to the toxic disturbance or the pressure. Such a group of manifestations, neurasthenic and phrenasthenic in character, if associated with a history of hearl injury, abscess t'<>ci else- where and especially ear trouble, should always put the physician on his guard and awaken a lively suspicion of a possible cerebral abscess. It is in the acute abscesses, following wounds about the head, and in the exacerbations of the chronic abscesses from the other c.c that the constitutional septic symptoms make themselves particularly obvious. They are the same as symptoms that occur in all septic poisoning — namely, chills, variable temperature, anorexia, coated tongue, constipation, headache, dullness of mind, general feeling malaise and emaciation. A general cachectic condition with icteric discoloration of the skin, loss of appetite, irritability, ness and mental confusion sometimes obtain. Tin- fever is vn\ irregular and slight. In some cases it is entirely absent. In no case does it i last verv long. Subnormal temperature is », , frequent as t<» be a &7° THE NON-NEURONIC DISEASES highly suggestive symptom. The pulse may be rapid and unsteady; often it is very slow, beating only 40 or 30 to the minute. With dis- turbance of the pulse and sudden elevation of the temperature the headache increases in severity. The respiration is sometimes irregular and slow. The pressure symptoms of brain abscess are much the same as those of any form of intracranial growth. They are headache, mus- cular twitching and rigidity, local and generalized convulsions, epilepti- form phenomena, paralysis, vertigo, vomiting and mental stupor. Con- traction and dilatation of the pupils, disturbed vision, optic neuritis, tinnitus aurium and other symptoms dependent upon the cranial nerves sometimes occur. The subnormal temperature, retarded pulse-beat and respiration and certain vasomotor disturbances are in part due to the intracranial pressure. Of all these pressure symptoms the headache is probably the most reliable. It is generally constant and severe and definitely localized by the patient. He usually points to a particular spot on the head, not unfrequently behind the ear or in the occipital region, percussion upon which causes him to wince. Sudden jars and disturbances of the heart action, as well as the onset of a febrile exacerbation, cause the headache to become almost intolerable. The same result follows a sudden flash of light or a sudden noise. Though more or less constant, the pain is often worse at night, causing insomnia. Partly on account of this constant pain and partly on account of the pressure and general septic condition, the patient becomes extremely melancholy, depressed, apathetic and stupid. Occasionally marked delirium occurs and passes into coma, which in turn terminates in death. Sometimes the headache diminishes and I have known it to vanish entirely after a time without any disappearance, however, of the other suspicious signs of abscess. Not always do these headaches indicate the location of the abscess. It may occur that the headache is frontal, while the localization symp- toms point to an occipital abscess. A characteristic of them having some diagnostic value is that they are usually unrelieved by medica- tion. Vertigo and vomiting are frequent symptoms, the latter especially so in cerebellar abscess. Round about the abscess minute hemorrhages occur. These being more marked in the early stage of the process, they, with the abscess itself, give rise to irritative symptoms, followed sooner or later by paralysis. Hence in cortical abscesses there are epileptic phenomena. focal and generalized, with monoplegic symptoms. In this irritative stage it is hard to determine at times whether the symptoms are due solely to the abscess or to a meningitis. Not unfrequently such abscesses are accompanied by a local meningitis, and then the symp- toms will be of a combined character. It is in the traumatic abscesses that the meningitis mostly occurs and the irritative symptoms are the more prominent. Among the pressure symptoms the optic neuritis, when present, is a most valuable one. It is of the so-called choked disc variety, Knies* obstructive optic neuritis. It is never so marked nor is it so con- THE NON-NEURONIC DISEASES 87 1 stant as it is in cerebral tumor. It is believed that ocular symptoms, including the motor and pupillary, are to be found in about half of the cases. Witherspoon asserts that cerebellar abscess gives rise to optic neuritis in from 75 to 80 per cent, of cases, whereas cerebral abscess is responsible for it in only about 30 or 40 per cent. The focal symptoms are of much consequence, though unfortu- nately they are often too indefinite. Abscesses lying in the so-called latent parts of the brain cannot, of course, be localized by any focal signs. Traumatic abscesses of the frontal lobes are usually devoid of localizing symptoms unless they are large or situated in the posterior part of the lobe, especially on the left side. By encroaching upon the precentral convolution, the base or near the posterior part of the third frontal, they may cause, along with psychic confusion and other mental disturbances, Jacksonian symptoms, aphasia, parosmia and ataxia. Hemiplegia or monoplegia may occur with abscesses located in and about the cortical Rolandic region. The metastatic embolic abscesses from septic foci in the thorax are apt to form in the area sup- plied by the Sylvian artery and give corresponding focal signs. The otitic abscesses are usually single and form in the tem- poro-sphenoidal lobe. The hearing consequently is affected. There is mind-deafness, auditory aphasia, and possibly by pressure amnestic aphasia and oculomotor paralysis, the frequency of which in this form of abscess has been pointed out by Wernicke. These abscesses when deep affect the underlying motor, sensory and optic conducting tracts. Hence there may be opposite-sided hemiparesis with rigidity or con- tracture. Or there may be hemiansesthesia and hemianopsia. In a case observed by Jansen and Oppenheim there were from a right-sided temporal lobe abscess left hemiparesis, hemihypaesthesia, hemianopsia bilateralis sinistra and conjugate deviation of the head and eyes to- ward the right. Ptosis from paralysis of the third nerve and internal strabismus from the sixth are rather common in temporal lobe abscesses. Blindness may occur when the abscess is in the occipital lobes, while hemianopsia occasionally accompanies abscesses of the occipital lobe and of the lower part of the parietal lobe. Cerebellar abscesses are usually due to mastoid disease and de- clare their presence by such localizing symptoms as ataxia, suboccipital headache, vomiting, cervical rigidity and rarely by bilateral amaurosis and pressure on the adjoining cranial nerves and their nuclei. Ac- cording to Macewen, the knee-jerks were absent in some eases ^\ cerebellar abscess. Abscess of the pons and medulla oblongata is very rare, yet when present has the typical bulbar symptoms to complete its clinical picture. Occasionally an abscess bursts into the ventricles, giving all the symptoms of a profound apoplectic stroke; namely, convulsions, fol- lowed by paralysis, deep coma, etc., all quickly terminating in death. Diagnosis. — In spite of the fact that cerebral abscesses an often latent and therefore cannot be diagnosed at all. and even when they have symptoms these are so often indefinite and variable, the gen era'l clinical picture is sufficiently unique and characteristic to enable 872 THE NON-NEURONIC DISEASES one in a large majority of cases to form a diagnosis, or at least a strong suspicion, especially when there has been a history of trauma- tism, local infection in the ear or septic processes elsewhere about the body. It is upon the history of the case, with the present symptomatol- ogy viewed en masse, rather than in detail, that the strongest diagnosis of cerebral abscess is made. It is the tout ensemble of the picture that here indicates the nature of the trouble, and not this or that particular symptom or small group of symptoms. There are quite a number of diseases with which brain abscess might be readily confused. This is because of the multiplicity of its symptoms, all depending upon so many different factors. From meningitis, particularly external purulent pachymeningitis or extra- dural abscess, it is distinguished by the greater degree of intracranial pressure, the more definite localization of symptoms and the absence of the characteristic irritative signs like the cervical rigidity, pupillary contraction and Kernig's symptom. In diffuse purulent meningitis, differentiation is often extremely difficult. The irregular and low fever, the slow pulse, the localization signs and the absence of most of the irritative symptoms of meningitis, such as the hyperesthesia, the muscular rigidity, the twitchings, the spinal symptoms and the mental restlessness are enough to make in typical cases a differential diagnosis easy. Lumbar puncture will assist in some cases. Other forms of meningitis that must be eliminated in the diagnosis are a localized meningo-ence phalitis and serous meningitis and tubercu- lar meningitis. In typical cases the differentiation is not difficult when one recalls that abscess produces only temporary or transient irritative symptoms, that its febrile characteristics are quite unlike those of meningitis, and that it has, like tumor, certain more or less definite intracranial localization symptoms. In the combined cases it is diffi- cult, but if the case is a combined one we can neglect the meningitis and diagnose the abscess, or in other words, a differential diagnosis is not imperatively called for. The distinction between brain abscess and the traumatic neuroses, especially when the former is due to a traumatism, must be carefully made. A careful consideration of the symptomatology of the traumatic neuroses, hysteria and neurasthenia, and its unlikeness to that of abscess, with its distinct organic and septic symptoms, ought, it seems to me, to make a differentiation here a matter of no very £reat difficulty. Tumor of the brain has many symptoms in common with those of abscess. In tumor, how r ever, the headache and optic neuritis are more constant and pronounced, the development of the disease is slow- er, there is absence of fever and septic symptoms and altogether more of a uniformity, steadiness and chronicity about the clinical picture. Prognosis. — Brain abscesses are both acute and chronic. Both forms are sooner or later fatal if not interfered with surgically. It is a rare thing indeed for an intracranial collection of pus to break into the nasal, orbital or buccal cavity ; still more rarely does it open out directly on the head. A spontaneous cure by this or any other means is not to be looked for. These cases are as fatal ultimately as any. THE NON-NEURONIC DISEASES 873 Cerebral abscess often runs a long course, with occasional remis- sions and changes in its symptomatology. When single and encap- sulated it may remain in a semi-quiescent state many years. Then it suddenly bursts into the neighboring ventricle or through the mem- branes and brings on alarming symptoms, with delirium, coma and death in a few hours. I have seen death follow a semicomatose condi- tion lasting several days. This coma is a certain indication of an early demise. Without interference, therefore, the prognosis of cerebral abscess is bad. With surgical intervention a little more than half the traumatic cases, and a little less than half the otitic cases, are saved and cured. Of course, the resulting scar tissue and damaged cerebral cells will in all probability leave some focal symptoms of a permanent character. Occasionally an abscess unfortunately recurs after a successful opera- tion. The most favorable location for an operable abscess is the tem- poral lobe ; the cerebellum is somewhat less favorable. Treatment. — The treatment cf brain abscess resolves itself into prophylaxis and surgkal evacuation. In the antiseptic management of fresh wounds about the head much can be done to ward oft intracranial infection. This is an added argument in favor of the maintenance of rigid asepsis in all operations in the nose and mouth, about the eye and ear. Forcible injections and irrigations, especially in the ear, must not be resorted to. Pus when present must be drained oil, and under no circumstance must a running from the ear be checked by closure of the opening. I repeat that the mere cessation of an otorrhoea has seemed to me in a number of cases to have been too fondly assumed as a cure of the ear disease. When an abscess of the brain is finally diagnosed, and its location is favorable for operation, there should be no further temporizing, but immediate evacuation with v drainage should be insisted on. There is no other treatment for an abscess. I cannot see the wisdom cf Oppenheim's suggestion to first try if removal of the pus in the tar or in the extradural region will relieve the cerebral symptoms in a where the diagnosis of uncomplicated otitic cerebral abscess can be definitely made. As a preliminary measure these operations will be done anyhow, and if the cerebral symptoms are thereby relieved in part, it is no proof that a cerebral absce-- does not exist. Therefore instead of recommending a measure that might readily be interpreted into meaning a delay, it seems to me the better part of wisdom to advise that every case of otitic cerebral abscess, where a diagnosis can be definitely made and where the location of the abscess is favorable for operation, should be operated at once or as early as possible. In view of their almost certain fatality without operative measures, I do n<>t feel that external purulent pachymeningitis, circumscribed purulent meningitis, diffuse purulent meningitis, sinus thrombosis or even beginning pyaemia should be looked upon as contra-indications t-> operative interference. The patient's life is never safe so long a- there is a nidus of pus within his cranium; it is safe in a large proportion i when that nidus of pus is opened and drained. Some operators report recovery by surgical \\^ in the 874 TH E NON-NEURONIC DISEASES cases of metastatic multiple abscess. Such cases, however, must nec- essarily be very rare. Moreover, so often is the original source of in- fection incurable that it would seem to be an almost useless task to attempt to cure, under such hopeless conditions, the trouble in the brain. ACUTE DELIRIUM. BELL'S MANIA. So varied are the opinions in regard to the nature of this brain disease that many names have been applied to it. The one that I have here selected is the one most commonly used. In the form described by Bell, and most of the authors who have followed him, there are no constant, distinctive post-mortem findings. Ford, Wood and others believe that it is a type of inflammation and should therefore be classi- fied as a menin go -encephalitis, or a periencephalitis. I am inclined to classify it with the brain inflammations in spite of the fact that the microscopical findings are negative. The symptoms are those of a profound intoxication, autochthonous or otherwise, and in a few cases which have been carefully examined signs of a diffuse inflammation have been discovered. The poison, either by reason of its extreme virulence or the peculiar susceptibility of the patient, so overwhelms the brain cells in some cases that death ensues before histological changes have had time to take place. In other cases by reason of the weaker character of the poison, the longer con- tinuance of its action or the greater power of resistance on the part of the patient, histological changes of a diffuse, inflammatory character are able to develop and so are revealed post mortem. Therefore, as in the analogous instance of Landry's paralysis, it seems to me that in Bell's delirium we are witnessing merely an early stage of a high de- gree of intoxication, which, if granted time to pass into a later stage, without the sudden intervention of death, would frankly declare itself as a form of toxic, diffuse encephalitis. This will be more clearly brought out in the further discussion of the disease. Etiology. — Most authors find that women are much more subject to the trouble than men, and that it occurs most frequently between the ages of twenty-five and forty-five. It occurred in a girl of eleven years of age in a case of Morel, quoted by Maudsley. Heredity does not seem to play a very prominent role. Emotional strain, distress, anxiety, worry, mental shock are more important as causative factors. Schule has attributed it to severe physical pain. Sunstroke, injury to the skull and the climacteric have been mentioned in its etiology ; to these must be added alcoholism, of course. More significant than any of these causes are those that involve infection. It has followed typhus and typhoid fever, epidemic, influenza, erysipelas and some skin affections. It has accompanied exhaustive dysentery, puerperal sepsis and epileptic attacks. Wood, Hurd, Berkeley, Dercum, Briand, Rohe and others consider a toxaemia or infection to be the primary cause, if not the essential cause in all cases. Pathology and Pathogenesis. — Some authors, as, for example, Pritchard, affirm pathology shows that under the name Bell's disease we have heretofore been describing two fundamentally different, though THE NON-NEURONIC DISEASES S75 symptomatically similar, conditions of disease ; the one organic, with a variable but always demonstrable pathology, essentially inflammatory. due to toxines, infection or trauma, the lesion varying from a gross menin go-encephalitis to limited lepto-meningitis or arachnitis, the other non-organic, or at least with findings indicating simply nutritional or dynamic changes without adequate or other than conjectural ex- planation either as to their etiological nature or pathological signifi- cance and interpretation. Most authorities would assume that this second, non-organic class was due to an intoxication. If this be so, there is no difficulty in recognizing both classes of cases as but two iypes of the same disease, for they agree in everything except the pathological findings, and in respect to this discrepancy the case is the same, as we have seen in Landry's paralysis and certain inflammatory conditions of the cord. The so-called non-organic cases are but the earlier stages of an overwhelming intoxication, which, given more time, less virulence or other conditions, would go on to provoke distinct gross signs of a diffuse inflammation. Granting the frequent con- founding of other forms of inflammation, such as traumatic meningitis -and encephalitis, with this disease, the findings are yet sufficiently sug- gestive of an incipient inflammation in the later stages of Bell's delirium. There is congestion of the vessels of the meninges and of the cortex and engorgement of the veins. In two cases examined by Osier there were perivascular exudation and leucocytes in the lymph sheaths and periganglia spaces. In Pritchard's case the pia mater was markedly cedematous and the blood vessels injected. The capil- laries of the cortex showed marked distention, but no petechial hem- orrhages were observed. The choroid plexus of the ventricles was cedematous. The cells were practically unchanged. ( )sler urges that a careful examination in fatal cases be always made of the lungs and ilium. In the majority of cases he says there is engorgement of the bases of the lungs and even deglutition pneumonia. I found this in a recent case of my own. Tnough the nature of the disease is thus not positively demonstrated, the findings point strongly to a condition of acute, severe infection. SYMPTOMATOLOGY. — The symptoms of this disease are almost en- tirely mental, which doubtless accounts for some of its names, as well as for the fact that it has been so long regarded as a pure psychosis. There are, in almost every case when closely studied, seen t<> he symp- toms that point to organic lesions and disturbances in the brain cortex. The abruptness of onset, the rapid advancement, the extreme violence, the utter incoordination and unsystematized character of them, their frequent remission, and their ultimate subsidence in death or dementia are the striking traits about the mental manifestations. The patient will be resting quietly when all of a Midden there will be a terrific outburst of wild delirium and intense frenzy. This i^ hys tero-maniacal in general character and is associated with hallucina- tions, great violence and motility, destructiveness and incoherence. Chattering and talking is incessant. The speech is absolutely uncoil trolled SO that obscene, boastful. lasci\i«uis ideas air voluminou poured forth. Laughing and screaming, alternating with wild singing 876 THE NON-NEURONIC DISEASES occur, all without the slightest trace of intelligence. The patient has been compared in this state to a maddened animal. The muscular movements are intense and constant until exhaustion puts an end to them. Vasomotor disturbances are obvious. Sleep is impossible.. Anesthesia and analgesia are apt to be complete. Food is obstinately refused. On account of all this the patient soon becomes utterly pros- trated and remains quiet with a more or less complete return to normal, consciousness. In a short time, however, another exacerbation comes on and the noisy, terrific scene is repeated. These remissions are a. striking feature of the disease. They are transient and become shorter and less frequent as the disease progresses. In them the patient may seem to have recovered complete health and for this reason they often give rise to false hopes. Pritchard says that delusions occur, especially in connection with the visual sense, and that strange and familiar per- sonalities arise. Shameless masturbation is practiced and attendants of the opposite sex are openly solicited. Sometimes in the speech there is observed a certain connected sequence and almost coherence, but the usual, absolutely unsystematized character of the ideas helps to dis- tinguish this trouble from a typical mania. Proper notions in regard to time, place and surrounding conditions are as a rule completely lost. Death almost always occurs in this disease and in the acute cases in about a week ; when partial recovery takes place the delirium is suc- ceeded by dementia, which remains more or less permanently. Such is the mental picture of these cases, but none of them are ever absolutely devoid of constitutional and somatic symptoms. The temperature usually rises rapidly, especially during the frenzy, and may attain to 105 degrees F. The pulse is variable, though it is always more or less disturbed. Both the temperature and the pulse as a rule remain above the normal. Convulsions and epileptiform manifesta- tions sometimes occur, as well as strabismus and various paralyses. Even more suggestive are the not unfrequent trophic manifestations,, such as alopecia, degenerative atrophy, petechias and other lesions of the skin and bones. Even bullae and pustules, with sores from abrasions and injuries, are frequently seen on the skin. The tongue is dry and apt to be coated. Sometimes the sphincters are relaxed, causing in- continence of urine and faeces. Just before death the bodily emaciation is apt to be extreme. Obviously we have here two fairly well marked sets of symptoms, the mental and the somatic. The latter are not known to be the direct results of the former under any other circumstances, though the for- mer are frequently caused by the latter. Therefore it would seem to- be a fair conclusion to draw that a profound toxic condition underlies the disease and is the cause of both sets of symptoms. Diagnosis. — Typical cases of Bell's mania do not: give much trouble in diagnosis. The febrile condition, the wild delirium with re- missions, the hallucinations, the extreme motility, the rapid course,. intense emaciation and somatic symptoms are characteristic. In sim- ple mania there are no somatic symptoms, very little febrile disturbance, if any, and the ideas are more systematized and coherent. In delirium tremens there is a clear history of alcoholism, much gastric disturbance* THE NON-NEURONIC DISEASES 877 peculiar visual hallucinations and a general and marked asthenia. A meningitis is accompanied by more or less nuchal rigidity and opistho- tonos, pupillary symptoms, motor spasm and paresis, vomiting, con- tinuous high fever, absence of complete remissions, Kernig's sign and the tdchc . Pigment. (After Heubner.) and not infrequently the brain will remain intact whih- many of its arteries show syphilitic arteritis. It is composed of a cellular, highl) vascular granulation tissue. It is never or rarely purulent, though in spots it may be caseated or fibrous. A round-cell proliferation extends directly along the adventitia of the blood vessels and also along the epineurium of the nerves. Between the bundles of the nerve fibres the thickened and infiltrated epineurium sends its pi everywh 882 THE NON-NEURONIC DISEASES like connective-tissue septa. The nerve itself thus becomes swollen, while the individual fibres undergo atrophy- In hereditary cerebral syphilis the lesions are not unlike those of the acquired form, though probably less intense. To sum up, the lesions of both hereditary and original brain syphilis involve the lymphatics, capillaries, veins and arteries. Endarteritis is the lesion par excellence. This may involve the dura or the pia, more especially the latter. A gummatous new-growth is formed, and as a result dural and pial gummata with gummatous leptomeningitis are seen. Focal and diffuse encephalitis with hemorrhages, necrobiosis from obstructive disease of the arteries and veins, and fibroid indurations from chronic vascular disease are all liable to occur. The wide extent, variable character and changeable nature of these pathological findings make clear the reason of the great diversity, variability and swift changes in the symptomatology of brain syphilis. Symptoms. — I sometimes teach that among organic diseases the clinical picture of brain syphilis is the homologue of hysteria among the psychoneuroses. Brain syphilis has such a wide range of symp- toms that it can simulate almost every other known form of cerebral disease. Somewhat like hysteria, its symptoms are remarkable for their appearance and disappearance, their transference from one part of the body to another and their involvement of all known functions of the nervous apparatus, sensory, motor, vasomotor and trophic. As the entire encephalon is liable to the disease, and as the disease process (soft and easily broken-down gummata, hemorrhage, emboli, etc.) itself is so inconstant in its minute manifestations, one can readily com- prehend how the symptoms or outward signs of these changing and shifting pathological phenomena should also be changing and shifting. It would be quite an impossible, or at least an herculean, task to attempt to classify the symptoms of brain syphilis. And yet there is a certain constancy and uniformity in their appearance and group- ing, so that after a time one comes quickly to recognize the clinical pic- ture as being more or less characteristic. Certain distinctive features belong to the symptoms in such a way that we can group them somewhat. The symptoms indicative of cen- tral irritation are quite distinguishable from those indicative of de- struction of the central nervous tissue. Sometimes these two classes of symptoms exist simultaneously in different parts of the nervous distribution ; at other times one or the other set alone predominate. Usually they exist in the same parts, the irritative being followed by the destructive signs. Epilepsies and stupor, delirious and comatose states, paresthesia and hypaesthesia, spasms and pareses, all thus alter- nate or occur together in the same patient or follow one another with remarkable persistency. Again, the symptoms may be subdivided into those that are due to the gummatous meningitis involving the gray matter of the cortex and the basal nerves in the irritative and destructive process, and those that have to be attributed to the closure of the circulation by thrombotic and embolic obstructions or to minute hemorrhages and inflammatory foci. The former are apt to be more focal and localized THE NON-NEUR0N1C DISEASES 883 in character and implicate special nerves and individual functions ; the latter are rather hemiplegic in character and are diffuse., spreading: over segments of the body known to the patient's mind and not in ac- cordance with distinct anatomico-physiological arrangements of the peripheral neuromuscular apparatus. All this great variability and wide extent of the symptomatology of brain syphilis are features that are almost pathognomonic of the disease. And yet. as I have tried to emphasize before, in spite of all this diversity in the details of the symptomatology, in its general manifestation there is a remarkable constancy, uniformity and individuality. I will now discuss the clinical picture of the commonest form of brain syphilis, basal gummatous meningitis. It must not be assumed that in these cases the disease is limited entirely to the membranes and vessels of the base. In fact, in these basal cases there is practically always some disease of the convexity and some neuritis of the cranial nerve roots. In the clinical picture, however, the symptoms of the basal meningitis predominate. These indicate irritation, compression and destruction of the neighboring nervous elements. Foremost among the general symptoms is headache. This is characteristic and peculiar. The pain is more or less generalized over the whole head, is paroxysmal and generally exacerbates with great severity at night. Sometimes it resembles the indefinite, indistinct, paresthetic sort of a headache com- plained of by neurasthenics ; at other times it is sharp, cutting and boring. It is rarely unilateral like hemicrania ; it is less definitely localized than the headache of cerebral tumor and abscess ; it lacks the dependence upon mental states like the clavus of hysteria ; and it is influenced by the stomach, the eyes and other sources of peripheral irri- tation which sometimes provoke pain in the head. It is not accom- panied by fever as a rule, though sometimes the temperature rises in cerebral syphilis. It is a sortr^of a cross between the headache of brain tumor and acute infective states; not as wearing and constant as the former ; more intense, definite and paroxysmal than the latter. / 'omit- ing and vertigo are frequent accompaniments of the cephalalgia. Con- vulsions of a generalized character, with unconsciousness, occur in periodical attacks. Between these there may be a progressive stupor as in all forms of progressive intracranial growths. Weakness of mem- ory, failure of intellect and power of attention, and general mental apathy are not uncommon. Even dementia of the true type may occur. In the midst of all this gradual obliteration of the consciousness, out- bursts of mania, severe attacks of excitation, delirium alternating with coma may be SO severe a- to require the incarceration of tin- patient ui- an asylum. The characteristic changeability of the symptomatology brain syphilis is shown in the occasional alteration of these mental! manifestations with periods of apparent mental restoration to health; The coma may recede, only to return again, however. It is obvious that the gummatous meningitic exudation is the cause of these symp- toms largely by the intracranial pressure it causes and the encephalitis it lights up. Doubtless the same explanation will answer for the casional polydipsia and polyuria that are seen in this form of brain. 884 THE NON-NEURONIC DISEASES syphilis. Tubercular meningitis of the base is easily differentiated by its general development and more or less elevated temperature. Most important are the symptoms dependent upon compression and destruction of the cranial nerve roots. The optic and oculomotor nerves are the most frequently implicated, usually somewhat more on one side than the other. The patheticus and abducens nerves are the next most frequently involved, but they are apt to be affected on one side or the other only. The fifth, seventh, eighth, ninth, tenth, elev- enth and twelfth nerves, as well as the olfactory, are all liable to be .affected, but with decreasing frequency in the order in which they are enumerated. Optic nerve trouble is particularly common, which for the purpose of diagnosis is fortunate. The ophthalmoscope, together with an examination of the vision, shows in most all cases some de- gree of atrophy. Typical choked disc and neuritic atrophy, with neuro-retinitis, are frequently seen on one or both sides. I have seen cases, on the other hand, in which the eye ground symptoms were prac- tically negative though the visual loss was certain. Almost every variety of visual disturbance may be met with, as, for example, bitemporal or homonymous hemianopsia ; amaurosis on one side with hemianopsia on the other, ultimately becoming bilateral amaurosis, which is not persistent as a rule, or complete. The visual field, when the optic nerve itself is diseased, may show an irregular, concentric contraction, decrease of central visual acuity, etc. All of these optic nerve symptoms, exclusive of the atrophy, show a remark- able degree of changeability and unsteadiness. The outlines of the contracted visual field may change from day to day ; the amaurosis may swiftly vary or change from one eye to the other ; the bitemporal hemianopsia may be of the most fugitive type. Paralytic symptoms, involving all or a part of the third nerve, are early and frequent. Ptosis on one or both sides is often seen. Sometimes one branch of the nerve gives way after another until the whole nerve is involved. Oppenheim declares, however, that a pure ophthalmoplegia, interior and exterior, is rare in basal syphilis. With involvement of the olfactory roots there results anosmia, which may be double or one-sided. I have several times seen the fifth nerve involved. This most fre- quently occurs on one side and may give rise to hyperesthesia, anaes- thesia or rather hypsesthesia of the face, with weakness in the move- ments of the lower jaw. The taste is disturbed on the anterior two- thirds of the tongue, and there is often a neuroparalytic keratitis on the same side. Hearing is affected by involvement of the auditory nerve ; at times the irritation produces most annoying tinnitus. When the facial is included, the paralysis resembles the peripheral type of Bell's palsy, without the involvement of the palate, the hearing and the taste. In other words, the paralysis partakes of the nature of a peripheral palsy, without, however, alteration in the electrical reactions. Facial diplegia occurs. The cranial nerve symptoms, various and scattered and change- able as they may be, are so constant a constituent of the clinical pic- ture of basal gummatous meningitis, that without them the diagnosis THE NON-NEUKONIC DISEASES 885 should be made with much hesitancy. It may be that for some brief period in its course basal gummatous meningitis may exist and pro- duce other symptoms than those showing implication of the cranial nerves, especially the optic and oculomotorius, but such cases must be exceedingly rare and such a clinical manifestation would probably be very brief. I have never seen either. \\ nen the gummatous meningitis is limited to the convexity of the brain of course the cranial nerves escape. Moreover, the symp- toms are quite dissimilar to those of basal disease. The headache seems to be more superficial and localized. Percussion on the outside of the skull will elicit sensitive spots or areas, especially above and behind the ear and in the occipital region. When the disease process is of wide extent, spreading over a large area, there will be epileptiform manifestations, convulsions, muscular Iremblings and slight muscular rigidity. If the motor region of the cortex is pressed upon by the gummata, aphasic manifestations or Jacksonian epileptic attacks, fol- lowed by monoplegia, will make their apearance. Eye symptoms, like choked disc may not be present until the disease is far advanced. Sometimes the motor and sensory disturbances will be of a very marked character on the opposite side of the body, such as cpilcptoid attacks, tremor, contracture and pai)is. In some cases the mind is affected by the associated involvement of the gray cortex. If the meningitis focal and scattered in character, the symptoms may be of a focal char- acter, depending upon the part of the brain and its localization func- tion, pressed upon. In this way occipital meningitis may produce hemianopsia, temporary disturbances of hearing, peripheral pains and other sensory phenomena. In the larger number of cases of brain syphilis these two forms of meningitis, that of the base and that of the convexity, occur together, though for a time one or the- other seems to predominate. Along with the meningitic symptoms are those which may be regarded as in a way secondary to the disease in the isolated vessels. In the larger vessels, especially those of the base, like the basilar artery and its branches, an endarteritis is the particular form of lesion present, while in the smaller and terminal capillaries of the cortex it is more of the nature of a periarteritis. In these cases thrombi form and emboli float off and occlude the circulation: or the thickening of the arterial walls brings about almost the same result by the narrowing of the calibre of the vessels. The location and size of the artery thus affected will of course determine the character of the symptoms, but these will partake of the nature of those of an encephalomalacia. When the softening is at the base, the great symptom is a p>. hemipL which, however, may come and go, first in the leg. then the arm and finally the face, several times before- it becomes permanent. The and mental functions as a rule remain intact. Premonitory indi- cations, like twitching, often precede the hemiplegic attack: on the other hand, the parah sis may conic on most unexpectedly and p away entirely in a few hours or days. 1 saw a case where tin- patienl had to take to his bed. and yet the next day lie was walking around almost as well as if nothing had happened. Speech \ ted, hut 886 THE NON-NEURONIC DISEASES there was no loss of consciousness. This hemiplegia is, of course, on the opposite side from that on which the affected artery rests. As Oppenheim well points out, the laws of localization for crossed hemi- plegia (superior and inferior) must be used with some caution in brain syphilis. Oculomotor paralysis of one side, and hemiplegia of the other, are here sometimes caused, not by a focal peduncular disease, but by the very conditions of the basal meningitic process. Involvement of the basilar and vertebral arteries give rise to pontile and bulbar symp- toms. When the arteries higher up are thus obstructed, the well- known apoplectic manifestations occur, such as prodromes followed by extensive hemiplegia, hemianesthesia, aphasia, hemianopsia. The syphilitic nature of these attacks is indicated by the headache and ver- tigo that long precede them, by the fugitive character of the apoplecti- form outbreaks, the stupor, mental confusion, and dementia that follow them. With these forms of brain syphilis, especially with the basal type, there is often conjoined syphilitic menin go-myelitis. The latter dis- ease, as a separate affection, will receive special attention under the general head of spinal syphilis. Here, however, a few words must be said in regard to the symptomatology of the combined diseases, com- monly referred to as cerebrospinal syphilitic meningitis. As a rule the brain symptoms stand out in overwhelming prominence. If a thorough search be made, however, a fugitive girdle pain, a tendency to para- plegic phenomena, irritability of the bladder, localized spinal root pains with vasomotor and trophic disturbances, and loss of one or both patella reflexes will be discovered. Later on these spinal symptoms may become more obvious and more constant. In some cases they may even obscure somewhat the cerebral symptoms. Almost any type of spinal syphilis may thus present itself with brain syphilis, the more common of which are Erb's type of syphilitic spinal paralysis, multiple sclerosis, Brown-Sequard's syndrome or Oppenheim's syphilitic pseudo- tabes which is not unlike tabes dorsalis in many respects. Closely related, pathologically and symptomatically, to this cerebrospinal syphilis is the multiple syphilitic neuritis of the nerve- roots of Kahler. There is nothing distinctive about this to call for special consideration except the fact that the paralysis of the various cranial and spinal nerves comes on insidiously and irregularly, and that the trouble may readily be mistaken for a peripheral neuritis. Oppen- heim says, anent this multiple syphilitic neuritis of Kahler, that it is just a little too schematic, a remark which I am inclined to apply to the former's own separate description of basilar and vortex forms of brain syphilis as though they were separate and distinct diseases. To be sure they each have a striking characteristic symptomatology and prognosis, but that is the result merely of the location of the disease process. To found a classification thus of the same disease upon a mere difference of location, is confusing. Especially is this true where, as in brain syphilis, the disease process so often occupies both and even all localities simultaneously. The symptoms do not materially differ in brain disease when the THE XOX -NEURONIC DISEASES 88,7 syphilis is inherited from those in which it is acquired. Occurring thus early in life, however, it is obvious why many mental defects and epilepsy should be so much more prominent than they are in the ac- quired form. Diagnosis. — It is easy to confuse brain syphilis with other dis- eases when there is not a clear history of infection. The wide extent of its pathology and consequent symptomatology account for this and the great changeability and transitoriness of many of its clinical phe- nomena. As the error is more often committed of attributing a non- syphilitic disease to syphilis than vice versa, the differential diagnosis is best considered under the headings of the diseases that are thus more likely to be mistaken. Prognosis. — The prognosis of brain syphilis, both as to life and as to permanent disability, depends very largely upon the location of the disease process. When it is most active at the base of the brain the prognosis is worse than when it is spread over the convexity. In neither form, however, is it absolutely unfavorable. Life is endangered the more the process approaches and affects the great vital centers in the pons and medulla. Involvement of the basilar artery and its branches is, therefore, a particularly unfortunate event. This i- especially in old age, and when the general health is below par. When hemiplegia occurs it may clear up, and later on many re- lapses follow. A hemiplegia, however, is always a bad sign, as it in- dicates both an advanced and wide state of disease. If the hemiplegia does not recede, and especially if contractures have begun, recover) is no more to be expected than in a hemiplegia of any other sort. In regard to the question of recovery. Gowers has stated that syphilis is practically an incurable disease, though he probably would not deny that by treatment its symptoms may be suppressed and its destructive influence held in abeyance. With this view I am in per- fect accord. Therefore it follows that recovery under treatment called recovery — can only be looked for when the tissue changes are strictly specific and have not gone on to complete degeneration, reor ganization, etc. An optic neuritis, for instance, is amenable t<> treat- ment, but an optic atrophy is not; a specific meningo-encephalitis m;i\ be made to clear up, whereas a necrobiosis due to an arte rial obliteration will not give any response to the most heroic medica tion. This is the reason that antisyphilitic treatment gives Mich vai i.t ble results in cases treated promiscuously and consecutively. Treatment. — The prophylactic treatmenl of brain syphilis should include the education of the young a- to the dangers of infec tion and the heroic treatment of the infection at once a fur it has been acquired. Xo half measures are permissible here. A thorough course of mercury for a long period, with now and then intervals o\ a tew weeks' rest, should be carried out. It is m) own practice to recom mend treatment of this sort for a period of not (ess than three years, and sometimes many years more. The mereurv may he given by the stomach or by inunction. I prefer the latter method myself, \bout a dram of unguentum hydrargyri is rubbed int.. the skin d.iih every other day, for a couple of weeks, and then a few w ' is 888 THE NON-NEURONIC DISEASES allowed or the mercury is continued in milder dosage by the mouth. It is best to use the ointment alternately on the inner side of first one arm, then the other, or on the inner side of one thigh and then the other, taking twenty minutes or half an hour to rub it in, and covering the part afterwards with a bandage. During this mercurialization the diet must be attended to, excluding all acids and stimulants ; the exer- cise must be regulated and the use of free bathing to promote the ac- tivity of the skin must be enjoined. A chlorate of potash mouth wash (about 5 per cent, strength) if used often during the day will prevent stomatitis. In a few cases even the most vigorous early treatment after the above manner, fails to prevent the disease attacking the brain or the trouble itself from relapsing. When either of these unfortunate events occur, the treatment must be at once renewed and conducted to the very point of ptyalism. It is the patient's only hope of avoiding the frightful ravages of the disease in the wav of the paralyses, etc. In some cases the iodide of potash may be employed with or with- out the mercury. It shows it's effects, as a rule, sooner than the mer- cury but the effects are not so lasting. The dosage of the iodides should be much larger than is usually given. I have myself given as much as one thousand grains a day. Beginning with twenty or thirty grains, well diluted in water and taken after food, three times a day, the dose should be rapidly increased daily until two or three hundred grains a day are taken. If the remedy .is well diluted, and the stom- ach assisted with a little pepsin and nux vomica, there is little danger of the manifestations of iodism. In some cases I have had such mani- festations disappear after suddenly advancing the dose to a still larger amount, say five or six hundred grains a day. The average case will hardly need more than about three or four hundred grains a day, con- tinued for two or three weeks or longer. I prefer the potassium salt to any of the others, and above all to the many iodonucleins and other substitutes put upon the market. Where atrophy has taken place, destruction of tissue -and sclerotic changes have occurred within the nervous tissues, of course no treat- ment, however vigorously applied, can be expected to produce any ben- eficial results. This is the reason why such varying reports are made in the treatment of consecutive cases of brain syphilis. Hem- iplegia, contractures, etc., are to be managed here on the same lines as when following other general causes. When complete, or what is more frequent, partial recovery, is obtained, the patient should be warned of the possibility of a relapse and told that the best way to avoid such a catastrophe is to lead a steady, regular life, to avoid all mental strain and worry, sunstroke. traumata, stimulants and excesses of all sorts, especially in eating and sexual matters. DEMENTIA PARALYTICA. Until quite recently this disease was discussed solely in works upon insanity. This was on account of the apparent supremacy of its psychic manifestations. Since we have learned more about its true nature, we find that pathologically and symptomatically it is an organic THE NON-NELJRONIC DISEASES 889 brain disease. While there may be some doubt as to the strict appli- cability of the name chronic diffuse meningoencephalitis, some still regarding the disease-process as primarily one of degeneration rather than inflammation, there can be no doubt as to the positive changes in the cortex and their relationship not only to the psychic but to the motor and other equally important symptoms. Hence it is proper to now discuss general paresis or dementia paralytica under the head of diseases of the brain. The old name progressive paralysis of the in- sane ought to be entirely dropped as it represents a conception of the trouble not at all in consonance with what we know about it to-day. Etiology. — In a few cases a neuropathic heredity can be traced but only in a few. The male sex fall victims to it much more fre- quently than the female. It occurs most commonly in the middle period of life, between the ages of thirty and fifty. It has been seen in childhood. It is very rare in old age. Nationality seems to have no deterring influence for or against it. It is on the increase every- where where the excitements and struggles of life are at their maxi- mum. In the cities and the crowded centers of civilization and in those walks of life where ambition to get ahead, mental strain, anxieties and hopes are the keenest. Nascent races, like the negro, when placed under these conditions become subject to it. Fournier ranks general paresis along with tabes dorsalis as a parasyphilitic disease. In from 70 to 90 per cent, of all cases a history of syphilis is obtainable. Like tabes it is regarded not as a syphilitic disease but rather as a sequel of or the remains after the syphilitic storm. Pathologically and clin- ically locomotor ataxia and dementia paralytica are certainly closely related. The difficulty of inoculating paretics with the syphilitic virus, as shown by Krafft-Ebbing, is a strong point in favor of the syphilitic theory of the disease. Acquired and congenital syphilis has been traced in some of the cases -seen in childhood. It is probably this in- fection, together with the strain and dissipation that lurk behind gen- eral paresis, that determine such factors in the etiology of it noted above in connection with sex, age, etc. In a happy epigram Krafft- Ebbing has summed up the etiology of the disease as covered by "civil- ization and syphilization." The particular truth concealed in this aphorism is that neither the syphilis nor the civilization alone is likely to give ri'se to the disease. The predisposing influence of the former and the exciting tendencies of the latter must cooperate. And yet one cannot elicit evidence of syphilis in ever} case. ( Hher predisposing factors must be assumed in its place by those who are not yet ready t«» accept the dictum that all paretics arc syphilized. In two instances in which the infection was strenuously denied, and in whom I could not obtain the slightest evidence either in the past or present histor) of the disease, typical dementia paralytica developed so., 11 after the residence of the patient was transferred from tin- countr) to the city. In both instances the new life was most strenuous, full of trouble and anxietj and wanting in either happiness < »r contentment. Nothing beyond a neuropathic heredity could be discovered as a predisposing cause. It is not unreasonable to suppose that in a certain -mall percent cases there may be such a congenital weakness, such a want "i vital 89O THE NON-NEURONIC DISEASES capacity, in certain of the central neurones that when subjected to the sudden and unwonted strain of competitive civilization they suc- cumb. Pathology and Pathogenesis. — The principal pathological changes are observed in the cerebrum, though there are changes often in the spinal cord also. Macroscopically the brain is smaller, smoother and harder than normal. There is an atrophic condition of the convolutions, making the gyri smaller and flatter and the sulci less deep. This is most no- ticeable in the frontal and parietal regions, though it may be seen also in the island of Reil and in the temporal convolutions. It is sugges- tive of cortical degeneration. The membranes are frequently adher- ent to the brain and when torn away often peel off spots of cortical substance with them. The pia is thickened and clouded while the dura looks as though there had been an internal hemorrhagic pachy- meningitis. There is an excess of cerebrospinal fluid and a variable degree of internal hydrocephalus with distention of the ventricles and granulation of their walls. The weight of the entire brain is diminished and when it is cut into it feels firm and cheesy. The borderlines of the gray matter are less sharply marked than normal and the thickness of the cortex is seen to be decidedly lessened. The degree of sclerosis varies in differ- ent cases. The white matter is also affected but not as much as the gray, being firmer in consistency and seemingly more compact. Areas of softening, old hemorrhagic remains and arterio-sclerosis are not un- frequently observed. In a word the gross appearances are such as we might expect from a prolonged inflammatory, degenerative, sclerotic process. The microscopic appearances are striking and quite typical though the nature of the process underlying these appearances is not clear. The cells of the cortex are more or less atrophied and the neuroglia and interstitial tissue is hardened and increased. The ganglion cells are variously altered, the nuclei and fibres of the neuroglia are increased, the spider cells are proliferated, the bloodvessels are hyaline and col- loid, and sclerosed, and the lymph tracts are dilated and displaced. Ac- cording to Tuczek the medulated tangential fibres of the upper cor- tex, especially of the frontal lobe and island, are degenerated. The whole condition, as Wernicke expresses it, suggests a chronic progres- sive process of the disease in the cortex that causes an ultimate atro- phy of its constituent parenchymatous elements. Similar appearances are sometimes observed in the basal ganglia, and gray matter of the crura, pons and medulla. The pyramidal and posterior tracts may also be implicated. It is not known whether the degeneration of the pyramidal tracts is primary or secondary. That of the posterior tracts is probably primary due to the same cause that produces the cerebral changes. The optic nerves, and occasionally some of the other cranial nerves, are wasted. Even the peripheral cutaneous nerves are now and then degenerated. It will be noted that this pathological picture brings to mind very forcibly the pathological picture of tabes dorsalis, the only difference THE NON-NEURONIC DISEASES 89 c being the location of the primary focus of the disease. Fournier him- self suggests that the two affections are but different expressions of the one and same morbid entity, possibly the one and same disease. Clinically it has long been remarked that locomotor ataxia often ter- minates in dementia paralytica and that paretic dements not in fre- quently develop tabes dorsalis. The question of the nature of the primary process in the patho- genesis of the malady is quite as unsettled here as it is in locomtor ataxia. There are strong contestants for the primary degenerative theory on the one hand, and for the meningeal inflammatory theory on the other. It is highly probable that whichever view is ultimately proved to be correct, it will apply to both dementia paralytica and loco- motor ataxia alike. Following the lead of Fournier many still maintain that as a more •or less remote result of the specific infection a primary degeneration takes place in the cortical cells and their processes, with a secondary sclerosis and inflammation in the interstitial tissues and cerebral mem- branes. Many facts seem to lend credence to this view and none more so than the one overwhelming fact that in the true syphilitic diseases of the central nervous system, which are distinctly meningeal and inflammatory no such characteristic changes are produced as are ob- served in dementia paralytica. The arteritis, gummata and gumma- tous meningitis of cerebral syphilis are never seen in these degenera- tive diseases, while on the other hand the characteristic degenerative appearances of the latter do not seem to occur with the inflammatory processes of cerebral syphilis. Either there must be two distinct forms of inflammation resulting from syphilis, the degenerative and the or- dinary, or the two processes must be distinct and separate. In the light of modern pathology the latter seems to me to be the only answer to the question. The primary inflammatory processes of syphilis of the central nervous system, whether immediate or remote, are not the same as the degenerative results of syphilis as seen in dementia para- lytica and tabes dorsalis. Those who contend that the disease is primarily inflammatory with the parenchymatous atrophy as a secondary result are tending back to the old view of a primary sclerosis, though they place the initial stages of the disease process in the cerebral meninges. This is somewhat in line with Obersteiner and Redlich's explanation of locomotor ataxia •on the basis of a particular form of spinal meningitis. Bevan Lewis partly sums up this view by stating that there are three Ma^'s in the cortical process: (1) the stage of inflammatory change in the tui adventitia of the arteries: (2) tin stage of hyper-development of the lymph-connective system of the brain, with degeneration ami disap pearance of the nerve elements and the axis-cylinders; and I 3 > tin- stage of general fibrillation with shrinkage- and extreme atroph) of the parts involved. In perfect consonance with this view the disc is sometimes called chronic diffuse meningoencephalitis. Whether the meningitis is an initial, synchronous or terminal phe nomenon with the encephalitis or not. the primary inflammator) na ture of the entire process is the essential feature of this view. If tins 892 THE NON-NEURONIC DISEASES be so we are at once confronted by the question as to the nature of the process in the similar disease, locomotor ataxia. If the two dis- eases are the same then primary inflammation, it would naturally be supposed, underlies the pathology of the latter. Many declare that it does but that it originates in the membranes. These theorists prob- ably then also believe that dementia paralytica originates in a chronic meningeal inflammation. Why discuss either disease then outside of the chapter or chapters devoted to cerebral and spinal meningitis ? The degenerative sequelae can then be easily treated of in a special para- graph and emphasized as one of the many results of these special forms of meningitis. If, however, the meningitis is not the primary source of the trouble, though it is still affirmed that the process is primarily an interstitial inflammation, then it is presumably the same in locomotor ataxia, and we are back to the old posterior spinal sclero- sis theory in which the degeneration was affirmed to be a secondary process. The only way of escape from this dilemma, it seems to me, is to deny completely the relationship between the two diseases, tabes dorsalis and dementia paralytica, and that we cannot do. The whole question is in need of more light, though for the present we are justified, in my opinion, in regarding the primary pro- cess as a degenerative one in the nervous elements in both diseases. This degeneration is the result of an exciting cause acting upon a nerv- ous system predisposed by syphilis. Secondary to the degeneration are the interstitial sclerosis, meningeal inflammation, and other vascular alterations. Symptoms. — It goes without saying that the most prominent symptoms of dementia paralytica are the mental exhibitions. For this reason the disease was formerly named and classified among the pure psychoses. Prominence and importance are not synonymous terms. There are other symptoms so important that were they absent, the mental symptoms would lose much of their significance as signs of this affection. The diagnosis should never be positively based upon the psychic symptoms, though they may furnish the strongest clue to the trouble. In every case search should be made for the motor signs, evidences of apoplectiform attacks, speech disturbances and Argyll-Robertson pupils. One or more of these will usually be found and when associated with the psychic exhibitions, the diagnosis of the organic cerebral disease, dementia paralytica, is assured. The special value that attaches to the menial change is that it be- gins to show itself so early that it almost assumes the role of a prod- rome and awakens a strong suspicion before any of the other symptoms can be clearly detected. The panorama of the psychic disturbances moves through the successive stages of gradual excitable dementia. A steady down grade in the intelligence, with a weakening of its chan- nels of expression, the judgment and conscience and morale, is the psychic side of the clinical picture. Those mental powers, with the high obligations that accompany them, which have been the last to have been laboriously evolved during long periods of evolutionary de- velopment in the race and in the individual, like the apex of a pyra- mid, are the first to topple. There is a gradual descent from manliness THE NON-NEURONIC DISEASES 893 to childishness, from altruism to egoism, from a high sense of obliga- tion to a low sense of responsibility. While the memory, the imagina- tion and the will may be affected, it is the intelligence, the comparing and relating faculty, that is especially damaged. *As this faculty ex- ercises an inhibitory control in health over all of the lower faculties of the animal personality, the more it is weakened the more the lower faculties and their personality come to the front. This explains the egoism, the selfishness, the shamelessness, the cunning, the unreason- able scheming, the emotionalism, the instability, unreliability and irri- tability, the abnormal ideomotor excitement, almost resembling mania, the expansive imaginativeness with its hallucinations and delusions, its volubility, loquaciousness and general bustling activity and self- satisfaction usually seen in these patients. The guiding reins of the intelligence have fallen and the steeds of the lower faculties are run- ning away. Only exhaustion brings them to a standstill in the ter- minal dementia. The whole picture is a sort of psychic analogue of spastic paralysis, or a failure of pneumogastric inhibition over the ac- tion of the heart. So fine are these psychic changes in the beginning that often they are not noticed except by the closest observers and the nearest 0/ kin. There is a tendency towards indifference to business, home ties and obligations of honor. The individual is not as scrupulous about little matters, his dress, his word, his promises, his speech, his en- gagements as he has been wont to be. He is a little impatient ni re- straint and seems to seek his own comfort and pleasure more deter- minedly. He becomes fickle and frivolous. He neglects his wife and children upon the slightest selfish whim. He grows inattentive and forgetful. He makes use of language and performs acts that are un- called for, unaccountable and often shameless. At times he is em- barrassingly indelicate, vulgar, cynical, obscene. He seems to have lost, as the disease advances, all sense of the proprieties, all finer ap- preciation of his proper relationship to the world outside of himself. He will expose his person, urinate or defecate upon the highway. utterly indifferent of publicity. This general apathy and indifference coupled with the unbridled egoism often brings him in conflict with his partners and associates in business, in opposition to those who are most interested in his welfare, and even in actual struggle with the law. Thus irritabilities at home and lawsuits abroad are his portion. Sharpers and tricksters observing and taking advantage <>f* his weak ness, often lead him into concocting vast schemes, spending vast sums of money, starting huge industrial undertakings, developing unwieldy financial combinations which help to enrich them but invariably spell ruin and disgrace for him. Who knows what financial crash* >. in- dustrial upheavals and great business failures have not been the legit- imate ending of the grand schemes of some unrecognized but irre sponsible paretic dement ! More disrupted family circles, more thiei erv in the world of high finance, more immorality, dishonesty, and general devilishness of a certain order may be due to incipient general paresis than we are willing to admit. Not only on the side of moral apathy and ethical indifference i 894 THE N0N- NEURONIC DISEASES dementia paralytica exhibit itself but also on that of positive self- satisfaction, unceasing activity, restlessness and excitement. The calm egotism and self-satisfaction of these patients is sometimes posi- tively exasperating. They know it all, they are absolutely impervious to instruction. They are therefore often apparently happy and pleased with the world, themselves and things in general. Their restlessness may exhibit itself in many ways ; in taking long walks, scribbling whole reams of paper, inventing a lot of useless gewgaws or perambulating about the house all night. The environment of such patients is in a constant state of turmoil. Those about him become worn out with his incessant volubility and his constant bustling without achievement, involuntarily irritate and cross him and drive his weak, irritable dis- position into frequent explosions of terrific anger. Though danger- ous at times, these passionate outbreaks are not lasting. Sometimes they are most violent and provoked by the most trivial circumstances. Loss of memory is characteristic of almost every case. It must not be confused with the mere apparent loss of memory caused by the overwhelming force of the selfishness. One of my patients was given some money by her husband, who was a poor man, to go out and pay some grocery and other bills in the neighborhood. She soon walked in with nineteen shirtwaists, to purchase which she had used all the money and declared she had forgotten all about the shop-bills which she still held in her hand. In the true amnesia, recent events are more easily forgotten than remote. If the patient is asked to describe his life he will be very loquacious about his early years but very chary in reporting what he did the week before. Inability for mental computation is particularly noticeable. A previous lightning calculator will struggle over the addition or multiplication mentally of a column of two figures. Amnesia is probably at the basis of this trouble. With the steady progression of the disease the signs of psychic irritability and intellectual weakness become more and more pro- nounced. They assume the character of grandiose hallucinations and delusions, particularly in regard to himself. On account of this folic de grandeur he identifies himself with kings, presidents and mighty geniuses. It is a favorite occupation with these poor creatures to pose as the reincarnated Messiah. Sometimes they imagine they are veritable Monte Cristos with unlimited wealth. The paretic dement is always a great artist, an inexhaustible inventor, a poet of world- wide renown, an astute detective and the scientist of scientists. While he does not hold long to these various poses, sometimes he does dabble in the work of the particular character he imagines he is. The vacuity of all his efforts and absolute nothingness of the sum total is at all times pathetic if not amusing. Instead of the feeling of well-being there is sometimes one of de- pression. Hypochondriasis or melancholia may occur early in the disease, though even these usually assume the grandiose, lie is a Byronic sufferer on account of the cruelty of the world. The torments ol Dante's Inferno are nothing in comparison to what one of my pa- tients declared he endured. Thev fancy thev are the victims of most THE NON-NEURQNIG DISEASES 895 extraordinary anatomical and physiological conditions. A man told me once that the food he ate flew out of the top of his head up against the ceiling. Unique, astonishing, surpassingly strange though sad and depressing are these delusions, and thus they are in keeping with the general psychic manifestations of the disease. As the excitement, volubility, and psychic irritability approach their climax the patient be- comes dangerous and more and more intolerable. He bursts out in passion, screams, rushes about, seizes those near him, scolds and pours forth a volume of senseless words and phrases and makes all sorts of strange and wild grimaces. The oncoming exhaustion soon shows itself in beginning bodily emaciation, especially as the patient refuses all nourishment, in a more prompt appearance of the condition of tire. in lessened volubility, in increasing inattention and dullness and finally in absolute stupor and dementia. The end is then not far off. Among the non-mental somatic symptoms of the disease the ear- liest to appear, and therefore of the greatest importance, are the Argyll-Robertson pupil, pupillary inequality y tremor, especially of the tongue and lips, special disturbances of the speech and alterations of the handwriting. These may be present when the mental manifesta- tions are quite elusive. Others may precede the mental and all other symptoms by months and years. They are never entirely absent when the mental symptoms are noticeably prominent. They should always be strenuously sought for and constitute the stronger foundation for the diagnosis of the disease in the entire complexus of symptoms. De- mentia paralytica is an organic disease with positive somatic representa- tion. The prominent psychosis that constitutes a part of its clinical pic- ture is the direct result of the organic damage in the cerebral cortical elements. It is not entirely and wholly an insanity in the usual mean- ing of the word. Pupillary rigidity to light stimulation, with preservation of re- sponse to accommodation, the Argyll-Robertson pupil, is an early - of general paresis. It is estimated that it occurs in si\t\ per cent. <>t" the cases. It may precede all other symptoms and be the only one present for many months and even several years, h is usually a con- tracted pupil; some ophthalmologists say always a contracted pupil. Mydriasis may exist, however, in some cases. It is usually bilateral. though it may be more marked in one eye than in the other, <>r ma\ be entirely unilateral. The pupils are sometimes unequal or irregularly contracted. Hippus has been observed. The vision is more or less disturbed and on account of the occasional headache with scintillat- ing scotomata the disease for a time has been mistaken for lu-mi- crania. Double vision from paralysis <»!" the external ocular nuts and even paralysis of accommodation have been rarel\ noted among the earlier manifestations. These eye symptoms, and others it will be remarked, are much like those sometimes seen in the preataxic si of locomotor ataxia. For a while in seme cases tin- distinction l><-tv, the two diseases cannot be made. Soon a peculiar, fine, fibrillary tremor announces itself. li i^ especially noticeable in tin- sides and tip of the tongue and in the lips. When the patient talks it can aK<» be slightly detected. It may be i 896 THE NON-NEURONIC DISEASES fined to certain muscles or it may appear in every part of the body. It shows itself when the patient write's and it is seen in the hand and foot sometimes when they are held out. The oscillations are small, rapid and sometimes accompanied by jerky movements. Together with and partly on account of the tremor and fine incoordination, the patient's speech and writing are strikingly characteristic. The former is slow, blurred, drawling, stumbling and interrupted. There seems to be difficulty in getting the words out and consequently delay and hesi- tancy. Syllables and parts of whole phrases may be omitted or slid over indistinctly. There is no distinct scanning of the speech, but rather uncertainty, jerkiness and irregularity. In the articulation the lips tremble and shake markedly, unusual parts of the face are worked and a nasal tone is assumed. Generally ordinary language will do to exemplify these manifestations. I sometimes demonstrate them more forcibly by getting the patient to repeat, as well as he can some such phrases as these : Peter Piper picked a peck of pickled peppers ; round the rugged rock the ragged rascal ran ; buy baby blue bottle of black- berry brandy, etc. Familiar, semi-automatic speech, like reciting the multiplication table, can usually be uttered with more facility than speech requiring constant attention and thought. The latter is always accompanied by a greater slowness of diction, partly on account of the growing dementia. In reading it is apt to be more noticeable than it is in automatic speech, but not quite so marked as in thoughtful conversa- tion. The same general features seen in the speech trouble are seen in the handwriting. There is tremulousness, jerkiness, irregularity, slow- ness, laborious effort, repetitions and omissions. Incoordination and dementia are behind these. Sometimes there is absolute want of sense, coherency and relevancy in what is penned. Paralexia or irrelevant reading has been noted in some cases. If the patient has been a letter writer, always ask to see some of the earlier letters. In some cases there may be detected slight occasional lapses, both of thought and diction, that now show themselves so prominently. As in all examina- tions of this sort, involving the patient's intelligence and education, the previous culture must be known and taken carefully into consideration. A man of mere ignorance may attempt to write and the product bear somewhat a resemblance to that put forth by an incipient paretic. So important are the speech and writing defects in the diagnosis of the disease that their examination must never be omitted, however slight the other symptoms may appear. They alone are almost path- ognomonic and can ofttimes be detected by special examination when otherwise they would not attract attention. A history of temporary syncope and paralytic attacks is extremely suggestive. These may be either apoplectiform or epileptiform. Without warning and without apparent immediate cause, the patient suddenly has a sinking spell with more or less coma and muscular weakness. He may turn pale and fall, or he may remain stationary in a sort of petit mal state. On the other hand, he may have all the indi- cations of a true apoplectic seizure; sudden unconsciousness, a fall, relaxed extremities with flushing of the face, stertorous respiration, rapid heart action, elevation of temperature and death. Hemiplegia. THE NON-NEURONIC DISEASES 897 monoplegia, aphasia and all the usual accompaniments and sequelae of cerebral hemorrhage and obstruction may appear in some cases. Usually these attacks are of the temporary type and sooner or later the patient is almost back to his old condition. The paralysis and the aphasia may be very brief and Meeting or they may be partially per- sistent. The phenomenon repeats itself, varying in time and location. True epileptic-like seizures, generalized and partial, usually without aurae, are perhaps more common than apoplectic strokes. Sometimes these precede the appearance of any of the other typical symptoms. All forms of epilepsy may be simulated by them, even the psychic and the status types. After these paralytic and epileptic attacks the pa- tient's general mental condition, speech, etc., are apt to be worse. In the cases that are hemiplegic the gait is characteristic, pains are absent and the reflexes of the knee are exaggerated. In some cases, however, there will appear, if not at first, then later on, loss of the knee-jerks, static and motor ataxia, lancinating pains and other sensory phenomena without much motor weakness. These, of course, are the manifestations of tabes dorsalis. They may appear before the mental indications of dementia paralytica ; in about eighty per cent, of cases they come on after them. Even perforating ulcer, arthropathies, bed sores, optic atrophy and other vasomotor and trophic conditions obtain late in the disease. With involvement of the pyramidal tracts together with the posterior, a combined spinal degenerative sclerosis with its usual clinical picture will be associated with the general paresis. So extensive is the clinical portrayal of the disease at times that one may well be in doubt as to what are the symptoms of the funda- mental malady and what the symptoms of the complications. There- is ground evidently for the contention of Fournier that the entire complexus of symptoms, cerebral and spinal, represents one uniform, wide disease of the entire nervous system, with its maximum repre- sentation, and usually limitation, in the sensory path. Diagnosis. — In the earlier stages the diagnosis of dementia paralytica is very difficult and this is the time when a correct diagtt is especially desirable. To save the patient, his family and his property a prompt recognition of the first signs of the trouble should be within the ability of every physician. A later diagnosis is neither especiall) brilliant or useful. By that time the patient is apt to In- so far ad vanced that no possible treatment will be of any benefit and so obstrep erous that the family will already have thought n\ putting him in con finement. Be very suspicious, therefore, when a strong, industrious, devoted man suddenly begins to neglect his engagements, his family and his usual duties; when he loses his power of concentration and attention; when his apperceptions become clouded and his judgments confused; when trifling causes excite him abnormally ; when lie dallies and delays and seems to want initiative force; when he i^ easil) wearied mentally and physically; when he lets loose in his sexual life is immoral, obscene and indifferent to all law and order; when h< unwarrantably egotistic, self-satisfied and selfish, or, on the other hand, is melancholy and hypochondriacal. If a history of syphilis can he obtained in addition t<» these mani- 898 THE NON-NEURONIC DISEASES festations in a man whose temperament is not of the neurotic type, the suspicion is strengthened. Ask to see some of his letters and note any changes in their style, both as to writing and contents. Especially look out for irrelevancy of phraseology, repetition and omission of words and syllables. Note his surprise when his attention is called FIGURE 199. General Paresis with arthropathies. (After Lloyd.) to these things, showing that they were done absent-mindedly, as it were. Search keenly for the somatic signs, such as the Argyll-Robert- son or other pupillary defect, the tremor, the speech disturbance. In the early stage the disease most liable to be confused with general paresis is neurasthenia. Very often these patients are neu- rasthenic and hysterical; it therefore behooves the examiner to be all THE NON-NEURONIC DISEASES 899 the more careful to detect the presence of the dementia and other signs of the trouble. The somatic symptoms are rarely liable to be mis- taken for signs of neurasthenia, certainly not the pupillary changes In nervous exhaustion there may be a kind of aphasia or a tremulous- ness, but there are none of the paralytic attacks of an apoplectiform or epileptiform character. In hysteria the latter may be simulated, but there are sensory phe- nomena as well as absence of the peculiar tremor that determine the latter. In neither neurasthenia nor hysteria is there that strange and almost weird-like absence of the old personality in the midst of all the bustling activity and garrulousness. Something seems to have dropped out of the mental life of the man that represents not so much a loss of force as a loss of control. In neurasthenia, and much more so in hysteria, the personality may be somewhat changed, but it is a more radical change ; nothing is lost, but everything is altered. In general paresis there is a distinct hiatus in the mental picture, and that which has left the vacancy is missed because of the absence of its controlling force over the entire man, mental and physical. The paretic dement does not, except in very rare cases, worry about his condition. He is not introspective or the victim of phobias and fixed ideas, as a neurasthenic or hysteric is. He is objectively egotistic, while the latter are subjectively egotistic. The paretic dement has the ego- tism o-f a child, selfish, grasping, indifferent to others, happy when its wants are supplied, enraged when subjected to restraint. The neu- rasthenic and the hysteric, to a lesser degree, has the egotism of the intense introspectionist. The former is then usually gay and exhilar- ated, the latter are more apt to be sad and whining and depressed. This difference between the general mental states of dementia paralytica and neurasthenia or hystero-neurasthenia, coupled on either hand with the other special symptoms of the two sets of diseases, ought to aid very materially in the making of the differential diagnosis. The paroxysmal character of the headache in hemicrania, its abrupt onset and cessation, the aurae, the peculiar eye symptoms and the absence of the typical psychosis of general paresis, will distinguish the former from the latter. And yet there are eases of the latter in which there are only headache and neurasthenic manifestations early in the disease, just as in some cases of hemierania. In even the- however, the headache does not come and go periodically, localize itself closely, and be associated with vomiting as in migraine. Tumor has been mistaken for early dementia paralytica. There are headache, neurasthenic symptoms, sometimes aphasia, epileptiform seizures and mental changes in tumor, but there are also choked disc, absence of tremor, absence of Argyll-Robertson pupil and none of the characteristic mental exhibitions. Mental Stupor and slow pulse are the traits of an intracranial neoplasm. Gutnmata and gummatous meningitis may cause symptoms that closely resemble those of dementia paralytica. And this is not markable when it is remembered that the arteriosclerosis and menu encephalitis set up by syphilis may conceivably provoke the d. five changes in the nervous elements that are observed in tli 900 THE NON-NEURONTC DISEASES syphilitic troubles. In cerebral syphilis there is a sharper localization of the symptoms and more of an inflammatory irritative character about them than there is in general paresis. The headache is distinct and sharply localized, the paretic symptoms are comparatively limited and the somatic signs are more pronounced than the mental, whereas in general paresis it is the other way. Argyll-Robertson pupil, tremor, speech difficulty such as are seen so frequently in paretic dementia are not traits of cerebral syphilis, but, on the other hand, ocular palsies, monoplegias, sharp epileptic seizures are more to be expected in the latter. In late cerebral syphilis, particularly of the vertex type, quite a typical form of general paresis may supervene; but by that time the essential nature of the disease will have been well established. Multiple sclerosis can be differentiated without much difficulty. The mental symptoms when present are of the stuporous or mildly de- lirious type, not so unique and sui genesis as they are in general paresis. Nystagmus, scanning speech, intentional tremor, paresis with some rigidity, ocular palsies with diplopia of a transient character, will help to easily distinguish a disseminated sclerosis. Senile dementia is a disease of old age and is not abrupt, but is clearly a condition running parallel with a slow wasting of the entire organism. It has not the more prominent somatic signs of general paresis. Subacute and chronic alcoholism produce an exhilaration, a weakening of the higher controlling mental faculties and mild delirium that in some respects brings to mind dementia paralytica. Tremor, speech troubles, restlessness, insomnia, loquacity, amnesia, belong to both diseases. In some of the cases of alcoholism that I have seen I have suggested the diagnosis of incipient alcoholic meningo-enceph- litis. Here, however, the irritative traits of the psychosis have seemed to me to be more pronounced than they are in general paresis. There are illusions and hallucinations and other typical phenomena that are rarely met with in the latter disease. The etiology of the trouble is almost quite obvious and to a large extent helps to determine the diagnosis, just as it does in the encephalopathy of lead poisoning and bromine, trional and other forms of drug intoxication. Prognosis. — This is absolutely bad. Most cases die in about three years, others have lived to eight and ten years. It has been re- ported that two or three cures have occurred. The only cases tha; hold out any possible hope of recovery are those that are recognized early, give a clear history of recent syphilitic infection and undergo vigorous antisyphilitic treatment. Death is caused by exhaustion, hypostatic pneumonia, bedsores, genito-urinary complications or other intercurrent trouble. As partial remissions in the course of the dis- ease sometimes occur, it is imperative to warn the relatives not to mis- take these for a cure. Treatment. — A paretic dement is always a nuisance and dan- ger to himself and his family. The proper place for him therefore' is in a sanitarium or asylum. Here he will be given careful nursing, plain, nourishing food and such occupation and diversion as will keep him out of harm's way and in the least excited state of mind. Tonics THE NON-NEURONIC DISEASES 0,01 may be employed on general principles but medicinally the only hope- ful treatment is the specific. Large doses of the iodides should be given a trial. If they show the slightest tendency to do harm, they should be withdrawn at once. Temporary remissions sometimes fos- tered by this treatment are mistaken for cures. Early in the disease- some improvement may follow massage, hydrotherapy and gentle ex- ercise. Xo severe measures of any sort should be attempted. Cold to the head may allay excitement. Other symptomatic measures in- clude the bromides for the insomnia and epileptic seizures, hypoder- mics of morphine, hyoscine, duboisine and ergotin for the delirious excitement, the usual measures for the apoplectiform attacks and forci- ble feeding for the refusal to take food. MULTIPLE SCLEROSIS. DISSEMINATED SCLEROSIS. CEREBROSPINAL MULTIPLE SCLEROSIS. This is a chronic, progressive malady of the cerebrospinal axis, occurring in the early part of adult life and notable for the wide diversity of its symptoms. While in the typical forms of the disease there is a certain degree of uniformity in its clinical picture, in many of the atypical forms it closely simulates a large number of other nervous diseases, from which it is sometimes extremely difficult to differentiate it. On account of the disseminated nature and wide distribution of the lesion, with the consequent variability and wide diversity in its clinical manifestations, a concise definition of multiple cerebrospinal sclerosis cannot be satisfactorily formulated. When con- fronted with a suspected case, the diagnostician must keep well in mind the nature of the lesion and apply most carefully his knowledge of nervous physiology and localization. Etiology. — Multiple sclerosis is almost unique in being the only chronic progressive organic disease that occurs so early in life. The vast majority of the cases make their appearance between the a of twenty and thirty years. It rarely occurs before fourteen, and still more rarely after fifty. Cases have been reported in infancy. In old age it is practically unknown. Young male adults are rather more frequently afflicted than females. Neither climate, race nor season enter into its etiology. Heredity in some instances has seemed to have played a minor role in the transmission of a neuropathic condition. Eichhorsl -aw it in an eight-month's child whose mother was a victim of the dis© An inherited degeneracy rather than a mere neuropathic state may underly some of these cases. There is now under my observation a man, thirty-two years of age. who exhibit- and gives a typical hi- tory of disseminated sclerosis, which began about eight years His family history is remarkably suggestive. IN- grandparents on the paternal side were first cousins. Mis father, an uncle, and an aunt, were born deaf-mutes. I lis mother became a deaf-mute in child- hood after a severe attack of scarlet fever. The patient himseli the younger and weaker of twins. Mi- twin brother and one Other brother are apparently perfect in every way. There can be discovered 902 THE NON-NEURONIC DISEASES no exciting cause whatever for the onset of the trouble. He has been temperate and has led an even, comfortable life. He was married over ten years ago and has two healthy daughters. Infection is the preeminent cause of multiple sclerosis. This is so true and it so frequently follows the infectious fevers that it may almost be called a post-infectious disease. This undoubtedly explains in part many of the other etiological factors of the disease, such as the age of the patient, sex, slight hereditary influence, etc. Typhoid fever and malaria seem to be the more frequent infections that pre- cede it. Variola, scarlet fever, measles, pneumonia, whooping-cough, diphtheria, cholera, influenza, erysipelas and acute articular rheuma- tism, has each seemed to have borne a causal relationship to it. It is not the result of syphilis. The disseminated form of spinal syphilis, with its multiple foci, is another disease. The puerperium has been followed by it and so has dysentery. Oppenheim believes that intox- ication from metals is sometimes the origin of the trouble and declares he has observed it follow carbonic dioxide poisoning. Gerhardt traced it to mercurial intoxication in one case. Trauma, shock and sunstroke are doubtful causes. It is probable that infection underlies these alleged causes. In some cases it is difficult to assign any cause for the disease. Pathology and Pathogenesis. — The gross appearance of the cerebrospinal axis in a case of multiple sclerosis is unique and striking. Even before the removal of the soft membranes, the glistening, hard, sclerotic foci may be seen irregularly scattered through the white matter of the cord and brain. These nodules vary, from 1-25 of an inch to an inch in size ; are irregular in shape ; and are harder than the surrounding tissue, though not so hard as connective tissue. There is no uniformity in their distribution. They are found most commonly in the w r hite matter of the centrum semiovale, internal capsule, crura cerebri, pons, medulla and columns of the cord. They are not con- fined to any tract or part of a tract. They sometimes involve a quar- ter, a half or entire cross-section of the cord. By extension they im- plicate the gray matter, though they never seem to originate in the gray matter. They are sharply outlined, though the neighboring tis- sues may be more or less indurated. This produces a sort of diffuse, as well as disseminated, sclerosis in some places. Under the microscope these nodules are seen to be fibrous in structure with a proliferation of the neuroglia and the other changes common in medullary degeneration. They are not connected appa- rently with the walls of the bloodvessels, though the latter may be proliferated and their walls thickened, distorted and otherwise sugges- tive of an inflammatory process. The degeneration is not of the sec- ondary type so that by many the disease is supposed to be a primary form of sclerogliosis. My own observations lead me to believe that some cases are of the nature of a congenital defect, in the same way that the cases of syringomyelia and Friedreich's disease are. The nature of the lesion, the marked influence of a neuropathic heredity, and the frequent absence of all exciting causes are very suggestive. Infections produce inflammatory and neuronic degenerative (lis- THE XOX-XEUROXIC DISEASES 903 orders, but in no other disease do they cause the unique patches seen in this disease. Infection probably acts as a prominent exciting cause in most cases. Running through the nodules may be seen axis cylinders. The myelin substance breaks down but the axis cylinders and ganglion cells show a great power of resistance to the sclerotic process. It is not FIGURE 200. Disseminated sclerosis of the brain and the spinal cord. (Partly schematic.) (After Lcyden.) probable that the axis-cylinders undergo any regeneration under these circumstances. The nodules and sclerotic process implicate and surround some- times the peripheral nerve-roots and cranial nervi The origin of this peculiar form of sclerosis 1- hidden in much mystery. The pathological finding- do not give absolute validit) to any one explanation, though they are highl There are three hypotheses held among the various pathologists. I have alreadv mentioned that one authorities hold that the 904 THE NON-NEURON IC DISEASES ease is a primary proliferation of the neuroglia, a sclerogliosis, and that many of the other presentations suggestive of inflammation and softening are purely secondary to the irritative presence of these sclerotic nodules. Other writers state that the pathological process is primarily a minute myelomalacia, the result of the presence of minute infected emboli. These infected emboli obstruct the circulation in the minute terminal bloodvessels of the brain and cord. The tissue in spots softens, breaks down and is absorbed. The vacant area is then occupied secondarily by the overgrown neuroglia. Still others maintain that the sclerosis arises from an inflammatory process com- mencing in the arteries. This as well as the preceding explanation is in consonance with the infectious nature and origin of the disease though many of the later findings do not lend either of them very strong support. Symptoms. — Two significant facts stand out in the symptomatol- ogy of multiple sclerosis, and while beautifully indicative of the char- acteristic nature of the lesion, are even of greater importance when it comes to the question of diagnosis. The first noteworthy fact is that the symptoms, whatever they may be, are rarely if ever complete. The paralysis, the anaesthesia, the trophic and mental disturbances, never go on to complete paralysis, complete anaesthesia, etc. It is exceedingly rare for an entire tract, center or cerebrospinal association to be com- pletely severed or obliterated. As a rule some fibres and cells re- main intact, and as a result the symptomatology exhibits such general characteristics as paresis, paraesthesiae, incoordination, tremor, jerking movements. This will be brought out in the further discussion of the symptoms. The other notable traits about the general symptomatology of the disease are the wide diversity and great variety of the individual symptoms, showing more or less implication of all parts and all func- tions of the cerebrospinal apparatus. This would naturally be looked for from such a lesion as multiple sclerosis usually reveals. And yet in spite of all this variability and diversity, the symp- tomatology of typical multiple sclerosis is singularly definite and uni- form. It is this clinical picture that I will now attempt to describe and when I have done this, I will point cut the possible variations in the picture. As a rule the onset of the disease is very insidious. The patient first notices a little difficulty in his walking. It seems to be stiff, irregular and a trifle jerky. The lower limbs feel zvcak and numb. As he has probably come through a severe infectious fever, and has been convalescing for several weeks, he attributes this weakness and numbness to his lowered health and vitality ; and therefore, he does not pay much attention to it at first. lie notices, however, that it does not improve but seems to get worse. Moreover he begins to have vertiginous attacks, reels slightly and has to take great care lest he fall when he turns suddenly. He is becoming cognizant of a slight tremor in his limbs, especially when he moves them voluntarily. His bladder appears to be weak and he has some trouble retaining his urine. All of these manifestations, not unlike the early manifestations THE NON-NEURONIC DISEASES 905 of myelitis, gradually increase, or at least continue unabated, until, becoming alarmed, he seeks the advice of a physician. A careful examination now reveals a partial paraplegia, spastic m type, with muscular rigidity and exaggeration of the reflexes. The gait is unsteady and much like that of alcoholic intoxication. It is spastic and paretic, incoordinate, ataxic, and trembling. It may reveal the characteristics of cerebellar ataxia. The tremor and incoordina- tion may be observed in all of the movements of the body, especially of the limbs, of the eyes and the muscles of speech and parts under the more immediate control of the will. The tremor is absent during rest and appears only upon voluntary movement. It has therefore been denominated an intentional tremor. At times it accompanies re- flex and automatic movements. It is irregular, wobbling and occurs in oscillations of five Or six to the second. Sometimes the tremor is coarse and choreic in appearance. On this account the patient cannot carry a glass of water to his mouth without spilling it, or write, or touch promptly the end of his nose. It is impossible for him to walk a chalk-line on the floor, climb a ladder, or kick squarely the leg of the table. This ataxia is also static in character and the Romberg symp- tom is more or less present. The muscular stiffness, weakness, ataxia and intentional tremor all render the patient exceedingly awkward in all of his movements. The head jerks and oscillates, the tongue is protruded in a jerking manner, the eyeballs jerk (nystagmus) and the speech is thick, slow and jerk- ing (scanni)ig or syllabic). With all this muscular disorder and weakness, there is no atrophy or changes of electrical reaction. The reflexes arc increased and usually an ankle clonus can be easily elicited. The sensory symptoms are not of very great significance, except those of vision. There may be numbness in the beginning and this may go on to a considerable degree of hypaesthesia. Complete anes- thesia does not occur, although a hemianesthesia has been reported. In such cases there is always some sensation. The ataxia in this dis- ease is not the result of the sensory disturbances so much as of the motor trouble. Even less frequent than the tactile disturbances are the disturbances of the muscular, temperature and pressure senses. Neu- ralgic pains are not uncommon and the Brown-Sequard symptom-com- plex has been observed. A rather striking and confusing characteristic "i' these sensor) symptoms is their instability and variability. They are temporary and come and go most unexpectedly. In this respect the) bear a resem- blance to the symptoms of hysteria and cerebrospinal syphilis. The reason for this is obvious. The visual symptoms of multiple sclerosis an important and should be sought for in every suspected rase. Diplopia occurs as the result <>t the involvement of the ocular muscles. In a very large number of the cases there is irregular and partial atrophy of the op' \ complete atrophy of the nerve is so ran mworth) of fur- ther consideration. An ophthalmoscopic examination of the fundi shows in most of th< n atrophic pallor of the "titer <>r temporal go6 THE NON-NEURONIC DISEASES • halves. Spots occur here and there and the two fundi never present the same appearance. These fundus changes appear in about twenty per cent, of all cases. As a result of these changes in the fundi, the optic nerve, and the central neurones that take part in the visual act, many symptomatic phenomena occur. The vision becomes blurred and misty and may be the first indication to the patient of his trouble. This diminution of vision scarcely ever advances to complete blind- ness. The Held is irregularly contracted and central scotomata, first for colors and then for white, become prominent. Scintillating sco- tomata are sometimes present, and sometimes there is simply achroma- topsia. Neuritic changes may precede the atrophy and the visual dis- turbance is usually the outgrowth of a partial retro-bulbar neuritis of one or both eyes. Thus the vision is sometimes markedly impaired without any noticeable alterations in the fundi. The pupils may be unequal and contracted. Ophthalmoplegia has been seen a number of times, though it is rare. A very important symptom when it does occur is an apoplecti- form attack. There is unconsciousness and hemiplegia, both very tem- porary and disappearing in a few hours or days. Sometimes the at- tack resembles an epileptic one, at other times it is like a severe stroke of apoplexy with prolonged after results. In such cases there is marked headache and vertigo. Sometimes the temperature is increased. The mental faculties are not usually affected. There may be dull- ness, however, hebetude, general apathy, with melancholia and weak- ening of the memory. The impulsive laughter is not a mental symp- tom. It is entirely involuntary and does not represent in any way a mental mood. Sometimes it is paroxysmal and exaggerated, and be- ing- frequently repeated greatly annoys the patient. It is exceedingly rare for illusions, hallucinations or delirium ever to be present. Diagnosis. — The above description represents a more or less typical case of multiple sclerosis. Such cases, however, are not the ones usually met with. The atypical cases, the aberrant cases, the so- called aborted types, the "formes frustes" are the ones that too often cause the confusion in diagnosis. Moreover, difficult is it in practic- ally all cases to make a diagnosis early in the disease, when an accurate diagnosis is especially desirable. Practically every case exhibits some approach to the general clin- ical picture which I have attempted to outline. Here and there a particular symptom may be absent, or the grouping of the symptoms may be quite unusual. This is all to be expected when one remembers the character and distribution of the lesion. If the symptoms are in- tracranial as well as spinal, rather insidious in onset, and slow in progress, to a certain extent variable and falling short of completeness, and above all suggestive of an interrupting lot of scattered foci within the nervous paths, the diagnosis of a disseminated sclerosis is almost certainly indicated. In the early period of the disease one should be keenly observant of the motor zveakness. As this may be the only symptom for many weeks or months, it should be closely studied. Its character is that of a spastic spinal paralysis. A slight nystagmus, a diplopia, a failure of THE NON-NEURONIC DISEASES 907 eyesight may accompany it. Sooner or later the nystagmus the optic atrophy, the dysarthria, the intentional tremor, the vertigo and even the temporary apoplectic attacks will announce themselves. Oppen- heim saw a unilateral optic atrophy as the only symptom in a case for twenty years. I have seen a tremor with merely a little vertigo cause a great variety of diagnoses to be made during a period of a year or more. In one case that I had under observation for nearly two years there were hysterical manifestations with merely a slight ataxia and misty vision. The case had been variously diagnosed as hysteria, loco- motor ataxia, neurasthenia and epilepsy from a possible incipient intra- cranial tumor. It gradually assumed the classical picture of a cerebro- spinal disseminated sclerosis. Too much caution therefore cannot be exercised in the early diagnosis, and it may be put down as an axiom that at this stage a positive diagnosis is practically impossible. It cannot be more than a strong suspicion. The atypical forms of the disease may show every sort of com- bination in their symptomatic presentation. The form that resembles a progressive spastic spinal paralysis is perhaps the commonest. The head symptoms, such as the optic atrophy, the diplopia, the nystagmus and the dysarthria, as well as the sensory manifestations, must be earnestly sought for and will usually be found, but more or less scured by the more prominent spinal symptoms. There is a hemi- paretic form that has been referred to by Oppenheim, Charcot. Ed- wards, Bickeles and others. A glycosuria, tachycardia and asphyxia may indicate a bulbar form. An acute ataxia of the upper extremities with bulbar symptoms giving rise to an alternate hemiplegia has been observed. Among the more common diseases with which insular sclerosis may be confused are transverse myelitis, spastic spinal paralysis. I< motor ataxia. Friedreich's disease, bulbar paralysis, amyotrophic lateral sclerosis, post-infectious disseminated myeloencephalitis, chronic men- ingitis, dementia paralytica, apoplexy, epilepsy, paralysis agitans and especially hysteria. So large a number of diseases is disseminated sclerosis liable to be mistaken for, that sometimes its diagnosis i- best made by exclusion. A careful consideration ^\ the clinical picture presented by these various simulated affections, therefore, should always be borne in mind. The reader is referred t<> their discussii ,;i in other parts of the book. A word must be said, however, lure in re gard to the differentiation of this disease from spastic spinal para! locomotor ataxia, dementia paralytica and hysteria. In spastic spinal paralysis the head symptoms, such a- the head ache, the optic atrophy, the nystagmus, the scanning speech, an- ab- sent. There is no intentional tremor and no ataxia. Th< and paretic, but it is not irregular and jerky. There are particularly no sensorv or mental alteration-. The muscular atrophy i^ a little i marked than in multiple sclerosis, though n<>t so marked a- in anterior poliomyelitis, or amyotrophic lateral sclerosis, or progi nuscular atrophy. The spasticity, together with the 1 tion of the ki jerks, is perhaps a little more pronounced in spastic spinal paral 908 THE NON-NEURONIC DISEASES In multiple sclerosis the rectal, and especially the vesical, functions are apt to be somewhat disturbed. Locomotor ataxia has been diagnosed from the ataxia which sometimes obtains in insular sclerosis. The former is a sensory dis- turbance, however ; the latter a motor. In locomotor ataxia Rom- berg's static ataxia is promptly manifested when the patient is asked to close his eyes while standing. The mere closure of the eyes does not so seriously discommode the victim of disseminated sclerosis. The absent knee-jerks, the fulgurating pains, the anaesthesia, the Argyll- Robertson pupil, the early loss of sexual power and other sensory,, degenerative symptoms will help to distinguish tabes. Dementia paralytica is a diffuse meningoencephalitis and has some symptoms not unlike those of multiple sclerosis in which the cerebral symptoms predominate. There is a form of post-infectious disseminated myeloencephalitis that cannot be distinguished from mul- tiple sclerosis, and sometimes changes into the latter. Dementia paralytica can usually be distinguished by the more distinctively mental character of some of its symptoms than the mental manifestations of multiple sclerosis, though it must be admitted that the differentiation at times is extremely difficult, if not impossible. In dementia paralytica the growing irritability, the weakening memory, the failure of con- tinued attention, the restlessness and excitability, the moral and sex- ual indifference, the exaltation of ideas and folie de grandeur, must be carefully taken into account. The incoordination and tremor, as shown in the penmanship and speech of the paretic dement, are char- acteristic. The finer and more complex voluntary movements of the hands are very noticeable, as well as the fibrillary tremor of the tongue. In multiple sclerosis the tremor and incoordination are much coarser. The unequal pupils, the Argyll-Robertson pupil and the absence of nys- tagmus characterize general paresis. Between no two diseases is a mistake more liable to be made in some cases than between multiple sclerosis and hysteria. Every symp- tom characteristic of the former may be simulated by the latter. More- over the two diseases not infrequently coexist in the same patient. When after a most careful examination there is still doubt, it is judi- cious for the practitioner to throw the weight of his suspicion on the side of the organic disease. In hysteria the temperament of the pa- tient, the psychic factors in the etiology, the psychic complexion of the entire clinical picture and the exceeding variability and instability of the symptoms speak plainly. The sensory manifestations are more pro- nounced in hysteria than they are in disseminated sclerosis. In the latter the intentional tremor, the optic atrophy, the characteristic dys- arthria, the nystagmus and the incoordination are to be closely noted. If these symptoms occur in a hysteric, excepting, of course, the optic atrophy, they can be made to show slight degrees of alteration by some sudden influence upon the patient's mind. It must be admitted that at times it is next to impossible to say whether a case is hysterical, a case of multiple sclerosis or a combination of the two diseases. I have seen a case of multiple sclerosis regarded for several years by different diagnosticians as locomotor ataxia, hysterical ataxia and in- THE NON-NEURON1C DISEASES sanity. Lately I saw a case diagnosed as hypertrophic pachymenin- gitis. Prognosis. — The prognosis of disseminated sclerosis in regard to life is favorable. Only when the disease attacks the vital centers in the medulla does it cause death directly. The fatal issue is usually brought about by some complication or intercurrent disease. In re- gard to ultimate cure, the prognosis is not good. A very few cures have been reported. Usually the disease runs a long course of years, progressively getting worse, but with occasional intermissions of quies- cence, or even partial remissions. Other degenerative and inflamma- tory troubles may be lighted up in the course of a multiple sclerosis. and the prognosis then becomes the prognosis of this particular disease. Treatment. — The treatment is not very satisfactory. Quiet, reg- ularity of habits, hygienic environment and all the other recommenda- tions for slow, progressive, chronic troubles are here in order. Above all avoid severe, radical measures. Why electricity is recommended I cannot understand. In my own opinion it had better not be em- ployed. Hot and cold baths, stimulants and over-exertion must all be prohibited. Special forms of the disease call for special lines of treatment. Syphilitic cases should be combated with mercury and the iodides. Apoplectic cases, myelitic and encephalitic complications de- mand a certain amount of antiphlogistic treatment. Local bleeding with leeches may be of some avail in such cases. In all cases the steady use of the iodide of potassium is about the best medicinal line i^i treatment to be employed. Arsenic, nitrate of silver, quinine and mild hydro- therapy, chiefly for their tonic effects, are useful at times. In spite of all treatment most of the cases steadily progress, often in an irreg- ular manner for many years. TUMORS OF THE BRAIN. Every variety of tumor attacks the brain, solid, granular and tic. In fact, the intracranial structures seem to be a favorite seat for new growths, whether they appear elsewhere in the bod) or not. \- compared with other organic diseases of the brain the) are not so com- mon. They seem from the reports to be more frequent than tumors of the spinal cord. They partake of the same characteristics in a gen eral way as they do when in other parts of the body. The only thin- that makes them of special interest in cerebral patholog) is the effect of their presence upon the functions and histological structures of the brain itself. Only in some few particulars does their intracranial seal cause them to exhibit special differences. For instance, fatty tumors are comparatively less frequent in the brain than the) are elsewh gliomatous tumors are never found outside of the skull except in the retina and spinal cord. It is more common for massive tubercular growths to appear in the brain than elsewhere, Aneurism constitutes a variety of tumor hut it will be discussed in a section by itself as it is more of a disease- of the circulatory apparatus than a new growth. Etiology.— There is nothing specially distinctive about the etiol Ogy of brain tumors more than what is true of them when located 9IO THE NON-NEURONIC DISEASES elsewhere in the body. Their essential causes are but little known. The granulomata, tubercle and syphiloma are, of course, infectious in origin; some of the gliomata and fatty tumors owe their existence to developmental anomalies and other congenital influences ; echinococci and cysticerci are parasitic ; actinomycosis is an infectious form of tumor. Heredity is supposed to exercise a slight influence in predisposing to the tumor itself and its particular variety. Males suffer about twice as often as females. This may be due to the greater incidence of cer- tain exciting influences in the former than in the latter in some cases but certainly not in all. The difference between the sexes in this respect varies with the nature of the tumor. It is with sarcomata that the sexes approach more nearly to an equality. All ages, from six months to advanced years, are subject to brain tumor. Certain tumors exhibit a choice of age in their appearance. In the first twenty years of life, one-third of all cases occur. The relative number diminishes then until it becomes quite small after fifty. The reason of this is obvious since the infectious and other conditions out of which the tumor is evolved are more common in these years. Tuberculous tumors are found most frequently in childhood and early adult life, though they may be seen up to old age. Parasitic tumors are incidents of early adult years. So also are gummata and gliomata. In early and middle life the sarcomata join the gummata and gliomata in the frequency of their occurrence. Carcinomata have been diagnosed at all ages but most of them have been seen between the ages of forty and sixty. The late tumors are the sarcomata, gummata and carcino- mata. The rarity of all the other varieties of tumors renders their gen- eral etiological characteristics of slight importance. Traumata of the head, such as blows and falls, are undoubtedly in some instances the excitants of a morbid growth. So also may be old cicatrices. In some instances the tumor may have been latent but the trauma initiated the secondary disturbances upon which notice- able symptoms arose. Hence traumata must not be given too much credit for the production of intracranial neoplasms. Pathology. — It is difficult to make a perfectly satisfactory classi- fication of all the tumors that attack the brain, for our knowledge in regard to them is not entirely adequate and they vary in so many par- ticulars. Neither their frequency, their location, their causation nor their intimate structure will form at present a basis for accurate clas sification. They may be enumerated, however, thus: tubercle, syph- iloma, glioma, sarcoma, myxoma, carcinoma, fibroma, osteoma, choles- teatoma, lipoma, psammoma, neuroma, echinococcus and cystic ere us. To these may be added erectile tumors or vascular enlargements, actin omycosis, abscesses and cysts due to hemorrhage and encephalomalacia. and dermoid cysts, which will not be discussed here. Some of these growths may be single or multiple, simple or com- plex. They may vary in consistency and intimate appearance in the course of their development. A broken down glioma looks not the same as an original one. A sarcoma may be limited or diffused over a considerable area. A tubercle may be single or multiple. An osteoma THE NON-NEURONIC DISEASES 911 and a fibroma may seem to be amalgamated into an osteofibroma. In the same way osteosarcomata, gliosarcomata, angiosarcomata and fibrosarcomata may be recorded. A tuberculous tumor is usually a single, rounded mass, varying in size from that of a nut to that of a hen's tgg or closed fist. These tumors are not miliary as they are seen to be in tubercular meningitis though rarely the whole brain has been infiltrated with minute miliary concretions. They are the most frequent of all the brain tumors and are slightly more often located in the cerebellum than in the cerebrum. There may be more than one tumor. They are readily detached, as they do not infiltrate though they damage somewhat the encircling brain tissue. The tumor itself is not vascular, hence when it breaks down it exhibits an opaque, cheesy aspect, sometimes softened here and there, but not very extensively. Calcification and pus formation are among its possible late alterations. It may surround itself with a fibroid capsule. Apparently the growth starts in the lymphatic sheath of the blood vessels. Vascular obstruction, thrombosis, meningitis, cerebral softening and inflammation are among its complications. The tubercle bacillus carried to the brain by way of the blood stream is, of course, the immediate cause of the morbid development ; hence tuber- culosis of the lungs, articulations and other parts of the body is apt to be present though not necessarily so. All the usual characters of a tubercle, such as the appearance of the cells and the presence of the bacilli, belong to these tumors as they do to the same tumors else- where. Sometimes a syphiloma so closely resembles a tuberculous tumor that only the presence of the specific bacillus in the latter enables one to distinguish them apart. It varies in size from a pea t<> a walnut and is irregular and nodular in shape. Like tubercle it is non-vascular and tends strongly to caseation. It does not become purulent like tubercle. On section it exhibits scattered, chees) spots separated by firm, reddish-gray fibrous tissue. The periphery is irregular, thick and grayish with sometimes numerous vessels and evidences of arte- ritis. The surrounding brain substance is not infiltrated though it is atrophied and otherwise damaged. Old gummata may be BOft and broken down though less markedly so than tubercle, or they ma\ rear shrunken, fibroid and encased in a capsule. All of the late changes are different from those of tubercle and help to differentiate the two. In these there is a recognizable connection with the menin and a tendency to extend along the surface of the brain in a grayish, gelatinous sort of membrane. With these i> often associated a gum- matous meningitis; with tubercle there may be a scattered miliar) tuberculous condition in the vicinity. Gummata are mosl frequently met with in the cerebrum and pons Varolii, rarely in tin cerebellum and basal ganglia. Tiny an- superficial and are attached t.. the pia mater. Even deep gummata haw their origin from the pia usually. Like tubercles they may sometimes ^tart in the dura mater and thence invade or only compress the brain. Tin- growth ma vascular walls* elsewhere than in the meninges, though The sarcomata are among the most interesting and ft' the 912 THE NON-NEURONIC DISEASES brain tumors. They form a large group and on account of their posi- tion, combinations and integral changes they assume various modifi- cations. They range in size from a nut to a fist. They are of connec- tive-tissue origin and therefore start from various tissues. They seem particularly to grow from the periphery into the cranial cavity. The bones at the base of the skull, for instance, the sphenoid, the temporal and the occipital often form their nidus. The dura, the periosteum, the other membranes or the brain substance itself may be their starting point. They are primary or secondary and their internal changes and amalgamations give rise to myxosarcomata, gliosarcomata, fibrosarcomata, osteosarcomas, etc. They are of ail shapes and may be single or multiple. Rapidly developing, as a rule, they are grayish or white in appearance or yellowish according to the admixture of cells and bloodvessels. They are harder than gliomata as a rule and only occasionally reveal patches of caseation and disinte- gration. They are rich in cellular elements, especially round and spindle cells. Sometimes they possess an alveolar structure and they may even break down into cysts. Pigmentary, calcareous and other changes may develop within them. They grow into the cerebral sub- stance but are as a rule quite sharply defined from it. They may even be encapsulated. As endothelioma they contain endothelial cells from the lymphatics. Sometimes they infiltrate without displacing the brain substance. Round about them the tissues are destroyed somewhat and they are often accompanied by meningitis. A curious condition is a diffuse, spread out sarcomatous manifestation in the meninges along with the tumor. Gliomata are not as frequent as seems to be commonly supposed. They are strictly neural tumors and are often due to congenital anomalies. They never involve the meninges and bones and usually appear in the cerebrum or cerebellum, rarely anywhere else. They de- velop out of the neuroglia tissue and so infiltrate the brain substance that they can never be shelled out as tubercle can. They range in size from a hazel nut to a hen's egg. The largest tumors of the brain are the gliomata. In the middle they are reddish-gray or yellowish-white, according to their age. Sometimes they look very much like the brain tissue itself and have the same consistency. They undergo various changes so as to be mucoid or fibrous in character. Especially prone are they to disintegrate and being vascular, to exhibit signs of old hem- orrhages. Cysts and cavities are liable to form in their center. The rapidity of their growth varies and they rarely, if ever, become en- capsulated. On microscopic examination the typical glia cells, small, spider- like cells with delicate, fibrous prolongations are seen, mingled with an abundant supply of bloodvessels. The formation of the tumor sug- gests strongly an inflammatory process with reactive neurogliosis. The tumors are much more easily detected by their appearance when situated in the white matter than when in the gray matter of the brain. Of course they damage the tissues which they infiltrate though sometimes intact fibres will be seen coursing through them. The brain is altered in shape and enlarged where they are located. The so-called THE XOX-XEUROXIC DISEASES 913 hypertrophy of the pons, for example, is simply a condition of glioma- tous infiltration. It has been confounded with sclerosis of the brain and optic neuritis accompanying hemorrhage or softening. The tumor is usually single though multiple glioma is a very rare possibility. As a primary growth carcinoma of the brain is very rare. Usually it is secondary to cancer elsewhere. It is a soft, vascular, irregularly outlined growth that partly infiltrates and partly displaces the braia tissue. It may spring from the dura and spread out or it may be a circumscribed or diffuse neoplasm within the brain in any situation. As compared with other tumors it is rather frequent in the central ganglia. It is both single and multiple, sometimes symmetrical in both hemispheres. Its contents are colloid, consisting of large cells sometimes with several nuclei. Wernicke refers to their tendency to softening and destruction of all the tissues. Fibromata are small, very rare tumors of the brain, occurring chiefly in the cerebellum and cerebellar peduncles. They have been seen multiple and single. Dana intimates that the enlarged and hard- ened pacchionian bodies may be regarded as of the nature of fibromata. Calcification in tumors and actual bony tumors occur in the brain and may be single or multiple. Osteomata or fibro-osteomata usually develop in the dura mater and its processes. Psammomata are small fibrous growths, filled with calcareous par- ticles, that develop in the meninges, pineal gland and choroid plexus. They are hemispherical, smooth, hard and gray. They show no marked tendency to grow. In the hypophysis adenomata may develop and be accompanied by acromegalic symptoms. Cholcstcatomata or pearly tumors are found sometimes post mor- tem at the base of the brain in the recesses of the skull. They are composed of epidermoid cells in concentric layers. They give the ap- pearance of white, shining nodules and are said to contain cholesterine and stearine. Usually they are innocent but at times they develop as large as a fist, growing into the cerebral substance'. Xcuromata, small growths containing nerve elements, melon- omata, lipomata, angiomata, enchondromata, and teratomata, are all SO exceedinglv rare as to be unworthy of lengthened consideration. Cysts of 'the brain, excluding those due to hemorrhage and sofl ening, are parasitic in origin. These arc hydatid or cysticercous. The former is usually single and found in tin- cerebral hemispheres, either in the ventricles or the white substance Rarel) it has been seen in the membranes, the central ganglia, the cms. pons, cerebellum and me- dulla. The echinococcus is tin- cause of tin- hydatid cyst Those due to the cysticerens cellulosa are- apt t<> be- multiple-, as many as tut;, and a hundred cysts having been counted. They are- si.,u in growth and are scattered over the- surface- of tin- brain and in the- ve-ntriele-s. are encapsulated and provoke no symptoms. It must not be forgotten that cysts are sometimes due- to degenerated m-. .plasms, a. is seen so frequently in gliomata. As an immediate- or remote result ol th< neo 914 THE NON-NEURONIC DISEASES plasm in the intracranium, certain changes are produced in the brain and surrounding structures. The gross appearance of the brain is altered. There is a flattening of the gyri and an arching out of the cortex. This is not noticed when the tumor is cortical or meningeal in origin. The softening and destruction of the brain substance around the tumor have already been referred to. Displacements and distortions are effected by the extension of the growing mass. Where the space is confined, as beneath the tentorium, the displacements, as for instance of the pons by growths in the cere- bellum, are sometimes very considerable. On account of the obstruc- tion of the passages between the ventricles internal hydrocephalus of- ten results. Tumors of the cerebellum and quadrigemina act thus. Compression of the venae Galeni which carry the blood away from the choroid plexuses is responsible sometimes for the hydrocephalic con- dition. Irritation and inflammation, especially in the meninges, is an- other effect of the presence of the tumor. And sometimes the cranial bones are thinned out, actual perforation having been observed over the tympanum. x\ll of these secondary results are produced by direct pressure and destruction of the growing mass ; sometimes by remote and indirect pressure as the result of the foreign body being thus confined within a fixed cavity. The choked disc, so-called, and other cranial nerve in- juries are the result of compression of the nerve by the growth. In children the general intracranial increase of pressure has caused a separation of the sutures. In a few instances even spinal cord changes have been observed, due possibly to the general increase of pressure in the cerebrospinal canal, to nutritive disturbances and to toxic fac- tors. Symptoms. — The brain is an organ inclosed within unyielding walls ; it is a compound organ with special functions belonging to each of its constituent parts. A tumor, therefore, will cause two distinct sets of symptoms, the general, due to the general increase of intracra- nial pressure, and the special, or focal, due to the particular location of the neoplasm and the part of the brain that is damaged. The important general symptoms of a tumor of the brain are headache, choked disc, retardation of the pulse, vomiting, vertigo and generalized spasms. While the objective symptoms are the most re- liable for diagnostic purposes, the subjective, especially the headache, are usually the first to appear or be noticed. Cephalalgia occurs in about two- thirds of the cases and is very variable in character. It is less paroxysmal and more progressive than most other headaches. It is usually generalized over the whole head, though there are at times tender spots, especially upon percussion with the fingers. Very often, in spite of its constancy it undergoes sudden and sharp exacerbations. As a rule it is dull, boring, aching and dis- tressing. Its very constancy is exasperating and wearing. At other times it is sharp, lancinating and lightning-like. Pa- tients are often driven to contemplate suicide, so agonizing is it. It breaks the sleep at night, it unnerves the patient and almost upsets his THE NON-NEURONIC DISEASES 915 mind. It is very often worse at night and occasionally manifes certain degree of periodicity in its daily appearances. The location of the maximum point of the pain is suggestive oi the location of the tumor. Occipital headache with the pain extending somewhat down into the neck is suggestive of a growth beneath the tentorium, in the posterior fossa or cerebellum. Frontal headaches occur when the neoplasm is in the centrum ovale whether anteriorly or posteriori)-. Superficial tumors very often lie immediately under the tenderest spot in the scalp. The focal pain due to implication of the origin of the superior branch of the trigeminus often combines with frontal head- ache. Forced expiration, sudden jars, difficult defecation, straining of all kind, sneezing and coughing usually increase the headache. An- other peculiarity about them is that to all forms of medication that overcome other headaches, these are steadily resistant. It is not clear entirely what is the immediate cause of these headaches, though it is assumed to be a pressure and irritation of the membranes. In all forms of tumor this symptom is a prominent one, though it is less so perhaps in the gliomatous variety and those that grow more slowly. The most valuable symptom of brain tumor for surety of diagno- sis is undoubtedly the appearance of the eye-ground. Choked disc occurs in four-fifths at least of all cases. For obvious reasons it is not always the first symptom noticed by the patient. He tampers with glasses and opticians for his failing sight for a long time, often before his headache, until eyesight and other suspicious signs finally drive him to consult a physician. It results from a form of optic neuritis which may be bilateral or less frequently unilateral. Most commonly it is bilat era! but more pronounced on one side than the other, generally the same side as the tumor. It is constantly present in tumors of the corpora quadrigemina, and in four-fifths of the cerebellar tumors. It is absent in nearly two-thirds of the cases of tumor of the pons, medulla and corpus callosum. The size of the tumor seems to have very little direct influence upon the presence, severity or course of choked dise. It seems to be less frequent, however, in tubercle and most frequent in glio- matous and cystic enlargements. Irritation, pressure, engorgement ^i the nerve sheath and associated meningitis have all probably something to do with the causation of the optic neuritis. Tin course of the in- flammation is variable though generally progressive. In the end com- plete optic atrophy usually obtains. The use of the term choked disc may be misleading, diverting tin- mind from the primary inflammatory nature of tin- trouble, but if this possibility is guarded against it is still a good one t«. emploj in the de- scription' of the appearance <>f the fundus caused b) the neopla forms of optic neuritis as distinguished from tin- syphilitic and some other forms. Primary optic atrophy never develops as a result of brain tumor. The sight is not much impaired if the neuritis is moder ate, but curiously enough is often diminished as the inflammation sub sides. The secondare atrophy that follows it is always a late sign and may be useful in indicating an older age in the tumor when the other symptoms have been slight or only of recent on 9l6 THE NON-NEURONIC DISEASES Vomiting is the next important symptom and should probably be added to the headache and the optic neuritis as one of the pathogno- monic tripod of indications of brain tumor. It occurs in tumor of all parts of the brain. It is most frequent, pronounced and obstinate in cerebellar and pontile neoplasms. It is an early symptom as a rule though it seems to be largely an accompaniment of the headache and vertigo. It is a true cerebral phenomenon and hence is peculiarly in- dependent of the taking of food or of the condition and contents of the stomach. It may come on at any moment and is projectile in char- acter. A lad whom I saw with a brain tumor had, before consulting any physician, adopted the habit of always carrying a towel with him for he had been often embarrassed by vomiting under most unexpected circumstances. There is not much nausea with the vomiting and the examination of the expelled matter fails to reveal any cause for it. In a general way it is more characteristic of rapidly growing tumors. In about half of the cases dizziness and persistent vertiginous, in- toxication distress are spoken of. In rarer instances there are definite attacks of rotatory vertigo. When the tumor is in the cerebellum espe- cially, these occur in conjunction with forced movements. Sometimes the patient complains of vertigo only upon rising or turning suddenly. Vertigo is least frequent in tumors of the cerebral hemispheres, some- what more frequent when they are located in the central ganglia and most frequent when the tumor is in the cerebellum, cerebellar peduncle, pons or corpora quadrigemina. It may be explained partly and in some cases by the disturbance of those important avenues of afferent impulses, the optic and auditory nerves, but it cannot be entirely so accounted for. Under the head of vertigo and Meniere's disease, I have discussed this symptom and its mechanism somewhat more in detail. In nearly all cases, sooner or later, the mind is affected. This is .a direct effect of the pressure and irritation and not due merely to the prolonged suffering caused by the headache. The latter, of course, has a depressing and exhausting effect, too. Stupor and coma are the common terminal manifestations, especially just before death. It is always a sign of great significance. Early in the disease and when the tumor is small it may be absent. In extracerebral tumors it is also less frequent. The patient seems to.be profoundly asleep and hard to arouse. He is dull, heavy and stupid when he is aroused. He seems to drop asleep most easily and on the most extraordinary occasions. At table he will drop his knife and fork, stop masticating and fall back in his chair. Defecation and urination may occur in the same way. Early in the trouble there may be only a slight failure of memory, with depression and partial lethargy. In other cases there is exhilaration, high emotional excitement, silliness, restlessness, delirium or mania with hallucinations and delusions. Asylum cases of insanity show upon post mortem quite a number of brain tumors. Sometimes the disease evokes only hysterical manifestations in individuals predisposed to this psychosis. Large tumors and frontal tumors are supposed especially to be characterized by these psychic manifestations. General convulsions, which, however, are not epileptic in character THE NON-NETJRONIC DISEASES 917 though they are often associated with unconsciousness, occur among the general symptoms of brain tumor. They occur in all stages of the dis- ease, closely resemble epilepsy, and in some instances are even pre- ceded by prodromata. One-fourth of the cases are subject to these attacks. Sometimes they are distinctly apoplectiform. True epileptic and Jacksonian manifestations occasionally occur. They may even pre- cede all other symptoms by many years. These functional attacks may range all the way from petit mal seizures up to those of brief, tetanic rigidity. Oppenheim suggests that when epilepsy thus occurs it is not so much a direct product of the tumor as an independent disease in a brain predisposed to the development of both tumor and epilepsy. Retardation of the pulse, to forty or thirty beats per minute, is not so very uncommon in intracranial growths. It is the result of the general intracranial pressure. It is never an early symptom. In tumors near the region of the vagus nucleus the pulse rate tends to unwonted rapidity. It has been found by Gray, Mills, Lloyd and others that the cranial temperature is often somewhat raised. As the normal tempera- ture of the scalp is uncertain, the abnormal cannot be employed very successfully as a means of distinguishing the presence of a tumor. According to Bruns, a typanitic or cracked-pot sound is obtained sometimes by percussion of the cranium immediately over the site of the tumor. In a limited experience I have not been able to observe this symptom. Oppenheim says it occurs under other conditions and is always present in sucklings. Before the days of cerebral localization it was not possible to carry the diagnosis of a tumor of the brain much beyond its mere pres- ence. Under such circumstances the above symptoms constituted prac- tically the whole of the clinical picture so far as the diagnostician was concerned. The order of fheir appearance, the relative frequency of sensory, motor and special sense phenomena, their limitations, degree of severity and other incidents in regard to them, were matters of in- terest, but of little practical value. Since the development of our knowledge in regard to the special functions of the various parts of the brain all of this has been reversed. It is not enough now to diag nose the presence of a tumor; we must attempt a definite localization of it within the intracranial mass. This undertaking presupposes a thorough knowledge of the anatomy and physiology ^>\ the brain, the chapters upon which, as well as the chapter np<»n cerebral localization, should be consulted by the reader. I hie. however, a nw words upon the focal diagnosis of intracranial growths art- in order to comprehend what is said elsewhere and to emphasize tin- clinical picture produced en masse by the peculiar localization of- the tumor. Whereas the study of tin- general symptoms upon which the diag nosis of the mere presence of the tumor is based takes precedence always in point of time tin- search for and stud) of the particular manifestations upon which tin- definite localization of the growth is found far outrank it in importance. \s the signs of localization | suppose and include all those that indicate th«- existence of the tumor. 9l8 THE N0N-NEUR0N1C DISEASES they alone would constitute the basis for the diagnosis if they were always present. Localizing symptoms are not always present. Their absence is to be accounted for in three different ways. In some cases the morbid growth is so large, and the intracranial pressure is so high that the general symptoms overwhelm and completely mask all localization signs when they are present. In other cases the neo- plasm, small and capable of provoking sharply defined localization symptoms, is embedded in one of the so-called silent parts of the brain, as, for instance, the frontal and temporal lobes of the right side. Multiple tumors may produce such a confused clinical picture that the focal signs, though present, are of little use in the diagnosis. Dana affirms that one-seventh of all tumors are multiple, including, chiefly, tuberculous, cancerous and melanotic growths. And finally in still other cases there may be all the while fine and definite localizing mani- festations present which our knowledge of brain physiology falls short of interpreting. Much as we know of the functions of the brain, there is much still, as, for instance, in the production of the mental phe- nomena, that we do not yet know. A few gross motor and sensory manifestations do not exhaust all of the functional activities of this wonderful organ and in our localization researches upon it we have only entered the outer portals of its mysteries. Not more than one- third of its cortex and its constituent elements are in direct communi- cation with the outer parts of the body by way of the nerves, and most of our symptomatologies are drawn from the body and neural mani- festations. What symptoms, beyond the few mental symptoms now in our possession, we are to attribute, directly or remotely, to the mys- terious workings of the remaining two-thirds time alone can tell. In many cases of brain tumor none but general symptoms occur throughout the entire course. In others the localizing signs are dis- tinct and definite, even in some instances overshadowing the general symptoms. As a general axiom it may be said that the smaller the tumor and the less marked the signs of intracranial pressure, the more definite and direct will the focal symptoms likely be. A small tumor may rarely be located in a functionally known part of the brain and yet no localization symptoms be manifested. Such a growth in the white substance of the cerebrum has slowly separated the fibres without functionally damaging them. Again, what is called action from a dis- tance may lead to an erroneous focal diagnosis, but this is exceedingly rare. Much more important among these indirect manifestations of the tumor are what are called the neighborhood symptoms. If carefully studied, these indirect symptoms sometimes add to the value of the direct and so confirm the focal diagnosis. The best way to discuss the focal symptoms of brain tumor is perhaps to take up seriatim the old established anatomical divisions of the encephalon, the fossae of the skull and finally some particularly prominent centers of the brain. Frontal Lobe. — Tumors in the anterior part of the frontal lobe, the prefrontal region, do not produce any characteristic motor or sen- sory symptoms. There is a change in the character and temperament. T H E NO X - N E L' RO N 1 C DIS EA S ES 910 generally, of the patient. He is elemented, is dull or extremely emo- tional. He laughs and cries easily, is silly and quickly angered. This is more apt to be the case when the left lobe is affected ; tumors oi the right lobe are as a rule absolutely silent. As the tumor grows and compresses and extends backward and downward other symptoms arc- added to the mental. Encroachment upon the posterior part of the frontal lobe and the central convolutions causes irritative motor signs such as muscular spasm. The speech center may be involved in the third left frontal convolution causing aphasia, preceded by a sort of FIGURE 201. (From Sprat ling.) distaste for speech, a mutism. Tumors in the inferior part of tin- may produce olfactory and visual disturbances b) compi the olfactory and optic nerves. In this way hemianopsia and optic neu ritis of a severe type ma> be produced. Even the orbit ma) U vaded and the ocular globe paralyzed and protruded. When Un- conjugate deviation of the eyes with muscular spasm in tin- face, the diagnosis of frontal tumor is positive. Brans' invesl have revealed to US why frontal tumors sometimes produ< Har ' 1 1 ' 1 \ 1 a Parietal Lobe.— Excluding the posterior central mors of the parietal lobe cause preeminentl) senson ma They are not prominent and with them are mingled neighborhood 920 THE NON-NEURONIC DISEASES symptoms. The muscular sense is lost, as a rule, and therefore the gait is ataxic. As with the frontal lobe, if the growth encroaches upon the central convolutions, spasms, contractions, convulsions and paraly- ses may occur in the related muscles. The lower limbs are affected by high situated tumors, the arms, neck and head by the lower ones. As many tracts between the occipital lobe and the central course through the white areas of the parietal lobe, tumors in its lower part give rise to alexia and visual aphasia. Still deeper growths may cause hemianopsia. Near the angular gyrus is the cortical representation of the third nerve and its damage has resulted in paralysis of this nerve. This may explain some rare cases of vertex gummatous men- ingitis with motorocular palsy. I once saw such a case. The symp- toms of the specific meningitis pointed clearly to the vertex of the brain and the only cranial nerve that was involved was the third. Some symptoms of a sensory character due to implication of the parietal cortex are probably neighborhood symptoms and due to the close connection between the cortical elements of the parietal lobe and those of the central convolutions. Astereognosis is a symptom of parie- tal tumor in nearly all cases. Central Convolutions. — These are the most responsive areas of the brain from the standpoint of localization. Being chiefly motor in function, and grouping the entire musculature of the body in their representation, their injury can be studied objectively and in detail. They constitute the motor zone and exhibit most pronounced focal manifestations. With the size of the tumor, of course, will vary the extent of the manifestations throughout the movements of the body. Irritative- signs are among the first to appear and as a result Jack- sonian epilepsy obtains. Sensory aurae often precede the local spasms. If the tumor is small the epileptic condition may be without disturb- ance of consciousness and be confined to the face or the arm or the leg. As the neoplasm enlarges, however, the muscular convulsions be- come more extensive and the consciousness may be lost, the whole clinical picture assuming the complexion of a typical grand mal seiz- ure. Even in these elaborate cases, the consciousness is not lost at first and the aurae and spasm usually begin always in the one place, thus enabling the localization to be made. The hemiplegic character of the primary manifestations should be carefully noted. As the destructive action of the tumor goes on, the spasms are replaced by a gradually deepening paralysis. Monoplegias and hemiplegias are the forms that this assumes. Sensory disturbances usually accompany them, con- sisting of various paraesthesiae such as numbness, pricking, pins-and- needles sensation, loss of pain, touch and temperature sense, and even complete hemianesthesia. Even the muscular sense may be involved and the stereognostic sense. Local twitchings in certain muscular groups, followed by spasm and paralysis in the same groups, asso- ciated with similar sensory phenomena in approximately the same localities, are the sign manual of a growing neoplasm in the central lobule. Aphasia and agraphia may also be present. The exact point of the cortex impinged on and the details of the localization can be worked out by reference to the chapter on cerebral localization. It THE NON-NEUHONIC DISEASES 0,21 must be remembered that the tumor may not actually be in the cen- tral cortex. It may be in the meninges over the center or in the white substance of the brain beneath it. Under such circumstances irritative symptoms of the motor centers will be very gradual in onset, paroxys- mal in character in the beginning and only very late become posi- tively and permanently paralytic in character. However, there is great uncertainty about this as well as about the age and size of the tumor from such localizing indications. A very large tumor, for instance, of the centrum ovale may just touch the motor centers, as it were, and produce only slight irritative symptoms. It is thus obvious that implication of the motor zone of the cor- tex furnishes us with our happiest localizing symptoms. Their origin, their location and their onward march from slight twitching to com- plete paralysis are seen in no form of lesion so beautifully and regu- larly characteristic as in tumor in or about this region. Temporal Lobe. — In right-handed people the right temporo- sphenoidal cortex is practically latent. In the left temporal cortex are the centers for hearing and smell and taste. Word-deafness occurs with tumor of the first and second convolutions, also amnesic aphasia and various forms of paraphasia. Pressure by a temporal tumor on the neighboring parts of the brain may cause a variety of symptoms. Posteriorly it may provoke optic aphasia : anteriorly it may disturb the centers for taste and smell both in itself as well as in the cornu ammonis. If the tumor is verv low in the lobe so as to compress the medulla and adjoining parts it may cause attacks of vertigo with forced movements and a mixed type of aphasia. Hemianesthesia, hemiplegia and hemianopsia may all be caused by such a localization of the neo- plasm. The reason is clearly the implication of the great conducting tracts in the brain stem. Occipital Lobes.— The" visual centers of the cortex are the great characteristics of these lobes. A bilateral tumor in them produces complete amaurosis. Hemianopsia is the distinguishing feature i>\ a tumor in one of the lobes. As lesions of the optic tract may cause the same visual disturbance, it is not a particularly valuable sign for locating the tumor in the occipital lobe. When, however, it is accom- panied by sharp occipital headache, extending down the neck the pre- sumption is strong that the growth is occipital. Psychic blindness, word-blindness, soul-blindness or the incapacity t«» comprehend the nature of things seen are some of the curious results of tumors in the occipital lobe. 'Extension of such a tumor int.. the parietal lobe and impingement upon the internal capsule may be surmised by the hemi- anaesthesia, unilateral ataxia, and even hemiplegia. Central Ganglia.— -Tumors of this region like those «-t tin- corpus callosum are only obscurely diagnosed and from the standpoint surgical intervention are of no interest whatever. Most of the symp- toms are due to the damage done to the internal capsule, either directlv or through pressure. Gradual and partial hemiplegi distinctive symptom <>r there may be unilateral, indefinite twitchi from a tumor in the striate- body <>r the thalamus irritating the 1 SUle and its fibres that go coursing down between them. Hemichoi 922 THE NON-NEURON IC DISEASES athetoid movements on one side of the body, involuntary and automatic jactitations, hemiansesthesia and hemianopsia of the opposite side, are all possible and their mechanism easily understood. There are no known symptoms produced by a growth confined solely to the cor- pora striata or optic thalami, unless it be located in the posterior part of the latter or pulvinar. Growths in the pulvinar cause hemian- opsia with distinctive hemiopic pupillary reaction or inactivity, a fact which distinguishes this form of hemianopsia from that produced by disease in the occipital lobe. Tumor of the basal ganglia is usually marked by a considerable degree of stupidity. Corpus Callosum. — Tumors here are not frequent and their focal symptoms are not very distinctive. There is a peculiar mental state consisting of intense stupidity, drowsiness, sopor, mutism and mental deadness. For hours the patient will sit immobile, seemingly lost to every stimulation from the outer world. The absence of most of the localizing signs of tumor and the gradual increase of those that are present are two of the more striking features of tumor in this re- gion. Absence of cranial nerve disturbances and absence of anaes- thesia are especially noteworthy. Hemiplegia with a lesser degree of paralysis on the other side, or bilateral hemiplegia of equal intensity on both sides, is suggestive. Coma usually terminates these cases. Dana thinks that symptomatically tumors of the great commissure are the same as or closely correspond with tumors situated in the third and lateral ventricles. In other words they belong to all of that class of tumors which begin in the central parts of the brain and gradually extend outwards toward the periphery. Corpora Qnadrigemina. — Tumors here usually involve also the crura cerebri and pineal gland, or vice versa. These structures are very complex and important organs. In them are located the pri- mary centers of sight and hearing. Their occupation by a tumor therefore causes hemianopsia or blindness, impaired hearing or deaf- ness. With this hemianopsia there is paralysis of the ocular muscles as a rule. The pupillary reflex is lost and there is nystagmus. Ambly- opia may occur and the same ocular muscles may be paralyzed bilat- erally. Incoordination, forced movements and an intention tremor, as well as a tremor like that of paralysis agitans, may often be ob- served. Most of these are neighborhood symptoms and occur whether the tumor have its origin in the pineal gland, the gemina or the crura. A special characteristic of implication of the latter is superior alter- nating hemiplegia, a condition in which there is oculomotor paralysis of one side with paralysis in the extremities of the other side. A curious and rare analogue of this is the alternation of oculomotor palsy with a hemiataxia of the other side, due, according to KrafTt- Ebing, to a tumor in the tegmentum. These latter conditions are ex- tremely rare. Cerebellum. — Cerebellar tumor of a certain type is perhaps the most definite of the localizable lesions. Its diagnosis is therefore com- paratively easy. Tumors in the lateral lobes of the little brain are usually silent until they press upon the medulla, the pons and the re- lated cranial nerves, when they produce close neighborhood symptoms. THE NON-NEURONIC DISEASES 923 Vertigo and incoordination are the distinctive symptoms, though not strictly focal, of a neoplasm in the middle iobe or vermis. Cerebellar ataxia is a reeling, drunken gait. It has been called a titubating gait It is unfortunate that the term unstable equilibrium is not used instead of ataxia, because the gait is not the same as that seen in tabes. A true ataxia may develop rarely in the extremities and be bilateral or unilateral. Sometimes closing of the eyes increases the disturb- ance of equilibrium and on account of it the patient may actually fall. A distressing and constant vertigo accompanies usually this symp- tom. Vomiting, appearing early and continuing during the entire disease, is a typical symptom. Forced movements, turning involun- tarily and automatically one way or the other, are also quite prominent in some cases. The general symptoms of this tumor are st rough marked, as a rule. The headache is occipital and extends with mus- cular rigidity into the neck. It may be frontal in some cases. ( )ptic neuritis is sharp and well defined. Opisthotonos, general convulsions. tetanic rigidity of the whole body, unilateral and bilateral hemiple- gia, may all occur from extreme pressure upon the great tracts run ning through the medulla. Other medullary symptoms that may not be unlooked for are glycosuria, cranial nerve and nuclei disturbance and the usual signs of bulbar paralysis. Internal hydrocephalus is also possible from pressure on the venae Galeni. Pons Varolii and Medulla Oblongata. — These parts of the brain are subject more frequently to tuberculous and gliomatous tumors than to any other variety. Occasionally no symptoms are produced by them for a long time but sooner or later a very characteristic clin- ical picture is presented. The details of this picture ma\ undergo a great variety of presentations but in the main its chief outline is quite uniform. The prominent feature in the symptomatology of tumors^ of the brain stem is the combination of cranial nerve palsies and hemiplegia. In most tumors the cranial nerve palsy will be on the same side as the tumor, the paralysis of the limbs on the opposite side. This is the well-known alternating hemi- plegia and is easily comprehended by a glance at the physiological anatomy of the parts. The paralysis of the limbs will, ^i course, be of the cerebral or central neurone type, while that of the cranial nerves will be of the spinal or peripheral neurone type. The size and exact location of the tumor must l>e worked out 1>\ a stud) of tin- particular cranial nerves involved and the extent of the henupKy In about one-quarter of the cases the cerebral nerves alone are in volved, in another quarter the limbs are affected, in nearh a half are both the nerves and limbs involved. In almost eight \ five per cent, of the latter the paralysis i^ of the alternating variety. In high tumors of the pons the hemiplegia is combined with oculomotor paralj sis; in low tumors with trigeminal paralysis. Lower down still in the medulla the other nerves ma\ hi- Involved seriatim such as the facial, the abducens, the auditory, etc. Bilateral cranial nerve palsy, bilateral hemiplegia and all the symptoms of a true progressive bulbar paralysis may appear when the tumor is large, bilateral ..r t , rather than upon any one or two of them. As many signs should be discovered therefore as possible. Some cases arc- latent and present no signs at all, for a long time at least. Others exhibit only on, two symptoms, like headache, for instance, long, even years, before the appearance of others. Some cases present only the general symp- toms ; now and then a rare rase may exhibit merely some one or two focal, without general, manifestations. M<>m cases present both gen- eral and focal symptoms sometime in their course though the loca tion of the tumor, its age. its nature and man) Other factors will v;ir\ very greatlv the relative values of tin- respective general and focal symptoms. Subjective symptoms are commonly tin- first t<> be no ticed; objective symptoms are tin- most reliable for diagnostic pur- poses. Though the patient usually presents himself on account the headache, the examiner should at once institute a minute search for objective signs. The most important of the latter is without doubt optic neuritis. No headache should be allowed t<> pass without a careful examination of the eye-grounds. From eighty t<> ninet) per cent, of the cases of intracranial tumor have optic neuritis and choked disc. It is a characteristic condition and is not exactl) like other fomu 9^8 THE N0N-NEUR0N1C DISEASES optic neuritis. It is practically confirmatory of the trouble. A some- what similar choked disc occasionally appears in meningitis, cerebral abscess, renal disease and hydrocephalus, but these can be distin- guished by other symptoms. Papillitis may be due to other causes than tumor, especially when chlorosis, lead poisoning, polyneuritis or alcoholism are present. A central scotoma for colors speaks for alcoholic intoxication. With the headache cerebral vomiting is almost always present. Vertigo is also a frequent accompaniment. The headache must be studied from every possible point of view. It is usually characteristic in brain tumor though often it varies from the typical and by resembling other forms of headache, like hemi- crania, the cephalalgia of hysteria, neurasthenia, constipation, infec- tion and toxaemia, may add much confusion to the diagnosis. It is well in such cases to diagnose the neoplastic headache by exclusion. A search should be made for all possible causes of the headache apart from tumor. If then papillitis and vomiting can be added to the symp- tomatology the diagnosis is complete. Cephalalgia, optic neuritis, cerebral vomiting, constitute strong grounds for the opinion that a tumor is present. As these symptoms are not always present all to- gether there are others that when added to any one of these makes the diagnosis strongly presumptive, practically positive in some cases. Vertigo is not of much importance. Progressive stupor is, however ; so also are retardation of the pulse, general convulsions, epileptic attacks of a particular type and various speech disturbances. The progressive character of the symptomatology is a significant feature. It will be noticed that all of these symptoms except the optic neuritis may occur in certain non-organic diseases, such as hysteria, neuras- thenia, epilepsy and certain psychoses. It is necessary to exclude these first, therefore, which may be generally readily done by search- ing for the other symptoms of these various neuroses. It is to be remembered, however, that as a rare possibility a latent tumor may be present with all the signs of a mere neurosis or psychosis. Of course, no differentiation can be made then. An organic disease, and that a tumor of some sort, being finally decided, the location of it is the next question to be considered. I have shown under the head of symptoms how the focal diagnosis may be both clear and obscure. I have also dwelt upon its supreme im- portance. While so strongly suggestive of a neoplasm, focal symp- toms are sometimes produced by other diseased conditions, as for instance, a focal meningitis, an aneurism, an old hemorrhagic cyst or spot of necrobiosis. Even in hysteria, epilepsy, alcoholism, uraemia and paralytic dementia localized manifestations both of a motor and sensory character in various parts of the body may occur in such a way as to closely simulate the focal symptomatology of brain tumor. The progressive character of the latter taken in conjunction with the characteristic general symptoms helps much in the differential diagnosis. It seems incredible how closely even focal organic symp- toms may be simulated by the neuroses and diffuse organic diseases. Gowers mentions the case of a hysterical little girl who after watch- ing for some time a genuine case of brain tumor, mimicked it almost THE XOX-XEUROXIC DISEASES 929 perfectly. Monoplegias, focal anaesthesia, limited irritative and de- structive simulacra and actualities may occur under many other cir- cumstances, functional as well as organic, than those of an intracra- nial growth. The keeping close to the typical symptomatology of tumor and contrasting it with the total symptomatology or entire clin- ical picture of these other diseases is the only safe way to determine the origin and focal value of the former. The presence of a tumor and its location having been established, it remains only to fix its nature. As multiple tumors are always in- operable, whatever their location may be, they must be carefully dis- tinguished from single growths. Such tumors do not, as a rule, afford any definite localizing symptoms by which an operation can be guided anyway and so the diagnosis of the single tumor is the great desideratum after all. It is not always easy by any means to deter- mine what sort of a growth the patient is afflicted with. Certain gen- eral infectious states, such as syphilis and tuberculosis, will afford a strong presumption as to the nature of the neoplasm. The existence of tumors in other parts of the body, like cancer of the breast, actino- mycosis of the jaw, hydatids of the liver, presupposes the same sort of a tumor in the brain. Tubercle and gliomata occur most frequently in the cerebellum and brain stem ; sarcomata at the base of the brain and outside of it; syphilomata in the cortex. The rapidity of growth, general course and extension of the tumor help slightly to suggest its nature. Cerebral gliomata are slow and gradual in their development Tumors that are rapid at first and then stationary are probably tuber- culous. The results of treatment will occasionally determine a syph- ilitic neoplasm from all other varieties. The main diseases from which intracranial tumor is to be care- fully differentiated are epilepsy, migraine, hysteria, alcoholism, lead intoxication, abscess, intracranial aneurism, dementia paralytica, acute encephalitis, disseminated cerebrospinal sclerosis and melancholia. The reader is referred to the sections upon these various affections for their differentiation from tumor. Lumbar puncture has been found useful in the diagnosis of a few obscure cases. Increased pressure, leucocytosis and the presence of bacteria may point to a tumor and meningitis, though their absence does not exclude the former. Lately skiagraphy has been employed to locate a cerebral tumor. In some instances, particularly when the growth was superficial or near the vertex, the shadow w. tiring. In deep tumors the Rontgen ray does not seem to be of much availability. Metal obj< such as shot and bullets are much more distinctly seen in skiagraphs than are the ordinary tumors. Prognosis. — The course of brain tumor is usuall) a prolonged one, ranging from a few months to - t number of years. Jackson ported a glioma that had apparently lasted for ten years, and I >sler one in which Jacksonian symptoms had persisted for fourteen y< Most of the cases run their career in about two or three duration of the tumor (Upends upon its nature somewhat and the ter mination of the case is often effected by the destruction of the neigh- 93° THE NON-NEURONIC DISEASES boring brain substance. Gliomata and fibromata are the most en- during tumors. Especially prolonged are cholesteotoma and angioma. Carcinoma, some varieties of glioma and soft sarcoma are apt to be rapid. Tubercle, especially when it appears in childhood, and even other forms of tumor, have become stationary. Syphiloma is the only tumor that seems capable of disappearing entirely, and then only un- der treatment. It, as well as partly degenerated gliomata, may leave permanent residua. By discharging their degenerative debris through the nose and mouth, the effects of certain growths have been quite ameliorated. Very rarely a brain tumor may become encapsulated or atrophied, with a recession of its symptoms. In other cases after a long period of quiescence" or slow progress it may suddenly light up most violent symptoms with coma and the patient pass quickly to his death. Altogether tubercle in children and gummata in adults offer the most favorable .prognosis : rapidly progressing gliomata and sarcomata the most. A sarcoma may prove fatal in six months. Lo- cation of the tumor has much to do with the prospect of the patient's continued existence. Bulbar tumors are particularly dangerous. Subsidence of the headache, and recession of the optic neuritis, are favorable signs of a possible recession or stationary condition in the neoplasm, or at least its environment. Marked mental stupor, con- vulsions, severe vomiting, rapid and intense optic neuritis and apoplec- tic seizures are bad omens. I have seen a marked recession of all symptoms with an ultimate optic atrophy remaining in a case which several years ago had been told by several competent neurologists that he could not possibly live the year out. Our prognosis should always be made guardedly in cases of brain tumor. A slow, chronic optic neuritis indicates that the disease will probably be of slow progression. Spontaneous recovery has been seen in the parasitic tumors, and spontaneous metamorphosis and retrogression are believed by some to occur sometimes in tubercle. It is believed that some reported cases of the disappearance of all symptoms of brain tumor were cases of Quincke's serous menin- gitis or idiopathic internal hydrocephalus. Death results from bulbar symptoms, exhaustion, infection, com- plications and intercurrent maladies. Treatment. — Every case of tumor presents a study for medical and surgical treatment. In this is included the question of radical cure or only amelioration. In most cases the entire treatment of the case resolves itself into the management of the patient and his comfort, without any hope or attempt at modifying in the least the morbid growth. The administration of medicines will have, therefore, for its twofold object the eradication of the tumor and the relief of the pa- tient's sufferings. A clear comprehension of the limitations of the medical treatment of brain tumor will not only lead to the avoidance of some ridiculous errors and absurd logic but it will enhance the value and efficacy of the small powers with the limitations. A mere temporary relief of the headache will not be interpreted then as a THE NON-NEURONIC DISEASES 93I cure, a diminution of the convulsions will not be looked upon as a hopeful sign of the disappearance of the neoplasm, nor will the diag- nosis previously well founded be shaken by mere temporary changes in the general clinical picture. On the other hand, what can be done by medicinal agents in a few cases will be so sharply defined that there will be no hesitancy or lack of energy in applying the few effective measures in the properly selected cases. Therapeutic sins of omis- sion as well as commission are sometimes strikingly enacted in eases of brain tumor. In no disease is prompt, thorough, correct treatment so much of a desideratum, and in no disease are the indications for it more obscure sometimes. Time must not be wasted with medicines if the tumor falls into the operable class soon to be mentioned. Sur- gery must not be coquetted with if the tumor is amenable to medical treatment or is inoperable. Symptomatic relief, by all available and surgical means, must be promptly and fulsomely given if all hope of affecting the tumor itself is past. In a word, given a correct diagno- sis of the location and nature of the growth, and a thorough appre- ciation of its proper relationship to medical and surgical interven- tion, the treatment should be prompt, thorough, rational and perma- nent. The treatment of syphilomata by appropriate medication is not only successful but it furnishes us sometimes with some of the most brilliant results known to medical science. The disappearance of some of the most threatening symptoms is accomplished almost as if by magic. The more recent the tumor and the shorter the time that the focal symptoms have existed, the more complete and satisfactory is the cure. After a syphilitic growth has existed for a long time and irreparable changes have taken place in the brain tissue, no treatment can be expected to banish the manifestations that depend upon those changes. And yet even irr many advanced and old cases of this sort. a decided and grateful retrogression of the symptoms is often brought about by antisyphilitic treatment. Every case that presents a history or suspicion of syphilis should be- at once put upon antispecific reme- dies. The iodide of potash, started at twenty grains three times a day, should be rapidly increased until two or three hundred grains ;i da\ are administered. If in a week or two this is not accompanied b\ s< change for the better in the symptoms, mercurial inunctions should be added to the regimen. For details of the treatment, consult the chapter on cerebral syphilis. In tin- case of tumor there is not any ground for fear in the use ot this heroic antisyphilitic medication ;i< there may be in the degenerative conditions of the parasyphilitic dlS eases, locomotor ataxia and paretic dementia. Within reasonable lim- its, such as would undoubtedl) show some result, in a genuine case of syphiloma, it can do no possible harm whatever the tumor max Nay, more, it is a fact that even in non-Syphilitic neoplasms the io,li,le of potash has very often caused .1 noticeable amelioration of the head ache and other symptoms. Henc< it is good pi i for .1 short time with antisyphilitic medication all 1 tumor in which there is a shadow of a doubt about its specific origin, and in which the evidences do not point most emphatically awnv from syphiloma. 932 THE NON-NEURONIC DISEASES By such a procedure under these circumstances there is everything to gain and nothing to lose. It is extremely doubtful whether any other than syphilitic tumors can be directly affected by internal medication. Cases of improve- ment and even cure have been reported in tubercle by the use of cod liver oil, tonics, rest and a general hygienic mode of life. Gowers says that more than once he has known the symptoms of cerebral tubercle, steadily increasing under country air and abundant food, to become permanently arrested when the patient was taken into a London hos- pital and cod liver oil and iron given. It is known, however, that this class of morbid growths very often, after a period of rapid and rather violent activity, become stationary, and especially so when the gen- eral constitution is put in the best condition of health. These happy results are most attainable in children, in whom it is believed that tubercle has been made to disappear completely. Little as we know about the reparative processes of the treatment in these cases, the latter is apparently so fortunate in some few instances, that it recom- mends itself for all cases of tubercle. The general hygienic and tonic treatment can only be of advantage to all cases of tumor, tuberculous or otherwise. It is to be hoped that some specific remedy will be dis- covered some day to act upon these tubercles as the iodides act upon syphilomata. The tuberculins that have lately been experimented with in tuberculosis of the lungs and joints, have not succeeded in giving any very promising results in cerebral tuberculosis. Arsenic has been recommended and said to have shown a cura- tive power in a few cases of sarcoma. It is a tonic, as we all know, and affects powerfully the metabolism of the body. If it only strength- ens the organism so as to give it a fighting capacity against these growths, or if it assists in their transformation into more stationary forms such as fibrosarcoma, it is a most available agent and should be given a fair trial. The same statements apply to phosphorus. No other tumors than those just mentioned respond to medical treatment and so far as we know no other remedy has any direct effect whatever on intracranial neoplasms. Symptomatic treatment therefore constitutes the only field for the further use of internal medication. This, however, is of great importance and may be called for whether an operation be a part of the question or not. The head- ache is the chief symptom that calls for relief. Most of the remedies that are useful in other forms of cephalalgia are distinguished, as a rule, for their incfficacy in this form. Hence in most cases nothing but morphine will avail. To be sure there is always the danger of the formation of the morphine habit to be taken into serious consider- ation. If the nature and location of the tumors are not clear and if there is a probability that it may prove to be operable when it an- nounces itself a little more definitely, morphine had better be with- held and temporizing undertaken with other drugs. Unless the head- ache is excruciating, threatening to drive the patient to suicide, as it almost does sometimes, all other possible means of relief had better be tried first before morphine is resorted to. I prefer to try the fol- lowing remedies in the order mentioned: cannabis indica, phenacetin. THE NON-NEURONIC DISEASES 933 acetanelid, antipyrin and large doses of sodium salicylate. Other measures commonly employed in the management of headache should not be neglected. An ice bag, cold water coil or hot cloths may be applied to the head. A mustard foot bath and a sinapism to the back of the neck may help somewhat. Purgation, free diuresis and diaphor- esis, even leeches and venesection may be considered. As a rule the case will prove to be desperate and resort will have to be had to mor- phine. In inoperable, severe or moderately severe cases with an un- favorable prognosis constantly hanging over them, it seems to me more beneficent to establish, if need be, the morphine habit and give the patient some comfort the few months or years he has to live, than to allow- him to be harassed and worn out, perhaps sooner than he would be even with the morphine habit, by the increasing and intol- erable headache, vertigo, vomiting and convulsive seizures. For these latter symptoms there is sometimes mitigation in full doses of the bro- mides, chloral and other narcotic antispasmodics. In nearly all cases the question of surgical intervention will arise. In not more than five per cent, of the cases will the tumor be found to be operable. The early enthusiam following the work of Hors- ley, Macewen, Godlee and others has calmed down considerably , but yet when we consider what is being done at the present day by such operators as Wernicke, Horsley, Bergmann, Keen and others, and compare the promising outlook offered by their results with the utter hopelessness with which these cases were regarded only a few decades ago, we may congratulate ourselves without undue elation upon the splendid advances of surgery. Five per cent, is not a large number of cases amenable to oper- ation but that is not the fault of surgery, it is in a large measure the result of the location and nature of these tumors. Multiple tumors are inoperable; so also are malignant and metastatic growths. Deep neoplasms usually cannot be reached without almost certain risk, hence they cannot be undertaken. Large infiltrating growths must also he left alone. The infiltration of a small, superficial tumor is not a ground for its being regarded as inoperable. The most favorable tumors are those that are single, superficial, not too large, more or less encap lated and distinctly localizable. These are tin- fibromata, growing from the dura and compressing the brain in and about the motor rone. Small tumors, early diagnosed, offer the best chances also, though large growths have been removed piecemeal in successive operations. In some cases in which the localization of tin- tumor is somewhat in doubt an exploratory operation is to be recommended. Tumora the occipital lobe, causing hemianopsia, and those in the temporal lobe which cause auditory disturbances max hi- regarded as amenable to the knife. As the opening of the cranial cavity is always a serious procedure, the relatives should always Ik informed of all p<>s M bie accidents. They should also be made well acquainted with the queke that are likely to follow the removal of the tumor or excision of the brain substance- with its removal. The continuance of the paralvsis should be carefully explained beforehand to them, if the 934 THE NON-NEURONIC DISEASES operator wishes to avoid most awkward questions after the recovery of the patient from the operation. In some cases, focal symptoms alone may justify an operation. A persistent aphasia, especially if it be progressive, may well suggest a trepanation over the third left frontal convolution. Cerebellar tumors do not encourage one very much to operate. Their removal may be possible in adults but is not to be thought of in children. The trouble is that if the tumor is in the lateral lobe it does not usually produce symptoms until it is too large for surgical in- tervention, and if it is in the middle lobe where it usually causes the liveliest symptoms, it is in too close proximity to vital centers that are liable to be damaged in the handling of the growth. Nevertheless it is to be remembered that both Macewen and Parkin operated success- fully upon a cerebellar tumor. The symptomatic treatment of brain tumor by operation is always to be considered when the removal of it is out of the question. Tre- phining, withdrawing some of the fluid and thus lessening the intra- cranial pressure have afforded temporary relief of the headache, stu- por and optic neuritis. When symptoms are severe and blindness is threatened one can readily see the commendability of such a procedure, though the benefit may be only temporary. In some cases the ventricles have been tapped in the hope of re- lieving the pressure symptoms. The results have been very doubtful, however. The same is to be said of lumbar puncture, a most doubt- ful procedure. INTRACRANIAL ANEURISM. An aneurism is a variety of tumor, soft, pulsating, attached to a bloodvessel, and terminating usually in rupture and hemorrhage. In- tracranial aneurisms may be within the brain, on the minute terminal arterioles that penetrate its substance, or between the brain and the skull on the large arteries found there. The former are called miliary aneurisms, are small and produce no symptoms until they rupture and allow the blood to pour out into the brain substance. They are of interest chiefly in connection with intracranial hemorrhage, under which head they have been considered. The large aneurisms may be formed anywhere where there is a large vessel in the intracranial cavity. By far the greater number of them occur on the large arteries at the base of the brain. Etiology. — The causation of intracranial aneurisms is in the main the same as that of aneurismal dilatations elsewhere in the body. Men are more subject to the disease than women, in the pro- portion of about three to two. The reason for this is probably not an inherent difference between the sexes, but a difference in their modes of life. The greater tendency to exposure, strain and dissipation in men leads to a greater deterioration of the walls of their arteries. This is shown in another way when the age at which these aneurisms occur are considered. For instance, they all appear between the years of ten and sixty, and within this period the greater number show themselves during the first half or when exposure, strain and dissipa- THE NON-NEURONIC DISEASES 935 tion are most rife. In some families there seems to run a slight in- heritance toward arterial disease. This has been mentioned before in connection with intracranial hemorrhage. The nature of this con- genital influence we know absolutely nothing about. It is an inheri- tance not of the disease itself but of the predisposition to the disease, a very important distinction to keep always in mind. With or with- out such a predisposition an aneurism may be initiated by various exciting causes chief of which are blows, shocks and other traumata to the head. The immediate cause of the dilatation is a diseased state oi the arterial walls. The muscular and elastic tissue is diminished and re- placed by a fibroid and otherwise degenerated tissue. Under the pulsations and pressure of the blood stream the wall distends but does not contract again as it should. In the course of time the bulging be- comes larger, more permanent and finally constitutes an aneurism with thin walls, pulsating and variable in shape. Primary degeneration, syphilis and emboli, especially when in- fected, are the chief factors in the production of the disease of the vascular walls. The first is largely an accompaniment of old age and with traumatism is perhaps the cause of most of the aneurisms in other parts of the body ; the second and third are conditions of early adult and middle life and are the principal elements in the produc- tion of intracranial dilatations. The primary degeneration may be -fibroid or atheromatous; even extensive fatty and calcareous break- down may be seen. Specific arteritis is a condition so well known that it need only be mentioned here. Emboli, especially infected em- boli, are the products of endocarditis. Minute vegetations break off from the diseased valves of the heart, float away to the brain on the blood current and finally become lodged at the bifurcation of some artery. Obstruction takes place, the vessel becomes closed and oblit- erated in front of the obstruction, inflamed and degenerated behind it. The cause of this inflammation is irritation and infection. These causes of the arterial disease explain clearly why the basilar and ver tebral system of vessels are so much more frequently affected in ad- vanced years while the anterior and middle cerebral-, especially the latter on the left side, are seen to be the choice site of the aneurism in earlier years. PATHOLOGY. — Scarcely more need be said upon the patholog) of this condition than what has been mentioned under tin etiology. The arteries that are afTected in the order of their frequency an- the mid die cerebral, basilar, internal carotid, anterior cerebral, posterior com municatingi anterior communicating, vertebral, posterior cerebral and inferior cerebellar. Those of tin- left side far outnumber thos< the right. Within tin- central ganglia or ventricles aneurism is i rare. Usually the swelling is single though a multiple condition ma\ obtain. Its histological attributes arc- the same as all aneurisms and need not be discussed here. In developing it compresses and mon less damages the neighboring brain tissue. Nerves in juxtaposition may also undergo destruction. Even tin- bone tis>iir maj be eroded 93^ THE NON-NEURONIC DISEASES by its steady pressure. The presence of the tumor is generally rec- ognized by the symptoms of this collateral damage to bone, brain and nerve. After a time the aneurismal sac bursts and a superficial, com- pressing, severe hemorrhage occurs, the blood escaping in most cases into the membranes at the base of the brain. This hemorrhage may be continuous or intermittent, through a small or a large opening. The ventricles may become gradually filled with the outpouring blood, or the latter may tear or disintegrate its way into the brain matter. From the internal carotid it may burst into the cavernous sinus. Symptoms. — The symptoms of an intracranial aneurism are prac- tically those of a tumor. They are general and focal. The terminal hemorrhage has its own special symptomatology which is closely sim- ilar to that of meningeal apoplexy. Headache is of a pulsating char- acter, constant, intense and accompanied often by local tenderness in some spot. Vertigo, giddiness, vomiting, projectile in character, stu- por and occasional convulsions are all characteristic symptoms and are due to the general intracranial increase of pressure. Cranial nerve palsies are frequent, though optic neuritis is not common. The usual aneurismal murmur is not often produced by the intracranial variety of dilatation. It was noted in aneurism of the internal carotid and left vertebral arteries. So many conditions give rise to cranial murmurs that the symptom is not very valuable here for diagnostic purposes. A pulsating vascular murmur may be heard sometimes in cases of tumor located near and compressing a large artery. It may be noted in hydrocephalus, anaemia, rachitis, and even in perfectly healthy children. Exophthalmic goitre and neoplastic compression of the sympathetic nerve have provoked cephalic murmurs. These general symptoms are the offspring mostly of moderately large aneurisms. With them may be associated certain focal symp- toms indicating where the tumor is located. In small aneurisms it may rarely happen that slight focal symptoms are presented but no general manifestations. The localisation of an aneurism by means of its focal exhibitions follows the same general rules that the localization of an ordinary tu- mor follows. Motor and sensory disturbances should be carefully noted and compared with one another and their correlation with the areas of the brain should be made in accordance with the known facts of brain physiology. When the aneurism is in the internal carotid artery there is com- pression of the optic nerves, the oculomotor nerves, the olfactory and the trigeminus. The sight of one eye therefore fails, ptosis occurs on the same side, the sensibility of the eye is lost with all its usual consequences and the power of smelling is damaged. Hemiplegia may occur from the pressure backward on the crus cerebri. On the left side the speech center may be implicated with the production of motor aphasia. The free communication between the ophthalmic and facial veins usually quickly relieves what transient fullness may occur in the retinal veins from pressure on the cavernous sinus. Michel finds that the occasional optic neuritis that is seen is not due to compression THE NON-NEURONIC DISEASES 937 but to extension of inflammation from the vicinity of the aneurism. When rupture takes place, as it does in a little less than half the cases, the cavernous sinus is invaded and a pulsating exophthalmus may result. Swellings on the anterior, middle and posterior cerebral arteries can be surmised from symptoms pointing to implication of the ad- jacent cortical centers. With the first, for instance, the symptoms are the same as those in aneurism of the internal carotid except for the involvement of the third nerve. Aneurism of the middle cerebral, or artery of the Sylvian fossa, provokes hemiplegic convulsions and speech disturbances especially. The cranial nerves are notably free from involvement. Being near the motor zone, Jacksonian symptoms, followed by monoplegia and hemiplegia, are the characteristic features. Sometimes they cause no outward evidences of their presence. About two-thirds of them rupture. A posterior cerebral aneurism is apt to compress the upper part of the pons, the crus cerebri temporo-sphe- noidal lobe, the third and sixth nerves. Alternating hemiplegia there- fore is to be expected. An enlargement of the anterior communicating artery produces, as a rule, no symptoms, because it is usually small and compres merely the inner sides of the frontal lobes. If it is large the olfactory and optic nerves may be affected. When the posterior communicating vessel is the seat of the dilata- tion hemianopsia may be produced by compression of the optic tract. The temporo-sphenoidal lobe and basal ganglia may be compressed. The third nerve is the one most commonly implicated. Hemiplegia is rare. Vertebral aneurisms do not often cause serious trouble. In fact the condition in these arteries is usually one of gradual extensive dilatation of the artery tharkthat of a typical aneurism and the symp- toms, such as they are, are notable for their slowly progressive char- acter. The symptoms are more or less similar to those- of basilar aneurism. The basilar artery is a frequent site of both aneurism and uniform dilatation. Its close proximity to the pons, the medulla and the c< bral crura indicates the possible symptomatology. The general symp- toms, especially the headache and vertigo, arc unusually pronounced. Pontile symptoms, such as alternating hemiplegia, upper cranial nerve involvement, and forced movements, occur when tin- swelling is high up. Bulbar symptoms with dysarthria, dysphagia, cardiac and respira- tory difficulties combined with hemiplegia appear when the enlai ment is lower down. Internal hydrocephalus occasionally occurs. I vulsions are rare. Occasional apoplectic attacks sometimes happen. About half the cases rupture. Cerebellar aneurisms are of no interest clinically. Prognosis. — An intracranial aneurism is a most dangero session. The greater number of those that have produced Sufficient symptoms to enable them to be diagnosed during life have soon< i later terminated by rupture. The duration of these enlargements 93& THE NON-NEURONJC DISEASES ranges from a few weeks to six years. An occasional spontaneous cure has resulted from the filling of the sac with a clot; Treatment. — The dyscrasia that causes the disease in the walls of the arteries would seem to invite treatment but it is hardly to be expected that such treatment will modify an aneurism after it has once developed from such a vascular condition. Syphilitic history there- fore recommends the use of potassium iodide and mercury to prevent at least the extension of the disease in the arterial walls. Atheroma and other degenerative conditions may be attacked, but little real benefit will accrue thereby to the aneurism. The use of potassium iodide in moderate dosage, thirty or forty grains a day, has seemed to favor intra-aneurismal coagulation and spontaneous cure in a few instances. It should be tried. Ergot is useless and irrational. In the general regimen and mode of living everything should be done to avoid momentary or continuous increase in the intracranial blood pressure. Tight bands about the neck must be eschewed. Strain- ing at stool, giving way to passionate anger, coughing, running to catch a train, must not be permitted. Stimulants, like alcohol and coffee and tea, must be left alone. An even, quiet existence with a bland, nutritious dietary and little drink should be constantly main- tained. In sleeping the head should be elevated and in waking never extended or flexed on the body too far. Surgical measures have been tried for the cure of these aneurisms but without any very flattering results. Only in the rarest cases is the diagnosis sufficiently precise to cause surgery even to be thought of. Dilatations on the internal carotid have been diagnosed and the artery itself ligated with success. The vertebrals have been tied for a basilar aneurism. The risk of the operation, great as it is, is less than that of the aneurism. Lumbar puncture must be discountenanced. Most disastrous results have followed it. When rupture occurs, the physician's special services are at an end. HYDROCEPHALUS. This is another term representing merely a symptomatic con- dition, which, it is to be hoped, will ere long be allowed to follow the terms paralysis, apoplexy and Landry's paralysis in neurological nosology. It means merely dropsy of the brain, represents an accu- mulation of fluid and is the result of a considerable number of dis- eased conditions. It is never strictly a primary condition. Its imme- diate causes may be congenital, developmental anomalies, mechanical obstructions or meningeal inflammations. It is therefore both a con- genital and an acquired condition. From the standpoint of duration, the former is sometimes spoken of as chronic, and the latter as acute, hydrocephalus. This is clearly an injudicious use of adjectives, for acquired hydrocephalus may be chronic and a congenital form may run a rapid, acute course. On the basis of pathology, when the fluid occu- pies the ventricles, the hydrocephalus is said to be internal; when it is outside of the brain in the subdural space it is spoken of as external. Internal hydrocephalus has a varied etiology, as will be seen later on. THE NON-NEURONIC DISEASES External hydrocephalus, or hydrocephalus ex vacuo, is seen in senile conditions, atrophy of the brain, hemorrhages, encephalomalacia and sclerotic conditions, in cancer and other cachetic diseases, in chronic nephritis, chronic alcoholism, rickets, meningeal cysts and other chronic troubles and pathological residua. This, of course, is not a true dropsy, but rather a pathological, secondary effusion. It will be practically convenient, though somewhat arbitrary. t<» discuss the malady under the three subheads, as Osier does. Serous Meningitis. Congenital Hydrocephalus and Acquired Chronic Hydro- cephalus. These are all internal types of hydrocephalus. The ex- ternal types might be added as a fourth subhead, but as they are merely symptomatic of so many different disease states, their discussion would be an unnecessary recapitulation of what has been said under the head of these diseases elsewhere. Serous Meningitis. — This is a form of internal hydrocephalus whose pathogenesis is obscure. Quincke has studied it very closely and arrived at the conclusion that it is due to a simple serous inflam- mation of the meninges of the ventricles with effusion, analogous to serous pleuritis. It is thus an ependymitis and one wonders at the significance of the name sometimes applied to it, idiopathic internal hydrocephalus. So much doubt is there in regard to its inflammatory nature that Quincke himself likens the process to that of angioneurotic oedema of the skin, and speaks of it as an angioneurotic hydro- cephalus. It is easy to understand the secondary nature of the effusion in this form of ventricular dropsy but the cause and nature of the primary trouble in the ependyma or related structures are far from being established. In acute cases the ependyma exhibits no abnormal appearance whatever; in the chronic cases it is rough and thickened. In some cases distinct signs of inflammation appear, not only in the intracerebral pia, the choroid plexus, but in the cortical pia also. In the latter case there is also an external hydrocephalus. < me hypoth< though far from being a satisfactory one. attributes the ventricular effusion to the closure of the foramen of Magendie by the simple basal meningitis. The disease occurs almost a> frequently, however, without any indications of meningitis, internal or external. The exudate is believed to arise chiefly from the choroid plexus and the walls of the ventricles. It does not materially differ from the normal cerebrospinal fluid. If its specific gravit) should be- ab 1009, w * tn albumin present in larger amount than two per thousand, tumor or stasis is more likely to be the original cause of the drop In some cases it has been thought that tin- entire disease owed inception to a trauma, mental strain, alcoholism. various acute ini tious diseases, purulent otitis and toxaemia. It is quite impossible, in our present state of knowled rately classify this form of hydrocephalus. It seems to occur as both a congenital and acquired affection. It appears in children but adults are more frequently attacked by it. It runs both an acute and a chronic course. In its essential origin it is not alv decide whether it is a primary or a secondary manifestation. It is usually internal though it may be accompanied b> an external dropsy. In 94° THE NON-NEURONIC DISEASES some cases inflammation, in others mechanical obstruction, and appa- rently in a considerable number of cases, a developmental functional defect may be at the bottom of it. No name, beyond hydrocephalus, that has hitherto been given to it is strictly correct. Symptomatically there seem to be various well-marked types of the affection. As an acute trouble it closely resembles sometimes either a purulent or a tuberculous meningitis. It is not improbable that some reported cures of the latter disease have been cases of this form of hydrocephalus. Headache is present. There are signs of increased intracranial pressure. The discs are choked. The pulse is retarded. The neck is retracted. The fever is slight, inconstant and usually ab- sent. It may be present at first and then disappear. In some cases loss of the knee-jerk has been observed. Lumbar puncture may aid much in the making of a differential diagnosis. In meningitis of the purulent or tuberculous type, the cephalalgia is apt to be keener, the retraction of the neck more forcible and all the other well known signs, such as occur in the pupils, in Kernig's phenomena, in the stu- por, in the gait, more pronounced. Excessive visual disturbance points rather to the hydrocephalic condition. Bacilli in the cerebrospinal fluid and the discovery of tuberculosis or local infection elsewhere may clinch the diagnosis. Sometimes a differential diagnosis is absolutely impossible. This acute hydrocephalus usually recovers completely or partially in a few weeks or passes into a chronic form of the trouble. When it has become chronic, this form of cerebral dropsy is very liable to be mistaken for tumor of the* brain and cause a false notion of a cure of the latter when the symptoms have disappeared. Like tumor it provokes both general and focal symptoms. The former in- clude intense headache, slight fever, vomiting, vertiginous attacks, somnolence, stupor, delirium, retardation and acceleration of the pulse. Optic neuritis with choked disc, and later on atrophy, are very fre- quent manifestations. Exophthalmos may occur. Hemianopsia, local spasms, muscular rigidity, temporary monoplegias and hemiplegias and signs of cranial nerve involvement are among the more important focal symptoms. As the clinical presentations of this hydrocephalus have the remarkable property of undergoing exacerbations and varying degrees of intensity from day to day, they can be distinguished from the progressive symptoms of tumor. Moreover the hydrocephalic skull is in evidence in most of the cases, showing that though the cases occur mostly in adults in this chronic form of the trouble there has been all along a congenital predisposition. This, of course, is not the case in tumor. The diagnosis of this hydrocephalus rests primarily upon its pressure symptoms, its characteristic remissions and intermissions, its temporary focal exhibitions or absence of them, its congenital hydro- cephalic predisposition, and its cranial nerve involvements. The nor- mal character of the cerebrospinal fluid as distinguished from the albuminous fluid of tumor is helpful in making a differentiation in some cases. In children the diagnosis is less difficult than in adults because the hydrocephalic deformities of the head are more prominent in them. The total clinical picture, studied in every possible way and with all THE NON-NEURONIC DISEASES 941 possible aids to examination, must form the basis for the diagnosis of so obscure a disease rather than one or two symptoms only. This picture must be both broadly and minutely contrasted with that pre- sented by intracranial tumor, purulent meningitis, tuberculous menin- gitis and some other diseases. Even then, unfortunately, the diagnosis cannot be made positively in a good many cases. Recovery frequently follows, in these cases, many weeks of suf- fering. In spite of the presence of intense headache, vomiting, stu- por and optic neuritis, mercurial treatment has eventuated in a com- plete cure after several weeks or months. The course of the disease is always remittent. Sometimes it becomes chronic and in children cannot be distinguished from the ordinary form of congenital hydro- cephalus. Death occurs rather infrequently. Here could be discussed logically some cases of acquired hydro- cephalus that run a course, and in many respect are, similar to Quincke's type of the disease. This is especially true of some of the cases of secondary external hydrocephalus. One form of the latter has already been referred to, as it is sometimes regarded as primary and aligned with similar cases of so-called angioneurotic hydro- cephalus. Most of the cases, however, are associated with atrophy of the brain, which is supposed to cause a vacuum with resulting effu- sion between the brain and the skull. Hence the name hydrocephalus ex vacuo. Other cases, both internal and external, are attributed to trauma, insolation, congestion, obstruction of the venae Galeni. of the aqueduct of Sylvius, or of the foramen of Monroe by various neo- plasms, cysticerci, cicatrices, etc. Purulent and tuberculous menin- gitis after the same manner may be accompanied by dropsy of the brain, as also may phthisis, nephritis, marasmus and various cachexias. In many of these cases a slight hydrocephalus, or at least hydrocephalic tendency, has probably existed from birth and the causes mentioned have only awakened it from its latency. At all events the cases are not in any way distinctive and are of no special clinical interest be- yond that of the disease with which the hydrocehalus is associated. Acquired Chronic Hydrocephalus. — This then is the affection to which what has just been said is largely applicable. It is always sec- ondary, even in what is sometimes called the primary or idiopathic form which is in great need of thorough pathological reinvestigation. Mechanical obstructions and meningitis are the primary cause of the trouble in nearly all of the cases. All of the etiological factors in this form of the trouble take their cue from tin- etiology of the primary cause. In adults, of course, the head will not be enlarged though there may be an intense degree of dropsy present. In children the skull may and usually does undergo early and steady expansion. Even in them the enlargement may not hi- commensurate with tin- severity of the other symptoms. The brain substance may he so compressed and attenuated that the ventricles are enormously distended while the gen- eral size of the head remains about normal. The external appearances are often the same in children as they are in the ordinary congenital type of the disease. The symptoms are extremely numerous and varia- ble, especially in the adult. They art- those chiefly of intracranial 94 2 THE NON-NEURONIC DISEASES pressure. In children idiocy and imbecility occur, in adults stupor, delirium and maniacal outbreaks. In rare instances acquired, men- ingitic external hydrocephalus is sacculated on account of the ad- hesions between the pia and the dura. The condition is a veritable meningeal cyst. The limited pressure upon the brain by such cysts may give rise to pronounced pressure focal symptoms. There is hardly any need of dwelling here upon the headache, the vertigo and vomiting, the optic neuritis, the cranial nerve palsies, the tremors, the spasms, the stupor and coma that are observed in these cases. Tumor of the brain probably causes the greatest difficulty in the differential diagnosis. (See the discussion of this in the preceding paragraphs under the head of internal meningitic hydrocephalus.) Congenital Hydrocephalus. — This is the typical form of the trou- ble, the most common and the one generally referred to when the term hydrocephalus alone is used. It is practically always internal. Its course may be acute or chronic. The etiology of the disease is ex- ceedingly obscure. It seems to be more than merely congenital at times. The appearance of the condition in several members of the same family and in two and three successive generations indicate a purely hereditary influence. In conjunction with this fact it is sug- gestive that various cachexias, alcoholism and syphilis have been reck- oned among its etological incidents. I have seen it in syphilitic fam- ilies. Oppenheim has several times observed a moderate hydro- cephalus in hereditary syphilitics with severe symptoms coming on later in life. Of the direct prenatal causes that are responsible for it we know nothing. Psychic excitement and traumata sustained by trie mother while she is carrying the fcetus, uterine disease, etc., are some of the hypothetical causes that have been enumerated. The pathology of the disease is capable of being described but its pathogenesis is still completely in the dark. The essential fact in its pathology is the excessive accumulation of fluid in the ventricles. The fluid varies but little from the normal. It is clear, colorless, has a specific gravity of from iooi to 1009 and contains a small and variable amount of albumin, a slight quantity of sodium chloride, occasionally some urea, cholesterin and other unimportant substances. The amount varies in different cases from a few ounces to several pints. It usually distends all of the ventricles, particularly the lateral. It may be lim- ited to particular ventricles by the mechanical obstruction or inflam- matory closure of the inter-communicating ducts. The other pathological findings are the direct result of the con- stant pressure and distention exerted by the fluid. The most remark- able of these is the atrophy and wasting, the thinning out and displace- ment of the brain substance. It is astounding at times to what an ex- tent the wall of the hemispheres can be reduced. Both compression and stretching is the cause of this. The entire brain may seem to be transformed into a huge cavity inclosed by walls not more than a few millimeters thick. It is said that Cardinal, Brigfht's famous patient, lived to be twenty-nine years of age, with a hydrocephalic head per- fectly translucent when the sun was shining behind it. The convolu- tions and sulci, of course, are obliterated. The corpus callosum is car- THE NON-NEURONIC DISEASES 943 ried upwards, the central ganglia are flattened out and the floor of the third ventricle bulges beneath. Sometimes the convolutions are only partially obliterated and the basal ganglia are found at the bottom of the sac. The skull is uniformly enlarged in correspondence with the cerebral distention. The falx is stretched and the tentorium is elevated anteriorly. The space beneath the tentorium is increased in size and is incompletely filled by the cerebellum. Either fluid or connective tissue may occupy the space between the cerebellum and the membranes. The choroid plexuses are thickened and seem to have been inflamed while the ventricular ependyma is often finely granular in appearance and sodden. The external appearance of the head is striking and characteris- tic. It is uniformly enlarged with a strong tendency towards being dolichocephalic or boat-shaped. As the face remains small the front part of the cranium hangs over it like a beetling brow. The promi- nences are all enlarged, smooth and rounded. A balloon-like appear- ance is thus strikingly given to the upper part of the head. The cir- cumference, measuring normally in the newborn about thirty-five to forty centimeters, may reach the enormous size of a hundred and sixty-seven centimeters. The bones are thin, the sutures gaping, the fontanelles large and bulging. On account of the downward pressure on the orbital plates, the eyeballs protrude and the eyes are directed downward and partially covered by the lower lids. Wormian bones sometimes develop in the sutures of the skull. The entire shape of the head is such that it can be readily distinguished from the square- shaped, angular, rachitic head. Ofttimes developmental defects are seen in other parts of the body, such as encephalocele, hare-lip, spina bifida, club-foot and a general condition of dwarfism. Albinism has been noted. In addition to the physical appearance of the child, other symp- toms enter into the composition of the clinical picture of this disease. The head may be so large as to obstruct the act of parturition ; very often, however, it does not begin to be very noticeable until the child is several weeks or months old. From that time on there is then a steady increase in the size of the cranium. The veins appear prom- inently beneath the skin. The hair fails to grow. Auscultation en- ables one to detect a brain murmur and percussion reveals a fluctua- tion wave. Idiocy and imbecility is the lot of these children. The intellect is weak even in the mildest cases and the speech slowly acquired. Rarely a hydrocephalic child will be bright mentally; the disease becomes ar- rested; and adult life may be reached with normal faculties. In still rarer instances the mental faculties have been retained with a high de- gree °f hydrocephalus. General spasms and epileptiform convulsions are common. The head cannot be held erect, partly on account of it> weight, partly on account of the weakness of the muscles. The voluntary movements are never learned. SO that the child carries his limbs about in an awk- ward manner. The legs are usually somewhat spastic, causing ;i de- cided difncultv in the rait. Many children never learn to walk. < Mhers 944 THE NON-NEURONIC DISEASES wabble and shift about in the most ungainly way. Incontinence of urine and feces is a mental rather than a motor trouble. Choked disc FIGURE 203. Hydrocephalus. Fitch's case. and optic atrophy are very frequent accompaniments of the disease, caused, of course, by the pressure upon the optic nerves. Sensation is not as often affected as motility and seldom are the cranial nerves THE NON-NEURONIC DISEASES 945 except the optic, implicated. Profound stupor, fever, vomiting and coma may herald the end. FIGURE 204. I [ydrocephalus. The outlook for these children is bad. They usually die- within the first four or five years of life. This is a blessing iu disguise if the case is a severe one at all, for the prospect <>t idiocy, n life contin 946 Till-: .\ON-NEURONIC DISEASES is strong. In some cases evacuation of the fluid has occurred through a spontaneous rupture, with amelioration of the symptoms. In the FIGURE 205. Hydrocephalus. mildest cases life has been prolonged to old age, the disease having doubtless become permanently arrested. The prognosis of hydro- cephalus is therefore always serious and full of uncertainty. THE NON-NEURONIC DISEASES 947 Treatment. — The treatment of hydrocephalus of all forms is far from satisfactory. In the acute and chronic acquired forms it is more hopeful than in the congenital. It is always uncertain, however, how much of the recovery was due to the therapeutic measures insti- tuted and how much to the natural events of the disease. In the ac- quired types of the trouble, treatment should always be directed, so far as possible, to the underlying primary disease. Quincke advises mercurial treatment in all of these cases, especially the internal men- ingitic cases, whether a history of syphilis be obtainable or not. Medi- cines cannot remove the fluid. Purgatives and diuretics, so much vaunted in earlier days, accomplish practically nothing, and are often dangerous if used with necessary vigor in the weakened state of these patients. Surgical measures, therefore, as a rule, are our only resort and these are not very promising. They consist of stead}- compression of the head, partial withdrawal of the ventricular and spinal fluid by punc- ture, violent revulsive measures and ventricular drainage. It does not seem very rational to transfer the pressure of the ac- cumulating fluid from the skull around it to the brain within it by ex- ternal compression of the head. And yet that is a practice that is honored both by age and by a few apparent cures. Trousseau, and others to-day. recommend the application of strips of adhesive pias- ter up over the vertex from one mastoid process to the other, from the nose to the occipit, and then in circles around the head horizontally. The case must be closely watched and the strips removed upon the first indication of undue pressure upon the brain. It was first hoped that this would favor the reabsorption of the fluid. Then a check to the enlargement of the head was all that was anticipated. Neither of these results, however, are accomplished, whereas an increase in the symptoms of compression almost always lakes place. West recom- mends the use of a broad elastic band instead of the diachylon plaster. He, with most other clinicians, however, recognizes the general futil- ity of this method of treatment. Withdrawal of the fluid by puncture is the most direct, but also the most dangerous, mode of attempting relief. Hippocrates re-com- mended puncture of the ventricles. Simple puncture under the strict- est kind of antisepsis, withdrawal of not more than an ounce of fluid at a time, and the operation not repeated at too close or frequent in- tervals, have in a few instances seemed to have resulted in a cure- In sixty-three cases. Eienschen reported fifteen recoveries, twelve im- provements and twenty-four deaths. ( )ften the first operation is borne well but the second or third proves fatal. Puncture, with after- drainage, and puncture with iodine injections, seem to have been particularly dangerous. The insertion of the aspirating needle or tro- car is usually best made at the outer angle of the anterior fontanelle. Much safer, though slower in action, is puncture of the sub- arachnoid sac of the spine between the third and fourth lumbar ver- tebrae. This measure has proved to be both curative and palliative, especially in acquired hydrocephalus. Revulsive measures consist of the use of the set<>n. inunctions 948 THE NO N- NEURONIC DISEASES with ointments of tartar emetic and even the once so popular appli- cation of spirit of turpentine freely to the scalp and then setting it on fire. These heroic measures unfortunately do not cure and only too often fail to relieve. It must be admitted, to the chagrin of both medicine and surgery, that the treatment of hydrocephalus is not a brilliant success and only too often nature and a laissez-faire policy accomplish about as much as do science and art. DISEASES OF THE PONS VAROLII AND MEDULLA OBLONGATA. It is not necessary to give a minute description of the pons and medulla here, for the main points in their anatomy have been touched FIGURE 206. pc ft !'* ale Diagrammatic representation of a section of the medulla at the level of the decussation of the pyramidal tracts: fp., posterior fissure; f, anterior fissure; p, crossing of the pyramidal tracts; fg, fasciculus gracilis; a, anterior horn; pc, posterior horn; R, substantia gelatinosa of Rolando; 1, lateral column; ac, anterior column; ap, pyramid. (From Quain.) upon in the earlier accounts of the anatomy of the brain and of the neuronic architecture of the nervous system. The medulla is but an upward extension of the spinal cord. Its tracts and gray matter are the same, with but a slight alteration in their relative positions. Usually that part is called the medulla which extends from the edge of the foramen magnum up to the lower border of the pons. As the tracts of the medulla continue to course on up- wards to be prolonged into the crura cerebri, they are embraced ven- trally and crossed transversely by the great commissural fibres of the cerebellum. These transverse fibres and all that lies behind them is known in gross anatomy as the pons varolii. It is better to regard the upward extension of the medulla, lying behind the transverse tracts of the pons, as the preoblongata. What is commonly called in anatomy the medulla may then well be called the postoblongata. Behind the preoblongata is the fourth ventricle, with the aqueduct of Sylvius above leading to the third ventricle. THE XOX-XEUROXIC DISEASES 949 There are three important facts to be noted in connection with the rearrangement of the spinal cord tracts in their upward extension into the medulla. The first is that there is a sort of an antero-posterior flattening out of the cord, with a gradual posterior displacement of the anterior parts thereof. The posterior columns separate, V-shape, and continue on up as the restiform bodies and inferior cerebellar peduncles. The central canal of the cord opens out into the fourth ventricle, which is bounded above laterally by the superior cerebellar peduncles. The central gray matter and anterior horns of the cord sink backwards until, with the widely separated remains of the posterior horns, they form a plate of gray matter just beneath the floor of the fourth ventricle. At FIGURE 207. TcM. — Section op the medulla oblongata in the region of the superior pyramidal decussation. (Schwalbe.) •£ a.m./., anterior median fissure ; f.a., superficial arciform fibres emerg- ing from the fissure ; py. , pyramid ; n.ar. y nucleus of the arciform fibres ; f.a'., deep arciform fibres becoming superficial ; 0., lower end of olivary nucleus ; o\, accessory olivary nucleus ; n.l., nucleus lateralis ; f.r., formatio reticularis ;/.a. 2 , arciform fibres pro- ceeding from formatio reticularis ; g., substantia gelatinosa of Rolando ; a. V., ascending root of fifth nerve ; n.c, nucleus cuneatus ; n.c'., external cuneate nucleus ; f.c, funiculus cu- neatus ; n.g., nucleus gracilis ; f.g., funiculus gracilis; p.m./., posterior median fissure; c.c, central canal surrounded by grey matter, in which are, n.XT. f nucleus of the spinal accessory, and, n.XIL, nucleus of the hypoglossal : s.d. f superior pyramidal decussation. the top of the posterior columns an expansion of gray matter becomes the nuclei of the posterior columns. The second great change in the medulla to be noted in relation to the cord tracts is that the pyramidal tracts course ventrally and decus- sate in the well-known decussation of the pyramids. The third change is that the sensory tracts in large part decuss in the fillet. With the aid of the accompanying diagrams and a knowledge of the gross anatomy, the reader can easil) trace every pathway and gray deposit in the spinal cord up into the medullar. The apparent con fusion is caused merely by their rearrangement and not by an) essen tial change. The cranial nuclei arc the special points of interest in the medulla in clinical neurology. These nuclei arc tin- cause of the special symp- tomatology of medullary diseases. Th< y arc all in the gra) matter making up the floor of the fourth ventricle and the aqueduct of Sylvius. 950 THE N0N-NEUR0NIC DISEASES They are the remains of the anterior and posterior horns and central gray matter of the spinal cord. The Figure 175 illustrates more accurately than any long description can the relative positions of these nuclei. Being but the upward extension of the gray matter of the cord, they are liable to the same sort of disease processes that the latter is. They may undergo primary and secondary degeneration and be subject to the distinctive effects of hemorrhage, inflammation, compression, etc. In this respect medullary diseases differ in nowise from spinal cord affections. The symptoms of disease of the medulla and pons are both general 7i.am. FIGURE 208. Section op the me- dulla OBLONGATA AT ABOUT THE MIDDLE OP THE OLIVARY BODY. (Schwalbe.) ± f.l.a., anterior median fissure ; n.ar., nucleus arciformis ; p., pyramid; XII., bundle of hypo- glossal nerve emerging from the surface ; at b, it is seen coursing between the pyramid and the olivary nucleus, o. ; f.a.e., exter- nal arciform fibres ; n.l., nucleus lateralis; a., arciform fibres passing towards restiform body partly through the substantia gel- atinosa, hemorrhage, softening, inflammation, abscess, tumor, atrophy and sclerosis. Occasionally it is congenitally under size and is the site of an agenesis. There is a cerebellar type of infantile palsy and in some cases of congenital atrophy the symptoms have a close resemblance to- those of Friedreich's disease. MALFORMATIONS OF THE BRA1X. These are of no clinical interest whatever, since they are monsters that never continue to live long after birth. There are many varieties, all of which are interesting from the embryological and developmental standpoint. Anencephaly accompanies acrania and is a condition in which, there is no cranial cavity or contents. The cerebellum and the primi- tive basal ganglia may be present in some cases. THE NON-NEURONIC DISEASES 957 Micrencephaly and microcephaly means a monstrously small head and brain. Porencephaly is a partially damaged brain. I have described it under the head of congenital, infantile hemiplegia. Cyclopia is where the anterior cerebral vesicle fails to separate or divide and the orbits remain together with a single optic apparatus. Meningocele, encephalocele and hydrencephalocele are hernias, the nature of which is sufficientlv indicated in the names. SECTION D. GEN ERALMALADIES WITH LEADING NEURO- LOGICAL SYMPTOMS. ALCOHOLISM. Alcohol seems to exert a particularly deleterious influence upon the nervous system. Its effects are somewhat different when taken in large quantity occasionally from what they are when it is imbibed in small quantities for long periods of time. It may be studied therefore under the heads of acute and chronic alcoholism. In the course of chronic alcoholism there are occasional outbreaks of an acute condi- t.on, known as delirium tremens. Acute Alcoholism. — The ingestion of a large amount of this poison results in disturbances of coordination, psychosis and narcosis. The condition is known preeminently as drunkenness, intoxication, and is of physiological and toxicological interest rather than neurological. There is a flushed or cyanotic condition of the face, the pulse is full, the breathing labored. The pupils are dilated. The temperature is generally below normal. I nconsciousness is present, but it is not so dee]) but what the patient can generally be aroused momentarily with a great effort. Muscular twitchings, occasional convulsions and great stupor are not infrequently seen. This is the form of alcoholism usually observed in the condition known as dipsomania, wherein there is a strong hereditary tendency toward drink and a yielding to it by the indulging in a periodical spree. Acute alcoholism is chiefly of interest to neurology in the role it plays in the differential diagnosis of cerebral hemorrhage, epilepsy, hysteria, syncope, uraemic coma and other affections accompanied by unconsciousness. Chronic Alcoholism. — The constant drinking of alcohol and alco- holic beverages in amounts that never arc- sufficient to cause actual drunkenness produces changes of a degenerative character in nearly all of the tissues of the body. In a work of this sort we are only con- cerned with the changes that are established in the nervous tissues and functions. I will pass by, therefore, with the merest mention, the well- known alterations in the digestive apparatus, the heart and arteries, the kidneys and the skin. The earliest manifestations of chronic alcoholic intoxication are 960 GENERAL INTOXICATIONS functional in character. There is a muscular tremor which affects principally the lips, the tongue and the hands. It is also in the legs, but it is not so observable. It is an intentional tremor in part, being empha- sized when any voluntary movement is attempted. It causes unsteadi- ness and is very obvious in the speech and the writing. It is a fine yet irregular oscillatory tremor, and is most noticeable in the morning. A dose of alcohol diminishes or puts a stop entirely to it for the time being. Insomnia is a troublesome symptom in these habitues and during their sleep the muscles often jerk and twitch. The mental state is characteristic of a mild degree of degenerative encephalitis or meningoencephalitis. In the morning until the ac- customed stimulant is obtained there is dullness and apathy and some- times confusing headache. The temperament gradually undergoes a change. The individual is irritable, restless, forgetful, morally low- ered. He may be emotionally depressed and maudlin. He is given to silly sentimentalism and is often suspicious. His judgment and reason- ing faculties are impaired. His volition lacks force. Later on actual dementia may supervene. If alcohol is not a cause of dementia paralytica, the clinical picture it induces is very similar to it. Mental breakdown, epilepsy and other serious results are certainly to be at- tributed to it in a certain percentage of cases. Delirium Tremens. — This is a condition bordering on but not classified as an insanity. It does not follow an excessive ingestion of alcohol in *a person usually temperate ; it is liable to occur in a habitual drinker who may take an overdose of the poison. It is therefore an incident merely in the course of chronic alcoholism. It may even occur as the result of the sudden withdrawal of the poison. The symptoms appear gradually but rapidly, attaining their maxi- mum in two or three days. They may be provoked by a shock in a heavy drinker, a sudden fright, an accident with a profound mental disturbance following it. As the attack approaches the individual sleeps badly, has horrible dreams, is restless, depressed in spirits and devoid of appetite. Flashes of light appear before his eyes and noises of a disagreeable character ring in his ears. To overcome these he takes more liquor, goes to bed, and at night experiences anew the vivid, distressing and terrifying dreams, the visual and auditory hallu- cinations, the restlessness and harassing motility. As the attack draws on to its climax absolute insomnia occurs, the visual hallucinations assume definite shapes, suspicions and uneasiness are prominent and a veritable delirium is in evidence. Loquacity is enormous. The patient talks incoherently without cessation. He is in constant motion ; tries to get up and go out ; rushes toward the open window or attempts to push past his guards to the door. He is subject to all sorts of distressing and suspicion-pro- voking hallucinations. At times he trembles and cries out in evident alarm and fright. He declares his enemies are in the room ; that they are trying to injure him. He points them out, though his finger indi- cates only empty space. He thinks he is surrounded and overrun with rats and mice and snakes and other crawling vermin. This of GENERAL INTOXICATIONS 96 1 course increases his terror. He is now the victim of the genuine "horrors." There is great muscular tremor and to all intents the poor fellow is in the state of a thoroughly terrified animal. The countenance is flushed and the conjunctiva congested. The pulse is soft, frequent and easily compressed. Perspiration is free and the temperature, except in mild cases, is slightly raised. The average temperature is about 102 or 103 degrees F. When it climbs to 108 or 109 degrees it is a most ominous sign. The tongue is coated heavily and there is absolute want of appetite. The insomnia continues and becomes a constant feature. In three or four days, if the case is favorable, the symptoms all aoate ; the rest- lessness subsides ; the hallucinations slowly vanish ; and the appetite comes back. In severe cases the delirium continues, and being less active is gradually replaced by signs of profound prostration. Stupor super- venes, the respirations grow shallow and the patient dies of gradual heart-failure. The symptoms caused by the polyneuritis which often constitutes a part of the clinical picture of chronic alcoholism will be found de- tailed in the section devoted to peripheral nerve lesions. Diagnosis. — The diagnosis of chronic alcoholism and of delirium tremens is not difficult. The entire condition of the patient must be taken into account. The cutaneous, gastric, circulatory, renal, nervous and mental appearances must all be given due consideration. If this is done it is almost impossible to make a mistake ,for scarcely any other disease, in fact none, presents so broad a picture. Acute mania and symptomatic delirium certainly do not, for they are wanting in the characteristic visual hallucinations and tremor. The tremor may be mistaken for the tremor of^dementia paralytica, but the latter is accom- panied by a somewhat different psychic state, more of an exaltation ana with more distinct delusions. The lungs should always be carefully examined, for in some intances an overlooked pneumonia, especially in a drunkard, may be the cause of the delirium starting. The same is to be said in regard to erysipelas and trauma. Pathology and Pathogenesis. — The changes observed in the nervous tissues in chronic alcoholism are slight and never in any sense characteristic. The dura may be hemorrhagic and inflamed. The pia-arachnoid is often opaque and thickened and chronically •changed. Actual signs of meningitis arc rare in either membrane. The underlying convolutions of the brain, in old cases, are wasted and depressed. Adhesions with signs of genuine meningo-encephalitis may be seen in some protracted eases. Berkeley, Buzzard, Hoch, Cole and others have found chromatolvtic and other degenerative signs in the neurones. Especially so in alcoholic neuritis described elsewhere. Though these findings, as well as others Outside of the nervous system, such as the congestion of the bases of the lungs, of the skin, of the face and of the kidneys, are highly suggestive in chronic alcohol- ism, they do not explain the delirium tremens. Tin- latter is patholog- ically as much of a mystery as ever. It is undoubtedly due to an acute 962 GENERAL INTOXICATIONS process of some sort in the cerebral cortex, which disturbs the function of the latter, runs a definite course and then tends to subside. We cannot even form a guess of the nature of that process. It is more than a mere intoxication, such as is evidenced in the findings of simple chronic alcoholic poisoning. Prognosis. — In private practice the prognosis, for obvious rea- sons, is better than it is in hospital practice. Many cases recover, but relapses are common if the use of the poison is resumed. It is well for the patient to get away from his home and usual haunts ; for this reason institutional treatment is more favorable than home treatment. Complications add to the gravity of the prognosis in all cases. The condition of the pulse is a fair indication of the outlook. If some improvement is detected after sleep, a favorable temination may be expected. If the patient wakes up and is as bad as ever, a fatal issue is likely to occur soon. Very high temperature, 103 or 105 degrees, is an unfortunate sign. Sudden rise in temperature, with old age, are bad prognostics. Associated pneumonia and nephritis cloud the prog- nosis ; so also do traumata, wounds and operative procedures. Treatment. — The treatment of chronic alcoholism practically re- solves itself into the removal of the poison. This had better be done absolutely. Only in very exceptional cases will it ever be found nec- essary, on account of the pulse or tendency to delirium tremens, to allow a little alcohol or to attempt to reduce it gradually. The craving for the liquor should be suppressed as much as possible. Capsicum is effective sometimes. General tonics like strychnine, quinine, iron and the bitters generally are always in order. Abundant nourishing diet also must be prescribed, and all other measures that tend towards reconstruction and the health of the organism. All of this can be carried out best in an institution. The special manifestations call for no particular consideration here, for their management will be found described under the various dis- eases and disorders throughout the book. The delirium tremens needs careful management. The patient must be put under restraint and vigilantly watched night and day. His strength and violence is such that two male nurses will usually be required to handle him. No open window should be allowed and all dangerous articles and medicines by which he might injure himself should be removed. It is rarely necessary to resort to a "strait- jacket." Other means can sometimes be devised by which he can be strapped and confined to his bed. Broad straps may be attached to his wrists and ankles and then fastened to the sides of the bed. A sheet, long and strong, may be wrapped around him and the bed at the same time. If the hallucinations are increased by darkness or a dim light, the room had better be kept well illuminated. The alcohol should be withdrawn at once, except in those few cases in which there is a dangerous feebleness of the pulse. Sometimes even in these cases ammonia, ether, cofTee or other stimulant may be found available. Digitalis, at one time so much used, is now not considered snfe, as it accomplishes little in the mild cases and has often proved disastrous in the severe ones. GEXERAL INTOXICATIONS 9O3 Nourishment is of supreme importance. It should be fostered as rapidly as possible, and in as thorough a manner as the condition of the organs will permit. Hence it is well to use a liquid diet for a time and give it every two or three hours. Beef tea, milk, lightly beaten eggs are available. The bowels should be gently and promptly moved, but severe purgation must be avoided. Ware has shown that a simple expectant treatment gives as a rule better results than that with medicines. However, though de- lirium tremens usually runs its course without being affected by medi- cines, a certain amount of medicinal treatment is in order. The object to be attained by this is the sedation of the nervous system, the produc- tion of sleep and the supporting of the strength. Bromides are usually disappointing. They may produce a certain amount of quietude, but they rarely cause sleep. Full doses are neces- sary. Paraldehyde was and to some extent is still popular. Bromides with tincture of capsicum are sometimes very effective. Chloral, unless markedly contra-indicated by the condition of the heart, is the most general available drug in this condition. Sixty grains can be given by the mouth and in many cases a refreshing sleep thus be secured. The hypodermic use of hyoscine, gr. i-ioo, or of morphine, gr. }4> cautiously repeated, is second in value only to chloral. Opium itself must be used with great circumspection. Moreover, it tends to lock up the secretions. Pilocarpine has been recommended, but it is dan- gerous to the heart. Cannabis indica has effected a desirable sedation in a few cases. Cold douching, cold bathing and a cold pack may be tried if the heart can bear the shock. A word of caution is necessary An the drug treatment of delirium tremens. Ofttimes in spite of the most heroic administration ^of hypnotics and sedatives the patient con- tinues wide awake. It is risky to go on plying the remedies under such circumstances, for as they accumulate in the system they may suddenly exhibit a poisonous effect and the patient die in complete stupor. THE OPIUM AND KINDRED HABITS. There are certain drugs which are taken medicinally at first, rarely as a luxury, and producing certain pleasurable or unpleasurable effects upon the nervous system,, are continued until the disastrous habit is formed. After alcohol, opium and its alkaloid morphia are most abused in this way. Then follows the use of chloral, cocaine, ether, chloro- form, paraldehyde, cannabis indica. Acute poisoning by these various drugs is a subject of special interest to physiology and toxicology more than to neurology. Chronic poisoning is pretty nearly the same, differing more in degree than in kind, in all of them. A description of chronic opium poisoning will cover more or lrss the clinical picture produced by all of the others. As in the- discussion of chronic alcohol- ism, I will briefly consider only the neurological manifestations. ppium Habit. — M or phino mania. Morphinism, In certain coun- tries where opium is used regularly there is established a partial im- 964 GENERAL INTOXICATIONS munity against its deleterious effects. The effects are present, but they are not so obvious as they are with us. In this country the habit is generally started by the use of morphia to relieve pain. A headache or an old sciatica, the pains of chronic rheumatism or of tabes are re- lieved for the nonce by the drug, even upon the prescription of the medical attendant. When the pains return the patient acquires the drug for himself, learns how to use it hypodermically and so starts the habit. The use of chloral for insomnia, and in fact all of the above-named drugs, is started in this way. Women and physicians themselves are the leading victims of these habits. Gradually the dose has to be increased to obtain the desired relief, not from the pains so much now as from the depressing after-effects of the drug itself, so that ere long the patient has lapsed completely into the ranks of the habitues. The origin of the habit should enforce the lesson upon the profession to be chary in the use of these drugs, especially in chronic, painful states. I have seen several lives ruined by the injudicious use of morphine by a physician at the beginning of a simple ailment. When the ailment returned the patient repeated the prescription upon his own responsibility until he found himself in the grip of the habit. Symptoms. — There has never been written a more vivid account of the symptomatology of chronic opium poisoning than De Quincey's "Confessions of an Opium Eater." Every physician should be familiar with this masterly essay. It both teaches and warns in terms that cannot be mistaken. The symptoms at first are not at all significant and sometimes whole months may go by without the health showing much deteriora- tion. Sooner or later, however, except in the most rare instances, the physical and mental signs of breakdown declare themselves. These are subjectively noticed most as the effect of the drug wears off. There is a feeling of weariness, lassitude, apathy, depression and distress. Sometimes there is nausea and gastric discomfort. The mind is so depressed and the patient feels generally so bad, that he resorts to the drug again to get relief. As this dose wears off he experiences the same depression in a little more marked degree. And thus the evil gTows. Physically he becomes sallow in appearance, emaciated, gray and aged. His appetite is deranged or gone entirely. His pupils are dilated or unequal, except when under the direct influence of the drug. A general condition of asthenia and extreme physical debility ultimately obtains. Showing the degenerative effects upon the nervous system, these patients are immoral, irresponsible, unreliable, liars and cheats. Los- ing their higher psychic inhibitions, they fall a step toward the mon- key ; hence they are silly, cunning, without a manly sense of obliga- tion, selfish, maudlin and sentimental. Their mental stamina is gone and they lack initiative force and self-control. They are restless, irritable and emotional. They are sometimes the victims of most vivid and terrifying visual and auditory hallucinations. I once saw a pa- tient reach out to put her hand into the pocket of her brother, who she thought was standing beside the bed, but who had been dead many years. Itching and other paresthesias are complained of. There are GENERAL INTOXICATIONS 965 occasional chills, followed by profuse sweats. Sometimes marked hysterical and neurasthenic symptoms dominate the clinical picture. Some of the profoundest neurasthenia I have ever witnessed was in morphine users. Unless the case is not too far gone, so that under heroic treatment recovery can be obtained, these patients usually go on increasing the dose of the drug until they become complete physical wrecks and mental dements, and die of inanition and heart failure. There is nothing to be said of the pathology of the condition. It is a progressive degeneration of wide extent, toxic in origin. The treatment is practically impossible outside of an institution. The patient must be isolated, systematically fed and deprived of all possible means of securing the drug. In mild cases the drug may be withdrawn abruptly and completely ; in severe cases only a gradual withdrawal had better be attempted. For the first week or so the patient had better be kept in bed and the drug gradually diminished. In the meantime, with the aid of baths, moral suasion, massage, etc., the restlessness, insomnia, nausea, vomiting and even the abdominal pains should be alleviated as much as possible. The bowels should be moved gently and regularly. The diet should be plain, nourishing and frequently given. It should consist of meat broths, milk, whipped eggs, matzoon, koumys and other light, semi-fluid articles. If the pulse calls for it, stimulation with aromatic spirits of am- monia may be given. Sleep should be fostered by warm baths, warm drinks and other measures ; but sometimes sulphonal, hyoscine or even morphia itself may have to be temporarily resorted to. Rapid, forced nourishment, with gradual withdrawal of the drug, is the sum total of the treatment in these cases. In this respect it follows along the same lines as that for chronic alcoholism. In spite of the occasional complete restoration to health, these patients too often lapse back into their old habit ; which makes the treatment of them a discouraging and thankless task. TETANUS. This is sometimes called lofkjaw. It is a most virulent form of infection in which there are severe tonic spasms in the muscles of the body. Etiology. — The cause of this disease is the tetanus bacillus dis- covered by Nicolaier and studied by Rosenbach, Kitasato and others. The germ enters the body through an open wound in the skin or mucous membrane. It is found in the earth. It has been isolated and cultivated. In appearance it is a slender rod which may grow into long threads, with one end swollen and occupied by a spore. It is an- aerobic, motile and develops at ordinary temperatures. The bacillus itself does not enter the blood or organs, but at the site of the wound it produces a tox-albumin, which is the most virulent form of poison that is known to enter the body. Pathology and Pathogenesis. — There are no distinctive patho- logical findings. The congestions observed in different parts of the body, the perivascular exudations and beginning chromatolytic changes 966 GENERAL INTOXICATIONS in the neurones may be the result rather than the cause of the symp- toms. There are local manifestations, as, for instance, in and about the wound and the inflammation of the umbilicus in tetanus neonatorum, and constitutional symptoms. Symptoms. — We speak of a puerperal tetanus, a tetanus neona- torum and of endemic and epidemic attacks of the disease. Essentially they are all the same. The symptoms do not develop immediately after the invasion of the bacillus, but gradually. It has been suggested that this is because the toxin undergoes a still further change in the system. In about ten days the earliest signs begin to appear. The first are a little stiffness in the neck, slight rigidity in the jaw and difficulty in mastica- tion. General toxic symptoms, such as chills, fever and malaise, may accompany these early troubles. Gradually trismus occurs, or tonic contraction of the muscles of the lower jaw. This cardinal symptom has given the name lockjaw to the malady. As the eyebrows are ele- vated and the corners of the mouth are stretched laterally, the counte- nance assumes a horrible sort of a grimace, risus sardonicus. In children the symptoms remain thus in the face often, but in adults the musculature of the body is generally affected also. The muscles of the back are most frequently involved after the jaw muscles. This causes a bowing of the neck and back, so that the patient rests on his heels and occipit. So violent is this opisthotonos at times that the abdominal muscles have been torn directly across. If the entire body and limbs are rigid, the condition is known as orthotonus. A lateral bending of the spine causes pleurothotonos. In rare instances the ab- dominal muscles are so violently spasmodic that the body is curved forward into a condition known as emprosthotonos. Sometimes the chest is compressed, the glottis thrown into spasm, the respirations diminished and the patient almost completely asphyxiated. There is excruciating pain with all this violent spasm and the patient, silent and immovable as if clamped in a vise, is bathed in a profuse perspiration and reveals untold agony. The paroxysms are variable in duration and are easily excited. The intervals between the paroxysms are usually very brief. The temperature is usually normal, though just before death it may rise to no degrees F. Even higher temperatures have been seen. Heart-failure, asphyxia or exhaustion puts a grateful termination to the patient's sufferings, usually in a paroxysm. What is called head-tetanus is the unilateral stiffness of the jaw and facial muscles, from a wound on that side of the head, usually near the fifth nerve. Diagnosis. — The characteristic clinical picture and the history of a wound make the diagnosis of tetanus a matter of no great diffi- culty. The similarity between this form of toxaemia and strychnine poisoning is obvious. There is no history of a wound, however, in the latter, and trismus does not lead in the symptomatology. In tetany the tonic spasms begin in and are usually limited to the ends of the extremities, especially the fingers and hands. Doubtful cases of tetanus GENERAL INTOXICATIONS 967 can always be decided by a bacteriological examination of the secre- tions in the wound. Prognosis. — This is decidedly bad in the traumatic cases, at least 80 per cent, dying in three or four days. In the so-called idiopathic cases the mortality ranges about 50 per cent. Children offer the worst prognosis. If the symptoms are late in coming on, if they confine them- selves to the jaw and face, and if there is little or no fever, hope of recovery may be entertained. Treatment. — Strict attention to the wound is the first requisite. It should be thoroughly excised, antiseptically washed and according to Tizzoni treated with nitrate of silver to destroy the germ in situ. A dark and perfectly still room should be the domicile of the pa- tient. Extraordinary care should be exercised to avoid every possible sort of irritation. Noise, light, drafts of air, change of nurses should FIGURE 211. Tetanus. (After Sir Charles Bell.) be guarded against. Even such strenuous efforts will not forestall the paroxysms in most cases. Feeding is a problem on account of the locked condition of the jaws. A tube may be passed through the nostrils into the gullet or into the rectum and liquid diet administered in that way. The control of the spasms is best secured with full doses of mor- phia. Chloroform inhalations and such drugs as the bromides, chloral, cannabis indica, calabar bean, curare are available agents. Recovery has sometimes followed their employment. Blood-scrum therapy seems to hold out some hopes in this disease. By Tizzoni and Cantani the blood-serum of immunized animals has been used with some degree of success. Behring and Roux haw each prepared an antitoxine serum. That of Tizzoni seems to be the most successful. It is recommended that j.j^ grammes of the Tizzoni anti- toxine be employed for the first dose and 0.6 grammes for subsequent doses. From the observations of Marie and Morax, of Meyer and Ransom 968 GENERAL INTOXICATIONS and of Rogers, it would seem to be advisable to expose the nerves of the part in which the primary infection lies as near the cord as possible and to inject directly into the substance of each nerve the antitoxine. This prevents the absorption of more poison from the wound. An injection should then be made low down in the spinal cord or in several places. The injection may be made as in an ordinary lumbar puncture. HYDROPHOBIA. This is sometimes called lyssa and rabies. It is an acute disease of warm-blooded animals ; is dependent upon a specific virus ; and is characterized by marked nervous symptoms. Etiology. — The poison that causes rabies is found chiefly in the nervous system and in some secretions such as the saliva. We know nothing of its essential nature. Many animals are susceptible to it, namely, man, the dog, the wolf, the cat and by inoculation the ox, horse, pig and skunk. The period of incubation is shorter in children than it is in adults. Bites are more dangerous in exposed parts of the body, like the face and hands, than they are in covered parts like the trunk and legs. Large, punctured wounds afford the most ready entrance to the poison. According to Horsley, only 15 per cent, of those bitten by dogs be- come affected with the disease. Wolf bites give a higher percentage. The incubation period in man ranges from two weeks to three months. The average time is about six or, eight weeks. Pathology and Pathogenesis. — The blood vessels of the cere- brospinal nervous system are congested, particularly in the medulla. There is an exudation of leucocytes in the perivascular spaces and minute hemorrhagic extravasations. The virus is found abundantly in the brain, spinal cord and nerves, but not in the kidneys, spleen or liver. Congestions are observed in the stomach, larynx, trachea and bronchi. Symptoms. — These appear after a variable interval after the in- oculation. For the sake of practical convenience, they are divided into those of the premonitory, excitable and paralytic stages. The earliest signs of the malady are irritability, restlessness, mental depression, fear of impending trouble, pain and numbness near the bite, headache, loss of appetite and insomnia. Often there is a general hyperesthesia to all forms of sensation. Light and noise irritate the patient. The larynx being congested, the voice grows husky and deglutition becomes embarrassed. The pulse is somewhat rapid and the temperature is slightly elevated. Gradually the hyperesthesia increases, the restlessness grows worse, the dejection passes into alarm and general spasms begin to develop. This is the beginning of the stage of excitement. As this advances, every sort of external stimulation, a noise, a bright light, a mere verbal suggestion, a draught of air may provoke a most violent spasm. The spasms are strikingly in the areas supplied by nerves from the medulla, namely, the muscles of the larynx and mouth. They are reflex spasms and are exceedingly painful. An attempt to drink GENERAL INTOXICATIONS 969 water sets up the spasm so violently that the patient dreads the very thought of drinking, hence the name of the malady, hydrophobia. The mind is usually affected along with this spasmodic condition. The patient is maniacal, though between the spasms he may be calm and rational. He is usually harmless and even takes care not to hurt any one unless his mania is furious. The temperature is generally ele- vated in this stage. After a few days the paralytic stage comes on, terminating in coma and death in a few hours. Heart failure and syncope close the distressing scene. Pseudo-hydrophobia or lyssophobia is in all probability a hysteroid trouble, developed upon a background of fear and dread. An indi- vidual who has been bitten may undergo and simulate all the phenome- na of true rabies in a mild degree. The condition lasts longer than in the genuine disease and the patient always recovers. Diagnosis. — This is easy in view of the history of a bite and the characteristic symptomatology. In cases of doubt, or better still, in all cases, so as even to forestall any possible doubt, the animal that has bitten the patient should be made to furnish inoculations from the region of the medulla. If these be tested upon other animals, especially the rabbit, the paralytic form of the disease will appear in from fifteen to twenty days. This will tell us what to expect in the patient and confirm the diagnosis. Treatment. — This is not the place to discuss the general prophy- laxis of hydrophobia and the laws that should be passed in regard to the muzzling of dogs. The preventive inoculations of Pasteur do cer- tainly seem to be effective in man, if we are to judge from the sta- tistics sent out by the various Pasteur Institutes throughout the world, and especially by the one in Paris. The management of a case of hydrophobia is a most unsatisfactory task. There is no specific remedy and so the treatment must perforce be entirely symptomatic. It must also be heroic. The wound, of course, should be antiseptically washed, cauterized and kept open for several weeks. When the disease has developed it is absolutely incurable. Chloro- form and morphia are about the only agents at our command to assist in alleviating the patient's sufferings. Chloral, bromides, hyoscine are practically useless. Cocaine may be painted on the throat, enabling the patient to swallow somewhat. Rectal nutrient enemata as a rule, however, will have to be resorted to. Arthritis Deformans. — I nder this name are probably masking a number of conditions, so that we arc only justified in regarding the term as merely representing a symptom-complex. The view is gaining ground that an infection or toxemia underlies the manifestations. The microbic origin of the disease is not satisfactorily established yet, though Schuller, Bannatyne and Blaxall and others have found micro- organisms in the fluids of the joints. The affection IS often associated with previous infections, as shown by the Statistics of Stewart and others. This local infection theory i> accepted by Baumler, but not by Osier and others. 97o GENERAL INTOXICATIONS An attempt is being made, and I think somewhat successfully, to bring the old neurotic theory of the origin of arthritis deformans in line with the infection or toxemic theory. There can be no doubt FIGURE 212. Arthritis deformans. (From a photograph in the Pathological Museum of the Med. Depart, of the University of Illinois.) of the tropho-neurotic nature of the malady in some of its phases at least. We are familiar with the acute and chronic forms of arthritis that often accompany gross disease of the cord. The arthropathies of GENERAL INTOXICATIONS 9/ E tabes are among the latter's cardinal symptoms. In dementia para- lytica, syringomyelia, hemiplegic conditions and diseases of the peripheral nerves, atrophy and other changes not infrequently occur in the bones and in and about the joints. Hence the dependence of trophic joint troubles upon disease of the central nervous system can- not be gainsaid. There are several features in rheumatoid arthritis that suggest most forcibly a central nervous explanation of the trouble. The changes in the joints are all but identical with those that occur in the spinal arthropathies just mentioned. Similar changes of a trophic nature occur in various tissues in arthritis deformans, just as they do in many spinal diseases. The skin becomes glossy, the nails get brittle, the bones and muscles undergo dystrophic alterations. The muscles sometimes markedly atrophy. The distribution of the joint and other changes is symmetrical, thus suggesting a central cause for them. And finally neuritis is often found clinically and pathologically in the disease. Osier notes that in a case of chronic arthritis from Dejerine's service, Triboulet and Thomas found upon post mortem a sclerotic condition of the posterior columns of the cord. Puerperal infection seems to have been the primary cause of the trouble. Llewelyn Jones has recently called attention to the relationship of rheumatoid arthritis to some other neurotic affections, particularly tetany, Raynaud's disease and exophthalmic goitre. In all of these troubles there is more or less similarity in the vasomotor phenomena, the muscular cramps and the periarthritic swellings. As Jones sug- gests, these diseases may all pass insensibly into each other as members of one family group, owning one common parent origin, a cerebro- spinal toxcemia. Macalister has adopted views similar to those of Jones. &• Upon these and other grounds, I am inclined to regard arthritis deformans as a cerebro-spinal tropho-neurosis, dependent upon some obscure form of toxaemia. I have not classified the malady among the tropho-neuroses in the earlier part of the volume, because there are needed more data, especially from examination of the central nervous system, to positively affirm that the disease is solely of neurotic origin. Other Toxemic Troubles. — There are many other neurotic mani- festations and symptom-complexes that are not sufficiently sui generis to warrant giving them the dignity of a special nosological position among nervous diseases. Such, for instance are the nervous types of influenza, with their severe headache, profound prostration, pains in the back and joints, depression of spirits, melancholia and even de- mentia; pertussis, with its spasmodic Spells; and many cutaneous ex- hibitions, such as urticaria, herpes, pruritis and other paresthesia. Most of these symptom-groups can be referred to neuritis, poliomyelitic processes, disease of the posterior rool ganglia and other disorders nf the lower or peripheral neurones. Finis. GENERAL INDEX. Abdomen, scaphoid, 803 Abiotrophy, 352 Aboulia, 106 Abscess, cerebellar, 871 pontile, 871 bulbar, 871 of the spinal cord, 555 cerebral, 867 Abnormal emotional states, 759 intellectual states, 756 Accommodation reflex, 63 Acoustic striae, 704 Acquired diseases, 100 Acromegaly, 339 Acute delirium, 874 ascending paralysis, 431 Acroparesthesia, 316 Actinomycosis, 910 Acrania, 956 Adiposis dolorosa, 345 ^sthesiometer, 49 Afferent diseases, 367 path, 98 Ageusia, 658 Agenesis, general, 422 Agitans, paralysis, 299 Agraphia, 767, 769 Age in etiology, 30 Agoraphobia, 163 Ainhum, 350 Akinesia algera, in Akinesis, 51 Akatama, 590, 603 Alopecia, 170 Allorhythmia, 169 Alcoholism, 959 Alcoholic coma, 761 polyneuritis, 592 Allocheiria, 48 Alternate hemiplegia, yyy, 924 Alternating anaesthesia, 954 Alexia, 762, 767, 770 Amblyopia, 787 Ambiguous layer. 683 Amyelinic neuroma, 609 Amimia, 771 Amnesia, 595, 762 Amnesic aphasia, 767, 769 Amyelia, 570 Amnestic aphasia, 769 Amyotrophic lateral sclerosis, 395, 401 Amaurotic family idiocy, 451 Anterior fundamental root zone, 476 radicular zone, 475 fossa, tumor of. 925 poliomyelitis, acute, 541 * commissure, 472 Antero-lateral tract, 476, 484 Anode, 65 Aphasia, 762, 767 sensory, 769 pressure. 771 visual, 770 amnestic, 769 ' tactile, 771 Aphonia, 766 hysterical, 124 Apoplexy, hemorrhagic, 828 ingravescent, 837 spinal, 529 Apoplectiform bulbar paralysis, 952 Apoplexia parva, 188 Apoplexie foudroyante, 830 Apoplectic coma. 762 Apparatus, nervous, 97 Area, auditory, 722 olfactory, 722 Analgesia, 48 Analgesic paralysis, (>23 Anaesthesia, 47 crossed, 779, 954 Anaesthetic leprosy, 568 Angular curvature. 501 Angioneurotic oedema, 318 Aneurism, intracranial, 934 Animal, a vertebrate. 459, Ankle clonus, 6l reflex, 6i Aneurisms, miliary. 826 Aneurismal erosion of vertebrae, s^x; Anaemia, spinal, 52; cerebral, Xjj hydrocephaK -id, « w -'4 974 GENERAL INDEX Anosmia, 779 Anencephaly, 956 Angioneuroses, 306 Anarthria, 766 Anidrosis, 74 Area, gustatory, 722 visual, 722 Areas, motor, 723 sensorial, 719 Argentic polyneuritis, 598 Arthropathy, 383 Arthritic palsy, 617 Arteriosclerosis, 828 Arterial system, cortical, 711 ganglionic, 713 choroidal, 714 Arteritis, degenerative, 829 syphilitic, 881 Arthritis deformans, 969 of spine, 510 Arteriae sulci, 465 Articular hydrops, 2> 22 ~ Articulate animal, an, 459 Argyria, 598 Arthralgia, 596 Arrhythmia, 169 Arachnoid, 462, 667 Arachnoidal granulations, 667 Arborization, 91 Arm palsy, lower, 616 upper, 616 Argyll-Robertson pupil, 63, 376 Architecture, neuronic, 95 Arbor vitae, 707 Arsenical polyneuritis, 597 Ascending current, 85 neuritis, 582 Asterion, 679 Astereognosis, 48 Astasia abasia, 107 Association centers, 747 tracts, 472, 735 Associated movement, 52, 776 Astrophobia, 163 Asphyxia, local, 310 Aspiration pneumonia, 660 Asymmetrical cord, 570 Atavism, 28 Ataxia, 377, 48, 54, 779 hereditary, 444 cerebellar, 444, 447 of thought, 449 locomotor, 367 Ataxic paraplegia, 439 aphasia, 767, 768 hemiplegia, 836 Atrophy, 74 deuteropathic, 393 optic, 784, 787 lipomatous muscular, 416 occupation muscular, 430 progressive muscular, 392 protopathic, 393 Atrophic spinal paralysis, 541 Attitude, 45 Atelomyelia, 570 Atheroma, arterial, 828 Athyrea, 334 . Athetosis, 53, 425, J77, 778 Aurium, tinnitus, 166 Aura, 198 Auditory symptoms, 792 area, 722 Aural vertigo, 153 Automatic reflex action, 476 Automatism, 142 Autohypnosis, 145 Axis — cylinder, 91 B Babinski reflex, 56 Baths, 79 Basedow's disease, 322 Baillarger, line of, 683 Basilar meningitis, 814 Bechterew, striae of, 683 Betz, giant cells of, 685 Bell's mania, 874 palsy, 653 Beriberi, 590, 603 Biot's respiration, 753 Bifida, spina, 569 Bladder crises, 382 Blepharospasm, 167 Blindness, word, 762, 770 psychic, 791 Blushing, 167 Blind spot, 781 Body, pineal, 739 pituitary, 739 Bodies, olivary, 705 Pacchionian, 667 Borborygmi, 168 Brain, diseases of, 662 anatomy, 662, 668 diseases, symptoms of, 662 weight of, 664 microcephalic, 664 megalocephalic, 664 membranes of, 665 conducting tracts of, 710, 735 circulation of, 710 GENERAL INDEX 975 symptoms of disease of, 749 localization symptoms, 750 general symptoms, 751 tumor of, 909 tumor of the base of, 925 vesicles, 668 membranes, diseases of, 793 circulatory diseases of, 821 the, 460, 663, 714 veins of, 714 malformations of, 956 sand, 696 structure, 680 Bregma, 679 Broca's center, 724 Brown-Sequard syndrome, 492, 530 Brachypncea, 382 Brachial plexus, 614 Bradycardia, 169 Bulbar paralysis, apoplectiform, 952 paralysis, hereditary, 953 paralysis, progressive, 395, 406, 951 abscess, 871 Burdach's column, 475, 484 Bursitis, 633 Capsule, internal and external, 689 internal, functions, 736 Cannabis indica habit, 963 Caisson disease, 570 Cauda equina, diseases of, 573 Case-book chart, 44 Caries of the vertebrae, 499 Catalepsy, 146 Calcaneus, pes, 548 Cajal, cells of, 685 Cardiac crises, 382 Cavities of spinal cord, 562 Cathode, 65 Canals, semicircular, 149 Carcinoma of the brain, 910 Caudate nucleus, 690 Cauda equina, 463 tumor of, 557, 559 Causalgia, 48 Cerebro-spinal meningitis, epidemic, 808 Cerebral anaemia, 822 hyperaemia, 825 hemorrhage, 828 embolism, 845 thrombosis, 845 palsy of childhood, 854 diplegia, 854 paraplegia, 854 abscess, 867 Cerebral — syphilis, 877 localization, 662, 714 cortex, 672, 681, 717 concussion coma, 761 epiphysis, 695 Cerebro-spinal fluid, 495 Cerebellar agenesis, 450 cortex, 707 abscess, 871 tract, direct, 475. 484 Cerebellum, 705, 740 tumor of, 922 diseases of, 954 Center, Broca's. 724 Centers, cortical, 717 spinal, 480 association, higher, 747 psychomotor, 729 Central convolutions, tumor of, 920 myelitis, 537 scotoma, 787 ganglia, tumor of, 921 Cells of Purkinje, 707 Cervico-brachial neuralgia, 251 Cervical enlargement, 463 opisthotonos of infants, 820 myelitis, 536 hypertrophic pachymeningitis, 514 Cephalic flexures, 670 Cephalaea, 234 Cephalalgia, 234 Chemosis, 811 Choked disc, 784 Choroidal arterial system, 714 Chloroform habit, 963 Chromophyllic substance, 93 Cheyne-Stokes respiration, 753 Chromatolysis, ^6 Chorea, 286 habit, 265 procursive, 299 magna, 132, 299 corpuscles, 289 festinans, 299 hysterical, 132, 299 Chronic chorea. 297 infantile meningitis, 820 meningitis, 820 Chiasm, optic. 694, 788 Cholesteatoma of the brain. 910 Chorda tympani, 701 Chloral hal.it. 963 ( livostek's sign, 260 Ci net nrc pain, 535 Ciliary body, 707 974 GENERAL INDEX Anosmia, 779 Anencephaly, 956 Angioneuroses, 306 Anarthria, 766 Anidrosis, 74 Area, gustatory, 722 visual, 722 Areas, motor, 723 sensorial, 719 Argentic polyneuritis, 598 Arthropathy, 383 Arthritic palsy, 617 Arteriosclerosis, 828 Arterial system, cortical, 711 ganglionic, 713 choroidal, 714 Arteritis, degenerative, 829 syphilitic, 881 Arthritis deformans, 969 of spine, 510 Arteriae sulci, 465 Articular hydrops, 322- Articulate animal, an, 459 Argyria, 598 Arthralgia, 596 Arrhythmia, 169 Arachnoid, 462, 667 Arachnoidal granulations, 667 Arborization, 91 Arm palsy, lower, 616 upper, 616 Argyll-Robertson pupil, 63, 376 Architecture, neuronic, g5 Arbor vitae, 707 Arsenical polyneuritis, 597 Ascending current, 85 neuritis, 582 Asterion, 679 Astereognosis, 48 Astasia abasia, 107 Association centers, 747 tracts, 472, 735 Associated movement, 52, 776 Astrophobia, 163 Asphyxia, local, 310 Aspiration pneumonia; 660 Asymmetrical cord, 570 Atavism, 28 Ataxia, 377, 48, 54, 779 hereditary, 444 cerebellar, 444, 447 of thought, 449 locomotor, 367 Ataxic paraplegia, 439 aphasia, 767, 768 hemiplegia, 836 Atrophy, 74 deuteropathic, 393 optic, 784, 787 lipomatous muscular, 416 occupation muscular, 430 progressive muscular, 392 protopathic, 393 Atrophic spinal paralysis, 541 Attitude, 45 Atelomyelia, 570 Atheroma, arterial, 828 Athyrea, 334 . Athetosis, 53, 425, 777, 778 Aurium, tinnitus, 166 Aura, 198 Auditory symptoms, 792 area, 722 Aural vertigo, 153 Automatic reflex action, 476 Automatism, 142 Autohypnosis, 145 Axis — cylinder, 91 B Babinski reflex, 56 Baths, 79 Basedow's disease, 322 Baillarger, line of, 683 Basilar meningitis, 814 Bechterew, striae of, 683 Betz, giant cells of, 685 Bell's mania, 874 palsy, 653 Beriberi, 590, 603 Biot's respiration, 753 Bifida, spina, 569 Bladder crises, 382 Blepharospasm, 167 Blindness, word, 762, 770 psychic, 791 Blushing, 167 Blind spot, 781 Body, pineal, 739 pituitary, 739 Bodies, olivary, 705 Pacchionian, 667 Borborygmi, 168 Brain, diseases of, 662 anatomy, 662, 668 diseases, symptoms of, 662 weight of, 664 microcephalic, 664 megalocephalic, 664 membranes of, 665 conducting tracts of, 710, 735 circulation of, 710 GENERAL INDEX 975 symptoms of disease of, 749 localization symptoms, 750 general symptoms, 751 tumor of, 909 tumor of the base of, 925 vesicles, 668 membranes, diseases of, 793 circulatory diseases of, 821 the, 460, 663, 714 veins of, 714 malformations of, 956 sand, 696 structure, 680 Bregma, 679 Broca's center, 724 Brown-Sequard syndrome, 492, 530 Brachypncea, 382 Brachial plexus, 614 Bradycardia, 169 Bulbar paralysis, apoplectiform, 952 paralysis, hereditary, 953 paralysis, progressive, 395, 406, 951 abscess, 871 Burdach's column, 475, 484 Bursitis, 633 Capsule, internal and external, 689 internal, functions, 736 Cannabis indica habit. 963 Caisson disease, 570 Cauda equina, diseases of, 573 Case-book chart, 44 Caries of the vertebrae, 499 Catalepsy, 146 Calcaneus, pes, 548 Cajal, cells of, 685 Cardiac crises, 382 Cavities of spinal cord, 562 Cathode, 65 Canals, semicircular, 149 Carcinoma of the brain, 910 Caudate nucleus, 690 Cauda equina, 463 tumor of, 557, 559 Causalgia, 48 Cerebro-spinal meningitis, epidemic, 808 Cerebral anaemia, 822 hyperaemia, 825 hemorrhage, 828 embolism, 845 thrombosis, 845 palsy of childhood, 854 diplegia, 854 paraplegia, 854 abscess, 867 Cerebral — syphilis, 877 localization, 662, 714 cortex, 672, 681, 717 concussion coma, 761 epiphysis, 695 Cerebro-spinal fluid, 495 Cerebellar agenesis, 450 cortex, 707 abscess, 871 tract, direct, 475. 484 Cerebellum, 705, 740 tumor of, 922 diseases of, 954 Center, Broca's, 724 Centers, cortical, 717 spinal, 480 association, higher, 747 psychomotor, 729 Central convolutions, tumor of, 920 myelitis, 537 scotoma, 787 ganglia, tumor of, 921 Cells of Purkinje, 707 Cervico-brachial neuralgia, 251 Cervical enlargement, 463 opisthotonos of infants, 820 myelitis, 536 hypertrophic pachymeningitis, 514 Cephalic flexures, 670 Cephalaea, 234 Cephalalgia, 234 Chemosis, 811 Choked disc, 784 Choroidal arterial system, 714 Chloroform habit, 963 Chromophyllic substance, 93 Cheyne-Stokes respiration, 753 Chromatolysis, 36 Chorea, 286 habit, 265 procursive, 299 magna, 132, 299 corpuscles, jtt; festinans, 299 hysterical, 132, 299 Chronic chorea, 297 infantile meningitis, 820 meningitis, Sao Chiasm, optic, 694, 788 Cholesteatoma of tin- brain. 910 Chorda tympani, 701 Chloral habit. 963 Chvosu-k\ sign, Cincture pain. 535 Ciliary body, 707 976 GENERAL INDEX Circle of Willis, 711 Circulation of the brain, 710 Circulatory diseases of brain, 821 Claustrum, 689 Clarke's vesicular column, 469 Clonus, ankle, 61 jaw, 62 Clonic spasm, 52 Climate in etiology, 32 Climato-therapy, 87 Classification of diseases, 22 Claustrophobia, 163 Clavus, 109 Cocaine habit, 963 Coccydynia, 251 Conarium, 695 Collateral, 91 Colonies, epileptic, 215 Coccygeal neuralgia, 251 Conus medullaris, 463 Cornual myelitis, 541 Cord, the, 460 tumor of, 555 abscess of, 555 malformations of, 569 spinal, diseases of, 526 spinal, anatomy, etc., 459 Cortex, cerebellar, 707 cerebral, 672, 681, 717 Cortical layers 684 arterial system, 711 deafness, 723 Corticopetal path, yz2> Conjugate deviation, 777 Conducting tracts of the brain, 710 Compression paralysis, 619 myelitis, 537 Compressed air paralysis, 570 Corpus callosum, tumor of, 922 Corpus dentatum, 707 Consciousness, 748 loss of, 760 double, 762 Concussion, spinal, 574 Congenital myotonia, 284 Conduction, spinal, 476, 481 Convulsion, 52, 772 Convolutions, cerebral, 672 Corticofugal path, 733 Coma, 761 Communicantes, rami, 308 Comma tract, descending, 475 Commissure, gray, 460 anterior, 472 Contraction of visual field, 780 Contracture, 53, 776 Confabulation, 595 Coprolalia, 270 Corpus callosum, 736 Corpora geniculata, 692, 738 subthalamica, 692, 695, 738 albicantia, 692, 739 quadrigemina, 692, 695, 738 striata, 689, 737 quadrigemina, tumor of, 922 Coxitis, 550 Coxalgia, 633 Cramp, 52, 167 writer's, 273 Cranial nerves, diseases of, 643 nerve nuclei, 698 Cranio-cerebral topography, 677 Craft palsy, 430 paralysis, 581, 621 Cretinism, 334, 337 Cremasteric reflex, 56 Crossed pyramidal tracts, 472, 483 paralysis, 655 paralysis, 924 paralysis, 51 anaesthesia, 779, 954 Cross-legged progression, 424 Crises, 381 Cry, hydrocephalic, 817 Crutch palsy, 581, 620 Crura cerebri, 739 Curvature, angular, 501 lateral, 509 Cup, physiological, 784 Cyclopia, 957 Cytoplasm, 93 Cysticercus of the brain, 910 Cyrtometer, 678 D Deafness, hysterical, 124 cortical, 723, 762 word, 723, 762, 769 Deep reflexes, 57 Deformans, arthritis, 969 osteitis, 343 Delirium, 760 hallucinatory, 107 acute, 874 grave, 874 tremens, 960 Dentate body, 707 nucleus, 739 Depression, 760 Deuteropathic atrophy, 393 Degeneration, 36, 584 reaction of, 170 primary, 580 GENERAL INDEX 977 Degeneration, secondary, 458, 580 Descending current, 85 comma tract, 475 Demarche tabeto-cerebelleuse, 448 Degeneracy, 29 Decentralization, 36 Dercum's disease, 345 Dentate ligament, 462 Deviation, conjugate, yyy Delusion, 758 Dendrite, 91 Dementia paralytica, 888 Dermographia, 74 Diatheses in etiology, 33 Diagnosis, general, 41 Diabetic polyneuritis, 603 Digiti mortui, 311 Dietetics, 78 Dipsomania, 959 Direct pyramidal tracts, 472 Diplegia, 774 cerebral, 854 Diplegic facial palsy, 654 Diseases, neuronic, 89 primary, 20 secondary, 21, 458 Disorientation, 595 Disseminated sclerosis, 901 nodal neuritis, 584 myelitis, 537 Dislocations of vertebrae, 496 Disc, choked, 784 Diplomyelia, 570 Diphtheritic polyneuritis, 599 Direct cerebellar tract, 475, 484 Diencephalon, 668 Diastematomyelia, 570 Diaphragmatic paralysis, 613 Diaphragm sellae, 666 Diplopia, 647, 792 Diuresis, 74 Diver's paralysis, 570 Doctrine, the neurone, 89 Dolorosa, adiposis, 345 puncta, 629 tubercula, 611 Double consciousness, 762 Dorsal nerves, 624 myelitis, 536 Dolorous paraplegia, 508 Dorsalis, tabes, 367 Dreaming, 141 Dropsy, sleeping, 148 Drunkard's paralysis, 581 Dura mater, 461, 665 Dysthyroidation, 323 Dysarthria, 407, j66 Dystrophy, progressive muscular, 409 Dyskinesis, 53 Dysphagia, 658 Dyspepsia, nervous, 168 Dynamometer, 52 Echinococcus of the brain, 910 Echolalia, 270 Echokinesis, 270 Eclampsia, 252 infantum, 252 parturientium, 254 ursemica, 255 Efferent diseases, 392 path, 98 Efferentes, rami, 308 Electric chorea, 299 Electro-diagnosis, 64 therapy, 85 Elephantiasis mollis, 610 Embolism, spinal, 528 cerebral, 845 Emissive centers, 717 Emission, seminal, 170 Emprosthotonos, 966 Emotional states, abnormal, 759 Encephalomalacia, 845 Encephalometer, 678 Encephalitis, 864 hemorrhagic, 865 suppurative, 867 Encephalocele, 957 , Encephalon, 663 Encephalopathia saturnina, 596 Endoneurium, 577 Endogenous diseases, 25 Endemic polyneuritis, 603 Endarteritis, spinal, 528 Enlargement, cervical and lumbar, 463 Epineurium, $jj Epilepsy, [88 hystero, 120 Jacksonian, 206 symptomatic, 206 traumatic, 190 reflex, 191 senile, 191 Epileptic fit, 200 coma, j()\ face, 209 colonies, 215 Epileptic equivalent, 205 Epilepticus, status, 189, Epiphysis cerebri. 695, 739 97 8 GENERAL INDEX Epidemic cerebrospinal meningitis, 808 Equivalent, epileptic, 205 Equivalents, hemicranial, 230 Equi no- varus. 548 Erythromelalgia, 314 Erigentes, nervi, 308 Erosion of vertebrae, aneurismal, 509 Erythrophobia, 167 Erb's sign in tetany, 260 palsy, 616 spinal paralysis, 523 Essential contracture, 256 Etiology, general, 25 Ether habit, 963 Examination of patient, 41 Excitation, reaction of, 260 Exaltation, 759 Exudative, inflammation, 39 Exogenous diseases, 25 Exercise, physical, 79 External pachymeningitis, 512 Exophthalmos, 324 Exophthalmic goitre, 322 Facts, 184 Facies epileptica, 209 Facial hemiatrophy, 348 tic, 265 Factitia, urticaria, 168 Failure, mental, 762 Familial diseases, 29 Falling sickness, 188 Fasciculus, solitary, 704 Fear neuroses, 163 Fetichism, 170 Fever, spotted, 808 Fibroma molluscum multiplex, 609 Fissures, cerebral, 672 Fibres, tangential, 683 Fibrillary tremor, 53 Fibroma of the brain, 910 Fit, epileptic, 200 Filum terminale, 461, 463 diseases of, 573 Flaccid paralysis, 51 Fluid, cerebro-spinal, 495 Flexures, cephalic, 670 Forced movement, 53 Focal neuritis, 629 Frankel's exercises, 391 Folie musculaire, 291 Folie de grandeur, 894 Frontal lobes, tumor of, 918 Friedreich's disease, 444 Fractures of the vertebrae, 496 Frontal lobes, functions of, 719 Functional diseases, 40 Funiculus, 577 gracilis, 475 cuneatus, 475 Gal77 Thermometry, local, 74 general, 75 Therapy, symptomatic, 81 Thermanaesthesia, 48 Thrombosis, cerebral, 845 of the sinuses, 850 spinal, 528 Thalamencephalon, 668 Thalami, optic, 691, 737 Thomsen's disease, 284 Thyroiodin, 323 Tic impulsif, 269 de pensee, 270 spasmodic, 264 convulsif, 264 facial, 265 douloureux, 248 Tinnitus aurium, 166 Titubation, 055 Tobacco heart, 169 Tonic spasm, 52 influence, spinal, 476, 481 Topoalgia, 48 Topography, spinal, 459, 485 cranio-cerebral, 677 Torticollis, spasmodic, 267 Toxic headache, 236 polyneuritis, 592 Traumata, 33 Traumatic neuroses, 182, 575 epilepsy, 190 Tracts, optic, 692 Tractus tegmento-cerebellaris, 695 cortico-spinalis, 698 Tremor, 53 intentional, 905 Tremens, delirium, 960 Treatment, general, 75 Triceps reflex, 62 Trigeminal neuralgia, 248 •Trismus, 966 Trophic symptoms, 74 influence, spinal, 476, 481 T*ophoedema, hereditary, 320 Trousseau's sign, 260 Trophoneuroses, 74, 306 Tube, neural, 668 Tubercular spondylitis, 499 Tubercula dolorosa, 611 Tuberculous meningitis, 814 Tuberosa, urticaria, 318 Tubercle of the brain, 910 Tubular vision, 112 Tumor of the spinal cord, 555 of the cauda, 557, 559 of the brain, 909 of the vertebrae, 507 Tumors of the nerves, 509 Turck's column, 472 Tussis hysterica, 119 Tympani, chorda, 701 Uraemic convulsions, 255 coma, 761 Uncrossed pyramidal tracts, 472, 483; Upper arm palsy, 616 Urticaria, 970 tuberosa, 318 giant, 320 factitia, 168 Urethrodynia, 252 V Vacuolation, 36 Valleix's points, 586, 629 Vasomotor symptoms, 72 reflexes, 63 action, 476 Vasocorona, 465 Veins of the brain, 714 Venae Galeni, 714 Ventricular hemorrhage, 836 zone, 475 Ventral cerebro-spinal fasciculus, 476* Vertebrate animal, 459 Vertebrae, diseases of, 496 caries of, 499 tumors of, 507 syphilis of, 509 erosion of, 509 Vertigo, 55, 149, 752 ocular, 152 aural, 153 Vermis, 705 Vesicular columns, 469 Vesicles of the brain, 668 Vicq d'Azyr, line of, 682 Visceral reflexes, 63 neuralgia, 252 GENERAL INDEX 989 Vision, tubular, 112 Visual disturbances, 780 aphasia, 770 field, contraction of, 780 area, 722 Vitse, arbor, 707 Vita sexualis, 191 Volitantes, muscae, 166 Voluntary speech, 169 Voluntaristic psychology. 754 Vomiting, 751 Von Graefe's sign, 327 W Wallerian degeneration, 37. 351, 584 Weber's test, 792 Weight of brain, 664 "Weir Mitchell treatment, 178 Weigert-Edinger hypothesis, Wet brain, 806 White matter, 97 Willis, circle of, 711 Word-deafness, 723, 762, 769 blindness, 762, 770 Wrist reflex, 62 drop, 566, 619 Wrisberg, nerve of, 700 Wryneck, spasmodic, 267 Writer's cramp, 275 Zone, hysterogenic, in sensitive-motor, 729 Zones, radicular, 475 Zona molecularis, 707 granulosa, 707 357 ■HM LIBRARY OF CONGRESS ,Y*T ■ .* ,**. 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