>0« "of ^ ••••• ,# ©*. •Sri-* ^ * d* * ^ P *»V* 4> O * & - - "oV* /% *^ -^ ** %■ Motor Points on Shoulder and Arm, Motor Points on Forearm and Hand, Motor Points on Inner Surface of Arm, . " Ape Hand," Extreme " Main en Griffe," Slight Atrophy of Interossei, . 20. Motor Points on Thighs and Legs, . 21 to 25. Deformities of Feet, 26. Pes Equinus, ...... Pes Equinus of Eight Years' Duration, . Motor Points on Anterior Surface of Leg Motor Points on Trunk, .... Patient showing Lordosis, Atro of the in Deeeneration of the Small 31 to 37. Distribution of Sensory Nerves, 38. Child in Convulsive Seizure, 39. Diffuse Neuroglia Sclerosis in Epilepsy 40. Various Phases of the Earlier Stages Ganglion Cells, 41. A Group of More Mature Neuroglia Cells Pyramids, 42. Epileptoid Stage of Hystero-epileptic Attack. 43. The Three Types of Distribution of Anaesthesia in Hysteria 44. Hysterical Loss of Color Sense and Limitation of Visual Field 45. Dilatation of Blood-vessels in White Matter of the Convolutions in Chorea, the Layer of S. PAGE 5 6 9 14 17 17 19 21 21 23 23 25 25 27 27 29 3i 32 32 32 33 34 39 50 76 76 77 90 99 100 XVI LIST OF ILLUSTRATIONS. 46. Changes in Purkinje's Cells in Chorea, . 47. Tetanus Bacilli and Spores, 48. Position of Hands in the Spasm of Tetany, 49. Location of Head Pains, .... 50. Case of Myxcedema with Idiocy, 51 to 55. Normal and Degenerated Nerve Fibres, 56. The Brachial Plexus ; Branches involved in Upper-Arm Type, 57, 58. Anaesthetic Areas of Hands after Section of Ulnar Nerve, 59. Palmar Surface after Section of Median Nerve, 60. Relation of Seventh, Eighth, and Glosso-pharyngeal Nerves, etc. 61. Boy with Facial Palsy in Stage of Recovery, . 62. Young Boy with Multiple Neuritis, 63. Chronic Interstitial Neuritis, 64. Diagram showing the Relation of the Vertebral Spines, etc., 65. Outlines of IV. and V. Cervical Segments, 66. Cross Section of Sixth Cervical Segment of a Boy, Two Years Old, 67. Diagram showing the Probable Relation of Cells and Fibres, etc. , . 68. Descending Degeneration after a Lesion in the Internal Capsule, . Plate I. Fig. I. Sensory Tract, Fig. II. Cross Section of Cord, Fig. III. Relation of Motor Tract to Nuclei of Cranial Nerves, 69. Secondary Degeneration Following a Lesion in the Left Cerebral Hemisphere, 70. Diagrammatic Cross Section of Spinal Cord, 71. Course of Sensory Fibres from the Posterior Roots to the Medulla Oblongata, 72. Division of Sensory Root Fibres — Human Embryo of Six Months, . 73. Blood Supply of Spinal Cord, 74. Case of Infantile Spinal Palsy, 75. Case of Acute Infantile Cerebral Palsy, 76. Paralysis of Upper Arm with Atrophy, etc., Section through Lumbar Segment in Acute Poliomyelitis, Poliomyelitis Anterior of Old Standing, etc., Poliomyelitis Anterior ; Chronic Stage, etc., Secondary Ascending and Descending Degeneration, Sketch of Section of Spine in a Case of Fracture Dislocation of the Seventh Cervical Vertebra, 82. Section through Pons showing Gummatous Infiltration with con- siderable Destruction of Tissue, 83, 84. Sections showing Specific Lepto-meningitis, . . . 339, 85. Degeneration of Cauda Equina in Multiple Sclerosis, 86. Sclerotic Patches in Multiple Sclerosis, 87. Vicinity of Locus Cceruleus in Multiple Sclerosis, .... 88. Patient with Paralysis Agitans, who also exhibited some Symptoms of Disseminated Sclerosis PAGE 123 154 161 171 206 218 219 221 222 229 231 241 249 264 265 266 267 269 270 270 270 270 273 275 276 281 290 291 293 300 301 302 314 329 333 340 348 349 35o 353 LIST OF ILLUSTRATIONS. XV 11 FIGURE PAGE 89. Section through Seventh Cervical Segment, showing Gliosis of Cord, 374 90. Section through Part of Fourth Dorsal Segment in Gliosis of Cord, 375 91. Case of Friedreich's Disease, ........ 378 92. Same Case, showing Marked Atrophy of Shoulder Muscles, . . 379 93. Deformity of Feet in Friedreich's Disease, ..... 382 94. Section through Middle Dorsal Region of a Case of Friedreich's Disease, 387 95. Section through Cervical Segment in same Disease, . . . 388 96. Brain in Cerebral Type of Hereditary Spastic Paralysis, . . . 397 97. Section through Cervical and Dorsal Segments in a Case of Me- ningoencephalitis. Probable defective development of pyram- idal tracts, etc., . . . . . . . . . 399 98. Section through Motor Cortex in a Case of Spastic Paraplegia, . 400 99. Diagrammatic Representations of Divisions of Progressive Mus- cular Atrophy, .......... 405 100. Two Brothers Afflicted with Peroneal Form of Progressive Muscu- lar Atrophy, 412 101, 102. Case of Peroneal Form of Progressive Muscular Atrophy, 414, 415 103 to 105. Case of Progressive Muscular Atrophy in a Child with a Spinal Lesion, 417 106 to 108. Three Brothers, illustrating Various Types of Progressive Muscular Dystrophies, 422-424 109. Boy with Pseudo-Hypertrophy attempting to Straighten Himself, . 425 1 10. Patient with Landouzy-Dejerine Type, 426 in, 112. Changes in Muscular Tissue in a Case of Primary Dystrophy, 433 113, 114. Changes in Muscle and Nerve in a Case of the Landouzy-De- jerine Type, 434 115, 116. Boy with Defective Development of Scapula and Shoulder Muscles, 435. 436 117. Meningocele, etc., 441 118. Child of Seven Years with Spina Bifida and Deformity of Feet, . 442 119. Right Hemisphere of a Simply Convoluted Brain, .... 446 120. Mesial Aspect of same, 447 121. Relation of Brain to Skull Lines, 44S 122. Localization of Cortical Centres, 449 123. Reid's Lines, 45° 124. Motor Fibres for the Facial Nerve and Extremities, . . .451 125. Diagram to show relative Position of the divisions of the Motor Tract, 452 126. Diagram representing Motor Innervation of a Muscle, etc.. . . 453 127. View from Before of Medulla Oblongata, etc., .... 456 128. Course of Visual Fibres, 460 129. Diagram representing Secondary Degeneration, etc 463 130. Arteries at the Base of the Brain, 4.65 131. Cortical Distribution of Middle Cerebral Artery 4' N o XV111 LIST OF ILLUSTRATIONS. 132. Distribution of Blood Supply at the Base, .... 133. The Veins of the Du"a Mater, 134. 135. Diagrams illustrating Aphasia, 474 136. Case of Unilateral Nuclear Palsy, etc. Hypoglossal nucleus of left side, 137. Same Case. Hypoglossal nucleus, right side, 138. Case of Left Hemiplegia, 139. Right Hemiplegia with Contractures and Retarded Growth of Arm 140. Congenital Diplegia — " Frog Girl," 141. Case of Spastic Diplegia ; cross-legged progression, 142. Child with Congenital Diplegia ; Microcephalic Skull, Strabismus and Idiocy, 143. Meningeal Hemorrhage at Birth, 144. Cyst due to Softening of an Obstruction of Middle Cerebral Artery etc., 145. Section through Portion of Motor Cortex, removed at Operation for Localized Epilepsy, 146. Variously Degenerated Cells from same Section, 147. An Old Hemorrhagic Cyst ; Tumor growing in its Walls, 148. Section passing through Posterior Quadrigeminal Bodies, showing Tumor, 149. Tumor (Sarcoma) of the Cerebellum, 150. Vertical Section through Cyst and Tumors in Brain, 151. Dissections showing the Guide adopted in successful Trephining for Abscess from Ear Disease, 152. Patient exhibiting Symptoms of Thrombosis of Cavernous Sinus, 153. 154. Brain with Large Anterior Defects, .... 592 155. Large Double Porencephalic Defect, 156. Skull of Child showing Changes four Months after Lannelongue's Operation, 157. Defective Development of Occipital Lobes, .... 158. Brain of Cerebral Type of Hereditary Spastic Paralysis, . 1 59. Child with Pleuroplegia, 160. Hydrocephalic Idiot 161. Congenital Idiot, 162. Case of Myxcedema with Idiocy, ..„.„„ PAGE 467 469 475 510 5ii 525 527 53i 533 536 54i 543 544 545 546 565 567 570 584 586 593 594 598 600 602 605 635 639 645 THE NERVOUS DISEASES OF CHILDREN. CHAPTER I. INTRODUCTION— METHODS OF EXAMINATION. The nervous system of the child is subject to many dis- eases. Some of these are identical with the nervous dis- orders of tne adult ; others are peculiar to the early years of life. The brain and the spinal cord do not attain their full development until months and years after birth, and even the peripheral nerves do not exhibit all their normal func- tions until the child is several weeks old. During this pe- riod of incomplete development the nervous system responds much more energetically to morbid influences than it does in later years. This is especially true of the brain. It is in a state of irritability and instability, and a perversion of functions may result from causes which would exert little or no influence over the nervous system of the youth or adult. Evidence of this is furnished by the behavior of a child in fever. The irregular choreiform twitchings and the delirium are often the outward signs of an unstable cerebral state, while the unusual irritability of the brain is proved by the occurrence of convulsions upon peripheral irritation to which the adult brain would not at all respond. In the early period of lite, too, hereditary affections of the nervous system are frequently manifest, and morbid psychic inheritance casts its shadows before. Inhibition of normal development may occur at any period ; family affec- tions are developed in the earlier years of life, and the acute infectious diseases of childhood are often followed bv serious nervous disorders. If we add to these, diseases due to traumatism, we have an array of nervous disorders equal 2 THE NERVOUS DISEASES OF CHILDREN. to, if not in excess of, those that occur after the age of puberty. Childhood is exempt only from the diseases due to senile deterioration, from degeneration and sclerosis of the brain and spinal cord, and relatively free from those due to toxic agents, such as alcohol, metallic poisons, and syph- ilis ; but the effect of such diseases in the parent is exhib- ited with cruel persistence in the offspring. The diseases of the nervous system during the period of incomplete development are to be the special subject of this treatise. Before proceeding to the description of dis- ease it will be necessary to adopt a correct Method of Examination.— First of all inquire into the ancestry of the child. A reliable history of the physical and mental condition of parents, grandparents, and other relatives is of the greatest value in establishing a diagnosis. The habits and the diseases of the parents should be care- fully determined, for of the ills the child is heir to, not a few are due to alcoholism, to syphilis, to epilepsy, to hysteria, in one or both parents. Next to heredity, environment plays the most important part ; it is well, therefore, to inquire into the home surroundings, the manner in which the child is watched and cared for ; how it has been fed, trained, and educated. The previous history of the child is next in order. In every case inquire into the manner of its birth ; whether it was protracted or not ; whether or not instruments were used ; whether the child was asphyxiated at birth or at once began to breathe freely. Make inquiries regarding the occurrence of spasms or convulsions ; the time at which the child began to take notice of things, to recognize parent or nurse, to stand, to walk, and to talk. The occurrence of other diseases, of the ordinary infectious diseases of child- hood, of whooping-cough, of pneumonia, scarlet fever, measles, meningitis, should be determined, and one should never forget to ask whether similar nervous conditions have been previously observed. Then proceed to the Examination of the Patient. — The art of making a diagnosis by mere inspection has gone out of date, and is cried down by many ; yet I am willing to say that in fully one-half of the nervous diseases of children the nature of INTRODUCTION— METHODS OF EXAMINATION. 3 the trouble can be suspected, if not made out, by a thor- ough inspection of the child without putting a finger to its body. I am not in favor of hurried examinations ; on the contrary, I wish to plead for the greatest accuracy in ex- amining for details ; but let the physician or student train his powers of observation and his diagnostic ability will be more acutely developed than that of the man who can never even suspect a disease unless he has all his tools (percus- sion-hammer, thermometer, aesthesiometer, electrodes) con- stantly at his command. In my lectures to students I insist that they shall study the general ap- pearance of a child, and should not feel satisfied until they learn to recognize peculiarities of facial expression, of gait, and of stature ; to distinguish be- tween the behavior of the normal child and the feeble-minded, between spastic and flaccid palsies, and to determine by the peculiar deformity of the foot or by the scraping noise which the patient makes in walking, which group of muscles is affected. It is important from the history of the patient, and from these general observations to get correct first impressions ; these first impressions are then to be confirmed by a careful detailed examination. Never make a diagnosis unless the child has been wholly undressed ; if this is not done a Pott's paralysis may be taken to be a traumatic myelitis, or a neuritis may be mis- taken for poliomyelitis anterior. Lay the child on a table or on another person's lap in order to get a full view of it ; of the relative size of head and body ; of the proportionate development of arms, legs, and abdomen. Remember also that the child has heart and lungs, liver, spleen, and intes- tines, which, if diseased, may hold an important relation to the nervous disorder present. In proceeding to a detailed examination it is best to begin with the head, including the face, then take up the upper extremities, the abdomen, and finally the lower extremities. The following scheme includes the more important points to be estab- lished in the examination of a child ; the exact order of inquiry is subject to slight modifications. EXAMINATION SCHEME. Head (Skull). — Size? Shape? Symmetrical? Dolichocephalic? Brachy- cephalic ? Fontanelles ? Hydrocephalus ? Bulging (Frontal or occipital) ? Mental condition ? Speech ? 4 THE NERVOUS DISEASES OE CHILDREN. Eyes (Fundus). — Vision? Field of vision? Pupils? Light and ac- commodation reflexes ? Ocular movements ? Nystagmus (Lateral or ro- tatory) ? Is cornea sensitive ? Hearing? Face. — Symmetrical ? Paralysis ? Tongue ? Deglutition ? Articula- tion ? Sensation in face ? Teeth ? Upper Extremities. — Are they symmetrical ? Position ? Circumfer- ence of arm and forearm ? Movements (Flexion, Extension of forearm, wrist, fingers) ? Paralysis ? Tonus of muscles ? Are muscles atrophied or hypertrophied ? Reflexes ? Contractures ? Electrical reactions ? Sensa- tion ? Trunk. — Respiration ? Sensation ? Reflexes (Abdominal, Epigastric, Cremasteric) ? Action of muscles ? Lower Extremities. — Are they symmetrical ? Circumference of thighs and calves ? Ability to stand ? Romberg's symptom ? Ability to walk ? Gait (Paretic, Spastic- paretic, Ataxic, Cerebellar) ? Movements of individual groups of muscles ? Is child able to raise thigh ? To flex and extend thighs, legs, toes ? To stand on tiptoes ? To elevate toes, keeping heels on ground ? Are muscles paretic or paralyzed, atrophied or hypertrophied ? Electrical reactions ? Reflexes (Knee-jerks, Ankle clonus, Achilles tendon reflex) ? Contractures ? Sensation ? Vesical and Rectal Reflexes? Examination of the head of a child often gives us val- uable information. The normal head should be well rounded and symmetrical. According to the age of the child the size will vary. The average horizontal circum- ference at birth (measured by a line passing from the gla- bella around the occipital protuberance) is between 38 and 42 ctm. ; at the end of one year between 45 and 52 ctm., and in later years it may grow gradually to 56 ctm. Any marked departure from these measurements is abnormal, but heads of tolerable size may be associated with deficient development of parts of the brain. I have seen cases with normal circumference in which the anterior defect was not evident in the measurement in consequence of a slightly ex- cessive development of the occiput. A normal circumfer- ence is also present at times, although the actual cranial ca- pacity may be very much diminished by a receding frontal bone. The following table will give the chief measurements of the skull in chil- dren ; a few centimetres should be deducted for the thickness of hair and scalp. INTRODUCTION— METHODS OF EXAMINATION. Table of Cranial Measurements in Children. New-born. End of 1st yr. 1st to 7th yr. 10th year. M. F. M. F. M, F. M. 47 i. Circumfer- ence 2. Binauricu- 34- 20.0 385 to 450 34.o 20.0 42.0 25- S 700 to 1, 000 28.0 42.0 25.0 34 to 46 ... . 27 49 Taken around glabella and occipital pro- tuberance. 3. Volume . .. 4. Naso- occi- pital arc. j B to opposite ext. aud. mea- tus. 1,300 ... Volume is to cir- i 20.0 22. a 28.0 10.0 10. cumference as 1,350 is to 50 (in the adult). N, 0, T. N, 0. 5. Naso-breg- matic arc. 7-7 9.0 7-7 9-o 12 6 Bregmatic lamb, arc. 12 e to A. 1 Fig. 1. — Craniometrical Lines. (Benedict and Peterson.) The formula for the cephalic index is length : breadth :: 100 : x. An in- dex below 78 is dolichocephalic ; 78 to 80 mesocephalic ; above So brachyce- phalic. The facial length is determined by a line passing from N to lowest part of chin. Both halves of the head should be symmetrical. Asym- metry occurs chiefly in connection with defective develop- 6 THE NERVOUS DISEASES OF CHILDREN. Fig. 2 Six Years. (Peterson.) ment of the brain and with early cerebral lesions. (Fig. 2.) The chief abnormalities of skull formation are as fol- lows: Dolichocephalus, a long skull, the anterior poste- rior diameter being pro- portionately greater than the transverse. Many new- born children are dolicho- cephalic as the result of compression of the head in the pelvic canal, but af- ter a few days or weeks the head is well rounded. Asymmetry of Skull in a Male, aged Brachycephalic — the skull Right Hemiplegia from Birth. j s s hort in the antero-pos- terior diameter. The terms microcephalus and macrocephalus need no further expla- nation. * Bulging of the frontal or occipital bones is important as an indication of hydrocephalus. If there is a very con- siderable increase of intracranial fluid the sutures may be pushed asunder and can be felt distinctly through the scalp. This same condition occurs in some cases of neo- plasm. In passing the hand over the head the fontanelles can be felt. The occipital fontanelle should be closed after a few weeks, the anterior remains open until the tenth or twelfth month. If it closes long before this period there is premature ossification of the sutures ; if it remains open much longer, it is a certain sign of rickets. After the examination of the head Ave may pass at once to the inquiry into the mental condition of the child. Ac- cording to its age we must ask whether it recognizes its mother or nurse; whether it has learned to play, to understand what is said to it ; whether it begins to imi- tate sounds, to articulate, etc. ; in short, whether it shows a normal awakening of the mind. In children a little more advanced in years it is necessary to determine whether the child is able to keep up with others of its age ; whether it * A few special terms have been in use for oddly shaped heads : Keel-shaped skull, scaphocephalus ; triangular skull, trigonocephalus ; steeple-shaped skull, oxycephalus ; obliquely deformed skull, plagiocephalus. INTRODUCTION— METHODS OF EXAMINATION. 7 has been able to acquire the ordinary rudiments of knowl- edge. The physician should assure himself of these points by a personal examination, and should not depend upon the statements of parents and relatives. A few extracts from Preyer's observations on his own child will show what may be expected of a normal child at different stages of its development: During First Month. — Recognizes difference between light and dark objects (even on first day) ; follows with its eyes object moved slowly before it (as early as eleventh day) ; begins to hear about the fourth day ; recognizes sounds toward end of first month ; learns to distinguish between bitter and sweet ; recognizes disagreeable odors ; first tears on twenty-third day during a crying spell ; expresses displeasure by turning head away, by shutting its eyes, and, of course, by crying ; begins to smile. During Second Month. — Recognizes human voices and direction from which sound comes ; turns head toward low sounds ; is quieted by song ; smiles when music is heard ; recognizes its mother. During Third Month. — Moves arms, expressive of pleasure ; listens attentively ; is able to support head a little ; uses definite sounds in crying. During Fourth Month. — Associated eye movements perfect ; stares at new objects ; recognizes strange surroundings ; reaches after distant ob- jects ; first attempt to sit upright. During Fifth Month. — Recognizes strangers as such ; likes to take hold of everything ; stretches out its arms to be taken up ; holds head straight ; sits alone ; moves legs as if to walk ; forms syllables. During Sixth Month. — Distinguishes faces ; stares at strangers ; smiles, if smiled at ; smiles with relatives, not with strangers ; turns its head toward a person leaving the room ; begins to creep ; " crows." During Seventh Month. — Follows objects dropping out of its hands ; recognizes its image in mirror with evident pleasure ; points with finger at pictures ; purposive movements ; associates persons and names ; extends hand when asked ; articulates a number of different sounds in cry- ing and in " lolling to itself." During Eighth Month. — Sits upright when it is carried ; some chil- dren attempt to stand and to walk. During Ninth Month. — Begins to imitate tunes ; laughs heartily ; begins to beg for things. During Tenth Month. — Takes an intense interest in its food ; rec- ognizes parent after absence of several days ; he begins to walk alone ; an- swers questions by motions and indicates where certain things are. During Eleventh Month.— Stands quite alone ; pushes chairs ; makes first attempt to repeat sounds impressed upon its mind ; begins to articulate its own name ; understands language fairly well. During Twelfth Month.— Imitates laughter of others ; stretches its 8 THE NERVOUS DISEASES OF CHILDREN. arms out to enforce its demands ; improvement in walking and standing ; looks at others attentively while they eat. During Fourteenth, Fifteenth, and Sixteenth Months. — In- dependent speech is acquired, and repeats spoken words easily ; in seven- teenth month may speak short sentences, using verbs ; from this time on there is steady improvement in memory of words and use of language. At two years child may learn to repeat rhymes, to detect colors, etc. If the mental condition of the child has been satisfac- torily determined, the special senses should be examined. " Does the child see? " is a question often answered affirma- tively by the mother, when a closer examination proves that the child is totally blind. Mothers are easily deceived in this, for the restless movements of the eyes in young children are supposed to be purposive and part of the vis- ual act. To test vision use a candle or a taper, and pass it in front of the eyes at some distance from the head, so as to avoid heat sensations, and note whether the child fol- lows the light. Do not be misled by accidental movements and avoid using rattles, for the child may turn its eyes in OD Grasset et Fig. 3.— Normal Visual Fields for Colors. the direction from which the sound issues without seeing the object. Use also simple substances of different colors (pencils, papers, glass, etc.), and see whether the child fol- lows these objects. It is a matter of still greater difficulty to test the field INTRODUCTION— METHODS OF EXAMINATION. 9 of vision. In very young children it may be altogether impossible, but after a child has reached the age of five months or thereabouts, it may be possible to make a rough test of the visual field by passing objects from above and below, as well as from the sides, into the visual field, and noticing when the child begins to turn its eyes toward this Fig. 5.— Field of Vision in a Case of Left-sided Hemianopsia. The Shading Repre- sents the Blind Part ; the Oval Outline is the Average Normal Field. (Gowers.) object. The ordinary test as applied in the adult cannot be employed for children until they have developed con- siderable intelligence. The condition of the visual field is often overlooked and the defects re- ferred to are much more frequent than they are generally supposed to be. An examination should be made in all cases of cerebral palsies, and in those in which cerebral tumor is suspected. In cases in which a very complex examination seems desirable, and these are relatively few, special tests should be made for form and color. The hemianopsic defects as they occur in tumors, and in cerebral palsies (first described by Freud), the concentric limitations occurring in hysteria, are the most common defects in children. The pupils are to be tested for their size and power of contractility ; both should be equal and contract promptly to light and during accommodation. The movements of the ocular muscles will be best understood by reference to the subjoined table. Conjugate deviation is due to disease in the hemisphere, and according to Grasset to lesions in the first and second frontal convolutions and in the angular gyrus and its vicinity ; the patient looks toward the lesion except in the case of spasm of the muscles, when the patient looks away from the lesion. Nystagmus occurs in many cerebral affections, particularly in cases of early cerebral disease or congenital defect. It is seen also in cases in IO THE NERVOUS DISEASES OF CHILDREN. which there are stigmata of degeneration as well as in multiple sclerosis. In the latter it may be bilateral or rotatory. The corneal reflexes (the prompt closure of the eyelids if the cornea is touched carefully with the head of a pin or a small lead-pencil) should be tested. In a great many individuals this reflex does not ensue upon irritation of the conjunctiva, a fact that has deceived many physi- cians. Muscles of the Eyes and Face. Diseases in Name of Normal Symptoms of Defi- Innervated Represented which Muscle Muscle. Function. cient Action. by in is commonly Involved. Ciliary . . . Makes 1 e n s e Loss of accommo- The thirdCorpora l"gS.c;S° more convex ; dation ; spasm of nerve. quadrigem- 3.2S M 3 - c? = aids in accom- ciliary muscle may ina and pe- modation. cause myopia. duncle. Sphincter Contraction of Paralytic mydria- The thirdCorpora P.^o-5 « iridis. pupil to light sis ; no contrac- nerve. quadrigem- ^»° V ■ — and during tion to light and ina and pe- .2 S, h o & accommoda- during conver- duncle. i go |-S| tion. gence or accom- modation. tmu theri in ; ti rior tary Dilatato r Dilates pupils; Pupil does not re- Sympathetic pupillse. as a result of spond to sensory •-,£3 5 « — « ~ • ft - sensory or stimulation. "S .~ ~ — u psychic stim- C .12 r, wS ■" ulation. Meni eye ofm lis ; drug Rectus su- Moves eye up- Upward movement The third Corpora , •->, perior. ward and in- limited; diplopia; nerve. quadrigem- .2 js ward. Acts false image ina and pe- with inferior above ; deficient duncle. O 3 ft_a oblique. rotation of eye- ball. "C ft Rectus in- Moves eye in- Strabismus diver- As above. Corpora ternus. ward. gens ; defective quadrigem- 13 w inward pull. ina and pe- duncle. c 3 • 2 o o a s ea Rectus in- Moves eye Imperfect move- As above. Corpora ferior. downward ment downward ; quadngem- •2o S and rotates it eye rotated out- ina and pe- g.S-'-S inward. Acts ward. duncle. ! --"3 rt with superior I S- 3 CJ O C 7? oblique. Obliquus Acts with rec- Imperfect move- As above. Corpora inferior. tus superior, ment upward ; eye quadrigem- : •-" "rt moving eye rotated inward. ina and pe- 5 8*S upward and duncle. ■a^a outward, and \ Z~ s rotates it up- ,- rt O — t- y ward. g-S- Obliquus Moves eye On looking down- The fourth Peduncle — u - — . superior. downward ward eye is pulled nerve (tro- near the and outward. inward ; conver- chlearis). corpora| £ --^ Acts with in- gent strabismus ; quadrigem- .£-s| ferior rectus ; diplopia on step- ina. m .5 g3 5 n 2 §.2 rotates down- ping downward. ward. LQ 5rt INTRODUCTION — METHODS OF EXAMINATION. Muscles of the Eyes and Face. — Continued. Diseases in Name of Normal Symptoms of Defi- Innervated Represented which Muscle Muscle. Function. cient Action. by in is commonly Involved. Rectus ex- Moves eye out- Out ward move^The sixth Pons As before, and ternus. ward. ment impaired ; nerve (ab- in disease of head turned in di- ducens). the pons. rection of para- lyzed muscle. Levator Raises upper Ptosis ; eye closed; The third Pedun c le Associated palpebrae eyelid. may be opened a nerve. and cor- with other superioris little by frontalis pora quad- third nerve muscle. rigemina. diseases; often congenital. Orbicular- Closes eyelids. Eyes cannot be The seventh Pons Peripheral fa- is palpe- closed (lagoph- nerve. cial neuritis ; brarum. thalmos). affections at base of brain (meningi t i s , tumor, and the like) ; in some dystro- phies. Fronta 1 i s Raises eye- Imperfect raising As above. As above. As above. and cor- brows ; fold- of eyebrows ; no Teg a t o r ing of skin of frowning ; lines of supercilii forehead, as in frowning. forehead d i s a p- pear. Orbicula- Move lips and Face distorted and The seventh Pons Cerebral apo- ris oris, cheeks, as in pulled toward nerve. plexies (es- buccin- speaking, kiss- healthy side ; ina- cape of upper ator, and ing, showing bility to pout lips branches of other of teeth, and or to whistle; drib- fa c i a 1 ) ; pe- muse 1 e s the like. bling of saliva ripheral facial of face. from paralyzed side ; flapping of cheek with each expiration, owing to paralysis of buccinator ; d i s- app earanceof nasolabial fold. palsy (of t e n due to expos- ure ) ; in all lesions of pons ; in dys- trophies. Masseter , Masseter, tem- Mastication imper- The fifth Pons Rarely affect- tempor- poral, and in- fect ; if in spasm, nerve. ed in children al, and ternal ptery- jaws cannot be except in tet- ptery- goid elevate opened; jaw anus ; in facial goids. lower jaw; moved toward hemiatrophy; external pter- paralyzed side by in lesions ygoid draws action of ptery- of pons. jaw forward ; goids. the two ptery- goid muscles of one side acting togeth- er move teeth toward oppo- site side. Mydriasis can be caused by paralysis of sphincter iridis (third nerve), or by spasm of the dilator muscles (mydriasis spastica). Atropin has a simi- lar effect by paralyzing the sphincter, and contracting the dilator. 12 THE NERVOUS DISEASES OF CHILDREN. Myosis may be due to irritation of sphincter supplied by the third nerve, and occurs in early stages of many cerebral affections, including apoplexy ; or it may be due to paralysis of the dilator muscle. Inequality of pupils is sometimes congenital, more often due, however, to cerebro-spinal disease (meningitis, syphilis, multiple sclerosis, hereditary ataxia). Muscles of both eyes act conjointly. If such action is deficient we speak of conjugated paralysis. There may be paralysis of lateral, upward or downward movements, without total palsy of any one muscle. If individ- ual muscles are paralyzed, or muscles in one eye only, double vision is certain to result. In diplopia the exact position of true and false images will help to de- termine the degree of ocular paralysis. If one muscle is paralyzed second- ary deviation of the associated muscle in the other eye may take place. Paralysis of the inner muscles of the eye is designated as ophthalmo- plegia interna ; paralysis of the muscles moving the eyeball, as ophthalmo- plegia externa, and such ophthalmoplegia may be total or partial. Total ophthalmoplegia externa and interna is the occasional accompaniment of dis- ease at the base ; partial ophthalmoplegia (only part of the muscles sup- plied by the third nerve, for instance, being affected) points to nuclear disease, but syphilitic infiltration of the root fibres may simulate nuclear disease. Narrowing of the orbital fissure (sometimes congenital) may be due to paralysis of smooth fibres in the lid, innervated by the sympathetic. Clonic movements of eye muscles we term nystagmus ; this may be lateral or rota- tory. An examination of the sense of hearing is often called for. In children with defective development this may be entirely wanting. The simplest tests are to clap the hands at a distance from the child's head, to whisper its name, to use a loudly ticking watch — even very young children will be attracted by the sound if it is perceived. In the case of older children tuning-forks may be applied to the head. In disease of the ear proper the vibrations would be perceived, but not so if the nerve itself, the labyrinth, or the auditory tract were involved. In continuing the examination of the head we must note whether the face is symmetrical. Asymmetry may point to congenital defect or to a preceding palsy. It is of some interest to know that asymmetry of the face and of the nose, adhesion of the ear-lap, malformations of the outer ear, the existence of a torus palatinus (felt as a ridge in the roof of the mouth) are the ordinary stigmata of a degenerative type, of which so much has been made by the Italian crimi- nologists of the present day. If such stigmata prove a INTRODUCTION — METHODS OF EXAMINATION. 13 predisposition to the development of nervous troubles, to crime, or to insanity, it is well that the physician should know of them. The teeth play a very important part in the life of every child. If not de- veloped between the ages of six months and one year, rickets must be suspected. Notched teeth are suspicious of syphilis. Unusually early de- velopment of the teeth is an evidence of premature ossification. Muscles of Tongue, Palate, and Pharynx. Name of Muscle. Normal Function. Symptoms of Defi- cient Action. Genio- glossus. Styloglos- sus. Li ngual muscle proper. Az y g o s uvulse. L e vat o r palati. P a 1 a t o- pharyn- geal mus- cles. Stylo-pha- ryngeus. Pushes tongue to opposite side. Raises tongue backward and upward. All movements of the tongue itself. Shortening of uvula. Raises the vel- um palati. Prevent food from passing 1 toward up-| per part of pharynx and| posterior' nares. Helps to draw. larynx up- ward so as to be closed by; epiglottis and; ov ertopp e d 1 by tongue. Tongue when pro- truded deviates to paralyzed side. Tongue cannot be moved backward or hollowed out (action deficient in many healthy subjects). When lying in mouth deviation to healthy side ; when protruded deviates to paral- yzed side ; if one or both halves are atrophied tongue looks shrivelled. Uvula deviates to- ward sound side ; if both sides are paralyzed there is nasal tone and regu r g i t a t i o n through nose. Arch cannot be raised in the in- tonation of " ah ; " if paralysis is bi- lateral flapping of arch and regurgi- tation of food through nose. Regurgitation of food; nasal speech. Imperfect degluti- tion ; food gets into windpipe. Innervated by Represented The twelfth Medulla, nerve (hy- poglossal). The twelfth Medulla . The twelfth Medulla, nerve. Probably Medulla pharyngeal plexus; seventh nerve (?). As above. Medulla. The fifth Pons nerve. Glosso -pha- Medulla, ryngeal. Diseases in which Muscle is commonly In- volved. Bulbar pal- sies (acute and chron- ic) ; in spe- cific and tu- ber c ul ar diseases of base ; dys- r o p h i e s (rare). As above As above ; see also seventh nerve affec- tions. Basilar tions. affec- Bulbar affec- tions and dis- eases of the base. H THE NERVOUS DISEASES OF CHILDREN. Muscles of Tongue, Palate, and Pharynx.— Continued. Diseases in Name of Normal Symptoms of Defi- Innervated Represented which Muscle is Muscle. Function. cient Action. by in commonly In- volved. Constric- Help to push Food is swallowed P h a ryngeal Medulla Diseases of the tors of food into gul-l very imperfectly ; plexus. base (bulbar). pharynx. let. sticks in throat. Laryngeal Movements of Hoarseness and Recurrent Medulla .... Bulbar troub- muscles. vocal cords in difficulty in laryngeal les (similar res pirat io n breathing ; laryn- nerve ex- symptoms and in articu- goscopy examina- cepting the maybe caused lation. tion reveals false crico- thy- by tumors and position of vocal roid mus- foreign bodies cords (see special cle. in larynx). text-books). The movements of the tongue have been considered in the annexed table. A short frenulum may cause difficulty in articulation, but it is often suspected to be the cause in cases in which the defect is due to a distinct cerebral lesion. Sensory disturbances of the face are rare in children, but, if suspected, tests should be made carefully with cotton, with the head and point of a pin, and by application of hot and cold ob- jects. Subjective sensory disturbances (neuralgia) may occur as in adults, and will vary in the distribution according to the branches affected. (Fig. 6.) In examining- the trunk and the four extremities no fact is more important to establish than the existence of paresis, or paralysis of individual mus- cles, or of groups of muscles. To be able to do this the ex- aminer must know the action of all the more important mus- cles in health and the disturb- ances due to disease of such muscles. These are the very facts in which the student's knowledge is, as a rule, most defective. In the appended tables I shall endeavor to give the principal points in concise fashion : Fig. 6. — Sensory Chart of Face. (C. S. Freund.) INTRODUCTION— METHODS OF EXAMINATION. 15 Muscles of Head and Neck. Name of Muscle. Normal Function. Symptoms of Defi- cient Action. mastoid. S t ern o- R ai s e s and Inability to raise c 1 e i d o- turns face toj head from bed, or opposite side; other horizontal head inclines position, if both to same side; muscles are affect- if both mus- ed ; if one muscle cles act con-j is affected, no jointly head is marked change of brought for- position, unless ward. opposite . muscle is contractured ; spasm of muscle frequent ; head in- clined to one side R e c t u s To flex head. Cannot flex head 1 capitis so as to bring antic us chin on chest, major. R e c t u s To flex head, capitis anticus minor. R e c t u s 1 Slight rota Innervated by capitis lateralis. tion. Seal eni ] Elevate ribs anterior medi- u s , e t po s t e- rior. L o ngus colli. when verte- bral column is fixed ; aid in inspira- tion ; slight lateral flex- ion. Flexor of ver- t e b r a 1 col- umn. Deficient rota- tion scarcely noticeable, un- less sterno-clei- do-mastoids are diseased. Deficient inspi- ratory move- ments. Imperfect flexion of upper spine. Spinal ac- cessory. Upper cervi cal. Represented Diseases in which Muscle is commonly In- volved. Medulla and In bulbar and second and third cer- vical seg ments. Lower cervi- cal nerves. Lower cervi- cal nerves. Upper cer- vical seg ments. Lower cervi- c a 1 seg- ments. cervical cord affections ; in later stages of p r o g r essive muscular a t r o p h i es ; o c c asionally in neuritis. Diseases of the cervical region (my- elitis, men- i n g i t is, tumor ; pro- gr e s s i ve wasting of muscles. The sterno-cleido-mastoid and the other muscles of the head and neck, mentioned in these two tables, are more frequently the seat of spasm than of paralysis. The spasm may be restricted to one muscle or may involve several. It may be the result of organic disease in the medulla oblongata or spinal cord. More often it is functional in character and related to other spasmodic conditions. The same group of muscles may be involved in the ordinary rheumatic affections (torticollis, caput obstipum). 1 6 THE NERVOUS DISEASES OF CHILDREN. Muscles of Shoulders and Upper Extremity. Name of Muscle. Normal Function. Symptoms of Defi- cient Action. Innervated by Trapezius. i. CI a -J v i c u 1 ar portion (respi- ratory ; outer third of; c lavicle to occipi- tal bone). | Pulls head backward; ro-| tates slightly toward side ot muscle , so that chin is 1 turned to op- posite side ; contraction ot both clavicu- lar portions, bends head backward; slight eleva- tion of shoul- ders ; aids in deep inspira- tion. portion; der - blade ; (fro m elevation of acromion acromion and outer (clavicle goes spine of along), scapula to! ligament. | nucha and up- per dorsal spines). I 3. Low e r Adduction ol portion scapula to- Deficient back- ward movement of head; not marked as rule because deep muscles perform this function ; shoulder does not move during in- spiration. Spinal ac cessory. I Diseases in Represented , which Muscle is m ! commonly In- volved. 2. Middle Raises shoul- Acromion depress- Spinal ac- cessory nerve. and ad- ductor. R h o m boids. ward median line. Oblique move- | ment of scap- I ula from be- low, upward and inward, so that infe-i rior angle is brought near- er the medi-j an line ; hold spinal margin of scapula down to tho- rax. ed by weight ot 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 Spinal ac- Medulla MedullaProgre ssive and second muscular and third cervical segments As above. is about ten ctm distant, instead of being five or six ctm. distant from median line ; loss of adductor may be covered up by action of rhom- boids ; rounding of back. (Fig. 8.) Deep groove be- tween inner mar-j 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- imated to median line. (According to Duchenne this can be affected by upper portion of latissimus dorsi.) cessory nerve. Fifth cervi- cal. wasting ; dis- eases of me- dulla and up- per cervical cord; clavi- cular portion least fre- quently in- volved. As above. and second and third cervical segments. Fourth and As above. Fifth cervi- c al seg- ments. INTRODUCTION-— METHODS OF EXAMINATION. 17 Frontalis Facial Nerve Corrugator Supercilii Orbicular. Palpebr. Muscles of Nose Zygomatic! Orbicularis Oris Median Division of ) Facial Nerve \ Masseter Levator Menti Hypoglossal Nerve Lower Facial Nerve Platysma Myoides Hyoid Muscles Omohyoid Anterior Thoracic Sup- ) plying Pectoral Mus- v cles ) ( Region of Central ( Convolutions Speech Area Temporal Facial Trunk of Facial Nerve ( Posterior Auricular \ Nerve Facial Lower Branch Splenius Sternocleido Mastoid Spinal Accessory Nerve Levator Anguli Scapulae Trapezius Scapular Branch Circumflex Nerve PosteriorThoracicNerve Phrenic Nerve Erb's Point Brachial Plexus Fig. 7.— Motor Points of Face. (Erb.) Fig. 8.— Patient with Hypertrophy of Infra and Supra Spinati, Showing Rotation oi Right Scapula and Deep Groove along Inner Margin of Scapula (Atrophj of Rhomboids and Slight Atrophy of Lower Portion of Trapezius). 2 1 8 THE NERVOUS DISEASES OF CHILDREN. Muscles of Shoulders and Upper Extremity. — Continued. Name of Muscle. Normal Function. Symptoms of Defi- cient Action. Innervated by Levator Draws superi- Isolated a n g u 1 i or inner angle rare, scapulae. ! of scapula up- \ ward ; aids in shrugging of shoulders. Represented Diseases in which Muscle is commonly In- volved. paralysis Third and Second and D y s t r o phies Serr a t u s Rotation o t Fifth cervi cal nerves, magnus. Scapula pulled up- Poster i or ward ; lower inner thoracic angle nearer the nerve median line ; armj cannot be raised above horizontal position ; if arm is stretched forward scapula is re- moved from tho- rax ( " winged scapula ") ; dur- ing abduction of arm, scapula is moved nearer to median line, and crowds trapezius and rhomboids forward. D e 1 1 o i d To raise arm Can raise shoulder Circumflex. 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. (three di- to horizontal visions). Infraspi- 1 natus. ! Teres j minor. J Sub scap- ularis. S u praspi- natus. position, and forward, out- ward, or back- ward ; move- ments possi- ble only if s c ap u 1 a 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 angle of scapula is de- pressed. 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 S u p r , a moved outward. _ sca P^r, Difficulty in writ ing (Duchenne). Arm cannot be Subscapu- moved inward ; lar nerve, scapula is rubbed against ribs. According to Du- Sup rascap- C ircum- flex. Fourth (?) and cervical c e r v i c a 1 diseases. segments. Fifth an d Progressive sixth cervi- muscular at- c al s eg ments. rophies (dys- trophies) ; ne u r i t i s of part of the brachial plex- us; after trau- matic injuries to shoulder ; i n cervical cord affec- tions. Fourth, fifth, As above; also and sixth in Erb's form cervical of obstetrical segments. paralysis. Fourth, fifth, j and sixth cervical segments. chenne, humerus is separated still further from acro- mion, if supra- spinal is affect- ed in addition to deltoid. ular. Fourth cer- vical. As in case of deltoid. As above. INTRODUCTION- — METHODS OF EXAMINATION. 19 h U s ■ ■ ^B^ \£W • • ■ H^^^^^^^^H ■ ■ ^^r & ^^\ > WK awik 41* \ % * A - M MHr YtwlnMm^m"' *&tt^L: ■ Wk ■^ : %m m Kg M ^ ma ^ mm ^ ^^B be .s • S ss &o £ S3 to o Q '53 o a a ^ *-■ « < "55 ^3 1/5 a £ rf 2 8 S: * 10 o Sir On 20 THE NERVOUS DISEASES OF CHILDREN. Muscles of Shoulders and Upper Extremity. — Continued. Diseases in Name of Normal Symptoms of Defi- Innervated j Represented which Muscle is Muscle. Function. cient Action. by in 1 commonly In- volved. L a t i ssi- Pulls the arm, Arm cannot be S u b s c apu- Sixth a n d As in progres- mus dor- when raised, moved backward ; 1 a r , also seventh cer- sive atrophies si. downward insufficient exten- branches of vical. and dystro- and back- sion of dorsal dorsal and phies ; in cer- ward ; if arm spine ; trunk can- lumbar vico-dorsal is at rest up- not be moved lat- nerves pass- lesions; in p e r portion erally. ing through' neuritis. brings scap- muscle. u 1 a nearer the median ■ line ; united action of up- per third of both muscles causes exten- sion of dorsal trunk ; single action causes lateral move- ment of trunk. Teres ma- Rotates raised Very few symp- S u b s c apu- Seventh cer- As above. jor. humerus in- ward; adduc- tion of arm to thorax ; slight elevation of shoulder. toms ; action sup- plied by other muscles. lar. vical. Pectoralis Clavicular Imperfect adduc- Anterior Fifth, sixth, Amyotrophies major. portion de- tion of arm ; par- thoracic. and seventh and dystro- presses hu- alysis can be dis- cervical. phies, chiefly; merus from covered best by also in lesions raised posi- extending arms of brachial tion to hori- and tryingto press plexus. zontal ; ad- volar surfaces du ct i on of again s t each arm, as in other. giving a bless- ing ; sternal p o r t i on de- presses arm c o m p letely, and if arm is at rest draws acromion for- w a r d and backward. 1 1 m Absolute paralysis of both upper extremities is rare in children. If an entire extremity is paralyzed, the arm either hangs limp by the side of the body, or is flexed at the elbow. If the arm is lifted it falls by its own weight. Children sometimes refuse to make an effort, or do not understand what is wanted of them. After a few trials it is generally possible to decide whether there is a loss of power or mere lack of effort. Total paralysis of the upper extremity occurs either from disease of the brachial plexus, from spinal cord affections, or from cerebral lesions. If due to the first or second causes, the INTRODUCTION—METHODS OF EXAMINATION. 21 Fig. ii.— Young Boy with Multiple Neuritis, showing Double "Wrist Drop" and Slight " Foot Drop." Fig. 12. — Appearance of Hand in an Early Stage of Progressive Muscular Atrophy Atrophy of Abductor Brevis and Opponens Pollicis. (Duchenne. ) 22 THE NERVOUS DISEASES OF CHILDREN. paralysis is flaccid ; if due to the third cause, the paralysis is spastic, and very often part of a hemiplegia. Plexus lesions are observed in very young children (obstetrical palsies), spinal lesions in children of any age, and cere- bral lesions in children under the age of four chiefly ; but the after-effects of these conditions are often visible late in life. From the causes enumerated above, as well as in cases of neuritis and progressive muscular diseases, the paralysis may be incomplete and restricted to definite groups of muscles only. To test the extent of paralysis, the patient should be asked to perform the various movements, such as raising the shoulder, putting out arm, flexing and extending the elbow, wrist, and fingers. In very young children these tests cannot always be satisfactorily made, but even in infants much can be inferred from their power to hold or to grasp objects placed in front, above, or below them. In older children further tests can be made by offering re- sistance to active movements, and asking them to overcome it. The ability to bring the fingers in opposition to one another, to make the thumb touch the tips of the other fingers, the moving of the fingers to and from the middle one, and the ability to write, are necessary tests of the action of the intrin- sic muscles of the hand. 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. . . Extends fore- Arm cannot be ex- Musculo- Six th, s e v- ] Poliomyelitis arm ; long tended except by spiral. "enth, eighth and other head of t r i-j its own weight ; if cervical affections of ceps, and cor- long head of tri- segments. cervical aco-brachialis ceps is affec ted cord ; trau- help to keep subluxa t i o n of matic inju- head of hu- head oi humerus ries ; amyo- merus in posi- occurs easily. \ trophies tion. j and dystro- Biceps Flexion and Flexion deficient, Musculocu- Fourth, fifth, phies (tri- supination of but can be carried taneous. sixth cervi- ceps es- forearm. out in part by other muscles. cal. capes in many pe- 1 ripheral j palsies). Supinato r Flexes fore- Flexion and prona- Musculo- Fourth, fifth As "above ; in- longus arm and aids tion deficient; spiral. cervical, j volved in pe- in pronation. muscle does not stand out pro mi- ne ntl y if arm is flexed and at- tempt is made by another to extend it forcibly; if mus- cle is atrophied arm is spin d 1 e- shaped ripheral neu- ritis (trauma- tic), not in lead palsy. Supinato r S u p i n a t e s Deficient supina- Musculo- Fifth cer- Diseases as brevis. han d when tionofhand. forearm is ex- tended. spiral. vical. above ; also in peripheral palsies. INTRODUCTION— METHODS OF EXAMINATION. 23 Fig. 13.— Motor Points on Shoulder and Arm. (Bernhardt.) Supinator Longus Extensor Rad. Long- Extensor Rad. Brev Extensor Digitorum Communis Extensor Indicis' Abductor Pollicis Longu: Extensor Pollicis Brevis- Dorsal Interosseil. and II. Extensor Carpi Ulnaris Supinator Brevis Extensor Minimi Digiti Extensor Indicis Extensor Pollicis Longus Abductor Minimi Digiti Dorsal Interossei III. and IV. Fig. 14.— Motor Points of Forearm and Hand. (Erb.) 24 THE NERVOUS DISEASES OF CHILDREN. Muscles of Arm, Forearm, and Hand. — Continued. Name of Muscle. Exten s o r carpi rad i a 1 i s longus et brevis. Exten- sor carp i ulnaris. Exte nsor d i g i t o- rum com- munis. Extensor indicis. Ext en s o r minimi digiti. F 1 e x o r c a r p i| radialis. | Fie x o r c a r p ulnaris. Pal m a r is longus. F 1 e x o r di gi to- rum sub- limis. F 1 e x o r d igi to- rum pr o- fundus. Normal Function. Symptoms of Defi- cient Action. Diseases in T , „ , which Muscle is Innervated Represented commonly In . b y in volved. Extens ion Wrist cannot be'M use u 1 o- Seventh cer- As before ; es- vical. peciallyin neuritis. and abduc- tion of wrist ; the shorter muse 1 e h a s pure ex ten- sion action only. Extension and! Wrist abduction ot wrist. flexed dor sally spiral, (extended) or ab- ducted ; flattening of forearm. (Fig. ii.) As above. canno t be flexed dorsally or adducted ; " drop I wrist " is charac- I teristic of paraly- j sis of extensors. | ] j B'irst pha langes Musculo | cannot be ex- spiral. Extension of j tended nor fingers I first pha-| abducted ; grasp \ langes of all is weak because | fingers and flexor muscles are abduction. ; sho rtened and I cannot contr act J I forcibly. Flexion o f Deficient flexion. Median. wrist and pro-l nation. Flexion of Flexion and supi- Ulnar, wrist a n d| nation impaired, supination. Flexion o f Flexion impaired ; Median, wrist only. no an o m a 1 o u s position of hand from paralysis of wrist as hand falls by its own weight; the flexors of I fingers may act as substitutes. Flexes second Second ph al an x Median, phal a n x to-i cannot be flexed, ward first. Flexes last Last two phalanges Ulnar and two phalanges cannot be flexed. Median, toward first. Seventh cer- As above, vical. Seventh cer- As above, vical. Eighth cer- As above, vical. Eighth cer- As above, vical. I Eighth cer- As above, vical. Eighth cer- As above, vical. Interossei Abduction and and lum-j adduction of bricales. j fingers if first i phalanges are i extended; flexion of first ph a 1 a n g e s and siraul- tane o u s ex- tension of sec- ond and third phalanges. Fingers cannot be abducte d or ad- duc t e d ; inte r- osseous sp a c e s are very marked ; " Main en griff e " due to extension of first phalanges and flexion of second and third phalanges. (Figs. 17- 18.) Ulnar, which also sup- plies th i r d and fourth lu m b rica- les; median supplies first two and sometimes third 1 u m- bricales. Eighth vical. Eighth vical, dorsal. cer- As above; muscle should be tested with I special care in ca s e s of traumatic in- juries, cer- As above ; of- fir s tj ten the first muscles to be affected in progre s s i v e spinal atro- phies. INTRODUCTION— METHODS OF EXAMINATION. 25 Triceps (External Head Triceps (Internal Head Ulnar Nervt Flexor Carpi Ulnar: Flexor Profundus Digitorui Flexor Subl. Digitor (III & IV Flexor Subl. Digit, dnde.x and Fifth Finger) ' Palmaris Brevi: Abductor Digiti Minim; Flexor Minimi Digiti Opponens Minimi Digit Lumbricales Musculo -Cutaneous Nerve Biceps Muscle Brachialis Anticus Median Nerve jpinator Longus Pronator Teres Flexor Carpi Radialis Flexor Sublimis Digitorum Flexor Pollicis Longus Median Nerve Abductor Pollicis Brevis Opponens Pollicis Flexor Pollicis Brevis Adductor Pollicis Fig. 15.— Motor Points on Inner Surface of Arm. (Erb.) Fig. 16.— Extreme Atrophy of Thenar Muscles-" Ape Hand." (Duchenne.) 26 THE NERVOUS DISEASES OF CHILDREN. Muscles of Arm, Forearm, and Hand. — Continued. Diseases in Name of Normal Symptoms of Defi- Innervated Represented which Muscle is Muscle. Function. cient action. by in commonly In- volved. The nar Extends first Impairment of ex- M usculo- First dorsal. As before; muscles : phalanx and tension and ad- spiral. more especi- Extensor abducts meta- duction ; flatten- ally in amyo- pol 1 i c i s carpal b one ; in g of ball of trophies and brevis. acts with ad- ductor polli- cis longus. thumb. neuritis. Exte n s o r Extends both Deficient extension M usculo- First dorsal. As above. pol 1 i c i s phalanges of and ad d uc t ion ; spiral. longus. thumb ; al so adduction ot meta c a r p al bone and backward movement of second phalanx is flex e d toward first. thumb. Abductor Abduction of Deficient abduc- Musculo- First dorsal. As above. po 1 1 i c i s metacar p al tion of meta- spiral. longus. bone ; aids in flexion o t hand. carpal bo n e ; if this muscle and extenso r pollicis br e v i s are par- alyzed adduction results. Abduct o r 1 Musculo- pol 1 i c i s spiral. brevis. Opponens pollic is and outer Opposi tion No opposition Median. First dorsal. As above. of thumb. movement. portion of the flexor brevis. J Abduct o r Flex first pha- No flexion; if Median and As above. pol 1 i c i s lanx and ex- muscles are par- ulnar. br e v i s ; tend seco n d alyzed and atro- fie xo r phalanx (like phied, ape hand is brevis int e r o s s e i, formed. (Fig. 16.) and ad- also have an ductor. abduction and adduc- tion action. Flexor Fie x e s end No flexion of end Median. As above. pol 1 i c i s phalanx. phalanx. longus. INTRODUCTION— METHODS OF EXAMINATION. 2J Fig. 17. — Extreme " Main en Griffe," after Injury to Ulnar Nerve A. Subluxation of Hypertrophied Heads of Metacarpal Bones B. (Duchenne.) Fig. 18. — Slight Atrophy of Interossei, and Beginning of "Clawed Hand. (Duchenne.) THE NERVOUS DISEASES OF CHILDREN. Muscles of the Pelvic Girdle and Lower Extremities. Name of Muscle. Normal Function. Symptoms of Defi- cient Action. Diseases in Innervated Represented which Muscle is by in commonly In- volved. Glute al muscles. Gluteus maxi- mus = exten- sion of leg at hip and slight rotation out- ward. Gluteus med- ius et mini- mus = abduc- tion of leg ; if leg is ex- tended also lateral move- | ment of trunk. Outward rota- ( tion of thigh. External rotators : Pyrifo r- mis. Gemelli. Quadratus femoris. Internal obturator. Extern al obturator. Ilio-psoas. Flexion and outward rota- | tion of thigh, i Tensor Flexion and in- fasciae la-j ward rotation, toe. Adductor 1 Adduction of muscles, thigh. Inferior Glu- teal (sacral plexus). No extension of thigh ; great dif- ficulty in climb- ing; no abduction of thigh; waddling gait, exaggerated Gluteal su- movement of pel-[ perior. vis. Deficient outward 1 rotation; leg | turned inward Fi r s t a n d In progressive second sa- atr o p h i e s ; cral seg- these muscles ments. are hypertro- phied or atro- phied in vari- ous forms of dystr o phy ; myelitis of lower cord; poliomyelitis ; traumatic in- juries to cord ! and cauda equina. Fifth bar. lum- As above. | S a c r a 1 ; plexus (muscular branches). Obtura to r nerve (lum- bar plexus) Crural (lum- Fourth lum- Sartorius. Flexion of hip and knee.j ! and slight! i outward rota-i tion of thigh. | Q u a d r i - Extensor of leg ceps fe- (rectus femor-! moris. is also flexes' , hip). "1 Flexion diffi- cult; in bed bar plexus), bar. [ thigh cannot be flexed; diffi- culty risingSuperior As above, from horizontal gluteal. position. Mo addu c tion; Obtu r a to r Third lum- thigh rolls out- nerve, great bar. ward. j sciatic and crural. Flexion impaired ; Crural. Third lum- As above, acts imperfectly. bar. Leg cannot be ex- Crural, tended ; to test it ask patient, who is lying down with hip bent, to stretch out the leg ; when patient is sitting down to extend leg ; or try to flex leg while patient extends forcibly in the sitting posture with leg hanging down. Fourth lum- As above bar. very fre q u e n t in poliomyelitis. INTRODUCTION— METHODS OF EXAMINATION. 29 Sciatic Nerve Biceps Femoris (Long Head Biceps Femoris (Short Head Peroneal Nerve Gastrocnemius (Outer Head Flexor Hallucis Longus— Fig. 19. Crural Nerve Obturator Nerve Pectinus Adductor Magnus Adductor Long. Crural Nerve Vasti Interni Gluteus Maximus Adductor Magnus Semitendinosus Semimembranosus Tibial Nerve Gastrocnemius (Inner Head) Soleus Flexor Digit. Comm. Longus Tibial Nerve Tensor Fasciae latae Quadriceps Rectus Femoris Vastus Externus Fig. 20. $0 THE NERVOUS DISEASES OF CHILDREN. Muscles of the Pelvic Girdle and Lower Extremities.— Continued. Name of Muscle. Normal Function. Symptoms of Defi- cient Action. Innervated by Diseases in Represented ! which Muscle is in commonly In- volved. Biceps; Flexors of leg, Deficient flexion ; Sciatic. Semite n- and extensors action of quadri-j di'nosus ofhipinordi- ceps may cause and semi- nary walking, excessive ext e n • membra-l not in climb-i sion; in standing ; nosus. i ing stairs. I thigh is flexed to I excess; trunkl moved backward. | Gastrocne- Flexion and Deficient flexion of Internal pop- mms (al- adduction of so plan- foot ; toes tariusand point inward; soleus). first phalang- es are extend- ed; last pha- 1 an g e s in plantar flex- ion. Peron e u s Slight flexion, longus. chiefly abduc- tion of foot ; elevates outer margin of foot. foot ; heel cannot liteal. be raised ; cannot! stand on tiptoes. Fifth lum- bar. Fifth lum- bar. As before. An t e r i o r Extensors Deficient ab d u c- Peroneal, tion ; plantar arch lessened; in- creased by con- tracture. (Figs. I 21-24) J flat-foot ; I walking tiresome, of Deficient e x t e n - Anterior tib- ial. tibial foot and toes; muscles' tibialis anti- (tibialis 1 cus is also ad- ant icus,{ ductor; ex- extensor tensor digito- digito-rum in ad- rum, and ! dition to mov- exten s o r ing toes, also pollicis elevates outer longus). ridge of foot and abducts I foot. Poster i o r Adduction o f 1 tibial foo t ; outer; muscle. margin of. J foot becomes \ J convex. Peron eu s Abduction of | brevis. foot. | j Inteross ei Abduction and Abduction and ad- Poster ior pedis et adduction of duction of toes; tibial. lumbri- toes; flexors] deficient; paral- cales. of first pha- 1 ysis of interossei ; lanx ; extend hyperextension of second and first phalanges ; third phalanx. : second and third flexed (clawed foot). (Fig. 25.) Adductor ; Flex first pha- Deficient flexion of P o s t e r i o r flexor lanx of big toe toes; foot cannotj tibial brevis and extend be pushed off and ab- second. | ground easily ductor hallucis. 1 sion; "drop-foot, toes scrape floor to clear this, ex- cessive flexion at knee and hip ; contr a c t u r e of flexors and pes equinus or equi- novarus. (Figs, 11, 26, 27.) Deficient abduc- Posterior tion or adduc- tibial nerve tion; deformities result from de-| ficiencies. j Peroneal. First and second sa- cral. Fifth lum- bar, first sa- cral. Most promi- nently affect- ed in dystro- phies and in poliomyelitis. As above, but particularly in poliomyelitis ; peripheral neuritis ; fre- quent also in peroneal form of progressive muscular atrophy. First and second sa cral. First and second sa- cral. As above. INTRODUCTION — METHODS OF EXAMINATION. 3i Fig. 21. — Submetatarsal Prominence. A Lower than Normal, and Increased Plantar Arch, resulting from Contract- ure of Peroneus Longus. Fig. 22. — Exhibiting, in Addition, Promi- nence of the Tendon of the Peroneus Longus F, (Duchenne.) Fig. 23. — Plantar Surface of Same Foot, exhibit- ing Changes due to Con- tracture of Peroneus Longus, shortening of Transverse Diameter A C, and Torsion of Foot. Fig. 24. — Same Foot. Valgus Position due to Same Cause. Fig. 25. — " Clawed Foot " from Atrophy of Interossei and Other Intrinsic Muscles of Foot. (Du- chenne.) 32 THE NERVOUS DISEASES OF CHILDREN. Fig. 26. — Pes Equinus in a Boy- Five Years of Age from Atrophy of Tibialis Anticus. Fig. 27. — Pes Equinus of Eight Years Duration. Tibialis Anticus Extensor Digit. Commun. Longus. Peroneus Brevis Extensor Hallucis Longus Dorsal Interossei Peroneal Nerve Gastrocnemius (Outer Head). Peroneus Longus Flexor Hallucis Longus •*- — - — 4- Extensor Dig-it. Comm. Brev. >l===&i Abductor Minimi Digiti Fig. 28.— Motor Points. (Erb.) INTRODUCTION— METHODS OF EXAMINATION. 33 Muscles of Back and Trunk. . Diseases in Name of Normal Symptoms of Innervated Represented which Muscle is Muscle. Function. Deficient Action. by in commonly In- volved. Erector Extens i n of Lordosis of lower Dorsal S e c n d to In spinal dis- spinee: lower dorsal spine ; perpendic- nerves. 12th dorsal eases and s a c r - and lumbar ular line from segments. progr e s s i ve lumbal- vertebras. shoulder falls be- muscular at- is ; 1 n- ll i n d os sacrum; rophies. gissimus unilateral palsy dorsi. causes deflection of spine toward sound side. (Fig. 30.) Quadratus Deflects lower Lateral movements Lumbar As above. lumbo- p r ti n of of lower vertebrae nerves. rum. spine lateral- iy. Aid in expira- imperfect. Abdomin- Lordosis, with pro- Dorsal S ec n d to As above. al mus- tion ; also in trusion of nates nerves. 12th dorsal cles. voiding blad- der and strain- ing at stool : in k e e p i n g vertebras i n position. and abdomen; other actions defi- cient ; cannot straighten up f r m recumbent position without assistance of hands. segments. Serratus Magnus Obliquus Abdominis Externus Transversus Abdom inis Rect. Abdominis Fig. 29. — Motor Points of Trunk, (Bernhardt.) 34 THE NERVOUS DISEASES OF CHILDREN. Paralysis of individual muscles is not as frequent in the lower extremities as in the upper ; large groups of muscles (the anterior tibial, the posterior tibial) are often paralyzed together. Symmetrical palsies are common (in- volving thigh groups, both post-tibial groups, etc.). Complete paraplegia of the lower extremities is seen in some spinal and cerebral lesions of children (spastic birth palsies, dor- sal myelitis (poliomyelitis and my- elitis), and in multiple neuritis. Pa- ralysis may be flaccid (spinal or peripheral) or spastic (cerebral, or spinal). If spinal and spastic, lesion is in lateral column. To test the exact extent of pa- ralysis, the child is to be placed on its back ; if it does not move the limbs at all voluntarily, suspicion of very great paralysis or of exces- sive pain is a safe one. If there is total paralysis, child cannot hold the leg if it be raised a few inches ; to make further tests, see whether child can abduct, adduct, flex, or extend the various parts. Failure to abduct may be due to contracture of adductors ; so also incomplete extension of knee may be due to contracture of flexors of knee. Contractured mus- cles must be considered with par- ticular care. In very young chil- dren, if it is desirable to determine whether there is any actual paraly- sis, pinch the toe steadily until the child draws the leg away, or may be expected to do so. With older children special tests can be made. Ask child to keep thigh flexed while you oppose ; to test flexors of thigh, rest your hands on its knee and ask it to push it away by lifting up thigh ; to test extension of thigh ask it to climb on a chair or observe whether it raises hip in walking. Place the child on a table and ask it to swing leg forward and backward ; to keep leg straight while the physician endeavors to flex it (for extensors of leg), or to keep leg flexed while physician attempts to straighten leg (for flexors of leg). To test flexors or extensors of foot and toes, it should be asked to perform dorsal and plantar flexion with and without resistance. Fig. 30. — Patient showing Lordosis of Verte- bral Column, clue to Weakness of the Ex- tensors (Erector) of the Spine. INTRODUCTION— METHODS OF EXAMINATION. 35 To raise on tiptoes, or to raise toes while keeping heel on ground are good tests for the posterior and anterior tibial groups. Paralysis of certain groups is attended by contractures of others. Deformities of the feet (pes equinus, equino-varus, and valgus) result from paralysis and contractures of the flex- ors, extensors, and of the intrinsic muscles of the feet. Examination of the gait is of the utmost importance ; an attempt should be made to have the child walk ; and even if it cannot walk, the position of the legs in stand- ing, or in attempting to walk, is often quite characteristic. Cross-legged position and cross-legged progression occur in children (spastic paraplegia or diplegia) ; the gait may be paretic, spastic, ataxic, or a combined form of all three. If the gait is simply paretic the child drags its legs carefully, walking slowly and without raising the feet. The spastic gait is common in the hemiplegias and other cerebral palsies of childhood ; also in spinal palsies (myelitis and disease of the lateral columns). The knee is stiff, the leg is moved as a whole, and the child walks either on the ball of foot or on its toes. The ataxic gait, variously modified, occurs in neuritis in cerebellar disease, and in hereditary ataxy. In walking the child reels, its legs are moved extravagantly, and it walks with a broad base. A swinging gait is very frequent in poliomyelitis. The gait varies according to the muscles affected ; in dystrophies, the gait is apt to be waddling, or a mixture of the waddling and swinging gait. To be efficient, muscular action must be well directed. The contractions of muscles may be entirely normal, but unless the functions of muscles act- ing together are properly co-ordinated, the contractions may fail of their prop- er effect. In all movements (in walking, standing, writing, grasping, talk- ing), co-ordination of the muscles therein concerned is essential. We speak of inco-ordination or of ataxia, if movements go wide of the mark. If a per- son, instead of touching the end of the nose with the tip of the fingers (.eyes being closed), fumbles all over the face, he has ataxia of the upper extremities : and if instead of preserving his balance accurately in walking, a person sways from one side to the other, and to keep his balance at all walks with a broad base, that person has an ataxic gait. The ataxia may result not merely from inco-ordination of the muscles, but from insufficient sensory (muscular) im- pressions of the whereabout in space of various parts of the body. It a person is asked to cross one knee over the other (with eyes closed), he must know exactly where the other knee is, if he is to perform the movement with nicety; and he must be able to gauge the exact extent of muscular action to 36 THE NERVOUS DISEASES OF CHILDREN. be employed. With the assistance of vision muscular co-ordination is more perfect. In children ataxia is less frequent than in adults ; it occurs in mul- tiple neuritis, in hereditary ataxy, and in cerebellar disease. (In the last- named, there is considerable reeling in addition to mere ataxia ; there is inco-ordination and great uncertainty.) Before leaving the muscular system it is well to note the tonus of the muscles. In children this is of importance ; for by the mere handling of a leg the experienced physician will be able to determine whether muscles are normal, flabby, atrophied, or contractured. Every normal muscle, if struck lightly with a percussion hammer, or with a finger, will exhibit a wavelike con- traction. This mechanical excitability may be increased, so that the entire muscle contracts forcibly upon the slightest tap (tapping the quadriceps femoris may produce movement like that of the knee-jerk) ; or instead of single contractions, small fibrillary tremors may be observed on tapping atrophied or degenerated muscles. Sensory Disturbances are not easily determined in children. Many of the finer tests cannot be applied at all, for young children are not sufficiently intelligent to give reliable answers. The disturbances of sensation may be either subjective or objective. i. Subjective sensations may vary according to character of sensory perception ; thus we may have subjective sensa- tions of heat, of cold, of pressure ; if these sensations are in- tense, they may be equivalent to pain (Goldscheider) ; there may also be perverted subjective sensations, such as formi- cations and other paraesthesias (feeling as though a part were swollen, enlarged, unusually heavy and the like). Pain may vary in character (boring, cutting, thumping, burn- ing) ; its description, as Goldscheider has shown, depending very largely upon well-known sensory impressions that have been associated with pain. Distribution of pain will vary ; it may follow the distribution of nerves (neuralgia and neuritis); it may be restricted to definite areas, and such areas of pain may hold definite relations to disease in distant parts (according to Head, Dana, Goldscheider, and others). Pain persistently referred to one spot is desig- nated as Topoalgia. 2. In testing for objective disturbances of sensations, we must note that ordinary sensation is of a fourfold char- acter ; that we have perception of touch, of pain, of heat and cold, and of muscular position, and all of these may be INTRODUCTION— METHODS OF EXAMINATION. 37 equally disturbed (complete anaesthesia), or that they may be dissociated (partial anaesthesia). To test sensation at the bedside, the simpler the tests the better; the aesthesiometer can be disregarded altogether. To determine tactile sense, passing cotton over the skin is generally quite sufficient ; or still better, let the physician close his eyes and touch the patient ever so lightly ; his own perception of contact will tell whether that of the patient is above or below his own ; asking the patient to state in which direction a finger is being passed is another good test ; a correct answer implies normal tactile percep- tion at every point ; writing numbers on the skin may be tried in older children. For pain, we may test by prick- ing with a pin, by using faradic current, by pinching and the like. In all these tests the child's facial expression should be studied carefully. To determine the temperature sense it is best to employ test-tubes with hot and cold water, or to place metallic objects on the skin and see whether differ- ences in heat or cold can be made out. Goldscheider has shown that there are special areas in the skin for the per- ception of pressure, of heat and of cold ; and that the acute- ness of sensory perception varies in different areas. His test for minimum or maximum heat and cold per- ceptions are not practicable, however, at the bedside — least of all in children. The muscular sense itself is complex. It includes a per- ception of passive movements, of active movements ; a per- ception of pressure and of resistance ; and a perception of the position of parts. To test the perception of passive movements it is best to take the joint firmly between the two hands ; then move the joint slightly and ask whether patient knows what has been done ; a deficiency of this sense can be made out easily. This test is an important one, for many cases of ataxia are depend- ent upon the loss of this special perception. To test perception of weight, it is best to use rubber balls of same size, but filled with varying quantities of shot. The balls are placed on the hand, on the leg. etc.. and the patient is asked to determine which is lighter or heavier. The patient's knowledge of the position of parts is tested by placing one part (a leg. a finger), in a def- inite position, or raising it to a definite height (eyes closed) and then asking patient to put a symmetrical part in relatively the same position. Loss of sensation is termed anaesthesia; diminished sensation hyp.; 38 THE NERVOUS DISEASES OF CHILDREN. merh'anu-s Fig. 32. Fig. 33. Distribution of the Sensory Nerves. (Freund.) Figs. 31-37- INTRODUCTION— METHODS OF EXAMINATION. 39 40 THE NERVOUS DISEASES OF CHILDREN. sia ; and increased sensory perception is termed hyperesthesia. Disturb- ances of sensation may vary with the distribution of sensory nerves (Figs. 6, 31-37). Hemianesthesia implies loss of sensation in one half of the body. This is rare in children, and occurs chiefly in hysterical affections. Anoma- lies of temperature sense are observed in gliosis of cord and in some cases of neuritis. Examination of Reflexes. — We distinguish between the deep or tendon reflexes, and the superficial or cutaneous reflexes. The former con- stitute the more important class. Among the deep reflexes the patella-tendon reflex or knee-jerk has the greatest clinical significance ; next in order is the ankle clonus, or Achillis-tendon reflex, and in many cases the behavior of the wrist reflex, the triceps reflex, and even the jaw-jerk is carefully to be considered. In children it is more difficult than in adults to elicit the deep reflexes, for the former cannot relax their muscles easily, and thus inhibit to a degree every reflex movement. In testing for the knee-jerks and for other reflexes, it is best to take the child unawares, before it has learnt to know what the test means. While busying one's self with other parts of the body, or while talking to the child, if the leg is in a favorable position, strike the tendon smartly, and the reflex movement will ensue if the conditions are normal. If this first test does not succeed, seat the child at the edge of a table with its legs dangling, and then quickly strike the tendon. If the child continues to inhibit the reflex, I am in the habit of placing my left hand be- tween the crossed knees of the child in such a way as lightly to grasp the posterior surface of the upper leg. The child is compelled to relax its muscles, and I can easily tell whether it makes the slightest effort to contract them. If the tendon is struck, the excursion of the leg is bound to follow, provided the reflex is present. In older children Jendrassik's method (clinching the fists while the test is made) may be employed ; but it is still better for the physician to ask the child to squeeze his fingers the very in- stant he strikes the tendon. To elicit the ankle clonus, it is best to perform sudden dorsal flexion of the foot, while keeping the knee resting on the other hand and in the position of moderate flexion. The same clonic movements can be elicited by striking the Achillis tendon while the foot is in moderate dorsal flexion. The paradoxical muscular contraction described by C. Westphal — a tonic contraction of a muscle when suddenly relaxed — is a mere curiosity without any known clinical significance. The wrist reflex is tested by striking the radial side of the forearm near the wrist ; a smart tap will cause contraction of the supinator and biceps muscles. The triceps reflex is elicited by striking the tendon while the arm is flexed. The " jaw jerk," a reflex contraction of the masseter, is brought about by striking the middle of the chin when the mouth is slightly open, or by tapping a pencil laid on the lower teeth or jaw. I have seen this distinct- ly present in a case of multiple sclerosis in a child. The power of inhibition is not so great over the other reflexes as over the knee-jerks ; and in testing for the wrist INTRODUCTION— METHODS OF EXAMINATION 4 1 or triceps reflexes, the most important point is to place the joints in a position of moderate flexion so as to give the muscles the fairest chance for contraction. The deep re- flexes may be present, diminished or exaggerated. The knee-jerk is present under ordinary conditions ; the mere presence of the ankle clonus is abnormal ; and the reflexes in the upper extremity are present in some and absent in other normal individuals. The reflexes are diminished or absent in peripheral nerve disease, in spinal-cord affections involving the pos- terior columns and the gray matter of the cord ; in disease of the cerebellum. They are exaggerated in diseases of the brain and in diseases involving the lateral columns of the cord ; also in some functional diseases. The diminution of the reflex is determined easily enough ; but the absence of the reflex is more significant than a mere diminution. The diminution or absence of a reflex may be apparent only. Thus the knee-jerk may be wanting in con- sequence of contraction of the posterior flexors of the leg, or there may be anchylosis of the joint preventing free movement ; but in such cases the contraction of the quadri- ceps can be seen or felt upon tapping the tendon. In some children the tendon is displaced or so imbedded in fat that it cannot be struck readily. It is more difficult to state when a reflex is exaggerated. In this matter comparison based upon experience is the best guide. We can safely speak of an exaggerated re- flex if there is an exceeding quickness of the response, if the slightest tapping produces a liberal movement ; or if clonic movement results from a single blow (as in patella clonus). If the reflex can be elicited indirectly by striking the finger placed over the tendon, the reflex is surely exag- gerated. Moreover, if a child of three years or more is so seated that its feet are in solid contact with the floor, and if when its patella tendon is struck, the foot is lifted from the floor, the reflex is considerably increased. Very lively knee-jerks do not necessarily imply organic disease, but exaggerated knee-jerks with ankle clonus are rarely due to functional disease. Exaggeration or absence of the reflexes in one half of the body is always suspicious 42 THE NERVOUS DISEASES OF CHILDREN. of organic disease, and so is the association of increased mechanical excitability and of contractures with increase of the reflexes. The superficial or cutaneous reflexes are not of great importance in child- hood. The abdominal, epigastric, and cremasteric reflexes are but poorly developed in children ; the last named is absent in many older children ; and no significance can be attached to its presence or absence unless the con- dition is not the same in both halves of the body. The plantar reflex (re- traction of the foot on irritation of the soles) deserves a few words of comment. Like the other cutaneous reflexes it is absent in diseases of the peripheral nerves, and in diseases of the spinal cord involving the course of the reflex arc ; if the lesion is higher than the level through which the arc passes, the reflex may be exaggerated, but this is not as constant a feature as is the case with the tendon reflexes. In all unilateral brain-lesions the cutaneous reflexes are absent as a rule on the paralyzed side. The plantar reflex is absent in condition of unconsciousness (as w r ell as in deep sleep), and its presence or absence may give a clew as to the degree of coma in a given case. ELECTRICAL EXAMINATION. The distribution of a paralysis, the flaccid or spastic con- dition of the muscles, the behavior of the reflexes, may enable us to make an accurate diagnosis in many cases, but in many more the diagnosis cannot, be safely established until a careful electrical examination has been made.* The chief object of such an examination is to determine the response of various muscles and nerves to the faradic and galvanic currents, and to compare such response with the conditions obtaining during health. The younger Westphal has shown that the nerves and * The author cannot undertake to give a detailed account of medical elec- tricity ; for this the student is referred to the treatises of de Watteville, of Beard and Rockwell, of Erb, Bernhardt or Lewandowski ; also to the special chapters in the text- books of Gray, Dana, and Gowers. The importance of a thorough knowledge of elec- tricity should not be overlooked. Whatever one may think of the value of electricity as a therapeutic agent, there can be no question of its great merit as an aid to diag- nosis. It is disappointing to find so good an observer as Moebius disputing the value of electricity in diagnosis. (Neurologische Beitrage, i., pp. 97-100.) Moebius argues that the experienced neurologist can make a diagnosis without the assistance of elec- trical tests, and that such tests are not wholly reliable. But they are reliable in fully ninety-five per cent, of all cases, and as for making a diagnosis without the aid of elec- tricity, that may be possible. A clever neurologist can also diagnosticate a cere- bral tumor often enough without the ophthalmoscope, yet he will not spurn the cor- roborative evidence which an examination of the papillae may furnish. INTRODUCTION— METHODS OF EXAMINATION. 43 muscles of the new-born, and of children up to the age of live weeks, do not respond except to very strong currents, and even then the contractions are slow. But after that age the normal nerve and muscle will respond to faradic and galvanic stimulation. A knowledge of the position of the motor points is essential. (See Figs. 7, 13, 14, 15, 19, 20, 28, 29.) Always begin the examination with the faradic current, and apply the one pole to the nerve or muscle to be examined, and place the other pole at a safe distance, say on the sternum. Use mild currents (children grow very restless under strong currents), and determine the weakest current with which a contraction is to be obtained, or whether any contraction can be obtained at all, or not. Place the arm or leg in a position favorable for contraction of the muscles to be tested, and make sure that the contraction which en- sues is the one that should naturally follow. Do not, for instance, claim that the electrical conditions are normal if on applying an electrode to the ex- tensor digitorum communis, a flexion contraction of wrist and fingers ensues. And if there is any doubt as to the strength of the current, let the physician apply it to himself ; he will be more certain to be merciful if he does ; or if there is a doubt whether a nerve or muscle should respond to a given strength of current, let that same current be applied to the same point in the opposite half of the body. Thus we can compare two peronei with each other ; or we can compare a peroneal nerve with a facial or a median nerve of the same or opposite side of the body,* or we can compare the degree of excitability with the figures given in tabular form by Stintzing for the minimum and maximum currents required to produce contractions of various nerves and muscles.! A simple diminution of faradic and galvanic response of nerves and mus- cle occurs in light cases of peripheral neuritis, in hysterical conditions, in atrophies following joint disease or disuse, and in many of the primary dys- trophies. A simple and continued increase of response is observed practi- cally only in cases of tetany. But in addition to mere quantitative changes there are also changes in the character and quality of the response to be ob- served in cases of degenerative disease of the peripheral nerves, of the mus- cles, and of parts of the spinal cord. The changes implying degenerative disease are referred to as the reaction of degeneration. * In children the motor points may be obscured by deposits of fat. t American faradic batteries are now provided with a sliding scale which enables the physician to record how far apart the primary and secondary coils wore when first con- traction was obtained. In recording the effect of the galvanic current, the strength should be stated in milliamperes. 44 THE NERVOUS DISEASES OF CHILDREN. The following table will give the salient features of nor- mal electrical conditions and of the reaction of degenera- tion : Normal Electrical Coxditioxs. Reactiox of Degexeratiox. Nerve and Muscle. Nerve. Muscle. Faradic cur- Contractions good rent. prompt. No response No response (except in partial (except in R. D.). partial R. j D.L Galvanic Contractions prompt and No response, current. quick. Order of i. K. C C* contrac- 2. A. C C. / interchange- 3. A. O. C \ able. 4. K. O. C. 5. K. C Te. (rare). Increased excitablity at first, then diminished ; contrac- tions sluggish. A.C.OK.C.C. With stronger currents. 1. A. C C. 2. K. C. C 3 A. O. C 4. K. O. C or A. C. C. = K. C C. * K. C C = Kathodal closure contraction ; A. C C = Anodal closure contrac- tion ; A. O. C = anodal opening contraction ; K. O. C. = Kathodal opening con- traction ; K. C Te. = Kathodal closure Tetanus. The preceding table states that in the reaction of degeneration (R. D.) the nerves and muscles fail to respond to the faradic current, but that the muscles continue to respond to the galvanic current in altered fashion ; first of all, the contractions are sluggish, and this is the most important point, and secondly, the order of contractions is reversed ; the anodal closure contrac- tion can be obtained with weaker currents than are required for the kathodal closure contraction. The mistake is commonly made to suppose that there is no reaction of degeneration present unless the galvanic order of contractions is reversed ; this occurs in the majority of typical cases, to be sure ; but the failure of response to the faradic current is early evidence of a reaction of degener- ation, and often precedes the development of abnormal gal- vanic conditions. In first examinations the faradic test is therefore much the more important. Many recent authors (including Remak) agree further- more in considering the sluggishness of contraction much more significant of degenerative changes than the reversal of the normal formula. INTRODUCTION— METHODS OF EXAMINATION. 45 It should be remembered also that muscles may be so much atrophied as to give no contraction to the strongest galvanic current. A partial reaction of degeneration is often overlooked ; it implies a mere diminution of faradic excitability of nerve and muscles, but the response of the muscle on direct galvanic excitation is slow, and the formula may be re- versed. Even if the faradic response is present, it is important to make sure that the galvanic response is prompt, not sluggish. The greatest service which electricity affords us in diag- nosis is in differentiating between cerebral diseases on the one hand and certain spinal and peripheral diseases on the other. The ganglion cell of the anterior horn and its ana- logue in the cranial nerve nuclei are responsible for the nor- mal electrical condition of peripheral nerves and muscles. Lesions involving this cell, or interfering with the transmis- sion of impulses from it to the periphery are attended by changes in electrical reactions; in all other diseases the electrical conditions are practically unaltered. Whence it follows that the electrical conditions are : Normal in i. All cerebral diseases,* excepting those of cranial nerve nuclei. 2. Diseases of lateral and posterior col- umns of spinal cord. 3. Functional troubles. 4. Mild peripheral troubles. 5. In some forms of muscular dystrophy. Distinctly Altered (R. D.) in 1. Bulbar paralysis (acute and chronic). 2. Polioencephalitis superior (Wernicke). 3. Poliomyelitis (acute and chronic). 4. Progressive amyotrophies. 5. Amyotrophic lateral sclerosis. 6. Myelitis, but only in muscles repre- sented in diseased level. 7. Gliosis and tumor involving gray mat- ter. 8. Anterior root disease (syphilis, tumor, etc.) 9. Vertebral disease (Pott's disease, tu- mor). 10. Peripheral neuritis (traumatic, rheu- matic, toxic). 11. In some forms of muscular dystrophy. A very few exceptions have been reported. To complete the examination, be sure to look for trophic symptoms which occur in many functional disor- ders as well as in disease of the peripheral nerves and in some diseases of the spinal cord (gliosis, tumor); and finally the conditions of the vesical and rectal reflexes should be 46 THE NERVOUS DISEASES OF CHILDREN. recorded ; though in regard to them the influence or lack of previous training of the child must be taken into ac- count. A sudden loss of control is often associated with serious cerebral and spinal disease. , BIBLIOGRAPHY.* Dana : Text-book of Nervous Diseases, 1893. Duchenne : Physiologie des Mouvements. Paris, 1866. German translation by C. Wernicke, Berlin, 1885. Fisher and Peterson : Cranial Measurements in Twenty Cases of Infantile Cerebral Hemiplegia. New York Medical Journal, April 6, 1889. Goldscheider : Diagnostik der Nervenkrankheiten, Berlin, 1893, and Ueber den Smerz, Berlin, 1894. Gowers: Diseases of the Nervous System. Second American edition. 1892. Gray : Nervous and Mental Diseases, 1893. Moebius : Diagnostik der Nervenkrankheiten, Leipzig. Second edition. 1894. Neurologische Beitrage, Heft I., 1894. Oppenheim, H. : Lehrbuch der Nervenkrankheiten. Berlin, 1894. Oppenheimer, Z. : Schmerz und Temperatur Empfindung, Berlin, 1893. (On Pain and Temperature Sense.) Remak : Deutsche Medicinische Wochenschrift, November 16, 1893. Preyer : Die Seele des Kindes. Third edition. Berlin, 1890. Sternberg: Die Sehnenreflexe. Leipzig, 1893. Stintzing : Deutsches Archiv f. klin. Medicin, vol. xxxix., p. 76. Westphal, A. : Archiv fiir Psychiatrie, vol. xxvi., p. i., 1894. * This list is necessarily incomplete ; it is intended to give the more recent works and articles chiefly which have been especially consulted in the preparation of this chapter. PART I. GENERAL NERVOUS DISEASES. CHAPTER II. CONVULSIONS— ECLAMPSIA INFANTUM. The peculiar seizures so common in ii fancy, and desig- nated as convulsions, constitute a symptom, not a form, of disease. The unusual frequency of convulsions in early childhood points to the greater excitability in the child of the motor mechanism of the brain. The motor centres in the cortex are more apt to " discharge," and the inhibitory power of the brain is less developed than in the adult. Eclampsia has been found to be a convenient term for the condition in which convulsions are apt to occur, but it shoald be remembered that it is neither a disease nor a sufficient diagnosis in any given case, for eclampsia in children, as well as in adults, may be the result of many widely different disorders. However frequent these convulsive seizures may be in early life, healthy children are as exempt from them as are healthy adults. If they are not the result of organic disease of the brain, their occurrence indicates functional involve- ment of the brain in connection with disease in other parts of the body. A convulsion is a motor discharge resulting in muscular contractions of one or more parts of the body. In the major- ity of cases these convulsive movements are associated with a number of other symptoms ; viz., an initial cry, turning of the eyes upward or inward, very sudden loss of conscious- ness, involuntary passage of urine and faeces, prolonged drowsiness, and a condition of stupor if not of coma. The muscular movements may be tonic at first, but are apt to lead to clonic spasms. No two convulsive seizures are exactly alike, but the student who has seen a single seizure will not forget the main features of the condition. The 4 50 THE NERVOUS DISEASES OF CHILDREN. convulsion may come on without warning, or the child may have complained of uncomfortable sensations in the head or stomach, of a little dizziness or nausea; the child gives a shrill cry and at once is thrown into tonic and clonic con- vulsions. These may be partial or general, unilateral or bilateral. The legs are stiff, the arms bend ^it the elbow, the wrists are flexed, and the hands clenched, with the fingers firmly closed upon the thumb. The head is thrown back or rotated to one side ; the back may be arched, the face is pale at first, but soon reddens and the eyes remain wide open. The pupils do not react. In older children the tongue may be caught between the teeth, and bloody Fig. 38. — Child of One Year Photographed During a Severe Convulsive Seizure. froth may be noticeable at the lips. In very young chil- dren Ave have the ordinary foam at the mouth. The respi- ratory muscles are in a condition of spasm ; the child often turns blue rapidly and is in danger of asphyxia. Fortu- nately the tonic spasm soon ceases, slighter clonic convul- sions then set in, and after a little the child groAvs quiet, and is apt to be in a dazed or stuporous condition for a period that may vary from several minutes to as many hours or days. While the clonic spasm lasts there is im- minent danger of injury to the child's head and limbs in consequence of the severe jactations, but this danger is not as serious as that from asphyxia during the period of tonic spasm. Causes. — Convulsions always denote " cerebral (cortical) irritation." It might be sufficient to state that any morbid CONVULSIONS— ECLAMPSIA INFANTUM. 5 1 process which brings about cortical irritation, directly or indirectly, is liable to cause convulsions. But it will be better to enumerate the chief conditions under which in- fantile convulsions occur. I. Convulsions occurring within the first few days of life are, as a rule, the result of meningeal hemorrhage, due either to protracted labor, or to instrumental delivery. If the child survives, the injury done to the brain often leads to the development of spastic palsies. (Cf. chapter on Cer- ebral Palsies.) Ii. The convulsions may be due to organic disease of the brain, such as tumor, abscess, meningitis, or vascular lesions ; in all of these cases they may be the earliest symptom of the disease, but other symptoms associated with them, such as headache, paralysis, optic neuritis, and the like, will be forthcoming. III. They may mark the onset of any acute infectious disease. The initial convulsion is almost as frequent in children as is the initial rigor in the adult. The former is the infantile equivalent of the latter. It is extremely com- mon at the onset of pneumonia, scarlet fever, and measles ; and I have also witnessed convulsions in malarial fevers, and at the onset of chicken-pox. The general practitioner is very apt to err in the interpretation of such convulsions. A child that has had a convulsion, preceded or followed by a rise of temperature, is often suspected to be in the first stages of a meningitis or of some other cerebral disease. While this is true in some instances, the fact is entirely overlooked that the seizure may be the initial symptom not only of any of the ordinary infectious diseases, but of the acute spinal diseases as well — poliomyelitis, for instance (probably an infectious disease). IV. The convulsions may be of reflex origin. Almost every possible peripheral disturbance has been supposed bv one author or the other to be a sufficient cause of convul- sions. I need merely mention ocular insufficiencies, nasal obstruction, narrow prepuces, not to call up a dozen or more about which there has been much, and often bitter, discussion. Of the influence of two conditions there can be no reasonable doubt. The first of these is dentition ; the 52 THE NERVOUS DISEASES OF CHILDREN. second, gastrointestinal irritation. There has been some question whether a tooth about to cut through the gum can cause an eclamptic seizure. Delayed dentition is so fre- quently associated with rickets that the latter condition is considered by many to be the chief factor, and this I be- lieve to be true ; but convulsions do at times occur in chil- dren who present no tangible signs of rickets. Moreover, there is good reason why a cutting tooth should produce convulsions in a child so disposed, say by rickets, or any form of exhausting disease. " The cutting tooth " is a direct irritant to the filaments of the trigeminal nerve, which car- ries this irritation easily enough to the convulsive centres at the base of the brain. I have little doubt of this special reflex origin of convulsive seizures since observing in the adult the occurrence of severe epileptic paroxysms in a case of trigeminal neuralgia. The influence of gastro-intestinal irritation is well illus- trated by the convulsions occurring in the course of an acute or chronic intestinal catarrh, in the ordinary summer diarrhoea of young children, or with the exhausting chronic diarrhoea in older children. The same phenomena are apt to occur in the presence of animal parasites (pin-worms, and, above all, tape-worms), and I have known severe con- vulsions to disappear promptly after the removal of a tape- worm in children between the ages of four and eight years never to return again. V. Convulsions may be due to poisons (organic or me- tallic) circulating in the blood. Under this heading we may include the convulsions of uraemia, those due to the presence of ptomaines in the stomach and bowels, and con- vulsions produced by the administration of lead and other metallic poisons. Toxsemic convulsions are not as common in the child as in the adult, unless, following recent studies by Chenbach and others, we consider the convulsions of in- fectious fevers and of intestinal troubles to be due to some form of auto-intoxication. VI. Convulsions may result from severe loss of blood, from any exhausting disease, or from such constitutional disturbances as scrofula and rickets. A vast literature has grown up on the subject of rickets and convulsions. What- CONVULSIONS— ECLAMPSIA INFANTUM. 53 ever theory one may be willing to adopt in order to ex- plain this close relationship, the fact remains that an enor- mous percentage of children having convulsions are af- fected with rickets. An hyperasmic condition of the cortex analogous to the condition of the bones in rickets, is the only explanation we can give of the frequency of convulsions during rickets, unless we are satisfied to accept the very vague statement that the latter is a simple predisposing cause of convul- sions. VII. A convulsion may be idiopathic, hereditary, or, if you choose, the first incident in the course of an ordinary epilepsy. This possibility must be kept in mind, but it is a more probable explanation if the child happens to be above, rather than under, four or six years of age. This inter- pretation of a convulsive seizure occurring in a child should be given only after excluding every other possible cause. It is surprising, but a fact nevertheless, to which I have called attention a number of times, that the more carefully we scrutinize cases of convulsions, or of epilepsy for that matter, the fewer of them appear to be truly idiopathic. It will be well not to make the diagnosis of an incipient epi- lepsy unless we are informed that the child has had previous convulsions at somewhat long intervals, or unless we have occasion to observe subsequent convulsions without a dis- tinct additional cause for each seizure. VIII. Convulsions occur after traumatic injury. In some cases actual hemorrhage over the motor centres is the im- mediate cause of the convulsions. In other cases no tan- gible injury to the brain has resulted, and we are therefore compelled to regard the fact as the expression of shock. Theories. — Many theories have been advanced to ex- plain the occurrence of convulsions. No theory is entirelv satisfactory ; but by physiological experiments and the ex- periment of disease we have learned to know the conditions under which convulsions are most apt to occur. There are, first of all, the time-honored experiments of Kussmaul and Tenner, showing that convulsions occur in a rabbit after suddenly tying the cervical arteries and thus cutting off the blood-supply. Their conclusions are op- 54 THE NERVOUS DISEASES OF CHILDREN. posed to the older theories that these seizures were due to an hyperasmic condition of the brain. The question arises whether the sudden withdrawal of blood deprives parts of the brain of their functions, or whether it simply acts as an irritant to the cortical centres. The latter explanation seems plausible, in view of the experiments of Hitzig, Fer- rier, Horsley and others. A mechanical irritation of the pons (floor of the fourth ventricle), as proved by Nothnagel, also produces convulsions. It is evident that toxic agents have the same effect as the electrical or mechanical irrita- tion just referred to, else we could not explain the convul- sions due to toxic poisons, those of uraemia, for instance, and those due to asphyxia from the accumulation of car- bonic acid in the blood. Every morbid process in the motor areas of the cortex, if not absolutely destructive, is apt to cause convulsions ; but what is the relation between the convulsive centres in the cortex and Nothnagel's epileptic centre in the pons? Does the cortex contain an actual convulsive centre? If you irritate the lower epileptic centre in an animal whose hemispheres have been divided from the rest of the brain you will get convulsive spasms of the entire body. The lower centres have the power, therefore, of " starting " con- vulsive seizures, and it is probable that they are under higher control in the fully developed brain, and as long as normal conditions exist. It is this power to control, this inhibitory force, that resides in higher centres. The re- moval of this inhibition through disease of such centres liberates the energy of the lower centres.* Age and Frequency. — By far the largest majority of convulsions occur during the first two years of life. The Philadelphia Health Reports, as condensed by Lewis, give ample proof of this. Seven thousand five hundred and eight deaths among * Neurologists, the world over, have adopted Hughlings Jackson's views without en- dorsing every detail of his theories. Jackson considers the central nervous system to be made up of three tiers of sensory-motor centres. All parts of the body are repre- sented in each tier, but the higher centre controls one or both below it, and has the power to inhibit the discharges of a lower centre. In a young child the higher tiers do not exert a controlling influence, since they are imperfectly developed ; hence the dis- charges are so much more frequent than in later years. C ONVUL SIONS—E CL A MP SI A INF A NTUM. 5. minors (during period of 1 876-1 883 inclusive) were due to convulsions and laryngismus stridulus. Of these were : Under 1 year. 1 to 2 years. 2 to 5 years. 5 to 10 years. 10 to 15 years. 15 to 20 years. Cases of convulsions .... Cases of laryngismus stridulus 4,993 24 i,335 7 893 9 178 5 38 26 Total 5, OI 7 i,342 902 183 38 26 While the statistics illustrate a general truth, some com- ment is necessary. First, convulsions occurring at this early age are not necessarily fatal, although those occur- ring in the first few days after birth are much oftener fatal than those occurring later. The brain has less power of re- sistance, and the processes giving rise to these early con- vulsions are a more serious menace to the life of the child. Secondly. It should not be forgotten that convulsions are so much more frequent during the first two years of life because the diseases and conditions causing convulsions are most frequent at this period. We must take into account the traumatic injury to the brain during labor, defective brain development, dentition, the acute infectious diseases, and almost all the infantile cerebral palsies and gastro-intestinal disorders. Convulsions may, however, occur at any age ; but since the child's brain grows less irritable the older it grows, a convulsion occurring later in life often signifies more serious trouble. The frequency of convulsive attacks in any given case will also depend upon the nature of the disease, of which it is merely a symptom. In the case of convulsions due to dentition, a convulsion or a series of convulsions may occur with the cutting of each tooth ; if due to gastro-intestinal irritation, convulsions may recur until the condition is im- proved or relieved. In acute infectious diseases we are apt to have but one initial convulsion, or at most two or three distinct attacks; if there were more than a few con- vulsions during an attack of an acute infectious disease. 1 should suspect some cerebral complication. It is a serious fact, and one well worth remembering, that 56 THE NERVOUS DISEASES OE CHILDREN. the initial convulsion is as little apt to be repeated as is the initial rigor. The first toxic invasion, and not the fever, seems to cause the convulsions. I have often observed convulsions with relatively low temperatures (103 F.) at the onset, without any repetition of the same during the course of an acute disease, even though the temperature reach 105 F. and over. I do not mean to deny, however, that high fever alone may cause convulsions, but it is not a common cause. I have often had occasion to observe that repeated con- vulsions are characteristic of cortical disease, and this is borne out by the cerebral diseases of childhood. In the majority of such cases the convulsions will be but one of a series of symptoms ; and if convulsions occur re- peatedly without any further evidences of cerebral disease, the suspicion of true epilepsy must be entertained. Symptoms. — It may seem odd that we should treat of the symptomatology of convulsions, which we have termed a symptom and not a disease, but the sequence of events during a convulsion is subject to great variations, and on this head there is need of further remark. A con- vulsion may be partial or general. Partial convulsions in the majority of the cases denote organic disease of the brain, and in this sense may be more serious than general convul- sions ; a slight convulsive twitching of a thumb may seem a small matter indeed, but it may be the first sign of a cere- bral tumor which is bound to be fatal before long ; while, as regards danger to life, a severe general convulsion may be entirely harmless. Partial convulsions are often unilateral, and sometimes limited to a single member. The cortical centre of the part convulsed is the chief site of the lesion. Partial convulsions often become general ; it is of importance, therefore, to inquire whether convulsions that affect all parts of the body start in any one particular member, or whether the person who is subject to general convulsions ever had unilateral partial convulsions. All the phenomena of a convulsive seizure need not to be exhibited in each attack. We may have convulsive twitch- ings without loss of consciousness, and loss of conscious- ness without twitching movements. I have clearly in mind a child about three months old, whom I had reason to see CONVULSIONS— ECLAMPSIA INFANTUM. S7 some nine years ago. Without any known cause the child was in a " faint spell," during which time the skin was pale, and the pulse became slow and irregular. Such a spell oc- curred about the same hour on successive afternoons, and later on the child had several each day. No other symptom could be made out, with the exception of a distinct enlarge- ment of the spleen. The attacks were evidently malarial and yielded quickly to large doses of quinine. The child has had no convulsions since. There is some difference of opinion among authors as to whether a convulsive attack begins with clonic or tonic movements, and which preponderate. It is well to know that, according to the researches of Unverricht and others, tonic and clonic convulsions are different in degree and not in kind — a tonic convulsion being practically a rapid suc- cession of clonic movements. In children tonic spasm is supposed to precede clonic spasm, and in this respect to differ from the epileptic paroxysm of later life ; but the dis- tinction is of no importance, and, moreover, I have seen many spasms in children which were purely clonic from beginning to end. One special form of convulsive seizure in children de- serves special mention. This is Laryngismus Stridulus. — Cerebral croup, inward con- vulsions, child-crowing, etc. The many synonyms are in- dicative of the frequency of this condition, which occurs more often in European countries than here, owing prob- ably to the greater spread of severe forms of rickets in England and the Continent. In its simpler form laryngismus stridulus implies merely a spasm of the glottis. A child that may have exhibited an occasional crowing, croaking noise, is seized quite suddenly with severe spasm of the adductor muscles ; the body be- comes rigid, the head is drawn back, the face grows pale and then livid. In a few seconds the spasm relaxes, a deep inspiration follows with a hissing sound, and all is over for the time being, except that the child presents a haggard, tired look. Several such spasms may follow quickly upon one another. After the attack is over the child often vomits, is badly frightened, and from sheer fatisrue falls 58 THE NERVOUS DISEASES OF CHILDREN-. into a more or less natural sleep. It is not rare to have as many as twenty and more of such attacks during the course of twenty-four hours ; in other cases single attacks follow each other at much longer intervals. It is quite excep- tional, however, to have but a single spasmodic attack. Other convulsive actions are apt to be associated with the laryngeal spasm. The diaphragm and other respiratory muscles are often convulsed ; the fingers are firmly clenched, and the toes are apt to be flexed under the feet (" carpo- pedal " spasm) ; general convulsive movements, and a total loss of consciousness may be developed before the seizure is over, proving the close relationship between the laryn- geal spasm and general convulsions." Causation. — There was much dispute among the older authors as to the true cause of laryngeal spasm. An en- larged thymus gland pressing upon branches of the vagus was supposed to be the most frequent cause; but this has been entirely disproven by the autopsies of Henoch and others, which showed that there was no correspondence between the state of the thymus and the occurrence of " in- ternal convulsions." An overpowering weight of opinion is in favor of a close causal relationship between rickets and laryngismus stridulus. The researches of Elsasser, of West, Gee, Gay, Henoch, Jacobi, Heubner, and many others have offered substantial proof of this position. Of late there has been a disposition to discredit this theory. Loos has denied the influence of rickets, while endeavoring to establish a relation between this convulsive disorder and tetany. To my mind the only proper relationship between the two is that they are both due, safely enough, to the in- fluence of rickets. Loos has been severely criticised, and, in fact, has been disproven, by Kassowitz, whose large ex- perience of rickets has stood him in good stead. Rachitic softening of the skull (craniotabes) has been supposed by Jacobi and others to be the active cause of laryngeal spasm. That the association of these conditions is unusually frequent cannot be doubted. Kassowitz states that only 48 of 370 cases of laryngeal spasm did not present * Children suffering from laryngismus stridulus are prone to the ordinary eclamptic fits, thus furnishing / vrurther evidence of the close relationship between the two conditions. i CONVULSIONS— ECLAMPSIA INFANTUM. 59 marked craniotabes ; but of these 48 cases 47 presented some other decided symptom of rickets. The fontaneiles were closed in only 4 of these 370 cases, although no of these children were over one year of age. When rickets is not present the fontanelle should be closed at the end of the first year (Kassowitz). But is a softened skull the direct cause of laryngeal spasm ? Scarcely. The centres for vocal movements are far away from that part of the brain which is apt to be compressed in cases of craniotabes. The hyperaemic condi- tion of the brain in rickets is of a piece with the hyperaemic condition of other structures and it is this hyperemia which causes an unusual irritability of the centres which would not under ordinary circumstances " discharge" upon the slightest peripheral irritation (cold air, dentition, slight gastro- disturbances). Indeed there may be spontaneous discharge of these centres without peripheral irritation. Laryngeal spasms occur most frequently between the sixth and eighteenth months, the very period during which rickets is most marked, if present, though it occurs often enough up to the age of three years and later. Patients sub- ject to attacks are most apt to suffer from them during the coldest months of the year, viz., January, February, and March. Much has been made of the supposed relation between laryngismus stridulus and tetany. Cheadle believes the two to be identical. Facial contractility may be present in cases of laryngismus, but cases of laryngismus in which the Trousseau symptom (spasm induced by compression of the artery in a limb) can be elicited during the interval be- tween attacks are surely rare. There is another form of convulsive seizure in children which reminds one of laryngismus. Not long ago a child, aged eighteen months, apparently healthy, was brought to me that would hold its breath in many a crying spell ; after holding its breath for a few seconds it would turn blue, and its head would drop forward as it lost consciousness. After a few more seconds the child recovered consciousness, and all was well again until the next crying spell, when the same sequence of symptoms would be apt to occur. Treat- 60 THE NERVOUS DISEASES OF CHILDREN. ment by bromides and small doses of chloral put an end to these attacks in a few weeks. Diagnosis of Convulsions. — It is scarcely credible that any one who has ever seen a convulsive seizure can mistake it for anything else. And yet it has happened within the author's experience that he was called to a case of supposed tetany, which was, however, nothing more than an ordinary convulsive attack. The difficulties of diagnosis that arise are concerned al- together with the possible etiological factor in a given case. The physician who witnesses a convulsive seizure will do well to be reserved in giving an explanation of the origin of such seizure until he has carefully examined into the previous history of the case ; and if he does this he will be able to say with great certainty whether the convulsion is due to some reflex irritation, to some cerebral or spinal dis- ease, or whether it is the initial -symptom of an acute in- fectious disorder. It is of the greatest practical importance, however, to be able to say distinctly whether the convulsion is the expression of functional disease or of organic disease of the brain. General convulsions are, in the vast majority of cases, of functional origin. Partial convulsions are, as a rule, the result of organic disease ; but a child that has gen- eral convulsions may at some previous stage of its history have exhibited partial seizures, or a general convulsion may have begun in a strictly localized fashion. The part first convulsed, or the part alone convulsed, indicates that the centre in the brain which governs the movements of this special part of the body is the chief site of the lesion. A twitching movement of the thumb, so slight that many might be tempted to regard it as a trifling nervousness, indi- cates disease in or near the centre governing the movement of the thumb. A convulsive seizure, beginning with a twitching of the eyelid, with the drawing up of one corner of the mouth, is certain to be the result of disease in the respective centres of these parts. The gravity of a convulsive seizure will depend very much upon the cause of the attack. In general terms a par- tial epilepsy pointing to organic lesions of the brain is a more serious matter than a general epilepsy, which may be func- CONVULSIONS— ECLAMPSIA INFANTUM. 6 1 tional ; but if a general convulsion is the expression of a typical epilepsy about to be developed, its importance is as grave as though it were due to gross disease of the brain. Convulsions occurring at the onset of acute diseases are much more apt to pass off without leaving a trace behind them than are those convulsions which occur during the further course of the disease. There is considerable difference of opinion among au- thors regarding the remote dangers of convulsions. Many are inclined to believe that convulsions are always sympto- matic, and that they rarely constitute a serious danger to the child's life ; but others, like myself, relying chiefly upon a careful study of infantile cerebral palsies and of epileptic convulsions in the adult, are confident that convulsions are capable of, and often do give rise to, serious disturbance. This is not surprising if we consider that extreme cyanosis, with an accompanying intense hypersemic condition of the brain, occurs during the acme of the convulsion. I have myself recorded a case of a child dying in an epilep- tic convulsion, in which at the post-mortem table a wide- spread recent subpial hemorrhage over the convexity and at the base was found to have been the immediate cause of death. Eustace Smith refers to a case with hemorrhage over the base, evidently the effect of convulsions, while Money and others have reported cases in which a palsy, setting in after a convulsion, was evidently due to hemor- rhage from the smaller vessels of the brain coming on dur- ing, and caused by, a convulsive seizure. The possibility of such occurrences makes a convulsion a serious matter. It must be the earnest endeavor of the physician in attend- ance on the child to bring a convulsive seizure to an end as soon as possible. The longer a seizure lasts, the more fre- quently it is repeated, the greater the danger becomes — if not to the life of the child, at least to its future mental condition. Hence it follows that we must be guarded in giving a prognosis of the child's condition after the seizure is over. the majority of them leaving no unfavorable trace upon the child, but every now and then cases occur in which the convulsions were the first of a long series of disasters. 1 shall never forget the case of a bright child who had a con- 62 THE NERVOUS DISEASES OF CHILDREN. vulsive seizure at the onset of chicken-pox ; a rapidly de- veloping - idiocy was the result. Seizures occurring in the first few days after birth, inasmuch as they are the expres- sion of serious disease in the brain, give a particularly un- favorable prognosis. Treatment. — The first object of treatment should be to check the convulsion. Many remedies will have been ap- plied long before the physician has arrived at the bedside of the patient. Hot baths, mustard poultices over the spine, the abdomen, to the soles of the feet are still in vogue and urged not only by knowing mothers, but also by many ph}'- sicians (by Meigs and Pepper, for instance) ; but all these measures are to be deprecated, for they imply a loss of valuable time. If a convulsion ends while the child is in a hot bath it does so because it has run its course. To check the convulsion inhalations of chloroform, or of chloroform and nitrite of amyl in equal parts, are much to be preferred to other forms of medication. A few drops poured on a handkerchief and held to the nose of the child are generally sufficient to diminish the severity of the convulsive seizure, and the nurse or physician sitting by can keep up this method of administering the drug until the tendency to convulsions has for the time being entirely disappeared. Recognizing the danger from convulsions, the physician will do well to wait until all danger from further attacks has passed. After the acute stage is over small doses of morphia (gr. -^ to -J) or of opium, according to the age of the child, may be given ; and after the first day has been passed safely I should advise the discontinuance of the opiates and the substitution of small doses of bromide (3 to 5 grs.) several times a day, or small quantities of chlo- ral hydrate (gr. v. to x.), either by mouth or in the form of a rectal enema. Absolute quiet is the first essential of treatment. If there is good reason to believe that the convulsive seizure was due to gastro-intestinal irritation, or to the presence of intestinal parasites, steps should be taken to purge the bowels thoroughly, say by the use of castor-oil, calomel, and the like, or by appropriate treatment for the removal of worms. As soon as the cause has been removed, treat- CONVULSIONS— ECLAMPSIA INFANTUM. 63 ment by small doses of opium, bromide, or chloral, will be in order for at least a week or ten days after the occurrence of the seizure. If the convulsive attack is associated with fever, there is of course every reason for giving a tepid bath, with sub- sequent cool sponging. The reduction of the temperature will, to a certain extent, diminish the probability of repeated attacks. Should a child be seized with convulsions while in the bath, it should be removed at once and made to in- hale chloroform and nitrite of amyl. It is far better in the cases of gastric disturbance to evacuate the stomach through the bowels than to attempt to give emetics, which of course cannot be readily swallowed, or to give hypo- dermic injections of morphia as suggested by Steffen. For to cause a child to vomit that has a tendency to convulsions is to increase the danger to which it is exposed. Cases of laryngismus stridulus can be treated on very much the same principle as convulsions, excepting that the child should not be allowed to remain in the recumbent position ; it should be carried about, and if breathing is inter- fered with, forcible means, such as pulling forward of the tongue, slapping of the chest with cold and wet towels, should be resorted to in order to bring about regular, rhythmical breathing. • If the convulsions are due to rickets, very active treat- ment, looking toward the improved nutrition of the child, will be necessary. It should be placed upon the albumi- noids, and only small quantities of the cereals allowed. Moreover, in these cases, Kassowitz's plan of administering phosphorus with cod-liver oil, as in the scrofulous diathesis, is of greatest importance. Sirup of the iodide of iron, or any of the many preparations of iron now to be obtained in the market, can be administered. If the attack is the be- ginning of an epilepsy, no time should be lost in placing the child upon the regular treatment of this condition, for the details of which the student is referred to the chapter on Epilepsy. In the cases of ursemic and other toxic con- vulsions, the treatment must be in accordance with the nature of the cause, and the condition is to be treated in every respect as the same condition would be treated it 64 THE NERVOUS DISEASES OF CHILDREN. it occurred in the adult. If the attack is supposed to be due to cutting teeth, the question arises whether incision of the gums is apt to be of any benefit. There has been much discussion on this point, and yet no definite conclusion has been reached ; and no one, so far as I have been able to learn, has proved that cutting* the gums has ever injured the child or impaired its future health. It is well enough, therefore, to try this method, and personally I have little doubt that a temporary relief of the hypersemic condition of the gums is thus afforded. That the mere incision of the gum, as Henoch and Lewis suggest, is apt to increase the peripheral irritation, I can scarcely credit, if the incision be made according to modern surgical practice. BIBLIOGRAPHY. Cheadle : The Lancet, May, 1887. Chenbach : Rev. Mensuelle des Maladies de l'Enfance, 1894. Dana : Convulsions. Wood's Reference Handbook, etc. Elsasser: Der weiche Hinterkopf, 1843, p. 133. Gay : Brain, vol. xii., p. 482. Gee : St. Bartholomew's Hospital Reports, vol. iii. Hammond, G. M.: Medical Record, vol. xli., p. 263, 1892. Henoch : Vorlesungen iiber Kinderkrankheiten, 7th ed., p. 149. Jackson : Brain, 1886. Also his article on Convulsions in Reynolds's System, vol. i., p. 737, Am. edition ; Lumleian Lectures, The Lancet, March 29, 1890. Jacobi: Anatomy of the Thymus Gland. Philadelphia, 1888. Also Pepper's System of Medicine, vol. ii. On Craniotabes, Am. Jr. of Obstetrics, vol. iii., 1870. Kassowitz : Beitrage zur Kinderheilkunde, Neue Folge, IV., p. 43. Lewis : Convulsions, Keating's Encyclopaedia, vol. iv. Loos: Deutsches Archiv fur Klinische Med.,Bd. L., 1892. Meigs and Pepper : Diseases of Children, p. 551. Philadelphia, 1874. Money: Diseases of Children, 1887. Nothnagel : Ziemssen's Cyclopedia, vol. xii. Sachs : Volkmann's Series, Nos. 46, 47, p. 452. Senator: Zeitschrift f. Kl. Med., Bd. VII. H. 3. (Ptomaines.) Smith, Eustace : Diseases of Children, p. 292 et seq. (Influence of Lead.) Soltmann : Gerhardt's Handbuch der Kinderkrankheiten, Bd. V., 1. Tarchanoff Arbt. fur Kinderheilkunde, 1879. Unverricht: Experimentelle u. Kl. Unters. iiber Epilepsie. Breslau, 1883. Deutsches Arch. f. Kl. Med., 1883, P- 44L West : Diseases of Children, 5th Am. ed., 1874. CHAPTER III. EPILEPSY. Few diseases have given rise to so much discussion as has epilepsy, the " morbus sacer " of ancient writers. Opinions regarding its cause and pathology have under- gone many radical changes. The causes of epilepsy were by most writers thought to be obscure, and it is only with- in very recent years that a little more light has been shed upon the morbid processes underlying epilepsy. Some claim that epilepsy is never a disease per se, always a symptom, while others are inclined to regard true idio- pathic epilepsy as a form of hereditary disease. Whether epilepsy is merely a symptom or a distinct disease the term may be used to describe a state in which convulsive seizures occur at varying intervals of time, and in which there is no special cause for each seizure. Thus a child may have a convulsive fit with the cutting of each tooth, yet we cannot say that it has epilepsy ; but if that same child, after dentition and its attendant troubles have passed, continues to have fits at shorter or longer intervals, the child is surely a subject of epilepsy. Fortunately, how- ever, only a very small proportion of those who have con- vulsions during childhood develop true epilepsy in later years. Webber reports that of 160 cases of epilepsy only 24 began before the age of five. The Symptoms of epilepsy vary with the character of the attacks. Unfortunately the intervals between the attacks are not marked by. as distinct a series of symp- toms as is the case in tetany. In epilepsy the only symp- toms that we can find in the interval are the effects of biting the tongue, the acne resulting from the bromide treatment, and the general stupor and indifference which arc as often due to the drugs administered as to the disease itself. 66 THE NERVOUS DISEASES OF CHILDREN. Epileptic attacks can be divided into two classes: i. Major attacks, or " grand mal." 2. Minor attacks, or "petit mal" It is not uncommon to see patients who are subject to both kinds of attacks, and there is every possible gradation between the graver and lesser seizures. The "grand mal" attacks differ but very little from the convulsive seizures as described in the preceding chapter. It will be sufficient in this connection to enumerate in the order of their importance and of their occurrence the chief symptoms of a major attack of epilepsy. i. Prodromata, generally of a sensory character. At times there is a vasomotor or psychic disturbance. 2. Initial cry. 3. Loss of consciousness (very sudden). 4. Pupils dilated ; no reaction. 5. Tonic or clonic spasm of muscles (unilateral, partial, or general). 6. Spasm of respiratory muscles, which may lead to asphyxia. 7. Spasm of the muscles of the jaw (biting of the tongue, bloody foam). 8. Spasm relaxes, movements become clonic and then intermittent. 9. Involuntary passage of urine or of fasces. 10. Gradual recovery of consciousness, followed by a prolonged stupor or profound sleep. In a typical attack of " grand mal " the majority of these symptoms will be present, but occasionally we meet with major attacks in which there are no distinct prodromata; in which the initial cry is wanting ; in which there is no biting of the tongue ; no relaxation of the sphincters. The most constant symptoms are the loss of consciousness, the dilatation of the pupils, the spasm of the muscles, and the stupor or sleep after the convulsive movements have ceased. As in many other diseases due weight should be attached to each symptom, and while the diagnosis of epilepsy should not rest upon a single symptom, the association of any two or three of the above-named symptoms will be sufficient for a diagnosis in any given case. EPILEPSY. 67 The prodromata are of great importance. In a fair pro- portion of cases the patient feels that an attack is coming on. A vague sensation at the stomach, a feeling of numb- ness or of tingling in any of the extremities, are by far the most frequent warning signs. In other cases the aura con- sists of slight twitching movements, of an altered psychic condition, varying from a general restlessness, or irritability of temper, to actual maniacal excitement. In many instan- ces the character of the aura points to the involvement of a definite portion of the brain, and this is true even of cases of general epilepsy which are not supposed to be due to any gross cerebral lesion. In not a few cases temporary aphasia marks the coming on of an attack. Auditory symptoms in the prodromal stage are not un- common. In a little boy whom I described -in a paper pub- lished some years ago, every epileptic attack was preceded by a hissing noise like that of a steam-engine letting off steam. A young lady, aged twenty-five, a school-teacher, who was subject to epileptic attacks at every menstrual period, is in awe of an old woman whom she sees regularly before each epileptic seizure. The vision of a ball of fire, of the colors of the rainbow, of a sudden change in the size of objects, are common premonitory conditions. Still an- other patient perceives a very foul odor during the aura ; but I have never met with a case of epilepsy in which a pleasant or unpleasant taste preceded an epileptic fit. This would seem to show that the sense of taste is not inde- pendent of the sense of smell, although some authors refer in a vague manner to a gustatory aura. Patients who have distinct auras are on the whole more fortunate than those who have not, for they are able to prepare themselves for the attack. They can secure themselves against injury during the attack and may have time to apply remedies which either inhibit or shorten the spells. The convulsive movements should be carefully studied in each case, for they help to define the character of the epilepsy. Generally speaking, universal convulsions point to hereditary epilepsy. Partial or unilateral epilepsy is indicative of organic cerebral disease. This distinction 68 THE NERVOUS DISEASES OF CHILDREN. would be a very easy one were it not for the fact that partial epilepsy may at any stage of the disease, and at any stage of an attack, become general, so that after the lapse of time the convulsions due to organic disease of the brain can in nowise be distinguished from those which are pre- sumably hereditary and idiopathic. It is of the utmost im- portance, therefore, in making the diagnosis of epilepsy, to inquire into the past history of the case, and to determine, either by personal observation or by close questioning, the exact manner of onset of each attack. However rapidly the attack may become generalized, if it begins each time with distinct twitchings of the thumb, of the wrist, even of the eyelid, there is good reason to remove such a case from the category of hereditary epilepsy and to range it with those due to organic lesions. The attacks may come on it definite hours of the day or night. In " nocturnal " epi- lepsy an abrasion of the tongue, involuntary micturition, a tired feeling or a headache in the morning may be the only evidences of an attack.* The typical '* minor " attacks consist of a very transitory loss of consciousness, without any muscular twitchings, without the peculiar cry, and without the involuntary pas- sage of urine and faeces. The loss of consciousness is often so slight that the child is supposed to have been " absent- minded " or merely " faint." Mothers and physicians, euphemistically inclined, are apt to speak of these attacks as " fainting spells." There is every reason to suspect epi- lepsy in any case in which " fainting spells " occur and re- cur without sufficient exciting cause. In some instances a sudden lull in the conversation of the child, a momentary stare, an unexpected stop when the child is practising upon an instrument, are the signs of petit mal ; a change in the child's mental condition, in its character and temperament (there is often increased irritability) and sometimes a mild form of mania, reveal the serious character of what was supposed to be a mere fainting spell. Bourneville has found a slight elevation of temperature during major attacks (not more than i° F.); in the status epi- lepticus, the condition of continuous spasms, the tempera- * For further details see description of convulsive attack. EPILEPSY. 09 ture may rise several degrees. Thomsen and Oppenheim have proved the existence of a concentric limitation of vision, and a diminution of general sensibility for some time after an epileptic attack. The term " procursive epilepsy " has been applied to attacks in which the patient is suddenly impelled to run some distance forward, or backward ; this may be a pro- dromal symptom, or it may be the only manifestation of an attack. This variety is very rare indeed. We have alluded above to the psychic symptoms of epileptic attacks. Instead of having a typical convulsive seizure, the child passes into a state of mental confusion in which it becomes entirely irresponsible for, and unconscious of, its actions ; it may also pass into a condition of trance or into an acute mania. Prolonged periods of double con- sciousness are rare in children ; nor have I seen in very young persons the condition of narcolepsy (sudden falling asleep) which has been described as an occasional symptom of epilepsy in the adult. These " psychic equivalents " may be the beginning of a post-epileptic insanity. The mental derangement following epileptic attacks is often marked by violent delirium. Under the influence of delusions and hallucinations, the patient may be impelled to deeds of cruelty. Such derangement may last for a few hours or weeks, or it may become chronic and lead to de- mentia. Causes. — In considering the causes of epilepsy we shall take up, first, causes of the disease itself, and secondly, the causes which lead to individual attacks. That epilepsv is often a sad heritage can scarcely be doubted if we regard the statistics collected by Gowers, who finds a distinct family history in two-thirds of 1,450 cases. The inheri- tance seemed to him to come more freely from the mother's than from the father's side. It is also beyond dispute that epilepsy in the ancestry is not the only predis- posing cause. Other chronic nervous disorders have a powerful influence in this respect. I have known children of extremely hysterical, neurasthenic mothers to develop idiopathic epilepsy. Chorea in the mother is very apt to lead to the development of true epilepsy in the child : and. 70 THE NERVOUS DISEASES OF CHILDREN. on the other hand, syphilis and alcoholism in the father exert a very powerful influence in this same direction.* According to Mendel's recent studies the majority of cases of hereditary epilepsy begin before the age of twenty years ; but up to the age of forty years idiopathic epilepsy may manifest itself. The same author proposes to call the disease " late epilepsy," if it is developed after the age of forty years; in twenty-five per cent, of these "late" cases Mendel found distinct hereditary influences. Accepting the truth of all these statements, I wish to impress one fact upon the mind of the student. Cases of hereditary (idiopathic) epilepsy are not nearly so frequent as they are supposed to be. If we examine carefully into the early history of our cases we shall find frequently that the child has either sustained some traumatic injury to the brain, or has acquired some cerebral lesion early in life. The paralysis and other symptoms which were due to the same lesion may have disappeared, but the epilepsy re- mains. I was led to this conclusion by my studies of infan- tile cerebral palsies, and my views have been quoted ap- provingly by various authors (among them Freud) who have become interested in the same subject. A very striking instance illustrative of this is that of a girl, about fifteen years old, who had been treated by many physicians for idiopathic epilepsy without any favorable result. On inquiring into the early history of the case I was told that several years previously the girl had suffered a slight paralytic stroke, every vestige of which had disap- peared with the exception of an increase of the reflexes on one side of the body. Testing carefully for a possible dim- inution of power, I found distinct traces of an old hemi- plegia. What was supposed to have been a case of heredi- tary epilepsy was clearly a case of epilepsy due to former cerebral disease. The number of cases of idiopathic epilepsy will be still further diminished if we exclude from the number those cases in which there is evidence of defective general devel- * Kowalewsky has insisted on the importance of syphilis in hereditary epilepsy, and has made a fanciful distinction between epilepsy due to paternal or maternal syphilis. His statements need corroboration. EPILEPSY. 71 opment of the brain ; and those associated with infantile cerebral palsies ; for the paralysis and the epilepsy are due to organic disease of the brain. The cases in which epi- lepsy is developed after an acute infectious disease cannot be considered " idiopathic." The most characteristic cases of idiopathic epilepsy are those in which the disease shows itself between the tenth and twentieth years. A few may come on between the twentieth and thirtieth, but those that occur later in life, or in the first few years of life, should be very carefully anal- yzed before the diagnosis of hereditary epilepsy is made. It must be remembered that even in cases with dis- tinct hereditary predisposition some other exciting cause must be present to develop the disease ; thus we find that masturbation, disturbances of menstruation, sexual excesses, great emotional excitement, a blow to the head, are causes which lead to the development of epilepsy in those predis- posed.* Causes which Lead to Individual Attacks. — In a patient suffering from epilepsy any interference or disturb- ance of the general health of the patient may bring on an epileptic attack. Indigestion is perhaps the most powerful of these causes. Herter and Smith, in an excellent research on this subject, are inclined to look upon putrefactive proc- esses in the intestine as a frequent exciting cause of epi- lepsy. They do not believe that this cause w^ould be suffi- cient to produce epilepsy in a person not predisposed thereto. The same may be said of eye strain,t of urethral * Reflex epilepsies are described by many authors as due to peripheral injuries (hand or foot) ; in such cases the aura begins with symptoms referable to the injured part. A permanent epilepsy is rarely established in this way, and I prefer to speak of reflex convulsions rather than of reflex epilepsy. t Ranney's contributions to this subject are put forth very earnestly, but he fails to prove more than that the eye-conditions (errors of refraction, muscular insufficiencies, etc.), may cause single attacks ; but they are surely not the cause of the epilepsy. Moreover, every neurologist knows that by treatment directed to the improvement of the eye-condition the attacks may be suspended temporarily, but the disease returns after a varying lapse of time. Ranney's cases are for the most part reported altogether too early. Hern and Dodd have given but little support to Ranney's (or rather Stevens's views), and their statistics are open to the same criticism. Instead of reporting the " cures " it would be valuable to publish the later history of all c;ise;< operated upon. Dr. Weir Mitchell's paper concerning the history of the discovery of reflex neuroses may well be read in this connection. 7 '2 THE NERVOUS DISEASES OF CHILDREN. stricture, of narrow prepuces, and of laryngeal irritation. I doubt whether any one of these conditions has ever been the sole cause of epilepsy, though I am willing to concede that they may be sufficient to produce attacks in persons with this special hereditary taint. Improper oxygenation of the blood is another frequent cause of attacks. This explains why so many epileptics have their seizures in crowded court-rooms, in theatres, and in poorly ventilated bedrooms. The irritation of the epi- leptic centres in the lower portion of the brain is directly responsible for these attacks. After allowing for all these various causes we are compelled to admit that many of the epileptic seizures occur at more or less regular intervals, for reasons that we cannot discern, though I am firmly convinced that the more carefully we regulate the life and habits of the epileptic patient the fewer attacks he will have. It is very largely a question of removing all excit- ing causes. A few powerful drugs have been known to cause epi- lepsy. Heiman described a number of cases of epilepsy due to poisoning by cocaine, and Tuczek has dwelt upon the potent influence of antipyrin. In the case of children cocaine need not be feared ; but the careless exhibition of antipyrin might be responsible for unpleasant occur- rences. Gray has called attention to the frequent occurrence of epileptic attacks in those suffering from chorea and mi- graine. I have seen several patients, in advanced years, in whom the epilepsy followed upon the cessation of mi- graine. Diagnosis. — The diagnosis of epilepsy offers but little difficulty if the character of the individual attacks has been clearly made out, and if such attacks recur at more or less regular intervals. The epileptic nature of a seizure is determined by the presence of some of the several symptoms which go to make up a complete attack. The chief difficulty will be experi- enced in differentiating between an epileptic seizure and an ordinary fainting spell ; also between epilepsy and hysteri- cal attacks. EPILEPSY. n The characteristic features of each attack will be found in the following tables : Epileptic Attacks. Loss of consciousness very sudden. Warning of short duration. Pupils dilated ; do not contract to light. Pulse unaltered. Tonic and clonic spasms in various parts of the body. Bloody foam at mouth. Involuntary passage of urine and faeces. Prolonged stupor after attack. Fainting Spells. Loss of consciousness gradual. Warning of some minutes before consciousness is lost. Pupils contracted or unaltered. Pulse feeble. No spasms. No evidence of biting of the tongue. No involuntary passage of urine or fseces excepting in rare instances. Recovery prompt after attack. Epileptic Attacks. Loss of consciousness sudden and absolute. Warning of short duration. Pupils dilated. Tonic and clonic spasm. Eyes turned upward and inward. Involuntary passage of urine and fasces. Prolonged stupor. Attacks at rarer intervals. Duration of attack short. Hysterical Attacks. Loss of consciousness not absolute. The attack often preceded by emo- tional excitement. Pupils not dilated. Tonic rigidity ; exaggerated conscious movements ; arching of back ; ex- cessive noises. Eyes staring, not turned, sometimes closed. No involuntary passage of urine or faeces. Recovery gradual ; no stupor. The patient may pass, however, into a trance condition. Attacks may be frequently repeated. Duration of attack much longer. The distinction between organic and idiopathic epilepsy can easily be made if we simply call to mind that organic epilepsies are, as a rule, partial, if not unilateral. Idio- pathic epilepsies are invariably bilateral or general in their manifestations. If organic disease is suspected, the manner of onset of the individual attack should be carefully determined, for partial 74 THE NERVOUS DISEASES OF CHILDREN. or unilateral manifestations often become general during the attack. Under the head of organic epilepsies we must include post-kemiplegic epilepsy. This form comes on with deplorable frequency after the paralytic attacks in early life. The epileptic movements as a rule affect the paral- yzed side, but after the disease has lasted for some years the child, although its paralysis is unilateral, is prone to have general epileptic attacks. Under these circumstances the relation between the general epilepsy and the hemi- plegic form of paralysis can scarcely be doubted. It will not be amiss to insist again upon the fact that the evidence of an existing hemiplegia may be so slight that it will be overlooked unless specially examined for; but however slight such traces may be, if it can be shown that the epi- lepsy was developed after the onset of the hemiplegia there is good reason to suppose that both the paralysis and the epilepsy are due to the same organic lesion. I consider it a safe rule in epilepsy beginning in childhood to examine particularly for exaggeration of the reflexes ; and an in- crease of the reflexes in one-half of the body is quite as safe a sign of a preceding hemiplegia as a marked paralysis with contracture would be. The sudden onset of epileptic movements in a child pre- viously healthy should lead one to suspect the possibility of intra-cranial tumor ; and a slight weakness of the parts con- vulsed, a possible increase of the deep reflexes in that same part, the presence of headaches and the development of optic neuritis, are the symptoms that we must look for in order to establish or to discard the diagnosis of tumor. The same series of symptoms may occasionally be pres- ent in cases of acquired or hereditary syphilis ; if so they are the result of general specific infiltration of the brain coverings, or of the development of gumma together with this general infiltration. Prognosis. — The prognosis of epilepsy, whether of the organic or idiopathic order, is altogether bad. This con- viction deepens the larger one's experience with this di- sease. Do what we will, the attacks recur as soon as treat- ment is abandoned. I have seen but very few cases of absolute cure of genuine epilepsy. One was the case of a EPILEPSY. 75 young girl, now twenty years old, in whom the disease was developed at the age of twelve, coincident with the onset of menstruation. She has been free from attacks for very nearly five years. I have known the attacks to be inhibited for a year, and even for two years, and yet they have re- turned after that period of time if the customary treatment was stopped. In this respect there does not seem to be a wide difference between organic epilepsy and idiopathic epilepsy, excepting that in the case of the former an early termination in death can be looked for if the attacks are due to malignant disease. The frequent development of de- mentia, idiocy, or of epileptic insanity, in epileptics makes the outlook gloomier still. In spite of all recent therapeu- tic efforts we have not been able to accomplish anything in the way of a radical cure of the disease. The only ray of hope that we have is that in the beginning of the disease the attacks may be due to special conditions which do not necessarily imply the existence of hereditary epilepsy. The fond hope that the child may outgrow the tendency to epi- leptic seizures is rarely realized. Pathological Anatomy. — Our knowledge of the mor- bid changes in epilepsy is very incomplete. Meynert laid great stress upon induration of the cornu amonis. Chronic hydrocephalus has been found in many cases, but this can hardly be considered a cause of epilepsy, for if it signifies anything it merely implies that the same process which led to the development of hydrocephalus also caused the epi- lepsy. It is much more probable that the morbid changes of true epilepsy will be revealed through a study of the corti- cal changes which give rise to a localized epilepsy in cases of traumatic injury to the brain, and of epilepsy associated with infantile hemiplegia. In these conditions the initial stages of a morbid process* leading to epilepsy can best be studied ; and it is possible, if not probable, that general epi- lepsy has such a beginning at a time and in a part of the brain unknown to us. Marie, Fere and Chaslin have led the way in these studies, and report the discovery of a neuroglia * For other morbid processes associated with epilepsy see chapters on Cerebral Pal- sies and on Idiocy. 76 THE NERVOUS DISEASES OF CHILDREN. wmmmm mMmm sclerosis which they consider to be the cause of epilepsy — such sclerosis starting from a focus of disease. Van Gieson has made a very valuable contribution to this subject by a study of cortical tissue, removed by McBurney, from two cases of Starr. Van Gieson demonstrates most sat- isfactorily the changes in the large pyramidal cells of the cor- tex and in the neuroglia. He finds that " the ganglion cells are affected by a series of de- generative changes, which in their most advanced stages re- sult in an almost complete dis- solution of the cell, and yet this degeneration is not extensive enough to involve the cells so universally as to interfere with their topographical distri- bution." Some of the degenerative changes of the cells will be evident in the figure below taken from Van Gieson's article. There is a decided change — a true hyperplasia — in the Fig. 39. — Diffuse Neuroglia Sclerosis of the Cortex in Epilepsy (after Chaslin). Fig. 40. — Various Phases of the Earlier Stages of the Degeneration of the Ganglion Cells. The thin lines enclosing the cells w and u represent the pericellular spaces ; the cells x and y show the earliest stages, w and s later stages, and k shows the ultimate destruction of the whole of the ganglion-cell body, leaving nothing but the nucleus lying in an empty space. (Van Gieson.) EPILEPSY. 77 neuroglia tissue ; clusters of an increased number of very young and seemingly proliferating neuroglia cells are vis- ible in the vicinity of the small pyramids. These findings were very much the same in two cases : in one the condition was due to a foreign body, and in the other an old cicatrix seems to have induced a similar path- ological state in the neighboring tissue. To appreciate such researches at their true worth, it is important to note that they have reference to the early conditions only. What the nature of the secondary changes is, and how these are m 1 #■'*£» ja §) Fig. 41. — A Group of More Mature Neuroglia Cells in the Layer of Small Pyramids. (Van Gieson. ) developed from the original forms of disease is a subject for further study. It will be a fertile field of research for any- one who undertakes with the ever-improving histological methods, to study the entire brains of epileptic children and adults. Pathology. — The pathology of the epileptic attack is identical with that of convulsive seizures in general. It is not necessary therefore to repeat in this chapter what was said in the chapter on convulsions. The onlv question of importance is whether in the diseased brain the convulsive centre on the floor of the fourth ventricle plavs as impor- tant a role as it does in physiological experiments. Bins- wanger found that electrical stimulation of the lower halt of the floor of the fourth ventricle in animals would pro- duce tetanoid attacks or spasm in the limbs and the trunk, but he did not succeed in causing an ordinary epileptic at- 78 THE NERVOUS DISEASES OF CHILDREN. tack. There can be very little doubt that in man an epi- leptic attack is in the vast majority of cases the result of cortical irritation. The character of the sensory auras is evidence of this, and so is the observation made by Oebeke and Gowers, that epileptic attacks cease after a lesion in the internal capsule.* As for the pathology of the disease itself, opinions have undergone a very marked change within the last few years, owing to the rather startling suggestion of Pierre Marie, that epilepsy is often of infectious origin. There is much in favor of this view, but we must not attach undue impor- tance to the element of infection, and should remember that the convulsions which occur so frequently in the acute fe- brile diseases of childhood give rise to serious vascular dis- turbances, and that a focus of disease, due to purely me- chanical causes, may be the beginning of general changes throughout the brain, which are responsible for the per- manent establishment of epilepsy. Treatment. — In discussing the treatment of epilepsy it is best to proceed on the theory that the attack is due to over-action of the cortical cells. Gowers explains the influ- ence of the bromides by stating that " if we regard the morbid state in epilepsy as an instability in the resistance of the nerve-cells, it seems probable that the effect of the bromide is to increase the stability of that resistance." Whatever the explanation may be, we have up to this time found no drugs that can in any sense be considered proper substitutes for the bromides. f It is certain that very few cases of epilepsy have been permanently cured by the administration of the bromides ; but unquestionably they serve an admirable purpose in checking the number of attacks and in diminishing their * Horsley pleads strongly for the invariable cortical origin of the epileptic attack, and believes that during the attack the cortex is in a congested rather than an anaemic condition. It will help us very little to say that the attacks are due to toxine poi- soning. This may or may not be true ; but in every case the most important ques- tion to settle is, why should certain individuals be prone to attacks and others not, while these same toxines from intestinal putrefaction, for instance, are present in hundreds of others who never have an epileptic seizure. t A recent writer explains the action of the bromides by their power to diminish the effect of the toxines circulating in the body. If toxines and bromides were in- jected into the veins of an animal at one and the same time, the result was less severe than if the toxine alone was injected. EPILEPSY. 79 severity. To accomplish this end the bromide salts should be administered according- to a definite plan. It has been my practice to give preference to the bromide of sodium, which I employ, according to the age of the patient, in ten or fifteen grain doses, three times a day. If given in a wineglassful of (alkaline) water after meals, the gastric functions will not be seriously impaired. Erlenmeyer sug- gested, some years ago, that a combination of the three salts — the bromide of sodium, the bromide of ammonium, and the bromide of potassium — would answer far better than the exhibition of any one of these salts singly. He went so far as to claim that if a single salt had produced acne, that that acne would disappear upon the administra- tion of the three salts combined. I have given this method a fair trial, but have not been able to convince myself of the truth of Erlenmeyer's claim. The bromides should be pushed in every case to the point of tolerance, and until the attacks have been successfully diminished or inhibited. The loss of the palate reflex is evidence that the patient has become thoroughly brominized, but in children intol- erance to the drug is very apt to be established long before this point has been reached. The bromides can be administered in divided doses, two or three times a day ; but if there is no good reason to give the drug during the day, there is a special advantage in giving the entire daily dose shortly before bedtime. The stupefying effect of the drug is not felt as distinctly as when it is administered during the daytime, and the sound sleep that ensues is an additional advantage to the patient. In the case of nocturnal attacks the medicine should be given before going to bed, and at no other time. In his recent admirable lectures on the treatment of certain func- tional neuroses Seguin advocates the plan of giving the bromides on very much the same principle that one would adopt in giving quinine in malaria. In other words, the bro- mides should be given according to the periodicity of the attacks, giving little in the interval and increasing the dose very much at the time when an attack is expected, or if the attacks are frequently repeated, to give the drug four to five hours prior to the time at which the seizures occur. 80 THE NERVOUS DISEASES OF CHILDREN. I can subscribe thoroughly to this recommendation, as it has done me admirable service in the treatment of epileptic at- tacks in children. If the tendency to attacks has been over- come, the drug should be continued for a period of at least one year after the last attack. The dose may be dimin- ished gradually, but it is wise to keep the patient in a state of mild brominism. If it is desirable to diminish the amount of bromide the patient takes, the good effect of the drug can be maintained by giving, in conjunction with the bromide, five to ten grains of chloral hydrate, a few minims of the tincture of digitalis, or of the tincture of belladonna. Innumerable other drugs have been suggested ; among these I will mention cannabis indica, which is particularly effective in cases of epilepsy with chronic headaches. The oxide and lactate of zinc have been freely recommended, but I have seen no beneficial results. The administration of iron and arsenic is thoroughly rational ; no doubt the restorative effect of these substances upon the blood is the indirect cause of improvement in the epileptic con- dition. Some years ago Gowers advised the use of fif- teen to thirty grains of borax several times daily. A fair trial has been given this drug in my clinic and in private practice, but I cannot claim more for it than for dozens of other drugs.* Flechsig has lately advised a combination of opium and bromides. Opium is given for six weeks ; beginning with one-half to one grain ; the dose is increased gradually until the patient takes eight, ten, or fifteen grains daily ; then it is stopped suddenly, and the bromides in thirty-grain doses, four times daily, are substituted ; after some time the dose is diminished to five or ten grains per day. I was ready to adopt this suggestion, as I had for many years been using codeine together with the bromide salts. In children the doses must be somewhat altered, but the plan of treatment, according to my own experience, deserves further trial, f * Dr. Joseph Collins has summarized the more recent measures suggested in the treatment of epilepsy. He has a kind word to say for borax, for nitroglycerine (in cases of vasomotor aura), and for Flechsig's method; but utterly condemns the use of osmic acid, simulo, hydrastinum, and other drugs. t While this chapter was going through the press Bechterew has advised a combi- nation of bromides and Adonis vernalis. EPILEPSY. 51 The treatment of epilepsy is by no means exhausted by the recital of a few medicinal agents. Success depends upon other factors. The greatest possible attention should be paid to the details of the patient's daily life ; regularity in the hours of sleep, in the hours of meals, and careful dieting, are essential to proper treatment.* I place my epi- leptic patients upon a general mixed diet, including small quantities of albuminoids and a liberal vegetable diet, but I forbid all indigestible articles of diet. I also limit the amount of cereals, and try to check intestinal fermentation as far as possible, being moved to this by the result of Her- ter's researches. Pastry and sweets of all sorts are strictly prohibited. Fresh air at all times, and particularly at night, should be insisted upon. Freedom from all emotional ex- citement is another recommendation that should not be overlooked. Marked ocular defects and other peripheral conditions which may cause an epileptic attack, are to be treated. Older patients who have distinct warning of their epi- leptic attacks should be provided either with the pearls of nitrite of amyl, which they can break up and inhale at short notice, or else they can be given a mixture of equal parts of chloroform and nitrite of amyl, which can be carried in a small phial and can be poured upon a handkerchief for purposes of inhalation as soon as the attack is signalled. In the case of partial epileptic attacks of definite onset a ligature applied around the part first convulsed, to be tight- ened quickly as soon as the warning comes, has been rec- ommended by Hughlings Jackson, and is serviceable in some cases. Surgical Treatment. — The surgical treatment of epi- lepsy has attracted great attention during the past ten years or more, ever since it has been known that the motor centres in the cortex can be safely and easily reached by the surgeon's knife. Surgical interference should be considered only in cases of partial epilepsy pointing to a definite focus of disease as the starting-point of the entire trouble. Nothing seems easier than to expose the centre * To carry out these measures successfully, I am now in the habit of placing such patients under the rest cure whenever feasible. 6 82 THE NERVOUS DISEASES OF CHILDREN. of the part first convulsed in an epileptic attack, to remove this centre, and thus to remove the seat of disease, but, un- fortunately, our ardent hopes in this matter have not been realized. Some years ago Dr. Gerster and myself reported the results of operation in ten cases of epilepsy. The cases were carefully selected, and if surgical operation could have been of avail in any case of epilepsy, it would have been so in those we selected for operation ; we were bound to admit that in our experience some slight improve- ment followed, but also that not a single absolute cure had been effected. Since that article was written my ex- perience has been fully doubled, and the conclusions reached are practically the same. An analysis of the cases reported by Starr, which included my own cases, does not justify one in taking a more hopeful view of this question. I have referred in former writings to the reason for this disap- pointment. After an initial injury to the brain, months, and sometimes years, elapse before the epileptic habit is established. In about the same length of time a general cerebral sclerosis has been developed in connection with the original focus of disease. We may remove the orig- inal focus, but the general sclerosis remains and will act as a constant irritant upon the remaining epileptic centres. On the other hand, it would be hasty to conclude that the surgeon's skill can be dispensed with in these cases. * The aim should be to watch carefully for the development of epilepsy, and to remove injured tissue at the earliest pos- sible date. More than this, I consider it important not to await the actual development of epilepsy ; and if the brain has sustained any considerable injur}^ to remove the in- jured tissues, which, if allowed to remain, constitute a per- manent menace to the future health of the patient. We shall be able to prevent the development of epilepsy very much more readily than we can cure it if once established. The surgical procedures in vogue at the present day for the cure of epilepsy are trephining and excision of diseased tissues. Trephining has been practised for centuries. It * The author wishes to direct particular attention to the excellent work done by Kocher. EPILEPSY. 83 is a method that entails no special danger upon the life of the patient operated upon, and can therefore be tried with perfect impunity except in very young children. White, of Philadelphia, and others believe that its only effect is the same as that of any operative procedure ; but I am inclined to think that if it has any good effect it is in the way of relieving increased pressure, and that increased pressure is more frequently a real factor in epilepsy than is generally supposed, on account of the frequency of cystic formations and of the increased ventricular fluid in a very large proportion of the cases. Excision of tissue would seem to be a rational procedure in the earlier stages of epilepsy developed after traumatic injury, or in connection with early cerebral disease; but the paralysis which so easily results from the excision of the motor centres mil- itates somewhat against the advisability of this practice, although according to Dana's and my own experience such paralysis disappears after a few weeks. In cases in which the epileptic attack is preceded by sensory or psychic phenomena, a removal of the auditory or visual centres, for example, would be a serious matter indeed, if not en- tirely unjustifiable. Many a patient would prefer the oc- casional repetition of an epileptic attack to a permanent loss of hearing or a permanent impairment of sight. The only other* surgical procedure which has been sug- gested for the cure of epilepsy is the ligature of one or both vertebrals, as was first done by Dr. Alexander, of Liv- erpool. The dangers of this operation are so great, and the results so uncertain, that it scarcely merits serious con- sideration. BIBLIOGRAPHY. Bidwell and Sherrington : British Medical Journal, 1893, November 4, p. 988. Binsvvanger : Arch. f. Psych., 1888. Bourneville ; Recherches sur l'Epilepsie, l'Hysterie, etc. Paris, 1893. Blocq-et-Marinesco : (Pathology) Sem. Med., 1892, p. 445. Collins : New York Medical Record, September 22, 1894. Dodd : Brain. Winter Part, 1893. * I do not consider it necessary to refer to the surgical treatment of the eve-muscles for the cure of epilepsy except utterly to condemn the practice. As I am treating of epilepsy in children, I need not discuss oophorectomy and similar procedures. 84 THE NERVOUS DISEASES OF CHILDREN. Echeverria : Epilepsy. New York, 1870. Erlenmeyer: Behandlung d. Epilepsie, Monograph., pp. 513, 573. Berlin, 1892. Fere : Les Epilepsies. Paris, 1890. Fischl : Zeitschr. f. Heilkunde, 1890. Flechsig : Neurologisches Centralblatt, 1893, p. 229. Francois, Frank : LeQons sur les Fonctions du Cerveau. Paris, 1887. Fraenkel : Beitrage zur Chirurg. Festchr. fur Th. Billroth, 1892. Gerster and Sachs : American Journal of the Medical Sciences, November, 1892. Goldstein : Schmidt's Jahrb., vol. ccxliii., p. 100. Gray : Keating's Cyclopaedia. Heiman : Dtsch. Med. Wochenschr., 1889. Hern : British Medical Journal, September 30, 1893. Herter and Smith : New York Medical Journal, 1892. Horsley ; British Medical Journal, April, 1892. Kocher : Zeitschr. fur Chirurgie, 1892; also 1893, xxvi., pp. 1-93. Kowalewsky: Syph. Epilepsie, Berl. Kl. Wochenschr., 1894, No. 4. Kiimmel : Dtsch. Med. Wochenschr., 1893, pp. 326 and 531. Kussmaul and Tenner : Publications of Sydenham Society, 1859. Marie, Pierre: Sem. Med., July, 1892. Mendel: Neurolog. Centralbl., 1893, p. 664. Nothnagel : Article " Epilepsy " in Ziemssen's Cyclopaedia ; also Wiener Medicinische Presse, 1893. Parker and Gotsch : British Medical Journal, May 27, 1893. Reynolds : Monograph, London, 1861 ; also in Reynolds's System of Medi- cine, Article Epilepsy. Ranney : New York Medical Journal, 1 893 and 1 894. Sachs : New York Medical Journal, February 20, 1892. Seeligmuller : Dtsch. Med. Wochenschr., 1 and 2, 1894. Seguin : Functional Neuroses, New York Medical Journal, 1890. Starr : Brain Surgery, 1893. Tuczek: Berl. Kl. Wochenschr., 1889. Trowbridge : Al. and Neurologist, February, 1892. Van Gieson : New York Medical Record, April 24, 1893. Webber : Boston Medical and Surgical Journal, 1893, vol. xxviii., p. 491. Weir Mitchell : Medical News, Philadelphia, April 28, 1894. CHAPTER IV. HYSTERIA. True hysteria is a relatively rare condition in adults. This may seem a very heterodox statement to those who have been ready to apply the term hysteria to many affec- tions of the nervous system which could not be attributed to organic lesions. Those who are advised of the recent conception of hysteria will not hesitate to indorse the au- thor's opinion. The determination of the chief symptoms of true hysteria, and the separation of this disease from many other functional disturbances which were once classed with it, have constituted a signal achievement in modern neurology. We have at last succeeded in establishing a number of symptoms, chiefly of a sensory character, which, when present, enable one to make the diagnosis of hysteria with a great degree of certainty, and which do away with the necessity of reaching this diagnosis by the process of exclusion. As the physician's knowledge of nervous dis- eases increases, the diagnosis of hysteria is made more and more infrequently. We see much less of true hysteria in this country than in Europe ; it is also far less common in England and in Germany than in France and Rus- sia. In the author's very large clinical service at the New York Polyclinic the diagnosis of hysteria is one of the rarest of all diagnoses made. In other in- stitutions with which the writer is connected, particularly in the Montefiore Home for Chronic Invalids, hysteria is found to be much more frequent, especially among the foreign-born inmates. Broadly speaking, the Anglo- Saxon race is less prone to the development of hysteria than the other races represented in our population. If hysteria is a rare disease in the adult, it is still rarer in the child, but when it does occur it is an important 86 THE NERVOUS DISEASES OF CHILDREN. factor in the future life of the child. Many of the writers on diseases of children have neglected this subject entirety, and others have given but scant notice of it. The older English and American authors have had very little to say upon the subject. Meigs and Pepper, as well as West, scarcely refer to it. In the large hand-bock of Gerhardt, Jolly has treated the subject in a very satisfactory manner, and in American Cyclopaedias and Systems of Medicine the subject has received careful attention at the hands of Mills and Lloyd. Henoch has written a very full chapter on the subject, but includes under this heading many forms of disease which are more properly designated by other names. The French authors have naturally had much to say on the subject, and they include so many different forms under this heading that one is puzzled to know whether there is any form of nervous or mental disease that is not in some way related to hysteria or influenced by it.* The symptoms of the disease are truly protean in char- acter. No one person, fortunately, ever exhibits even a majority of them. Hysteria in one person is very different from the disease as it is exhibited in others. It is difficult, therefore, to give any one clinical picture of the entire dis- ease. It will be best to take up the chief symptoms as they occur independently of other diseases, or as they are known to complicate other functional and organic diseases of the nervous system. Hysteria is characterized by symptoms which point to a defect in the various systems and organs of the body. Thus we have : i. Psychic manifestations. 2. Motor manifestations. 3. Sensory manifestations and vasomotor disturbances. 1. Psychic or Mental Hysteria. — Properly speaking, every case of hysteria would come under this heading. For, if there is any one feature that distinguishes hysteria from other diseases, it is the defect of will-power and the excess of the emotional faculties. " I can't " is the pet phrase of all hysterical subjects, or still better, " I will not." It is not so much a direct lack of power to exert the will, * Moebius proposes to designate as " hysterical " all those morbid physical states which are engendered by concepts. HYSTERIA. 87 as a tendency to exert it in perverse fashion. This peculiar mental condition is easily recognized, and often leads to the diagnosis of hysteria in persons who have other symptoms pointing to a much more serious disease. But aside from this general hysterical state of mind there are other mental conditions which are very typical of hysteria. The most pronounced psychic form of hysteria, as observed in chil- dren, is that known as hysterical mania. Under great men- tal strain or excitement a child is seized with a crying or laughing spell, after which it passes into a state of nervous excitement in which, as in a little girl, aged eleven, under my observation, the child becomes violent, attempts to strike others, to injure herself, and to tear her clothes from the body, and to do all possible injury irrespective of conse- quences. Another condition, sometimes classed under the heading of hysteria, is observed in young girls, particularly at the age of puberty, and often ends in a condition of marked acute mania. I have seen a number of such cases in girls who were over-ambitious and eager to pass their school ex- aminations. They would keep up under the excitement of the examination, but immediately thereafter, whether suc- cessful or not, would become irritable, excitable, sleepless, would have laughing and crying spells by turns, would re- fuse to take nourishment, and eventually would either re- cover under proper treatment, or else pass into a condition of typical acute mania with absolute loss of reason, with in- tense excitement, and confused delirium. There would be no reason to consider these cases in this connection if they did not occur in children who have shown either a marked nervous predisposition, or who have exhibited hysterical symptoms of one kind or another at previous periods. Such children, if subjected to severe strain or severe emo- tional excitement, are very apt to pass into a condition of hysterical mania. Other mental conditions which cannot well be separated from hysteria, are those known as hystero-epilepsv, cata- lepsy, hysterical trance, and the like. Of these, hystero- epilepsy is by far the most important, and the gravest dis- order. Mills is inclined to consider this form of disease 88 THE NERVOUS DISEASES OF CHILDREN. very rare among children. In this he is undoubtedly cor- rect; but it has been my good fortune to see a number of classical instances of this special form in children, and the account I give is based entirely upon personal impressions. The gravity of hystero-epilepsy is increased by the fact that the children so afflicted are met with in mentally de- generate families. Insanity, epilepsy, chorea, chronic alco- holism, are the predisposing conditions in the ancestors of those who suffer from hystero-epilepsy. In one family I have during a period of ten years treated the mother for severe hysteria, a daughter at the age of nine for hysteri- cal convulsions, and another son and daughter for typical hystero-epilepsy. On account of this close relationship to true epilepsy, cases of hystero-epilepsy cannot be regarded with the in- difference which many physicians still display toward hys- terical subjects. In many cases it is difficult to decide whether the attacks as they occur are more hysterical or more epileptic ; and of the attacks occurring in one individ- ual some may be typically hysterical, while others may be typically epileptic ; and there is some danger in every case of severe hystero-epilepsy that with the progress of years the hysterical symptoms may vanish and true epileptic at- tacks may occur. It is of the utmost importance, before passing judgment upon any case, to determine whether the attacks are epileptic or hystero-epileptic. 2. Motor Manifestations. — Every variety of clonic and tonic movements occur in hysterical subjects. Many of these will be understood best by referring in detail to a few cases of typical hystero-epilepsy. Some seven years ago I was called to see a lad, then thirteen years old, who had been seized with violent con- vulsions during the night. I had been informed that these convulsions were preceded by great emotional excitement, caused by a severe upbraiding which the boy received for his misbehavior. After this little domestic scene was over the boy went to bed, and was seized with the first attack. During the attack he gave a shrill cry ; then began to bark like a dog, snapping at everyone who approached him, and would pass through the most severe contortions, touching HYSTERIA. »9 the bed at times only with the head and heels, the back be- ing- deeply arched as in the famous drawings of Richer representing this condition in women. After this the most violent jactations of the entire body occurred. During these convulsive movements he would snap, bark, and bite, then he would suddenly give a leap to the foot of the bed — almost tumbling out of bed — yet he always saved himself in time. This whole performance would last about two or two and a half minutes, then thorough relaxation of all the mus- cles would take place, he would fall back exhausted, and would then regain consciousness. During the attack there was no evidence of consciousness, at least no impression could be made upon him through any of the special senses. The patient often had as many as two hundred such attacks in the course of twenty-four hours ; he was sleepless, refused food, and became very much emaciated. After careful nurs- ing for a period of six weeks the attacks gradually lessened, and eventually he recovered entirely ; but he has shown since a deficiency in his moral and mental make-up, and although he has been cured of the hystero-epilepsy, it was found necessary to place him in a reformatory. This last fact is of some interest as showing the relation which hystero- epilepsy bears to degeneration of all the mental and moral faculties. Another case was that of a young girl, aged fourteen, who was much worried over the fear that she and the family would starve, as the father had daily drummed into the child's ears the necessity of economy and the difficulties of supporting a family in hard times. The young girl, the* daughter of a very hysterical and emotional mother, took the warning to heart, and for the first time in her life de- veloped typical hystero-epileptic attacks. Without warning- she would fall from a chair, from a sofa, or even on the street would pass suddenly into a condition of apparent un- consciousness. Wherever she lay she would pass through all sorts of contortions, would shriek, now and then would give agonizing yells, and would finallv end up by a pro- nounced crying spell. At other times she would become violent, tearing her own clothes, the bed-linen, and every- thing that was within her reach in the room. These spells 9 o THE NERVOUS DISEASES OF CHILDREN. HYSTERIA. 91 were repeated very frequently during a period of three weeks, and then gradually subsided. During all these at- tacks the movements seemed to be more or less purposive, and yet there was good reason to think, and the girl later on confessed, that she was absolutely unconscious of what she was doing at the time. These two cases will suffice as a general indication of the common form of hystero-epilep- tic attacks. The distinction between the attacks of epilepsy and of hystero-epilepsy is brought out in the following table : Epilepsy. Aura frequent. No distinct cause for each attack. Hystero-Epilepsy. No aura ; but, Some emotional excitement, direct cause of attack. Onset sudden or gradual. Noises of all sorts during attack. Onset sudden. Initial cry. Eyes open or closed ; pupils often Eyes turned up ; pupils normal ; often ecstatic expression of coun- tenance. Either tonic rigidity of muscles or extravagant movements (some- times purposive). No impairment of vesical and rectal reflexes. Attacks last much longer, followed by a condition of trance, or else patient recovers consciousness as soon as convulsive movements cease. The patient falls softly, without per- sonal injury. Biting of the tongue rare. Patient may pass into condition of trance, or may exhibit signs of fa- tigue ; recovery often rapid. dilated widely ; do not react ; roll- ing of eyes upward and inward. Tonic and clonic convulsions. Involuntary micturition or defeca- tion. Duration of attack only a few min- utes. The patient often injures himself. Biting of the tongue. After cessation of clonic movements, stupor or somnolence. In addition to the typical attacks of hystero-epilepsy there are other forms of motor disturbance due to hysteria. The hystero-epileptic attack, while it is the gravest, is in fact much rarer than the ordinary hysterical convulsion. The latter is characterized by a temporary and imperfect loss of consciousness ; by irregular, though sometimes violent, twitchings of all extremities, sometimes by the repetition 92 THE NERVOUS DISEASES OF CHILDREN. of one special form of convulsive movement, such as re- traction of the head. When these various convulsive move- ments are over, the muscles pass into a state of tonic rigid- ity, after which rapid recovery takes place. The entire episode is wound up by a fit of crying or laughing, or by some other expression of an emotional character. Some of the patients pass into a condition of melancholy which may last for hours, or into a state of exaltation and even of ec- stasy. This is as true of the hysterical attacks as they oc- cur in children as it is of those that occur in the adult. These attacks can be distinguished from the hystero-epilep- tic and from epileptic seizures by the incomplete loss of consciousness, by the absence of all regular rhythmical convulsive movements, and by the fact that there is neither biting of the tongue, nor involuntary micturition, nor any of those symptoms which are often associated with hystero- epileptic and epileptic attacks. Other hysterical attacks consist of spasm of the head and neck muscles, the well- known spasm of the oesophagus which gives rise to the sensation known as globus hystericus, or to difficulties in deglutition if the spasm affects the lower part of the gul- let. This oesophageal spasm which occurs in hysterical and otherwise neurotic children is a symptom which has not met with the appreciation which it deserves. If a sound is passed into such a gullet it will be seen that there is an actual muscular spasm at the point of constriction, and that it requires considerable patience, and sometimes a little force, to overcome the contraction ; but the age of the child and the fact that the constriction easily disappears, to return again under the slightest emotional excitement, is sufficient to remove any fear of the constriction being due to an organic cause. In this same category we may place spasmodic movements of the diaphragm, of which singultus is the common manifestation. This special phenomenon is much more frequent in later periods of life than during the period of childhood ; but 1 have had under observation an entire family afflicted with this special form of hysteria who upon the slightest provocation would exhibit this symptom. On one occasion I examined the mother, in the presence of two daughters, respectively twelve and ten HYSTERIA. 93 years of age. Under the excitement of the examination the mother was seized with severe singultus, and before I had completed the examination both the daughters were favoring me with a similar exhibition. The scene would have been a comical one if it had not brought home to me the powerful influence of example and suggestion, if not of heredity, in such cases. Spasm of the bladder, increased peristaltic action of the stomach and bowels resulting in diarrhoea, upon the least emotional excitement, fits of crying or laughing, of sneezing and of coughing, are the various forms of hyster- ical spasm met with in children, as well as in persons of more advanced age. If the hysterical seizure, or better said, the hysterical spasms, assume a more extravagant character, and if it affects a larger number of the groups of muscles that generally act in unison with one another, we may have that peculiar condition which is known as cJiorea major. In this form the jactations are often severe and ex- hausting. The child or young person assumes the most im- possible positions, often suggesting the intention of dra- matic effect. From this condition it is but a short step to a condition of mild tonic contracture, the limbs retaining any position which may be given them. This state of cata- lepsy is very often witnessed in conjunction with chorea major, sometimes independently of it, but it is a mistake to assume that catalepsy occurs exclusively in conjunction with hysteria. It occurs in connection with various forms of mental derangement, and a condition not unlike cata- lepsy is met with in some forms of infantile cerebral paraly- sis. In hysteria the muscles of the body are not only sub- ject to convulsive movements but also to absolute paral- ysis. There is not a part of the body that may not be the seat of such palsy, but it is most frequently mani- fested in the extremities, in the tongue, and in the vocal cords (hysterical aphonia). In some instances there is no absolute paralysis of any one muscle or group of muscles, but certain functions are paralyzed. The best known ex- ample of this is the form which has recently been described as astasia-abasia. A person thus afflicted is neither able to 94 THE NERVOUS DISEASES OF CHILDREN. stand nor to walk, but is perfectly well able to use all mus- cles while lying on the back. Many have claimed that this is simply due to a psychic condition dominated by the idea that standing or walking is impossible. This is in all probability the proper interpretation ; but the point that concerns us here is that it rarely, if ever, occurs except in those who exhibit other symptoms of hysteria. Hysterical aphonia is very frequent in children, and par- ticularly during the period of development. The hysteri- cal character of the aphonia is easily recognized by the fact that (as the laryngoscope reveals) there is no organic cause for the loss of voice, and that the single attacks come on very suddenly, as a rule, in the wake of some nervous ex- citement, and disappear as rapidly as they have appeared. I have had young girls come to my consulting-room who would not be able to speak above a whisper, and it has al- ways been a special pleasure to have them sing the entire scale before leaving the room. A strong faradic brush ap- plied to the neck over the trachea is the most persuasive master in these cases. Hysterical mutism, an absolute loss of speech, is generally the result of severe emotional ex- citement. In some instances it is associated with delusions, and with other symptoms of mental derangement. Other forms of hysterical paralysis are not so easy to diagnosticate, and in order to be able to differentiate them from paralysis due to organic causes the physician must have all neurological facts and doctrines at his command. It is indeed one of the most difficult problems in neurology to distinguish hysterical from organic forms of paralysis, but this distinction can be made safely enough if the fol- lowing points are kept in mind : Hysterical paralysis does not as a rule adhere to anatomical distribution, with the one exception that an hysterical hemiplegia may be quite as complete as any hemiplegia due to an organic disease. But I have not yet met with a single case of hysterical hemi- plegia in a child that has in any way suggested, even for a moment, the possibility of a hemiplegia due to an organic lesion in the brain. In hysterical paralysis the paralysis, as well as the anaesthesia which is associated with it, is apt to be regional ; thus we have a paralysis of the hand, or a pa- HYSTERIA. 95 ralysis of hand and forearm, or paralysis of an entire extrem- ity, with an anaesthesia that, as a rule, develops in propor- tion to the paralysis. This association of regional anaesthe- sia and regional paralysis is, to my mind, very characteristic of hysteria. From cerebral palsy, hysterical paralysis can be distin- guished by the fact that it is not accompanied by increased reflexes ; and by the lack of marked sensory changes in the majority of cases of brain paralysis. The deep reflexes may be lively in cases of hysterical paralysis, but I have never seen them markedly exaggerated, nor are they accompanied by the spasticity and tonic contractures which are so fre- quent a symptom in the case of paralysis due to brain dis- ease. The presence of ankle clonus was at one time sup- posed to prove the non-hysterical character of an affection ; this may be true in the majority of cases, but since I have met with the presence of ankle clonus in some hysterical, and even in neurasthenic, affections, I cannot attach much importance to this one point. The electrical reactions, it should be remembered, are normal both in hysterical and in cerebral diseases ; we cannot therefore utilize them in any way in making the differential diagnosis. If the question arises whether paralysis of the upper or lower extremities (more frequently of the latter) is due to spinal disease, or whether it be purely hysterical, the diag- nosis should be based upon the absence of vesical and rec- tal symptoms in a case of hysterical paralysis, though to be sure there are many cases of spinal disease too in which these are not affected. Retention of urine may occur in cases of hysteria, but it is only of short duration as a rule, and is rarely accompanied by the symptoms of cystitis, as is the case in a large number of spinal palsies. In many cases of spinal and peripheral nerve disease the electrical reac- tions are altered, and the reaction of degeneration may be present. This is not the case in hysteria ; but these elec- trical reactions are normal also in all those cases of spinal disease which are due to disease of the lateral columns and not of the posterior columns, or of the central gray matter. All these points of differential diagnosis may in some cases prove insufficient, and the diagnosis of hysteria or of 96 ' THE NERVOUS DISEASES OF. CHILDREN. organic disease of the nervous system will depend upon the general agreement or disagreement of symptoms. If the physician is aware that flaccid paralysis of a single group of muscles, or of one or more extremities, is generally as- sociated with changes in the electrical reactions, and with loss of reflexes in case these symptoms are due to organic disease, and if he remembers, furthermore, that spastic forms of paralysis are associated with increased reflexes, with nor- mal electrical reactions, and with normal sensations, he will have little or no difficulty in arriving at a correct diagnosis. Hysterical paralysis is subject, moreover, to greater changes than the organic palsies are. While under certain condi- tions the very persistence of the symptoms for a long period of time, followed by a sudden change, is extremely char- acteristic of hysterical paralysis, the diagnosis becomes still more certain if the hysterical paralysis is associated with other symptoms, such as are known to be purely hys- terical. I refer particularly to the presence of rigidity, anaesthesia, or typical and complete hemianassthesia, and to the presence of major or minor hysterical attacks ; but the difficulties are still further increased by a fact to which Seguin called particular attention some years ago, that hysterical paralysis or hysterical symptoms often compli- cate organic disease. A characteristic case is that of a young girl, aged thirteen, who some years ago had acquired a typical poliomyelitis. The sudden onset, the in- volvement of the anterior tibial group, the flaccid and atrophic paralysis, the presence of the reaction of degeneration, the absence of the knee-jerk placed this diagnosis beyond a doubt. Two years after the onset of the poliomyelitis the young girl was seized with a complete paralysis of the fore- arm and hand of the same side. The arm could be raised a little at the shoulder with flexion at the elbow ; flexion at the wrist or any movement of these parts was impossible. There was at the same time an anaesthesia ex- tending from the tips of the fingers of this right hand to a line drawn around the arm at about one and a half inch below the elbow. Every form of sensation was absent in this part. The hand and forearm showed no ten- dency to atrophy, the electrical reactions were entirely normal, but the hand remained stationary in the position resembling that practised by the accou- cheur (a favorite position in hysterical subjects). There was no increase of the reflexes in the upper extremity, and there were no symptoms pointing to any organic lesion, either of the brain or spinal cord. There was no reason therefore to hesitate in making the diagnosis of hysterical paralysis, and I HYSTERIA. 97 have on many occasions exhibited this young girl to my students as an ex- ample of a patient who exhibited both an hysterical and an organic affec- tion. Another case will illustrate the same point. A young girl, aged fifteen years, was brought to my clinic, with a history of a fall upon the arm, causing a dislocation of the head of the humerus, a year previously. The arm was set, but marked paralysis, with excessive pain along the median and ulnar branches, remained. The pain disappeared, but in spite of every form of treatment the paralysis would not yield. The girl was surrounded by an over-sympathetic mother and very loving sisters, who insisted on providing her with pillows and all sorts of appliances in order to protect her against pain, and the arm against injury. The family had made her a confirmed in- valid. At the time of my examination I found that the arm was but very little atrophied, that there was no objective disturbances of sensation, that the electrical reactions had returned to an almost normal state, the faradic and galvanic excitability of the median and ulnar nerves still being somewhat diminished ; but the galvanic reactions of all the muscles were good, their contractions were prompt, and the formula was not altered in any muscle, yet the paralysis stubbornly persisted. It was evident that an hysterical form of paralysis had been superimposed upon a paralysis which was origi- nally due to a peripheral neuritis. The separation from the family, the en- couragement given her by a competent nurse, the use of electricity and of hydro-therapeutic measures, brought about a most marvellous improvement in less than two weeks — a change which could never have been effected so quickly by these same remedies if the paralysis had not been of an hysterical character. French authors have reported a number of cases of extreme progressive wasting of muscles in hysteria. Hirt has recorded a case in a young girl, so astounding in the rapidity and degree of development of the atrophy that it almost challenges belief. 3. Sensory Symptoms. — Disturbances of sensation con- stitute a very characteristic symptom in the majority of cases of hysteria. As has been intimated in the preceding pages the sensory symptoms often enable one to make a positive diagnosis of the hysterical character of the affec- tion when other symptoms would leave some room for doubt. Every form of sensation may be affected, and it may either be exaggerated or diminished. Hysterical hyperassthesia and hysterical anaesthesia are of common occurrence. If hyperassthesia exist there is an unusual sensitiveness to the slightest touch or to the slightest pain- ful impression. This hyperesthesia is most prominently developed in the region of the ovaries, in the skin over the 7 98 THE NERVOUS DISEASES OF CHILDREN. spinal column, each spinous process being so sensitive to touch that the patient cries out from pain, however light the touch may be. The hyperaesthetic areas, if stimulated, are very apt to cause distinct hysterical attacks in those prone to such seizures, and the hypersesthetic areas may become true hysterogenic zones. These hysterogenic zones have been studied with great care by the French school, but we cannot enter upon the subject more fully here, and leave it with the simple statement that there are some hysterogenic zones which are not hypersesthetic. Hysterical anaesthesia is still more frequent than the exaggerations of sensor)' impressions. The anaesthesia may not only include every form of ordinary sensory per- ception but also the special senses. The distribution of this anaesthesia is one of the most valuable signs of hysteria. It occurs in the form of a complete hemianaesthesia, or in the form of a regional anaesthesia. The hemianaesthesia is limited strictly to one-half of the body, but in this half it is often absolute, and the completeness of the anaesthesia is the very feature which should make us suspicious of its hysterical nature. If a girl or a boy, or for that matter any person at any age, present absolute anaesthesia to all forms of ordinary sensation, and in addition exhibits blindness of one side or a typical hemianopsia, deafness, loss of taste, and of smell in the same half of the body, such a person has undoubtedly hysteria. In this country hysterical hemianaesthesia is relatively rare, excepting in the Russian and French elements of our population, but in these it can be studied easily and satisfactorily. If an entire half of the body is not anaesthetic, the re- gional character of an anaesthesia is sufficient to lead one to the diagnosis of hysteria. By regional anaesthesia I mean anaesthesia of a well-marked division of the body, say of the hand, of the forearm, of the leg, the hips, or of circum- scribed areas in any part of the body without reference to the anatomical distribution of the sensory nerves. (Fig. 43.) As this regional anaesthesia is frequently associated with a regional paralysis the hysterical nature of both becomes very evident. The hysterical nature of the anaesthesia is not only made evident by its peculiar manner of develop. HYSTERIA. 99 ment, but also by the manner in which it often disappears. In a number of cases in which I have studied this anaesthesia carefully it would recede inch by inch, but always main- tained a certain level in the arms or legs without any ref- erence to the well-known sensory areas. Hysterical pa- tients do not present anaesthesia of the anterior or posterior surface of a limb, but the anaesthesia generally involves both surfaces. The hysterical nature of sensory disturb- ances is also established by the powerful effect of hypnot- FlG. 43.— The Three Types of Distribution of Anaesthesia in Hysteria : Hemianaes- thesia, Segmental, and Disseminated. Z, Hysterogenic Zones. (Dana.) ism, and of mere suggestion, which is often sufficient at least temporarily to dislodge a complete anaesthesia. The anaesthesia may furthermore be transferred from one limb to another, not merely by the action of magnets, or bv the action of metals as was formerly supposed, but, I am con- vinced, by any form of suggestion powerful enough to pro- duce a strong psychic impression. It is on the theory of suggestion that we must explain the miracles ascribed to magneto-therapy or metallo-therapy, that played such an important role some ten or fifteen years ago in France. Of the special senses vision is most frequently subject to hysterical disorder. In some there is true photophobia. in others a diminution of visual perception is more common. 100 THE NERVOUS DISEASES OF CHILDREN. and the patient may indeed be unaware of the existence of such diminished visual sensation, which is the best proof that it is not intentional or simulated. The retina may be entirely insensitive to light, there may be distinct limita- tion of the visual field, or there may be a complete loss of every form of visual perception in one eye. (Fig. 44.) Bilateral loss of sight is said to occur in hysterical patients, but as a rule it is simply transitory. I have not had op- portunity to see this special visual defect in children. Visceral hysteria deserves a passing notice ; the paral- ysis of the bladder and increased peristaltic action of the o. s. O. D. Fig. 44. — Hysterical Loss of Color Sense and Limitation of Visual Field. Color Sense Absent in Left Eye and Field Contracted ; in Right Eye Field less Con- tracted ; Order of Appreciation of Colors from Without In was Yellow, Violet, Blue, Red, Green. (After Peck, from Dana.) stomach and bowels have been mentioned in connection with the motor disturbances of hysteria, but there are two other forms of hysterical manifestations that are worthy of further mention. The one form is hysterical anorexia. Patients in this condition absolutely refuse food, and if they take it, vomit it at once. In some instances this is accom- plished without effort on the part of the patient and seems entirely unintentional. In other instances, again, the pa- tient deliberately sets to work to eject the contents of the stomach, and does not give up the effort until she has suc- ceeded. In my service at the Montefiore Home I frequent- HYSTERIA. 10 1 ly had occasion to witness the antics of an hysterical girl, aged fifteen, who immediately upon taking food would go into a corner of the room, begin violent gagging movements, and would succeed within a period of three or four minutes in ejecting the entire contents of the stomach. In this girl, who was not possessed of any insane delusion regarding the character of the food, the act seemed purely volitional, and it was a surprising fact that like many other hysterical patients with anorexia, she was not so much reduced in her physical condition as a person would have been who had persistently starved herself. She evidently obtained food and retained it ; but how she got it and when she took it it was difficult to determine, since she was watched care- fully night and day. She was treated by rather heroic measures in the form of forced feeding and hydro-thera- peutic measures ; she was allowed to leave the institution after she had shown her willingness to take food and re- tain it. Distention of the stomach and bowels, representing a total paralysis of the muscular walls of the stomach and in- testines, occurs frequently enough, and more particularly in children. The stomach is apt to be distended to an enor- mous degree, and the bowels dilate in such a way as to give the appearance of the abdomen in a case of large-sized tumor. I have in mind the cases of twin brothers who pre- sented the following peculiar condition : The one brother, at the age of fourteen, was true to the neurotic stock from which he descended by suddenly developing a tremendous meteorismus, which increased from day to day until the abdomen was so distended that the skin seemed ready to burst. There was some anorexia and constipation. The constipation was occasionally relieved, with the result of temporarily diminishing the distention of the bowels, but in a very few hours the intestines would resume their for- mer state. Every possible measure was resorted to, to re- move this distention, but it persisted stubbornly for some weeks, when finally, after the introduction of large enemata of an infusion of valerian, the distended abdomen suddenly collapsed. A few days later the twin brother of this patient began the same performance, and went through exactly the 102 THE NERVOUS DISEASES OF CHILDREN. same experiences. The abdomen became distended to almost the same degree as in the brother, and after it had lasted about the same length of time his abdomen also collapsed in very much the same way. These boys not only came from very neurotic stock, but had been very much pampered in their early training, which fertilized the soil that had been prepared for the development of every form of hysteria. Diagnosis. — It would be impossible to state the points of differential diagnosis between hysteria and other affec- tions without repeating everything that has been said in the preceding paragraphs. It is worth while stating once more that the diagnosis of hysteria should be made only in case an organic affection can be positively excluded, and if the well-recognized symptoms of hysteria, particularly hys- terical seizures and hysterical sensory changes are present. Let the student also bear in mind that hysteria and hypo- chondriasis are not one and the same disease, though one is often mistaken for the other. If a boy is fearful of a dis- ease with which he supposes he is afflicted, we have no hesitation in saying that he is a hypochondriac ; but let a girl exhibit exactly, the same symptom, and she is at once put down as an hysterical subject. To distinguish between the two conditions is not always an easy matter, but in hypochondriasis the patient is generally possessed of a few notions regarding his own bodily condition, and these make such a deep impression upon him that his entire ego is af- fected by it. His supposed affliction is constantly before his mind, and dominates his entire being. In cases of hys- teria there is no such introspection as in hypochondriasis. The bodily ailments or bodily peculiarities are far more numerous, but they are subject to greater changes, disap- pear for a time and then return again, and are not pushed to the fore quite as much as in cases of hypochondriasis. In the latter, moreover, the depression is, as a rule, greater than in hysteria, and in hysteria there are other symptoms which prove the presence of an hysterical affection. The difficulties of a differential diagnosis are still further in- creased by the fact that a mixture of the two conditions (hysterical hypochondriasis) is not uncommon, and it must be left to the physician to determine, by a close analysis HYSTERIA. 103 of the symptoms, whether there is more hysteria or more hypochondriasis in the symptoms which the patient pre- sents. Pathology. — In discussing the pathology of hysteria there is much room for theorizing, but there are very few facts to guide us. That it is a functional disease is con- ceded on all sides; but there is no other disease in which the loss of function may be so absolute as in hysteria. By some mechanism or by some influence which we cannot yet understand, an entire hemisphere is temporarily invali- dated, or else we could not explain the typical and com- plete hemianassthesia so common in hysteria. Meynert endeavored to give an anatomical explanation, but failed. How this loss of function of one hemisphere is effected, and whether it is similar or not to the occurrences that take place under hypnotic influence, we are not prepared to say. The transfer of sensory disturbances from one-half of the body to the other, would go to show that the two halves of the brain are evidently in sympathy with one another (to put it broadly) in this disease, and that they can be al- ternately affected. The highest centres are evidently im- paired in hysteria, cortical inhibition is removed, and the lower centres have full sway.* It is quite in keeping with this view that even the reflexes are frequently exaggerated in hysteria as they are in organic disease in which the changes in the motor fibres of the pyramidal tract interfere with the proper transmission of cortical influences. We cannot properly speak of the morbid anatomy of hysteria, for the entire conception of the disease would have to be altered if we could suppose the existence of such. Some post-mortem findings have been reported, but they were evidently accidental complications. Duration and Course. — Once hysterical always hys- terical, would seem to be the general opinion with regard to these subjects, but this is not quite accurate. The mani- festations of this disease often disappear for years, and it is * Breuer and Freud have attempted a psychological explanation of hysterical phe- nomena and have ascribed these to a " hypnoid " condition in which there is a division of consciousness, and a limitation of the power of association. Freud has directed at- tention to the diminution in the strength of concepts in hysterical subjects, and to the ready change from psychic to physical innervation. 104 THE NERVOUS DISEASES OF CHILDREN. one of the commonest experiences to record the disappear- ance of an hysterical paralysis or of an hysterical anaes- thesia. The tendency to relapses is extremely great, and symptoms that have disappeared for years may occur un- der any emotional excitement. Treatment. — The treatment of hysteria must be con- sidered with reference to the hysterial predisposition and with reference to the special hysterical symptoms. The hysterical predisposition is unfortunately either inherited from a neurotic ancestry or it has been specially fostered by an hysterical environment. In hysteria, as in other men- tal affections, I have often doubted whether heredity is the marked factor that it is generally supposed to be, or, if we grant the hereditary tendency, whether the hysterical man- ifestations would be as frequent as they are if a serious at- tempt were made to change the surroundings of such chil- dren. In the majority of cases the early training has been defective, and an example constantly before the child of an hysterical mother or other hysterical relatives is sufficient to engender the disease in its fullest form. The first and most important principle of treatment, therefore, is the ab- solute separation of the child from the family. It is a great pity that this is so rarely urged by the physician, and still more rarely permitted by the parents. Only the more in- telligent parents can be made to understand that an utter stranger, if properly qualified, may train a child far better than its own mother can. At all events there is nothing in the treatment of hysterical children as important as plac- ing them under the influence of a sober-minded, intelligent nurse or teacher who will devote herself or himself to study- ing the peculiarities of the child, and who will make a seri- ous attempt to foster the good qualities and to counteract the vices. If this course that has been suggested is per- sisted in, it should be followed not for weeks or months but for years, and I am confident that if this is done during the formative period of a child's mind and character, an excel- lent result will follow in many instances. I base this upon my own experience, and have often stated to parents that if they will not allow me to pursue such a plan of treatment I prefer not to attempt any. HYSTERIA. 105 The Weir-Mitchell treatment, which is so effective in many cases of adult hysteria, is also of the greatest value in the hysteria of children ; but I consider it to be one of its special advantages that it presents the first and easiest op- portunity for the separation of the child from its immediate surroundings. Select a nurse carefully, place the child en- tirely in her charge, and many a parent will soon be con- vinced of the good that comes from such separation. In addition to the moral influences which may be exerted in this way the physical health of the child can be looked after. Bad habits of diet and of general hygiene can be corrected, and a child whose general physical condition has been far below par up to the time of its entering upon the rest cure, may be turned into a strong and vigorous be- ing. Drugs, I am very certain, have but little influence over such conditions. The usual hysterical remedies, such as asafcetida, valerian, and the like, may be employed, but they will accomplish little which cannot be accomplished by other means as well. If the effect of a drug depends upon its disagreeable taste, suggestions of a different char- acter will prove quite as efficient ; and above all the phy- sician should avoid the possible risk of engendering the idea in a growing child that there is a special drug which it may resort to for any annoying or painful sensation. There is nothing more disgusting than the habit so freely practised by many physicians of giving valerian or asafce- tida or morphine to children or adults, whenever they pre- sent symptoms which smack of hysteria, or which cannot be interpreted otherwise by the attending medical adviser. The special symptoms of hysteria call for distinct forms of treatment, but whatever these special symptoms may be, I always consider it wise to bear the general hysterical condi- tions in mind, and to employ, in addition to the special treat- ment, those general measures which have an excellent invigo- rating effect upon the nervous system. I refer more particu- larly to the proper use of hydrotherapeutic measures. The treatment of hysterical aphonia was referred to above. Sim- ple faradism is all that is needed in most instances, and if this is not sufficient, regular vocal exercise in the hands of an in- telligent teacher or nurse, will bring about the desired effect. 106 THE NERVOUS DISEASES OF CHILDREN. I have never found it necessary to apply the faradic current to the inside of a throat, but of course would not hesitate to do so in case the external application did not prove suf- ficient. Blistering or the use of the static machine act as powerful forms of local suggestion. In hysterical paralysis of the limbs, the use of a strong faradic current is gener- ally the most effectual remedy. The current applied know- ingly, not mercilessly, will gradually induce the patient to attempt similar contractions ; and if to the use of the faradic current be added the encouraging words of the nurse or physician, who should not, however, accuse the child of simulation, and if other measures, such as massage, be em- ployed, the hysterical paralysis will disappear in the course of time. But many of the cases are extremely stubborn and may require more vigorous measures, or more power- ful suggestion, before they yield to treatment. It is of some importance at times to prove to the child that it can use its limbs very much better than it supposed. Place it in the middle of a room quite by itself, make it stand or crawl or walk, and if once shown that it can do so, the para- lytic symptoms may rapidly disappear. But the treatment of hysterical patients of all ages and of all classes demands unusual tact and patience on the part of everyone con- cerned in the treatment of the case. The sensory disturbances of hysteria are best influenced by the use of the cold douche or of the faradic current, particularly by the employment of the faradic brush. If such measures as I have suggested are not sufficient to re- move an hysterical paralysis or an hysterical anaesthesia the effect of suggestion, hypnotic or otherwise, may be attempted. The visceral disturbances common in hysteria also de- mand special treatment. Some of the measures to be em- ployed were referred to in connection with the hysterical twins mentioned above. In cases of hysterical anorexia patient efforts should be made to induce the patient to take small quantities of food, and if these are not retained forced feeding must be resorted to ; but never, if you can avoid it. resort to rectal feeding, for the patient who has discovered that she can be fed in that way will continue to refuse food HYSTERIA. 107 very much longer than she would otherwise. Lastly, the treatment of hysterical attacks may be managed in very much the same way as other hysterical manifestations are treated. First of all, the patient should receive the general anti-hysterical treatment, and should be given some drug which will act as a powerful irritant or a powerful form of suggestion when the attack is imminent. 1 have found nothing better than a sudden douche of cold water, or cold flagellations on the head and chest, or the inhalation of ni- trite of amyl. The latter has this to recommend it, that it may be used with all the more assurance in those cases in which the suspicion of epilepsy cannot be altogether ex- cluded. Lavender and ammonia are also efficient in some cases in which the hysterical attack can be inhibited by any such simple measure. If an hysterical attack continue for a prolonged period of time it may be brought to an end by the brisk use of cold douches, by the application of a strong faradic current, or by pressure over the ovaries, after the age of puberty, if these organs be oversensitive. BIBLIOGRAPHY. For detailed information see the text-books of Grasset, Henoch, Hirt, and Gowers. Babinski : Atrophy with Hysterical Palsies, Arch, de Neurologie, July, 1886. Bourneville : Progres Medical, 1880. Breuer u. Freud: Neurologisches Centralblatt, 1893, Nos - l and 2 - Bussard : Archiv der Phys., 1887 ; Neurolog. Centralbl., 1893, I. and II. Bruns : Review of Traumatic Forms, in Schmidt's Jahrb., 1892 and 1893. Charcot : Lecons du Mardi a la Salpetriere. Paris, 1889. And in numerous other publications : Progres Me'dical, 1882. Dana : American Journal of the Medical Sciences, 1892. Freud: Neurologisches Centralblatt, 10 and 11, 1894. Gillette : New York Medical Journal, 1882 (in a child of eighteen months). Gilles de la Tourette : Treatise on Hysteria. Paris, 1S91. Hirt: Deutsche Med. Wochenschr., 1894, Extreme Muscular Atrophy in Hysteria. Jacobi : American Journal of Obsterics, 1876. Janet : Archives de Neurologies, 1893, XXXV.-XXXVI, Jolly: In Ziemssen's Cyclopaedia, and Berl. Kl. Wochenschr.. 1892. No, 34. Lowenfeld : Neurasthenie und Hysteric Monograph. Wiesbaden. 1804. 108 THE NERVOUS DISEASES OF CHILDREN. Mills : Article in Keating's Cyclopaedia (with ample references). Moebius : Ueber d. Begriff d. Hysterie, Centralblatt fur Nervenheilkunde, 1888 ; also Neurologische Beitraege. Part I. Remak, E. : Berl. Kl. Wochenschr., 1892. Riesenfeld : Hysterie bei Kindern. Inaug. Diss. Kiel, 1887. Tuczek: Berl. Kl. Wochensch., 1886, No. 31. Weir Mitchell : Lectures on Nervous Diseases. CHAPTER V. CHOREA. Among the neuroses of childhood none is worthier of careful study than chorea. The name is made by some to include a number of varying conditions, but its use should be restricted to designate a functional disease characterized by irregular, involuntary twitchings of some or all of the muscles of the body. These movements cease, as a rule, during sleep. Synonyms. — This neurosis is also known as St. Vitus's Dance and the Chorea of Sydenham. Both these names deserve to be used, since the former implies its historical origin, and in the latter is preserved the name of the famous English physician who first described its most characteristic symp- toms. Scelotyrbe and Melancholia saltans, are terms occasionally used, though almost obsolete. In German text-books the disease is called Chorea Minor, in contradistinction to Chorea Major, a neurosis of a purely hysterical character.* Etiology. — Chorea is distinctly a neurosis of childhood and early adolescence. The vast majority of cases begin in very early youth, though Sinkler, a few years ago, reported two cases in persons over eighty years of age. Careful sta- tistics have been gathered with reference to this disease, the most elaborate being those reported by Dr. Stephen Mackenzie, in 1887, f° r tne British Medical Association Col- lective Investigation Committee. Of four hundred and thirty-nine cases reported by this committee thirty-four per cent, occurred between the ages of five and ten Years, forty- *The name chorea, Greek x°P eia , can be traced back to the dancing mania of the middle ages. During a severe outbreak of this psychic disturbance in Strasburg, in the early part of the. fifteenth century, the chief magistrate of that city ordered those affected with this dancing mania to repair to the chapel of St. Vitus, in Zabern, a small village not far from Strasburg. The name, St. Vitus's Dance, is the only point of affin- ity between the dancing mania of old and the typical chorea of the present day. IIO THE NERVOUS DISEASES OF CHILDREN. three per cent, between the ages of ten and fifteen years, and sixteen per cent, between the ages of fifteen and twenty years. The largest number of attacks were found to occur in the thirteenth year of life. Some cases, however, oc- curred very much earlier than this average percentage would indicate. I have seen several cases of genuine chorea in children under one year of age, and many more in children up to the age of three years. That chorea is also occasionally congenital must be admitted ; Sinkler re- fers to a case of this description due to fright of the mother during pregnancy. That there is hereditary predisposition to chorea is also evident, for the disease is developed more readily in children of choreic mothers and also in those whose parents are afflicted with epilepsy and migraine. All authors are agreed as to the greater liability of the female sex. Within the last three years I have seen 184 cases of chorea, in which 136 were females and 48 males. Sinkler among 328 cases reports 232 females and 96 males. Gowers, who has tabulated the largest number of cases, finds but 365 boys among 1,000 cases. Dr. Weir Mitchell has studied the relation of races to the development of chorea, and claims that the negro race is almost exempt. I have no means of saying whether this is approximately true, but from my own experience I can assert that it is by no means rare among that race, but that among them it is if anything more frequent in boys than in girls. The disease is very common in Hebrews, as are man) 7 other neuroses. Climate seems to exercise but very little influence upon the development of the disease proper, or upon the causa- tion of the individual attack ; it occurs quite as freely in cold countries as in warm, in northern as in southern lati- tudes, but there is an undoubted seasonal influence. The great majority of attacks occur in the spring. Drs. Mitch- ell and Lewis have made an elaborate research on this point, and claim that it is not so much variation in temperature or in humidity that causes the attacks, but that there is a decided correspondence between the number of attacks of chorea and the number of rainy and cloudy days ; and then again between the attacks of chorea and the number of storm- CHOREA. Ill centres that pass over Philadelphia. Too much reliance should not be placed upon these statements until confirmed by similar researches in other cities. Putnam could not trace the same influences in Boston, and Gowers cannot accept the conclusions of Lewis for the conditions under which chorea occurs in England. These factors, if power- ful at all, are of more importance in bringing about a recur- rence of attacks that in giving the first impetus to the dis- ease. The causes which lead most directly to the development of St. Vitus's Dance are (in the order of their importance) fright, various acute diseases, such as articular rheuma- tism, scarlatina, and cardiac disease which so often ac- companies the acute affections just mentioned. Gowers is inclined to regard fright as " the only immediate cause that can be traced with any frequency." It was the direct cause of chorea in 56 of my 184 cases. As a rule, the first symp- toms of chorea appear within a few days of the fright ; in some cases within a few hours, and even within the first hour. I had under observation for a long time a child that lived near the Brooklyn Theatre at the time it was destroyed by fire ; the child was startled by the sight of the flames, and within a few hours began to twitch, and soon developed a severe attack of chorea which lasted for months. In one case the sight of a street brawl, in another the sight of a dead body, was directly the cause of the choreic attack. In chil- dren who have once had chorea very trivial occurrences are apt to bring about a recurrence. I have known a slight overstrain at. school, the unexpected report of a pistol, a severe thunderstorm, or a scolding by a parent to be suf- ficient to bring on an attack. The relation of acute rheumatism to the development of chorea has always been a matter of dispute. Some claim an absolute causal relation between the two, others insist that it is nothing more than a coincidence if one disease follows upon the other. Statistics upon this point are not so satisfactory as they might be, for cases have been reported in which it was stated that acute rheumatism has occurred, but it is not clearly made out that the rheumatism actually preceded the onset of the chorea. Sec reported the occur- 112 THE NERVOUS DISEASES OF CHILDREN. rence of acute rheumatism in about one-fourth of his cases, and in these figures he is supported by the conclusions of the British Medical Investigation Committee and by Gowers.* It is a curious fact the rheumatism does not seem to pre- cede chorea nearly so frequently in earlier years as it does in those cases which occur between the ages of ten and fif- teen years. From this we must infer that accidental coin- cidence plays a very much greater role than many are will- ing to concede. This must unquestionably be the case with the statistics furnished by Dr. Hamilton, who found twenty per cent, of all school children choreic or affected with some similar disorder. 1 could find a satisfactory history of the chorea coming on after rheumatism in only 20 of 184 cases.f Hirt, in his recent text-book on Nervous Diseases, thinks that there is a common toxic agent which, if it affects the cor- tex, will produce choreic movements ; if it affects the joints chiefly, will give rise to acute rheumatism. This may be a rather hasty conclusion, but there is enough truth in it to say that the development of chorea is more probable in per- sons who have had rheumatism than in those who have not. The majority of recent writers, including Herringham, Mackenzie, Bouchard, Gowers, Osier, and others insist that an intimate relation exists between chorea and rheumatism ; but the question arises whether heart disease, which is so frequently associated with rheumatism, may not be in part responsible for the development of chorea. Statistics, in order to be satisfactory, should be collected very much more carefully than they have been hitherto, in order to prove in how few, or in how many, cases of chorea the first attack has been preceded by rheumatism or cardiac disease. We can readily understand that fright should be a more powerful agent for evil in cases in which cardiac disease has preceded the existence of chorea. I have found satis- factory evidence % of cardiac disease preceding the devel- * Starr has tabulated 2,476 cases (by various authors) ; in 662 (twenty-six per cent.) there was a history of preceding rheumatism ; in 502 there was cardiac disease. t In his monograph (which appeared while this chapter was going through the press) Osier states that there was a history of rheumatism in 15.8 per cent, of cases which he had examined carefully. + The mere existence of a murmur has been taken by many as evidence of cardiac disease ; in the fewest reports is there any accurate diagnosis of the cardiac condition. CHOREA. I I 3 opment of chorea in only 20 of 184 cases. The bearing that heart disease may have upon the pathology of chorea we shall have occasion to refer to later on. In this treatise we need not consider the chorea of preg- nancy, except to record the fact that it occurs, as a rule, only in women who have had attacks of chorea in early life. There has been much talk about reflex chorea, as about the reflex origin of many other neuroses, but he who sees with only half an eye will soon convince himself that these reflex theories are but a poor makeshift. Of all the cases of chorea that I have seen, I have found but very few that I could consider due to any peripheral exciting cause. I have convinced myself that in a few cases the presence of intes- tinal parasites was the cause of a transitory chorea, which disappeared as soon as the parasites were removed, but I am not convinced that nasal or ocular trouble, of which so much has been made of late, ever leads to true chorea. If these troubles prove an inconvenience to the child, some choreiform habits may for a time be established, but in such cases the cardinal symptoms of St. Vitus's Dance are wanting. There is a curious relation between epilepsy and chorea. Gowers refers to epilepsy developing from chorea, and I have seen a case in a woman of forty, and another in a child of ten years, in which severe chorea set in after the cessa- tion of epileptic attacks. Symptoms. — Involuntary and irregular movements of any muscle or group of muscles of the body constitute the chief symptom of chorea. The muscles of the hands and fingers, and of the face and tongue are most often affected, but the leg and trunk muscles are at times involved. These movements are aggravated by volitional effort either of the muscles affected or of some other group of muscles. Thus the choreic movements of the hands will often become very much more intense if the child is told to stand absolutely still, or if while one hand is being examined it is asked to grasp something with the other hand. If the patient at- tempt to keep the affected part absolutely quiet he may succeed in doing- so for a few seconds, but after that the movements will become more intense. s 114 THE NERVOUS DISEASES OF CHILDREN. The choreic movements may affect only one extremity ; they may involve one-half of the body (hemichorea), or they may be generalized. In 184 cases, 35 were cases of right hemichorea; 32 of left hemichorea; and in 117 cases the choreic movements were general. As a rule the choreic movements are so evident that no special examination is needed. As the child enters the con- sulting-room the most superficial inspection is sufficient for a diagnosis ; but in other cases, particularly during the early stages of the disease, the choreic movements are discovered only upon careful examination. If there is any doubt what- ever about the condition, I ask the child to place its hand quietly upon my own, or between my two hands ; the irreg- ular choreic movements will, if present, be easily seen or felt. The true nature of the trouble, which may have ap- peared to be nothing more than "a slight nervousness," may thus be detected. If any further corroboration is needed, an examination of the tongue, as a rule, reveals the true char- acter of the disease, for there are very few cases of chorea in which if the movements of the extremities are ever so slight, the tongue does not exhibit very marked choreic twitching. These tongue movements are slow, coarse, sometimes rhythmical. In advanced cases, if the tongue is protruded the mouth is opened much more widely than necessary, the eyelids and eyebrows are raised in the same effort, and then through a choreic movement of the raas- seters the tongue may be caught between the teeth. These movements combined give rise to what, in a former article, I called the " facies " of chorea. The movements of the choreic patient are not only irregular but are often awkward to the extreme. This is clearly shown in the attempt to open or button the cloth- ing, in raising a glass of water to the lips, or in attempting to hold the pen in writing. This awkwardness often in- duces great irritability on the part of the child ; but how- ever annoying the movements may be, it is only in a very small proportion of the cases that they lead to a condition of exhaustion. A few years ago I had occasion to observe a little girl, six years of age, the child of healthy and intel- ligent parents. It had passed successfully through one CHOREA. 115 attack of chorea , and in the second attack, coming on after a fright, the movements were extreme, and sleep was so poor that within a few weeks the child died from exhaus- tion. A weakened but not diseased heart unquestionably assisted in bringing about this early fatal termination. It is a fortunate circumstance that in almost all these cases the movements cease during sleep, and that the child is thus able to recover partially from the exhaustion caused by the movements during the day. Some weakness of the muscles is frequently associated with choreic movements. The term paralytic chorea has been proposed for those cases in which there is marked paralysis, but as there is more or less weakness in the majority of the cases, and often more awkwardness than weakness, there does not seem to me to be sufficient excuse for the introduction of this term. Speech is frequently involved. This is in the nature of a dysarthria rather than an aphasia, the choreic movements of the tongue and laryngeal muscles making speech dif- ficult and often unintelligible. In some cases there is a little awkwardness of articulation, in others hasty articu- lation leading to the repetition of words, and in some a peculiar condition of speech which is in part due to diffi- culties of articulation, and in part to choreic movements of the respiratory muscles necessitating rapid breathing. Deglutition may be difficult, the tongue is frequently bitten, and from the awkwardness in the use of the knife and fork, and in passing food to the mouth, the little patient is much annoyed and is an ungainly sight for others while at his meals. Laryngeal chorea, pure and simple, occurs somewhat rarely, and consists of choreiform movements of the muscles controlling the vocal cord. The result is a peculiar ex- piratory noise like a bark, which is repeated at short in- tervals. These cases are often mistaken for cases of hys- terical bark; but the general restlessness, the age of the patient, the choreic movements of the tongue and fingers, should leave little doubt regarding the diagnosis. I re- member a little girl, aged ten, who began to bark after a sudden fright ; her case had been diagnosticated as hysteria Il6 THE NERVOUS DISEASES OF CHILDREN. by several eminent physicians, but there was no element of true hysteria in the case. She recovered promptly under the usual rest treatment. There is little doubt in my own mind that this represents the rarest form of chorea. The electrical reactions are sometimes slightly altered in cases of chorea. Rosenthal, Benedict, and others have found an increased response to the faradic and galvanic currents on the part of the muscles and nerves of the af- fected side. Some have even asserted that the reaction of degeneration with qualitative galvanic changes occurs in some instances, but I am inclined to doubt the correctness of this statement. I have never found a similar condition although I have frequently examined patients with this end in view. If such electrical changes were present I should suspect the presence of multiple neuritis, as this has been known to occur together with chorea ; just as I might sus- pect this same complication in cases of marked sensory dis- turbances, for the rule is that in uncomplicated chorea sen- sation remains undisturbed. Mental disturbance has been frequently referred to by many writers as a complication of chorea. It is surely not a very frequent occurrence, except that in the cases of chronic chorea (probably a different disease) the ten- dency to dementia is very marked. The impression I recorded a few years ago seems to me to represent the truth of the matter : " The mental calibre of children who develop chorea is rather above than below par. Children who by means of a better mental development stand head of the class, who work for prizes and earn them, children who are under constant mental strain, and about whom parents and teachers make much ado, are the ones most apt to be attacked by chorea." In some instances a violent mania is developed early {chorea insaniens), but it is much rarer to find this sequence of events than to observe cases of acute mania, particularly among young girls, in whom the movements of the extremities and of the tongue are typically choreic. Irritability of temper is perhaps the most frequent mental condition associated with chorea ; but this is natural enough if we consider the very annoying movements and the difficulty the child sometimes expe- CHOREA. 117 riences in making itself understood. At times, instead of a condition of mania, a condition of apathy and depression is present in patients afflicted with chorea ; but I am inclined to think that this is only true of patients who inherit a pre- disposition to mental disease. The temperature has been studied in chorea. In mild cases it is normal throughout the entire course of the disease. In severe cases it may be raised a degree or two, but any greater elevation is undoubtedly due to some other condition. Complications. — By far the most frequent complica- tions are rheumatism and heart disease. Rheumatism, if present, is discovered easily enough both by the fever and by the painful swellings ; but it is well to remember that the acute rheumatism of children is often a very much vaguer disorder than the acute rheumatism of the adult. If pain is much complained of in any case of chorea the joints should be examined carefully. Heart disease is the com- plication most to be feared. The heart should therefore be examined frequently and carefully. Mitral regurgita- tion is by far the most frequent form of cardiac disturb- ance. In the statistics of the British Investigation Com- mittee there were 116 cases of mitral disease and only 6 of aortic disease. Gowers found but two instances of aortic regurgitation among 252 cases of chorea. Sinkler found cardiac murmur in 82 .of 279 cases, but he does not decide how many were due to organic cardiac disease. It may often be difficult to determine this question, but if a patient whose heart was normal develops a murmur while under observation the probability of organic lesion is very great ; yet since anaemia is very frequent in cases of chorea, we must allow, in judging cardiac conditions, for the possibilitv of haemic murmurs and slight dilatation of the heart. Brown and J. K. Mitchell have described patients covered with subcutaneous nodules. These have a more direct re- lation to the rheumatic fever than to the chorea.* An excess of urea and of phosphates has been found in the urine of choreic patients. It is questionable at best * Osier is of the same opinion ; he considers this condition a great rarity in this country. Il8 THE NERVOUS DISEASES OF CHILDREN. whether they are not in some way the result of the inces- sant restless movements. Convulsive attacks are referred to as a complication by several authors. These are not of a typical epileptic character, but appear to be half-choreic and half-spasmodic movements. Mitchell and Burr have recently reported a case of this sort. The cases in which epilepsy is associated with chorea are more than likely cases of organic brain lesion, in which both the hemichorea and the epilepsy are symptoms of one and the same process in the cortex. Duration. — It is difficult to give any accurate informa- tion with regard to the duration of chorea, as the disease can hardly be said to be ended if upon the slightest prov- ocation another attack sets in. A single attack may last from a few weeks to many months. The average duration is generally considered to be about ten weeks. In my own cases the duration of attacks varied between four and twelve weeks. Two and three attacks are much more common than a single attack. I found among 104 cases which were analyzed for this purpose that 50 cases had one attack; 17 cases had three attacks ; 26 cases had two attacks ; 7 cases had four attacks ; 3 cases had five attacks, and 1 case had eight attacks. Notwithstanding this tendency to relapses the disease is an eminently curable one. It is only in a few cases that the disease becomes chronic, as in a patient of Meldner, who developed chorea in early life and remained choreic until his death, at the age of sixty-six years. The interval between the relapses is also subject to great variation. In a few cases the relapse may set in after sev- eral weeks ; in others after several years ; and in the case of chorea of pregnancy we often find that ten years or more have elapsed since the preceding attack. The female sex, for reasons too evident to mention, is more prone to re- lapses than the male sex. The second and third attack is generally supposed to be milder than the first, but there are exceptions to this rule, for the very worst cases of chorea that I have ever seen have been in patients Avho were passing through second and third attacks. Later attacks, as a rule, simulate the earlier ones. If the first attack has been a hemichorea it is very probable that CHOREA. 119 later attacks will be of the same character. The severity of development, with the exceptions just mentioned, is very much as in the first or in the earlier attacks. Diagnosis. — The diagnosis of chorea rests entirely upon the character of the movements. These are, as a rule, un- mistakable, and are so typical that when they occur in con- nection with other diseases we speak of such movements as " choreic " or " choreiform." In practice the question is most frequently raised whether a child is suffering merely from general nervousness or from typical chorea. It has been my habit to decide this point not merely upon the character of the movements, though it would be safe enough to do this, but chiefly upon the presence of other symptoms, which I consider of still greater diagnostic importance. I refer particularly to the characteristic movements of the tongue, and to what I have previously alluded to as the " facies " of chorea. In rare instances a child may be able to imitate the choreic movements of others and thus simu- late true chorea, but if it be mere simulation the attempt will not be a prolonged one nor will it be successful. Hys- terical chorea can be distinguished very readily from the chorea of Sydenham by the more rhythmical character of the movements, by the peculiarity of the onset, by the longer free intervals between the attacks of twitching, by the longer duration of the disease, and by the presence of other stigmata of hysteria. It is not generally appreciated that the choreiform movements associated with infantile cerebral palsies are apt to be mistaken for true chorea. This post-hcmiplcgic chorea is very similar to the ordinary form, but it is more strictly unilateral ; it is more persistent, and it is invariablv associated with other symptoms which prove the previous existence of paralysis. The difficulties of diagnosis are in- creased by the fact that in every case of severe chorea there is more or less weakness of the affected members, but in such cases I would advise examination for the existence of contractures and for increase of the reflexes, symp- toms which are characteristic of preceding paralvsis, even though there be little actual weakness at the time. Rigid- ity and increased reflexes, moreover, are never present in 120 THE NERVOUS DISEASES OF CHILDREN. cases of uncomplicated functional chorea. As this post- hemiplegic chorea is as much the expression of organic lesion of the brain as is post-hemiplegic epilepsy, it is nat- ural that the choreic symptoms should continue as long as the cerebral lesion which has given rise to them continues in force. In a collection of cases of infantile cerebral pal- sies I have found this post-hemiplegic chorea present in about six per cent., from which it is evident that choreic movements are not nearly so frequently found in conjunc- tion with these cerebral diseases as athetoid movements are. I have been consulted a number of times for persistent chorea, and in several such cases it has been my experience that the family physician has overlooked a preceding hemi- plegic attack which could have been made out readily enough if attention had been paid to the existing contract- ures and to the increase of the reflexes. I would urge that in every case of chorea a careful examination be made for other evidences of organic brain trouble. Confounding chorea and epileptiform convulsions is scarcely conceivable, for the convulsive movements of epi- lepsy come on at rarer intervals, there is generally some momentary loss of consciousness, and there are other symp- toms pointing to epilepsy. In a previous publication I re- ferred to the child of a colleague who would make sudden and very quick twitchings of an arm and of a leg. If these twitchings occurred while the child was walking or running across the room it would stand still, evidently surprised by these movements. It was natural to think of petit mal, but the frequency of the movements, the character of the twitchings, and the general choreic behavior of the child helped me to exclude petit mal and to recognize the case as one of true chorea. The diagnosis was corroborated by the very prompt result of antichoreic treatment. Morbid Anatomy and Pathology. — In considering this part of the subject we meet with very much the same difficulties which we encountered with regard to epilepsy, and the resemblance between the two is also a close one in this respect, that we not only have a general functional dis- ease, which in the one case we call epilepsy and in the other chorea ; but, like epilepsy, chorea is also frequently enough CHOREA. 21 the expression of actual cerebral disease. It is natural therefore to infer that ordinary chorea must be due to dis- turbances similar to those which we find in cases of organic lesion. Almost every conceivable change in brain structure has been at one time or another held responsible for the de- velopment of chorea. See collected 84 cases of chorea on Fig. 45.— Dilatation of Blood-vessels in the White Matter of the Convolutions of a very Chronic and Severe Case of Chorea. (Dana.) which a post-mortem examination had been made. In 16 no changes were found in the central nervous system, in 32 there were lesions in the brain and in the nerve-centres ; in the remainder there was congestion of the serous mem- branes. Ogle, Pye-Smith, and others refer to a hypersemia of the brain and cord. As long ago as 1868 Steiner report- ed upon a careful examination of three cases of chorea. He found cerebro-spinal anaemia and some connective-tissue proliferation in the upper part of the spinal cord ; conse- 122 THE NERVOUS DISEASES OF CHILDREN. quently he considered chorea to be the result of spinal irri- tation. Meynert and Elischer found hyaline degeneration in the nerve-cells of the central ganglia. The latter author also found changes in the vessels of the central ganglia as well as extravasation of blood into the connective tissue of the brain, and also numerous emboli in the smallest vessels of the cortex. He described peculiar corpuscles — highly refractile bodies — but Wollenberg has found them in the brains of non-chore^c patients. Flechsig has found hyaline changes in the anterior divisions of the lenticular nucleus. More recently Dana has observed not only a general hyper- emia of the brain, but a degeneration in the walls of the blood-vessels, in the white substance of the brain, and con- siderable perivascular exudation Avith an accumulation of leucocytes. Many others, chief among them Hughlings Jackson, have insisted upon the embolic origin of chorea, a theory that would be plausible enough since Dickenson has found that in 17 of 22 fatal cases endocarditis was associated with the chorea ; and yet this theory will not explain that large number of cases in which there is no involvement of the heart. Furthermore, an examination of the brain in fatal cases of chorea by competent observers has failed to reveal the presence of emboli. This view of the relation between capillary embolism of the brain and chorea was suggested by Angel Money, who noticed after injections of a fluid into the carotids of animals movements closely resembling those of chorea, and this condition was found after death to be associated with capillary embol- ism of the brain and cord. Lockhart-Clarke found changes in the nerve-elements and connective tissue in the spinal cord ; Garrod speaks of an overgrowth of connective tissue in the nerve centres, and thus we might go on quoting as many different find- ings as there are authors who have Avritten upon this sub- ject. One of the latest contributions to this subject is by Anton, who found a lesion or old scar in the outermost division of the lenticular nucleus. As the same lesion was present in both halves of the brain, and the chorea was also a symmetrical one, the author is inclined to attribute the choreic disease to these lesions. By way of contrast CHOREA. 123 this same author reports the case of a man, sixty-five years of age, in whom spontaneous and associated movements were entirely wanting in the left half of the body. In the brain of this man the thalamus was very considerably dis- eased, and was supposed to be the cause of the defective movements. I need not discuss the author's theory at- tributing the excessive movements to the disease of the lenticular nucleus, and the defective movements to the thalamus. Other authors, basing their conclusions upon a number of autopsies, have attributed choreic movements to disease of the thalamus. In a recent article on rabies Golgi refers incidentally to his studies on cortical changes in chorea, which were published in 1874, and have been quoted since by v. Ziemssen and others. As a matter of historic interest the annexed figure is reproduced, showing the changes in the nerve- cells of the cerebellum ; Gol- gi also reports that the gan- glion cells of the cortex, and the cells of Purkinje, in the cerebellum, were calcified. It is doubtful, surely, whether there is any causal relation between these changes and others pointing to a chronic interstitial encephalitis, and the disease proper. More- over, Golgi's patient died at the age of thirty-two years, and in him chorea was associated with chronic mental disease. Up to the present time the results of bacteriological research are not very promising. Berkeley found the staphylococcus pyogenes aureus in cultures from the blood of a fatal case of chorea. Dana has published the history and autopsy of a case of chronic chorea. The patient was thirty-four years of age at time of death ; he had his first attack of chorea at fourteen, and repeated attacks after that. The post- mortem findings included a chronic leptomeningitis of the convexity of the brain, hyaline bodies in the brain cortex, slight meningitis of the upper part of spinal cord, and slight meningo-encephalitis. Diplococci were found in the proliferating tissue between the meninges and the brain. The case is of unusual interest, showing that choreic symptoms may be Fig. 46. — Changes in Purkinje's Cells in Chorea ; Varicose Swelling of the Nerve-processes. (Golgi. ) 124 THE NERVOUS DISEASES OF CHILDREN. associated with a wide-spread affection ; but the true pathology of chorea can- not be made out in any case of fourteen years' standing, nor can such a case be relied upon to prove the " germ-theory of chorea." * The only just inference from the preceding account is that the accurate pathology and morbid anatomy of chorea are still unknown. Of the changes that have been reported bv various writers, many, if not most of them, are secondary and not primary. All that we can claim at present is that there is considerable change in the gray matter of the central nervous system ; that the entire motor tract may be involved, but that the changes occur more frequently in the cortex than in other parts of the brain. These choreiform movements are often associated with gross lesions in the cortex ; they are for this reason more common in the child than in the adult, and a lesion anywhere in the brain so situated that it cuts off cortical impulses may give rise to chorea. The occasional development of mental symptoms, the association of chorea and epilepsy, the one following upon the cessation of the other, are the symptoms which not only indicate a cerebral but also a cortical origin. With more recent methods of examination, such as those described by Golgi and Cajal, we may be able to make out the permanent changes in the nerve-elements of the brains of choreic persons, but even such changes may be second- ary to alteration of the blood-supply ; in fact the tendency of the present day appears to be to regard chorea as due primarily to vascular changes, and such vascular changes may be the result of infection. Prognosis. — Complete recovery is the rule in the ma- jority of cases of chorea. The prognosis may be a little doubtful in regard to the recurrence of the disease and the duration of an attack. A child that has once had chorea has acquired a distinct predisposition to the disease, and often an occurrence which would leave no impression upon a healthy child's nervous system is sufficient to bring about a relapse of the disease. Later attacks are generally as * Osier refers to the bacteriological researches of Pianese (Naples, 1893). Animals inoculated with a culture of bacillus taken from a choreic patient died " with muscular twitchings and convulsions," and the same bacillus could be obtained in pure cultures from the central nervous system. But it is doubtful whether these animals had chorea. CHOREA. 125 mild as the first, and there is no special reason to fear an unfavorable issue in later attacks unless severe complica- tions set in. Under such circumstances not the chorea, but the complicating rheumatism, or endocarditis, is the actual source of danger. Death occurred in only two per cent, of the cases collected by the British Medical Investigation Committee, and Sinkler states that in Philadelphia, in seventy-four years, there have been but sixty-four deaths from chorea. This latter statement does not mean very much, as the majority of the cases of chorea ending fatally would be reported as cases of one of the complicating con- ditions. There is no way of predicting positively the duration of a choreic attack. The milder an attack at the beginning the more likely it is to run a short course, whereas the severer forms are apt to be much more chronic, but severe cases under proper treatment will yield much more quickly than mild cases that are handled improperly. Under com- petent medical care the first attack may be recovered from in a period varying from four to ten weeks unless serious complications arise. A few get well more quickly, but they are the exceptions rather than the rule. Treatment. — A severe case of chorea puts the skill of the attending physician to a severe test, while in a mild case the less medical interference the better for the child. Increased experience, both in private and in dispensary practice, prompts me to urge the simple plan of treatment which I outlined a few years ago. The most important factor in the treatment of chorea is rest, absolute rest, often to the exclusion of all other thera- peutic measures. Take a choreic child that has been ac- customed to roam about at will and put it to bed ; it will be a little restless for the first few days, but it soon quiets down and shows the great advantage of a thorough rest in bed. There is difficulty occasionally in carrying out this plan, for mothers and nurses are only too likely to be disheartened by the first show of resistance on the part of the child and its unwillingness to remain quiet ; but with a few exceptions this restless opposition on the part of the child disappears within a few days, and the little patient feels very much 126 THE NERVOUS DISEASES OE CHILDREN. happier in bed than out. After a few days of enforced rest a decided improvement is noticeable. In the milder forms all movements cease, and in the severer forms the child is no longer troubled by the annoying jactations of the limbs. According to the severity of the case rest should mean en- tire rest in bed day and night. If the disease is taking a favorable course, after a week or two the patient may be taken out of bed. for half an hour, an hour, or two hours, and then returned again for the remainder of the day. I have met only very few cases in which it seemed impossible to carry out this treatment ; but I am firmly convinced that it was never the fault of the little patient but always the fault of incompetent and unintelligent relatives. If the dis- ease has assumed a mild form we can endeavor to keep the child quiet without keeping it in bed the entire day ; a few hours' rest will be better than none. It will also be of bene- fit to the child to forbid its taking any violent exercise, such as running, riding, dancing, or bicycling. I am so convinced of the value of this rest treatment in chorea that I have made it a rule, even in dispensary prac- tice, to insist upon this point of rest ; we go to the extent of preferring the mothers to report to us about the child rather than to have the child taken out of bed and brought to us at short intervals. Next in importance to rest is a nutritious and easily di- gestible diet. Milk and rest will do more for most cases of chorea than any other two measures. The nutritious diet will have a peculiarly good influence upon the many cases that are associated with profound anaemia. The monotony of this special form of rest-cure can be varied with advantage by the use of lukewarm baths. Im- mersion into a cold bath, or the wet pack, with subsequent friction, cannot be recommended. It is better to place the child in lukewarm water, then reduce the temperature by adding cold water, and with this water, that is growing colder and colder, to sponge the spine thoroughly in order to get the effect of the dripping water upon the skin. After the bath the patient should be kept quiet and wrapped up warmly. In every case proper hygienic and dietary meas- ures are of far more importance than medicinal treatment, CHOREA. 127 and yet we are bound to consider the various drugs that have been suggested for the cure of this disease. Among these arsenic holds the first place. Its praises have been warmly sung by some, while others have decried it as but little better than any other drug that might be substituted for it. Only a few years ago Dr. Seguin, in his remarkably lucid lectures on the various forms of functional neuroses, placed arsenic first and rest second in the treat- ment of chorea. This order I think should be reversed, as I have yet to see the first case of chorea that got well more quickly with arsenic than without, as long as it was getting the benefit of rest. Dr. Seguin insists that physicians, almost without exception, give nearly useless doses of ar- senic. He regards eighteen to twenty-seven drops of Fow- ler's solution after each meal as the really efficacious dose. In my own experience very few children will tolerate these large doses, which should at all times be given in some alka- line water within an hour after meals. I have always con- tented myself with smaller doses, varying from four to twelve drops three times a day, and if the cases resisted treatment I prefer abandoning arsenic rather than pushing it to the extreme which Seguin recommends. In cases of excessive restlessness I have been in the habit of prescrib- ing the arsenic, together with the elixir of the bromide of sodium, or if the sleep is disturbed I give the evening dose only in this way and administer the arsenic alone during the day ; the free use of chloral and bromides is to be con- demned. If one must use any drug in the cases of chorea, arsenic is to be preferred ; but it cannot be expected to per- form miracles, and we must not regard it in any sense as a specific therapeutic agent. Many other remedies have been proposed, all have been tried, and almost all have been abandoned. For some time the tincture of cimicifuga was in great favor. From fifteen to thirty drops, three times daily, may be administered in those cases in which arsenic is not well borne h\ the stomach. I have seen no good reason to resort to the use of hypodermic injections of arsenic, as recommended by Eulenburg and Hammond ; Weir Mitchell some time ago proposed the use of salicylates; Simon and Legroux suggested the use 128 THE NERVOUS DISEASES OF CHILDREN. of antipyrin ; and the oxides and sulphate of zinc have long been in popular favor in the treatment of this disease ; but no one, I think, would venture conscientiously to recom- mend any of these drugs as a specific against chorea. The preparations of iron and of arsenic fulfil this role better than any other drugs, for they at least help to tone up the general system. Hirt recommends the use of morphine. If this drug were as effective as it is claimed to be, its use in young children is not to be encouraged. In the earlier stages of chorea it is essential for patients to obtain sleep. According to the age of the child chloral in five, ten, or fif- teen-grain doses per rectum may be given. If it is neces- sary to substitute another drug I would suggest the use of chloralamid, of sulphonal, or trional (ten to fifteen grains each), and if there is a great deal of mental excitement I should favor the hypodermatic use of the hydrobromate of hyoscine (one two-hundredths to one one-hundredth grain). In the majority of cases a heart tonic will be necessary. Digitalis in drop doses of the fluid extract or the tincture of strophanthus should be given in cases of heart weakness or feeble pulse. Blaud's pills, the sirup of the iodide of iron, various preparations of cod-liver oil, good stimulating wines, all these will be called for in some cases of chorea, but whatever else one may be induced to give the only matter of importance is that absolute rest shall be enforced. Erb has advised the use of electricity. A weak gal- vanic current may be employed safely enough and may be applied to the nape of the neck, and over the motor areas ; in which case, if given late at night, the current will help to bring on normal sleep. A moderate stabile current of from fifteen to twenty cells (about ten milliamperes) ap- plied to the spine will help to allay restless movements of the body. I am opposed to the use of a strong current to the head, as it may do much more harm than good, and also to the use of the faradic current to the head or spine, which would act as a direct irritant rather than as a seda- tive. Massage may be given in some cases in which the general nutrition is at a very low ebb and in Avhich the circulation is poor. A special caution is necessary as regards the question CHOREA. 129 of attendance at school. Every choreic child, however mild its attack may be, should be kept from school both for its own sake and for the sake of the other pupils who might imitate the disease. I have sometimes allowed my- self to be persuaded to permit a child with a mild form of chorea to continue at school ; in almost every instance I have had reason to regret it, for nothing is better calculated to bring out severe chorea than the competitive spirit that obtains in most schools. Periods of examination are fraught with greatest danger to those children who have had attacks in earlier life. The atmosphere of the school-room seems to have a depressing influence upon such children, and among the wealthier classes far better progress can be made in the ordinary studies if the child is instructed at home than if it is taught at school. It is necessary for the phy- sician to take a firm stand on this question or else his treat- ment of the case will be thoroughly unsatisfactory. BIBLIOGRAPHY. Anton: Neurolog. Centralbl., 1893. Benedikt : Nervenpathologie und Elektrotherapie, p. 256. Berkeley : Johns Hopkins Hosp. Reports, 1891, vol. ii., No. 6. Brown : Journal of Nervous and Ment. Dis., 1891. Dana : Brain, vol. xiii. , April 1890. (Cases with autopsy collected.) On the Microbic Origin of Chorea, Am. J. of Med. Science, January, 1894. Elischer : Virch. Arch., vols. lxi. and lxiii. Erb : Elektrotherapie, p. 587. Golgi : Ueber die Pathologische Histologic der Rabies Experimentalis, Berl. kl. Wochenschr., No. 14, 1894, and Rivist. Clin., iv., p. 361, 1874. Koch : Ueutsch Arch. f. Kl. Med., 1887, p. 40. Knapp : Review of Infectious Origin of Chorea, Boston Med. and Surg. Journal, January 25, 1894. J. K. Mitchell : Univ. Med. Magazine, vol. i. Naunyn : Schmidt's Jahrb., January 15, 1889. Osier : Chorea and Choreiform Affections. Philadelphia, 1894. Roger: Arch. Gen de Medecine, December, 1866, and January, 1867. Rosenthal : Klinik der Nerven Krankheiten, 1875. Rothschild : Arch. f. Kinderheilkunde, vol. xvi.. p. S^. 1893. Sachs : Article Chorea, Keating's Cyclopaedia, and Hare's System of The- rapeutics. Sahli : Deutsches Arch. f. Kl. Med., vol. Ii. ; on rheumatism. See : Memoires de^l'Acad. de Med., tome xv., 1850. Q 130 THE NERVOUS DISEASES OF CHILDREN. Sinkler : In Pepper's System of Medicine. Soltmann : Article on Chorea, in Gerhardt's Handbook, contains Litera- ture up to 1880. Starr : Article Chorea, in Am. Text-Book of Children's Diseases. Virchow: Hirsch Jahresbericht, 1889, Bacteriological Investigations. Wollenberg : Zur Pathologie d. Chorea Minor, Arch. f. Psych., vol. xxiii., p. 167. V. Ziemssen : Article Chorea, in Ziemssen's Cyclopaedia (full Literature), 1877. CHAPTER VI. CHOREIFORM DISEASES. HEREDITARY OR HUNTINGTON'S CHOREA. This disease is, on the whole, extremely rare. In view of its hereditary character we must consider it in connec- tion with the diseases of children, although it generally appears about the age of thirty or forty. The disease was first observed by Huntington, a Long Island physician, and it has since been designated by his name. It appears, how- ever, that another New York physician, Waters, described the disease in a letter to Dunglison. For a long time the disease was little known beyond America. Of later years it has been observed and discussed by a number of English, German, and French authors. The disease runs in families and is spread from one community to another in connection with the migration of the afflicted families, so that now, ac- cording to Gray, there are a number of communities in America in which the disease is prevalent ; but, as he further states, there is great secrecy maintained with regard to it, as the affliction is looked upon as a distinct stigma resting upon the family. The disease affects several members of the same genera- tion, but may skip as many as it selects. The descendants of healthy members of a family enjoy immunity from the disease as a rule. In King's case, which I quote on the au- thority of Gray, the great-grandfather was choreic. He had ten children, but only four were afflicted with this disease; they also had children who were choreic. One of these four had nine children, eight of whom were healthy, but the ninth was choreic. The ninth had live children, of whom four were choreic. Of these four, three had no children, but the fourth had a chorea whilst he was still young and 132 THE NERVOUS DISEASES OF CHILDREN. was cured of it, when again at thirty-five he passed gradu- ally into the typical Huntington's chorea. Symptoms. — The disease begins, as a rule, between the ages of thirty and forty years, though at least a dozen or more cases are on record which have begun before the age of twenty. Males and females seem to be afflicted with the disease in equal numbers. The chief symptom is a motor disturbance, which for lack of a better name is termed choreic, yet it is very different from the twitchings of chorea minor. The muscular movements of Huntington's disease are coarse and grimacing, and may be distributed over a large area of the body. In Sydenham's chorea the affection is more strictly localized. In the former disease grimaces and all sorts of extravagant posturing are much more pronounced than those which we observe in the ordinary chorea of children. The difference in degree of muscular activity is so very great that one naturally doubts the connection between the two diseases. As a rule, the movements are slight at the start, affecting the face and upper extremities only. In the course of years they in- crease in intensity, and become widely distributed over the entire body, until in the end every single muscle or group of muscles may be involved. The muscular disturbance is in part subject to the will, and patients afflicted with this disease can, if they make a serious effort, inhibit the twitchings very much better, and for a much longer period, that those suffering from ordinary chorea can. The motor power is, as a rule, not diminished, but on account of the irregularity of muscular actions it is extremely uncomfortable for the patient to walk about, and he easily becomes an object of pity or of ridicule. There are no sensory disturbances, and the reflexes, while they may vary much, are not morbidly exaggerated or diminished. By far the most characteristic symptom of Huntington's chorea is the association with it of a progres- sive dementia. The first sign of a mental change may be a simple depression which is deepened by a knowledge of the hereditary and incurable character of the disease. Under the circumstances the occurrence of suicide is not unnatural. The mental condition at the start varies a little ; HEREDITARY OR HUNTINGTON'S CHOREA. 1 33 the patient may be either irritable or apathetic. In the course of time the deterioration of all the mental faculties is very marked and a typical dementia is developed. Speech is, as a rule, thick and indistinct, sometimes nasal and con- fused. Unless some intercurrent disease puts an end to life these patients may linger on for many years, may be- come absolutely bedridden, a burden to themselves and a torment to their families. The course of the disease is a very chronic one, and unfortunately does not tend to shorten human life. Sev- eral of those who have exhibited the first symptoms of dis- ease at the age of thirty have lived to sixty and seventy years. Whether cases beginning very early in life live as long, cannot be distinctly stated, but there is nothing improbable in supposing that they do. Etiology. — Among the causes of this disease none is more potent than heredity. It is distinctly a family dis- ease, and unlike other such diseases it does not seem to skip a generation. Persons with this disease can generate healthy children whose descendants may not be afflicted with this trouble. To all appearances an exciting cause is needed to develop the disease. In many of the cases the first symptoms appear upon severe emotional excitement or after some acute disease. In this respect the resem- blance between the hereditary chorea and chorea minor is very striking. Rheumatism does not, however, play the part in the hereditary form that it does in ordinary chorea. Differential Diagnosis. — The strong factor of hered- ity and the appearance at a relatively advanced age are sufficient to distinguish these cases from ordinary chorea. There is some danger, however, of confusing these cases with the post-hemiplegic chorea that is developed early in life, and this danger is all the greater if the onset of the paralytic symptoms is uncertain, or if the paralysis has disappeared and the chorea remains. From Friedreich's ataxia the disease can be easily differentiated bv the fact that in the former malady the disturbance of motion is truly ataxic and not choreic, and the reflexes are totally absent, whereas in Huntington's disease the reflexes are not markedly altered. In Friedreich's disease there is also 134 THE NERVOUS DISEASES OF CHILDREN. ataxic gait, made worse by closing of the eyes, whereas in Huntington's disease nothing of the sort is observed. Some forms of hysterical chorea might be confounded with this disease, particularly if on inquiry the statement is elic- ited that a similar affliction has been observed in other members of the family, and it should not be forgotten that the hereditary trouble may be of an hysterical order. The examination of the patient and the general hysterical tem- perament, the fact that the choreic movements come on in the nature of attacks, and the determination of other hys- terical symptoms will help to distinguish one disease from the other. Pathological Anatomy. — The morbid changes in Huntington's chorea have not yet been definitely made out. Innumerable changes have been reported by various authors. These include pachymeningitis, hsematoma, tu- mors of the dura, general atheroma, atrophy of the motor convolutions, increased fluid in the ventricles, foci of soft- ening in various parts, even in the ganglia. The most frequent states are pachymeningitis and changes in the cortical tissue. In a case of Charcot's the meninges were adherent in certain places, and the cortical substance was evidently sclerotic. But these varying conditions are largely secondary processes and do not in any way explain the true pathology of the disease. Dr. Osier summarizes the changes in a series of sections from the brain and cord which he examined as follows : " The arteries were thick- ened, and, in places, show hyaline degeneration, and in the smaller arterioles the fatty changes were very marked in the fresh specimens from the cortex. The perivascular lymph-spaces were large, and contained leucocytes. The ganglion cells, in many sections, showed very slight changes, not more than are often seen in chronic cases associated with atrophy of the convolutions. There was the common vacuolation, and many cells seemed laden with pigment. The increase in the connective-tissue elements was more evident to the touch and upon section than microscopically. Sections of the pons and medulla showed no foci of disease. Beyond the thickening of the arteries and the shrinkage of the cells of the anterior cornua — probably an artificial HEREDITARY CHOREA WITHOUT DEMENTIA. 135 change — the sections of the cord showed no important lesions."* For the present we can say nothing more than that the disease is, in all probability, due to some chronic cerebral process, the exact nature of which will have to be determined by further post-mortem examinations with the aid of improved methods of staining. Prognosis. — The prognosis of Huntington's chorea is grave as regards the cure of the trouble, and in view of the marked dementia which is associated with almost every case. Unfortunately, however, it is not a fatal disease, and patients so afflicted may live on to a very old age. Treatment. — As for the treatment of these cases the same principle should be observed as in ordinary chorea — rest, freedom from care, and excitement, separation from family, and change of climate, and possibly a course of arsenic treatment may be of some benefit. If movements are excessive and the sleep of the patient is unsatisfactory, the exhibition of hyoscine, in doses of one one-hundredth of a grain, or of trional or chloralamid, in ten- to twenty-grain doses, once or twice a day, is worthy of a trial. But, in the nature of things, every form of treatment will be simply palliative. HEREDITARY CHOREA WITHOUT DEMENTIA. There is another form of chorea of which a slight mention should be made in this connection. The disease has a distinct hereditary tendency, and is either transmitted direct from parent to child or from a more remote ances- try. The disease appears early, beginning as a rule at about the age of puberty and continuing during life. It is characterized by distinct choreic movements of the hands and tongue and the facial muscles. The move- ments may become so marked that they interfere with every voluntary effort, such as riding, sewing, buttoning of clothes, and the like. The lower ex- tremities do not become affected, and the general health of the patient is not much impaired, except that the annoyance of the disease may bring on a despondent feeling ; and in one case that has come under my notice distinct melancholy has occurred. It is distinguished from Huntington's chorea above all by the entire absence of symptoms pointing to dementia. The persons afflicted with this form remain bright throughout life and are able to attend to their ordinary affairs. It is furthermore to be distinguished from chronic chorea from the fact that there is no distinct history of an acute attack after which the chorea has been developed ; that the choreic move- * Greppin claims that the disease is due to a morbid process (not unlike an encepha- litis), which is developed gradually and in various parts of the brain. 136 THE NERVOUS DISEASES OF CHILDREN. ments have never been as wide-spread as they are in such cases of chorea minor as develop into chronic chorea. They generally begin a little later in life, too, than the acute chorea minor does, and there is no distinct history of individual attacks. If the disease has once been established it continues without any marked remission, but also without any rapidly progressive changes. Some confusion might also arise with the post-paralytic chorea, but under such circumstances an examination into the past history of the patient, and for the evidences of persistent paralysis, will help to clear up the diagnosis. The prognosis is entirely favorable as regards life, but less can be said as regards the cure of the disease. I have placed such patients under the com- plete rest-cure, and have for a time obtained distinct improvement in the choreic movements. General tonic measures should be employed, and the proper feeding of the patients carefully looked after. HABIT CHOREA. It is well Known that children are subject to " tricks " of movements. These include simple or co-ordinated movements of various muscles, gen- erally of the muscles of the face, of the eyes, of the shoulder, and even of the thighs. These " habit spasms " or " tics " may resemble chorea ; often the jerkings are so violent in character as to suggest " epileptoid " rather than " choreic " disease. It is not always an easy matter to determine how the " trick " was en- gendered ; in some instances a true chorea has been the starting-point, the children keeping up some form of twitching movement after the St. Vitus's dance has disappeared. Through imitation, habit chorea may be developed in children who have watched others with chorea minor. A habit chorea developed in early years is often continued throughout life. Among professional men (artists, literary men, and even physicians) such " tricks " are not uncommon, and the doubt arises whether serious ef- fort has been made to dispel the habit. Among the commoner forms of " habit chorea " are blinking, facial contortions, sniffing, shrugging of the shoulders, or some trick in speech or gesture. A friend of mine, now a well- known astronomer, has since boyhood never answered a question without first saying, Hm ? ah ? eh ? In former years this utterance was accompanied by an exaggerated raising of the eyebrows, as if to intensify the interrogation. Such habits cannot be condemned sufficiently in children. Parents should discipline children severely in order to rid them of the habit. If there is any peripheral condition (such as nasal obstruction or eye- strain) which helps to keep up the habit, the condition should be treated carefully. It is important also to make certain that the " habit spasm " is not true chorea. This can be done by examining for other symptoms of St. Vitus's dance. The general condition of children with some form of habit spasm may require treatment ; many of them are anaemic or scrofulous. Complex Co-ordixated Movemexts (Complex Ticsl— In children as well as in adults, complex movements of a definite character are repeated COMPLEX CO-ORDINATED MOVEMENTS. 1 37 at intervals, or may be continuous. They bear only a very slight resemblance to chorea ; but, as a matter of convenience, they may be discussed in connec- tion with "habit chorea." Some of them, the gyrospasms, for instance, occur in very young infants ; others occur in older children and are frequently associated with slight mental derangement. Some authors have included them under the heading of Imperative Movements, and have described them as head-shaking, head-rotating, head-banging, head-nodding, etc. These movements are often associated with nystagmus and with defective mental de- velopment, thus indicating that in some instances they may be due to organic disease of the brain. These peculiar disorders have been studied carefully by Henoch, Hadden, Gee, Peterson, and Mills, and have been referred to by Osier and others. Gyrospasms of the Head is a term applied by Peterson to peculiar ro- tary movements of the head in children, associated at times with strabismus or nystagmus. Two of Peterson's five cases were observed by him in my clinic. Both these cases were in young infants, eight and nine months of age respectively ; in one the movements came on after a fall ; in the other there was no history of traumatism. The chief points of Case II., as described by Peterson, are as follows : The child was nine months Old when examined. Since the age of four months he had a rotary movement of the head. When the child was quiet, the head kept oscillating from right to left and from left to right at the rate of eighty to one hundred oscillations per minute. The movement ceased at times, particularly if its attention was riveted upon some object. There was lateral nystagmus of the right eye, also ceasing for minutes at a time. The patient had measles two months previously ; no convulsions ; no fall ; labor had been entirely normal. The general health was good. These gyrospasms, which are evidently of a piece with head-nodding, head-jerking, etc., are scarcely to be confounded with epilepsia nutans. If developed in later years, they may constitute a habit, but if the movements begin in early infancy and are associated with nystagmus, strabismus, or idiocy, one need not hesitate to ascribe them to cerebral disease. The re- gion of the cranial-nerve nuclei would be the most probable seat of the trouble ; and the lesion must be supposed to be irritating in character. The chief etiological factors appear to be rickets, intestinal irritation, and dentition. The prognosis is good on the whole, except when these move- ments are associated with idiocy. A mild course of bromides has proved satisfactory in the majority of cases. Mills recommends, in addition, the use of two or three minims of tincture of belladonna, or one minim of the fluid extract of conium. Imperative movements associated with arithmomania (repeating every- thing a definite number of times) are clearly the result of mental derange- ment, and do not seem to me properly to belong to this group of cases Several other motor neuroses bearing a more or less close resemblance to chorea minor have been described in 138 THE NERVOUS DISEASES OF CHILDREN. connection with the chief disease. While the cases may have a superficial resemblance to St. Vitus's dance they are, as a rule, of a much more explosive character. They occur in families with a neurotic heredity, and represent, on the whole, a more serious disturbance of the central nervous system. It is not an easy task properly to classify all these conditions. There has been much confusion in the discussion of these subjects, and no two authors entirely agree in the designation of these disorders. As a majority of them come on later in life, or at least are fully developed in later years, we need not in this book treat them in a very exhaustive manner. CHOREA ELECTRICA. It is certain that two distinct forms have been described under this term. Henoch includes under it a form of choreic disturbance which re- sembles in part what is commonly designated as habit spasm, but some of his cases are more distinctly allied to myoclonia or paramyoclonia multiplex. In the latest edition of his book Henoch does not insist on the old designa- tion, yet many authors have followed him and are continually quoting his cases under the name of chorea electrica. The term electric chorea should, however, be restricted entirely to a very rare disease that occurs chiefly in the northern part of Italy. I have had opportunity to see one case of this description in Italy, and one in an Ital- ian woman who came to my clinic. The condition was first described by Dubini, in 1846, and to avoid confusion it would be as well to speak of it as Dubini's disease. The disease is apt to occur in boys and girls as well as in men and women of advanced years. It has been supposed to be due to some infectious agent, but the rarity of the disease, even in those districts in which it does occur, would seem to militate against this view. The chief symptom of the disease is a series of violent spasmodic move- ments, affecting particularly the muscles of the neck and head, as well as of the extremities. Some of the cases begin with movements in an arm, which spread to the leg of the same side, and finally involve the opposite half of the body, and also the trunk, neck, and head. After some months the choreic members of the body become paralyzed, there is wasting of the mus- cles and a loss of faradic irritability. Epileptiform seizures occur, and these may either be partial or general. In the course of a year or more the pa- tient may become entirely paralyzed, and, as in the case I saw, the patient was confined to his bed, unable to move a limb, while the severest choreic jactation continued in the muscles of the neck. The majority of these cases end fatally within a few weeks or months. During the course of the disease the patient suffers a great deal from pain, has slight elevation of tempera- ture, but, as a rule, does not have loss of consciousness. MALADIE DES TICS CONVULSIFS. 1 39 The disease is so rare that sufficient pathological examinations have not been made to warrant definite statements with regard to its nature. It is probable that it is a form of cerebro-spinal disease, as is indicated by the con- vulsions on the one hand and the paralysis with atrophy on the other. MALADIE DES TICS CONVULSIFS. This disease, also known as Gilles de la Tourette's disease, rep- resents another form of motor neurosis, not unlike some of the symptoms presented by electric chorea. The chief symptoms of this condition are con- vulsive twitchings of the facial muscles and other regular systematic move- ments, explosive conditions of speech known as echolalia and coprolalia, and lastly, imperative conceptions and impulses. The disease, as a rule, appears in children between the ages of seven and fifteen years. It is more apt to occur in those predisposed by inheritance to neurotic troubles than in others. In many instances there is a very distinct history of heredity, the same dis- ease occurring in succeeding generations. The first symptoms that appear are sudden and explosive twitchings of the muscles of the face or of the eyes. The mouth may be twisted, and all sorts of grimaces may constitute the early symptoms. In a case which I have had under observation, the boy would begin by making grimaces and then turn about quickly as though he was snapping at some one. These movements were performed in regular succession. Movements of the sterno-cleido mastoid, and of the trapezius are often repeated so systemat- ically that they seem to be purposive. The child may at the same time begin to spit or to blubber, and the entire combination of symptoms suggest the possibility of simulation or intention, particularly if jumping or leaping move- ments are associated with the other symptoms. Whatever the symptoms may be that have been developed in a given case they are persisted in with remarkable regularity, and can be distinguished from intentional movements by the fact that the patient is evidently surprised by the suddenness and vio- lence of the movements. Smacking, hissing sounds are sometimes heard, but none of the symptoms is more characteristic than the habit of repeating words or sounds that are heard (echolalia), or the involuntary sudden explo- sive utterance of foul language (coprolalia). A little patient of mine uttered the worst curses I ever heard, which she had evidently picked up on the street, but would regret the utterance the very next moment after they had passed her lips, and would contritely declare that she was entirely irrespon- sible for the same. The symptoms frequently continue for years, and while the intelligence of the person so afflicted does not show any deficiency only a few are able to become masters over these explosive seizures. In some an attempt to con- quer the disease produces great restlessness and general excitement. In others the symptoms become manifestly worse under the effort to control them, and it is advisable under such conditions to divert the attention of the patient from his symptoms as much as possible. Imperative conceptions are frequently associated with the motor symp- 140 THE NERVOUS DISEASES 0E CHILDREN. toms, and usually seem to represent a psychic explosion, the equivalent of the physical symptoms. Some of the children feel compelled to utter words in a definite sequence, to pronounce certain letters in a peculiar way ; rolling the " r " and making a hissing " s " are particularly frequent. In others impera- tive actions, such as occur in connection with neurasthenic disorders, are quite common. They are compelled to do their sums over and over again, to re- trace their steps, to pick up everything they see lying on the street or in the room, and are compelled to be in a state of continued activity in consequence of these imperative impulses. The course of the disease is extremely chronic ; by some it is considered to be absolutely incurable. But this is too extreme a view, since some of the cases get well. The prognosis should, however, be studied carefully in each case, as there is no telling in advance whether the patient will respond favorably or not to the treatment. The resemblance between this disease and other choreiform neuroses is at times so close a one that a differential diagnosis becomes ex- tremely difficult. It can hardly be confounded with ordinary chorea since the movements are much more systematic and more intermittent than they are in St. Vitus 's dance. Moreover, echolalia and coprolalia never occur in ordinary chorea. The difficulties of speech in chorea are the very reverse of the explosive speech in this maladie des tics. Hysteria and hysterical chorea may bear a superficial resemblance to this disease ; but, on the one hand, the stigmata of hysteria are generally wanting, and in hysteria there is no such constant repetition of more or less convulsive movements as there is in this disease. There is, no doubt, a close resemblance between the maladie des tics and the jumpers and many other forms of disease which are differently designated according to the peculiar variety of muscular action. The one point, however, which the maladie des tics has in common with other similar conditions is its occurrence in neurotic families. The distinction between maladie des tics and para-myoclonia will be evident after a discussion of the latter trouble. Treatment. — The most important factor in the treatment of these pa- tients is their complete isolation from all other children and from their usual surroundings. They should be subjected to a moderately severe discipline by a competent nurse or parent, and should be taught self-control as far as that may be possible. The mere seclusion from other persons generally serves to lessen the excitement and the number of explosions. If the disease has led to a condition of exhaustion from loss of sleep or from inanition it is well to secure quiet during a considerable part of the entire day. This can be done best by the administration of chloral or of small doses of morphine. I can- not see the wisdom of using chloroform, as has been suggested by some. If the motor restlessness is extreme, hypodermic injections of hydrobromate of hyoscine, in doses of one one-hundredth grain, carefully administered, may prove of some benefit ; the ordinary nerve-tonics, such as arsenic and quinine, will do little good, and it is best not to waste much time in administering these drugs. I am confident that if any good is to be accomplished we must depend entirely upon isolation, proper feeding, and the use of tonic hydro- THOM SEN'S DISEASE. 14 1 therapeutic procedures. Regular gymnastic or calisthenic exercises should be tried and may be of advantage. thomsen's disease (myotonia congenita). In connection with the various forms of disordered movements which have been considered in this chapter, we may discuss in a very brief way the disease known by the name of the physician who gave the first thorough description of it as it occurred in his own family. Those who object to nomenclature of this sort will prefer the term Myotonia congenita, but as Thomsen was also a subject of the disease an exception may well be made in his case. The disease is unusually rare. In this country very few cases have been described, and the only one which the author has had an opportunity of see- ing was the one presented to the New York Neurological Society, in 1886, by G. W. Jacoby. The best accounts of the disease are those furnished by Thomsen himself, a description of it in Leyden's work on " Spinal Cord Diseases," and the monograph of Erb, who summarized the chief points of twenty-two cases, and added a full account of peculiar electrical changes in this disease, for which he proposed the term " Myotonic Reaction." The chief symptoms of the disease are a rigidity of the muscles (myoto- nic contraction), which is developed whenever an attempt is made to use a muscle or muscles after a period of rest. If the first difficulty has been overcome, the action of the muscles may be entirely normal for some period of time. This muscular rigidity is most noticeable in the attempt to rise after a person has been quietly seated for some time ; in the attempt to use the hands in lifting, grasping, or writing, and at times considerable difficulty is experienced in the movements of the tongue and of all muscles concerned in articulation. The impression created by a patient grasping the physician's hand and then not being able to let go, or by his falling to the ground and remaining absolutely rigid until the spasm is relaxed, is one not easily for- gotten. The spasms are generally limited to a few groups of muscles, but in some cases the entire muscular system, with the possible exception of the ocular and respiratory muscles, is involved. The rigidity of the muscles is in- creased under the influence of emotional excitement and under the influence of cold and damp weather. Under alcoholic stimulants the rigidity is said to lessen, and prolonged periods of rest undoubtedly have a favorable influence upon the disease. The disease occurs in families, and very often affects several members of the same family. In Thomsen's family, five generations have been affected by this same condition. There seems to be no distinction as regards sex, and the disease may come on at any period of life. A number have been re- corded during the first decade of life, but the disease is apt to reach its maxi- mum during the period of greatest muscular development, and the symptoms are, therefore, pronounced between the ages of fifteen and twenty-five. In Jacoby's case the patient, who was twenty-four years of age at the time of 142 THE NERVOUS DISEASES OF CHILDREN. examination, stated distinctly that he could not, as a boy, take part in the outdoor games of his comrades on account of the stiffness of his muscles. He attempted to play upon the organ, but found that his hands were clumsy. He was not able to whistle, and even in chewing his food the muscles be- came stiff and rigid, simulating a condition of trismus. In this case there was distinct rigidity of the muscles of the eyes. The entire muscular system is, as a rule, well developed ; there is often an increase of normal volume. According to Thomsen, the more the muscles are used the less the spasm becomes, and he advises a very active life as pos- sibly the only hope for an improvement in the disease. There are a few objective symptoms which render the diagnosis of the disease easy enough. The mechanical excitability of the muscles is mark- edly increased, a single tap of the hammer on a muscle produces a slow, tonic contraction of the fibres, but the contraction is not relaxed for some period of time. In contradistinction to the symptoms of tetany it may be stated that the nerves do not show any increased mechanical excitability. There is, furthermore, a very remarkable change in the behavior of the muscles un- der electrical stimulation. Erb has proposed to speak of this as " myotonic reaction." The faradic excitability of the nerves is not changed, but on the use of a strong current the muscles innervated by the nerve will be forcibly contracted and remain contracted for some period of time after the current is broken. The direct faradic excitability of the muscles is very much in- creased, mild currents being sufficient to produce contractions of long dura- tion. The galvanic excitability of the nerves is surely not increased — possi- bly diminished — but the same muscular phenomenon can be observed on galvanic stimulation of the nerve as in the case of faradic excitation. The direct galvanic excitability of the muscles is increased ; the anodal contrac- tion is, as a rule, greater than the kathodal contraction ; opening contrac- tions either with the anode or kathode cannot be readily obtained. The con- tractions are sluggish, and are continued for some time after the elec- trical stimulation ceases. But the most characteristic symptom of this myotonic reaction is a peculiar, rhythmical, wave-like contraction which pro- ceeds from the kathode toward the anode. This phenomenon can best be observed with the use of strong currents of at least 20 or 25 milliamperes, and if the negative pole is placed over the tendinous end of a muscle. If the kathode is placed in the palm of the hand, the anode on the shoulder, a wave-like contraction will appear and gradually work its way from the mus- cles near the wrist to those of the shoulder. These are the chief symptoms of the disease. A few complications have been observed in some of the cases, among which we may specially mention increased mental irritability and hypochondriasis, both of which are not un- natural in view of the annoyance which the disease causes the sufferer. Epilepsy and migraine have also been recorded in connection with Thorn- sen's disease, but as both these diseases are to a great degree hereditary the association maybe simply a coincidence. The pathology of the disease is still obscure, and up to the present time no post-mortem examination has been made. The muscular tissue has been PARAMYOCLONUS MULTIPLEX. 1 43 excised from the living body and examined. The changes noted were an enormous hypertrophy of the fibres, with considerable increase of the nuclei in the sarcolemma, and an increase of the interstitial connective tissue. In one case examined by Erb, there was a striking vacuolization of the individual muscular fibre. These histological findings do not enable us as yet to explain the character of the disease. All that we can say is that there must be some congenital condition which causes a hyper-excitability of the ganglion cells of the cord. Whether the condition causing this hyper-excitability is in the muscular tissue itself, in the nerves, or in the spinal cord, cannot be definitely stated. Thomsen considers defective cortical innervation to be the prime cause, but cerebral changes alone could not account for all the symptoms ; these must be ascribed to disease (primary or secondary) of the muscles or to disease of the cord. The disease can be mistaken only for tetany, and possibly for chronic mus- cular dystrophies ; but as Erb suggested, several years ago, a tap with the percussion-hammer and a few closure contractions with kathode and anode upon certain muscles will suffice for a diagnosis of Thomsen's disease. The prognosis is favorable as regards life, unfavorable in regard to a cure of the disease. Much can unquestionably be done by a continued exercise of the will and by regular gymnastic exercise. No further treatment of the dis- ease is indicated. CONGENITAL PARAMYOTONIA. Eulenburg * has described a somewhat similar affection, which he terms " Congenital Paramyotonia." This also is a distinctly family disease which may be traced through six generations, the disease becoming manifest im- mediately after birth. The chief feature of these cases is a tonic spasm last- ing from a quarter of an hour to several hours, and coining on chiefly after exposure to cold. There is distinct weakness associated with rigidity ; the facial muscles may be involved as well as the orbicularis palpebrarum and the orbicularis oris, making speech impossible during the continuance of the contraction. The disease is differentiated from Thomsen's disease by the absence of increased mechanical excitability and by the absence of anything resembling a myotonic reaction. Eulenburg states that there is a distinct tendency to kathodal and anodal tetanus. PARAMYOCLONUS MULTIPLEX (MYOCLONIA). A short reference is necessary in this connection to the above disease, which bears a resemblance to electrical chorea and to tic convulsif. By some it has been regarded as a form of hysteria, but this is not warranted. for cases have been observed in which there were none of the characteristic signs of hysteria. It is best to restrict the term paramyoclonus multiplex to the disease as it was originally described by Friedreich. The chief symp- * Neurologisches Centralblatt, June 15, 1SS6. 144 THE NERVOUS DISEASES OF CHILDREN. torn of the condition is a rapidly repeated clonic spasm, occurring in attacks and affecting individual muscles or groups or muscles, and as a rule symme- trical muscles are involved. The muscles of the extremities are more fre- quently affected than those of the other parts ; the face muscles are gener- ally exempt, and in this respect the disease differs markedly from other forms of choreic and clonic spasm. These clonic convulsions are not severe enough to cause actual movements of the extremities, but in a few cases sud- den contractions of the diaphragm have been accompanied by hiccough and by peculiar respiratory sounds, and in the Case described by Starr the move- ments were strong enough to produce jactitation of the head and of the trunk. In contrast to some of the other diseases described in this chapter, the myotatic irritability is increased only a little or not at all. Electrical ex- citability is not altered. The disease may come on at any period of life between the age of pu- berty and fifty years. It is rare in young children, and the short account of it here is simply given for the purpose of showing its close relationship to the various forms of choreic disorders. Heredity is not a prominent factor in the cases which have been described, but it has been found in several mem- bers of one family. Mental or physical strain and emotional excitement are the chief causes. Cases closely resembling, yet not identical with paramyo- clonus multiplex have been described by Hammond and others, under the title of " Convulsive Tremor." The prognosis is serious as regards a cure of the condition, though some of the cases have been reported improved and a few entirely cured. In the treatment of the disease nothing more can be attempted than regular gym- nastic exercise, and the exhibition of mild sedatives, such as chloral and the bromides, in small doses, in case the spasms are excessive. A mild galvanic current is said by some to have exercised a favorable influence over the dis- ease. BIBLIOGRAPHY. HUNTINGTON'S CHOREA. Berkeley : Medical News, Philadelphia, 1883, vol. xlii., p. 200. Bourneville et Pilliet: Arch, der Neurol, 1887, 1888. Charcot : Progres Medic, 1878. Ewald : Zeitschr. f. kl. Med., vol. vii., 1884, p. 51. Fere: Arch, der Neurol., January, 1884. Greppin, L.: Archiv. fur Psychiatrie, vol. xxiv., p. 155. Gray : Text-book, pp. 321-338. Herringham : Brain, vol. xi., p. 415. Hoffman: Virchow's Arch., vol. cviii., 1887. King : New York Medical Journal, 1885. Marie : Progres Medic, 1886. CHOREIFORM DISEASES. 1 45 Menzies : Journal of Mental Science, October, 1892, to June, 1893. Oppenheim and Hoppe : Arch. f. Psych., vol. xxv., p. 617. Osier : Journal of Nervous and Mental Diseases, February, 1893. Peretti : Berl. Klin. Wochenschr., 1885, No. 52. Phelps, R. M. : Journal of Nervous and Mental Diseases, 1892. Schlesinger : Zeitschr. f. Kl. Medicin, 1892. Zacher : Neurologisches Centralbl., January 15, 1888. HABIT CHOREA, GYROSPASM, ELECTRICAL CHOREA, ETC. Gee : St. Bartholomew's Hospital Reports, vol. xxii. Gowers : Diseases of Nervous System, vol. ii., p. 633 (" Habit-spasm "). Hadden : Lancet, June 14, 1890, and St. Thomas's Hospital Reports, 1890. Henoch : Lectures on Diseases of Children. Janowitz : These de Paris, July, 1891. Mills : Chapter on Imperative Movements, etc. American Text-Book of Diseases of Children, p. 712. Osier: Monograph. On Chorea, 1894. Peterson: Medical News, October 1, 1892. Weir Mitchell : Medical News, March 4, 1893. MALADIES DES TICS. Charcot : Lecons du Mardi, 1887-88. Dana: New York Medical Record, 1887. Gilles de la Tourette : Archives de Neurologie, 1885 ; Nos. 26 and 27. Grasset : Arch, de Neurologie, 1890. Guinon, G. : Revue de Medecine, 1886, i., 1887, p. 500. Hammond : New York Medical Record, February 27, 1892. Jolly : Charite Annalen, 1802, p. 740. thomsen's disease. Bernhardt: Virchow's Archiv, 1879, vol. lxxv. Dejerine et Sottas (Case with autopsy) : Compt. Rend, de Biol., Paris, 1893 (not accessible to author). Erb : Monograph. Leipsic, 1866. Eulenburg and Melchert : Berliner Kl. Wochenschr., No. 38, 1885. Eulenburg : Neurologisches Centralbl., 1886, p. 265. Jacoby, G. W. : Journal of Nervous and Mental Diseases, March, 1887. Marie : Revue de Medecine, 1883, p. 1064. Seifert: Arch. f. Kl. Med., 1891, vol. xlvii., p. 127. Striimpell : Berliner Kl. Wochenschrift, 1881. Thomsen, J.: Archiv f. Psychiatric vol. vi., 1876, p. 702; also vol. xxiw, 1892, p. 918. Westphal : Berliner Kl. Wochenschr., 1883. White: Digest, Brain, vol. ix., p. 112. 146 THE NERVOUS DISEASES OF CHILDREN. PARAMYOCLONUS MULTIPLEX. Bechterew : Neurolog. Centralblatt, 1887, p. 181. Friedreich : Virchow's Archiv, vol. Ixxxvi. Homen : Arch, de Neurologie, 1887. Jolly: Charite Annalen, 1892 (On " Tics "). Marie : Progres Medical, 1886. Remak : Arch. f. Psychiatrie, vol. xv. Seeligmuller : Deutsche Med. Wochenschr., 1886, No. 24. Starr : Journal of Nervous and Mental Diseases, July, 1887. Unverricht : Die Myoclonic Vienna, 1891. CHAPTER VII. TETANUS. In view of recent bacteriological researches tetanus may be described as an infectious disease of the central nervous system, characterized by continuous tonic spasms with oc- casional attacks of clonic movements. The muscles first affected are, as a rule, those of the neck and jaw. Opistho- tonus and lock-jaw (trismus) are the first and most startling symptoms of the disease. According to its origin we dis- tinguish between traumatic tetanus resulting from a wound, and idiopathic or rheumatic tetanus if the cause is unknown, or if, in our ignorance, we attribute it to exposure to wet and cold. We must take special note of the tetanus oc- curring in new-born children (tetanus neonatorum), and may make a passing reference to puerperal tetanus. There is every reason to believe that traumatic injury, with the attendant invasion of the bacilli of tetanus, is the primary cause of the trouble in every instance. Etiology. — Tetanus is far more frequent in males than in females. This is particularly true of those cases in which the traumatic factor is evident, and is easily explained by the greater liability of the male sex to traumatism. The very fact that of 46 so-called idiopathic cases recorded by Gowers only 9 were in females would lead one to doubt the spontaneous origin of these cases. While tetanus may occur at any time of life, it is most frequent between the ages of ten and forty years. It occurs within the first few days of life between the fourth and eleventh days, but be- tween this time and the age of five years there is, practically, immunity. Between five and ten years the tendency to the disease is on the increase, and continues to increase un- til after the age of forty years. These statistics strengthen 148 THE NERVOUS DISEASES OF CHILDREN. the impression that traumatic injury alone, plus infec- tion, is the only feasible explanation of the occurrence of the disease. Colored races, and particularly those living in tropical countries, are affected more frequently than white people. This has been amply proved in regard to the population of India and the West Indies. The filth and uncleanly habits, so common among these people, should be borne in mind. Puerperal tetanus, which is unquestionably infective in character, occurs very frequently still in these same coun- tries, but it is becoming less and less frequent in those countries in which modern surgical principles prevail in obstetrical practice. The previous health of the person seems to have little to do with the development of the disease. The most robust as well as the feeblest are equally liable to the infection. Irregular wounds, burns, scratches, and frost-bites are more often the cause of tetanus than the large aseptic incisions made by the surgeon's knife. Tetanus has been known to follow the extraction of a tooth, and, in the popular belief, lock-jaw is much feared after slight injuries to the toes in paring nails, etc. With greater cleanliness among the masses the disease will no doubt soon diminish. Tham- hayn states, from a large collection of cases, that tetanus is developed most frequently from wounds in the hand, leg, foot, head and neck, arm and trunk, in the order of fre- quency just mentioned. Injuries to the head have often been supposed to be peculiarly liable to produce tetanus ; but as such injuries are generally the result of a fall and contact with the earth, as in a fall from a horse, it will be seen that the danger of infection is greater than in many other instances. The same is true of tetanus following a splinter which has been run under the nail, and the old- fashioned method of stopping bleeding by putting cobwebs around the bleeding part may be very largely responsible for the tetanus. The micro-organisms of tetanus evidently do not require a large abraded surface, and it is question- able whether, even in those cases in which tetanus comes on after flogging, there was not sufficient abrasion of the epithe- lium to permit of the invasion of these minute organisms. TETANUS. 149 As a rule, tetanus is developed within a period of five to fourteen days after the initial injury; but, in some cases, a few hours are sufficient for the development of the disease, and in cases in which the crural nerve has been enclosed by a ligature, full-fledged tetanus of the severest type was observed at once after the occurrence. The development of tetanus is not rare within three or four weeks after the injury, but beyond such a period the occurrence of tetanus could not well be explained. That infection alone will not explain all the circumstances in connection with the de- velopment of tetanus will be insisted on in the section on pathology. Symptoms. — The first symptoms of tetanus, if we except vague pains at the seat of injury, and a dull headache, is a gradual stiffening of the muscles of the neck and of the jaw until the head is markedly retracted (opisthotonus) and the jaws are firmly clenched (trismus). While the cer- vical muscles are apt to be involved at the beginning the dorsal and lumbar apparently escape ; but retraction of the head is soon followed by arching of the back. As the disease continues the legs become rigid, but the arms es- cape or are but little involved. The muscles of the face are often affected at an early stage of the disease. The eye- brows are raised, the ocular fissure becomes narrow, the mouth is distorted, the lips press against the teeth and the entire face may assume what is known as the " risus sar- donicus." The rigidity of the muscles is painful, and the oc- casional clonic exacerbations which occur tend to make the condition still more distressing. The difficulties of degluti- tion and of respiration, from spasm of the thoracic muscles and of the glottis, increase the agony of the patient and are a continuous menace to life. Epigastric pains (pains possibly due to spasm of the diaphragm) are frequently complained of. During sleep the spasm usually ceases, but no sooner does the patient Avake up than all the distressing symptoms return in full force. The pulse is rapid and feeble. Whether vasomotor spasm is the cause of this peculiarity of the pulse has not been sufficiently determined. The temperature varies considerably ; in some instances it remains normal during 150 THE NERVOUS DISEASES OF CHILDREN. the entire course of the disease, in others there is a con- tinuous fever of from two to three degrees. H. C. Wood refers to the serrated tracings of the temperature chart, the rise coinciding with the paroxysm, and the fall of the line with the interval. Occasionally the temperature rises to io8° or even no F., and according to Wunderlich this high temperature continues for some hours after death, reaching even to 1 14 F. It was formerly supposed that the rise of temperature was due to excessive muscular action ; but cases in which muscular action is greatest are not nec- essarily those with highest or even high temperature. Gowers is inclined to consider the fever to be of nervous origin, which is probably correct if modified to mean that the toxines circulating in the system exercise a special ir- ritating influence upon the centres in the pons and spinal cord, or upon the heat areas in the cortex. Thirst is a fre- quent and most distressing symptom of the disease, and as it cannot be easily quenched, on account of the difficulty of deglutition, it adds much to the discomfort of the patient. The thirst is also increased by the very profuse perspira- tion which sets in and is a prominent factor in many of the cases. The urine is scanty and high-colored, of high specific gravity, all of which can be explained by the excessive ac- tion of the skin. Micturition is often irregular, possibly in consequence of spasm of the sphincter. The bowels are, as a rule, constipated. All the symptoms are apt to increase in intensity as the disease progresses. The majority of cases run to a fatal termination in less than a fortnight, often in four to five days. Death occurs, as a rule, either from failure of the heart or from asphyxia in consequence of spasm of the re- spiratory muscles. In other cases exhaustion from the con- tinued spasms and from the inability to take food may be the ultimate cause of death. Cases lasting above a fortnight are supposed to tend toward recovery. The spasms be- come slighter, the clonic spasm less frequent, and all the symptoms gradually recede until nothing but a slight rigid- ity of the muscles remains. The parts last involved are first released, and the spasms last longest in the muscles of the TETANUS. 151 neck and jaw, the very parts which were first affected. A single recovery seems to provide immunity against the dis- ease for all time. While the evidence upon this point is not absolutely conclusive, the fact of immunity after the first attack would be in keeping with what we know about immunity from other infectious diseases. Varieties.— The majority of the cases are so much alike that it would be quite useless to speak of variations from the clinical type. An exception must be made, however, in favor of what Rose has termed " cephalic tetanus." This form develops, as a rule, after injury to the head, and partic- ularly to the region of the face supplied by the fifth nerve. The special characteristics of this form are the association of paralysis of the face on the same side as the injury with tetanic spasms of the other side. In addition to this we find spasms of the respiratory muscles and great difficulty in deglutition. The behavior of the patient is very much like that of one suffering from hydrophobia, hence this form has also been spoken of as " tetanus hydrophobicus." There is some doubt whether injury to the peripheral nerve is the cause of the facial palsy. Bernhardt and Remak proved conclusively that there was no reaction of degen- eration in the nerve or muscles, and the nerve has been found healthy on post-mortem examination. We must suppose, therefore, that the facial palsy in such cases is of reflex origin. Some support is given to this view by the fact that if recovery sets in, the tetanic spasm and the facial palsy disappear at about the same time. The only other variety worthy of special mention is the so-called abortive form of tetanus described by Kussmaul. In these cases spasm of the neck muscles and trismus are the only symptoms, and these gradually disappear. Tetanus neonatorum can hardly be considered a variety of tetanus, for it resembles in every way the tetanus of the adult, but in this book it will naturally deserve some spe- cial consideration. The tetanus of the new-born child sets in, as a rule, be- tween the fifth and tenth days after birth, though it may be delayed as long as twenty days. The first symptom noticed in the child is difficulty in taking the nipple or in drinking 152 THE NERVOUS DISEASES OF CHILDREN. from the bottle ; with every attempt there is distinct rigid- ity of the masseters and of the muscles about the mouth, which interferes with the act of sucking and swallowing. Other facial muscles are apt to be in a state of contracture leading to a distinct deformity of the face. In some in- stances food can be poured into the mouth by a spoon with- out exciting a spasm, but in many others the mere touch of the spoon to the lips, or contact of the food with the lips and mouth, is sufficient to produce a spasm. In the earlier stages of the disease the child is quiet un- less food is given; but before long the spasms come on spontaneously without excitation by food. Spasm of the respiratory muscles soon forms a part of the clinical symp- toms, and periodic cyanosis is often one of the early symp- toms of the tetanic condition. Rigidity of the muscles of the neck and back, typical opisthotonus, and arched back soon follow, and to make the symptoms thoroughly com- plete the upper and lower extremities become the seat of muscular spasms. There may be slight remissions for a time, but the tetanic stage becomes more and more perma- nent, or, if absent when the child is quiet, will surely come on with the merest touch in lifting the child, in trying to give it an enema, in washing it, etc. The temperature, as a rule, remains normal ; in a few instances it reaches 102 or 103 F. The disease is steadily progressive. One is often de- ceived by slight remissions and is flattered by the tempor- ary good result of sedative treatment, but is only too often disappointed by the reappearance of all the symptoms, with even greater intensity. Death may follow within a period varying from one and a half to eight or nine days, and re- sults either from mere exhaustion, due to the inability to take food, or from asphyxia, due to spasm of the inspiratory muscles. A few cases of tetanus neonatorum get well, but surely only a very few. Cases with fever seem to be invariably fatal. In those who get well the tetanic spasm disappears very slowly, and Henoch states that in some instances a rigidity of the muscles existed three weeks after the onset of the disease. TETANUS. 153 The cause of this early tetanus is in every way the same as that of tetanus in the adult, except that the en- trance of the bacilli into the body occurs by way of the umbilical cord, the care of which has been neglected. In former days, and particularly in those countries in which obstetrical cases were managed exclusively by midwives, whose ideas of cleanliness were very defective, tetanus neonatorum was very much more frequent than it is now. Ritual circumcision, if performed without due regard to modern surgical principles, may be considered an occasional cause of tetanus. Bathing a child at low temperatures is referred to by many of the older writers as a common cause of tetanus. The use of a bath thermometer is said to have put an end to an epidemic of tetanus occurring in the practice of a midwife who was not able to distinguish by the hand alone a high temperature of water. In view of the modern bacillary theory of tetanus such an origin of tetanus would be rather difficult to explain ; but we must concede the possibility of other than mere toxic agents affecting and irritating the peripheral nerves of a newly born child. Pathological Anatomy and Morbid Pathology. — Careful search has been made by many writers for the act- ual lesion of tetanus, but up to the present time with few positive results. It has been noted that rigor mortis sets in with unusual promptness, some claiming that the tetanic spasm passes without relaxation into the post-mortem con- tracture. All the organs of the body have been examined ; no special changes have been found in them. Some writers have reported oedema, hypostatic pneumonia, pleural ex- travasation, which can be accounted for by the inter- ference with the circulation and respiration. The muscles of the body are sometimes ruptured ; some contain small hemorrhages, but under the microscope the fibres present no anomalies, though Bowman refers to occasional granular degeneration. It has been natural to look for changes in the nerve filaments in the peripheral wound which has been the starting-point of the tetanus. In some instances noth- ing abnormal has been found ; in others there has been neuritis, with considerable swelling of the nerve, which ex- tended from the periphery to the spinal cord. 154 THE NERVOUS DISEASES OF CHILD REX. In the case of tetanus neonatorum an inflammation of the umbilical vessels has been traced for some distance within the abdomen, and the peritoneal covering of the vessels has also been found inflamed. As for the central nervous system, the condition most frequently discovered is one of passive distention of the vessels, associated with minute hemorrhages : but both these conditions are surely secondary to the tetanic spasms and not in any sense the cause of them. In the spinal cord some investigators have reported granular disintegration of the gray substance, the formation of cavities containing gran- ular material, and changes also in the large ganglion cells, such as were found by Xerlich in the motor root of the fifth nerve. All of these changes are not suffi- ciently distinct to represent the actual pathological con- ditions of tetanus ; they are secondary effects, and it is probable that in tetanus, as in other of the diseases hitherto considered, definite though the symptoms may be, the changes are of a transitory character, every trace of which disappears after death. It would be more important, therefore, to refer to the morbid pathology. The pathology of tetanus can be readily understood in the light of modern researches if we regard the spasms of tet- anus as the result of increased irritability of the convul- sive centres in the brain. This increased irritability is di- rectly due to the influence of germs or germ products introduced into the system from some external injury. The tetanus bacillus (Nicolaier) is a rod-shaped microbe whose spores are attached to one end of the bacillus, which with its spores resembles in appearance an ordinary pin. This microbe is found in the soil as well as in the dust of dwellings. These bacilli are capable of resisting great heat, Fig. 47. — Tetanus Bacilli and Spores. (x about 1,000. ) (Kkasato.) TETANUS. 155 retaining their activity even after exposure to 175° F. for an hour (Kitasato, ZeitscJirift fiir Hyg., vol. vii.). Their spores are capable of resisting the same heat for six hours, but the spores are not formed if the temperature exceeds 108° F. The bacilli thrive in an atmosphere of hydrogen or in vacuo. They are killed by exposure to the oxy- gen of the air and by a five per cent solution of carbolic acid. As products of these bacilli we have several poisons (tetanin, tetano-toxin, and also tox-albumhis, Brieger), which have been derived from pure cultures and which are ca- pable of exciting the disease. It is probable that the tetanus bacillus itself does not cause the disease, but that the poi- sonous substances formed in the blood by the presence of this bacillus are the direct cause of tetanus. It is an inter- esting fact that granulating surfaces do not offer a suitable soil for the production of these toxic substances. The presence of oxygen seems to prevent the penetration of the bacilli into the tissues underneath ; hence we can readilv understand why scratches and hidden injuries should be far more dangerous than large abraded surfaces. Tetanus was supposed to result from direct irritation of the peripheral nerves ; if so, an ascending neuritis would have to account for the development of the symptoms of tetanus. It is at present altogether impossible to deny that in some way or other the bacillus must have been intro- duced into the system even in these cases.* Lesions of the occiput were supposed to lead more fre- quently than other lesions to tetanus, but in this instance, too, it is more than probable that the infectious element cannot be disregarded. In no disease have bacteriological researches led to more satisfactory results in treatment. Tizzoni cultivated the virus, inoculations of which in increasing strength pro- duced tetanus in dogs. The blood-serum of such dogs destroyed the activity of the virus. He inferred the pres- * Since the above was written Goldscheider has given a different explanation based upon recent anatomical conceptions. The poison of tetanus acts upon the peripheral nerve-fibre. This fibre being the peripheral portion of the neuron, an increased ex- citability of the ganglion cell is produced, and this hyper-excitability brings about the tetanic contractions of the muscles with which it is connected. 156 THE NERVOUS DISEASES OF CHILDREN. ence of an albuminoid body, which he called "anti-toxin/* with which he succeeded in arresting the disease in rats. The now famous researches of Behring and Kitasato have revealed the fact that the blood-serum of tetanic ani- mals produces immunity in others, and that the serum of the animals thus rendered immune had still more active anti-toxic qualities, and during the past year (1894) Behring has shown that the blood-serum of immunized animals not only produces immunity in others, but also has a distinctly curative effect. Tarnuffi (Centralblatt fur Barter iologie, 1892) reports a successful use of anti-toxin injections in the case of a peasant, aged seventy-four, who had developed tetanus as the result of a lacerated wound of the little finger of the right hand. One hundred and fifty grammes of the anti- toxine was given in this case, when on the eleventh day after the beginning of the injections a complete recovery ensued. The blood-serum from this patient did not produce tetanus when injected into a rat. No doubt other success- ful cases of this kind will soon be reported, and there is much to hope from this serum therapy in the case of tet- anus. Differential Diagnosis. — The diagnosis of tetanus is easy in the majority of cases. It is possible to mistake tetanus for strychnia poisoning, but in the latter the symp- toms never begin with distinct trismus. The symptoms also develop with much greater rapidity, and the severe pain referred to the stomach, as well as the absence of ex- ternal injury, would lead one to suspect the effect of strych- nia. If there is great difficulty in deglutition the symptoms of tetanus may resemble somewhat those of hydrophobia, but the entire absence of trismus, and the fact that all the symptoms are excited only if an attempt is made to swallow, and the previous history of the case, will enable one to dis- tinguish hydrophobia from true tetanus. As for mistaking tetanus for tetany, such confusion is scarcely possible if the mode of onset from the periphery inward, in cases of tetany, is kept in mind, and if we also remember that no such interval exists in tetanus as in the lesser disease. The favorable course of the disease will TETANUS. 157 render still further aid, after the lapse of a few days, in dif- ferentiating between tetany and tetanus. Prognosis. — The prognosis is invariably grave. A mor- tality of eighty -five per cent, does not overstate the facts. Other things being equal, the prognosis is the more grave the more thoroughly a lacerated wound has been infected. Recovery in a case of tetanus after compound fracture of the limbs is very rare. The longer the interval between the injury and the first onset of the symptoms the better the prognosis, and cases which begin after ten days usually take a more favorable course than those which come on very early after the injury. If the symptoms have lasted more than ten days there is some reason to expect a fa- vorable result. On the whole it is a disease very much to be feared, and a guarded prognosis is quite in order until a very decided improvement sets in ; but possibly the recent discoveries of Behring may in the course of time enable us to be more hopeful. Treatment. — In the treatment of tetanus, prophylactic measures are by far the most important. There is no good excuse at the present day for the infection of any surgical wound, or if the wound has been received under conditions which make infection possible, thorough antiseptic measures should be at once employed, and even after the first symp- toms of tetanus have been observed the condition of the peripheral wound should be carefully examined into and everything should be done to prevent further contamina- tion and infection. If recent investigations are carried to a successful issue, the most satisfactory and the most rational treatment of tetanus will unquestionably be that by injections of anti- toxin or any of the substances which may hereafter be proved to possess the property of counteracting the poison of tetanus in the human system. It is a consummation most devoutly to be wished, but though we are nearing the goal the struggle is not yet at an end. In the majority of cases, and particularly in those occur- ring at a distance from large medical centres, from which for years to come these anti-toxins will have to be obtained, the older methods of treatment will still have to be resorted to. 158 THE NERVOUS DISEASES OF CHILDREN. The general management of the patient is the first im- portant consideration. Absolute quiet should be secured by darkening the room, keeping out all visitors and noises. Nutritious but liquid diet should be given, and given slowly, so that the difficulties in deglutition may not be increased. If the trismus is very marked a wedge may have to be in- serted between the teeth to secure enough space to intro- duce a tube through which liquid can be taken into the mouth. If this cannot be done, the patient must be fed through the nose or else by the rectum. In former times it was considered wise to remove a peripheral scar, or even to amputate the part that included the seat of injury ; but according to our present conception the presence of tetanus bacilli in the system would prove that the disease was no longer a local one, and that excision of the lesion would not prove sufficient to bring about re- covery. Among the drugs employed in the treatment of this dis- ease chloroform by inhalation is unquestionably the most satisfactory. Woods, of Philadelphia, has recommended the use of nitrite of amyl, and Gowers reports that its use at Guy's Hospital has proved that the spasms became more in- tense at first but slighter afterward. Both chloroform and nitrite of amyl are palliative measures, but not curative in any true sense. Chloral hydrate has an advantage over the preceding drugs, inasmuch as it helps to produce sleep, and can be given continuously for a long period of time. To be effectual large doses of five to fifteen grains should be given at a time, and repeated several times during the day according to the needs of the case. There can be no reasonable objection to the use of opium and morphia in the form of suppositories, or by hypo- dermatic injection. Morphia may be exhibited in doses of one-twelfth to one-sixth grain, according to the age of the child, and can be given in connection with other nar- cotics. The use of conium and of gelsemium has been warmly recommended by various observers, but the results are not satisfactory enough to advise the general adoption of such measures. Curara in doses of one-fiftieth to one- half grain every hour has been urged by some ; but it is TETANUS. I 59 surely a dangerous drug, and its application would be rather in the nature of an experiment than of safe thera- peusis. Continued warm baths, electricity (galvanism to the spine and the muscles), all these measures have been ad- vocated by some, but without much result. If the bromides, chloral, opium or morphia, and chloroform, judiciously ad- ministered, do not bring about a favorable result it is safer not to exhibit other drugs. In the treatment of tetanus neonatorum the same drugs may be employed as are recommended in tetanus of the adult, but the dosage should be modified in keeping with the age of the patient. BIBLIOGRAPHY. Behring and Kitasato : Deutsche Med. Woch., Dec. 4, 1890, and 1892, p. 348. Brieger: Deutsche Med. Wochenschrift, 1887. Conner, P. S. : Article Tetanus, Pepper's System, vol. v. Frothingham : Tetanus Bacillus, American Journal of the Medical Sciences, May, 1894. Goldscheider : (Action of Tetanus Toxin, etc.) Zeitschrift f. kl. Med., vol. xxvi., p. 175. Gowers : vol. ii., p 674. Kitasato : Zeitsch. f. Hygiene, Bd. vii. ; also Deutsch. Med. W., 1889, No. 31. Nicolaier : Inaug. Dissert. Gottingen, 1885. Nerlich : (Cephalic Tetanus) Arch. f. Psych., vol. xxxiii. Rose, Pitha and Billroth : Handb. d. Chir., 1872. Runge : Die Krankheit. d. ersten Lebenstage. Stuttgart, 1893. Rosenbach: Arch. f. kl. Chirurg., 1887. Schultze, F. : Neurolog. Centralbl., 1882. Tarnuffi : Centralblat f. Bacteriologie u. Parasitenkunde, 1892. (Quoted in an Editorial, Medical and Surgical Reporter, Nov. 2, 1893.) Thamhayn : Schmidt's Jahrbiicher, 1861. Tizzoni u. Cattani : Berl. kl. Wochenschr., Dec. 4, 1893, and June, 1S94. CHAPTER VIII. TETANY. Tetany or tetanilla is a disease which occurs with far greater frequency in Europe than in this country. Osier and Weir Mitchell speak of its exceeding rarity in the United States. It occurs with about equal frequency in adults and children. , As the name indicates, " the little tetanus" is characterized by attacks of tonic spasms of various groups of muscles, particularly of those of the upper extremities. It is only within recent years that the disease has been prop- erly studied, though the first account of it goes back as far as the year 1830, when Steinheim described this group of symptoms as a special form of articular rheumatism. In the following year Dance published an article in which he expressed the view that the intermittent character of the spasms proved the disease to be of a malarial order. The name of the disease we owe to Corvisart, who in 1852 re- viewed the entire subject. His observations were preceded, however, by those of Trousseau, who had observed the disease in nursing women, and supposed a connection be- tween tetany and the function of lactation. He therefore termed it " contractu re rheumatismal des nourrices," but later on he observed the trouble in children and in adults with intestinal obstruction, and so abandoned the lactation theory. It was Trousseau who first discovered the very important fact that these attacks could be excited by com- pression of the arteries and nerve-trunks of the affected ex- tremity. Our knowledge of this disease has been enhanced by the observations of Chvostek, Koppe, Baginsky, Von Jaksch, Bernhardt, and others. The most comprehensive mono- graphs on the subject have been written by Weiss, of Vienna, in 1 88 1, and by Frankl Hochwart in the same year. TETANY. 161 Symptomatology. — The symptomatology of tetany in- eludes the symptoms to be noticed during the attack and those during the period of latency. The attack is preceded Fig. 48. — Position of Hands in the Spasm of Tetany. (After Oppenheim,) by vague tingling pains, by formication in the hands, fore- arms, and legs; a feeling of stiffness soon follows, and subse- quently the spasm of the muscles sets in. This tonic spasm 1 62 THE NERVOUS DISEASES OF CHILD REX. occurs more frequently in the upper extremities, and gives rise to such a marked rigidity of the muscles that passive movements are for the time being impossible. The position of the hand during the spasm will necessarily vary accord- ing to the groups of muscles affected. It is a common occurrence for the hand to assume the shape of the accoucheur's hand. Occasionally also the thumb is very firmly pressed upon by the flexed fingers and the nails are buried in the skin of the palm. In some rare cases there is complete extension of all the fingers. The forearms are gen- erally flexed, and the upper arms closely pressed against the chest. If the lower extremities are involved the thighs may be adducted, the legs extended or flexed, while the toes are apt to assume the position of talipes equinus. The spasms may also affect the muscles of the abdomen, of the chest, and of the back. These tonic contractions of the abdomi- nal and thoracic muscles may interfere with the movements of the diaphragm, and with the respiration, causing dyspnoea and cyanosis. If the muscles of the neck be also involved, the return of the venous blood from the brain may be re- tarded, as reported by Weiss in one case, in which loss of consciousness was the result of such neck spasm. Opistho- tonus is not infrequent, but trismus is rare and never occurs in the beginning of an attack as in tetanus. In severe cases spasms of the ocular muscles have been observed, of the oesophagus, of the larynx, and of the muscular apparatus of the bladder (a desire to urinate but micturition impossible). During the attack the patient complains of severe pain in the affected muscles ; there is also a diminution of tactile sensibility in the extremities, the muscular sense is often deficient, and while standing on the floor patients have a feeling as though they were walking on velvet. Elevation of temperature as high as 104 F. has been ob- served ; but this is exceptional, as Weiss records an average of only one such case in twelve. Headache, vertigo, tinnitus aurium, and excessive perspi- ration are other symptoms which have been noted during an attack of tetany. The attacks may last only a few minutes, or may cover a period of hours, or even days. Se- vere attacks of tetany may bear a striking resemblance to TETANY. 163 genuine tetanus ; but it should be noted that there is no ini- tial spasm of the masseters in tetany, and that in this form the spasm spreads from the periphery inward, and not cen- trifugally, as is the case in tetanus. Moreover, the reflex excitability, is not nearly as great in tetany as in tetanus. In tetany the patients may be entirely free from attacks for hours, and even for days, whereas in tetanus the attack, as long as it lasts, is continuous. Etiology. — The disease occurs chiefly in very young persons. The cases observed by Koppe were all from one to two years of age. Ganghofner reported 40 cases ; of these 5 were between two and three years of age and the remaining 35 between the ages of one month and two years. Gowers tabulated 142 cases ; of these 64 occurred from one to four years, and 36 from ten to nineteen years of age. On going over the literature of the subject a few years ago, taking all cases of tetany into consideration, it was evident that the majority of cases of tetany were observed between the ages of sixteen and thirty-five years. At one time the disease was classed among the professional neuroses, but this fallacy was corrected by Kussmaul. Any exhausting disease may be regarded as a possible etiological factor, but exposure to cold and wet is referred to most frequently as a predispos- ing cause. Intestinal irritation is another cause, and may be associated with eclampsia and laryngospasmus, two con- ditions which we know are also frequently excited by in- testinal irritation. Riegel instances a case in which attacks of tetany were inhibited by the removal of the ova of taenia medio-canellata and tricocephalus dispar. Weiss reports the occurrence of tetany as a complication of typhoid fever. It has also been observed together with small-pox, Bright's disease, malaria, chorea, and even after severe mental shock. The only just inference from all this is that tetany is liable to occur after any exhausting disease in those who are predisposed to this form of spasm. Weiss deserves special credit for bringing into prominence the re- lation between tetany and extirpation of the thyroid gland. The disease sometimes appears in epidemic form, if we are to credit the account of such occurrences in the schools and prisons of France. A similar epidemic occurrence is re- 164 THE NERVOUS DISEASES OF CHILDREN. ported by J. Lewis Smith, Escherich, and others. That it is much more frequent in some countries than in others has already been alluded to. The male sex is affected a little more frequently than the female ; Rilliet and Barthez recording 20 out of 28 cases in boys. In Gowers's statistics of 142 cases, 76 were males and 66 females. Although the disease sometimes occurs in families there seems to me to be insufficient evidence of actual hereditary predisposition to this disease. Symptoms of the Latent Period. — In the interval between the attacks the patient may appear to be entirely well, but if examined carefully he will exhibit a weakness, with slight rigidity of the affected muscles. The calf mus- cles are commonly the seat of mild contractions. Chvostek observed slight contractions of the orbicularis palpe- brarum. The intervals between the attacks of tetany may vary in duration from several hours to a few days, and even a few months ; but, of course, we can speak of a latent inter- val only in case the disease can be proven still to exist. This can be done by eliciting Trousseau's symptoms and by proving an increased electrical and mechanical excita- bility of the parts affected. Trousseau 's Symptom. — The famous French physician discovered that in persons afflicted with tetany a charac- teristic attack can be elicited by pressure upon the large nerve-trunks and the arteries of the extremities usually affected during an attack. The attack ceases as soon as pressure is removed. Kussmaul and Quincke maintain that in some cases pressure on arteries only is necessary r while in other cases the slightest pressure on a nerve-trunk is sufficient to produce contractions of all the muscles sup- plied by this nerve. Trousseau's symptom is absolutely pathognomonic of this disease. Increased electrical excitability has been found to be characteristic of tetany by Erb, Chvostek, Weiss, and oth- ers. According to these authors the faradic and galvanic responses of the motor nerves are enormously increased dur- ing the interval between the attacks of tetany. They were not only able to obtain kathodal closure contractions (K. TETANY. 165 C. C.) with very small currents, but were able with moder- ate currents to obtain a kathodal closure tetanus, and even an anodal opening tetanus, which has not been observed in any other condition. Chvostek and Weiss claim that this phenomenon is exhibited in the facial and in other periph- eral nerves. Erb found the electrical excitability greatest at the time when the attacks were most frequent. Increased mechanical excitability is a still more striking symptom of the conditio*??, a simple touch with a percus- sion-hammer upon a nerve-trunk being sufficient to pro- duce contractions of the muscles supplied by the nerve. I remember distinctly the cases in the Vienna General Hospi- tal in which pressure with a lead-pencil upon the focal point of the pes anserinus was followed by contractions exactly like those which a strong faradic current applied to this part Avould have produced. This is by far the most con- venient test to make in cases in which the existence of tetany is suspected, and it is far better to endeavor to establish this fact of increased mechanical excitability than to excite an attack by pressure upon a large nerve-trunk or a large artery. Differential Diagnosis. — There can be but little difficulty in differentiating between tetany and other con- vulsive disorders. From tetanus it can be distinguished sufficiently by the intermittent and centripetal character of the attacks, by the absence of trismus at the beginning of the attack, and, above all, by the presence, during the in- terval, of Trousseau's symptom, and the increased mechan- ical and electrical excitability. I have known a case of frequently repeated epileptiform convulsions to be mis- taken for a case of tetany, but the loss of consciousness, the universal tonic and clonic movements, the turning of the eyes, the stupor following an attack, and the extreme short- ness of the attack, are the symptoms that will help to dif- ferentiate the epileptiform attack from one of tetany. Morbid Anatomy and Pathology. — Post-mortem ex- aminations of cases of tetany have been made bv Langhans and Weiss, but up to the present dav there have been few positive pathological findings. Langhans claims to have found a periarteritis and periphlebitis of the white commis- 1 66 THE NERVOUS DISEASES OE CHILDREN. sure and of the anterior horns of the cervical portion of the spinal cord. Weiss failed to find any such changes in his cases. He formulated an ingenious theory of the disease according to which the attacks of tetany are due to an irri- table condition of the gray matter of the medulla and spinal cord. This irritable condition is the result of sympathetic disturbances causing irregularities in the vascular innerva- tion of the blood-vessels of the spinal cord. Gowers is in- clined to look for the chief changes in the motor cells of the spinal cord, but all this is in the nature of speculative pathology. Schlesinger has very recently reviewed the various theories concerning the nature of tetany. He con- cludes that none of them is entirely satisfactory, while, ac- cording to his own ideas, tetany is a disease of the entire nervous system, some of the symptoms being due to an af- fection of the peripheral nerves ; but the tetanic spasms, as well as Trousseau's phenomena, are held to be the result of increased excitability of the gray matter of the central nervous system, chiefly of the brain, medulla oblongata, and spinal cord. Peripheral irritants of various kinds may give rise to vasomotor disturbances in the spinal cord, and these may be the cause of functional changes. This last theory is but a little more satisfactory than the preceding ones, for nothing is known as yet of that special peculiarity and special irritability of the central nervous system which give rise to this disease. Irritability of the nervous system is so common that something more is needed to explain these very unusual manifestations of the disease. That a zymotic factor enters into the etiology of tetany can scarcely be doubted, but the exact nature of such infection is a matter for future study. The occurrence of tetany after extirpation of the thyroid gland points the way to a future investigation regarding the origin of the disease. If it is the function of the thyroid gland to eliminate mucin from the body, it is natural to infer that the continuance of this toxic substance in the sys- tem is responsible for the tetanic spasm that sets in when the gland is removed by surgical interference. A special caution is in order not to confound ordinary carpopedal spasm, or any of the short clonic and tonic TETANY. 167 spasms that so frequently occur in children, with true tetany.* The term should be restricted to those cases that present distinct attacks and a free interval, characterized by Trousseau's symptom and increased excitability. Prognosis. — The prognosis is favorable excepting in those few cases in which serious lung trouble may result from continued spasm of the respiratory muscles ; but the prognosis should be very guarded with respect to the dura- tion of the disease. It may vary from a few weeks to many months. Treatment. — The removal of every possible peripheral irritation is the first sine qua non of treatment. If intestinal irritation is suspected to be the cause, free purging of the bowels and removal of intestinal parasites are called for. A change of abode may become necessary, and absolute rest will have to be secured at any cost. To shorten the attack the physician will probably have to resort to the hypodermic use of morphine, and possibly to hyoscine. Weiss reports a single case in which the attack was inhibited by the application of ice to the back of the neck. Go wers, who seems to think the spasms of tetany very closely related to ordinary infantile convulsions, advises treatment similar to that employed in convulsions. Proceding on this basis, inhalations of nitrite of amyl and of chloroform may be tried. As soon as the attack is over it would be well to administer chloral hydrate in daily doses of about one-half drachm, or the bromides in doses of from ten to twenty or more grains per day, according to the age of the child. Small doses of sulfonal, or of trional, are worthy of trial. During the interval careful electrical treatment (stabile ascending currents through the peripheral nerve- trunks) as well as prolonged lukewarm baths should be given. There is little doubt but that the majority of cases will get well without any therapeutic measures. The chief duty that devolves upon the physician is clearly that of in- vigorating the central nervous system by the best known methods and remedies, in order to enable it to resist the ordinary irritants which produce the disease in those who are predisposed to it. If the child exhibits any symptoms * Some of the cases reported by Vaughan arc not distinct cases of tetany. 1 68 THE NERVOUS DISEASES OF CHILDREN. of rickets, cod-liver oil and iron will be the best remedies, and treatment, to be successful, will have to be directed entirely to the improvement of the child's defective bone development. The bromides may be administered if the condition is not intense enough to call for narcotics and opiates, and if, for some reason or other, these latter reme- dies should not be employed. Gowers states that a dose of digitalis, given at bedtime, has been found to be the most useful remedy for nocturnal tetany. The action of the digi- talis could be explained only on the theory that the blood- supply of the central nervous system is deficient, and that there is need of increased activity. TETANOID CHOREA. Under this title Gowers refers to a case which has been under his care that exhibited symptoms intermediate between those of chorea and that of tetany. The disease ran a fatal course, but no demonstrable lesions were found after death. The patient was a boy, ten years of age, with a history of three other relatives having suffered from diseases resembling chorea. In the patient the symptoms began seven months before death. They consisted of tonic spasm, which was continuous, and varied by paroxysmal attacks of similar but more intense spasm. The face was involved on both sides so as to cause a constant peculiar smile. The tongue was pressed up against the pal- ate, impeding swallowing and preventing speech. The arms were extended, pronated, and rotated inward so as to bring the back of the forearms outward, while the fingers were flexed at the joints, but at times were extended and moved slowly in a way characteristic of athetosis. The lower extremities were extended at all the joints, the feet being extended in talipes equino- varus, and the toes were flexed. The muscles of the trunk were also in- volved in the spasm. At first the left side was the more severely affected, but afterward the spasm became equal on the two sides. The electrical irritability of the muscles was normal, and there was no mechanical excitability of the nerves. There was considerable pyrexia during the more severe stage of the disease. The boy became thoroughly emaciated, and died from, exhaustion. There is very little of true tetany, and still less of chorea in this case as described by Gowers. While I have seen no case exactly like this one of Gowers, it is not rare to find irregular spasmodic movements of all sorts that may remind one at times of chorea, at times of athetosis, and then again of a cataleptic condition during febrile disorders in children. TETANY. 169 BIBLIOGRAPHY. Abercrombie : On Tetany in Young Children. London, 1890. Baginsky: Tetanie bei Sauglingen, Arch. f. Kinderh., 1886, vii., H. 3. •Chvostek : Zeitschr. f. kl. Med., vol. xix., H. 5 u. 6. Ewald: Allgemeine Med. Zeitung., 1893, lxii., p. 489. Erb : Electrotherapie. 2d Ed. 6. 188 Eulenburg : Tetanie, Eulenburg's Encyclopaedia. Frankl, Hochwart : Jahrbuch f. Psych., ix. ; Deutsch Arch. f. kl. Med., 1886, xliii. Griffith, J. P. C. : Tetany in America, with a Report of Five Cases, Trans- actions of the Association of American Physicians, 1894, ix. Hoche : Versuche mit Curarin bei Tetanie, Neurolog. Centralbl., April 15, 1894. Hoffman : Deutsch Arch. f. kl. Med., 1888, xliii., H. 1. Von Jaksch : Ztschr. f. kl. Med., 1890. Pvifgel : Deutsch Arch. f. kl. Med., vol. xii. Sachs, B. : Article Tetany, in Wood's Reference Handbook. Schlesinger, H. : Versuch Einer Theorie der Tetanie, Neurolog. Centralbl., 1892, p. 66. Schultze : Deutsch Med. Wochenschr., 1882, Nos. 20, 21. Vaughan : Some Cases of Tetany, Medical Journal, Dec. 24, 1893. Weiss : Volkmann's Sammlung, No. 189, 1881. CHAPTER IX. HEADACHES. It requires no little skill on the part of the physician to discover the true cause of headaches. They do not consti- tute a form of disease, but are symptomatic of many or- ganic and functional conditions. For this reason we must give them the very closest study. The diagnosis of the kind of headache in the adult is difficult enough, but it is doubly so in the case of children. As the headaches that come on in early life are frequently continued in later years and give rise to much suffering, it is particularly im- portant that an effort should be made to nip them in the bud. The most distressing form of headache and the one most stubborn in its resistance to treatment, migraine, will be considered in a separate section. The remaining forms may be classified as follows : Headaches due to : i. Anaemia and malnutrition. 2. Neurasthenia. 3. Transitory hyperaemia, as it occurs in heart disease or at the period of menstruation. 4. Gastric disturbances. 5. Genital irritation. 6. Ear disease. 7. Organic disease of the brain or its coverings (includ- ing specific disease). 8. The prodromal stage of acute infectious diseases. 9. Malaria. 10. Uraemic poisoning. n. Other toxic conditions. It was the fashion formerly to make a differential diag- nosis of the forms of headaches according either to the HEADACHES. 171 character of the headache or to the seat of the distribution of the headache. Thus frontal headaches were considered to be due to gastric disturbances. Occipital headaches were considered to be almost pathognomonic of uraemic poison- ing ; but he who has an opportunity of seeing a large number of cases of headaches in the adult, as well as in children, will soon convince himself that these signs are often misleading. I have seen frontal headaches with disease of the kidneys, and occipital headaches from gastric disturbances and in cases of specific disease, of tumor of the brain, etc. The diag- nosis of the kind of headache can, as a rule, be made after carefully examining into the general health of the patient, Constipation ) Caries of Incisor ) Errors of Refraction Gastric Dyspepsia i Anaemia J Eye Decayed Teeth ( Pharyngitis 1 Otitis Media Fig. 49. — Location of Head-pains. After Dana (slightly altered). Area I. Trigem- inus and Facial Nerve Strands. Area II. Upper Four Cervical Nerve Strands. and endeavoring to find what the fundamental disturbance may be. It will be better for us, therefore, to take the eti- ological conditions mentioned above, and to endeavor to connect with each condition a few signs that are generally associated with a particular form of headache. 1. Headaches due to anaemia. These headaches occur frequently in children between the ages of eight and fifteen years ; they are sometimes frontal, generally vertical, and are described, as a rule, as a dull, boring headache, and in the majority of cases are most pronounced earlv in the morn- ing. They are frequently attended by a slight vertigo and a feeling of faintness, but none of these symptoms is char- acteristic enough to make the diagnosis of anaemic head- ache unless the headaches due to eve-strain, to neurasthenia. 172 THE NERVOUS DISEASES OF CHILDREN. or to urasmic conditions can be excluded. The pallor of the conjunctivae, which may be obscured by a catarrhal condition very frequent in children, and above all the pal- lor of the gums and of the roof of the mouth, and a defi- ciency of red blood-corpuscles are the signs which help to make the diagnosis of anaemic headaches certain beyond peradventure. The result of treatment will also furnish corroborative evidence. The child should be placed at rest, fatigue should be avoided in every possible way, and its diet should be nutritious to the extreme. It is important not only to give nutritious food, but to exclude everything that is of no decided value to the child's condition. If we allow an excess of fruit or of sweetmeats the child will very natu- rally look for these rather than for meat, fish, eggs, oysters, which should constitute its main diet, with a slight admixt- ure of farinaceous substances. One to two quarts of milk per day, three or four eggs, a pound or more of meat will be all of far more benefit to the child than any number of drugs. They may help a little, but I am certain that it is only a very little; and if there is any question of the child's power of assimilating these drugs I much prefer to give them up rather than to diminish the amount of food that the child takes. In the treatment of children's diseases the practical necessity of prescribing something often puts physicians at a great disadvantage. The era of rational therapeutics has not yet fully dawned. These anaemic headaches will be relieved in some in- stances by the proper use of hydrotherapy. It is a good plan to bathe these children every morning immediately upon rising. Sponge the head and spine first with warm water and then with cold, allowing the water to drip from a little height, so as to get the effect of the impact of cool water. After this they should be briskly dried and allowed at once to dress. In the case of very weak children it would be better to have the child take a little nourishment, say a glass of hot milk, before putting it through this pro- cedure. In addition to this the child should be given regular calisthenic exercises, which help to improve the general condition and to develop the muscles, and to pre- HEADACHES. 1 73 vent the accumulation of fat, which is not infrequent in children of anaemic disposition. The meals should be given at regular intervals, and in cases in which the appetite is poor the plan of giving small quantities at frequent intervals, say every two hours, is to be preferred to that of only three meals during the day. The most useful drug to be employed in the treatment of these headaches is caffeine in doses of one to two grains every hour, or at least three times a day. Black coffee will often answer the same purpose. From the combination of phenacetine and caffeine I have seen very little benefit, and it surely has no advantage over the administration of caf- feine alone, and is possessed of the disadvantage that phe- nacetine acts unfavorably upon the hearts of some children. If iron is to be given let it be administered in the most easily digested form, either in the form of reduced iron, or in one of the many preparations of the peptonates or albu- minates of iron that are now in the market. Arsenic can be given in small doses of from three to four drops of the Fowler's solution several times a day. In cases in which a general tonic effect is desired, we can give a palatable com- bination of iron and quinine with small doses of strychnine, from one-eightieth to one-sixtieth of a grain, three times a day. Why phosphorus should be recommended by some I cannot understand. I am certain that no one can conscien- tiously assert that he has seen any good effect from its use, and whether given in pill form or in the form of Thomson's solution, the effect in my experience upon cases in which I have known it to be tried has been absolutely nil. The fewer the drugs employed, and the more attention is paid to the hygiene and the diet of the child, the more quickly this anaemic condition will disappear. 2. Headaches due to neurasthenia. These headaches occur so frequently in persons who are at the same time anaemic, that the treatment of one cannot be considered apart from the treatment of the other. In anaemic persons headaches very often do not occur until they have been subjected to some emotional excitement, or have passed through some fatiguing ordeal. These headaches can be recognized as due to some other cause than to anaemia 174 THE NERVOUS DISEASES OE CHILDREN. alone by the presence of other symptoms expressive of the neurasthenic state, such as slight tremor of the tongue and fingers and the exaggeration of the deep reflexes, partic- ularly the knee-jerks ; but above all by the very, persistent location of these headaches on the crown of the head, and by the description of them as a pressure or a feeling of heat on the top of the head. This description of the headache is as characteristic for children as it is for older patients ; and in children emotional conditions, the strain of school work, the rivalry between classmates, is quite as apt to bring about a neurasthenic state and to cause neurasthenic head- aches as are the more serious struggles for existence in later life. The treatment of neurasthenic headaches may be con- ducted on exactly the same lines as was referred to for those due to anaemia. The hydrotherapeutic procedures and rest are by far the most important, and strychnine and quinine in small doses will be of far more benefit than if anaemia were the only cause ; but a cure cannot be easily ef- fected unless all exciting conditions are removed, and to that end the child should be taken from school, and in some instances a change of climate and of home surroundings may be necessary. If this is not sufficient a short rest in bed with forced feeding may be attempted, for the " rest- cure" plan is as efficient in children as it is in adults. 3. Headaches due to transitory hyperaemia. Fluctua- tions in the blood-supply of the brain sometimes cause severe headaches in children. I have seen many young persons who after some unusual excitement or intense emo- tion develop severe headaches, associated either with deep flushing, or with marked pallor of the face and with cold extremities. In others, again, persistent headaches come on after an acute exhausting disease, in whom it must be sup- posed that if there is any transitory hyperaemic condition it is rapidly followed by a lack of blood in the vessels of the brain and its coverings. A chronic hyperaemic condi- tion is probable in children afflicted with heart disease, who are often subject to periods of painful headaches, and a similar explanation must be sought for in young girls just beginning to menstruate, who have severe headaches for HEADACHES. lj$ several days preceding and during each menstrual period. While such headaches are often strictly periodic they are very different from ordinary migraine, and can be distin- guished from the latter, too, by the late onset of the trouble and by their disappearance as soon as the menstrual func- tion has been properly established. 4. Headaches due to gastric disturbance are a very common occurrence in children of all ages. They are generally frontal, sometimes frontal and vertical. They come on suddenly and persist, as a rule, until the gastric condition has been relieved. These headaches are easily recognized by the symptoms associated with them, viz., fetid breath, coating of the tongue, distress in the epigas- tric region, flatulency, and constipation or diarrhoea. The diagnosis is easily corroborated by the effects of treatment, a single dose of oil or of calomel being sufficient, as a rule, to dispel them until the next serious error in diet is com- mitted. 5. Genital irritation is referred to by some writers as a cause of severe headaches. Seguin refers to a case of oc- cipital headache, the worst he ever saw, which was cured by circumcision. I have not seen such a case, but I do not wish to doubt their occurrence, and if the physician is convinced of the cause of the disturbance the remedy is close at hand. In older boys, and even in girls, headaches are not infrequently due to self-abuse. The general nervous condition of the patient under these circumstances calls for treatment even more distinctly than do the headaches which are associated with a general restlessness, with pains in the back, with irritability, and in severe cases with a tendency to mental apathy or even dementia. There is every reason to look for this etiological factor in any case of persistent headaches in children between the ages of six and fifteen years. The treatment of the headaches under these circumstances is not an easy matter, and practically they can be cured only by the closest watchfulness on the part of parent or nurse. 6. The headaches associated with ear disease are char- acterized by intense pain, located either in the mastoid or in the temporal region, and are most frequently present in 1/6 THE NERVOUS DISEASES OF CHILDREN. the earlier stages of the disease, before the formation of pus. One of the worst cases of this sort that I have ever seen was in a little girl, two years of age, who was un- manageable and showed by her movements that she was in great distress. Every touch on the head was followed by a shrill cry, and the region of the ear was so sensitive that the slightest touch with the finger seemed to cause in- tense agony. No treatment was of any avail until after a few days the pus was freely discharged through the outer canal, and from that moment every trace of headache seemed to have disappeared. The child's behavior is exactly like that of an adult with acute ear disease, only that in children this special cause of headache is often dis- covered only by the merest accident. The practitioner and the specialist in ear diseases will meet with these cases so often, particularly in connection with the acute infec- tious diseases, that they should be on their guard. The cure depends entirely upon prompt treatment of the ear condition. 7. Headaches due to organic disease of the brain or its coverings should be suspected in every case in which the pain is persistent and strictly localized. The pain may vary a little according to the position of the head, but it is present whether the child be sitting up or lying down, and is always elicited by the gentlest percussion of the skull. The headaches constitute a most valuable symptom in the diagnosis of tumors of the brain or of the meninges, and in cases of meningeal inflammation from any and every cause. Thus after slight traumatic injuries to the head the onset of intense pain, particularly at the seat of external in- jury, must lead one to suspect the development of a mor- bid condition at this point. If due to tumor, the diagnosis can be strengthened by the discovery of other symptoms, which are bound to arise sooner or later. These are ver- tigo, nausea, and disturbances of vision due to optic neuritis. If a traumatic meningitis is the cause of pain in the head, slight rigidity of the neck, and possibly an inequality and immobility of the pupils, will help to prove the diagnosis. Among the organic headaches we might also include those due to specific disease ; but these headaches, if I may HEADACHES. 1 7/ trust my own experience, are relatively rare in cases of hereditary syphilis or in syphilis acquired at a very early date. They are surely not nearly as constant nor so im- portant a symptom as are the specific headaches of later years. The diagnosis should be made with great reserve, and only if other symptoms are present which point to an active syphilitic process. 8. The headaches which mark the prodromal stage of acute infectious diseases often give rise to very serious er- rors in diagnosis, and are occasionally suspected to be symp- toms of cerebral tumor, or possibly of meningitis. In the prodromal stages of typhoid and scarlatina, and of diph- theria, these headaches are very common indeed ; but the possibility of such a cause for headaches should be enter- tained if they are associated with a general malaise and with slight rise in temperature. There is no need of dis- cussing the treatment of this special form. Unless one chooses to apply cold cloths, or an icebag, or possibly to give small doses of phenacetine, there is nothing else to do but to wait for further developments, and to treat the more serious disease of which the headaches constitute the pro- dromal stage. 9. Malarial headaches are not observed nearly so often in this climate as in the more southern States, and in those countries in which severe types of malaria are prev- alent. Malarial headaches are almost invariably neuritic in character. The supraorbital and infraorbital points are painful, and the entire head may be sensitive to touch. There is a distinct periodicity in the development of these headaches, or if the headaches are continuous there are at least periodic exacerbations. In making a diagnosis of malarial headaches I follow the practice of examining the spleen and the blood, and not contenting myself with the diagnosis unless I can prove the existence of enlarged spleen, or the presence of the Plas- modium in the blood. If such evidence is obtained, a few large doses of quinine — from five to ten grains several times a day, according to the age of the child — will prove the best cure. 10. Uraemic headaches are not as frequent in children as 12 178 THE NERVOUS DISEASES OF CHILDREN. in the adult. These headaches are generally occipital, and are associated with slight disturbances of vision, with ver- tigo, nausea, and sometimes with epigastric pain. The con- dition of the kidneys should be carefully determined, if severe headaches arise during or after any of the acute in- fectious diseases ; above all, in scarlatina and diphtheria, which are known to be followed by renal complications. 11. Other toxic headaches deserve special mention. They are not frequent, but do occur often enough to make it necessary to examine for this possible cause. Among toxic substances lead is most easily productive of severe headaches. The poison is apt to be taken into the system not only with milk that is kept in leaden jars, but with drinking-water passing through leaden pipes, and I have known it to result from chewing-gum, and all sort of vile sweetmeats that are wrapped up in attractive papers, the children licking these papers in order that they may get the full value of their purchase. Not long ago I saw at my clinic a young girl, of about fifteen years, suffering from headaches, and from a typical lead palsy, who had evidently taken the poison into her system in the course of her daily work, which consisted of gluing together the paper in which chewing-gum was wrapped. She was in the habit of putting her tongue to the glue and the paper instead of moistening the former with a sponge. I have reserved for the last, the consideration of head- aches due to eye-strain. Not that I think them the least frequent, but because in my opinion undue importance has been attached to them. The cases that are due to eye-strain are those in which the headaches come on after reading, or in studying. They may persist for some time after the effort is made, but frequently disappear after the effort is relaxed. Serious errors of refraction may be the cause of headaches, and of continuous headaches, even though no effort be made to use the eyes ; but I have seen headaches persist so frequently after the fitting of glasses by the most competent oculists that I am firmly convinced that eye- strain is the sole cause of headaches in relatively few in- stances. These headaches are located in the frontal region, be- MIGRAINE. 179 tween and over the eyebrows (Fig. 49) ; in some instances with evident eye-strain the headache is referred to the occi- put. I am thoroughly in favor of giving every child the benefit of the doubt, and of making a careful examination into the condition of the eyes ; but I wish to protest against the excessive enthusiasm of the day which implies that if the slightest error of refraction is discovered in a child the error must be the cause of all ills. What is claimed for headaches is claimed under similar conditions for epilepsy and for chorea. The above considerations will convince the student that the diagnosis of headaches is no easy matter. To make an accurate, or even plausible, diagnosis of this condition is one of the most difficult tasks in neurology. It can be done properly only by a careful consideration of the general con- dition of the patient, of the symptoms associated with the headaches, and of the patient's health before and after the headache has been developed. MIGRAINE. Among the neuroses of early youth few are more troublesome or more interesting than migraine, or "sick headache." While the disease does not, as a rule, attain its full development until the age of puberty or later, it begins so often in the earlier years of life that it belongs very properly to the special subjects of this book. The symp- toms of the disease as it occurs in children are so very much like the adult form that in describing one we picture the other. Hemicrania (megrim), or sick headache, is characterized by occasional attacks of intense headaches, frequently unilat- eral, which are associated with a feeling of nausea, or with vomiting. The unilateral headache is by far the more im- portant symptom of the two, for in many cases the charac- teristic headache is present for months and years without nausea ever being associated with it, though I must record cases from my own experience in which periodic attacks oi nausea have occurred in children without any headaches. Such attacks I have interpreted to be the equivalent of or- l80 THE NERVOUS DISEASES OE CHILDREN. dinary migraine. The correctness of this view has been proved by the occasional occurrence of attacks in which both nausea and headaches were present, and by the fact that the ordinary treatment for migraine and no other helped to dispel these peculiar gastric attacks. Barring such occasional cases, it is better for us to consider the typical attack in which neither headache nor nausea is wanting. In this disease we find a number of very important symp- toms associated with headache. Peculiar visual disturbances constitute a prominent feature of the disease ; these have been variously described by many sufferers, and amount, as a rule, to a temporary and partial loss of sight during the attack ; or there may be every possible form of visual dis- turbance, from simple balls of fire to distinct figures, which appear as regularly in the attacks of migraine as similar phenomena do in attacks of epilepsy. In many cases a sim- ple dark spot is observed ; in others, flashes of lightning that surprise the patient are the first symptoms of a full- fledged attack of migraine. A young patient of mine would regularly see a bright zigzag line, which she compared to a distant staircase ; as soon as she seemed actually to ap- proach the stairs intense headaches would set in, and she would feel dizzy, but there was never loss of consciousness, and nothing resembling an epileptic attack. The patient would then pass through a typical attack of migraine, which would last for several hours, and during this entire time would exhibit very marked photophobia. In some cases other special senses are affected. Tinnitus is much more frequent, on the whole, than any other form of sensory disturbance excepting those of vision noted above. Disturbances of sensation in the limbs are a frequent ac- companiment of migraine. These sensations generally take the form of tingling, of pins and needles, or of burning sen- sations. In some instances there is a general numbness, very much like the numbness that precedes an attack of epilepsy ; the sensations, however, last very much longer than the sensory aura would, and can, of course, be distin- guished from the latter by the entire absence of any typical clonic movements. Motor symptoms are not present as a MIGRAINE. 1 51 rule. As there is a close association, at times, between mi- graine and epilepsy it is possible that we may see cases every now and then in which the auras resemble very much those that precede an attack of migraine, but the clonic movements are characteristic of epilepsy. Temporary aphasia I have met with as an accompani- ment of an attack of migraine. I recall the case of a young girl, ten years old, who had inherited migraine from her mother, and who greatly alarmed her parents by the sudden development of aphasia in connection with an attack of mi- graine. The girl when spoken to was able to mumble a few words indistinctly, but could not find the word she wished to say. She was in intense pain and extremely irritable, but, after a good night's rest, the headache had disappeared and with it the aphasia. This aphasia is associated with right-sided hemiplegia in right-handed persons. The occur- rences during these attacks of migraine adhere closely to the physiological laws of the cortex, and we may anticipate what we have to say upon the pathology of the subject to the extent of implying that the sequence of symptoms evi- dently proves that the entire motor district of the brain must be affected by the temporary defect in its blood- supply. Vertigo is another symptom that is associated with the headaches, at times preceding it, at other times following it. The vertigo is not, as a rule, as marked as it is in Me- niere's disease or in some organic diseases of the brain, but it is quite sufficient to make the patient unsteady on his- feet, and to give rise to a great deal of discomfort. The aspect of the patient varies considerably during the attack. In some there is distinct pallor of the face and a feeling of coldness in the extremities. The eyeball may seem a trifle retracted, the vessels of the conjunctiva may be engorged, and the pupils may be contracted. In other cases the face may be extremely flushed, the pupils dilated rather than contracted, and the ear distinctly reddened. These two distinct conditions are often present in one and the same attack, and, in rare instances, the one-half of the face may present pallor with its associated symptoms. whereas the other half presents a flushed condition with 1 82 THE NERVOUS DISEASES OF CHILDREN. the symptoms that go with it. The latter symptoms are evidently of the paretic order and the former of the spas- tic, and both are evidences of a change in the sympathetic nerves. Sweating of one side of the face has also been ob- served, as well as retardation of the pulse during the parox- ysms. On examination during an attack, I have found the various points of the trigeminal nerve quite as sensitive as in the milder cases of trigeminal neuralgia. In some pa- tients — and this is particularly true of children — the entire face and head is so sensitive that the patient can scarcely bear to rest the head on a pillow, and I have known one patient who insisted on " walking off " the headache rather than to rest the head against anything during the parox- ysm. Gowers refers to a transitory pyrexia during an attack of migraine in children. I have often taken the tempera- ture during attacks, impelled to do so by a flushed condition of the face, but have never found any elevation above ioo° F. If a higher temperature is present I should be disposed to look for some other condition, say some gastric disturb- ance, as a possible cause both of the migraine and of the fever. Etiology. — The hereditary predisposition to migraine is too manifest to be denied for a single moment. Not only do children of mothers who are thus affected inherit the disease, but it also occurs in the progeny of persons afflicted with other grave forms of functional nervous disturbance. Among these epilepsy, hysteria, and hystero-epilepsy are the most potent sources. The disease usually sets in in the earlier years of life. Some begin between the fifth and tenth years, a fair number of the cases between ten and twenty years, but the majority set in between twenty and thirty years. The female sex is evidently more disposed to the disease than males. The first manifestations of migraine are generally excited by emotional disturbances, by overwork, or worriment. In not a few instances I have known the rivalry among class- mates to have been the final cause of the development of migraine ; in others, some severe gastric disturbance has MIGRAINE. 183 been the starting-point of the entire trouble, but it should be remembered that, if a predisposition did not exist, the derangement of the stomach might have produced a tem- porary headache, but would not have started a series of attacks of migraine. After the first attack an interval of some weeks, or months, may intervene before a second seizure takes place ; but some form of periodicity is soon established in the majority of the cases, and in many, even in young children, the attacks are apt to return at stated intervals — every two, every four weeks, or every second or third month. It is with migraine very much as it is with epilepsy, that the slightest disturbance of the physical or- ganism is sufficient to develop an attack. It is, therefore, of the greatest importance in migraine, as in epilepsy, that the most careful attention should be paid to the general hygi- enic and dietetic management of the child. Pathology. — We cannot expect to demonstrate actual changes in the brain, or in any other part of the central ner- vous system in a person afflicted with migraine, for persons so afflicted rarely die after a paroxysm, and the attack of mi- graine evidently represents a transitory change ; but since the anatomical basis is wanting, the opportunity for theo- retical speculations regarding the pathology of the disease is all the greater, and has been improved by numerous writers. There can hardly be a doubt that changes in the blood-supply of the brain or its coverings are primarily responsible for the symptoms of migraine. There is also reason to believe that the sympathetic nervous system is largely involved in this disease. According to the varying conditions present many neurologists are inclined to sup- pose that in some instances we have an angio-spastic condi- tion, in others an angio-paralytic state, and if we wish to imply our belief in the role played by the sympathetic nerves we can speak of " sympathetico-tonic " or " svmpa- thetico-paralytic " forms. The behavior of the superficial blood-vessels in the two forms of migraine lends reasonable coloring to this view of the vasomotor origin of migraine. Some, not satisfied with this vascular theory, argue that there must be some inherent alteration in the nerve-cells of the brain, but no sufficient reason is given why. if such de- 1 84 THE NERVOUS DISEASES OF CHILDREN. rangement exists, there should be such violent periodic ex- acerbations of all the symptoms. To say that the symp- toms are due to a " nerve storm " is merely substituting a vague term for a vague conception ; yet we must acknowl- edge that a peculiarity in the structure or in the function of the cortical cells may be present in these cases, and that changes in the vasomotor apparatus are sufficient to pro- duce the phenomena of migraine in a person whose nervous system is thus altered, whereas the same vasomotor changes would be entirely insufficient to produce any such symptoms in persons whose brain -cells are altogether normal. It seems to me, therefore, that we must rely upon these two causes for an explanation of the disease ; surely one cause alone would not be sufficient to explain all the phenomena. We are constantly losing sight of the co-operation of forces and of causes that produce disease, and are hampered so frequently by the supposition that we must make out a single cause or none. The disturbances of vision, as well as the temporary aphasia occurring in some cases, prove conclusively that the cortical centres are involved in the disease. The visions of migraine can be regarded as a symptom of irritation the hemianopsia as a symptom pointing to temporary paral- ysis of the functions of the visual centres. Whether a de- rangement of the cells or some other change in the consti- tuent parts in these centres is responsible for the loss of function cannot yet be proved or denied. The relation of migraine to epilepsy makes it also more probable that some primary peculiarity of the cortical cells is responsible for this painful affection. The resemblance is so close, and the sequence of the two diseases so strik- ing, that the two forms of disease may possibly represent a different degree of affection of the cortical structure. As for the headaches of migraine, they can be best ex- plained, it seems to me, on the supposition of a marked distention of the blood-vessels of the coverings of the brain ; and the fact that various points in the face are as painful as they are in typical trigeminal neuralgia, would lead to the inference that the trigeminal nerve, as it passes from the brain outward, is affected by this general hyperasmic con- MIGRAINE. 185 dition. The nausea and vomiting are an expression of gen- eral cerebral disturbances, such as is seen in cases of gross disease anywhere in the brain, particularly in the lower centres in the pons and medulla, and such as we sometimes find in cases of simple cerebral shock. I cannot see suffi- cient reason to connect these symptoms directly with an affection of the sympathetic nerve. Within the last few years much has been made of ocular insufficiencies as a possible cause of migraine. This matter has been much overdone, thanks to the labors of Dr. Stevens and others. As a neurologist I could pass over the rather elaborate discussion that has been held on this subject, were it not for the fact that so high an authority as Dr. Seguin has given his approval to this special doctrine in his lectures on the treatment of neuroses {New York Med- ical Journal, 1890). Dr. Seguin states that he has not met with a case of migraine in a person with normal eyes, al- though he has been told of two or three by oculists of good repute. He implies, furthermore, that if the matter has been overlooked in the majority of cases of migraine, it is because the ocular examination has not been a thorough one. He argues, furthermore, that the ocular origin of migraine is made probable by the remarkable fact that in many persons of both sexes the attacks diminish and then cease between the ages of forty and fifty years, at the time the power of accommodation becomes exhausted, and a large part of the unconscious strain which has been going on from early youth is removed. Seguin implies still further that migraine is hereditary, chiefly because ocular defects are hereditary. The fallacy of these arguments seems to me to be evident enough from the experience many of us have had, that the eyes are normal according to the exami- nation of competent oculists in many children who have migraine, and that the attacks continue long after the ocu- lar difficulty has been corrected by glasses. We are willing to concede that these ocular difficulties have an important bearing upon headaches in general, and may influence the occurrence of attacks in those who have inherited migraine ; but far stronger evidence will have to be advanced before it will be safe to adopt Seguih's conclusions, and sonic other 1 86 THE NERVOUS DISEASES OF CHILDREN. sufficient reason will have to be given for the action of mydriatics, such as belladonna, atropine, hyoscyamia, and cannabis indica, than the effect these have upon the accom- modative effort. In my own experience with the drugs mentioned, cannabis indica is the only one which has any decided effect upon the course of migraine or upon single attacks. Diagnosis. — The diagnosis of migraine is easily made. The early onset of the disease, the periodic attacks, and the character of the headaches will, as a rule, leave little room for doubt. It is only in those cases in which a distinct sen- sory aura exists that a confusion with epilepsy might arise, but the preservation of consciousness and the entire absence of clonic movements, and the duration of the single attack, will help to distinguish migraine from epilepsy. It may be a little more difficult at times to distinguish between migraine and headaches due to some other cause, but this difficulty will cease as soon as the periodicity in the occur- rence of the headaches has been noted, and every other form of headache has been ruled out by the method of ex- clusion. I have found some little difficulty also, particu- larly in young children, to distinguish between migraine and ordinary trigeminal neuralgia, for owing to the sensi- tiveness of younger patients it is not so easy to determine whether there are distinct painful points, or whether the whole face is painful, merely as a result of the fear of ex- amination ; but the long and free interval between attacks, together with the periodic occurrence of the headaches in migraine, will serve to distinguish this form from typical trigeminal neuralgia.* Prognosis. — The prognosis of migraine is entirely fa- vorable as regards life, but not so promising with reference to the cure of the disease. It is a matter of common ex- perience that a person who has inherited migraine will be subject to the disease for a long number of years, until it begins to disappear with age. It is a satisfaction, however, to be able to assure the patient that under proper treatment the attacks may be either inhibited or their severity may be diminished. The only grave feature about the disease is * Trigeminal neuralgia is a rare affection in children. MIGRAINE. 187 the possible development of migraine into epilepsy, but even this is an infrequent occurrence. Treatment. — In the treatment of migraine two distinct objects must be kept in view. First — the cure, if possible, of the disease ; secondly — the amelioration of the attacks. The disease often defies the skill of the most experienced practitioner. As in epilepsy, no effort should be spared to accustom the patient to regular hygienic and dietetic habits. It is important that the child thus afflicted should sleep in a well-ventilated room ; that it should have regular and moderate physical exercise, a point of the greatest im- portance in those who have inherited gouty or lithaemic tendencies. The child should be relieved also, as far as possible, of all mental and physical strain. While the dis- ease is at its worst the patient should be kept from school, and all close application to study should be avoided. If it can be shown that the effort of reading or of studying helps to bring on an attack, even study at home and reading may have to be prohibited. As a matter of fact, however, a little mental occupation is often to be preferred to idleness, which permits the child to wait for the onset of an attack and often induces distinct hypochondriacal tendencies. My own experience has been very largely in favor of mod- erate employment of mind, which helps to prove to the child that its lot is not a worse one than that of other chil- dren, and helps also to avoid the idea of invalidism which is so frequent in children, even in those who do not for one reason or another care to simulate disease. If the child is anaemic, or if it exhibits scrofulous ten- dencies, iron in any of its various forms, arsenic and quinine in tonic doses, as well as cod-liver oil, are indicated. Phos- phorus is of so little use that we can disregard it alto- gether. A single attack can be relieved best by putting the child in bed during the period of the attack, and in a dark- ened room. On the whole mere rest in bed is as important a factor as any in the treatment of the attack. During this time the child should be placed on a mild diet ; an excess oi liquids as well as of nitrogeneous food should be avoided. Small amounts of meat, fish, and eggs, and a moderate 1 88 THE NERVOUS DISEASES OF CHILDREN. amount of milk, will answer the purpose better than if the patient is placed altogether upon nitrogeneous or upon an excess of farinaceous food. It is important to regulate the bowels thoroughly, and if the kidneys are not very active to increase the quantity of urine passed. To this end some mild alkaline water, such as Vichy or Seltzer, can be safely recommended, either alone or in conjunction with milk. Innumerable drugs have from time to time been warmly recommended. Some years ago antipyrine in doses of five to ten grains, according to the age of the patient, was quite in vogue ; but I da not consider it promising enough to urge its use in the face of the well-known risks attending its exhibition. I am certain, too, that phenacetine in five to ten-grain doses has little or no effect. I have seen some good results from the combined action of the phenacetine and caffeine in one- to three-grain doses, but I am certain that the combination is not in any way superior to the use of caffeine alone. Caffeine is beyond a doubt one of the most serviceable drugs to be employed. I am in the habit of giving it to children under fifteen years of age in one-grain pills or powders, which are to be repeated every twenty min- utes until distinct relief is felt ; and after the first relief has been procured the drug should be continued in the same dose at longer intervals, first every two, then every three hours, and later on, for a period of about a week after the onset of the attack, it can be given safely in small doses several times a day, according to the condition of the child. Caffeine can be combined with iron, with quinine, or with arsenious acid. I have also had excellent results from a combination of caffeine and cannabis indica. The various preparations of the latter are so unreliable that it is as well to use Herring's extract (gr. T V - -J-). A caution is neces- sary, however, as regards the use of cannabis indica, for some patients are peculiarly intolerant toward this drug, and I have seen distinct symptoms of poisoning from small doses repeatedly employed. While the drug is not in reality a dangerous one, the symptoms due to the admin- istration of large doses are so disagreeable and are so apt to alarm the patient and the family that great care should MIGRAINE. 189 be exercised in the use of the drug ; but if the fact of tolerance on the part of the patient has once been estab- lished no trouble is apt to ensue during subsequent trials. Seguin thinks that the drug exerts a sedative, even paralyz- ing, influence upon the third cranial nerve and its attached muscles, including the ciliary, but the drug has as good an effect in cases in which the ocular apparatus has been de- termined to be entirely normal. The nitrite of amyl has been suggested on physiological grounds as a proper remedy, particularly in those cases in which there is marked pallor of the face. We might also expect it to act favorably, as it does in epilepsy, in those cases of migraine in which there is a distinct sensory aura. I quite agree with Dr. Seguin in the statement that the drug is of little use in actual practice, and that it rarely affords the expected relief. The same can be said of acon- itia and of hypodermic injections of hyoscyamine. The bro- mides and chloral help at times to allay the nervous excite- ment and to induce sleep, as do also sulfonal and trionaland chloralamid in five- to fifteen-grain doses ; but I cannot re- gard them as in any sense specific remedies in migraine. Nitro-glycerine (one drop of the one per cent, alcoholic solution) has occasionally helped to diminish the severity of an attack. In this form it is not a dangerous drug, and can be safely administered under the supervision of a physician, of a nurse, or of an intelligent mother. The use of tea and coffee in moderate quantities is strongly to be recom- mended ; I have known children suffering from migraine keep simple black coffee in the stomach with beneficial effect who would vomit almost everything else that was given. I feel that I owe much to Dr. Seguin's suggestion that black coffee, without sugar and without milk, is easily digested, and that the general prejudice regarding this beverage is due to the milk and sugar that have been in- variably added, and that have given rise to fermentative processes in the gastro-intestinal tract. As for the correction of the ocular defects, I do not wish to oppose sensible treatment by competent oculists, but I do oppose treatment based upon extreme theories. I would have the eye condition corrected on the principle 190 THE NERVOUS DISEASES OF CHILDREN. that in migraine, as in epilepsy, every physical defect should be remedied ; but I must protest against the as- sumption that such slight errors in refraction and in ac- commodation may be the cause of this neurosis. The more liberal our therapeutic view, the more successful the treatment Avill be. BIBLIOGRAPHY. (A bibliography of headaches it is quite impossible to give. Many text- books on diseases of the nervous system treat the subject very fully ; in addition to these, the reader is referred to Seguin's article in Keating's Cyclo- paedia, and to one by Sinkler in Pepper's System.) MIGRAINE. Charcot: Lecons du Mardi, i., 1887. Collins, J. : New York Medical Record, April 14, 1892. Eulenburg : Article " Migraine," Ziemssen's Cyclopaedia ; Wiener Med. Presse, 1887. Fere: Ophthalmic Migraine, Rev. de Med., 1883. Haig: British Medical Journal, 1888. Hammond, G. M. : Journal of Nervous and Mental Diseases, 1892. Liveing : On Megrim, Sick Headaches, and some allied disorders, etc. Lon- don, 1873. Mitchell, S. W. : American Journal of the Medical Sciences, vol. 94, 1887. Moebius: Die Migrane, Vienna, 1894. (Contains recent literature; an ex- cellent monograph appeared after this chapter was written.) Oppenheimer, H. S. : Boston Medical and Surgical Journal, vol. 119, 1888. Ranney: "Eye-strain," etc., Medical Record, 1889. Sinkler : Medical News, July, 1890. Thomas, L. : La Migraine, Paris, 1887. CHAPTER X. THE DISORDERS OF SLEEP. The healthy new-born child sleeps during the entire day and night, except when it is being nursed or dressed. Dur- ing the first month the infant is awake only three or four hours out of the twenty-four, falling soundly asleep imme- diately after nursing. By the end of the sixth month fifteen hours' sleep per day is a fair average, which is gradually de- creased until at the age of one year the healthy child sleeps about twelve out of the twenty-four hours. From one year to four years an average of ten hours' sleep should be main- tained, and up to the age of ten or twelve years the health of the child demands that it shall sleep quietly for at least nine hours in the day. If there is a marked departure from these averages, the child is either in pain or some of its functions are not entirely normal. In the acute fevers or in any febrile condition children are alternately drowsy and wakeful. Disturbances of digestion are also a frequent cause of restless sleep or of prolonged wakefulness. In addition to these special conditions, others cause in- somnia in children as in adults. Poorly ventilated and over- heated rooms often cause restlessness, and for this reason it is important that a window should be kept open in every bedroom during the entire night, and that the child should be covered as lightly as possible, according to varying at- mospheric conditions. If a child is sleepless in the absence of unfavorable conditions, we must seek some other cause for the insomnia. In many cases the mother or nurse has not been careful to engender the proper habits of sleep. Infants that are much pampered, that are taken up as soon as they utter the first cry, or that are wilfully disturbed by parents or nurse, soon become poor sleepers. From the first week of life the child should be laid down quietly and 192 THE NERVOUS DISEASES OF CHILDREN. not rocked to sleep, and should not be disturbed except for good reasons. If the room is darkened a quiet sleep will come on very naturally. As the child grows older it is well not to excite it just before the hour at which it is ex- pected to fall asleep. With children up to the age of eight years the evening meal should be a light one, and their brains should not be disturbed by exciting stories. This should be observed more particularly in the case of chil- dren who exhibit nervous tendencies, or of those who have passed through exhausting diseases. The normal child does not fall asleep unless its hunger has been appeased. Insufficient nourishment is, therefore, to be suspected in cases of persistent wakefulness in young infants. With the change of nurse, or after weaning, a restless sleeper will often develop normal habits of sleep. If insomnia continues without apparent cause, we must sus- pect some graver trouble. In the earlier stages of tubercu- lar meningitis, in tumor, and in other painful diseases of the brain, sleeplessness is often one of the earliest symptoms. If the cause of insomnia cannot be easily made out, it is well to fall back upon faulty methods of training. Not very long since a child of five years was brought to me by its mother, who stated that it would not fall asleep if it awoke after midnight. The mother claimed to be ignorant of any mistake that she had made in the training of the child, but on closer questioning I heard that she had been in the habit, whenever her husband was away, of taking the child out of its bed and putting it in her own bed. After she had done this a number of times the child woke up regularly at midnight and would cry until the mother took it up. The mother, a very nervous woman, excited the child by her caresses and her despair over its not sleeping, and thus made matters worse and worse. The child had been sleeping very poorly for several weeks before I was consulted. The cure was a very simple one. The child was placed under the care of a sensible trained nurse, who would not yield to its solicitations, and after a few nights of restless sleep it began to develop proper habits and soon slept an average of ten hours. The prognosis is invariably favorable unless the condi- THE DISORDERS OF SLEEP. 1 93 tion is due to some grave disorder. It is most important to insist upon absolute regularity in putting the child to bed at a definite hour and keeping it there unless it is neces- sary to take it up. If this should prove insufficient a warm bath at bedtime will be conducive to sleep, and small doses of bromide or chloral will be useful to engender the habit. The newer hypnotics, such as sulphonal or trional, in five- to ten-grain doses, may be substituted for the bromide or chloral in children who are anaemic or poorly nourished. All hypnotics should be withdrawn as soon as possible, for there is great danger of accustoming the child to these drugs. In some cases the mere regulation of the meals, and the avoidance of all indigestible articles of diet, will be suffi- cient to restore sleep. In children who are impressionable it is of the greatest importance to avoid telling them stories or even engaging them in play in the latter part of the day. Pavor Nocturnus, or night fear, is a condition which is often troublesome both to the parents and the physician. Children affected with this disturbance wake up a few hours after they have entered into a sound sleep, are pos- sessed as a rule by great fear, fail to make out their sur- roundings, and act as though in a temporary dazed con- dition. They do not understand the soothing words of parent or physician, and often continue in a state of excite- ment until they return to full consciousness or are over- come by sleep. There seems to be no good reason to give any other in- terpretation of this condition than that the child has had a horrible dream, and that the substance of the dream is con- tinued in the half-waking state, or that the child is possessed by fear, which is the natural result of the dream and can- not shake off the fear until it fully realizes, if old enough, that its experiences have been unreal. This condition may occur in any child, but it is decidedly more frequent in children who have shown previous nervous symptoms, or in children of highly neurotic parents. It is not infrequent in those who are pressed by school duties. and who go to bed with a fear that their lessons have not been properly done for the next daw This knowledge is sufficient to prevent sound sleep, and during the hours oi 13 194 THE NERVOUS DISEASES OF CHILDREN. restlessness vivid dreams are very apt to occur. It was formerly supposed that late and indigestible meals were the chief cause of these noctural disturbances. Inasmuch as any indigestion may give rise to restless sleep, this factor may have to be taken into account ; but over-work, over- excitement, the reading of horrible or fascinating tales just before bedtime, constitute a much more important etiologi- cal factor. These noctural attacks often occur a single time, but with most children are frequently repeated during the ear- lier years of life, say between the ages of three and eight years. With the growth of the child and the improvement of its nervous system the attacks cease. The prognosis of this condition is entirely favorable ; though I can recall one case of a girl, aged six years, the child of an hysterical mother, in whom the fright connected with one of these nocturnal attacks was the cause, or at least the first beginning, of a severe chorea, which lasted for many months. In another child, about nine years of age, who had had attacks of epilepsy ever since early childhood, and who was particularly subject to nocturnal attacks of epilepsy ; the epileptic attacks ceased upon bromide treatment, but every now and then the child would have a very marked spell of pavor nocturnus, which would disturb her almost as much as an epileptic seizure would. Conditions similar to those of pavor nocturnus occur at times during the day. A little patient of mine, aged five years, would, in a very unaccountable manner, while walk- ing with her mother on the street, suddenly hide her face in the folds of the mother's dress, and bury her head for some time, in order (as she explained later on), not to see the hor- rible black things coming toward her. This child showed no other symptom of a nervous disposition, but was for a time in great dread of the recurrence of these experiences. That they were the result of visual hallucinations during the waking hours, I can hardly doubt. They disappeared entirely under sedative treatment, which was given carefully for some months. In regard to treatment, the most important point is to ex- ercise every possible precaution in order that unnecessary THE DISORDERS OF SLEEP. 1 95 nervous excitement, late in the day, may be avoided. I prohibit all school-work after five in the evening ; do not allow the child to read or be read to after this hour, and in- sist on a very light meal in the evening. After this meal the child is to remain awake for at least an hour, and may indulge in some simple game, or is allowed to frolic about in a quiet manner. Small doses of the bromides, from five to ten grains, according to the age of the child, about an hour before bedtime will be the best remedy. If neces- sary, small doses of chloral may be given with the bromides. If the attacks return very frequently I have found it more satisfactory to give five- or ten-grain doses of chloralamid, or of trional, every night, or every second or third night, until all excitement has subsided. Enuresis Nocturna is another very common form of disturbed sleep. Children who have been well trained, and who have learned to observe all the habits of cleanli- ness during the day, are frequently disturbed by involuntary micturition during the night. This is most apt to occur during the earlier hours of sleep, when sleep is most pro- found. The soundness of sleep alone may be a sufficient cause for this phenomenon, inasmuch as children do not perceive in sleep those symptoms of vesical irritation which prompt them to evacuate the bladder during waking hours, and during light sleep. In others, and possibly in the ma- jority of cases, it is due to indifference on the part of the child, or to an aversion to allow its sleep to be interrupted. In some few cases enuresis is the result of frightful dreams, the sudden impulse to evacuate the bladder accompanying such a dream as it would any emotional condition during wakefulness. If due to this latter cause the enuresis is not frequently repeated. Enuresis has been observed to be a symptom of nocturnal epilepsy. If so, it occurs at rare in- tervals ; the child is entirely unconscious of it, and often shows some other symptom, such as drowsiness and head- ache on the following morning. The condition is a very stubborn one, and all possible methods of treatment have been suggested. I am con- vinced that the cure of the condition is dependent chiefly upon careful training. The necessity of evacuating- the 10 THE NERVOUS DISEASES OE CHILDREN. bladder should be impressed upon children after the age of two years. The child should be made to void urine im- mediately before going to bed, and should be aroused one and a half to two hours after it has fallen asleep, and should be induced to pass water again. By giving relatively few liquids after four or five o'clock in the afternoon, I have found that the tendency to enuresis is often checked. If these simple measures are not sufficient, a few drops of the tincture of belladonna, given at bedtime, will be use- ful, and if the condition is due to frightful dreams, or to cerebral excitement continued during sleep, small doses of bromides may be given. Many medical men have seriously suggested that corporal punishment applied to the nates is the only efficient remedy, and some have gone so far as to suggest that the cutaneous hyperaemia caused by such pun- ishment explains the relief afforded. This explanation is not satisfactory ; but whatever the mode of action may be, it is very certain that such punishment need not be inflicted in the 'majority of cases, and that the desired end can be attained by other methods of training. Somnambulism is by no means rare in children, and occurs most fre- quently in those who are of a nervous temperament, or who have passed through some severe excitement. I have not known them to perform any of the marvellous tricks which are generally accredited to somnambulists, such as climbing out of windows and on the roof, and the like ; but they are apt enough to walk through the house, from one room to another, and to go through a number of purposive actions without being at all conscious of what they are doing. Nightmare is evidently closely related to this condition of somnambulism, and is generally associated with horrible dreams, from which the child awakens badly frightened, but is quieted much more rap- idly than in the condition which is described as pavor nocturnus. Night- mare and somnambulism occur, as a rule, at rare intervals and can generally be traced to some emotional excitement, or to some acute indigestion. The treatment of these conditions is exactly the same as that of night-terror. BIBLIOGRAPHY. Lyman : Insomnia and other Disorders of Sleep. Chicago, 1885. Putnam, C. P. : Article in Keating's Cyclopaedia (literature to date, 1890). Ringer: Medical Times and Gazette, 1867. Silberman : Jahrbuch fr. Kinderheilkunde, 1883. Soltman : in Gerhardt's Handbook, p. 325. Also the text-books of Henoch, Baginsky, and West. CHAPTER XL VASOMOTOR AND TROPHO-NEUROSES. In this chapter a brief description will be given of a number of rare and peculiar diseases, which must for the present be classified under the rather vague title of vaso- motor and tropho-neuroses. Grouping them together un- der one head is simply a matter of convenience ; they are as dissimilar as diseases can well be. Raynaud's disease and facial hemiatrophy have nothing in common ; myxcedema and Graves's disease very little ; moreover, myxcedema must now be removed from the category of " neuroses," if by the latter term we mean a disease of the nervous system with unknown organic changes. The nervous system is in- volved secondarily, and the marked changes underlying the symptoms are well known. In former days several of these vasomotor disorders were considered under the head- ing of diseases of the sympathetic nervous system ; but the sympathetic has nothing to with myxcedema, and very little with Graves's disease, or facial hemiatrophy. But if they differ in the marked changes underlying them, they have at least this in common, that, in all of them, changes in the skin and the subcutaneous tissues constitute the most striking symptom. EXOPHTHALMIC GOITRE. This disease, which is generally termed Graves's disease by the English writers, and Basedow's disease by the Ger- mans, occurs chiefly in adult life, yet a sufficient number of cases begin during childhood to make it incumbent upon us to give a brief description of the disorder. Moreover, two conditions resembling Basedow's disease, viz.. tachy- 198 THE NERVOUS DISEASES OF CHILDREN. cardia and enlargement of the thyroid in girls at the age of puberty occur often enough to make it necessary to dif- ferentiate between these various conditions. Von Ehrlich and Kronthal have called special attention to the occur- rence of exophthalmic goitre in children. The chief causes of Graves's disease in adult life are un- questionably great emotional excitement, some constitu- tional or exhausting disease, and occasionally some serious cardiac disturbance, but no cause is as frequent as fright or emotional excitement. In children these same factors may be at work, but heredity comes much more distinctly into play, particularly in children of persons suffering from epilepsy, chorea, or of those suffering from chronic al- coholism. The symptoms of this disease are very numerous. We may distinguish between the cardinal and accessory symp- toms. The cardinal symptoms are : 1. Excessive cardiac action. This is by far the most constant symptom of all, and is often present in the earlier stages of the disease when the other two cardinal symptoms — swelling of the thyroid and the protrusion of the eyes — are not yet in evidence. The pulse-rate may vary between 90 and 120, or it may even reach the enormous rate of 200. Under great excitement the pulse-rate is increased. Under mental and physical rest the pulse-rate may not exceed 90 or 100. In spite of the acceleration of the pulse-rate the other signs do not point to any organic disturbance of the heart. The percussion figure is normal, with only this ex- ception, that in the later stages of the disease the left ventri- cle, and rarely the right ventricle, may become hypertro- phied and dilated. The heart-sounds are clear but unusu- ally loud. All the arteries pulsate very distinctly, so that the pulse of the abdominal aorta may possibly be observed through the abdominal walls, and the pulse in the carotids may be so strong as to cause distinct pulse-like vibrations of the entire head. The pulse may even be noticeable in the retinal arteries, and a further disturbance of the entire vascular system may be noted by a dilatation of the capil- laries in the skin, by the presence of distinct tdches cere- brates, and a dilatation of all the larger superficial veins. If VASOMOTOR AND TROPHO-NEUROSES. 1 99 venous stasis is marked, cedema may occur in various parts of the body. It is also owing to the increased tension that hemorrhages are frequent from the nose, and even in the stomach and bowels. Cerebral hemorrhages have also been described in Graves's disease. A particularly characteristic symptom, which is found in exophthalmic goitre, and not in any form of enlargement of the thyroid, is the whirring felt if the finger is placed upon the goitre. 2. The second cardinal symptom is the enlargement of the thyroid gland. It is present in the vast majority of the cases, though a number of authors have taken pains to describe cases of this vasomotor disease in which the thyroid was not enlarged ; but the enlargement, as a rule, comes on some time after the tachycardia. The gland, after it has once become enlarged, increases rapidly, and may give rise to a great deal of inconvenience. The enlarged gland, or struma, is extremely vascular, and the tissue of the gland is not only hyperaemic but very soon enters into a state of hyperplasia with a tendency to a fibrinous degeneration. In keeping with these changes the goitre may in the begin- ning be soft and yielding, later on it will become hard to the touch. The enlargement of the thyroid is, as a rule, bilateral, but generally asymmetrical. The right half is more fre- quently enlarged than the left half. 3. The third cardinal symptom is exophthalmus, or pro- trusion of the eyes. This is the least constant of the three symptoms. If present it is generally bilateral, but in some instances one eye only may be affected, or the protrusion of one may be very much greater than that of the other eve. The causes of this protrusion have been in dispute, but it is more than probable that the interference with the venous current, as well as the arterial congestion, are the prime causes of this protrusion. The great variability of this symptom during life, its disappearance almost immediately after death, and the pulsation of the vessels in the fundus, lend support to this view. It can hardly be doubted, how- ever, that in some cases the development of fat and of cellu- lar tissue in the retro-bulbar space may makejthe protrusion still more marked. Vision is not affected in spite of the exophthalmus, and 200 THE NERVOUS DISEASES OF CHILDREN. the only changes observed were those recorded by Kast and Willbrand, who have noted a limitation of the field of vision in this disease without any evidence of hysteria. The pupils may be unequal, but react promptly. Ulcerations of the cornea, occurring in consequence of insufficient protec- tion of the bulb, have been noted ; as well as some interfer- ence with the lachrymal secretion. Among the accessory symptoms those relating to the movements of the eyes are the most important. Graefe's symptom refers to defective movements of the upper eye- lid on vision downward. The upper lid does not follow promptly the downward movement of the bulb, and the con- junctiva remains visible between the pupil and the margin of the lid. This symptom evidently bears some relation to the phenomenon described by Stellwag consisting of a dila- tation of the palpebral fissure. Graefe's symptom, as far as can be ascertained, is caused neither by the protrusion of the eyes nor by a spasm of the levator or a paresis of the orbicularis, but is explained most readily as the result of Stellwag's phenomenon. Moebius has described another symptom implying a deficiency in the power of accommo- dation. There is defective convergence of the axes of the eyes. These three symptoms are not absolutely pathogno- monic of Basedow's disease, as each one has been described in connection with other neuroses; but if they are associated with either one of the cardinal symptoms they help to cor- roborate the diagnosis of Basedow's disease. The next most frequent, and perhaps most important, symptom is a tendency to profuse diarrhoea. This is en- tirely independent of any gastro-intestinal disease, often resists treatment, and adds greatly to the patient's discom- fort and to the general depreciation of his health. Gastric crises have been described in some cases, and frequent vomiting is not an unusual symptom. Most patients exhibit considerable dyspnoea, resembling true asthma, and Louise Bryson has referred to defective expansion of the chest on inspiration. Glycosuria and albuminuria have been re- ported ; the former, according to the investigations of Chvo- stek, is of the alimentary order. In some few instances, however, diabetes mellitus has occurred as a complication VASOMOTOR AND TROPHO-NEUROSES. 201 of Basedow's disease. This association is not a strange one, since both diseases are often developed after severe emo- tional excitement. Of the disturbances in menstruation we need take no account, as we are, after all, concerned with the disease as it occurs in children. Trophic and vasomotor disturbances of the skin are fre- quent. Pigmentation, leukoderma, loss of hair, premature grayness of hair, and even scleroderma, as well as herpes, have been observed. A very remarkable symptom of Graves's disease is a tendency to profuse sweating. This may be universal or partial, sometimes strictly unilateral. This tendency must be held to account for the remarkable diminution in electrical resistance which has been observed in the skin of patients suffering from this disease. This fact was first insisted upon by Vigouroux, and later by Eulen- burg. Kahler and others have shown that this reduction in resistance to the galvanic current is due to the fact that the relative minimum of resistance is reached much more easily in patients suffering from Graves's disease than in healthy individuals. Kahler has also shown that this diminished resistance is not due to an hyperemia, as was supposed by some, but it is due to excessive moisture of the skin. A faint tremor is frequently observed, and by some is con- sidered to be one of the cardinal symptoms, but it is not as constant as the three cardinal symptoms we have mentioned. The tremor is rhythmical and consists of eight or nine vibra- tions per second. Choreiform tremor occasionally occurs, and true chorea and epilepsy are complications met with in a certain number of cases. These complications are in all probability purely accidental, and cannot readily be ex- plained on the supposition that the vasomotor changes in Basedow's disease are the cause of the chorea and epi- lepsy. In addition to the preceding symptoms mental changes deserve some notice, and among these we may note irrita- bility of temper, sudden changes of temperament, and the occasional occurrence of mania or melancholy. Some oi these psychic changes are developed in the earlier stages of the disease, others are later, and possibly accidental compli- cations. If we add anaemia, albuminuria, and a general 202 THE NERVOUS DISEASES OF CHILDREN. cachexia, we have completed the list of the symptoms typi- cal of Graves's disease. The morbid anatomy of Graves's disease has been the subject of much discussion. The cervical sympathetic has been given a very important part. A number of authors have reported actual changes in the cervical ganglia as the result of connective-tissue proliferation, and others have re- ported atrophy and degeneration of the nerve-elements ; but since Hale White has proven that similar changes occur in persons who have not exhibited the symptoms of Graves's disease, and that in persons who have died from this disease such changes have not been found, the force of all these findings has been much diminished. The same may be said of hemorrhages into the fourth ventricle, or atrophy of the corpus restiforme, to which Mendel and Leube have attached some importance. The heart has naturally exhibited hyper- trophy or dilatation, insufficiency of the mitral valve, and symptoms of mild endocarditis, but these changes are clearly secondary and do not at least hold any causal rela- tion to the disease. The theories regarding the pathology of the disease have been still more conflicting. Basedow supposed the disease due to chlorotic changes in the blood ; but this ex- planation is evidently insufficient, as the disease frequently affects persons in robust health who are far from anaemic. The close resemblance between the symptoms following upon section of the cervical sympathetic and those of Graves's disease has led many writers to attribute exophthal- mic goitre to disease of the sympathetic ! Section of the sympathetic produces irritation, and the symptoms follow- ing the experiment upon animals are clearly the result of irritation ; but we cannot suppose any condition of irritation to last for years, and must therefore seek some other expla- nation. Friedreich was inclined to the theory that dilatation of the vessels is the result of paralysis of the sympathetic; that dilatation of the coronary arteries caused tachycardia, and that this increased flow of blood to the cardiac muscle produced an increased activity of the heart, and that all the other symptoms of Graves's disease, the exophthalmus and the thyroid swelling, were the result of the arterial hyper- VASOMOTOR AND TROPHO-NEUROSES. 203 semia. Granting that the cardinal symptoms can be ex- plained in this way, the many accessory symptoms cannot be accounted for on this theory. Other authors have in- sisted on the bulbar origin of Graves's disease, and have sup- posed that a lesion involving the nuclei of the vagus was sufficient to account for all the symptoms, and some post- mortem findings have given support to this view. All these theories have proved more or less unsatisfac- tory, and while some cases may actually be due to one or other of the causes mentioned, a thoroughly satisfactory theory is still to be established. Within recent years a number of authors have insisted on the probability of the toxic origin of the disease, and have supposed that by the increased action of the thyroid gland a toxine is produced which is primarily responsible for all the symptoms, but it is questionable whether the diseased thyroid produces such a toxine, or whether it is not more probable that the toxine is the result of deficient function of the gland. The course of the disease is, as a rule, extremely chronic. The onset may be very sudden, and even violent, but after a while the symptoms subside in severity and remain con- stant for a long period of years; but the prognosis is not necessarily as grave as it is generally supposed to be. I have myself seen a number of cases of very marked im- provement and of total disappearance of all the symptoms. If death ensues it is, as a rule, due to some complicating disease, or to extreme exhaustion from diarrhoea, or from cardiac weakness, or possibly from acute mania. Treatment. — Absolute rest and careful feeding are the most important measures. The recoveries which I have seen have been in patients who have been placed on the rest-cure. The entire freedom from excitement, and regular feeding have tended to lessen all the nervous symptoms and have influenced the diarrhoeas which are often the most ex- asperating and exhausting symptom. The tachycardia is remarkably lessened and the dyspnoea is naturally benefited by rest in bed. The diet should be simple. It is well to avoid stimulants and excitants as well as carbonated waters. The excessive dilatation of the stomach, resulting from the 204 THE NERVOUS DISEASES OF CHILDREN. last-named, acts unfavorably upon the heart. Hydrothera- peutic procedures, consisting of cold baths followed by massage, of douches and drip baths, have been recom- mended by many. Mineral baths may have some effect upon the constitutional symptoms and thus favorably in- fluence the disease. Electricity has been warmly recom- mended by many, with special reference to the part played by the cervical sympathetic and vagus. To give this method a fair trial it is best to place the anode by turns over the goitre, the heart, and over the abdomen. The cur- rent should be mild and continuous. Recently Rockwell has recommended the use of the faradic current. As far as medicinal treatment is Concerned the drugs in- fluencing the heart's action have been most in favor ; above all, digitalis and strophanthus. While these drugs act favorably in some cases they unquestionably exert an un- favorable influence in others. I have found that exces- sive palpitation of the heart could be controlled very much better by cold applications to the cardiac region, and by the use of the galvanic current, with the anode over the heart. I am willing, however, to concede that the effect of the latter may be due to suggestion. If there is great emotional excitement bromides in small doses may be given with advantage. Nitrite of amyl and nitro-glycerine have been recommended, but they are to be used with great cau- tion, especially in children, and the result is a doubtful one at best. Ergot has been administered in some instances, and the subcutaneous injections of ergotin into the goitre have been practised, but the results have not been favorable enough to warrant its continuance. Thyroidectomy, or excision of a part of the enlarged gland, has come into vogue, and has been reported by many German, English, and American writers. Improve- ment following the operation has been observed, and in my opinion is sufficiently marked to warrant further trial of this procedure ; but I am not aware that the operation has, as yet, been attempted in children. Sufficient time has not yet elapsed to decide whether the relief obtained by the operation is merely temporary, or whether a permanent cure has been effected. If the enlarged gland presses upon VASOMOTOR AND TROPHO-NEUROSES. 205 the trachea and menaces life, the operation is surely justi- fied. Thyroid Enlargements at the Age of Puberty. — In connec- tion with the consideration of Graves's disease, attention should be directed to the occurrence of thyroid enlargement in girls between the ages of twelve and fifteen years, a condition for which I have been consulted a number of times. At this period the enlargement is often developed quite suddenly, and is so marked as to arouse suspicion of incipient exophthalmic goitre. As such girls are often anasmic, and have a high pulse-rate the suspicion of serious disease becomes still stronger ; but such enlargement of the thyroid gland does not continue for any great length of time, and none of the other important symptoms of Graves's disease are developed. By attention to the general health of the girl, and by direct galvanization of the thyroid gland, the symptoms soon subside. Tachycardia should also be mentioned, as it may occasionally give rise to the suspicion of Graves's disease. An excessively high pulse-rate is fre- quent in children, but true tachycardia in children has, to my knowledge, rarely been reported. I have seen several interesting cases of this kind, and one of them was of unusual interest. It was the case of a boy, of twelve years of age, who had been under my treatment for infantile cerebral hemi- plegia, and had sufficiently recovered from this condition to be able to go about freely, to attend school, and to play with boys of his age. While playing on a cold winter's day he was struck in the neck by an icy snow- ball. Directly he felt faint, but did not lose consciousness. As soon as he was put to bed the tachycardia became evident, and his family physician was sent for. From the first day until about three weeks after the accident the pulse was at no time less than 200 per minute, and often exceeded this rate, so that the number could not well be counted. None of the drugs ex- hibited — such as bromides, strophanthus, digitalis, aconitia — had the slightest effect upon the heart ; but after a period of about three weeks the symptoms subsided quite suddenly, and the boy has been entirely well for several years. The injury to the cervical region, and possibly to the cervical sym- pathetic, causing tachycardia, is of interest, if we recall the part that the cer- vical sympathetic is supposed by many to play in the causation of Graves's disease. The treatment of such cases should consist of rest, and the appli- cation of an ice-bag to the heart and to the cervical spine ; bromides, heart tonics, and aconitia may be tried according to the indications of the case. myxcedema. Myxcedema is a form of trophic disease to which attention should be directed. The disease is of unusual interest, but we need not go into a full discussion of the subject, as the one form of it which occurs in children — myxedematous idiocy — will be referred to in the Chapter on Idiocy. Myx- cedema was first fully described by Gull, in 1873; four years later similar cases were reported by Ord, and since that time innumerable authors in 206 THE NERVOUS DISEASES OF CHILDREN. England, America, France, and Germany have studied this tropho-neurosis. Special interest has been attached to this disease, since Kocher described a condition following upon removal of the thyroid, resembling myxcedema, to which he gave the name, cachexia strumipriva. The Commission that re- ported to the Clinical Society of London, in 1888, agreed that myxcedema, cachexia strumipriva, sporadic cretinism, represented one and the same mor- bid entity. The condition following upon removal of the thyroid gland, the results of physiological ex- periments (implantation of the glands into the abdomen of animals — Schiff, Horsley, and others), and above all, the brill- iant achievements of thyroid feeding, have proved conclu- sively that deficient action of the thyroid gland is the most important factor in the etiology of myxcedema. Symptoms. — Of the symp- toms of myxcedematous idiocy (the congenital form) we need here merely state that the skin is glossy or hard ; the lips and tongue are thick and large ; the hair is stiff and dry ; the child is dwarfish in stature ; the men- tal condition is that of complete idiocy or of imbecility. There is always absence of the thyroid gland. Myxcedema resembling that of the adult may come on at the age of puberty, or even ear- lier. It is characterized by the following symptoms : A gen- eral increase in the bulk of the body ; the skin is firm and in- elastic, does not pit upon press- ure, and is dry and rough ; the folds of the skin disappear, and there is a general obliteration of all the lines of the skin, particularly in the face, giving, as a rule, an older and more stu- pid expression to the face ; the nostrils and lips are very much enlarged. There is distinct apathy and slowness of speech, as well as of action, in some instances ; the mental changes include delusions which lead to dementia. Albuminuria and glycosuria have been observed ; but these are accidental complications, no doubt. The disease cannot be mistaken for any other, except possibly chronic Fig. 50. — Case of Myxcedema with Idiocy. Pa- tient Twelve Years Old : Dwarfish in Stature. VASOMOTOR AND TROPHO-NEUROSES. 20 J nephritis ; but the lack of pitting, the examination of the urine, and the pe- culiar expression of the face will help to remove all doubts. The disease is slowly progressive, and may last for ten, fifteen, or more years. The prognosis was hopeless until the recent discovery of thyroid feeding, and success of this warrants us in rejecting for the present every other form of treatment. Treatment is to consist of the administration of the pulverized thyroid gland of the sheep, which is to be had in all countries. An English prepara- tion is, up to the present time, by far the most satisfactory. Care should be taken to begin with small doses ; in children one grain twice a day should be the maximum dose at the beginning ; this may be in- creased to three or live grains twice daily until the amount is established which the patient needs to keep in a normal condition. A word of caution is in order not to continue the remedy if muscular pain and cardiac attacks occur. In two cases I have been compelled to discon- tinue the use of the gland on account of extreme and rapid emaciation. Tonic measures should be employed promptly if the general health of the child has been impaired. ANGIO-NEUROTIC CEDEMA. Angio- Neurotic (Edema is a trophic disorder, first described by Quincke, in 1882, although conditions closely resembling, or identical with it, have been known ever since 1827. It has passed under the designations of "acute circumscribed oedema," "periodic swelling," "Australian blight," "non-inflammatory cedema," etc. The disease is characterized by the appearance of circumscribed swell- ings in various parts of the body, more especially in the face, throat, and the extremities. These swellings appear without any direct cause, and are often associated with gastro-intestinal disturbance, which is thought to be due to a condition of the mucous membrane of the stomach and bowels similar to that of the skin or larynx when these parts are diseased. The disease comes on in attacks, which are precipitated most easily by exposure to cold, and by slight traumatic injuries. The attacks are most apt to occur between the hours of 1 and 5 A.M. In 71 cases the parts first affected were : The face in 29 cases ; the extremities in 22 cases ; the larynx in 5 cases ; the genitals — penis, scrotum— in 3 cases ; the trunk in 6 cases ; the stomach in 3 cases; the gums, neck, ear, each in 1 case. The cedema may be fully developed within one-half to two hours. The color of the skin is either whitish, or a dull roseate hue with a whitish shad- ing near the centre of the cedematous patch. There is no pitting on pressure. and none of the signs of an inflammatory swelling. The swelling, if in the face, may be marked enough to cause disfigurement. The sudden jumping of the swelling from one part of the body to another is quite characteristic. Subjectively there is a sensation of scalding or burning, or an itching on the part affected ; and after the cedema subsides a " heavy wooden " feeling is complained of. The disease is not a serious one unless it affects the mucous 208 THE NERVOUS DISEASES OF CHILDREN. membranes of the larynx and pharynx ; if the (Edematous swelling in the larynx increases rapidly, death may take place from asphyxia, as in two cases re- ported by Osier. One case has been reported that proved fatal from oedema of the glottis (Krieger). The gastro-intestinal tract, if affected, may exhibit severe disturbance, such as pain in the epigastrium, vomiting, colic, retraction of the abdomen, and constipation or diarrhoea. (Edema of the lungs has been reported as a symptom of this disease ; but proof of this is wanting. Collins has analyzed 75 cases. According to this author the average age at the onset of the disease is twenty-seven ; childhood is by no means exempt. Dinkelacker has reported the case of a child that had its first attack when three months old ; similar cases have been reported by Widonitz and Goltz. The disease occurs nearly twice as often in males as in females. It may come on after any exhausting condition ; but heredity, as well illustrated in a family described by Osier, is the most important factor. In this family five successive generations have been affected. Severe emotional excitement and hysterical conditions are the forerunners of the disease. The ingestion of certain foods was followed by this special form of oedema in cases reported by Osier, showing an etio- logical resemblance at least between angio-neurotic oedema and urticaria. Matas and others have described cases of this disease coming on after or during malarial fever. The diagnosis is made readily enough ; it may be confounded with the blue oedema of hysteria, as described by Sydenham, or the white cedematous swellings of hysteria described by Charcot ; but the pres- ence or absence of the stigmata of hysteria will help to disprove or corrobo- rate the suspicion of angio-neurotic oedema. There can be little doubt that angio-neurotic oedema is a vasomotor neurosis ; it is analogous to the non-inflammatory swellings which Weir Mitch- ell described after injury to the peripheral nerves, and to similar swellings, which appear after stretching a nerve. I have seen cedematous swellings in the face, after section of the trigeminal nerve, very like the circumscribed oedema under discussion. The serous exudation in all probability results from a retardation of the blood-current, and this in turn must be attributed to a local paresis of the vaso-constrictors, or a reflex stimulation of the vaso- dilators. Treatment of this disease as it occurs in children is more difficult than in the adult, in whom the removal of toxic causes, such as alcohol and tobacco, is all that is needed. In children the hereditary element is most marked, and all we can hope to do is to inhibit the attack and to strengthen the general nervous system. To check the attack we may compress the affected part by an elastic bandage, or apply dry heat. Small doses of morphia or codeia may be given. In the case of swelling in the pharynx or larynx, sur- gical interference may be necessary. To improve the general health of the child it will be best to administer strychnia in small doses (grain one-hun- dredth to grain one-sixtieth), and to give blood-tonics such as iron and ar- senic. VASOMOTOR AND TROPHO-NEUROSES. 209 RAYNAUD S DISEASE. Raynaud's Disease, or symmetrical gangrene, is a very rare tropho- neurosis, which occurs, however, quite as frequently in children as in adults. Indeed, says Morgan, " if there be any period when it is especially prone to occur, it is in childhood." Of 93 cases which he analyzed 24 were in children under ten years of age, and 5 of Raynaud's original cases were children be- tween three and nine years old. Mendel observed a case in a child fifteen months old. The symptoms of the disease are practically the same as those first enu- merated by Raynaud in 1862. A localized ischaemiaor asphyxia in symmetri- cally situated parts is the most constant symptom ; this asphyxia may lead to gangrene, but does not invariably do so ; nor is the disease invariably sym- metrical, and for that reason the designation of symmetrical gangrene is not entirely appropriate. The order of development of the symptoms may vary somewhat. Before the local syncope is fully developed, there is often a general numbness and some pain in the part to be affected. The affected area becomes pale and waxy in appearance ; if pricked, little or no blood flows from it. This condition may be recovered from, and several such attacks may occur, each one ending in recovery ; finally, however, the condition per- sists, and local asphyxia is developed ; in some instances the condition is reached at once and is persistent. The affected areas become deep red, then blue, and finally black in color ; the parts are swollen, and the local temper- ature is lowered. Extravasation of blood into the surrounding tissues may occur. Recovery at this stage is still possible ; but if the morbid process is continued for some time the parts grow thinner, the fingers become atten- uated, and finally gangrene results. The gangrene comes on either some months after the first symptoms of local asphyxia, or it is developed at the earliest period of the disease. The tips of the fingers and toes may be de- stroyed by this process, or an entire hand or foot or some other considerable part may be destroyed by gangrene. The parts most frequently affected, are the fingers, toes, ears, the buttocks, the calves, and the nose. The gan- grenous area is generally separated from healthy tissue by a sharp line of demarcation ; the dead part may either become mummified, or it may be cast off after suppuration. Moist gangrene also occurs at times, with the for- mation of large bullae and pus centres. If a gangrenous area heals, it behaves like a torpid ulcer (Elliot). Intermittent haemoglobinuria and glycosuria have been observed. The pathology of the disease is still obscure. Raynaud and his successors were of the opinion that the local syncope is produced by a contraction or spasm of the blood-vessels (both arteries and veins). The disease of the blood- vessels is by several authors attributed to syphilis or Bright's disease. The symptoms of Raynaud's disease have also been observed in association with tabes, syringomyelia, myelitis, and neuritis. The prognosis is generally good ; if death occurs, it is due to sonic in- tercurrent disease. Recoveries are frequent, even if local gangrene has 2IO THE NERVOUS DISEASES OF CHILDREN. existed ; but if large portions become gangrenous, the general health of the patient may be seriously impaired. In such cases tuberculosis may be de- veloped and hasten death. Treatment should be directed toward the improvement of the patient's general condition, and freedom from all emotional excitement should be in- sisted upon. The affected parts should be wrapped in cotton, and placed in a position most favorable for the circulation. Dry heat or warm baths may be employed to sustain the temperature of diseased areas. Nitro-glycerine and nitrite of amyl have been recommended, but they have not been exhib- ited successfully. Electric (galvanic) baths, and the application of the gal- vanic current through the cervical spine and the affected area have also been favored by some ; but very little good has resulted from such treatment. The greatest benefit will be derived from surgical measures after gangrene has set in. FACIAL HEMIATROPHY. Facial Hemiatrophy is a rare form of disease. Often it begins in childhood, and has important relations to other diseases of the nervous sys- tem. It has been described by some with exophthalmic goitre and with mi- graine, but its relations appear to me to be very much closer to scleroderma, which occasionally precedes it, and I have recently seen a case of universal scleroderma in which there was double hemiatrophy of the face. The disease is characterized by a distinct diminution in the size and bulk of the subcutaneous tissues, and by the alteration in the bony structure as well. In one case, on the left side the distance from the middle of the chin to the angle of the jaw was 1 1 ctm. ; on the right side, 12^ ctm. ; from the upper margin of the naso-labial fold to the middle of the ear was 10^ ctm., while on the right side this distance measured 1 1 ^ ctm. The first indications of atrophy are found in the skin. A single spot, generally in the cheek, be- comes thin and white from the disappearance of pigment. The loss of fatty tissue underneath causes a depression also at this point. The atrophy pro- ceeds from this and gradually involves the entire half of the face. The eye is retracted, there is distinct wasting of one-half of the nose, and even the ear of one side may be smaller than that of the other side. In some few in- stances a slight glossiness of the skin has been observed, but in the majority of cases there is unusual dryness, the condition resembling scleroderma. The hair also is apt to be stiff and hard. In some instances the teeth in the affected half are small, and undergo decay. There is no disturbance of sensation, and no interference with any of the special senses in the ordi- nary run of cases. The temperature sometimes varies a little. In a case which I reported the temperature was one degree higher in the normal ear than in the ear of the affected side. The muscular movements, in spite of the increasing atrophy, as a rule, remain normal. Several other authors, and myself, have reported cases in which there were distinct tonic and clonic contractions of the muscles supplied by the fifth nerve of the affected side. Atrophy of one-half of the tongue also occurs in some cases, but it is a rare complication. VASOMOTOR AND TROPHO-NEUROSES. 211 The causes of the disease are unknown, except that several cases have been developed after traumatic injuries to the face. Thus in one case re- ported by Skryme, and quoted by Gowers, in a child three and a half years of age, the disease was developed six months after an accident in which "the face was jammed and the neck twisted." The disease has also been devel- oped after acute infectious diseases, and after other causes which give rise to neuritis. There can be no doubt, however, that in many cases the disposi- tion to the disease is congenital. Pathology. — There was much dispute regarding the origin of this dis- ease, some claiming that the cervical sympathetic was responsible, others making it a disease of the trigeminal nerve. There is no doubt that a con- dition very similar to hemiatrophy will follow upon lesions of the cervical sympathetic ; but an autopsy performed by Mendel on a case which had been observed by many other men, proved beyond a doubt that in that case, at least, the disease was due to a proliferating interstitial neuritis of the left fifth nerve, and that this neuritis was most marked in the second branch ; in that case the facial nerve was entirely normal, but the left musculo-spiral had undergone the same changes as the left trigeminal. My own case, in which there were marked clonic and tonic contractions of the masseters, would also tend to show that the trigeminus was the chief seat of the trouble, and that in some instances the motor, as well as the trophic fibres in the trigeminus can be involved in the same morbid process. The occurrence of a neuritis of other nerves, as well as the association of facial hemiatrophy with sclero- derma, would seem to prove that the morbid process may in some cases ex- tend beyond the fifth nerve. The course of the disease is, as a rule, steadily progressive, though after the lapse of time there may be a complete standstill of all the symptoms. The disease does not call for any active treatment, excepting in cases like my own, in which the spasmodic contraction of the masseters was extremely painful and had to be relieved by opiates. The disfigurement of the face is the most serious feature of the disease. In the case of one patient I suc- ceeded in rounding out the cheek again by inserting a light rubber plate, which was attached by a dentist to the patient's upper teeth.* BIBLIOGRAPHY. EXOPHTHALMIC GOITRE. Booth, J. A. : Journal of Nervous and Mental Diseases, 1894. (Thyroidec- tomy.) Charcot : Gaz. des Hopitaux, 1893, Nos. 13 and 14. V. Ehrlich : Dissertation. Berlin, 1890. (Collection of cases in children.) Eulenburg-Guttman : Pathologie des Halssympathicus. Berlin, 1873, p, 32. Eulenburg : Berliner klinische Wochenschrift, 1889. Nos. 1 and 3. * In view of the excellent results obtained by thyroid feeding in a case of hemi- atrophy with scleroderma, this treatment deserves a fair trial. 212 THE NERVOUS DISEASES OF CHILDREN. Eulenburg: Centralblatt f. Nervenheilkunde, 1887, and Centralblatt f. klin- ische Medicin, 1890. Jacobi: New York Med. Record, 1879, p. 9. Kronthal: Berliner kl. Wochensch., 1893, No. 27. (Cases in mother and child.) Moebius : Zeitschrift fur Nervenheilkunde, vol. i. , p. 460 Roberts: American Journal of the Medical Sciences, 1876, p. 374. Sickinger : Dissertation. Strassburg, 1893. (Operative treatment.) MYXCEDEMA. Gull: Transactions of Clinical Soc, London, 1874. Hammond : Neurological Contributions, I., 1881. Hamilton: New York Med. Record, 1882, p. 645. Hun and Prudden : Am. Journal of Med. Sc, 1888. Horsley: British Med. Journal, 1885. Kocher : Arch. f. klin. Chirurgie, vol. xxix. Marie: Revue de Med., 1886, p. 207. Meltzer: New Yorker Med. Monatsschrift, April, 1894. Ord : Transactions of the Medico-Chirurgical Society, London, 1878. Reverdin : Revue Medicale de la Suisse romande, Oct., 1882; June, 1883. Schiff : Arch. f. Experimentelle Pathologie, vol. xviii., 1884. ANGIONEUROTIC CEDEMA. Charcot: (Euvres completes. Paris, 1885. Collins: American Journal of the Med. Sciences, December, 1892. (Full literature.) Dinkelacker : Dissertation. Kiel, 1882. Elliot : Journal of Cutaneous and Genito-urinary Diseases, 1888. Elliot: New York Med. Record, 1891, p. 559. Goltz: Deutsche Med. Wochenschr., 1880, No. 17. Leyden : Zeitschrift fur klinische Medizin, vol. iv., p. 605. Lovett: Boston Med. and Surgical Journal, 1890. Matas: New Orleans Med. Journal, Oct., 1887. Osier : American Journal of the Med. Sciences, April, 1888. Quincke: Monatsschrift f. prakt. Dermatologie, 1882, 1. Smith : Med. News, 1889, nv -> P- 3 2 °- Starr: New York Med. Journal, Sept., 17, 1892. Weiss : Wiener Med. Wochenschr., 1882, No. 14. Widonitz : Jahrbuch f. Kinderheilkunde, 1889, N - F-» v °l- xx i x - RAYNAUD'S DISEASE. Abercrombie: Archives of Pediatrics, Oct., 1886. (In a child aged three years alternating with haemoglobinuria.) Bramann : Semaine Medicale, May, 1, 1889. (Three brothers, affected at the ages of seven, nine, and thirteen years.) VASOMOTOR AND TROPHONEUROSES. 21 3 Elliot : Morrow's System, vol. iii. Mills: American Journal of the Med. Sc, Oct., 1878. Mendel: Semaine Medicale, 1893. Morgan : The Lancet, July, 1 889. Pitres et Vaillard : Archives de Physiologie, 1885, vol. v., p. 103. Raynaud : Translation by Barlow, New Sydenham Publications. The origi- nal These de Paris appeared in 1862. Shaw : New York Med. Journal, Dec. 18, 1886. Vulpian : Gazette des Hopitaux, 1884, No. 9. Weiss: Zeitschrift f. Heilkunde, 1882, vol. iii., p. 233. FACIAL HEMIATROPHY. Bannister : Journal of Nervous and Mental Diseases, vol. iii., 1876. Eulenburg : Ziemssen's Cyclopaedia, vol. xiv., 1877 ; also Zeitschr. f. kl. Med., 1882. Jessup and Brown: St. Bartholomew's Hospital Rep., 1882. Lowenfeld : Muenchener Med. Wochenschr., 1888, Nos. 23 and 24. Mendel: Neurologisches Centralblatt, 1888. No. 14. Mills, C. K. : Pepper's System of Medicine. Muraton : Neuroglisches Centralblatt, 1891. Newmark : American Journal of the Med. Sc, 1892, vol. civ., p. 294. Rosenthal: Berl. kl. Wochenschr., 1889, No. 34. Sachs, B. : New York Med. Record, March 15, 1890. PART II. ORGANIC DISEASES OF THE NERVOUS SYSTEM. CHAPTER XII. DISEASES OF THE PERIPHERAL NERVES. SOME PERIPHERAL PALSIES. Trumatism, toxic infections, exposure to wet and cold, rheumatism, the causes which lead to disease of the pe- ripheral nerves in the adult are not nearly as frequent in children. When the same causes are operative, however, the symptoms do not vary much from those observed un- der similar conditions in more advanced years. It will not be necessary, therefore, to give the various forms of pe- ripheral nerve lesions, of peripheral palsies and neuralgias, in full detail. A description will be given of those forms which are commonly met with in the earlier years of life. A nerve-trunk is made up of a connective-tissue sheath or epineurium, which encloses the nerve-fibres proper. These are held together by a finer connective tissue, or endoneurium. If properly stained and examined micro- scopically, it is seen that each nerve-fibre consists of an axis-cylinder, with fine longitudinal striations and surrounded by a myelin sheath. If the nerve be teased or lacerated the myelin runs out and collects in drops, behaving in every way as a semi-liquid fatty substance would. Like fat it is stained a deep black by osmic acid. The myelin sheath is surrounded by a thin con- nective-tissue membrane, the sheath of Schwann. The nuclei of this sheath are visible at various points. The entire nerve-fibre is divided into segments by the nodes of Ranvier. At these nodes the myelin sheath is interrupted, and the axis-cylinder is covered merely by the membrane of Schwann. In addition to this the nerve-fibre presents the incisures of Lanterman. Blood- vessels and lymph spaces complete the nerve bundle. The morbid changes in peripheral nerves are either of an inflammatory or of a degenerative order ; and the two cannot always be distinguished from one another. Inflammation may be restricted to the perineurium (peri- neuritis), to the endoneurium (interstitial neuritis), or it may affect the nerve- tissue itself (parenchymatous neuritis). In the last-named form the changes are very much the same as in degeneration of a nerve-fibre after traumatic in- jury, or after any cause which separates the nerve-fibre from its nutrient cell. 218 THE NERVOUS DISEASES OF CHILDREN. In the vicinity of the actual lesion or of the cut ends (in physiological ex- periment and after traumatic injuries) degeneration of the nerve is estab- lished. Toward the periphery, the whole of the nerve undergoes degenera- o Ml c rm o In u m >- Wft a; to £ ¥ *-> \v o \ h <*« o ■£ c o 1 rt / OJ cd J ^ S / m £ | S £ IS ^v u be _, 1 H £ tion, but of the part that remains in contact with the nutrient cell, only the stump becomes diseased. During the process of degeneration the myelin becomes disintegrated and is divided into irregular masses ; the axis-cylinder is also broken up and the process may finally leave nothing but a granular dibris within the sheath of DISEASES OF THE PERIPHERAL NERVES. 219 Schwann. The nuclei of the sheath undergo proliferation, and Ranvier is of the opinion that this is the primary factor in the entire process of degenera- tion. Figs. 52-55. This degeneration may be continued into the finest ramifications, and even into the muscle-plates. The cells of the endo- and peri-neurium also un- dergo proliferation, and the entire nerve-fibre may ultimately be changed to a mass of connective tissue. If degeneration has not lasted too long, regen- eration may set in. If the fibres have been cut, regeneration may set in if the cut ends are not too widely apart ; the new fibrils are offshoots from the cen- tral stump, and have a tendency to grow toward the peripheral stump, and M.TIioracic XSupra-Scap. MCircumflex. JtMusc.Cut Fig. 56. — The Brachial Plexus : the Branches involved in the Upper Arm Type of Paralysis are indicated by Shading. can grow even if decalcified bone be inserted between the cut ends (Gluck and others). In perineuritis there is swelling of the connective tissue surrounding the nerve-fibres, the blood-vessels are engorged, and the lymph spaces enlarged. The connective-tissue cells are increased. If the perineuritis last any length of time, an insterstitial neuritis will also be developed. This latter form is char- acterized by similar changes ; if the morbid process is continued, the nerve- fibre invariably suffers. Gombault has described a periaxillary or segmental neuritis in which normal segments of the nerve alternate with degenerated segments. In this form the myelin alone is altered and the axis-cylinder re- mains normal. This occurs particularly in those cases which are due to toxic infection. In diseases of the peripheral (mixed) nerves, whether there be an inflam- matory or degenerative process, the symptoms are. as a rule, motor and sen- sory in character. The paralysis is limited to the muscles supplied by the diseased nerve and by branches coming off below the site of injury or dis- 220 THE NERVOUS DISEASES OF CHILDREN. ease. The sensory symptoms may be a hyperesthesia or an anaesthesia, ac- cording to the degree of change. Paresthesias are very common, and if the nerve is inflamed it is painful on pressure. It is a striking fact, however, that the motor fibres suffer much more readily than the sensory filaments ; possibly because the former are farther removed from the nutrient cell, and Fig. 57. — Dorsal Surface after Section of Ulnar Nerve. (In this and the two follow- ing figures the depth of the shading indicates the amount of anaesthesia. After Bowlby.) probably because a collateral innervation between sensory fibres is more easily established. Sensory fibres are also regenerated more easily. Further symptoms are, marked atrophy, loss of reflexes in the diseased member, and changes in electrical reaction. The degree to which the last are developed gives an indication of the amount of degeneration in the nerve. DISEASES OF THE PERIPHERAL NERVES. 22 Trophic disturbances are very common in connection with peripheral neuritis and degeneration of peripheral nerves ; but these disturbances do not, as a rule, appear except in severe cases. Local cyanosis, oedema, and ulcers are common ; the skin becomes smooth and glossy, and the growth of the nails may be interfered with. Fig. 58. — Wasting of Muscles and Areas of Anaesthesia after Section of the Ulnar Nerve. (After Bowlby.) In children, as in adults, the nerves of the brachial plexus are most fre- quently affected. The symptoms referable to lesions of the brachial plexus vary according to the part of the plexus involved. As in the adult, we can distinguish between several distinct types ; the upper-arm type, the lower-arm type, and the paralysis due to total plexus lesion. 222 THE NERVOUS DISEASES OF CHILDREN. In children the entire plexus may be involved, more especially in ob- stetrical palsies ; but often the upper arm, or Erb's type, is present. In this type the deltoid, biceps, brachialis, internus, and the long supinator muscle are affected ; at times the supinator brevis, the infraspinator, and the sub- scapular muscles are also involved. In Fig. 56, that part of the plexus sup- Fig. 59. — Palmar Surface after Section of Median Nerve. (After Bowlby.) plying these muscles is indicated by shading. These fibres issue from the fifth and sixth cerebral segments. These muscles can be excited conjointly if the electric pole is applied to the point discovered by Erb (Fig. 7), and now known by his name. The diagnosis of a partial plexus lesion can be made readily by testing the action of the various muscles (see tables, pages 18 and 22). Madame Klumpke has described a lower-arm type of paralysis which is rare in adults as well as in children ; it is due to an involvement of the DISEASES OF THE PERIPHERAL NERVES. 223 nerves or root-fibres coming from the eighth cervical and first dorsal seg- ments. In this type the smallest muscles of the hands and the flexor muscles of the hands and the fingers are the ones chiefly involved. Whatever type of brachial plexus lesion may be present, the sensory dis- turbances are relatively slight. There is generally a diminution of tactile sensation, as is evidenced by the ease with which children with obstetrical palsies tolerate strong faradic currents. The sensory disturbance will be restricted to the fibres involved ; in Erb's type, for instance, sensory changes must be looked for in the vicinity of the shoulder and the outer surface of the arm. The details of sensory supply the reader will gather from Figs. 31-34, also Figs. 57-59. THE OBSTETRICAL PALSIES. As the name implies, these palsies are due to manipula- tions during labor. They are very frequent in cases in which the physician has had to insert a finger or a hook in the axilla, and in those in which the arm has been severely pulled upon, or the arm has had to be replaced. Any manipulation which would press the shoulder and clavicle backward and upward would be liable to injure the plexus. In cases of breech presentation pressure upon the supra- clavicular spaces may cause a partial plexus paralysis. I have seen several cases in which this paralysis was bilateral. The disturbances that ensue upon these obstetrical manipu- lations may be the result of a simple traction upon some of the fibres of the brachial plexus (Erb's type most frequent), or of pressure upon the entire plexus. The clinical picture will vary according to the injury done. The affected arm hangs down quite limp. Although the child moves the fingers and the arm of the normal side, there is little or no power on the paralyzed side. This condition is the immediate result of the accident during labor, but as a rule it is not observed until a few davs, sometimes a few weeks, after birth. The affected extremity is a little colder than the normal one, and the muscles of the affected side rapidly undergo atrophy, which may for a time be obscured by the frequent excessive development of fat in children. A change of response to galvanic and faradic stimulation will be found according to the severity of the case and the age of the little patient. In the first 224 THE NERVOUS DISEASES OF CHILDREN. few weeks after birth, if electrical examination is attempted, it will be found that the muscles of the affected extremity do not respond to the electric currents. At later periods, in the milder cases, the galvanic response of the nerves may be a little altered, but the muscles, as a rule, do not exhibit any marked change. If the case is a more severe one the diseased branches of the brachial plexus will fail to respond both to the galvanic and faradic current, and the muscles supplied by these branches will show either an altered galvanic response, the anodal closure contractions being greater than the cathodal closure contractions, or the af- fected muscles may fail to respond altogether to the inten- sity of current which can be employed in children. The ordinary tests for sensory disturbances cannot easily be ap- plied in very young children. There is generally some slight diminution in all forms of sensation, but we can only state with certainty that the sensations of touch and of pain are impaired in all but the mildest cases. In considering the imperfect sensation of a very young child we must bear in mind that, as Soltman and Westphal have shown, the peripheral nerves are not fully developed until several weeks after birth. Evidently the entire central and periph- eral apparatus conducting sensation is developed later than that transmitting motion. The appearance of the affected limb changes a little as time goes on. In some of the milder forms of obstetrical paralysis a complete restoration takes place within a few weeks, or a few months, with or without medical interfer- ence. In the severer forms the atrophy is apt to increase ; the development of the limb is retarded as compared with the normal side. It is common enough to have contract- ures at the elbows and at the wrists develop in the course of a few months, or after a year or more, in those cases in which, either from lack of treatment or from the severity of the injury at birth, the initial paralysis remains unaltered. Pathology. — It is naturally a difficult matter to deter- mine the exact changes which take place in any part of the brachial plexus in consequence of obstetrical manipulations, for the opportunities of a post-mortem examination are very rare. Roger describes a case in which the facial DISEASES OF THE PERIPHERAL NERVES. 225 nerve, and the arm of the same side were paralyzed imme- diately after birth from pressure of the forceps. Large extravasations of blood were found post mortem in the vicinity of the stylomastoid foramen, and of the brachial plexus. Oppenheim, who examined one of Henoch's cases, discovered degenerative changes in the brachial plexus. In the milder forms of these peripheral lesions we must suppose that the disturbance of function was due to slight mechanical injuries, to a stretching or tearing of some of the fibres, possibly to slight injury of the nerve-sheath, or a mild form of inflammatory reaction in the nerve-tissue. In the severer cases the lesion may amount to an actual tear, to a severance of the nerve-fibres, or, in the case of subluxation of the head of the humerus, to actual compres- sion, with loss of function, of the nerve-fibres. Every pos- sible degree of peripheral neuritis may therefore be the re- sult of these obstetrical lesions. Diagnosis. — The diagnosis of obstetrical palsies is one that demands a most careful examination of the paralyzed member. The differential diagnosis is to be made between these obstetrical palsies and the so-called " birth palsies " due to cerebral lesions. Peripheral Palsies (Obstetri- Cerebral Birth Palsies, cal Palsies of Brachial Plexus). i. Arm only affected. I. Hemiplegia or diplegia common ; brachial monoplegia rare. 2. Flaccid paralysis with atrophy. 2. Spastic paralysis, with or without atrophy, with tendency to rigid- ity. 3. Deep reflexes absent, surely not 3. Deep reflexes increased. exaggerated. 4. Changes in electrical reaction from 4. No changes in electrical reaction. simple loss of faradic response to complete reaction of degenera- tion. 5. No convulsions. 5. Convulsions apt to occur and to be repeated. 6. Deformity and arrested growth of 6. Flexion contraction of fingers. entire extremity. wrist, and elbow. 7. Sensation may be impaired. 7. Sensation not affected. T 5 226 THE NERVOUS DISEASES OF CHILD REX. There is no other condition from which these obstetrical palsies need to be differentiated if the child is examined within a reasonable time after birth ; but if, as is so often the case, the child is brought to a physician when it has reached the age of six, seven, or even twelve years of age, the true cause of the palsy may be difficult to fathom. At my clinic I often see cases for which nothing has been done, and the cause is elicited only after the closest in- quiry from the mother. In older children it is a question whether the palsy dates from birth, or whether some acci- dent in the first few years of life may not have been the cause. The pulling of the arm in some of the games the children play, wrenching and twisting it as they do with considerable force, may result in palsies very much like obstetrical palsies. Poliomyelitis is not easily to be con- founded with these obstetrical palsies, for the former is rare in the first year of life, and relatively rare in the upper extremities ; the atrophy is generally more extreme, sensa- tion is not involved, and complete recovery is not common in the spinal paralysis of children. Prognosis. — If properly cared for, recovery may be ex- pected in all but the most severe forms of obstetrical pe- ripheral palsy. According to the severity of the symptoms, the length of time that it will take before recovery sets in can also be foretold, with some degree of certainty, and for this purpose the electrical behavior of the nerves and muscles constitutes a fair test for the amount of damage that has been done. Even though the paralysis be complete, if the faradic response of the branches supplying the affected muscles is not entirely lost, the probability is that complete recovery will set in within a period of two to three months. If the faradic response is lost, but the galvanic formula is not altered, the restoration of function may be expected within a period of six months ; but if both the faradic and the galvanic responses are entirely gone or seriously im- paired, it may be a year, or even two years, before the arm can be properly used. In the severer cases, in which a great deal of injury has been done to the brachial plexus, it will not be well to promise much regarding the time or the possibility of complete recover)'. Some of these cases DISEASES OF THE PERIPHERAL NERVES. 227 are extremely stubborn ; this is to be emphasized, for the majority of authors seem to regard these conditions too favorably. Much will depend upon the accuracy of treat- ment. One point which I wish to impress upon the reader, however, is that in a fair proportion of all these cases the physician may reassure the parents regarding the future condition of the paralyzed extremity. Treatment. — If the child is seen immediately after birth, and the diagnosis has been properly made, the best the physician can do is to let the limb severely alone for a period of at least two weeks ; at the end of that time the exact damage that has been done can be properly estimated and the necessary therapeutic measures can be employed. Wrapping up the arm carefully, so as to avoid any further injury to it, and allowing it to be bathed as the rest of the body is, will be all that is necessary. After a period of two weeks I would advise the use of light friction and massage, so as to keep up the nutrition of the muscles, and I am also in favor of directing an intelligent nurse or rela- tive to move the parts systematically so as to overcome any tendency to contracture. After a period of four to six weeks electricity may be employed simply as a mode of exercise. If the parts respond to a mild faradic current such may be employed, but if the faradic current fails to elicit a response it is sheer waste of time to employ this form of electricity, and it will be better to use a mild gal- vanic current, using that pole which will produce contrac- tions. Sittings of from five to ten minutes every day, or every other day, are quite sufficient, and will be of some benefit in the way of preventing excessive atrophy and of preventing the formation of contractures. As soon as there is a tendency to the development of contractures, it will be well to place the arm in a splint in such a way as to oppose the contracture by simple me- chanical force, and to take the arm out of the splint dailv for the purpose of treating it by proper massage and elec- tricity. If deformities have arisen as the natural result of disease, or from neglect, the child should be placed in the hands of an orthopedic surgeon and an attempt should be made by every known surgical procedure to correct the 228 THE NERVOUS DISEASES OF CHILDREN. existing trouble. The old belief that a child will outgrow such deformities is absolutely unfounded. It is an unfort- unate fact, but true, nevertheless, that children outgrow only the very mildest disturbances of the nervous system, which is equivalent to saying that some troubles undergo spontaneous cure. Paralysis of the lower limbs due to obstetrical manipulations is reported every now and then, but it is extremely rare. The lumbar and sacral plexuses are so well protected at every point of emergence from the spine and in their course through the gluteal and pelvic regions that they are not easily disturbed by any amount of manipulation or traction upon the legs. An injury of these parts is, however, conceivable. If present, the paralysis will resemble the paralysis of the adult in the number of muscles involved and in the character of the palsy. It is interesting in this connection to note that Ross, some years ago, supposed that traction of the legs was occasionally responsible for spastic paraplegia in children. He does not refer to the effect upon the nerve-roots, but supposes that such traction would be followed by derange- ment of the fibres in the spinal cord itself. This view has not been accepted, and it has been shown that cases of spastic paraplegia due to traumatism during birth are due, in the vast majority of cases, to cerebral and not to spinal injuries. Still another obstetrical palsy occurring quite exceptionally is facial paral- ysis due to compression of the peripheral portion of the nerve by the blade of the forceps. This is an unusual occurrence, for if the blades of the forceps are properly applied they should not be in the vicinity of the facial nerve ; but accidents will happen and the blades occasionally slip even if applied by skilful obstetricians, and thus cases of obstetrical facial nerve palsy are not entirely unknown. This may be the result of actual pressure, and in other cases an extravasation of blood into the region of the parotid is likely to com- press the nerve sufficiently to cause temporary palsy. Such a palsy bears all the evidences of peripheral facial palsy, in contradistinction to the facial palsy that is associated with hemiplegia. (See page 234.) The prognosis in these cases is, on the whole, favorable. In a few cases, however, a degeneration of the nerve occurs in consequence of the severity of the compression, and under these circumstances permanent disfigure- ment of the face may be the result. Henoch reports two such cases. Par- rot and Troisier have examined such nerves post mortem. I have the rec- ords of two cases, one in a girl of twelve, the other in a woman of some twenty-two years of age, in whom facial palsy with unequal development of the two halves of the face is the result of this obstetrical accident. The treatment of these cases is exactly the same as that for all other pe- ripheral palsies. Light massage and the use of the electric current are the only measures that can be safely recommended. In the use of the current, which I would not advise applying until the child is at least four weeks old, DISEASES OF THE PERIPHERAL NERVES. 229 great care should be exercised not to give too strong a current to the head nor to cause any sudden interruptions of the current, for the effect upon the brain would be decidedly disagreeable. Medication by drugs is not in order. FACIAL PALSY (BELL'S PARALYSIS). Disease of the seventh nerve is not infrequent in children from the same causes that result in facial paralysis in the adult. We have to con- sider, first, rheumatic fa- cial palsy, more properly perhaps refrigeration pal- sy ; second, facial palsy due to ear disease ; and third, facial palsy as an accompaniment of disease at the base of the brain. Rheumatic facial pal- sy may occur in children at almost any age. Al- though I have seen it in a child nine months old, it is rare before the age of three years, and is most frequent between the ages of six and fifteen years. Sudden draughts of cold air are by far the most frequent Cause. The Side FlG - 60. -Diagrammatic figure showing the Re- lations of the Seventh and Glosso-Pharyngeal Nerves and the Course of the Taste Fibres. The lines I, 2, 3, 4 mark off different segments of the nerve. N.f., facial nerve ; N. a. , Acous- tic nerve; G. ph., glossopharyngeal nerve; G. g., geniculate ganglion ; N. p. s., great su- perficial petrosal from fifth nerve ; vV. c, c.p. t., tympanic plexus ; the dotted line vS". /. s. indi- cates salivary fibres ; the other dotted line in- dicates taste fibres ; C/i. /., chorda tympani ; N. st. , stapedius nerve; F. st, stylo-mastoid foramen. (Modified from Erb by Dana.) of the face exposed to the draught is generally the one that is paralyzed. In a few instances it is diffi- cult to make out the ex- act cause, but even under such circumstances re- frigeration is very prob- able. I am not aware that facial palsy occurs in connection with the various diatheses in children as it does in the adult, where it is met with in association with diabetes or tuberculosis. 230 THE NERVOUS DISEASES OF CHILDREN. The symptoms of facial palsy will be readily under- stood by an examination of Fig. 60. The distal portion (1) is the one which is generally affected in these rheumatic cases. All the branches of the facial nerve are involved, but there is no interference with taste, with hearing, with movement of the palate, or with the secretion of saliva. The paralysis involves the muscles of the eyes, the nose, the cheeks, and the lips. On inspection of the child's face it is easy to make out which side is affected, as the eyelids of the diseased side are wide open (lagophthalmos). If the child is asked to close the eyes it does so with ease on the sound side, but the diseased side fails to respond.* In this effort there is over-action of the frontal muscles and of the corrugator supercilii on the sound side ; the diseased side showing either diminished action or a total paralysis. The child cannot pull up its nose ; in bloAving out the cheeks the diseased side is flabbier than the sound side, and if the attempt is made to whistle or to pout the lips, as in kissing, the insufficient action of the muscles on the diseased side becomes very evident. If asked to show the teeth, the muscles of the sound side overact, and the mouth is drawn toward the healthy side. By this movement the paralysis is often revealed, even if the parts appear perfectly normal while at rest. The tongue when protruded deviates toward the sound side ; this deviation was formerly considered to be apparent, not real ; but Hitzig has shown that there is an actual deviation toward the sound side, and supposes that this is due to the fact that the tongue wishes to avoid contact with the corner of the mouth on the paralyzed side. Contractures of the muscles of the paralyzed side occur in the later stages of a facial palsy. The lower branches re- cover, as a rule, more slowly than the upper, and in several cases I have observed that the lower and middle muscles could be contracted only if the attempt was made to close the eyes (Fig. 61). Sensory disturbances are entirely absent, as the facial nerve is a purely motor nerve, the sensory fibres of the face * A few cases of double facial palsy have been reported (Mott, Huebschman) ; a suc- cessive involvement of the two sides is more frequent. The author has observed it in cases associated with diabetes in the adult. DISEASES OF THE PERIPHERAL NERVES. 231 coming from the fifth nerve. I have seen a few cases of severe neuralgic pains complicating facial palsy in children, but to account for it there has always been some additional cause, as in a boy of fourteen, whom I recently examined, in whom decayed upper teeth offered the explanation for the neuralgic supraorbital pain. Whatever part of the nerve is involved, provided it be between the nucleus of the facial nerve in the pons and the periphery, electrical changes will be found. In all but Fig. 61. — Boy with Facial Palsy, in Stage of Recovery. Slight contracture of para- lyzed side (B) ; in figure A mouth is pulled to the paralyzed side when boy attempts forcibly to close left eye ; also deepening of naso-labial fold. the very mildest cases the faradic response of the nerve as well as of the muscles is lost. My own experience has proved, however, that there is an exception to this rule, and that is, that if the patient be examined within the first forty- eight or seventy-two hours after the onset of the palsy, the faradic response may still be present ; it rapidly diminishes, however, and after a period of three days, in the vast ma- jority of cases, it is entirely lost. The galvanic response of the nerve may be increased during the first few da vs. but it is soon diminished or lost. The galvanic irritability of the muscles supplied by the facial nerve shows many in- teresting changes. In the earlier stages of the disease, and 232 THE NERVOUS DISEASES OE CHILDREN. in all but the severest cases, there is an increased galvanic excitability for a period of about two weeks. The same currents which produce very strong contractions on the diseased side give weaker contractions on the sound side. This can be easily demonstrated in the case of the orbicu- laris oris. If a very weak current be applied to the sound half of the muscle, contractions may be seen in the diseased half and not in the normal half, the increased excitability in the latter being so great that the small amount of current diffusing to the paralyzed side is sufficient to produce a contraction, while the full current is not sufficient to make the healthy half contract. During this period the cathodal closure contraction of the diseased muscle is generally greater than its anodal closure contraction. After a little while the increased excitability diminishes and soon falls below that of normal muscular tissue. The polar con- tractions also indicate a change, the cathodal closure con- traction first being equal to the anodal closure contraction ; later on in the severer cases the order is entirely reversed. As the disease approaches the period of recovery the galvanic formula approaches more nearly to the normal, the cathodal closure contractions being equal to the anodal closure contractions. Finally, the cathodal closure con- traction becomes greater than the anodal contraction, and the contractions that have been sluggish once more become prompt and short. The faradic response is, as a rule, rather longer than this in returning, and it is not uncommon to see the full power of all the muscles return before the nor- mal electrical reactions have been established. In the case of a boy (Fig. 61) the faradic response of the muscles and a normal galvanic formula were not restored until several months after the contractures had set in, and after most of the muscles could be innervated tolerably well. In facial palsy due to ear disease, the symptoms can be easily accounted for by the position of the nerve in the Fallopian canal. The nerve may be involved during an attack of otitis media, and as this ear affection occurs often enough in children in association with several acute infectious diseases, notably scarlatina, this form of facial palsy is quite a frequent occurrence. It does not DISEASES OF THE PERIPHERAL NERVES. 233 occur as readily in the earlier stages of an otitis as in the later suppurative stages, when, in consequence of caries of the bone, the facial nerve is directly involved. According to the severity of the disease the paralysis will be more or less complete, and as far as the facial nerve is concerned the symptoms will resemble those of the rheumatic form, but naturally the association of facial palsy with defective hear- ing or the persistence of a purulent discharge from the ear will point to the actual cause of the paralysis. I have seen facial palsy occur also after mastoid opera- tions for ear disease. In these cases an accident during the operation is often sufficient to break through the fine bony plate separating the facial nerve from the ear structures, and a more or less severe facial palsy is the result. This occurred in a little patient whose case I reported a few years ago, in whom the mastoid operation was done for the relief of epileptic attacks which were caused by the reten- tion of pus. On his recovery from the operation a distinct facial palsy on the side of the operation was noticed, and existed for a period of very nearly three months, after which complete recovery took place. As in the adult so in the child, paralysis of the face may accompany lesions at the base of the brain and in the pons. If the lesion is at the base, other cranial nerves are involved as well as the facial, and the diagnosis can therefore be readily made. It is common enough to find the facial nerve partially or totally paralyzed in cases of basilar men- ingitis, whether tubercular or non-tubercular. In cases of injuries to the skull, or in tumors occupying the middle or posterior fossa, facial palsy is one of a large series of symp- toms and has but little diagnostic value excepting in its as- sociation with affections of other cranial nerves. It may be stated in this connection that in cases of cerebellar tumor, which are by no means rare in children, both the acoustic and the facial nerves, which lie in very close juxtaposition to each other and are easily pressed upon by a cerebellar growth, are often paralyzed and give rise to symptoms which are almost pathognomonic of cerebellar disease. If the lesion is in the pons, and particularly if it be below the decussation of the facial nerves, paralysis of the face is 234 THE NERVOUS DISEASES OF CHILDREN". associated with alternate hemiplegia. In these cases every branch of the facial nerve is apt to be involved. If the lesion be a neoplasm other symptoms pointing to compres- sion of the cranial nerves and indicating the presence of tumor within the cranial cavity will naturally be associated with those mentioned before. The diagnosis of facial palsy is easy to make, and the only difficulty that arises at times is to determine whether the palsy is indicative of a central or a peripheral lesion ; but this difficulty can be readily met by keeping in mind the fact that if the lesion is in the brain anywhere above the pons the upper branches of the facial escape altogether and the electrical responses remain entirely normal ; furthermore in such a case the facial palsy is associated with hemiplegia on the same side of the body as is the paralysis of the face. It is far more difficult to determine the exact part of the nerve that is diseased. As the intensity of the affection depends somewhat upon the extent of the nerve tract that is diseased, this inquiry into the accurate localization of the disease has a practical as well as a scientific interest. Taking the diagram on page 229 we can study the variation of symptoms according to the part that is affected. If the disease is limited to that part outside of the Fallo- pian canal, there is complete paralysis of every branch of the facial nerve with the exception of the posterior auricular. Reflexes are wanting, but there are no disturbances of taste or hearing, no paralysis of the palate, and no dis- turbances of salivary secretion. If the disease happens to affect the part of the facial nerve as far as, but not including, the chorda tympani (Fig. 60 line 2), the symptoms are paralysis of all the branches of the facial, including the posterior auricular. The same symptoms as the above, with the addition of a disturbance of taste in the anterior portion of the tongue and a diminution in salivary secre- tion points to an involvement of the facial nerve from the point of junction of the chorda tympani to the junction of the stapedius with the main trunk (from 2 to 3). If that part of the nerve lying between the stapedius and the ganglion geniculi is diseased, the symptoms, in addition to total paralysis, are changes in taste, diminution in salivary secretion, and abnormal acuity of hearing, but there is no paralysis of the soft palate. The above symptoms, with the addi- tion of paralysis of the soft palate, point to a lesion in the region of the gen- iculate ganglion (division 4 to 5) ; but on this point there is much doubt. See table, p. 13. Paralysis of all the peripheral branches, a*s well as paralysis of the soft palate, abnormal acuity of hearing, decreased salivary secretion, but no involve- ment of taste, help to locate the lesion above the geniculate ganglion, and be- tween it and the exit of the facial nerve from the brain (division 5 to 6). If this part of the facial tract is diseased other cranial nerves are frequently affected. In such cases we are apt to have paralysis of the abducens, loss of hearing on the same side as the facial palsy, tinnitus aurium, and the reaction of degen- DISEASES OF THE PERIPHERAL NERVES. 235 eration of the acoustic nerve. If we have total palsy of every branch, palsy of the soft palate without disturbance of taste and with simple diminution of electrical excitability, without any other marked symptoms, we may locate the lesion in the facial nucleus ; but this form of palsy is recognized more definitely if other cranial nerves are simultaneously affected, such as the hypoglossal, the spinal accessory, the vagus, the trigeminal nerve, etc. The association of the symptoms just mentioned with paralysis of the arm and leg of the opposite side, refer the lesion to the pons. If there is but par- tial paralysis of the face, if the electrical excitability is not affected, if the re- flexes are normal, and if the extremities of the same side are affected, the lesion is in the crus or in the hemispheres, say in the internal capsule. If the facial nerve and the oculo-motor nerve of opposite sides happen to be affected the lesion is positively in the crus on the same side as the ocular palsy. The course and prognosis of all forms of facial palsies will depend entirely upon the nature of the morbid proc- ess. If the disease is due to caries of the petrous por- tion of the temporal bone, to malignant tumor, to fracture, to tubercular meningitis, or to any form of meningitis, the prognosis is extremely grave. Even those cases depending upon specific diseases at the base are not as amenable to treatment as one might expect. Those due to parotitis or to internal otitis recover if the disease itself is not of the destructive type. Cases due to simple section of the nerve, as in cases of operation, yield a tolerably favorable result. The prognosis of the rheumatic forms of facial palsy varies according to the intensity of the disease, but it does not depend apparently upon the amount of nerve-tissue that is affected, for, on the whole, cases in which the nerve within the canal is involved take about as favorable a course as do those in which the pes anserinus is the only part affected. Erb distinguishes three forms — a mild form, a medium form, and a severe form of facial paralvsis. His division depends entirely upon the electrical conditions present at the end of the first week. I cannot agree alto- gether with his statement that if the electrical excitability is entirely normal at the end of the first week, as in light forms, recovery will set in in two or three weeks. If at this same period the faradic and galvanic excitability are slightly diminished recovery may be expected in from four to six weeks, and if at the end of the first week the elec- 236 THE NERVOUS DISEASES OF CHILDREN. trical excitability of the nerve is very much diminished or totally lost, the disease will run a course of many months. I have seen cases get well practically within four weeks, in which the faradic and galvanic excitability of the nerve was diminished very much from the very start, and I should be inclined to formulate the following statements : I. If at the end of the first week, or, still better, at the end of the second week, the nerve responds at all to the faradic or galvanic current, a prompt recovery in about four weeks may be expected. II. If at about the same time the nerve fails to respond, but the muscles show a diminished or altered galvanic re- sponse, the disease is likely to run a course anywhere be- tween one and three months. III. If the muscles respond but feebly to strong cur- rents, if the galvanic formula is altered, and if the contrac- tions are extremely slow, the disease may run a course any- where between six months and a year, or even longer. If after a period of two months no electrical reaction can be observed, the degeneration is very complete, and a paral- ysis lasting at least a year, if not longer, may safely be pre- dicted. Treatment. — We need say little of the treatment in cases in which the facial palsy is only one of many symptoms pointing to gross cerebral disease. Under such circum- stances treatment directed to the relief, or possibly the cure, of these conditions will have to be employed. In the cases of facial palsy due to ear disease, proper surgical treatment should be recommended at a very early day. Much doubt has been expressed as to whether any treatment can cur- tail or cure the rheumatic palsies. The milder forms will undoubtedly get well even if no treatment is attempted. In the more chronic cases the use of the electrical current, particularly of the galvanic, can be safely recommended, for I have the distinct impression that cases so treated, and particularly those that have been properly treated from the very start, run a somewhat shorter course than those in which no electrical treatment has been attempted. Remak's recent article gives statistical evidence in favor of this view, and puts a quietus for the present upon those DISEASES OF THE PERIPHERAL NERVES. 237 who think that there is nothing in electro-therapeutics but hypnotism and suggestion. The electrical current, even if it have but slight influ- ence in restoring the power of conduction in the diseased nerve, is surely of value, as it is in paralysis of the extremi- ties, in exercising parts that cannot be moved by the will, and for this reason alone it deserves to be employed. Counter-irritation has been repeatedly tried. This may be accomplished by blistering either with emplastrum can- tharidum just in front of the ear or over the mastoid, or by the use of the actual cautery. I am free to confess, however, that the value of counter-irritation seems questionable in all cases, and particularly in those in which counter-irritation is not indicated to allay pain that is so frequently associated with neuritis. The application of leeches is to be thoroughly condemned, except possibly in those cases in which the fa- cial palsy is associated with ear disease. This practically limits us to the use of electricity as the only therapeutic measure which promises some relief in the severer cases. In these cases the faradic current, as recommended by Erb and Duchenne, may be employed for the first week or two, but after that a stabile galvanic current applied over the mastoid and along the peripheral divisions of the facial nerve is more in order. As soon as the ex- citability of the nerves and muscle is increased, or shows an approach to the normal, regular electrizations of the paralyzed muscles, in sittings of ten minutes or more, two or three times a week, should be practised. I wish to cau- tion the physician, however, against the use of strong cur- rents or against the use of the electrical current without the safeguard of a galvanometer. A current varying be- tween two and four milliamperes is quite sufficient for all purposes. I have also directed my patients, as soon as the least power returns, to practise contraction of the muscles (closing of the eyes, pouting of the lips, etc."), while look- ing into a mirror. As for medicinal measures, they may be discarded alto- gether. In many cases I have tried strychnia both per os and in the form of hypodermic injections, and I am firmly convinced that the effect of such medication is absolutely 238 THE NERVOUS DISEASES OF CHILDREN. nil, nor can we expect much more from the use of irritat- ing ointments or douches to the face. If drugs must be administered it is far better to give the ordinary tonics which will help to build up the general condition of the child ; and with this end in view, iron, arsenic, cod-liver oil, will be of far more service than any other remedies. Other Peripheral Palsies. — Almost any peripheral palsy which is known to occur in the adult may at times occur in the child. Of these I will make special mention only of a peripheral palsy, generally of traumatic ori- gin, affecting the peroneal nerve. Injury to this nerve occurs readily enough if a child happens to be violently struck upon the leg, or if the leg happens to be squeezed against a post, a chair, or, as in one case I have seen, against the edge of the bed. I mention this special palsy because it may lead to slight difficulties in diagnosis, inasmuch as confusion may arise with acute anterior poliomyelitis which affects the muscles supplied by the peroneal more often than those supplied by any other nerve. The onset of the paralysis without fever, the presence of considerable pain, the marked and rapid tendency to recover} 7 , and the history of a trau- matic injury will help to distinguish this peripheral palsy from the essential paralysis of children. The course, prognosis, and treatment of such a pero- neal neuritis will be exactly the same as in the case of other nerve palsies. Spasm of various peripheral nerves is common in young persons. The facial and the spinal accessory nerves are frequently affected ; the trigeminal in rare instances only. Spasm of the facial nerve (tic convulsif) is much less frequent than in adults ; a spasm involving an entire facial nerve of a child is very rare indeed ; but partial spasms are observed in the form of nic- titation (clonic spasm) or of blepharospasm (tonic spasm) of the obicularis palpebrarum. These movements are generally due to the influence of cold, to irritation of the trigeminus, but are also frequently of psychic origin. In the cases of clonic spasm of the entire nerve, all sorts of extravagant grimaces are indulged in. These spasms must not be confounded with habit chorea. Spasm in the distribution of the spinal accessory nerve involves either the sternocleido-mastoid or the trapezius (spasmodic wryneck). The spasm may be reflex in origin, or due to irritation of the nerve in its course from the medulla and spinal cord to the periphery. The position and movements of the head will vary according to the muscles involved. These spasms may be unilateral or bilateral. If we remember that spasm, whether clonic or tonic, denotes an excess of normal function, the symptoms can be easily understood by reference to the tables on pages 15 and 16. Ordinary wryneck, so frequent in children, is due to a rheumatic myo- sitis. Symptomatic wryneck occurs in connection with cervical adenitis, abscesses and caries of the cervical spine. Congenital wryneck is due to some pre-natal disturbance ; it may be of intra-uterine origin, and denote im- perfect development, in which case it is generally associated with atrophy DISEASES OF THE PERIPHERAL NERVES. 239 of one-half of the face ; at times it is due to obstetrical injuries in cases of breech and foot presentations. The treatment of spasms of the peripheral nerves consists in the use of sed- atives, such as opium, conium, codein, chloral, and bromides ; in some cases the valerianate of zinc, or the ammoniated tincture of valerian, might be given. The galvanic current may be applied in the milder cases. The attempt may be made to check the spasms by hypodermic injections of hydrobromate of hyoscine, one two-hundredth to one one-hundredth grain, or by the use of atropia (one-fiftieth to one-tenth grain, as recommended by Leszynsky). Tenotomies are in order in the congenital and chronic cases. Keen has suggested resection of the posterior branches of the upper three or four cervical roots ; but the advisability of this procedure is still in doubt. Spasm of the hypoglossal nerve I have not seen in children. The com- plex co-ordinated spasms have been considered in Chapter VI. BIBLIOGRAPHY. PERIPHERAL PALSIES. Bernhardt, M. : Arch. f. kl. Med. vol. xiv., 1874, p. 433; Zeitschr. kl. Med., 1882, p. 415 ; Neurologisches Centralblatt, 1886, p. 141. Erb : Diseases of the Peripheral Cerebro-spinal Nerves, in Ziemssen's Cy- clopaedia. (Contains full literature to 1876.) Hirt : Lehrbuch ; Second ed., p. 364. (Contains ample references.) Hitzig : Berliner klinische Wochenschrift, 1892, p. 1270. (Position of tongue in facial palsy.) Hoffman : Zeitschrift f. Nervenheilkunde, vol. v., p. 72. Huebschman : Neurologisches Centralblatt, November, 1894. Klumpke : Revue de Medecine, July, September, 1885. Leszynsky : Journal of Nervous and Mental Disease, January, 1890. Mott : British Medical Journal, December 2, 1893. Oppenheim and Siemerling : Arch. f. Psych., 1887. Parrot et Troisier : (Facial Paralysis in the New-born), quoted by Henoch, p. 227. Pfeffer: Klumpke 'sche Lahmung., Deutsche Zeitschr. f. Nervenheilkunde, 1 891. Remak : Zeitschrift f. Nervenheilkunde, vol. iv., p. 377. Roger: Journal fiir Kinderkrankeiten, 1864 (quoted after Henoch). Ross and Bury: On Peripheral Neuritis. London, 1893. Van Bibber : In Keating 's Cyclopaedia, page 788. CHAPTER XIII. MULTIPLE NEURITIS. Neurology has few more signal achievements to show than the advances that have been made in the study of multiple neuritis within the past decade. Many diseases which were formerly supposed to be due to an affection of the spinal cord, and which were often confounded with poliomyelitis, and with various forms of acute and sub- acute myelitis are now positively known to be due to poly- neuritis. The term multiple neuritis or polyneuritis is used to embrace all those diseases in which the symptoms are due to disease of several or many of the nerves of the body. There is not only a tendency to multiplicity but also a tendency to symmetrical development. Many of the older writers recognized the possibility of multiple nerve af- fections, and a multiple neuritis, as such, was recognized by Dusmenil as far back as 1 864, and even a few years earlier Virchow had described a multiple neuritis occurring together with leprosy. Going back still further, the histor- ical investigations have led to the discovery that Dr. Jackson, of Boston, had recognized peripheral multiple neuritis as early as 1822 ; but all these re- searches, even including those of Duchenne, did not help to place the knowl- edge of multiple neuritis upon a firm basis. The subject was revived by Jof- froy, iri 1879, an d Leyden, in 1880, and since that time a legion of articles have appeared by writers of almost every nationality. Comparatively few writers, however, have had much to say regarding multiple neuritis occurring in children. Diphtheritic paralysis, to be sure, was discussed clinically long before it was known that it was due to peripheral neuritis. Multiple neuritis is not so frequent in children as in adults, for the simple reason that the causes leading to it are not as powerful in early life as later on ; and yet cases are common enough to necessitate a discussion of them in this book. Symptoms. — Whatever the cause of the multiple neuritis may be the symptoms are, with few modifications, practi- MULTIPLE NEURITIS. 241 cally the same in all cases. Though the development of the various symptoms is subject to great variations, the symp- toms are such as we might well expect on physiological grounds from disease of the peripheral mixed nerves. The Fig. 62. — Young Boy with Multiple Neuritis, showing Double "Wrist Drop" and Slight " Foot Drop." most characteristic feature of multiple neuritis is the asso- ciation of motor paralysis with sensory paralysis, the dis- tribution of each harmonizing with the other and showing- very definite anatomical limits. The paralysis is of the flaccid order, leads at an early date to atrophy of the at- 16 242 THE NERVOUS DISEASES OF CHILDREN. fected muscles, and the electrical conditions are altered so that Ave may find almost every possible form of the reac- tion of degeneration from a mere loss of faradic irritabil- ity to an absolute loss of galvanic response on the part of the nerves and muscles. The distribution of the paralysis is, as a rule, entirely symmetrical, and may affect either the upper or lower, or all four extremities ; it may involve every part of all the extremities, and is the one affection which, perhaps more frequently than any other, leads to a complete paralysis of every limb of the body. It is one of the diseases, therefore, which the physician should bear in mind if he finds a patient who is totally paralyzed with the bare exception of the head and tongue, the jaws and eyes ; sometimes some of the latter parts may be paralyzed as well. Certain groups of muscles are more readily af- fected than others ; thus we find that the extensors of the wrist and the extensors of the feet are very frequently in- volved, and at an early period of the disease " wrist-drop " and " foot-drop " are characteristic phenomena of the dis- ease. With the paralysis sensory symptoms go hand in hand ; paralysis associated with pain may be noted in some cases of spinal-cord disease, as in the acute stages of a poliomyelitis, but the persistence of pain in those parts which are paralyzed, and along the nerve-tracts, points very strongly to a multiple neuritis. Whenever we suspect that a paralysis or a deformity may be the result of a multiple neuritis, the previous occurrence of a painful affection and the duration of the same must be determined with the greatest care. If the patient is seen during the acute stage of the disease the tracts of the peripheral nerves will be found painful, and this objective proof will be added to the sub- jective symptoms which the patient describes. But the ab- sence of pain, as evidenced by cases reported by Barrs, need not militate against the diagnosis. The motor and sensory paralysis is not developed as rapidly as is the case in central affections. There is a premonitory period during which numbness, tingling, sensations of heat or cold, in the parts to be affected later on, are the annoying symptoms, and after a premonitory period that may vary from a few days to a few months the development of the paralysis may be acute, subacute, or chronic. In the majority of the cases it is sub- MULTIPLE NEURITIS. 243 acute or chronic, and it often requires a period varying between one and two weeks until the weakness of the parts has advanced far enough to be con- sidered an actual paralysis. The onset is marked occasionally by the oc- currence of fever, and if the disease is due to any toxic agent that has con- stitutional effects the beginning may be signalized by convulsions as well as by rigors. Under the latter circumstances the symptoms may very closely resemble those of an acute poliomyelitis, but it is rare, after all, to find as stormy an onset in cases of multiple neuritis as in spinal infantile paralysis. Before the paralysis becomes absolute, if it ever reaches that stage, tremor of the parts and inco-ordination may be associated with loss of power and loss of sensation. The patient may be able to stand or walk, but does so imper- fectly, sways with the body when attempting to stand, and may fall if the eyes are closed. The reflexes connected with the parts affected by the paralysis are almost inva- riably diminished or lost ; this is particularly true of the knee-jerk, since the trouble so often begins in and involves the legs to a greater extent than any other part of the body. The reflex is often absent before the paralysis is developed. An exception to the rule of loss of knee-jerk has been reported by Striimpell. A few years ago he de- scribed a case in which the reflexes were increased, and this increase he attributed to irritation of the afferent por- tion of the reflex arc. The more distal parts of the extremities are, as a rule, the first to be affected. In the lower extremities the ear- lier stages of the disease are marked by an inability to raise the toes while the heel remains on the ground, show- ing a weakness of the extensors of the toes, and implying a loss of function of the branches of the anterior tibial nerve. It is a curious fact that the muscles supplied bv the anterior tibial nerves are not only most frequentlv af- fected in multiple neuritis, but they also innervate the re- gion which is frequently involved in poliomyelitis. In the upper extremities the extensors in the forearm are affected in the earliest stages of the disease, and from these parts the paralysis may extend upward in both extremities until the greater part of the muscles are affected or until total paralysis of all the extremities is established. The thenar and hypothenar groups are hot affected at the outset. It 244 THE NERVOUS DISEASES OF CHILDREN. will be seen from this that the musculo-spiral nerve in the upper extremity, and its homologue, the peroneal nerve, in the lower extremity, are the nerves first attacked by the disease in a large number of the cases. This peculiar pre- disposition of these nerves to the disease cannot be ex- plained on the ground of anatomical position, and for the present is as inexplicable as are the facts of elective affin- ities in so many other diseases. In some cases the nerves supplying the trunk muscles, including the diaphragm, may be affected, but this is, after all, a very great rarity. A paralysis of the vocal cords due to multiple neuritis is quite as rare as is also an in- volvement of the facial nerve and of the hypoglossal. I have, however, seen a number of cases, particularly after diphtheria, in which the ocular muscles, and these only, were paralyzed from neuritis of the third and sixth nerves. The paralysis, although it may involve a number of groups of muscles, does not affect all equally ; those less affected may undergo contracture, such contractures occurring quite as often in the lower limbs as in the upper extremities. Deformities quite as serious and quite as persistent as those in poliomyelitis may therefore result from a chronic multiple neuritis. As regards the sensory symptoms it is important to note that every form of sensation is about equally impaired ; touch, pain, temperature, and muscular sense may suffer equally except in the earlier stages, in which a hyper- esthesia is a more common than hypassthesia. In a few instances the pain sensibility may be diminished while the sensation of touch remains, and even if the sensibility to pain is intact the conduction of pain impressions may be very much delayed. While pain and paralysis are the dis- tinguishing symptoms of a multiple neuritis it should be remembered that in not a few instances one or the other may be absent, and in some cases pain may never have been present to any marked degree, or may have lasted for so short a time as compared with the paralysis that the pa- tient who is examined at a later stage of the disease fails to remember that pain was ever present. The electrical re- actions may exhibit every form of change from a very slight diminution of faradic and galvanic response to a MULTIPLE NEURITIS. 245 partial or complete reaction of degeneration. Atrophy is also a prominent and early symptom. The entire absence of bladder and rectum symptoms is of great importance in the diagnosis of multiple neuritis. Vasomotor changes in the paralyzed parts constitute a frequent symptom of multiple neuritis. (Edema and glos- siness of the skin, which are observed in the majority of the cases of peripheral nerve disease, are present in many cases of multiple neuritis. If the hand is involved we have not only the changes just mentioned but also a peculiar taper- ing of the fingers, which is quite characteristic of disease of the peripheral nerves. The course of the disease will vary very much according to the intensity of the cause. As a rule the symptoms in- crease during the first four to six weeks and then diminish. While the disease is increasing sensory symptoms are par- ticularly prominent. As soon as the acme of the disease has been reached these symptoms become less troublesome. The motor paralysis is recovered from much less quickly than are the disturbances of sensation. This is due not only to the actual loss of innervating power but to the atrophy of the muscles and to the contractures which have been formed. But even in severe cases a favorable change sets in sooner or later. The atrophy diminishes and the strength of the paralyzed limbs slowly increases until com- plete recovery is established. In some instances the onset of the disease is a very sudden one. The paralysis spreads rapidly and may resemble the course of Landry's paralysis ; but the fact that in multiple neuritis the trunk muscles generally escape and that the paralysis jumps from the lower extremities to the upper without in- volving the trunk, will help to differentiate it from an acute ascending palsy. While we have good reason to expect every case of multiple neuritis in a child to lead to recovery, death may result in consequence of complicating conditions, such as paralysis of respiratory muscles, or of the heart from disease of the vagus. Bronchitis and pneumonia are complications very much to be feared in children who have any form of multiple neuritis. Cir- rhosis of the liver and gross cerebral disturbances, due to alcoholism, which are common causes of death in cases of multiple neuritis in the adult, do not play a similar role in children. On the other hand, the involvement of the kidneys in cases of diphtheritic palsy is. a frequent cause of death. The psychoses described in connection with the multiple neuritis of adults are not met with in children. 246 THE NERVOUS DISEASES OF CHILDREN. Multiple neuritis may result from a number of different causes: I. Toxic forms, including those due to metallic and non-metallic poisons. Among the former lead, arsenic, and alcohol are the most potent; and in rare instances mercury, carbonic oxide, and phosphorus may bring on a neuritis. In a very careful article in Keating's " Encyclo- paedia," Dr. Putnam, of Boston, has discussed the palsies of children due to lead and arsenic. He finds that poisoning by arsenic occurs most frequently through the mixtures used to destroy vermin, such as " Rough on Rats," " Paris Green," " German Fly-paper," and the like. Furthermore, arsenic is contained in many of the coverings used for books, toys, confectionery, labels, etc., as well as for arti- ficial flowers. The same author also believes that wall-paper and some special forms of carpet may be the source of poi- soning by arsenic. The arsenic is supposed to be commu- nicated to the child in the form of dust distributed in the currents of air, or in the form of some volatile compound which may be formed in a room which contains arsenious wall-paper. That some cases occur from the continued administration of arsenic in diseases like chorea there is very little doubt, and it is well to watch children carefully, while they are undergoing this form of treatment .* As for lead, it may enter the system from glazed cards, acid fruits and vegetables put up in tins, also from chocolate and other articles wrapped in tinfoil. Lead also enters largely into the composition of rubber, and has been found in the rub- ber nipples of nursing-bottles. Various kinds of cakes and candies are treated with lead, and prove a prolific source of poisoning in children ; but children as well as adults are exposed to lead-poisoning very much more fre- quently through drinking-water than in any other way. This is more com- mon in country districts, where lead pipes are connected with pumps, or where lead pipes are used to convey water from wells or springs at varying distances to the house. In the large cities this special source of lead-poison- ing is extremely rare. Alcoholic poisoning in children would naturally be con- sidered a rare condition, and so it fortunately is : but among the ignorant classes of every large population par- ents will be found who take special delight in the fact that their child can partake of everything, including beer or * I have seen one case of neuritis in the adult after the use of an arsenic paste over a large denuded surface. MULTIPLE NEURITIS. 247 whiskey. A case of alcoholic neuritis in a child five years old has recently been reported from Strumpell's clinic. The child was raised in the saloon of its father, and not only drank considerable wine, but also two quarts of beer, every day. The father is quoted as saying that " the child was always thirsty, and of course we could not give it water." 2. The toxasmic forms of multiple neuritis are far more important — at least in children. Among these we class, first of all, the septic forms of multiple neuritis which may occur in children as in adults after injury of any sort. The inva- sion of micro-organisms is unquestionably the direct cause of the neuritis. A similar invasion of specific organisms would account for the neuritis of leprosy and of beriberi, but these are so rare in this country, and particularly rare in children, that we need not make any further mention of them. Malarial neuritis is of great interest to us, for though it has been described chiefly by authors in Eastern countries, it is not unknown in these parts. I have seen several cases of this description in children who were brought to my clinic from the swampy parts of Long Island. Dr. Browning, of Brooklyn, has reported several such cases, which were supposed to be cases of poliomyelitis. These cases resemble poliomyelitis in their onset and devel- opment, and may be easily mistaken for it. In its general symptoms this form differs but very little from other cases of multiple neuritis except that it is associated much more frequently with the intermittent form of fever, that the dis- ease itself undergoes marked remissions, and that the en- largement of the spleen and examination of the blood are likely to give positive proof of the origin of the neuritis. Recovery is a little more rapid, too, in these cases than in those due to other causes. Among the toxasmic conditions which give rise to mul- tiple neuritis we must include those blood states which fol- low upon the various acute infectious diseases (diphtheria. small-pox, typhoid fever, tuberculosis, and also syphilis). As the nerve affection is developed a long time after the initial infection it is probable, in the light of recent bacteriological 248 THE NERVOUS DISEASES OF CHILDREN. researches, that some chemical product of the micro-organ- isms, and not the bacteria themselves, are the direct cause of the neuritis. With the exception of diptheritic multiple neuritis (p. 253) all the forms just mentioned are compara- tively rare in children. In obedience to the ordinary belief, we must concede the existence of the" rheumatic forms of multiple neuritis ; but I am free to confess that I have seen only a very few cases of multiple neuritis in children which could with some degree of probability be referred to rheumatic causes. In cases of articular rheumatism in children the nerves are sometimes involved, as is proved by pains radiating down the limb through special nerve-trunks. Refrigeration neuritis occurs in children. In one instance this was due to ice-cold baths which a mother gave to her child daily all the year around. Now and then cases come under one's notice of severe forms of polyneuritis in which the true cause cannot be ascer- tained. Pathological Anatomy. — The chief lesions are re- stricted to the peripheral nerves. It is natural to infer that the changes are very much the same as those to be found in cases of ordinary simple neuritis (see page 217). In mul- tiple neuritis the changes are chiefly of a parenchymatous order, or interstitial in character ; the perineurium is not often involved. In some cases, however, the nerve is swol- len, the nerve-sheath is hyperaemic, of a deep purplish color, and, according to Eichhorst, may be covered with minute hemorrhages. There may be some slight changes also in the consistence of the nerve. All these conditions, and the appearances of degeneration, will vary according to the stage of the disease at the time of examination. In the earlier stages we often find the nuclei of the fibres en- larged, a proliferation of the connective tissue between the nerve-elements, and numerous round or spindle-shaped cells with myeline granules near the sheath of the nerve. If the specimen is examined at a later period of the disease the acute condition will have passed and the nerve presents the simple appearance of subacute or chronic degeneration of nerve-fibres. (Fig. 63.) In some cases the muscular tissue is slightly involved, MULTIPLE NEURITIS. 249 the fibres are smaller and paler, the transverse striation is less distinct than under normal conditions, while the nuclei of the fibres and of the interstitial tissues may be increased. The changes in the muscle may be both parenchymatous and interstitial. In considering the pathology of multiple neuritis, the most striking fact is the peculiar action of toxic agents upon the peripheral nerves, without affecting the spinal centres. The farther away the nerve-fibre is from the mother-cell the less able it is to resist morbid agencies ; for this reason the more distal parts of the peripheral nerves are affected at an earlier period in multiple neuritis than are the parts of the nerve nearer the spinal centres. This argument is strengthened by the re- cent conceptions of the close relationship between the ganglion-cell and the peripheral nerve -fibre as parts of a neuron. More- over, recent researches have shown that the same poison which often affects FlG 63 ._ Chronic interstitial Neuritis with Atrophy of the peripheral nerve-fibre may occasionally involve the ganglion-cell. Stieglitz has proved this to be the case in lead-poisoning. He found a distinct poliomy- elitis in animals compelled to inhale lead salts ; yet we cannot deny, in view of various post-mortem findings, that in man lead is prone to cause disease of the peripheral nerves. Oppenheim has described slight changes in the spinal cord in cases of alco- holic neuritis, and I have little doubt that similar changes will be made out in cases of arsenical neuritis. Diagnosis. — With our present knowledge of the charac- teristic symptoms of multiple neuritis there should be no difficulty in differentiating between the various forms oi this disease and other diseases with somewhat similar symptoms. The chief characteristic of these peripheral nerve diseases is the association of sensory with motor some Nerve-fibres. (Ziegler. ) a, cross-sections of normal nerve-fibres ; b, cross-section of thin, but nor- mal, nerve-fibres ; c, endoneurium ; d, cellular in- filtration and proliferation of connective-tissue strands and blood-vessels ; f, thickened endoneurium with small empty nerve-spaces and some normal thin nerve- fibres ; g, longitudinal section of a blood-vessel. 250 THE NERVOUS DISEASES OF CHILDREN. symptoms, the distribution of both pointing to an in- volvement of the same nerve-areas. Persistent sensitive- ness of the nerve-tracts and subjective pain in the course of the peripheral nerves help to corroborate the diagnosis. It is easy to confuse multiple neuritis with poliomyelitis, and until recent years this mistake was made by the ablest diag- nosticians ; but in poliomyelitis the initial symptoms are of a rather more violent character, and the entire central nervous system often shows the influences of the disease very much more than it does in multiple neuritis ; the paralysis is more symmetrical in neuritis than in poliomye- litis. In poliomyelitis there is no pain along the nerve-tracts and these nerve- tracts are not sensitive, but vague pains are very often present in the first week or two of poliomyelitis. [ have the record of a child, two years old, whom I saw two days after the onset of the paralysis, in whom the entire limb was so painful that the child would scream violently if the limb was suddenly touched, and this was not due to the fear of examination. The pain was not distributed along any nerve-paths, and I did not hesitate to make the diagnosis of poliomyelitis, which proved to be the correct one by the subsequent course of the disease. The electrical reactions are very much the same in both disorders. The difficulties of this problem are increased by the fact that, as Gowers has reported, cases occur in which a typical anterior poliomyelitis is compli- cated by a peripheral neuritis. In spite of all efforts at differentiation cases will occur every now and then in which it will be impossible to make an ac- curate diagnosis at the start, and the physician must watch the course of the disease before coming to a definite conclusion. From pachymeningitis, which is rare in children, the disease may be dif- ferentiated more easily, since in the former the legs are rarely affected, the anaesthesia is apt to spread to the upper limbs and to the trunk of the body, and there is no distinct sensitiveness to pressure on the part of the larger nerve-trunks, except late in the disease, and the atrophy is restricted to muscles innervated from the diseased spinal segment. Landry's paralysis is to be recognized by the rapidly ascending palsy pro- ceeding from segment to segment, involving the abdominal and thoracic muscles, and not skipping from the lower to the upper extremities, as is the case in poliomyelitis. The differentiation between the ataxic forms of multiple neuritis or the so-called pseudo-tabes from true tabes need hardly be considered in the case of children, as in them true tabes is a disease of exceeding rarity. But it may be well to bear in mind that the symptoms of hereditary ataxia (Friedreich's disease), including the loss of knee - jerk, ataxic gait, weakness, and awkwardness in walking, may bear a superficial resemblance to polyneuritis ; but the gradual development of the symptoms in MULTIPLE NEURITIS. 25 I this hereditary disease, the occurrence in various members of the same family, the peculiar appearance of the face, the disturbance of speech, the mental peculiarities, will render the diagnosis positive enough. As children are subject to hysterical palsies the question of differential diagnosis may occasionally arise, but hysterical palsy is not characterized by the loss of reflexes ; nor is the ataxia, if present, like that of polyneuritis ; and, moreover, the anaesthesia accompanying the palsy in hysterical cases is of the regional order, and not in keeping with the anatomical distribution of the nerves. If there is any reason to suspect myelitis, the presence or absence of bladder and rectal symptoms, the development or non-development of bed- sores will weigh heavily for or against the diagnosis of cord disease. Trichinosis must be suspected in cases of great painfulness of the ex- tremities, but the other attendant symptoms (gastric disturbances) and the excessive swelling of the muscles will help to clear up the diagnosis. The recognition of the cause of multiple neuritis is an important point in the diagnosis of the disease. In this country the most frequent cause will undoubtedly be found to be a preceding acute infection, either by poisoning or by the toxic products of some acute infectious disease, such as diphtheria, variola, and typhoid fever. Next in frequency I would place the probability of a malarial origin, and last, the possibility of tubercular, syphilitic, or alco- holic infection. Treatment. — First of all we must determine the pri- mary cause of the disease, and, if possible, the deleterious agent must be removed or its influence arrested. If the child lives in a malarial district it should be taken to a place where there is no danger from further malarial infection. If there is any suspicion of alcoholic, or of lead, or of arseni- cal poisoning, the sources from which such poisons have emanated must be absolutely cut off. If some acute infec- tious disease has preceded, we can, of course, do nothing to prevent the natural course of the neuritis, but b} 7 improving the child's general health will enable it to conquer the dis- ease much more rapidly. For the relief of pain, which is by far the most annoving symptom at the beginning of the disease, the application of heat is the best remedy we have. If heat is applied at later stages of the disease, when con- siderable anaesthesia may be present, it is of the greatest importance to watch the condition of the skin, or else seri- ous burns and ulcerations may result. It is on this account. also, that the use of the actual cautery and of other forms of counter-irritation is not to be recommended. Prolonged warm baths will often help to alleviate the suffering, and 252 THE NERVOUS DISEASES OF CHILDREN. will contribute largely to the comfort of the patient. If the pain is so intense that it cannot be relieved by these simple measures it will be well to give small doses of mor- phia, codeia, or rectal injections of chloral hydrate. There is no drug which has any specific action upon multiple neuritis. I have given the salicylates invariably, but am not convinced that they have done much good. Mercurials have been praised by some, particularly in cases in which the nerve-sheath is involved rather than in the parenchymatous form, yet I have not been able to persuade myself of the benefits to be derived from mercurials, even in many cases of multiple neuritis in the adult ; but if there is any good reason for the employment of mercury let it be given in the form of inunctions of unguentum hydrargyri or of a ten or twelve per cent, oleate of mercury. The administration of mercury, either by the mouth or in the form of hypodermic injections, is to be avoided in chil- dren, if possible. Iodide of potash or iodide of sodium has little or no influence upon the disease ; arsenic, which has gained such popularity in every form of chronic nerve dis- ease, is scarcely a safe remedy to use, for in small doses it will have no effect, and if given in large doses there is a de- cided danger of increasing the neuritis. We are compelled, therefore, in all cases of multiple neuritis in children, par- ticularly in those occurring after diphtheria and other acute infectious diseases, to resort to general tonic remedies. Among these none is better than cod-liver oil, and next in order, though far inferior to it, are iron, quinine, and strychnia in very small doses. In all cases of malarial neuritis, quinine should be given in the same doses that would be administered to combat other symptoms of mala- rial poisoning. A word of caution should be added with reference to the use of anodynes. In children the careless exhibition of morphia may result in the formation of the morphia habit, and morphia itself, if given in any consid- erable quantities, may help to intensify rather than to diminish the symptoms. If insomnia is persistent in consequence of the pain in the initial stages, one of the newer hypnotics, such as chlor- alamid, trional, or sulphonal, in doses varying between five MULTIPLE NEURITIS. 2$$ and fifteen grains, according to the age of the child, should be given a trial ; if they do not produce sleep, codeia in doses of one-sixth to one-half of a grain may be adminis- tered. After the first two or three weeks of the disease the paralytic symptoms become rather distinct. In all those cases in which there is considerable paralysis with atrophy the use of the galvanic current, both as a sedative and as a means of exercising the paralyzed parts should be em- ployed. The faradic current will be of little service if it fails to produce contractions, and as it has a distinct irritat- ing effect upon the sensory filaments in the skin it should not be applied. When all pain has disappeared and the progress of the disease has been stayed, light massage can be employed to advantage ; it is specially to be recom- mended at the time when contractures are about to be de- veloped, or when the atrophy of the muscles is on the in- crease. If permanent contractures result from multiple neuritis surgical interference may be necessary, but as the disease generally leads to spontaneous recovery, every possible means of bringing this about should be employed before the child is given over to the surgeon ; yet I can see no harm in having a simple tenotomy performed if that will enable the child to bring its feet to the ground, or to walk before complete recovery from all the symptoms has set in. In some of the cases some simple orthopedic ap- paratus will help the child to learn to walk, and will enable it to use muscles which cannot be depended upon to sup- port the body ; but there is also a danger in allowing chil- dren to wear an apparatus too long a time, as they are very prone to depend entirely upon such artificial support, and, as a rule, lack the energy to exercise weakened muscles. DIPHTHERITIC PARALYSIS. Paralysis after diphtheria occurs in a very large percent- age of all the cases, and is relatively more frequent after diphtheria of the adult than after the same disease in earlier life. In earliest infancy, say up to the age of two years, diphtheritic palsy is extremely rare. It bears no absolute 254 THE NERVOUS DISEASES OE CHILDREN. relation to the severity of the diphtheritic infection. 1 have often seen typical diphtheritic palsy set in after an infec- tion so slight that it was considered to be nothing but a mild sore throat ; but, on the other hand, it also occurs in connection with severe diphtheritic infection, and is fre- quently associated with other complicating diseases, such as nephritis and endocarditis, following upon the original disease. The previous health of the child has no distinct bearing upon the development of the palsy, strong and weak children being affected with equal frequency. I have seen the paralysis developed within the first week, while the throat was still covered with diphtheritic membrane, but the majority of cases do not occur until two, three, or four weeks after the termination of the disease. Diphtheritic palsy differs from other forms of multiple neuritis in the order in which the various parts become paralyzed. The palate is generally the first, often the only, part affected. Nasal speech and regurgitation of liquids through the nose are evidences of paralysis of this part, and the loss of function can be determined by an inspection of the mouth and throat. Loss of accommodation is next most frequent. If the paralysis extends further, the upper and lower extremities are apt to be involved. At first a mere Aveakness is noticeable, associated with tingling and numb- ness of the parts; later on this weakness increases, and develops into a full-fledged paralysis, and the disturbance of sensation may become more marked. The ocular mus- cles show a peculiar predisposition to diphtheritic infec- tion ; the rectus externus, supplied by the sixth nerve, is frequently, and often singly, affected, and the oculo-motor nerves are also involved at times, but a complete ocular motor palsy in diphtheria is rare. Ptosis and paralysis of one or more muscles supplied by the third nerve have fre- quently been noticed. The optic nerve fortunately escapes, so that blindness is not a common result of diphtheria, un- less by some untoward accident the diphtheritic poison should cause ulceration or purulent disease of the eye, with subsequent loss of vision. The ciliary muscle is often par- alyzed ; the reaction of the pupils to light is sluggish, but the contraction during accommodation may be preserved, MULTIPLE NEURITIS. 2$$ although the act of accommodation itself is carried out im- perfectly. Paralysis of the pharynx may occur, but is not frequent ; the larynx, however, often comes in for a share of the pa- ralysis ; the epiglottis then fails to perform its function, and food may reach the larynx and cause severe fits of cough- ing. Hoarseness and imperfect phonation point to an in- volvement of the muscles of the larynx and of the vocal cords. This special order of paralysis is generally sym- metrical. The reflexes are diminished or lost, even when no dis- tinct paralysis of the adjacent parts exists or existed (Bern- hardt). I have had opportunity to examine four children whose knee-jerks were absent after diphtheria, but who had never presented any paralytic symptoms. The same condition is found occasionally after scarlatina and typhoid fever. In the limbs the change in the motor and sensory functions is very like that of other cases of multiple neuritis, and does not, thererefore, require any special mention. It is questionable whether cardiac failure coming on after diphtheria is due to a neuritis of the pneumogastric, or whether, as Leyden would have it, it is due to a degeneration of the heart muscle itself. The irregularities of respiration associated with the heart symptoms are so characteristic of vagus affections that it seems much more probable to attrib- ute the loss of function to disease of the nerve rather than of the muscular tissue. Some diphtheritic palsies occur which do not in any way resemble mul- tiple neuritis ; a hemiplegia may follow upon diphtheria, but this may be the result of a vascular lesion so common in connection with other infectious dis- eases of childhood. Cases of this character have been described by Mendel and Bonath. Paralysis of the masseters has been reported by Grant and quoted by Gowers. As the paralysis was permanent fourteen months after the disease Gowers thinks the affection due to an acute nuclear inflammation. A degen- eration of various cranial nerves has been observed to come on after a con- siderable lapse of time, following upon diphtheria, but it has seemed a little doubtful whether such paralysis could be traced directly to a diphtheritic poi- son. The general course of the disease leads to recovery. It may last from six to eight weeks ; the cases in which single nerves only are affected recover more rapidly than those with multiple nerve-lesions. The ocular nerves recover 256 THE NERVOUS DISEASES OF CHILDREN. very much more quickly than the nerves of the lower ex- tremities do. If there is paralysis of the legs, it takes, as a rule, from four to six months before the symptoms disap- pear. The reflexes are late in returning, and in most cases cannot be elicited until some weeks after all other symp- toms have passed away! If death ensues during diphtheritic palsy, it is either due to some compli- cating disease, such as nephritis or pneumonia, or to paralysis of the heart or respiratory muscles. Mere exhaustion from inability to take food in cases of paralysis of the pharynx is an occasional but rare cause of death. Pathological Anatomy. — The morbid changes are essentially the same as those found in other forms of multi- ple neuritis ; the inflammatory and degenerative changes in the nerves are in these cases probably the result of micro- bic poisoning, or the effect of chemical poisons formed by the diphtheritic micro-organisms ; but bacteriological re- searches have not yet yielded a very satisfactory explana- tion of all the changes that take place in the peripheral nerves. The rod-shaped bacteria, the bacilli of Klebs and Loeffler, have been found in the blood-vessels of the nerve- centres, and other micrococci have been described by Oer- tel as occurring in these vessels ; but further study Avill be needed to explain the origin of the inflammatory process in the nerves. The degeneration is found in the nerve supply- ing the paralyzed part ; often the entire nerve is affected, at times some of the filaments only. In others, the degenera- tion extends to the anterior or even the posterior roots. The myelin and nerve-filaments undergo segmentation, the nuclei of the sheath are increased, and granular corpus- cles mark the decay of nerve-tissue. The axis-cylinder remains intact until the destruction of the medullary sheath has advanced considerably, and both these parts may then be entirely destroyed. The interstitial tissue between the nerve-fibres is but little affected, thus marking the condi- tion as a true parenchymatous degeneration. The nerves supplying the palate are most frequently and most severely affected, but the same changes may occur in the peripheral nerves of the extremities in all the ocular nerves, and even the phrenic nerve may be similarly affected. MULTIPLE NEURITIS. 2$J In diphtheritic paralysis the muscular tissue is more distinctly changed than in other forms of paralysis due to multiple neuritis. In cases of long- standing the muscular fibre of the palate, for instance, is found to have under- gone granular and fatty degeneration. In some there are signs of parenchy- matous as well as of interstitial inflammation, and every possible grade may be found between a simple inflammatory condition and complete degenera- tion of the muscular fibres. In cases in which the disease leads to implica- tion of the heart muscle, the pallor of the heart after death is the external evidence of complete degeneration of its muscular fibres. Much has been made of changes in the spinal cord in cases of diphthe- ritic paralysis, but these are restricted entirely to changes of the ganglion cells of the anterior horns, which have been found swollen, homogeneous in character, and with processes somewhat altered or entirely shrunken ; but it is doubtful whether these changes are primary or secondary, though there is no good reason why a poison which acts so vigorously upon the peripheral nerves should not invade the various tracts of the spinal cord. The frequent disease of the palate is probably due to the proximity of the nerves supplying the palate, to the diphtheritic membranes, and the eas- ier invasion into these nerves of the micro-organisms lodged in the tonsils, or in the back of the throat. And yet if we remember that a considerable period of time may elapse between the deposit of the diphtheritic membranes in the throat and the appearance of the palsy, and that the palsy may appear after very mild throat affections, it is not altogether easy to explain why these nerves should be so frequently the seat of the disturbance, unless we suppose that from their proximity to the original diphtheritic lesion the nerve-tissue has become altered and thus rendered more susceptible to the diphtheritic microbes, or to the microbic toxins. The poison attacks other nerves in the course of time ; the peroneus, the musculo-spiral, and the median are the most susceptible to it. The diagnosis of this palsy often depends upon the history of preceding diphtheria, or of some throat affection, however slight it may have been. The beginning of the palsy in the palate, and gradually spreading to the lower and upper extremities, will at once suggest the probability of diphtheritic paralysis. It is only in cases in which the original disease has been overlooked or forgotten that an examination of the patient some weeks after the onset of the trouble may, through the atrophic paralysis and the ab- sence of knee-jerk, suggest spinal infantile palsy ; but a closer examination of the patient, and the comparatively rapid development of the palsy, with its tendency to recov- ery, will place the diagnosis beyond doubt. Diphtheritic palsy, like other forms of paralysis in children, may con- 17 258 THE NERVOUS DISEASES OF CHILDREN. tinue for an inordinate length of time, and may be super- seded by an hysterical palsy. Thus it happens not infre- quently, as in the daughter of a clergyman whom I examined a few years ago, that a chronic paralysis is the outcome of a diphtheritic palsy which had set in a few weeks after a severe throat affection. The prognosis in diphtheritic paralysis is tolerably good, if we except the possibility of cardiac and renal complications. The paralysis of the palate, however dis- agreeable it may be to the child, rarely leads to serious complications, but if the muscles of the pharynx are in- volved there is danger of food entering the respiratory tract, and of a subsequent pneumonia. Other things being equal the earlier the paralysis appears after the initial disease, and the more quickly it reaches an extreme development, the more serious the case and the more imminent the danger to life. It is difficult to forecast the exact length of time which will elapse before complete recovery sets in, but it is safe on the whole to be guided by the same rules as in the case of any other peripheral nerve palsy. If the electrical examination shows an entire loss of response to the faradic current with an altered response to the galvanic current, many weeks will in all probability elapse before recov- ery sets in. If there are no marked changes in the electrical conditions of the nerves and muscles the degeneration or the inflammation of the nerve is very slight and the paralysis will be recovered from more quickly. Treatment. — The majority of cases of diphtheritic palsy will get well without any treatment, but it is of the utmost importance at all times to maintain the strength of the patient at par, and to be prepared for any sudden com- plications. If the palate is paralyzed and there is regur- gitation of food through the nose it will be better to feed the child by solids than by liquids ; if in consequence of paraly- sis of the pharynx and of the upper air-passages there is danger of food entering the respiratory tract it may be best to feed the child for a time per rectum, or else to use the feeding-tube through the nose or the mouth. In the case of children weakened by the diphtheritic process there is danger of death from exhaustion, and no time should be lost in employing this method of feeding rather than to starve the child while hoping for a spontaneous recovery. On the other hand, even while the rectal feeding is kept up MULTIPLE NEURITIS. 259 efforts should be made every now and then to have the child take its food in the natural way. If in any case of diphtheritic palsy there is the slightest irregularity or weak- ness of the pulse, cardiac stimulants should be given, above all digitalis and small doses of strychnia. If respiratory weakness threatens, the prompt use of the faradic current, as advised by Duchenne, is quite in order. Direct excita- tion of the phrenic nerves by a slowly interrupted faradic current will have the best results. The electrical currents should be employed, according to the principles laid down in other chapters, for the treatment of paralyzed parts. The usual tonics — iron, quinine, arsenic, digitalis, and strychnia — should be given, but no one would venture to assert that any one of these has any special curative action except in the way of keeping up the general condition. Indeed there is no better method of treating diphtheritic palsy than by carefully administering food, and making sure that everything taken into the system will help to improve the nutrition of the child.* LEAD PARALYSIS. A few pages are to be devoted to this special form of paralysis, in order to emphasize the fact, which was proved conclusively by Dr. Putnam, that chil- dren are no less susceptible than adults to the effects of lead-poisoning. Dr. Putnam has not only shown that entire families have been poisoned by lead, but in a series of examinations, made for the purpose of determining the presence of lead in the urine of persons who were supposed to be healthy, he found considerable quantities in the urine of father, mother, and two of the children of one family, all of them being in fair health ; a third child, how- ever, for some unknown reason, did not exhibit any traces of the poison. The water drank by this family ran for some distance through lead pipes and was found to contain a large quantity of lead. If present in small quan- tities, lead is practically harmless. Knowing the varied susceptibility of different persons to lead, and having proved its presence in the urine of per- sons who exhibited no symptoms of lead-poisoning, Dr. Putnam is right in urging that the possibility of lead-poisoning should be taken into considera- tion in all obscure cases. As for the symptoms of lead-poisoning in children, they differ but little from those presented by adults, and are in entire keeping with the symptoms * Since the above was written, Behring's important discoveries have been made known. Whether diphtheritic palsy can be affected by injections of the serum remains to be seen ; but it is to be hoped that paralytic symptoms will become less frequent after injections of the serum for the treatment of the earliest stages oi diphtheria. 260 THE NERVOUS DISEASES OF CHILDREN. of multiple neuritis due to other causes. The paralysis is most frequently developed in the extensor groups of the forearm, resulting in characteristic drop-wrist (Fig. 62 ). The muscles affected are supplied chiefly by the mus- culo-spiral nerve ; the supinator longus, and the extensor of the thumb usu- ally escaping. This escape of the supinator longus is frequently of service in helping to distinguish the disease from a simple traumatic musculo-spiraL neuritis, for in the latter case all the muscles supplied by this nerve are about equally affected, whereas lead, like other toxic agents, such as alcohol and arsenic, shows a selective preference for groups of muscles that are function- ally, not structurally, related. In adults it is rare to find other muscles seri- ously affected, except those of the upper extremities, although if careful examination is made of such patients, a slight weakness in the lower extrem- ities and a diminution of the reflexes will be found to be present. I have myself seen two cases of lead palsy in the adult in which the entire affection was restricted to the lower extremities. In one case the adductor muscles of the thighs were the only ones affected. In children the involvement of the lower extremities is not exceptional, and when it does occur, is just as apt as not to occur earliest in the course of the disease, the upper extremities being affected after the lower extremities have been paretic or paralyzed. While the symptoms are practically identical with those of the adult, the diagnosis of lead paralysis will be all the more readily impressed upon the physician by the occurrence of other symptoms. Thus the child is apt to ex- hibit a general cachectic appearance ; also a peculiar ashen hue of the skin , severe headaches, and marked disturbances of digestion. Soltmann expressed the opinion some years ago that lead colic was rare in children. It is more correct to assert that it is difficult to distinguish lead colic from other forms of colic so frequent in children. Disturbance of digestion due to constipation is the rule, but constipation may alternate with diarrhoea and with severe attacks of colic ; the colic being distributed over the entire abdomen and not in the vicinity of the umbilicus, as is generally the case in the adult. Vomit- ing frequently occurs, and the vomitus may be yellowish in color. The ab- domen may be retracted, but in many of the cases one or all of these symp- toms may be absent. If the lead poison attacks the brain, the tendency to convulsions will be very pronounced, and convulsions may possibly be the cause of death. In the cases reported by Karsch and Stewart (quoted by Putnam), the children died in convulsions, one of them with a mild form of delirium. Gowers supposes that epileptic attacks may occasionally originate in lead-poisoning, but Put- nam, whose experience in this matter is greater than that of any other author, is inclined to think that such a sequence of events has not been firmly proven. The lead line, which is of such assistance to us in diagnosticating lead- poisoning in the adult, is not so frequently observed in children, although in one epidemic forty-two cases of the sort were reported. The better condition of the teeth in children is unquestionably the explanation of this. The pathology and pathological anatomy of lead-poisoning are still sub- jects for discussion. That lead acts most powerfully on the peripheral nerves cannot be doubted, and that in the majority of cases lead paralysis is the ex- MULTIPLE NEURITIS. 26 1 pression of a neuritis it is also safe to assume. Gombault insists that lead causes a periaxillary neuritis, the sheath of the nerve being more affected than the axis-cylinder and relatively healthy portions of nerve intervening between diseased parts. These changes were found in guinea-pigs that were not paralyzed, and Putnam suggests very correctly that such changes in the myelin without paralysis may account for the fact that in man the elec- trical reactions are very often altered before any actual paralysis appears. Harnack and others have suggested that lead has a direct action upon the muscles rather than upon the nerves, but the experimental researches of Stieglitz, published a few years ago, have brought the entire discussion to a temporary and satisfactory close by showing that lead produces changes in the spinal cord as well as in the peripheral nerves. Prognosis. — The prognosis of lead-poisoning is, as a rule, entirely favor- able. There is more danger from the general nutritional disturbances and from the effect of the poison upon the brain, than from the lesion of the peripheral nerves and of the spinal cord, which may have given rise to the paralysis. If the cachectic condition is recovered from it is more than likely that the patient will regain considerable power in the legs in the course of a month or two, but the actual time that elapses before complete recovery sets in may vary as much in lead palsy as it does in other forms of multiple neuritis, and it need not be a matter of surprise if a child poisoned by lead shows the effects of such poisoning for six months, or even a year ; but under all conditions it is a source of satisfaction to be able to predict complete re- covery eventually. Treatment. — If the patient is in the acute stage of lead-poisoning the same measures should be employed which are advised in other cases of acute metallic intoxication. If the patient is in a subacute or chronic stage, an at- tempt should be made to eliminate the poison through the kidneys and skin. The iodides have become the classical remedy. It is better to clear the sys- tem of the lead gradually than to attempt to eliminate the poison in a very few days. Doses of from five to ten grains of the iodides three times a day will be quite sufficient for all purposes, and these should be administered in some alkaline water which will stimulate the kidneys to greater activity. Warm baths should be given every day, or every other day, in order to help on the same cause. After everything has been done to eliminate the lead, the attention should be directed entirely to the treatment of the paralyzed parts, and in this re- spect the use of active massage and electricity, in the manner so often re- ferred to, is advisable. The general condition of the patient will also call for the active exhibition of blood tonics, such as iron, arsenic, and cod-liver oil. BIBLIOGRAPHY. Barrs : American Journal of the Medical Sciences, February. 1SS9. Bernhardt: Zeitschr. f. kl. Med., Bd. XL, p. 365. Blocq et Marinesco : Nouvelle Iconographie de la Salpetriere. 1S90. p. 137. 262 THE NERVOUS DISEASES OF CHILDREN. Bonath : Neurolog. Centralblatt, 1893. Bramwell : American Journal of the Medical Sciences, June, 1888. Browning, William : American Journal of the Medical Sciences, December, 1 891. Brooklyn Medical Journal, January, 1893. Brissaud : Arch. d. Neurologie, 1891, p. 161. Buzzard: Monograph, 1885. Eichhorst : Virchow's Arch., Bd. CXXVIII. Eisenlohr : Deutsches Arch, f. klinische Med., 1880. Gombault : Arch, de Physiologie Norm, et Path., 1873, p. 593. Gowers : Multiple Neuritis and Poliomyelitis. Reprint. Grasset et Rauzier : Vol. ii., p. 87. Hirt : Neurol. Centralbl., 1884, p. 21. Jacoby : New York Medical Journal, August, 1889. Joffroy: Archives de Physiologie Norm, et Path., vol. xi., p. 177. Jolly : Bed. Med. Ges., December 7, 1892. Kast : Arch. f. Psychiatric Bd. XI. Klumpke : Des Polyneuritis, These de Paris, 1889. Leyden : Zeitschrift f. kl. Medicin, Bd. I. Lloyd: Medical News, 1892. Meyer : Virchow's Arch., Bd. XCV. Mendel: Neurolog. Centralbl., 1885. Oppenheim: Zeitschr. f. kl. Med., 1885, Bd. XL, p. 232. Pitres et Vaillard : Rev.de Med., 1885,1886. Also, Arch, de Neurolog., 1886. Putnam: In Keating's Cyclopaedia (Lead Palsy). Rosenheim : Arch. f. Psych., Bd. XVIII., 3. Starr: Middleton Goldsmith Lecture, New York Medical Record, 1887. Stieglitz : On Experimental Lead Palsy, Arch. f. Psych., Bd. XXIV. Striimpell : Arch. f. Psych., etc., 1883, Bd. XIV., 2. Berl. kl. Wochen- schrift, August, 1885. Neurolog. Centralbl., 1884. Muenchner Med. W., 1886, No. 34. Zeitschr. f. Nervenheilkunde, 1893. Vierordt : Degeneration d. Goll'schen Straenge, Arch. f. Psych., Bd. XVIII. DISEASES OF THE SPINAL CORD. CHAPTER XIV. ANATOMY, PHYSIOLOGY, AND PATHOLOGY OF THE SPINAL CORD. In this chapter it is proposed to give those facts merely which have an important bearing upon clinical diagnosis. Many details of the normal and morbid anatomy of the cord will, therefore, be omitted, however great the scientific in- terest attaching to tfiem may be. Structure of the Cord. — The spinal cord cannot be differentiated from the brain by any sharp line of demarca- tion. As a matter of convenience the former is said to be- gin below the decussation of the pyramids in the medulla ; but the medulla and pons are practically the extension of the spinal-cord structures, rather than integral parts of the brain proper. In-structure, in function, and in their relation to disease, the pons and medulla incline very much more toward the spinal cord than toward the brain. For all practical purposes, however, we may adhere to the accus- tomed plan of division, and may hold that the spinal cord begins back of the metencephalon, that is, below the part which gives rise to the medulla, and comprises all of the neural axis from this point to the filum terminale. The spinal cord of the child is not sufficiently distinct from that of the adult to call for any special discussion. The points which we wish to bring- out would, therefore, apply with equal force to the spinal cord of adult man. the difference in size and thickness remaining proportionate to similar differ- ences in the bony parts surrounding the cord. The only points to be noted are the following : In the foetus the cord at first occupies the entire length of the vertebral canal, but from the third month on, the roots of the lumbar and sacral nerves grow much more rap- 264 THE NERVOUS DISEASES OF CHILDREN. ci idly than the cord itself, so that at birth the lowest part of the cord is oppo- site to the third lumbar vertebra. After birth, the cervical and dorsal portions of the cord grow more rapidly than the other parts, so that in the infant the roots of the lower ! c dorsal nerves come off relatively higher than at a • 2 later age (Schaefer and Fitzner). • 3 The spinal cord is not as long as the spinal 4 column ; its lowest portion is generally taken to - 5 be situated opposite to the second lumbar verte- 6 bra. For that reason a given spinal-cord seg- ■ 7 ment is a little higher than the vertebral body • s through which the roots of any given segment •I D pass. Still more confusion is added thereto by •a the fact that the vertebral spines, which alone can . 3 be felt through the skin, are not always on the 4 same level with the vertebras. Gowers has made a careful study of these relations, and the exact correspondence of the various parts is well dem- onstrated in the annexed figure (64). This rela- 1 tion between the spinal vertebras and the seg- - 8 ments underneath has become a matter of much practical importance since operable diseases of the spinal cord have compelled the physician * 10 and the surgeon to localize disease of the spinal -" cord as accurately as it has long been their cus- . I2 torn to do with regard to many diseases of the brain. THE NERVOUS DISEASES OE CHILDREN. sels of the spinal cord. The author concludes that a condi- tion of irritation is present in the walls of the blood-vessels which leads to a dilatation of these vessels and to a prolif- eration of their endothelial elements. From this the morbid process extends to the neuroglia and produces a prolifera- tion of its cells. The changes in the ganglion cells are of a Fig. tj. — Poliomyelitis Anterior ; Part of an Acute Myelitis. Death of child (age, two and a half years) eight days after onset of complete palsy of legs and arms. (Sie- merling.) Section through lumbar segment, showing disruption of anterior gray matter from hemorrhage into it. B, marginal blood-vessel ; A, branch of anterior spinal artery. degenerative nature, and the changes in them, as well as in the nerve-fibres, are secondary and due to disease of the blood-vessels. Goldscheider has also shown that these de- generative changes occur most distinctly in the vicinity of INFANTILE SPINAL PARALYSIS. 30E altered blood-vessels, and that the degenerated ganglion cells lie in vascular areas. The cases reported by Siemerling, while corroborating the views of Goldscheider and others, have a still deeper significance. They show that in some instances a poliomy- elitis anterior is merely a part of a general myelitis of both the gray and the white matter. The frequent restriction of the process to the anterior cornua must be ascribed alto- gether to the peculiarities of arterial blood supply. It is Fig. 78. — Poliomyelitis Anterior of Old Standing, showing Disappearance of Ganglion Cells on Right Side, and Shrinkage of Right Half of Cord. Drawn from a section through lower cervical region. doubtful, however, whether the hemorrhage into the an- terior horns is often as destructive as in one of Siemerling's cases (Fig. yj). All these investigations prove that in in- fantile spinal palsy the inflammatory process is interstitial not parenchymatous. It may be limited to a few segments of the cord, or it may involve the greater part of the cord, and may extend to the medulla and pons. For the changes that occur in consequence of this early inflammation, and for the appearance of the cord in the later 302 THE NERVOUS DISEASES OF CHILDREN. years, we may refer safely enough to the famous publica- tions of Charcot and others. There is, first of all, a disappearance, often complete, of the larger ganglion cells of the anterior horns, and the few that are visible are altered in appearance. The nucleus has disappeared, the cell-body is shrunken, and the cell con- tours are entirely different from the normal, every trace of the cell processes having disappeared. But the ganglion cells are not the only parts that undergo changes ; the en- tire gray matter shrinks, and, as can be seen in the annexed cut, the entire gray matter in one-half of the spinal cord is shrunken in size, and the white matter of the same side is also very much less in volume than that of the opposite half. In consequence of the changes going on in the gray matter, the columns of Clarke dis- appear, together with other nervous struct- ures. These changes are unquestionably sec- ondary to the changes in the anterior horns, and considering the intimate relation between the anterior horns and the remaining part of the gray matter, as has been brought to light by recent anatomical investigations, we can readily understand why, in consequence of disease in one part of the gray matter causing destruction of nerve- cells, the nerve-fibres which owe their life and nutrition to such cells disappear as well. The anterior nerve-roots are smaller than the corresponding roots of the sound side. These changes are also, in all probability, secondary to the changes in the ganglion cells. So much for the changes to be observed in the spinal cord itself. The atrophied muscles also present character- istic conditions. The fibres are very much diminished in size, many of them have disappeared altogether, and the place Fig. 79. — Poliomyelitis Anterior. Chronic stage ; section through sixth cervical segment, showing diminution of anterior gray matter and of en- tire half of right side. (Drawn from a specimen kindly furnished me by Dr. Collins. ) INFANTILE SPINAL PARALYSIS. 303 once occupied by the normal fibres is largely filled by adi- pose tissue. There is in these cases no such nuclear prolif- eration and no hypertrophy of fibres such as are found in the muscles of patients suffering from various forms of muscular dystrophy. But even the presence of a few hypertrophied fibres would not be unusual, as the stage of hypertrophy seems to indicate an incipient irritation which precedes the condition of atrophy. Marie has gone to some trouble to show that even the bones in cases of poliomye- litis undergo trophic changes. The bones are smaller than those of the corresponding healthy member and appear more rounded on cross-section than the healthy bone does. Theory of the Disease. — That poliomyelitis repre- sents an acute inflammatory condition of the anterior gray matter of the spinal cord is conceded on all sides, but the question arises what the origin of such inflammation may be. The only satisfactory explanation at the present day is to suppose that the inflammation is the result of an acute infection which happens to be located in the spinal cord, just as other acute infectious diseases show a predilection for other sites in the body. The microbic origin has not yet been satisfactorily demonstrated, but all the clinical facts point toward this view, and the close dependence of the myelitic process upon the distribution of the blood-ves- sels lends further color to this theory. The infectious ori- gin of poliomyelitis is also rendered highly probable by the frequent observation of the epidemic occurrence of infantile spinal paralysis. Such epidemics have been recorded by Medin and Briegleb in Europe, and by Colmer (1843) anQ " Caverly (1894) in this country. For several years past I have recorded carefully the cases of poliomyelitis in dispensary and private practice, and have noticed that at least 75 per cent, began between the months of July and October. Medin observed rive cases in the spring of one year, and between August and November he had examined altogether forty-four cases of poliomyelitis which had begun during this period. Sinkler states that of 270 cases 213, or 78.8 per cent., were at- tacked in the hot months of the year, from May to September, inclusive. Marie supposes that an infectious embolism or thrombo- sis in one or more of the branches of the anterior spinal 304 THE NERVOUS DISEASES OF CHILDREN, artery may be the direct cause of the attack of poliomye- litis. Marie is inclined also to infer the infectious nature of poliomyelitis from the close resemblance between polioencephalitis and poliomyelitis, and quotes approvingly the two cases of Moebius occurring in one family, in which one child was attacked with the form of acute cerebral palsy, and the other child with an acute spinal palsy. But surely this proof of the infectious theory of acute cerebral palsy is extremely slender, and Moebius's cases might well be due to a coincidence rather than to an infection, which is supposed to have caused a cerebral paralysis in the one, and a spinal paralysis in the other child. Nor can two cases occurring in the same family be considered evi- dence of an epidemic character of the disease. A few. years ago two chil- dren, cousins, were brought to me, who had developed acute spinal palsy within two weeks of one another. The one child has remained severely paralyzed in both lower extremities up to the present day, the other child escaped with a slight paralysis of the anterior tibial group. The theory of infection would be a very simple one to hold in such cases, but on closer examination it was found that both these children had developed the symp- toms of their disease shortly after exposure to an extremely cold surf bath. The infectious theory of poliomyelitis is a very plausible one, but we cannot disregard other possible causes, and refrigeration, as in the two cases just cited, may in some instances be a powerful factor in the development of poliomyelitis. It seems to me that those authors make a mistake who insist on a single origin for such a frequent disease as poliomyelitis. Many, pos- sibly most, cases may be due to an infectious cause, but some may be due to refrigeration, others to slight traumatic injuries. Marie is right in holding- that dentition is never the actual cause of poliomyelitis, but during the period of dentition other influences may bring about the disease at a time when the spinal cord, as well as the general nervous system, is in a condition of extreme irritability. What we have just said regarding the theory of the disease also includes all that can safely be stated regarding the etiology. Differential Diagnosis. — Poliomyelitis is most fre- quently confounded, during its acute stage, with acute cere- bral conditions, such as meningitis or some form of acute cerebral palsy. Meningitis can be safely excluded if there are no other signs of a meningeal process, except possibly coma and convulsions. In cases of meningitis these consti- tute the first of a series of many cerebral symptoms, such as vomiting, rigidity of the neck, headaches, cranial nerve affections, and the like. In poliomyelitis none of these symptoms appear, and the coma and convulsions last but a relatively short time. There is little difficulty in distinguishing between well- INFANTILE SPINAL PARALYSIS. 305 developed cases of acute spinal and acute cerebral palsy of children, but the less pronounced types of these diseases cannot be easily distinguished from one another unless a very careful examination is, made of all the accompanying symptoms. The mode of onset may be exactly similar in both ; it is, in fact, on the close resemblance between the two diseases in this respect that Striimpell was led to build up his theory of the analogy between the two. But aside from the symptoms of onset, the clinical features are almost diametrically opposed to one another. The follow- ing table will bring out these symptoms in the clearest pos- sible manner: Acute Spinal Palsy. Acute Cerebral Palsy. Onset sudden, with fever, coma, and Onset sudden, with fever, coma, and convulsions. Convulsions rarely convulsions. Convulsions apt to be repeated after first few days. repeated. Paralysis flaccid, associated with Paralysis spastic ; no atrophy ; asso- atrophy. ciated with rigidity and contrac- tures. Paralysis widely distributed, possibly Paralysis generally hemiplegic, some- involving all extremities, or nar- times diplegic or paraplegic. Mono- rowly limited to one member, or plegia rare, even a single group of muscles. Electrical reactions altered (R. D.). Electrical reaction normal. Deep reflexes diminished or lost. Deep reflexes exaggerated. Intellect never permanently involved ; Intellect often involved ; epilepsy fre- no epilepsy. quent. Doubt may arise as to the differential diagnosis in some cases between poliomyelitis anterior and a peripheral (mul- tiple or simple) neuritis. The onset may be equally sudden in both, though in many cases of neuritis the onset is much more gradual than it is in cases of poliomyelitis. In neu- ritis there are, as a rule, fewer symptoms of general ner- vous disturbance than in poliomyelitis ; but in those forms of neuritis in which there is a distinct toxic infection the toxic poisoning may produce cerebral symptoms very closely akin to those met with in the earlier stages of polio- myelitis. The distribution of the paralysis may be the same in both instances, but after all it is much more likely to be distributed according to strict anatomical lines in 306 THE NERVOUS DISEASES OF CHILDREN. neuritis than in poliomyelitis. In the latter, muscles that have a common function are very apt to be paralyzed to- g-ether. In former days the presence of pain along nerve- trunks and along nerve-branches supplying the paralyzed muscles was supposed to be a safe feature of differential diagnosis, and this holds good in a majority of cases ; but according to my own experience pain may be present in the acute stage of poliomyelitis, and if the child is too young to give accurate information to the physician, it is well- nigh impossible to determine whether the pain is a gen- eral one in the joints or whether it is along the distribution of the peripheral nerve-branches. But 1 have never seen pain persist for any great length of time in poliomyelitis, while it persists, as a rule, for days and weeks in cases of neuritis. The atrophy, the electrical reactions, and the re- flexes may be as thoroughly affected in one disease as in the other. The differential diagnosis can, in many in- stances, be made only after a close observation of the en- tire course the disease has taken. (See Chapter on Multi- ple Neuritis.) The various forms of progressive muscular dystrophies may occasionally be mistaken for poliomyelitis and vice versa. In cases that are seen years after the onset of the trouble, the initial history of the case will often be an important guide to diagnosis. If a case of typical mus- cular dystrophy is seen during the stage of atrophy, a su- perficial inspection of the case may suggest an old polio- myelitis ; but in the progressive dystrophies the atrophy affects an entire limb rather than single groups, or if it has spread to several extremities it is, as a rule, much more general than in cases of poliomyelitis, while in the latter the electrical changes are, as a rule, much more complete than in cases of progressive dystrophies. One special form of progressive muscular atrophy is easily confounded with the chronic stages of acute or subacute poliomyelitis; I refer to the peroneal form of progressive muscular atrophy, the Charcot-Marie type, cases of which have been recorded by Tooth, Hoffmann, myself, and others. This atrophy, beginning in the peroneal group of muscles, may for a time simulate a poliomyelitis involving this same group ; but the upward march of the disease in the case of progressive muscular atrophy, the progression INFANTILE SPINAL PARALYSIS. 2>°7 rather than retrogression, the involvement of the upper extremities, the strictly bilateral character of the atrophy and the slow development, the incomplete changes in electrical reactions, will help to distinguish the peroneal form of progressive muscular atrophy from poliomyelitis ; but the differential diag- nosis requires the most careful examination, and in some instances the hered- itary or family taint, in cases of progressive muscular atrophy, will give the correct clew to the nature of the disease. Prognosis. — Altogether too gloomy a prognosis is gen- erally given in poliomyelitis. This is based upon the fact that some palsy always remains, but the actual res- idue of palsy may be so slight that one should be care- ful not to depress the hopes of parents and patient. Above all, there is no need of predicting that the child will re- main a hopeless cripple for life. There is no telling at the outset of the disease to what extent the retrogression may take place ; but, of course, the more widely distributed the paralysis is at the beginning the larger the remaining palsy is apt to be, although some cases which begin in very stormy fashion exhibit more progress than those which be- gin less violently. Cases in which but a few muscles are paralyzed at the start often recover with very little per. manent injury. Very little change need be expected in the first few weeks of the disease, but there is reason to hope that those parts which show any improvement within the first few weeks or months after the onset of the disease will recover power before long, and only those parts will remain permanently paralyzed which after months show no signs of improvement. Muscles which are paralyzed, but which exhibit slight, or no changes of electrical reaction, may be regarded in a hopeful light, and, on the other hand, those which very soon after the onset of the palsy exhibit distinct reaction of defeneration, and for months afterward show no sign of change in this respect, are apt to be per- manently paralyzed. The more complete the wasting of the muscles, the less likely these muscles are to recover ; and if contractures form in the opposing groups permanent disability is the probable result, but even such disability can often be remedied by surgical procedures. The prognosis as regards life is, with few exceptions, en- tirely favorable. Cases that end fatally are apt to do sc-> 308 THE NERVOUS DISEASES OF CHILDREN. within the first few weeks of the disease. But parents are often most grateful for the assurance that if the child sur- vives, however great the paralysis may be, its mental de- velopment will in nowise be impaired. This assurance can only be given if the physician is certain that the palsy is due to a spinal lesion and is not a form of cerebral paralysis. Treatment. — During the acute stage of an anterior poliomyelitis the general condition only should be treated and little attention need be paid to the paralysis. The child should be kept in a quiet room, mild antipyretic measures may be employed, such as small doses of phenacetin, of anti- pyrin, of the salicylates, and the like. In the earlier stages cold applications, or mild counter-irritation over that part of the spine which is involved in the given case, are quite in order ; and the attempt should be made to limit the spread of the inflammation by the administration of small doses of bromide and of ergot (a few drops of the fluid extract). Iodides and other drugs I have found to be utterly ineffi- cient. The child should during this period be carefully fed, and the bowels should be thorougly purged by the use of small but sufficient doses of calomel. After the acute stage is passed the paralyzed muscles demand treatment. Elec- tricity and massage are the most effective therapeutic measures. Avoid electrization of the spine ; first, because it is not at all certain that the electric current reaches the spinal cord, and secondly, because the use of strong cur- rents makes the child extremely restless and may do more harm than good. In the treatment of paralyzed muscles an important use of the electric current is to exercise muscles which are no longer subject to the will ; it supplies, in other words, a convenient form of gymnastics. The cur- rent may, in addition, improve the state of the paralyzed and atrophied muscles, but whether or not it increases the conductive powers of paralyzed nerve and muscle I am not willing to assert. Since we wish to make the muscles con- tract, the only form of current that is serviceable is that form to which the muscle will respond. If the reaction of degeneration is complete the faradic current is quite useless, and if the diagnostic tests have shown that the muscles re- spond to the anode better than to the cathode, exercise by INFANTILE SPINAL PARALYSIS. 3 00. anodal opening or closing of the current is the only proper method. This should be done in sittings of ten to fifteen minutes once or twice a day, and that strength of current should be employed which is sufficient to produce mild contractions. Excessive contractions are not called for and help to increase the difficulties of application. If a muscle responds to the faradic current, however slightly, that current should be employed together with the galvanic, and it has been my habit, even in the more severely par- alyzed cases of poliomyelitis, to make occasional tests with the faradic cur- rent, first, in order to determine whether there is any sign of improvement in any group of muscles that have been paralyzed, and, secondly, to give such muscles the benefit of both currents. If the muscles react at all to both cur- rents, both may be employed in one and the same sitting, or given in alter- nate sittings. I am a thorough believer in the good influence that massage has upon atrophied and palsied muscles. It helps undoubtedly to keep up the nutrition of such parts, and in cases in which there is an incipient ten- dency to contracture such tendency may be overcome by the proper use of massage. But this should, if possible, be intrusted to skilled manipulators and not to the mother of the child or to a nurse, whose " rubbings " are, as a rule, wholly ineffective. Passive movements are entirely in order, and some good results have been attained by the regular use of Swedish movements, such as are given by trained rubbers or by a regular system of treatment in a well-equipped Zander institute. Unfortunately such institutes are for the present found only in a few large cities. During the chronic stages of poliomyelitis orthopedic measures should be employed without reserve. If the con- tractures have persisted for years they will not disappear without treatment. Tenotomies are followed by results fully as favorable as those in chronic ocular palsies. The general condition of the child and the condition of the paralyzed muscles improves markedly after the orthopedic surgeon has done his work, and such tenotomies need not be restricted to the tendon Achillis, but many of the other muscles which are in a state of contracture can as well be similarly treated. The application of splints according to the best orthopedic principles is also of great assistance to the child, and this, too, should not be delayed too long, for it is far better to have a child walk in splints than to have ugly deformities of the joints develop which may cripple him for life. Since I have recognized the truth of these principles I have had the satisfaction of seeing children, 310 THE NERVOUS DISEASES OF CHILDREN. and even very young children, walk within a few months after the development of a poliomyelitis, while in former years such children were compelled to be carried about in the arms of a nurse or to be wheeled about in chairs. I am satisfied that few cases of poliomyelitis are so severe that much cannot be clone by the proper application of ortho- pedic measures. In cases in which the joints are entirely use- less on account of the complete atrophy of the muscles, the operation for arthrodesis, as suggested by Wolff and others, may be resorted to. In this way a leg that would other- wise be entirely useless may be made to subserve the func- tion of standing and walking, though of course the station and the gait of the person will always be far from normal. At every stage of poliomyelitis active interference and active treatment are called for, and much harm and misery may be avoided if the physician, instead of quietly sitting by and saying that nothing can be done, will exercise his own mechanical ingenuity in every case to put the limb in the best possible condition for walking, whether such is to be attained by the use of splints or by one of the several or- thopedic measures that have been suggested. SUBACUTE ANTERIOR POLIOMYELITIS. Subacute anterior poliomyelitis is practically a mere variety of the acute form of the disease, and for that reason needs but little special mention. The entire difference between the two diseases is in the mode of onset, and ac- cording to our present views of the character and origin of acute spinal palsy, the subacute variety necessarily implies a milder form of infection than in the cases with a more acute and more violent beginning. The difference in the symptoms is also confined entirely to the difference in the manner of onset and the manner in which the paralysis is developed. In these cases of the subacute variety we find that the disease comes on very gradually. The child is ill at ease for some days or weeks, complains of weariness in walking, of pains in the joints and muscles. After some little while a decided paresis of one or more groups of muscles is observed, generally in the lower extremi- ties. This increases, and after a week or more a distinct paralysis is devel- oped. The paralysis then increases in the affected muscles, and is apt to spread somewhat after the fashion of a progressive muscular atrophy from one group of muscles to another. There is, therefore, a progression in these cases at the start, but the limit is very soon reached, and from this time on a retrogression again sets in, though not in such a marked degree as in the acute cases. The fact that the retrogression occurs after a given period of INFANTILE SPINAL PARALYSIS. 311 time will dispel all fears as regards the possibility of a progressive form of muscular atrophy. As a good illustration of this type, I may refer to a young girl who has been under my observation for many years. When the disease began she was thirteen years of age. A slight difficulty in the use of the toes was the first symptom the girl complained of, with the exception of a general weakness and an inability to walk long distances. From the exten- sors of the toes this spread to the tibialis anticus, and to the other muscles of the anterior tibial group. After a little the anterior thigh muscles, too, be- came involved, so that the girl was totally paralyzed for a period of several months. There was no history of fever at any time, or of any other symp- tom of an acute onset. The disease then came to a standstill ; the anterior thigh muscles recovered completely, and the paralysis is now, after the lapse of four years, entirely restricted to the tibialis anticus, and the extensor mus- cles of the toes. But even in the tibialis anticus the electrical reactions are quite normal, and the marked symptoms of reaction of degeneration are present only in the extensor muscles of the toes, which were those first affected. The knee-jerk, which was absent for many years, can now be elicited by Jendrassik's method, and the girl, who was once unable to use the limb at all, is now able to walk with but a slight halt in her gait. The differential diagnosis of such cases is often difficult, and cases are confounded easily with cases of progressive muscular atrophy, particularly of the peroneal form, and with cases of chronic neuritis ; but the points of dif- ferential diagnosis which were given between these diseases and the acute form of anterior poliomyelitis w r ill also help us in arriving at a correct diagno- sis of the subacute variety. Treatment. — The treatment should be conducted on the same princi- ples as were enunciated in the preceding discussion on acute spinal palsy. In those cases in which the original disease is not nearly so violent as in the acute form even more can be hoped from an early application of therapeutic measures, but it should be remembered that the natural course of the disease tends much more to recovery than does the acute form, so that tenotomies and other surgical procedures should be delayed until the disease has become entirely stationary, and there is no reason to think that further spontaneous recovery will take place. BIBLIOGRAPHY. Briegleb : Inaugural Dissertation. Jena, 1890. Caverly : New York Medical Record, 1894, vol. xlvi., p. 673. Charcot et Joffroy : Arch, de Physiologie, Paris, 1870, p. 134. Collins, J. : New York Medical Journal, January, 1894. Colmer, G. : American Journal of the Medical Sciences. 1843. Dauber : Zeitschrift f. Nervenheilkunde, vol. iv. Goldscheider : Ztschr. f. kl. Med., vol. xxiii., 1893, p. 494. Jacobi, Mary P.-: Article in Pepper's System. Kahlden, C. Von : In Ziegler's Beitrage, 1893, vol. xiii. Centralbl. fur Path. September 14, 1894. 312 THE NERVOUS DISEASES OF CHILDREN. Leyden : Arch. f. Psychiatrie, vol. vi., p. 271. Marie, P. : Maladies de la Moelle. Paris, 1892. Medin : Proceedings of Tenth International Congress, vol. ii., div. vi. Roger etDamaschino : Comptes Rend, de la Soc. d. Biologie, 1871. Schultze : Neurolog. Centralbl, 1892, No. 19. Virchow's Arch., vol. lxviii. Seeligmiiller : Gerhardt's Handbuch, vol. v. Siemerling : Arch. f. Psychiatrie, vol. xxvi., p. 267. (Full literature to 1894.) Sinkler : Keating's Cyclopaedia, p. 683. Volkmann : Volkmann's Sammlung No. 1. Wolff, J.: Berl. kl. Wochenschr., 1886, No. 52. CHAPTER XVII. ACUTE MYELITIS. Myelitis, or inflammation of the spinal cord, has been made to cover a multitude of diagnostic sins, both in the adult and in the child. If we subdivide the cases accord- ing to the mode of onset, we may distinguish between an acute, a subacute, and a chronic form ; and if the classifica- tien is based on the origin of the myelitis we have an idio- pathic form, a traumatic myelitis, a tubercular, and a syphi- litic variety. As the symptoms are very much the same, whatever the original cause of the disease may be, it will be better to describe the characteristic features of acute myelitis, which occurs frequently enough in children to de- mand special study. Symptoms. — The symptoms in a given case will vary ac- cording to the site of the inflammation, and the intensity of the process. Its clinical features will depend upon the amount of cord tissue involved. In such cases more than in any others an accurate knowledge of the functions con- nected with each segment of the cord is of importance if a satisfactory diagnosis is to be made. In every case of com- plete transverse myelitis, at whatever level the area of in- flammation may be, motion and sensation are chiefly af- fected, the reflexes are disturbed, and the functions of the bladder and rectum are deranged. Bilateral paralysis (para- plegia) is the natural result of a myelitis. If the lesion is in the cervical portion of the cord, both upper and both lower extremities will be paralyzed ; the first because the very parts which are most intimately connected with the motion of the upper extremities are destroyed ; and the lower ex- tremities are involved because the fibres going to them are interrupted at the site of the lesion. The bilateral character 3H THE NERVOUS DISEASES OE CHILDREN, of a palsy, is after all, the one symptom which points more frequently to a spinal lesion than any other. Almost the only exceptions to this rule are those cases in which a multi- ple neuritis, an ascending Landry's paralysis, or double cerebral lesions give rise to a bilateral form of palsy. If the lesion is in the cervical portion of the spinal cord, the paralysis of the upper extremities will be of a flaccid order; the paralysis of the lower extremi- ties will be spastic in character. Anaesthesia will be present in the four extremities and in the trunk to the level of the diseased seg- ments ; pupillary symptoms, Unilateral blush- ing (due to lesion of the sympathetic), and paralysis of the diaphragm are present in some cases. If the lesion is in the lumbar portion of the cord, the paralysis is re- stricted to the lower extremities and will be of a flaccid character, with more or less atrophy. If the lesion is in the cervical or dorsal portion of the cord, and the lumbar portion is entirely free from disease, the paraplegia of the lower extremities is of the spastic order, and the reason of this can be easily understood if we recall the fact that after a transverse lesion in any por- tion of the spinal cord the lateral columns will degenerate downward from that level (Fig. 80), and that such degeneration of the lateral columns in the presence of normal gray matter of the lumbar segments will produce a spastic form of paralysis with rigidities and contractures. Taking all cases of myelitis, the largest number affect the dorsal re- gion \ and in these the arms go free, as their spinal centres are above the site of the lesion, but the lumbar segments are affected by secondary degeneration. Sensation is impaired at a very early period of the dis- ease, often from the first moment of onset. In fact, in the cases of traumatic origin loss of power and loss of sensa- Fig. 80. — Seconda- ry Ascending and Descending De- generation follow- ing a Transverse Lesion in the Up- per Dorsal Cord. (Struempell. ) ACUTE MYELITIS. 315 tion are effected almost instantaneously, so that a person thus afflicted feels not only that his legs are powerless, but that they appear to be dead as well. Anaesthesia exists in all the parts that are supplied by nerves coming off be- low the site of the spinal lesion, and in the typical cases all forms of sensation are equally involved. Thus we gener- ally find that a spastic paraplegia is associated with the loss of touch sense, of pain sense, of thermal sense, and of muscular sense in the affected parts. As the anaesthesia is strictly dependent upon the conduction of impulses inward through the posterior roots of the cord, the extent of the anaesthetic area will naturally give us a sufficient clew as to the upper limit of disease in the spinal cord. (See pages 277 and 278.) At the upper limit of the anaesthetic area a small zone of hyperaesthesia, as a rule, begins. This is evidence of the fact that in the segment supplying the hy- peraesthetic area there is a condition of irritation affecting the posterior spinal root-fibres, but not the chief morbid and destructive process. Above the hyperaesthetic area sensa- tion may be expected to be entirely normal. In other cases, instead of a distinct hyperaesthesia we have a girdle sensa- tion, which also marks the level between the normal and diseased segments. The state of the reflexes helps us also to determine the area involved. If the lesion is in the cervical region all the reflexes of the upper extremities are destroyed, those in parts below will be exaggerated. If the lesion is in the dorsal region the reflexes connected with these segments, such as the abdominal and epigastric reflexes, will be lost and the lower reflexes will be increased. If the lesion is in the lumbar region the knee-jerk will be lost and the ankle clonus will be absent also. In some cases in which there is a very narrow band of inflammation these reflexes may behave differently and may give one a direct clew as to the exact extent of spinal inflammation. Thus in one case un- der my observation the knee-jerk was lost, but the ankle clonus was present. This, taken in conjunction with a line of anaesthesia showing an involvement of the lower dorsal segment, proved that the upper lumbar region was slightly involved, but that the lower lumbar and sacral segments 3l6 THE NERVOUS DISEASES OF CHILDREN. were not directly implicated by the disease. Since dorsal myelitis is the most frequent form, it is also common to have exaggeration of the reflexes associated with spastic contractures of the legs. The electrical reactions will vary according to the seg- ments involved. In cases of cervical lesion the reaction of degeneration will be found present in many, if not all of the muscles of the upper extremities, at least after the lapse of a few days or weeks ; those in the lower extremities will remain unaltered. But if the lesion is in the lumbar seg- ments the reaction of degeneration will be present in the muscles supplied by nerves coming off from the diseased area. In cases of cervical and dorsal myelitis we may, therefore, expect entirely normal electrical reactions in the lower extremities, however thoroughly paralyzed these parts may be. A few other symptoms must be noted which are ex- tremely characteristic of acute myelitis and often serve to reveal the disease when other symptoms in the case have left the diagnosis in doubt. In all such cases of myelitis, in whatever region the lesion may be, the vesical and rectal reflexes are disturbed or completely abolished. The re- sult of this is retention of urine, with possible overflow, or constant dribbling of urine, and either retention of stool or involuntary defecation. Since the centres for these reflexes are in the lowest portion of the cord, and every part of the cord must be intact if such sensation is to be conducted to the higher centres, we can understand why these symptoms should be present, whether the lesion be in the lumbar, in the dorsal, or in the cervical segments. Loss of sexual function is hardly to be mentioned in cases of myelitis in children ; but priapism, due to irritation of the spinal sexual centre, is not infrequently present in children, and is some- times a very annoying symptom. Involuntary spasmodic twitchings occur in nearly every form of myelitis. I know of no symptom which is on the whole more characteristic of spinal lesions, and which often serves as a differential symptom between supposed functional and spinal paralysis. This spasmodic cramp is evidently due to an irritation of the normal ganglion cells, and the irritability is often so ACUTE MYELITIS. 317 great that the mildest form of sensory impulse is sufficient to elicit such involuntary spasms. Under these circum- stances the mere touch of the paralyzed part — of a toe, for instance — is sufficient to produce contraction of the entire limb. Trophic disturbances are exceedingly common and much to be feared. Bed-sores are easily developed in all parts on which pressure is exerted : under the shoulder- blades, over the sacrum, on the hips, and even over the internal malleoli or on the inner surfaces of the knees and thighs — in short, wherever parts touch, are pressed upon, or are pressed against each other. In the sacral region the constant wetting of the bed and the uncleanliness of the patient may increase the danger and size of bed-sores ; and while it is true that such bed-sores may occur even without any external irritation, they are greatly aggravated by the dribbling of urine or the involuntary evacuation of the bowels. These bed-sores begin, as a rule, as a mere redden- ing of the skin ; the epidermis is soon worn away, the cutis is bared, this too disappears, and gradually the ulcer may eat away all the subjacent parts until the bone itself is laid bare, provided the patient lives a sufficient period of time. Every form of acute myelitis may be accompanied by fever, which may vary between ioo° and 104 F. and higher. The fever is unquestionably due to the myelitic process, but is often increased, and sometimes maintained altogether, by the complicating conditions of myelitis. Such compli- cations are deep bed-sores with the absorption of putrid matter and the danger of phlebitis ; furthermore, the oc- currence of cystitis and pyelonephritis, which are not un- common. In cases of myelitis, in which fever suddenly increases, with chills, with deep remissions and sudden ex- acerbations, the probability of this fever being due to some pysemic process is very great indeed. As soon as the dis- ease has passed the acute stage the fever lessens and the temperature will remain entirely normal until some com- plicating condition is established. Whenever a majority of the above symptoms are pres- ent, the diagnosis of an acute (transverse) myelitis can safely 318 THE NERVOUS DISEASES OF CHILDREN. be made. The modification of the symptoms if the mye- litis is not complete, or if it is subacute and chronic can be easily inferred from the preceding account. The question of greatest interest, in every case, is to determine what the origin of the myelitis may be. Idiopathic myelitis is, ac- cording to our present notions, scarcely conceivable, and here, as in so many other instances, it is better to say mye- litis from unknown cause than to concede that in some cases the origin is truly spontaneous. In cases occurring in children, without known cause, I am inclined to suspect slight traumatism. A child of eight years, a healthy, beautiful girl, while walking on a coun- try road, had a desire to urinate. Her mother urged her to do so on the road. The child hurried to one side, and in attempting to place its right foot on a stone not more than a few inches in height, lost its bal- ance and struck on the middle of the back. The child experienced pain at once, but was able to walk some little distance ; soon the power of its legs di- minished; it had to be carried, was put to bed, and within twenty-four hours had developed a most pronounced form of acute transverse myelitis. After three days anaesthesia was complete up to the umbilicus. The paralysis was absolute in the lower extremities. There was retention of urine and faeces, bed-sores were developed, and the child died from these complicating conditions within three weeks after the accident. In other cases traumatic injury is much more severe, and a complete destruction of the cord may be the result of in- jury to the spinal column. From the effect of concussion alone, without actual destruction of the bony parts surround- ing the cord, an acute myelitis may result. The myelitis which occurs in connection with tumors of the cord, with tubercular affections of the meninges, need not be separately considered, as it constitutes merely a part of the more seri- ous disease. The two forms of myelitis which are most common in children are those due to Pott's disease and to syphilitic infection ; but both these forms are so distinct and so important that they deserve special consideration. Acute myelitis does, however, occur in connection with other acute infectious diseases, such as typhoid, scar- let fever, small-pox, and the like. Some of these cases of supposed myelitis have probably been cases of multiple neuritis. Rheumatic or atmospheric influences (refrigera- ACUTE MYELITIS. 319 tion), as a direct cause of myelitis in the child and in early youth, should be considered duly. A young girl, of about sixteen years of age, was brought to me from the South, with the following history : On a very warm day in early spring she had taken a warm bath and had sat down at an open window in the evening, immediately after the bath, with nothing but a light chemise to cover her body. She sat there for hours and fell soundly asleep. The next morn- ing she experienced considerable difficulty in the use of both upper extremities. Within a few days these became absolutely paralyzed, and the legs at the same time grew stiff and motionless. She has since that time, a period of at least six years, been suffering from the effects of this myeli- tis, and even now presents an atrophic form of paralysis of the right upper extremity, with slight involvement of the left, and with a complete spastic paralysis of both lower ex- tremities, with incontinence of urine and faeces, and with considerable disturbances of sensation, though the latter have been recovered from very much more than has been the paralysis or the atrophy. Refrigeration is an important factor in the causation of spinal diseases. We have recognized it as a possible factor in acute anterior poliomyelitis, and we must recognize it as an equally potent factor in some cases of acute transverse myelitis. Pathology and Morbid Anatomy. — The delicate structure of the spinal cord seems peculiarly liable to in- flammatory disease. The cervical and lumbar enlargements of the cord are less frequently the seat of such inflammation than the dorsal portion. The reason of this is not easy to explain, although it must in all probability be sought in the peculiarity of the blood-supply. There can be little doubt that if the blood-supply of the cord is interfered with, necrotic softening follows as in the case of other organs. In the case of one form of myelitis — that due to specific disease — the relation of the my- elitis to disease of the blood-vessels can be clearly demonstrated. Under these conditions the smaller vessels are blocked by thrombi, and the result of this obstruction is a necrotic softening of the surrounding parts. In the eases of traumatic myelitis the earliest changes are due to mechanical injury of the parts, with compression of the delicate structures of the cord by effusion of 320 THE NERVOUS DISEASES OF CHILDREN. ' blood, and to the necrosis that follows such compression. It is more difficult to explain the exact manner in which myelitis is developed in the cases of a toxic character, unless we suppose that the chemically altered states of the blood produce coagulation of the blood and obstruction of blood-vessels, with the same result as in those cases in which these conditions are brought about by other disease of the blood-vessels themselves. The origin of myelitis from refrigeration and rheumatic influences in general cannot be satisfactorily ex- plained in this way, nor can any other plausible explanation be substituted. The myelitis which results from mere concussion without any visible anatom- ical changes must be explained on the supposition that minute changes in the gray and the white matter are present, such as were found in the spinal cords of animals experimented on by Schmauss. If the exact mode of origin of various forms of myelitis is still unknown, the morbid anatomy is no longer a matter of doubt. As for the macroscopical appearance of myelitis, the cord so diseased is generally surrounded by the hyper- asmic meninges, and the cord itself, if inflammation is recent, may appear to be congested and slightly swollen. The dis- tinction between the white and the gray matter is often not so marked as in the normal cord. There is, furthermore, a change in the consistence of the cord, which may be either slightly softer than normal or else so diffluent that as soon as the pia is cut open the cord flows out like creamy pus. This is the condition often found on post-mortem exami- nation ; in all probability the cord is not nearly so soft during life, but, like other necrosed tissue, softens consid- erably immediately after death. In acute myelitis minute hemorrhages are extremely frequent, and the altered cord may present the appearance of red softening. There is every degree of change between simple red softening and the condition of hemorrhagic myelitis in which the extrava- sation of blood, being considerable, for the time obscures all other changes. If the blood has been exuded for some time before death its color may have changed, and the con- dition be that of yellow softening. We also may discern a condition of white softening in which the white matter has become diffluent without any admixture of blood. If ex- amined microscopically the cord is found to contain ample evidence of inflammatory changes. Among these are dilated blood-vessels, with leucocytes, granules of myelin, and, fur- thermore, bodies well known as corpora amylacea. The ACUTE MYELITIS. 321 softened tissue also contains axis cylinders in various states of disintegration. In many cases of diffluent myelitis these changes of the individual ele- ments of the cord are the only ones that can be distinctly made out. But in the parts directly surrounding the focus of most intense inflammation further changes can be made out after proper hardening and staining with the various dyes. On such sections the blood-vessels will be found dilated, and innumer- able leucocytes can be seen in the vicinity of such vessels. The nuclei of the smaller arteries and capillaries will be found to be enormously increased and in a state of proliferation. The sheath of the blood-vessels is very much distended, blocked in part by the coagulation of blood, and round blood- corpuscles may even be found in the adjacent tissue. In the gray substance the large nerve-cells are swollen and granular, many of these granules showing distinct evidences of degeneration. The proc- esses of the cells are either shrivelled up or entirely lost, the contour of the cells less distinctly defined, and changed from the polygonal form to spher- ical or oval-shaped bodies. The neuroglia of the gray matter will appear denser than under normal conditions. In the white substance similar changes will be found in the blood-vessels and in the interstitial tissue. The white substance often has a distinctly fibrous appearance, containing many spider-cells or cells of Deiters. The white nerve-fibres themselves undergo degeneration. The axis cylinders are irregular, swollen, and often trans- versely divided. In some cases the entire nerve-fibre is destroyed or disin- tegrated and the space once occupied by such fibres is left vacant or occupied by granular matter. In some cases of myelitis the nerve elements are more intensely affected than the interstitial tissue, while in others the changes in the interstitial tissue are the more prominent feature in the cross-section, and the nerve-tissue has evidently been destroyed secondarily. In transverse myelitis the changes may be distributed equally through the entire cross-sec- tion ; in other cases the changes may be more intense in the gray than in the white matter, and in some more intense in the ventral half than in the dorsal half. In cases of meningo-myelitis, particularly in those of traumatic or specific origin, the most marked changes are near the periphery. Here the pia will appear thickened and the morbid changes can be traced along the connective tissue passing from the pia into various portions of the cord. If the myelitis is of the disseminated order, small foci of disease may appear in various portions of the cross-section and in various segments of the cord, intervening parts maintaining a tolerably normal appearance. If the myelitic changes are most prominent in the vicinity of the central canal and the parts surrounding it, we speak of a central myelitis ; but it is rare to find such central myelitis without some additional symptoms of a diffuse inflammatory process. The nerve-roots in connection with the inflamed segments are. as a rule, altered, and will present appearances somewhat similar to those found in the white matter of the cord. The vessels are dilated, the nuclei and the tissues about these vessels exhibit various degrees of proliferation, the myelin 21 322 THE NERVOUS DISEASES OF CHILDREN. is disintegrated, and the axis cylinder either swollen or distorted ; but these degenerative changes can, as a rule, be traced only a short distance from the diseased cord. Secondary changes follow upon the area of inflammation, and the tracts will be affected in an upward or downward direction, according to the direc- tion in which they transmit impulses. Thus, after a transverse myelitis the lateral columns will degenerate downward throughout their entire extent, but an ascending degeneration will occur in the parts that transmit impulses in a centripetal direction. (Fig. 80.) Among those exhibiting ascending degen- eration are the posterior columns, the cerebellar tract, and the antero-lateral ascending tract. The inflammation spreads a short distance upward and downward by contiguity, and those parts which one would suppose to be subject to descending degeneration only may be affected for a short distance above the lesion ; but such changes are of a distinctly inflammatory charac- ter and altogether different from the purely secondary changes, which rarely offend against physiological principles. These degenerations are, as a rule, developed very promptly after a transverse lesion, and often continue to exist after the initial inflammation has pretty well disappeared. Whether fibres that have once been seriously altered, or even destroyed, can ever regain their function or can grow anew, is a matter of serious doubt, and yet recovery takes place in a fair number of cases in which absolute paral- ysis, with signs due to descending degeneration, had existed for a number of months, or sometimes for a year or more. We must suppose in such cases that some fibres were so little altered that when the inflammatory products were ab- sorbed they still retained the power of conduction, and it is more probable that such fibres may be restored to absolute health than that entirely new fibres can be formed within a nerve-sheath, or that destroyed fibres can be replaced by new ones. Differential Diagnosis. — The more or less acute on- set, the often sudden loss of power, the rapid spread of anaes- thesia, the permanency of all these symptoms, together with the retention of urine and faeces, and the flaccid and atro- phic symptoms at the level of the injured part, together with the spastic symptoms in the parts supplied from seg- ments below the level of the lesion — all these symptoms will leave little doubt of the diagnosis of acute myelitis. In addition to this the etiological factors in the case — the occur- rence of traumatism, a preceding syphilitic infection or pre- ACUTE MYELITIS. 323 ceding bone disease, or marked rheumatic influences — will help to corroborate the diagnosis. Acute myelitis may resemble hemorrhage of the cord, but in cases of hem- orrhage the onset is more sudden than in cases of acute inflammation, all the symptoms being developed within a very few minutes. There is, as a rule, too, much more pain than in cases of myelitis ; but hemorrhage is frequently enough the first stage of a myelitis, and if symptoms indicating a spread of dis- ease follow upon what is supposed to be an initial spinal hemorrhage, it is fair to conclude that a myelitis haemorrhagica has followed upon the initial extrava- sation of blood. Direct injury to the spinal column may be another factor tending to corroborate the diagnosis of hemorrhage. A rapidly ascending myelitis may suggest the acute ascending (Landry's) paralysis, but in cases of myelitis the progress will be clearly from the level of the first injury, and is not apt to attack the parts in succession from below upward, including the trunk, as in cases of Landry's paralysis. In ascending myelitis, moreover, sensation is disturbed from the start, and all the trophic, as well atrophic, symptoms are much more characteristic of a myelitis than they are of Landry's paralysis. But if the myelitis begins in the lumbar por- tion of the cord, and gradually spreads upward, the difficulties of diagnosis may be extremely great. Landry's paralysis is unusually rare in children, whereas myelitis is relatively frequent. The distinction between meningitis and myelitis is not of great practical importance, for meningitis is rarely present without some involvement of the cord, and if the symptoms are purely meningeal, they are generally associated with other symptoms pointing to a wide-spread affection. A pri- mary spinal meningitis is a great rarity, except as a part of cerebro-spinal disease or after disease or injur)- of the spi- nal column. The involvement of the meninges in a given case will be indicated by considerable pain in loco morbi, and by the presence of distinct neuralgic pain along the nerves emanating from the diseased portion of the cord. The question at times arises whether a case is one of myelitis or multiple neuritis. In the latter symmetrically located pains are a more prominent symp- tom, trophic disturbances are not so marked as in myelitis, and the symptoms never include vesical and rectal disturbances. Moreover, we never have that combination of paralytic and spastic symptoms which we so frequently find in cases of myelitis. In cases of multiple neuritis affecting all four extremities the symptoms in all the extremities are entirely the same, whereas in cases of myelitis they would be of the flaccid order, say, in the upper extremity, and of spastic order in the lower extremity. 324 THE NERVOUS DISEASES OF CHILDREN. A more difficult task it is to distinguish between a mye- litis and hysterical paralysis, and yet a careful examination of the patient should reveal important points of diagnosis. Thus, in hysterical paralysis, legs that cannot be used in standing or walking may be moved freely in bed ; the rigidity is not so marked as in cases of myelitis, and if pres- ent can be more easily overcome, as a rule, than in myelitis. Bed-sores are rarely present in hysteria, and anaesthesia, if present, is anomalous in distribution. The reflexes, too, are not so distinctly exaggerated in hysteria as they are in cases of myelitis. If the symptoms should point to a lumbar af- fection, the lack of atrophy and the persistence of the knee- jerks will help to differentiate the hysterical paralysis from a spinal paraplegia. The bladder and rectal symptoms are also not so marked in hysterical as in myelitic cases. The very suddenness of the onset in hysterical cases, the fact that the paraplegia is frequently due to a sudden fright, or a deep emotional condition, may also point to hysteria rather than to myelitis. But in all such cases the fact that hysteri- cal subjects may suffer from organic lesion should be borne in mind. Prognosis. — The prospects in cases of acute myelitis will vary according to the level affected. Cervical myelitis is naturally a more serious disease than myelitis of lower por- tions of the cord, for in the former an extension upward to the respiratory and cardiac centres constitutes one of the grave possibilities of the case. In these, as well as in dor- sal and lumbar myelitis, the danger to life arises chiefly from the complicating conditions, particularly from bed- sores, from cystitis, and pyelo-nephritis. The earlier these symptoms set in the graver the prospect of the case ; but not a few of such cases get well in spite of all compli- cations, and if the myelitis can be proved to be due to spe- cific disease, or to some other form of mild toxic infection, recovery is more probable than in the traumatic cases of myelitis or those in which the etiological factor was en- tirely unknown. The prognosis will also vary according to the intensity of the affection and the extent of cord involved. If all the symptoms are developed rapidly, then become stationary ACUTE MYELITIS. $2$ and show not the slightest sign of improvement for weeks or months, the probability of spontaneous recovery is ex- tremely slight ; but any improvement which sets in, either in the form of diminution of anaesthesia, of the disappear- ance of bed-sores, or of a slight gain in motion, is a hopeful sign of greater improvement later on. I have myself seen complete recovery in cases of myelitis in which the palsy was absolute for a period of nearly six months, with marked contractures and increase of the reflexes and with slight vesical symptoms, but I cannot recall a single case in which complete recovery set in if deep bed-sores de- veloped at an early day, and marked cystitis appeared very early in the disease, the only exception to this rule being in cases of distinct specific myelitis. Treatment. — In the treatment of myelitis the follow- ing plan should be pursued if the patient is seen during the acute stage. An ice-bag should be applied to the greater part of the spinal column ; counter-irritation may be used, but the danger of trophic changes in the skin should be remembered, and such trophic changes should not be encouraged or started up by an excessive use of counter irritants. The patient should be placed absolutely at rest, if possible with some form of extension. His bow- els should be thoroughly purged, best by the use of calo- mel, and the bladder catheterized by careful hands and watched for the first signs of a cystitis. (If cystitis should develop, no time should be lost, even during the acute stage, in beginning the usual treatment for such conditions.) All these measures will tend, first, to make the patient more com- fortable, and, secondly, they will surely lessen the danger from complicating conditions. The diet should be of a mild, non-irritating kind, and the kidneys should be encouraged to greater activity. Under such conditions the administra- tion of small doses of digitalis or of the acetate of potash will be quite in order. Ergot was recommended years ago by Brown-Sequard, and may be administered with the idea of limiting the area of inflammation. 1 cannot say that 1 have ever seen any direct results from ergot, but it seems to do no harm, and considering the seriousness of the dis- ease it may well be tried. In cases in which there is reason 326 THE NERVOUS DISEASES OF CHILDREN. to think that there is much inflammatory exudation, and particularly in those of specific origin, the administration of the iodides is quite in order, or of the mercurials and iodides combined ; but if mercurials are exhibited it is use- less to give them in any other way than by inunction. The main objects are to prevent the serious complications so common in these diseases, and to give the diseased or- gans a fair chance of spontaneous recovery. It is of the utmost importance, therefore, in cases of myelitis, to keep the patient absolutely clean — a task not so easy in view of the frequent dribbling of urine and of the involuntary passage of fseces. The child should be placed upon a water-bed. Nurses should, invariably, receive instructions to keep the bed-linen absolutely smooth, and to promote this end it will be best to have the bed dusted very liberally with some slightly aseptic powder. In the case of female patients, pads should be put in place to catch the dribbling urine and to prevent its soaking adjacent parts ; in the case of boys, urinals should be used from the Start. It is a common practice with me to order the patient's posi- tion in bed to be changed at least every hour, so that no one part is pressed upon for too great a length of time. If in spite of all these precautions bed-sores should form, these should be treated according to the best surgical prin- ciples. Latterly I have been in the habit of dusting the sores with dermatol or aristol and covering them com- pletely with light antiseptic dressing, shielding the dress- ing as well as may be from contamination by urine or fasces. It is better to change the dressing frequently than to allow any infection of the sores through uncleanliness. In hospitals or among the poorer classes less expensive sub- stances, such as bismuth, may be used ; and if cystitis has been set up, the bladder should be washed several times a day with some weak antiseptic solution. After the symptoms of the acute stage have been suc- cessfully treated, and the patient has passed into a more or less chronic condition, the question arises as to the proper treatment of this latter stage of the disease. I am in favor of making the attempt again and again of affecting the focus of inflammation by the administration of the ACUTE MYELITIS. 32 J iodides. In nine cases out of ten this will be unavailing*, and yet there is no good reason why the effort should not be made. But the possibility of recovery should be well weighed in the scales as compared with the gastric disturb- ance which these drugs so often excite. The nutrition of the child must be maintained at all odds, and it is far better to abandon the iodides than to permanently impair the as- similation of food. If the iodides cannot be given, inunc- tions of the oleate of mercury or of the usual mercurial ointment should be substituted for the iodides. Counter- irritation may be attempted, either by the cautery, by blis- tering, or by mere cupping ; but little direct good is to be expected from these remedies. The child should be kept absolutely at rest, and the spinal column should be disturbed as little as possible. Electricity applied to the spinal cord is of very doubtful utility, but there is all the more reason for using it in the treatment of the paralyzed parts. It does excellent service here as a form of exercise for the maimed limbs. Massage has very much the same, and, I be- lieve, a better, effect than electricity. It tends, in addition, to maintain the nutrition of the parts, and to overcome the tendency to contractures, which so frequently give rise to the most disagreeable symptoms in these cases ; but nei- ther electricity nor massage should be pushed if the invol- untary spasmodic contractions become more frequent, as they often do in consequence of these measures. These contractions are not harmful, but are extremely irritating to the patient, and if inordinately increased may disturb his rest, and thus interfere with the general nutrition. As soon as the child has sufficiently recovered, it is of the greatest importance to give it all the fresh air possible, and, if necessary, to provide it with a wheeled chair, so that it can be given its regular outing. Its diet must be care- fully looked to, and all unnecessary excitement should be avoided. Tonic measures may be employed ; and if the child is in the charge of a competent person give lukewarm baths, followed by cool or cold douches of the spine, and let this be done before the massage is given. Iron, quinine, or arsenic may be administered for their general tonic ef- fect. Strychnine may be given in very small doses in those 328 THE NERVOUS DISEASES OF CHILDREN. cases in which there is reason to think that the substance of the cord has not been absolutely destroyed, and that the function of the diseased parts could be increased by the use of this drug. I know that it is an extremely popular drug in these diseases with all physicians who are not specialists, but I have found it to be a double-edged weapon. If it in- creases nerve conduction for a time, such improvement is very apt to be followed by a further diminution of func- tion, and in other cases again it produces annoying mus- cular contractions, which are as disagreeable as those that result from an excessively strong electrical current. In these cases, too, the physician who watches his patient carefully, and does not attempt to do too much, will suc- ceed far better than he who is continually meddlesome and ever anxious to change treatment. TRAUMATIC INJURIES OF THE SPINAL CORD. The direct results of jumping, of falls, and of injury due to falling weights are, on account of the more delicate nature of the spinal vertebrae, more seri- ous in childhood than in later years. A fall downstairs, or a simple fall out of bed, or a mild blow inflicted by another child, may be a sufficient cause to start the symptoms pointing to actual injury of the spinal cord. The symptoms of spinal-cord injury may be extremely varied. I do not propose now to discuss those cases in which traumatism is the remote and questionable cause of a subacute or chronic form of spinal disease de- veloping months, or even years, after an accident ; but I wish particularly to direct attention to those cases in which the traumatic injury is followed im- mediately, or within a period of a few weeks, by symptoms which point to direct injury. In the severest form of traumatic injury to the cord there is immediate paralysis both of the motor and sensory functions. Inasmuch as the injury more frequently involves the dorsal and lumbar portions, a spastic paraplegia with anaesthesia of the lower extremities, with loss of vesical and rectal control, constitute the chief symptoms which may be developed within a few minutes or within a few hours after the injury. If examined more in detail we can determine by the character of the symptoms, first, the exact portion of the cord involved by the injury, and sec- ondly, the amount of injury done at any level. The question of the exact extent of the injury up and down the cord is determined by the parts paral- yzed, and more particularly by the extent of the anaesthesia. These cases are indeed well calculated to illustrate the principles of spinal localization. In cases of cervical lesion the upper as well as the lower extremities are in- volved. In the upper extremities the paralysis is of an atrophic order, while spastic paralysis is present in the lower extremities. The anaesthesia in- ACUTE MYELITIS. 329 volves both the upper and lower extremities as well as the trunk, to or from a level supplied by the nerves coming off from the injured segment. In cases of cervical injury the sympathetic may be involved. In cases of in- jury to the dorsal region, which are the most frequent, the arms are not af- fected, the lower extremities are in a condition of spastic paralysis, the bladder and rectum may be involved, and the anaesthesia extends from be- low upward to a level corresponding to the segment or segments injured. The band of hyperesthesia, or the upper level of the anaesthesia, will cor- respond to the upper limit of injury. If the injury has been done to the lumbar enlarge- ment, the paralysis of the legs is of a flaccid order, the reflexes are diminished or lost, the vesical and rectal reflexes lost, and the areas of anaesthesia will vary according to the seg- ments involved. Injuries to the cauda equina are of particular interest because of the pecul- iar character of the anaesthesia, upon which alone an accurate diagnosis can be based.* The exact study of the symptoms will often help us to determine the upper as well as the lower limits of injury. Thus in one case which I have had occasion to observe, there was spastic paralysis with slight dimi- nution of the knee-jerks, but presence of ankle clonus. There was spasmodic twitch- ing of the legs, loss of vesical and rectal reflexes, as well as a tendency to bed-sores. The difference in the behavior between the knee-jerks and the ankle clonus proved con- clusively that the injury had slightly involved the upper lumbar segments, chiefly the lower dorsal region, but that every part of the cord below the upper lumbar segment had escaped injury, or else the ankle clonus would surely not have been present. In determining the extent of the cross-section involved, a very important question, and one which helps us to decide whether a complete crush has taken place or not. we must keep the physiology of the cord in mind, and endeavor to make out whether the anterior horns as w T ell as the lateral or the posterior columns have been involved. Thus, in a case in which the lesion was in the lower dorsal region, and in which there was complete anaesthesia with marked spastic paralysis, I found that the muscles of the back showed, after a lapse of weeks, neither a tendency to atrophy nor to changes in electrical reactions. I argued from this, and correctly too, as the event proved, that the chief injury was done to the posterior and lateral portions of the cord, and that the ventral portions had entirely escaped. * See the paper by Starr in American Journal of the Medical Sciences, 1 r.h , Fig. 81.— Sketch of Section of Spine in a Case of Fracture Dislocation of the Seventh Cervical Vertebra. (After Thorburn. ) 330 THE NERVOUS DISEASES OF CHILDREN. Another aid to accurate diagnosis of the lesion will be found in the sen- sitiveness to pressure over the spinal column at the seat of injury. The parts that are sensitive should correspond to the segment of the cord which an examination of the patient has shown to be diseased, and before coming to any definite conclusion the physician should remember the relation of the external parts to the segments of the cord, as given in Figure 64. If these two sets of facts do not accurately correspond, the preference should, to my mind, be given to the seat of injury as determined by the study of the paralysis and the anaesthesia ; but if the level determined in this way is not far distant from the seat of pain on pressure, both should be included within the area to be operated upon. The course of the disease will depend largely upon the region affected and upon the extent of injury done. Injuries to the cervical region are, on the whole, more serious than those to the dorsal and lumbar portions of the spinal cord. Injury to the lumbar spine is generally followed by more serious symptoms than is the case after dorsal injury. If the initial symptoms in- dicate a comparatively slight lesion at any level, the progress of the disease is apt to be more favorable than if a complete or nearly complete crush of the cord has occurred. If the symptoms show a tendency to improvement after a few weeks, or after a month or more, the possibility of complete recovery may be considered ; but if they remain stationary for a long period without the slightest indication of improvement, actual recovery is rare, unless re- lieved by operation. The danger to life is greater in the cervical cases, in which the proximity to the vital parts is of much importance ; and in severe cases of dorsal or lumbar injury the complicating conditions, such as cystitis and bed-sores, may bring about a rapidly fatal issue. After an initial injury that is relatively slight, de- generation may set in, which will be characterized by the onset of rigidity and contractures, and from the onset of these symptoms the prognosis as regards complete recovery may become very much graver. Pathology.— The actual anatomical lesions in traumatic injuries of the spinal cord may vary greatly. Even without injury to the vertebral column hemorrhage may occur from the effect of the shock, and this may be either epidural or subdural. The probability of the seat of the hemorrhage will have to be argued from the general character of the symptoms. In persons whose arteries are fragile, hemorrhage is much more likely than in persons whose vascular system is entirely normal. Persons with syphilitic disease will, therefore, be much more liable to traumatic hemorrhage than those not so affected. If the hemorrhage is considerable, whatever its location may be, the cord will suffer from compression and may undergo softening unless the blood that is exuded is rapidly absorbed. In all cases, inflammatory prod- ucts, which may be tinged by blood, will be found at the seat of injury. At times, in spite of a sudden paralysis developed immediately after an injury, no tangible lesion can be discovered at the time of operation or on the post- mortem table. We must then suppose either that the evidences of the initial lesion have disappeared, or that the traumatism has resulted in functional changes, or in such, at least, as are beyond the discovery by our present ACUTE MYELITIS. 33 I methods. If examined months or years after the initial injury, the cord may present nothing but the ordinary symptoms of chronic myelitis with consider- able shrinkage and wasting of the entire substance of the cord. Such wast- ing may be at times more marked in the white columns, at other times more distinct in the gray matter. In addition to the local changes found, the cord, if examined carefully, will reveal ascending and descending degenerations in accordance with the intensity of the process at any given level. I have seen several cases of spinal injury with severe spinal symptoms in which at the time of the operation no tangible changes were found in the cord, but the cord was evidently compressed by inflammatory exudations that had collected between the bone and the dura. Adhesions also form under these conditions between the dura and the surrounding parts. The impairment of function is, therefore, due to extra-spinal conditions. The breaking up of such adhesions and the removal of such inflammatory products are followed by improvement in the condition of the patient, if such removal is effected within a relatively short period of time after the accident. Treatment. — In cases of traumatic injury of the spinal cord, absolute rest is essential. The case may be treated in every respect for the first few days as though it were a case of non-traumatic myelitis, that is, by applica- tion of cold to the spine and by extension. As soon as the condition of the patient will permit, a careful examination should be made in order to de- termine the amount of injury done. The details of the treatment will not vary from that advised in cases of myelitis. The old habit of using the actual cautery, of blistering, and the like, is not to be recommended, for little good can follow it. As was said in the case of myelitis, the danger of trophic disturbances in the skin is great enough without such additional en- couragement. The most important question that arises is whether anything can be done for the patient by surgical means. There is still much hesita- tion on the part of physicians and surgeons in this respect. If the patient is in good general condition, and is able to stand the shock of the operation, the advisability of such surgical interference should be con- sidered purely upon the merits of the case, and if the evidence points to the fact that injured bone is pressing upon the cord, or that severe hemorrhage has occurred and that there is danger of permanent harm to the cord, the sur- geon should be permitted to expose the injured region. This can be done with considerable impunity by competent surgeons at the present day, and. as I have said, should be done as early as practicable after the injury. The cases in which surgical interference is useless are those in which all the symptoms indicate absolute crush of the cord, or in which the rapid devel- opment of all the symptoms points to a probably fatal issue ; or those in which so long a period of time has elapsed since the injury that there is no good reason to believe that the conditions can be relieved by operation. I have advised operations upon the spinal column in four cases, two of which were in children under the age of fifteen. The results were satisfactory, al- though neither one attained complete recovery ; but I attribute this to the fact that the cases were sent to me at too late a period after the accident. The operations are not nearly so dangerous as those upon the brain, and 332 THE NERVOUS DISEASES OF CHILDREN. much encouragement may be derived from the statistics of various surgeons who have not lost a single case of spinal operation for traumatic injuries. If for some reason or other the operation has not been performed and the pa- tient is left with a chronic spinal disease, the attempt may be made to bene- fit the patient by the administration of the iodides ; and the crippled con- dition of the extremities may be somewhat improved by ordinary surgical and orthopedic measures, such as have been frequently referred to in the discus- sion of myelitis and other forms of palsy. LANDRY'S PARALYSIS. Acute ascending paralysis (Landry's paralysis) is extremely rare in child- hood. It has been considered in connection with multiple neuritis, from which it is to be differentiated. It comes on after acute infectious diseases and from exposure to cold ; it is supposed by some to be due occasionally to syphilis. The paralysis begins in the legs, spreading from one to the other, then it involves the muscles of the abdomen, the thorax, the upper extremi- ties, and may spread to the muscles of the pharynx, larynx, and eyes. The paralysis is generally flaccid, sensation may be disturbed, and the reflexes are lost. The electrical conditions are generally disturbed, but not to the extent we find in poliomyelitis or in neuritis ; fever is sometimes present, sometimes absent. Enlargement of the spleen has been noted. The case may prove fatal within several days or a week. Some of the cases go on to recovery ; the paralysis, as a rule, disappearing first from the upper extremities. It is questionable whether the disease affects the peripheral nerves only, or whether it involves the central nervous system. The probability is that in this, as in other toxic affections, any or all parts of the nervous system may be involved. BIBLIOGRAPHY. MYELITIS. Chavier et Fevrier : Revue de Medecine, December, 1888. Church : American Text-book of the Diseases of Children, 1894. Eisenlohr : Deutsche Med. Wochenschrift, 1890. Jacobi, Mary P.: In Keating's Cyclopaedia. Leyden : Zeitschr. f. kl. Med., Bd. I., 1880; Neurolog. Centralblatt, 1892, p. 115. Moeli : Arch. f. Psychiatrie, Bd. XL, p. 757. Oppenheim: Berl. klin. Wochenschr., August 3, 1891. Raymond: Revue de Medecine, March, 1886. Schmaus : Compressionsmyelitis, etc. Wiesbaden, 1890. Other articles bearing upon this same subject are quoted in the chapters on Syphilis of the Spinal Cord, Compression of the Cord, etc. CHAPTER XVIII. SYPHILIS OF THE SPINAL CORD: SPECIFIC MYELITIS AND MENINGO-MYELITIS. In the adult, syphilis of the spinal cord can now be rec- ognized by a definite combination of symptoms. This is due to the researches of Erb, Gowers, Marie, Oppenheim, Siemerling, Hoffman, and others. In the child syphilitic disease of the spinal cord is not nearly so frequent as in the adult, but I do not hesitate to devote a special section to this subject, both because I think such cases can be easily overlooked and because they present a number of points in differential diagnosis which are of the greatest interest and which must be carefully considered if grave errors in diag- nosis are to be avoided. Symptoms. — In former days it was customary to make the diagnosis of syphilis of the spinal cord if the symptoms pointing to spinal lesion were irregular and would not fit into any of the ordinary types of spinal disease. As a mat- ter of fact the nature of the morbid process underlying these syphilitic spinal diseases is such that irregularities in distribution, and in the development of the symptoms, are very apt to occur, and yet we should be able to recognize the symptoms of syphilitic disease whether or not the pa- tient reveals, or we can prove, previous syphilitic infec- tion. In all but a few of the cases the onset of the disease is gradual. By degrees the legs or the arms, or both the lower and the upper extremities, which have shown some weakness, become paralyzed. One leg, or one arm, is at times more paralyzed than the other. This paralysis may be of the atrophic kind, but is much more apt to be of a spastic order. It is often associated with intense pain (par- 334 THE NERVOUS DISEASES OF CHILDREN. aplegia dolorosa), or with anaesthesia — the anaesthesia and paralysis may be crossed (Brown-Sequard type). The re- flexes are generally increased, rarely absent. If the specific process is situated in the cervical segments there are atrophic paralysis with loss of reflexes in the upper extremities, spas- tic paralysis with rigidities and contractures in the lower extremities. The vesical and rectal reflexes may be inter- fered with. If the dorsal or lumbar segments are involved, the symptoms will closely resemble those following upon myelitis of the respective segments ; trophic disturbances may occur ; bed-sores may develop ; in short, we may have all the symptoms of a wide-spread spinal affection. It is evi- dent that if we wish to distinguish between specific disease of the cord and the various forms of acute or chronic my- elitis we must look for some distinct points of differential diagnosis. Erb, referring to be sure to the conditions in the adult, has established a type of spinal-cord disease Avhich he pro- posed to call syphilitic spinal paralysis. This special type bears the following characteristics : First, the usual symp- toms of spastic paraplegia, with its peculiar gait, carriage, and movements ; second, marked exaggeration of the deep reflexes ; third, muscular contractures, which are slight as compared with the exaggeration of the reflexes ; fourth, involvement of the bladder ; fifth, a slight yet distinct dis- turbance of sensation ; sixth, gradual onset of the disease ; seventh, a decided tendency to improvement. There is no doubt that this type of spinal disease, so well characterized by Erb, does occur. The same series of symptoms has been recognized by Rumpf, and since Erb's publication cases of this description occurring in children have been observed by Friedmann and by myself. But I have taken some pains to prove that there are other types, quite as frequent as this one, and that it is a great mistake to hesitate in making the diagnosis of spinal-cord syphilis unless the symptoms of Erb's type are present. It is the author's conviction that, if we wish to make a positive diagnosis of syphilis of the spinal cord, we should pay attention to the following points, and not exclusively to those presented by Erb as character- istic of the special type he has described. SYPHILIS OF THE SPINAL CORD. 335 First, the most striking feature of syphilis of the spinal cord is the unusual distribution of the disease over the greater portion of the cord, involving, as it often does, the cervical and dorsal, as well as the lumbar enlargements. Second, the slight intensity of the morbid process at one level as compared with the extensive area involved, as evidenced by the preservation of some of the functions of the cord with complete loss of others. Third, the rapid dwindling of some of the symptoms and the very chronic persistence of others. Thus in some of my cases the anaesthesia lasted but a very short time, while the paralysis was recovered from with extreme slowness. Fourth, the very frequent history of other symptoms pointing to specific disease in the same or distant parts of the central nervous system. To emphasize these views let me state that I should be inclined to suspect specific disease of the cord if the patient presents symptoms of paralysis, whether they be of the spastic or flaccid character, and whether the contract- ures be slight or not, provided he furnish evidence of a morbid process affect- ing a very large part of the cord, and yet showing a relatively slight intensity at any given level of the cord. He may, for instance, exhibit the symptoms of extreme paralysis, spastic or atrophic, with partial or slight anaesthesia of the parts paralyzed, with little or no involvement of the bladder ; or, as often hap- pens, he may present traces of specific disease in other parts of the central nervous system. The chief difference, according to this, between the ordinary forms of myelitis and the specific diseases of the cord can be understood if we remember that the symptoms of an acute or subacute myelitis prove that the entire cross-section of the cord is affected almost simultaneously and to an equal degree, whence it follows that in such cases severe paralysis is likely to be associated with severe anaesthesia, with marked contractures, with abso- lute loss of vesical and rectal control, with serious trophic disturbances, and so on ; whereas, in the cases of spinal syphilis the morbid process invades the cross-section of the spinal cord partially and slowly. We may, therefore, find symptoms which point to a very marked affection of one or more of the systems of the spinal cord and to relative immunity from disease of the gray matter or other portions of the cord. Thus we may have extreme paralysis, but only slight ansesthesia ; or extreme loss of power, with relatively slight rigidity, as Erb pointed out. Furthermore, in the ordinary cases of myelitis, the symptoms point to a certain portion of the spinal cord at which the dis- ease is most intense, while in cases of syphilis of the spinal cord the clinical symptoms show that the disease involves a very large portion, if not the en- tire spinal cord, yet affects each single segment but relatively little. A still further aid to differential diagnosis is the very frequent involvement of the brain, at the same time that the majority of the symptoms point to disease of 336 THE NERVOUS DISEASES OF CHILDREN. the spinal cord, or if these two sets of symptoms do not set in simultaneously, we frequently have in a patient who presents symptoms of a specific myelitis the history of a preceding illness in which the symptoms were of a cerebral rather than of a spinal character. (Unequal ocular, and, above all, unequal pupillary symptoms, are very common symptoms of cerebral syphilis.) The rapid and often unexpected recovery, as well as the relapses, help also to dis- tinguish these cases from the usual forms of myelitis. In view of the rarity of these diseases, or possibly of the failure to recognize them when they do occur, I wish to give a few typical cases. The first one I take from Fried- mann's article on relapsing, probably specific spastic, spinal paralysis in childhood. A boy, five years of age at the time of examination. Nine months before the birth of this child the mother miscarried with twins at the end of the sec- ond month of pregnancy. The birth of the boy was entirely normal, but the head was said to have been very large. Relative reduction of the size of the head in the next few months. Four weeks after birth a skin eruption appeared which covered the entire body; it was vesicular at first; later on it ulcerated, and terminated in desquamation. The child began to ex- hibit normal mental development, learned to talk at the end of one year, and began to teeth at the age of six months. At the age of three months there was distinct difficulty in moving the arms and legs. Three months later the right arm could be moved, and at the age of one and a quarter years all four extremities appeared to be entirely normal. In the second year, the child having learned to walk well, paralysis again gradually developed in the left arm, and disappeared once more after six weeks. From the second to the fourth year the boy was healthy, and passed through measles and diphtheria without any disagreeable sequelae. In the fourth year he complained much of headache, particularly in the occipital region, and gradually his gait became weaker and weaker, and he began to drag the right leg, frequently falling in the attempt to walk. There was slight difficulty in micturition and the legs were somewhat rigid. In this attack the arms were entirely free, and there was no history of spasms or convulsions at any time. The patient was in this condition when examined by Friedmann. The only other points of in- terest in the case were the very bad condition of the teeth, slight increase of the reflexes, and entirely normal sensation. Very recently J. Hoffmann has reported the case of a boy, who, at the age of twelve years, developed a typical spastic paraplegia, evidently due to hereditary syphilis, manifest signs of which appeared in the first years of life. The following case, observed by myself, presents many similar features : SYPHILIS OF THE SPINAL CORD. 337 E. S , a girl, aged six years, born in this country, of German parents, was brought to my clinic in June, 1893. The mother gives a history of pro- tracted labor, but child was entirely normal ; began to cut teeth at six months, to stand and walk at the age of one year, and learned to talk well before she was two years old. The child had been perfectly well, with exception of mild attacks of whooping-cough and measles. At the age of five years the mother noticed that the child began to walk in a peculiarly stiff manner, and that its mental development was somewhat retarded. On examination we noted spastic par- alytic gait ; spastic paraplegia of lower extremities, more marked on left side ; left upper extremity slightly paretic and rigid ; both knee-jerks exaggerated ; triceps and wrist reflexes lively on left side. Pupils unequal ; left pupil dilated and does not react to light ; right pupil reacts sluggishly to light ; both pupils react sluggishly during accommodation. No sensory symptoms. The suspicion of syphilitic disease was strengthened by an examination of the mother. She has had two miscarriages ; three children died in early life. Five years ago (at the age of thirty years) she had left hemiplegia ; no loss of consciousness ; recovered in a few weeks ; has distinct " rheumatic " attacks ; gets dizzy while washing her face in the morning ; her pupils are unequal ; no reaction to light or during accommodation ; knee-jerks absent ; slight Romberg symptom ; no bladder trouble ; delayed sensory perception in the lower extremities. Thus we have a tabic, if not a purely syphilitic affection in the mother, and spinal syphilis in the child. Differential Diagnosis. — It seemed to me best to in- troduce most of the salient points of differential diagnosis in the description of the symptoms of the disease, and the comparison with myelitis was inevitable. On the distinc- tion between myelitis of the ordinary types and specific disease of the spinal cord, I need say nothing more. Syph- ilis of the spinal cord might be confounded with a spastic infantile palsy, particularly with a spastic diplegia and paraplegia resulting from meningeal hemorrhage occurring during the period of labor ; but in these latter cases the trouble can be traced distinctly to the earliest period of life, and there is never any history of relapses, and rarely of any marked improvement followed by relapse. Moreover, in the typical cerebral palsies the contractures are apt to be more extreme, and defective cerebral development is much more frequent than in the syphilitic cases coming on subsequently to the birth of the child. An acute infantile cerebral palsy might possibly simulate specific disease. 333 THE NERVOUS DISEASES OF CHILDREN. were it not for the far greater frequency of the hemiplegic form of paralysis in these cases than in the purely syphi- litic types of disease. Special difficulties might, however, arise in cases in which the acquired infantile hemiplegia would be proved to be due to syphilitic disease of the ar- teries, and under such conditions a combination of spinal syphilitic disease with cerebral disease would not be im- possible, although I have not yet come across a case of this description. Spinal syphilis in a child should not be con- Fig. 82. — Case of Multiple Cerebro-spinal Syphilis. Section through pons showing gummatous formation {g) in ventral portion, with considerable destruction of tissue. Weigerfs hasmotoxylin stain ; low power.* founded with hereditary spastic paralysis ; possibly the latter may be developed on a specific basis. Morbid Anatomy. — The anatomical changes in the spinal cord, due to syphilis, may be quite as varied in the child as in the adult. It is well known that syphilis is apt to cause disease of the blood-vessels. Obliterative endoar- teritis, with subsequent softening of the area supplied by the diseased vessel, is perhaps the best known anatomical process directly attributable to syphilis ; but a general ar- * Figs. 82-84 are reproduced by the courtesy of the editor of the New York Medi- cal Journal. SYPHILIS OF THE SPINAL CORD. 339 teritis is quite as frequent as an inflammation of the endo- thelium alone. Bruce has directed attention to changes in the adventitia (nodose periarteritis); moreover, veins are subject to syphilitic changes quite as often as the arteries are (phlebitis obliterans, Greiff). All these vascular changes are much more pronounced in the pial covering than in the substance of the spinal cord. The investigations of other authors, as well as my own, Fig. 83. — Section from Ventral Surface of Medulla Oblongata (high power), show- ing Infiltration of Pia and Substance of Medulla {a) and Typical Syphilitic Arteritis ; Marked Thickening of, and cellular proliferation in Intima (/) ; Narrowing of the Lumen ; Cellular Infiltration of Adventitia {b~). have shown that syphilis of the spinal cord is more often associated with a subacute or chronic meningitis, or me- ningo-myelitis, than with anv other process. The disease starts, as a rule, in the pia, and subsequently invades the spinal cord. There can be little doubt, however, that the changes may in some instances be developed in the reverse order. In the gross specimen we find the pia thickened and 340 THE NERVOUS DISEASES OF CHILDREN. often covered by a thick gelatinous substance. On micro- scopical examination this thickening is seen to be due to a proliferation of all the tissues. The cells are multiplied in number, the nuclei have increased, and the blood-vessels show the characteristic changes of specific arteritis. The walls of these vessels are thickened, all of the coats partici- pating to an equal degree. The process very often starts in the intima, but not invariably so, as was maintained some years ago by Heubner. The pia is generally adherent to <#& Fig. 84. — Section through a Portion of Dorsal Cord (Ventral Surface). Marked thickening of pia ; cellular infiltration of same, seen best in that portion which pro- jects inward ; infiltration of substance of cord. the spinal cord, and the cellular infiltration extends from it into the substance of the spinal cord. This invasion is a very gradual one, and may begin at almost any point of the cross-section of the cord ; but the lateral columns are more frequently involved than any other region, and for this rea- son the spastic symptoms are, as a rule, the first to be de- veloped. In other cases in which the morbid process in- vades the posterior columns the symptoms may resemble those of tabes rather than those of a spastic form of paral- SYPHILIS OF THE SPINAL CORD. 341 ysis. If the invasion occurs from the ventral surface, as it rarely does, the symptoms will naturally be of an atrophic order rather than of a spastic or sensory kind. But wherever the first point of attack may be, the morbid process gradually works its way inward from the periph- ery, and its plan of attack is generally from symmetri- cally situated points. The cellular proliferation and the slight increase in neuroglia tissue, as well as the gradual disappearance of the integral elements of the cord, give to these cross-sections, if examined under the microscope, the appearance of ordinary myelitis, and if it were not for the characteristic changes in the blood-vessels and for the evi- dence that the process has worked its way inward from the periphery, and, indeed, from the pia, the anatomical proof of a specific process would be very difficult to establish. As it is, there are not a few cases in which satisfactory proof cannot be given, and the pathologist is compelled to rely upon the clinical evidence in the case to prove the syphilitic nature of the disease.* But the wide-spread character of the disease, and the very gradual destruction of the different systems of the cord, as well as the impor- tant part played by the pia and the blood-vessels, leave little doubt as regards the true nature of the inflammatory proc- ess, and at the same time help us to understand the pecul- iar behavior of the clinical symptoms. The Figures 82-84 will give full details of the specific meningo-myelitis as seen in one of my adult cases. The meningo-myelitis may be associated with special gummatous deposits in any part of the cord or in the brain. Thus, in one of my cases a gumma in the pons was asso- ciated with a wide-spread specific meningo-myelitis at the base of the brain and throughout the entire extent of the cord. If such a complication exists the child may present the symptoms of tumor of the brain or cord, together with the symptoms of extensive meningo-myelitis. The fact that spinal syphilis has a distinct tendency to improvement makes the prognosis favorable, although this tendency implies also a danger of relapses. But syphilis in * Unfortunately the bacteriological investigations of Klebs, Disse and Taguchi, of Lustgarten, and others, have not yet disclosed the true bacilli of syphilis. 342 THE NERVOUS DISEASES OF CHILDREN. the spinal cord of the adult, as well as of the child, is amen- able to treatment, and the prognosis is distinctly more fa- vorable than in cases in which the same symptoms might be present and not due to syphilis. The prognosis is, for instance, very much more favorable than in cases of con- genital diplegias or paraplegias. The possibility of recovery should therefore be kept in mind, but the hope of complete restoration can be entertained only if the symptoms recede promptly upon anti-syphilitic treatment. If the symptoms do not in any way yield to treatment, and if, after a number of careful trials the condition remains practically the same, the prognosis is as unfavorable as it would be in any other case of myelitis. The prognosis will also depend very largely upon the evidence of the amount of damage that has been done by the specific process. If the symptoms show that there has been a complete destruction in one or more systems of the spinal cord the possibilities of recovery are naturally less than in cases in which the symptoms point to but a slight involvement of these parts. Yet whatever the outlook may be at the time the patient is examined, it is the physician's duty to warn the parents against the possi- bility of relapses and to prepare the relatives for the fact that later attacks may involve more vital parts, and may, therefore, be more dangerous than the one through which the child is passing at the time. Treatment. — Syphilis of the spinal cord in the child, as well as in the adult, calls for very prompt treatment. We must depend upon the usual remedies — the mercurials and the iodides. It is wrong to depend upon either one alone, for in some cases the mercurials are more effective than the iodides, and in others the reverse is true. It is my practice invariably to begin treatment with both,* and to stop one or the other only if either is not well tolerated or if the im- provement is such that I find the patient will do well on one drug alone, or if there is no improvement whatever, and I feel satisfied that the morbid process cannot be influenced either by iodides or mercurial preparations. Mercury should be given in the form of inunctions, either of the ten * I have never seen any untoward results from the simultaneous use of iodides and mercurials in the doses here recommended. Lewin thinks there is danger in this. SYPHILIS OF THE SPINAL CORD. 343 per cent, oleate of mercury or of the unguentum hydrargyri. According to the age of the child, one-half to one gramme of the ointment may be rubbed in daily, and in addition it will be well to begin giving small doses of the saturated so- lution of the iodide of sodium, the daily dose to be increased slowly. Thus I begin with three or four minims of this solution, to be given in milk three times a day, and increase the daily dose by one minim until a child, according to its age, takes ten, fifteen, or twenty minims of the iodide three times a day. As soon as decided improvement takes place the quantity of the mercurial inunction given may be re- duced and soon stopped altogether; but the iodides should be pushed for some time after this improvement has been noticed. In cases in which no improvement occurs, in spite of the proper administration of these drugs, it is well to stop both, for a time at least ; but I would advise, under all circumstances, if the diagnosis is safely established, to make repeated trials of these drugs in the manner indicated above ; and if a child has passed through several attacks of specific myelitis it would be well to subject it to periodic treatment by the iodides in the same manner that one would administer treatment for constitutional syphilis. In addition to the anti-syphilitic treatment, the physician will have to prescribe tonics, sufficient exercise, and nutritious diet, according to the needs of the patient. Moreover, in each attack, or as long as the paralysis lasts, it will be neces- sary to use electricity and massage, as one prescribes them in all other cases of myelitis, whatever the origin of the my- elitis may have been. BIBLIOGRAPHY. Bruce : On Syphilitic Nodose Periarteritis, Edinburgh Medieo-Chirurg. Soc. May 2, 1894. Reprint. Erb: Neurolog. Centralbl., March 15, 1892. Friedmann : Zeitschrift f. Nervenheilkunde, vol. iii.. p. 182. Gerhardt : Berliner kl. Wochenschrift, 1893, P- 1209. Greiff : Arch. f. Psych., vol. xii. Hoffman J. : Neurolog. Centralbl., July 1, 1894. 344 THE NERVOUS DISEASES OF CHILDREN. Hoppe (Cincinnati) : Berl. kl. Wochenschrift, 1893, p. 233. Kuh : Zeitschr. f. Nervenhk., vol. iii. Lamy: These. Paris, 1893. Oppenheim: Report. Berlin, 1890. Berl. kl. W., 1888, 1889. Rumpf : Monograph. Wiesbaden, 1887. Sachs, B. : New York Medical Journal, Sept. 19, 1891. . Brain, vol. xvi., 1893 (contains literature). Siemerling : Arch. f. Psychiatrie, vols, xix., xx., xxii. Sc human : Arch. f. kl. Med., 1889. CHAPTER XIX. DISSEMINATED SCLEROSIS. Disseminated, insular, or multiple cerebrospinal scle- rosis is distinctly a disease of early life. The first pro- nounced symptoms appear, generally, in the second and third decades of life, but a few prodromata can be traced back to a much earlier period.* The disease, as described by Charcot, is characterized by a ver}' definite set of symp- toms ; but variations from this type are not infrequent. Symptoms. — The prodromal symptoms of this disease consist of a weakness of the upper, and sometimes of the lower, extremities, of a slight awkwardness and trembling of the fingers, and of slight subjective sensory disturbances in the arms and legs. The chief symptoms develop gradu- ally, and are present from the early periods of the disease to the very end, but vary much in intensity at different periods of the disease. The most characteristic symptom is the tremor which is observed on voluntary motion of the af- fected parts. It is not observed when the parts are at rest, and only when a voluntary effort is made ; it is spoken of as " intention tremor." The tremor consists of about six to seven oscillations per second (Grasset). It becomes most * In a recent dissertation on Multiple Sclerosis in Childhood, by Totzke, it is stated that some of the symptoms were manifested in two cases at birth ; in one case at the age of five months, in one at fourteen months, and in two at the age of two years ; and of thirty- one cases which the same author analyzes, a very fair proportion began between the fourth and fourteenth years. He infers from this that the earlier years of life show a par- ticular predisposition to this disease ; but there is some reason to doubt whether all these cases were typical cases of disseminated sclerosis. In my own practice the youngest child that I had occasion to observe with distinct symptoms of multiple sclerosis was fourteen years of age at the time of observation, and her symptoms did not come on be- fore the tenth year. Marie, who reported three cases of "sclerose en plaques," in infants, in 1883, now states that he would not diagnosticate the cases as such, and be- lieves the " disease very rare in children.'' 34^ THE NERVOUS DISEASES OF CHILDREN. distinct if the patient is asked to take hold of any object, to pass a glass of water to the lips, or to take hold of a pencil, as in drawing or writing. At first this tremor is so slight that it does not interfere with the execution of the simpler movements, but as it increases in intensity it be- comes a bar to the use of the hands and legs, and may be an annoyance in walking. In attempting to put a glass of water to the lips the water is spilt (" the patient's face and garments are inundated " — Marie), and in writing the tremor is so marked that the pen tears the paper and the writing becomes altogether indistinct. Next in importance to this tremor is the difficulty in speech. The patient speaks deliberately, slowly, pronounc- ing each syllable with unusual care (scanning speech), and yet a distinct tremulousness of the voice is apparent. The consonants 1, p, g, and r, are most difficult to pronounce. The words in which these letters occur become unintelligi- ble at a very early period of the disease. Ocular symptoms are very common ; we may have, at times, an inequality of the pupils. (Uhthoff noted this in eleven per cent, of his cases.) The reactions to light and during accommodation are diminished, and a myosis is gen- erally present. Nystagmus appears at a very early day, particularly if lateral movement of the eyes is attempted. Upward or downward motion is unaccompanied by this phenomenon. Visual disturbances are not as common as in tabes, but do occur frequently, according to the excel- lent studies made of this special symptom by Uhthoff some years ago. Clinically we find a narrowing of the field of vision, particularly of color, and a diminution of the actual visual power. The trouble may be unilateral or bilateral. These symptoms are associated with atrophic discoloration of the papillae, which persists even though an improvement in the subjective symptoms may take place. The eye symp- toms are so similar to those observed in hysteria, that on this account alone the two diseases have frequently been mistaken for one another. A distinct tremor of the tongue and a peculiar vague expression of the countenance are the other head symptoms present in this disease. Charcot described a special facies DISSEMINATED SCIEROSIS. 2)47 of multiple sclerosis, and refers to a vague and uncertain look, the lips hanging down and half open. On the whole there is an expression of mild stupor on the face ; this ex- pression is not altogether unnatural, for the mental condi- tion is in many cases abnormal. The memory is weak ; there is lack of attention on the part of the patient, who passes easily from laughing to crying, and vice versa. Many of the patients are emotional to an extreme degree, and this is particularly true, according to my own experience, of those in whom the disease begins very early in life. The paralytic symptoms are of a spastic order. The legs and arms are moved awkwardly at first, later with dis- tinct evidence of rigidity. A spastic or spastic-paretic gait is present, and is associated with those other symptoms characteristic of all spastic paralysis and of involvement of the lateral columns of the cord, viz., rigidity of the muscles and increase of the deep reflexes. All these symptoms may for a long time be slight, but as the disease progresses they become more and more intense ; the spasticity and weakness grow worse until the patient becomes completely bed-ridden ; and the tremor increases in intensity and inter- feres with the use of the extremities. Speech becomes almost unintelligible, the memory grows weaker and weaker, until a truly pitiable condition is reached. Ob- jective sensory disturbances are not uncommon ; Freund has observed them in twenty-nine out of thirty-three cases. In fourteen there was a mere transitory anaesthesia, while in six cases he states that there was a more or less perma- nent and complete loss of sensation. He also refers to the occurrence of hyperassthesia, and even to the dissociation of sensation, but this latter form of sensory disturbances is surely a great rarity. Fortunately, or unfortunately for many, the disease covers a long period of years, during which a tolerable condition of health is maintained. In other cases, again, the symptoms described are developed more rapidly, and a few others are added which are not in- variably present. This is true of the bulbar symptoms leading to difficulties not only of speech but of deglutition. and occasionally to interference with the respiratory func- tions. 348 THE NERVOUS DISEASES OF CHILDREN. i\\' Paralysis of the ocular muscles, above all of those sup- plied by the sixth and third nerves, are not infrequent. Ves- ical trouble has been recorded by Erb and Oppenheim. If we add to the above symptoms the complete paralysis resulting from apoplectiform attacks it will be evident that the disease may become almost pro- ' )$ { \ I , - ; f v tean in its manifestations. Para- doxical contractions and impulsive laughter are frequent symptoms. As important as the positive symptoms hitherto recorded are those of a negative order. Atrophy of the muscles, paralysis of the blad- der and rectum, and changes in elec- trical reaction are entirely wanting. How such a variety of symp- toms is possible in this disease will be understood easily enough when we refer to the morbid anatomy. The course of multiple cerebro- spinal sclerosis is slow, and though invariably leading to a fatal issue, death is, in the majority of cases, the result of some intercurrent dis- ease. Etiology. — Disseminated scle- rosis comes on most frequently after acute infectious diseases, after pow- erful emotions, and occasionally af- ter traumatic injuries. Oppenheim has shown that the disease appears after intoxication by metallic poi- sons, while Marie believes it to be invariably of infectious origin, com- ing on after typhoid fever, pneumonia, measles, scarlatina, and small-pox. Heredity is supposed to play an important role in the eti- ology of insular sclerosis. In the broader sense it is true ; for multiple sclerosis may indeed affect children who are descended from a neurotic stock ; but it is extremely rare Fig. 85. — Degeneration of the Cauda Equina in Multiple Sclerosis. (After Taylor. ) DISSEMINATED SCLEROSIS. 349 to find the disease in both parent and child. The cases of Pelizaeus, quoted approvingly by some authors, are not to my mind typical cases of multiple sclerosis ; they belong rather to the hereditary form of spastic paralysis. (See page 391.) Pathological Anatomy. — We can infer from the name that the chief lesions in this disease are irregular sclerotic patches distributed throughout the greater part of the cen- tral nervous system. These patches sometimes occur in the brain as well as in the spinal cord ; but there is no rule de- FiG. 86. — Sections through the Pons, Medulla, and Spinal Cord, showing Sclerotic Patches. (Taylor.) termining their first appearance either in one or the other, and there is, therefore, as little reason for establishing a spinal form or a cerebral form on anatomical as there is on clinical grounds. The plaques have, however, a few fa- vorite sites ; the white matter of the brain, the pons and medulla, the lateral columns in the dorsal and lumbar regions are those most favored ; but they also appear in the cortex, in the posterior columns of the cord, in the cervical region, and even in the cauda equina. (Fig. 85.) Charcot was of the opinion that the foci of disease were very rare in the cortex of the hemispheres or of the cere- bellum ; but Taylor, in a very careful paper on this subject. has shown that the patches may be in these parts as well as in others. The sclerotic changes may also affect the spinal 350 THE NERVOUS DISEASES OF CHILDREN. roots and various cranial nerves. The optic chiasm is fre- quently diseased. Whether the sclerosis appears in the cen- tral nervous system itself, or in the nerves emerging from it, the character of the changes is very much the same, the main point being that a few, and not necessarily the major- ity, of the nerve-fibres are destroyed. These patches present slightly elevated, at other shrunken and contracted. ^00mir^ , a bluish-gray appearance and are sometimes times the parts in which they occur appear In size they vary from one twenty-fifth to one inch. They are, as a rule, a little harder than the sur- rounding tissue, and Morris calls especial attention to the nicety with which they can generally be easily dif- ferentiated from it by the naked eye. On microscopical ex- amination these patches are found to consist of a dense fibrous tissue, due undoubt- edly to the increase of the neuroglia and of the con- nective tissue. Weigert in- sists that the proliferation of neuroglia tissue is more marked in this disease than in any other. In these sclerotic portions the my- elin sheaths, according to the stage of the disease, are more or less diseased, but the axis-cylinders in the majority of the cases remain normal. The entire process may, therefore, be characterized as an interstitial inflammation, and it is owing to the preservation of the axis-cylinder that secondary degenera- tion in multiple sclerosis is a rare occurrence, indeed; although Buss has recorded a descending degeneration in the lumbar cord and an ascending de- generation of the columns of Goll, and of the direct cerebellar tract from the eighth cervical segment into the medulla oblongata, but his observations are surely exceptional. The absence of secondary degeneration also accounts for the frequent absence of contractures.* * The tremor of multiple sclerosis has been attributed by some to defective isola- tion (loss of sheath) of the axis cylinders, on the supposition that the nerve current resembles an electric current, and the axis-cylinder an electric wire ; but this is rather fanciful pathology. Fig. 87. — Vicinity of Locus Cceruleus. blood-vessels. (Taylor.) r, cells ; v, DISSEMINA TED SCLER OSIS. 351 Considering the importance which has been attached to the acute infec- tious diseases as an etiological factor in multiple sclerosis, it would be nat- ural to expect a very early involvement of the blood-vessels in this disease. Such an involvement has been found by Ribbert and urged by Marie. Taylor, the most recent author on this subject, whom I am willing to follow in this matter, because the autopsy in his case was on a young subject, proves that in the earlier stages of the disease there is a distinct increase of the smaller blood-vessels and capillaries. (Fig. 87.) The specimens examined also ex- hibited small hemorrhages and migrating white blood-corpuscles. In some of the vessels there was a slight thickening of the walls and an increase in the number of the nuclei. The perivascular spaces were dilated. The same changes were seen in a second case of Taylor's. In a third case there was absolutely no change in the blood-vessels, but in the second and third cases the disease had lasted longer, and it is, therefore, doubtful whether similar changes in the blood-vessels might not have been present also in these cases in the earlier period of the disease. Taylor thinks that we are not justified in connecting these vascular changes with the sclerotic process, for the patches are not distinctly related to the diseased vessels. Furthermore, vessels are often entirely normal in degenerated areas, and in some cases the disease of the blood-vessels is wholly wanting, however wide-spread the lesions may be. Further autopsies, particularly of cases that have been of short duration, are needed to help decide these various points. Atypical Forms and Differential Diagnosis. — It would be an easy matter to enumerate a large number of atypical forms of multiple sclerosis, for the clinical symp- toms as developed by Charcot are not distinct in many cases which have, however, a direct relation to this disease, repre- senting either early forms of the same, or a peculiar local- ization of the sclerotic patches. One variety of multiple sclerosis with bulbar symptoms bears a close resemblance to amyotrophic lateral sclerosis. But in children the com- bination of symptoms giving rise to such difficulty in diag- nosis is very rare. Then there is the bulbar, or, as Spitzka prefers to call it, " the oblongata type " of multiple sclerosis, in which the usual symptoms are not nearly as well devel- oped as are those pointing to an involvement of the lowest cerebral centres. Difficulties in deglutition, in mastication, and in articulation as well as phonation, are most promi- nent, and for a long time may obscure the usual symptoms of the disease. A hemiplegic form of multiple sclerosis has also been observed in the adult, but I have not yet met with it in 35 2 THE NERVOUS DISEASES OF CHILDREN. children ; nor have I seen an apoplectiform beginning of the disease in children, as sometimes happens later in life. Multiple sclerosis, and forms closely allied to it in children, should not be confounded with transverse myelitis. Acute and chronic forms of myelitis in children are not rare. The acute symptoms and the inflammatory condition upon which they depend disappear promptly enough, but a degeneration is set up which leads to the development of spastic and paralytic symptoms. These might be taken to be symptoms of multiple cerebro-spinal sclerosis unless the history of the patient is carefully considered. The very acute onset and the involvement of the bladder, the persistence of sensory symp- toms for a shorter or a longer period of time, will help to differentiate myelitis and its secondary degeneration from insular sclerosis. From congenital spastic paraplegia the disease can be differentiated by a consideration of the mode of onset, and by the very early appearance of the congenital disease, for insular sclerosis does not generally come on until very nearly the first decade of life is passed. Moreover, in the congenital cases of spastic paraplegia there is frequently some cerebral defect ; and the cardinal symptoms of sclerosis are wanting in these cases. Westphal described a pseudo-sclerosis, a condition closely simulating that of multiple sclerosis ; but it is not an important disease, for to my knowledge it has not been clearly established in any other instance excepting the one reported by Westphal. Cases of hereditary tremor might be mistaken for multiple sclerosis, and I have myself seen one patient, a girl aged twelve, in whom there was much doubt as to whether or not this tremor, which became very much aggravated on voluntary motion, was the first symptom of a multiple sclerosis ; but having observed the patient for fully five years without the addition of any other symptoms referable to a multiple sclerosis, I am confident that the girl is suf- fering from an hereditary form of tremor, and have since learned that her father and an uncle have suffered from the same disease since very early childhood. The question also arises in some cases whether an insular sclerosis or an hysteria is present, but a consideration of the symptoms will soon remove all doubts as to the diagnosis. Nystagmus, scanning speech, and intention tremor are exceedingly rare in hysteria, while the sensory disturbances of hys- ' teria are too typical to be mistaken for the slight subjective and objective dis- turbances of sensation that occur in multiple sclerosis. In the adult the chief point in differential diagnosis is considered to be that between multiple sclerosis and paraly- sis agitans. To this we need pay comparatively little atten- tion, since paralysis agitans is distinctly a disease of later years, and is rare indeed before the age of forty ; but I can- not forbear referring to the case of a patient, still under my DISSEMINA TED SCLER OSIS. 353 care, who, although but fifteen years of age, presented all the symptoms of genuine paralysis agitans. As will be seen from the accompanying cut, the position of the body, the expression of the face, the position of the hands, are typical of paralysis agitans, yet the symptoms have changed in such a way that at the present time they point to a multiple sclerosis rather than to a shak- ing palsy. (Fig. 88.) The patient fell at the age of five years, and had an attack of convulsions with loss of consciousness, which was followed by a slight weakness of the legs, but from this condition he rallied rapidly enough, and was entirely well, exhibiting good physical and mental de- velopment. He was bright in school, and perfectly well until the age of fifteen, when he met with another accident ; he fell from a carriage and was badly fright- ened. The first symptoms he noticed were pains around the left ankle, then a shak- ing of the left leg. Soon thereafter the arm began to shake. Five months later he noticed the same symptoms on the right side of the body. On my first examination of the boy, three years after the accident, I found him well nourished ; the muscles in good condition, the color of the skin normal, but considerable vasomotor disturb- ances. He exhibited the mask-like ex- pression of the face, and his speech, as well as the rhythmical tremor of the hands, legs, and head, were exactly those of a patient suffering from paralysis agi- tans. Soon another set of symptoms ap- peared, which proved to me that this " senile " disease, when occurring in a young individual, was subject to peculiar modifications, which allied it much more closely to multiple sclerosis, a disease common in earlier life. While under observation he developed characteristic nystagmus, scanning. 23 Fig. 88.— Patient with Paralysis Agi- tans, who also exhibited some Symp- toms of Disseminated Sclerosis. 354 THE NERVOUS DISEASES OF CHILDREN. and tremulous speech. All the reflexes became greatly exaggerated. The typical tremor- of paralysis agitans, involving the head, lips, and tongue, and the extremities, remained unchanged. He still exhibits the propulsive move- ment so characteristic of paralysis agitans. The hands have the position of shaking palsy, and the contractures are much like those seen in the senile form. Motion is almost impossible, and with the symptoms pointing in part to multiple sclerosis, and in part to paralysis agitans, he presents a very unusual appearance.* Prognosis. — Like many other chronic disorders of the central nervous system, multiple sclerosis is not a rapidly fatal disease. It may run a continuous course for years Avith- out seriously endangering life, but any intercurrent disease is likely to lead to a fatal termination. At all events, everyone will acknowledge that it is rare to see cases of multiple scle- rosis that have exceeded the age of forty-five or fifty years, and as for those beginning very early in life, the facts are altogether too few to warrant us in stating that such pa- tients live much beyond the middle period of life. The danger to life is greatest in those cases in which the ob- longata type predominates. The invasion by disease of the vital centres might lead to early death. The invasion is, however, a very gradual one, and sudden death need not be looked for. In those cases in which such a termination is threatened, periods of asphyxia, of aphonia, and other symptoms of vagus disturbance would occur, and would give warning of the terminal possibilities. The disease is absolutely incurable, although remissions occur in which all the symptoms excepting the tremor or the nystagmus disappear, and in some cases the disease may come to a complete standstill for a period of several years. In this respect multiple sclerosis is not unlike tabes. My notes of the cases beginning in early life would go to show that the disease is even more slowly progressive than it is in the most favorable adult cases. Treatment. — Keeping in mind what was said relative to the prognosis of the disease little can be expected from our attempts at treatment. But if we cannot cure the dis- ease we can at least secure greater comfort for the patient. As the tremor is most marked when the patient is ac- * Schultze has reported a similar case in Virchow's Archiv, Bd. LXVIII. DISSEMINATED SCLEROSIS. 355 tive — and the disease is exhausting at best — it is wise to keep the patient in bed for a prolonged period of time. The rest cure I have found as efficient in these chronic organic diseases of the central nervous system as I have in the treat- ment of purely functional troubles. It is often surprising to note how much benefit, and, above all, how much comfort, patients derive from a complete rest when they have been attempting to go about from place to place, often from phy- sician to physician, with their weak and shaking limbs. With this rest treatment I am in the habit of com- bining mild hydro -therapeutic procedures. Generally a tepid bath in the morning, followed by lukewarm and successively colder douches down the spine, or by the use of the drip-sheet. At night it is well to give a tepid bath without douches, as the former contributes much to the sleep and the general contentment of the patient. The galvanic current may be employed to advantage. Stabile currents of 10 to 15 milliamperes may be passed down the spine and through the extremities. The faradic current should, however, be avoided, as it tends to increase the spastic rigidity of the muscles. Massage is also in or- der in these cases, if intelligently adimnistered. Spastic rigidities yield a little to such manipulations, and accord- ing to the recent investigations of Mitchell and his son, the general condition of the patient is improved by massage. If treatment is directed in this way and adapted to each individual case it will be far more satisfactory than send- ing the patient away to various springs, where a general routine treatment is adopted and the patient subjected to it, whether he suffers from rheumatism, gout, syphilis, or what not. Various drugs have been employed and recommended in cases of multiple sclerosis. Among these nitrate of silver, the iodides, and mercurials are the most prominent. I am confident that the first never does any good excepting pos- sibly to allay pain, and the last two will not be needed, for it is very certain that multiple sclerosis is rarely developed on a syphilitic basis, and there is no indication for the use of resorbents. 356 THE NERVOUS DISEASES OF CHILDREN. BIBLIOGRAPHY. Charcot: Lecons, etc., vol. i., p. 399, 1892; On Visual Troubles, Semaine Med., February 14, 1891. Freund : Arch. f. Psych., etc., 1891, vol. xx. Grassetet Rauzier : vol. i., p. 796. Kahler u. Pick : Prager Vierteljahrsschrift, 1879. Marie : Maladies de la Moelle, p. 108. Mensi : Riforma Medica, November, 1892. Nolda : Arch, fur Psych., vol. xxiii. Oppenheim : Berl. kl. Wochenschrift, 1888. Pritchard : Keating's Cyclopaedia. Ribbert : Virchow's Archiv, vol. xc. Sachs : New York Medical Journal, September 1, 1894. Schultze : Virch. Arch., vol. lxviii., p. 120. Spitzka : Article on Scleroses of Spinal Cord, in Pepper's System, vol. v. Taylor : Zeitschr. f. Nervenheilk., vol. v. Uhthoff : Arch. f. Psych., vol. xxi. Westphal : Arch. f. Psych., vol. ix. CHAPTER XX. COMPRESSION OF THE SPINAL CORD— POTT'S PARALYSIS. Injury to the cord may be due to specific diseases in the bone, to compression by an aneurism or to growths orig- inating in the membranes. These conditions are very rare, however, in children, and whenever the symptoms point to compression of the cord, caries of the bone may be taken to be the primary trouble. Pott's disease of the spinal column begins so insidiously that the recognition of it and of the symptoms resulting from compression are reached frequent- ly by exclusion only. While the disease may develop at any period, it is more common in childhood and in early life ; it is the result of a tubercular or scrofulous diathesis ; and evidence is easily adduced in the majority of cases of the existence of such diathesis in the same family and in the same or in preceding generations. Slight traumatic injuries are often sufficient to bring about marked disease of the bone, but evidently only in those children who are predis- posed to such disease, so that here as elsewhere the trau- matic incident simply helps to bring out the latent tendency to disease. There is often a period of weeks or months between the time of injury and the first symptoms of the disease. In consequence of the caries of the bone, the bodies of the vertebras become softened and are displaced, two or three of them, or even more, forming a marked angular curvature. Such angular curvature may in rare cases be the result of a septic process, in which case an abscess may have formed in the bone and caused the softening. Gen- erally the bodies of the vertebrae become diseased and the intervertebral substance undergoes inflammatory changes as well. The pressure upon the softened parts is sufficient to account for the deformity. 358 THE NERVOUS DISEASES OF CHILDREN. The deformity resulting from the disease of the bone is of such a character that a sharp angle is usually formed at one point, at which the cord is generally much compressed. In some instances the cord becomes quite thread-like. It is remarkable that the cord may sustain a very large amount of compression before its functions are seriously interfered with, or the symptoms of disturbed function appear. This accounts for the fact that in many instances the angular curvature may be extreme and yet neither paralysis nor anaesthesia is observed during life. Lateral displacement may occur without its having any influence upon the cord. Very naturally the spinal cord suffers under these condi- tions, and the roots emerging or entering through the in- tervertebral foramina are compressed by the thickened sheaths or by other inflammatory products. This accounts for the frequent occurrence of pain in these cases, and for the occurrence of paralysis in cases in which the cord itself shows tolerably normal structure. The causes of injury to the spinal cord in the subjects of Pott's disease are twofold : first, the cord is actually com- pressed in many cases, and, secondly, it suffers from the in- flammatory products gathering between the cord and the bony parts. The latter cause is often quite as active as the former. The changes in the cord itself will vary very largely according to the degree of compression. The cord is somewhat altered, the normal white having given way to a grayish or reddish-gray tint. The cord is less hard than under normal conditions, both these changes being clearly due to inflammatory processes. It has been found by Schmauss and others that the amount of inflammation of the cord is not always in keeping with the degree of com- pression. Recent French authors have attached consider- able importance to an oedema of the cord produced by compression of the veins as they issue from the cord. The oedema is subject to fluctuations ; and a diminution of the same may be responsible for an improvement in the pa- ralysis. The symptoms of Pott's paralysis often set in long be- fore there is any possibility of actual compression. In those cases we must suppose that the symptoms are due to a COMPRESSION- OF THE SPINAL CORD. 359 pachymeningitis constricting the spinal roots and blood- vessels ; the occurrence of rapidly developing spinal abscess, associated with painful paralysis, and the disappearance of the paralysis as soon as the abscess is emptied, lend support to this view. By degrees an extension of the inflammatory process takes place into the substance of the cord. The. myelitis as it occurs Avith Pott's disease shows a general in- crease in the interstitial tissue, with proliferation of the various cells and degenerative changes in the more impor- tant nerve elements. Granular corpuscles, corpora amylacea, and globular masses of myelin are found in sections that are properly hardened. The gray matter may have become almost indistinguishable from the white, and the larger ganglion cells may have entirely disappeared from the for- mer. In addition to these changes the usual ascending and descending degenerations will occur, as from any other focus of inflammation in the cord. The changes in the pe- ripheral nerve-roots will be those that would be expected from actual compression and inflammation. The interstitial tissue will be increased, the nerve-fibres themselves will be wasted, and the axis-cylinders will show the usual changes of degeneration. Symptoms. — The chief symptoms in this disease are a very gradual development of a spastic paralysis, generally of the legs and possibly of arms and legs ; the reflexes are increased, and radiating pains are present at a very early period of the disease. The symptoms often resemble those of a dorsal myelitis, since Pott's disease attacks the dorsal region more frequently than any other. If other regions of the spine are diseased the symptoms will vary as the)' would in the various forms of myelitis, but root symptoms will always play a very prominent part. The symptoms due to compression of the root - fibres will also help to differentiate between a myelitis following Pott's disease and the ordinary traumatic or non-traumatic myelitis. The compression of the sensory nerve-roots causes seyere pains. It may also interfere with normal sensation, and if both these functions are disturbed, an anaesthesia dolorosa may be well developed and associated with the paralysis. Be- fore the condition of anaesthesia is established a transitory 360 THE NERVOUS DISEASES OF CHILDREN. hyperesthesia may have existed. The irritation of the motor root-fibres would lead us to expect a condition of involuntary contractions of the muscles, but, as a matter of fact, these are not so frequent, and probably because the ventral gray matter of the cord, being nearest the verte- bral bodies, is affected at an early day ; and since the func- tion of this gray matter is impaired, irritation of the an- terior roots connected with it will not be able to exhibit symptoms of irritation. According to the location of the disease in the bone the symptoms may for a time remain unilateral; but, as a rule, the disease spreads rapidly enough to bring about a bilateral, and often symmetrical, set of symptoms within a very short period of time. Fur- thermore, as the disease progresses the paralyzed parts may become atrophied, the vesical and rectal reflexes may be impaired, and we then have all the ordinary symptoms of myelitis, even including bed-sores and the usual trophic disturbances of the skin. The course of the disease may be inferred from the ra- pidity with which all these symptoms are developed. If the disease is of a slowly progressive character, the symp- toms will naturally be developed one by one, whereas if the bone disturbance is of a more pernicious and rapidly pro- gressive character the symptoms of a complete myelitis will be reached at a very early day.* Diagnosis. — Paralysis due to Pott's disease must be distinguished from subacute or chronic myelitis. This dis- tinction is not always an easy one to make. It is reached most readily in those cases in which the course of the dis- ease is a very slow one and the usual causes leading to a transverse myelitis are wanting. It is rendered probable by the coexistence of pain with the paralysis, and above all things by the discovery of tenderness to pressure over the spinous processes. These same symptoms might occur in cases of tumor of the cord, but the unilateral development of symptoms in the latter disease will soon lead to a safe differential diagnosis. (See next chapter.) The earlier symptoms of a Pott's paralysis may simulate the condition * In some cases, like the one reported by Daxenberger, the caries may be latent for years, before spinal-cord symptoms are developed. COMPRESSION OF THE SPINAL CORD. 36 1 of spastic paraplegia, either of the congenital order or of that form due to specific disease. In several cases, under the author's care, the symptoms of Pott's paral- ysis were developed long before any actual evidence of disease of the verte- brae could be elicited. Whitman also states that in a number of cases the paralysis precedes the deformity. The diagnosis must at times be reached by exclusion ; but the condition may be suspected in cases of a gradually de- veloping spastic and painful paralysis, in which there is no trace of traumatic injury, and no history of any acute infectious disease immediately preceding the onset of the symptoms. The age of the patient and his general appearance will also help to establish a differential diagnosis. Prognosis. — The progress of the paralysis will depend mainly upon the development of the disease in the bone. If the disease has lasted long enough to produce complete compression at one point, the spinal-cord symptoms may per- sist during life, although the bone disease be checked or re- covered from. In other cases the course of the spinal-cord disease may be checked, although the disease of the bone steadily progresses. With the subsidence of the inflamma- tion the sensory symptoms may diminish, but the condition of paraplegia may continue years thereafter as a natural re- sult of the changes that have been set up in the spinal cord. In a number of cases, however, there is a proportionate de- velopment between the disease of the bone and the disease in the spinal cord. In the more severe cases the paralysis re- mains unaltered, or is steadily progressive, and all those complications arise, such as bed-sores, cystitis, and the like, which help to bring about a fatal issue in many cases of my- elitis. In the milder cases recovery is to be expected, and many a child who has not been able to walk for years during the active period of caries has attained a tolerably normal gait in later life. On the whole, the motor symptoms are much more persistent than the sensory. The prognosis is much more grave in the cases of cervical Pott's disease than in those occurring in the lower vertebral reo-ions, for rea- sons which can be readily understood. Treatment. — In the treatment of paralysis resulting from Pott's disease surgical methods should be resorted to 362 THE NERVOUS DISEASES OF CHILDREN. at the very beginning. If the spinal cord is to recover, the tubercular process in the spinal column must be checked first of all. The old method of absolute rest should be em- ployed, and tonics, such as cod-liver oil and iron, should be given. I do not wish to enter into the discussion as to which of these measures, the rest or the tonics, is more impor- tant. Some will be cured by the one, and some by the other, method, but I have not the slightest doubt that if all these measures are employed conjointly the result will be more satisfactory. In addition to rest, absolute immobility should be secured, either by forcible extension in bed, or by the application of a plaster-of- Paris jacket. The plaster-of- Paris jacket is not so useful during the period of rest as it is during the period of partial recovery, in which it is desira- ble for many reasons that the child should be taken out of bed and enabled to walk in the open air. I am very certain that the application of counter-irritants, which is still recom- mended by many, is of no use, and simply helps to increase the tortures that these children suffer. Of late years active surgical interference has been suggested in these cases by Macewen, Horsley, and others, but the wisdom of this may still be doubted. The surgeon's skill should, at all events, be restricted to those cases in which the clinical symptoms prove that there is evidence of compression at any one spe- cial point by inflammatory products or by displaced bone. If the child is strong enough to tolerate such surgical pro- cedures, the removal of these products of inflammation may be attempted ; but it is well to caution the physician who may be eager to try new surgical procedures that chil- dren are less tolerant to spinal and cerebral surgery than adults. After surgical measures have been given a fair trial, the physician and neurologist will be called upon to remedy the paralytic condition. Some assistance can be given by the orthopedic surgeon, who, by the ordinary tenotomies, may enable the child to use its feet to greater advantage. His treatment will be supported in every case by the discreet use of electricity and massage. The ordinary blood and nerve tonics will help very much more than the iodides or mercurials, but these should be given in the earliest COMPRESSION OF THE SPINAL CORD. 363 stages of the trouble. I have seen a favorable result from the latter in one case in which the process was suspected to be gummatous and not tubercular. BIBLIOGRAPHY. Charcot: Lecons, etc., 1873. Daxenberger : Zeitschr. f. Nervenheilkunde, vol, iv., p. 136. Erb ; Ziemssen's Cyclopaedia, vol. xi. (literature to 1878). Grasset et Estor : Revue de Medecine, Feb., 1887. Kahler: Zeitschr. f. Heilkunde. Prag, 1882. Kahler u. Pick : Arch. f. Psychiatrie, vol. x. Leyden : Klinik der Rickenmarkskr., vol. ii., p. 151. Schmaus : Monograph. Wiesbaden, 1890. (Contains a very full summary of cases with autopsies.) Whitman : Differential Diagnosis of Pott's Disease in Childhood, New York Polyclinic, May, 1893. CHAPTER XXI. TUMORS OF THE SPINAL CORD AND ITS MENINGES. The study of tumors of the spinal cord was at one time consdiered to be of small practical value on account of the rarity of the affection ; but far greater interest attaches to it now from the fact that the removal of these tumors has been rendered possible by the recent advances in sur- gery. A few years ago Mills and Lloyd collected and analyzed fifty cases of cord tumors ; fourteen per cent, of these were under twenty years of age. Of these four oc- curred before the age of ten and three between ten and twenty. Causes. — The actual cause of tumors of the spinal cord can be deter- mined as little as in the case of cerebral tumors. They are rarely primary, and are commonly due to some constitutional diathesis, such as tuberculosis or syphilis, or are the metastatic products of malignant tumors in other organs of the body. The tendency to proliferation of nerve-tissue is not so marked in the spinal cord as in the brain of the child. Gliomata originate in the vicinity of the central canal, and here are of special interest, as they often break down and lead to the formation of cavities, and hence have given rise to the discus- sion whether these cavities are invariably the result of such disorganization of a neoplasm, or whether the condition of syringo-myelia may be the result of congenital enlargement of the central canal. Small hemorrhages and em- bolism, though much rarer than in the brain, may give rise to cystic forma- tions, but among cystic growths those due to parasitic infection, particularly to the echinococcus, are the most common. As in the case of brain tumor, traumatic injury has been declared responsible by many for the occurrence of spinal tumors. It is doubtful, however, whether traumatism plays any further role than that either of eliciting a latent tendency to such disease, or of merely calling attention to the symptoms of the disease which may have antedated the injury. Symptoms. — The symptoms of tumor of the cord might be deduced from a consideration of the disturbance of TUMORS OF THE SPINAL CORD AND ITS MENINGES. 365 function due to a very gradual invasion of the cord. Thus in many cases the symptoms are strictly unilateral for a time, and become bilateral with the extension of the disease from one part of the cord to the other. The symptoms will naturally vary also according to the seat of the disease, ac- cording to the more or less rapid destruction of the cord, and according to the development of the tumor in the meninges or in the spinal cord itself. It is of the greatest importance to distinguish between extra-medullary and me- dullary tumors. Extra-medullary tumors are characterized by the early appearance of root symptoms, more persistent pain in the back, and by sensory disturbances which point to compression of the posterior root-fibres ; by paralysis and atrophy if the growth is on the ventral surface. These distinctions will not hold good if, as in a case of my own, the intra-medullary tumor starts near the entrance of the posterior root-fibres and actually compresses these. In some cases, however, the disproportion between the sen- sory symptoms and the paralysis will direct attention to the seat of the tumor outside of the spinal cord, particu- larly if all symptoms pointing to involvement of the cen- tral gray matter are absent. The focal symptoms in ex- tra medullary, as well as in intra-medullary tumors will vary greatly according to the seat of the tumor. There may be but slight sensory disturbances in a single area or in one limb; the sensory disturbances may be uni- lateral, and these may or may not be associated with par- tial paralysis, either unilateral or bilateral ; or there may be crossed sensory and motor symptoms. Any set of spinal, and more particularly hemi-spinal symptoms, whether of a motor, sensory, or vasomotor order, if they be slowly pro- gressive, are suspicious of tumor. There will be additional reason to suspect tumor if there is evidence of a distinct constitutional diathesis. All the symptoms of various spinal-cord affections may be simulated by tumor, accord- ing to the part of the spinal cord affected, and in this respect the symptomatology of tumors may be quite as varied as that of syphilis of the spinal cord. If we endeavor to analyze the symptoms in detail, we find that disturbances of sensation are by far the most com- 366 THE NERVOUS DISEASES OF CHILDREN. mon. If the tumor is in the cervical region, the pain radi- ates into the upper extremities, and generally into definite parts of the brachial plexus ; if in the dorsal region, the pain is relegated to the region of the ribs, the thorax, or the abdomen ; if in the lumbar region, the pain is described as limited to the various parts of the lumbar or sacral plexus. Whatever the level of the lesion may be, the sensory dis- turbances may be unilateral for a time and gradually be- come bilateral. Vasomotor disturbances occur, similar to those in peripheral neuritis ; the skin becomes glossy, livid, and painful. With these symptoms muscular wasting and paralysis will soon be associated. As the disease progresses the hyperalgesia or the paresthesia gives way to complete loss of sensation, the paralysis becomes complete, and the vasomotor disturbances increase, and serious trophic dis- turbances may set in. The sensory changes may vary in still another respect. Since the tumor need not invade all sensory fibres at once, some may remain entirely intact, while the function of others becomes seriously impaired. Thus dissociated sensation may be present in some cases, but is rarely as distinctly developed as in the case of syringo- myelia, which, as we have seen above, may or may not be connected with the formation of a central neoplasm. Ac- cording to well-known physiological laws, and to the old- time teachings of Brown-Sequard, one would expect in many of these cases of tumor to find distinct symptoms of unilateral lesion of the cord, namely, paralysis in one mem- ber or in one-half of the body, with loss of sensation in the opposite half ; but this, curiously enough, is not often the case. The paralysis will be developed in accordance with the ordinary laws relating to spinal-cord affections. The mus- cles represented in the segment or segments involved by the neoplasm (see tables in Chapter I.) will not only be paralyzed, but will also undergo atrophy, while the mus- cles represented in lower segments will show spastic forms of paralysis without any atrophy. The changes in electri- cal reactions will also vary in accordance Avith these con- ditions, the only muscles exhibiting distinct reaction of degeneration being those governed by cells in the affected TUMORS OF THE SPINAL CORD AND ITS MENINGES. 367 segments. Spasmodic contractions, the ordinary rigidities and contractures, will be observed in these cases under the same conditions as in cases of myelitis. All the symptoms will be largely influenced by the fact that an active my- elitic process is started up sooner or later in the vicinity of most of the tumors. This is particularly true of tubercu- lar and gummatous deposits, whereas other forms of tumor may exist for some period of time without seriously affect- ing the tissue in their vicinity. Paralysis of the sphincters of the bladder and rectum will occur in cases of tumor as in cases of myelitis. In the case of tumor in the lumbar region, absolute paralysis of the sphincters and retention are the natural results, while if the tumor is above this level incontinence will be the more frequent condition ; but in the later stages retention with overflow is the rule. The conditions of the sphincter ani will vary in very much the same way. As tumors are more frequent in the cervical and dorsal regions, I propose giving the condensed history of a case, which I had occa- sion to observe some years ago, of cervical tumor of the cord, although this occurred in an adult and not in a child, but the age of the person would have no effect upon the character of the symptoms. The patient's symptoms were, pains and weakness in his left arm, in- ability to move the fingers, and glossy appearance of the left hand. There was no history of syphilis nor of tuberculosis in the family. Four weeks before I saw the patient he felt what he termed rheumatic pains in the left shoulder ; these moved down to the left arm. The hand grew weaker after the lapse of a week or more, and then the fingers became puffy and the skin glossy. The examination revealed the following conditions : The left arm could not be raised as readily as the right ; the fingers were slightly flexed, and voluntary extension or further flexion was impossible. There was hyperassthesia of the entire forearm and hand, most marked over the distri- bution of the ulnar nerve, and particularly over the outer dorsal surface. The mere touch with the finger was painful. A prick was so disagreeable that he could not bear to be touched with an cesthesiometer. Cold water dropped on the arm produced excessive pain. The skin was swollen be- tween the joints of all the fingers of the left hand, and there was a peculiar cedematous appearance of the skin of the entire hand, including the fingers. The electrical conditions were normal. There was no ataxia in the lower extremities ; no inco-ordination of movements in the right arm. All super- 368 THE NERVOUS DISEASES OF CHILDREN. ficial reflexes were gone except a very slight scrotal reflex on the right side. At this examination the only other symptoms were a slight dragging of the left foot, an increase of both knee-jerks, and absence of ankle clonus on both sides. Passing over the change in the symptoms during the first few weeks, I will merely state that the paresis in the left leg gradually increased. There was continued hyperesthesia of the whole leg for several weeks, and a gradual weakening of the right leg as well. The abdominal muscles also were weakened so that he could not press properly when going to stool. Incontinence of urine gradually developed. For some time there was no disturbance of sensation in the right half of the body. About one month after the first examination the patient began to exhibit a slight cough, and was annoyed by the lack of sufficient muscular power to expectorate freely, but the physical examination revealed no distinct signs of disease excepting a slight catarrhal condition. The paralysis increased rapidly, so that the patient was unable to leave his bed. There was also some tenderness over the seventh cervical vertebra. The right hand gradually grew weaker and sensory disturbances appeared in the right forearm, but his right hand did not show the trophic disturbances so prominent in the left. The hyperses- thesia which had existed at the beginning gradually gave way to an increas- ing anaesthesia. The anaesthesia, six weeks after the first examination, in- volved both sides of the body, the only parts retaining normal sensation being the neck, face, and head The various forms of sensation were very much disturbed. Heat could not be appreciated, but cold was painful for a time. The muscular sense was impaired both in the left and in the right leg, but in the right arm muscular sense was not disturbed. The only further changes noted were an increase in the paralytic symptoms in the left upper extremity, though the patient retained considerable power in the pectoralis, the deltoids, biceps, and brachialis during the entire disease. The temperature was only little above normal. The pulse was small and accelerated, often about 1 20. Respiration varied very much, generally about 25. The urine was free from sugar and albumin. There was no history of night-sweats, but the patient stated of his own accord that he was frequently subject to unilateral sweating of the right side. The patient died very suddenly, exactly six weeks after my first examination. The autopsy revealed a round tumor of the size of a hazel-nut between the sixth and seventh cervical seg- ments, pressing closely upon the emerging posterior fibres without displac- ing these and extending inward as far as the median line. At its caudal end the tumor had exceeded the median line by a small fraction of an inch. The tumor proved to be a typical solitary tubercle. In addition to this we found that the cord between the seventh cervical and the fourth dorsal seg- ments was practically a diffluent mass revealing no trace of structure. There were also minute tubercular deposits discovered in both lungs, and in both apices there were incipient cavernous spaces. The variation of symptoms in other cases would depend solely upon the different site of a tumor, but the general course and variability of the symp- toms are well illustrated by the above case. TUMORS OF THE SPINAL CORD AND ITS MENINGES. 369 Pathology. — The morbid anatomy of tumors of the cord differs very little from that of tumors of the brain. The central nervous system is in this respect a unit, and the same growths which are apt to occur in the brain occur, though with lesser frequency, in the spinal cord. In the cases of Mills and Lloyd, only four were of a tubercular character, while twelve were either sarcomata or gliomata. Parasitic growths occurred in three cases of the fifty, syph- ilitic growths in five, and the remainder were distributed among various kinds of neoplasms, such as myxomata, fibro- mata, carcinomata, and so forth. Neuromata of the cauda equina must be included in the list. The largest collection of cases of solitary tubercle of the spinal cord has been made and carefully analyzed by Dr. Herter. His experi- ence proves that the disease is one of early life, twenty of the twenty-six cases occurring before the age of thirty-five. A few authors have reported cases of tubercle which they supposed to be primary ; but, as in the case reported by myself, although it was difficult to prove any other focus of tubercular disease during life, such focus of disease evi- dently precedes the spinal disease in every instance. Spe- cific infiltration of the spinal cord is, on the whole, more fre- quent than the same infiltration of the brain. To be sure, relatively few cases of this description are on record, but I am certain that more careful examination will hereafter re- veal a larger number of gummatous deposits, if not gum- matous growths, in different parts of the cord, associated with the specific meningo-myelitis, which is, as has been stated in another chapter, the most frequent form of syphi- litic disease of the cord. Tumors of the cord may occa- sionally erode the surrounding bony parts and may work their way to the surface, but this is very much rarer indeed than a similar invasion of the skull in cases of brain tumor. The reverse course, from the bone to the cord, may also occur, but is a great rarity. Diagnosis. — Tumors of the spinal cord are to be differentiated from hemorrhage into the cord, from spinal caries with Pott's paralysis, from pachymeningitis cervicalis, transverse myelitis, syringomyelia, neuritis, and from traumatic injuries of the cord. In the case of hemorrhage the paralysis and other symptoms resulting 24 370 THE NERVOUS DISEASES OF CHILDREN. therefrom appear with great suddeness. The condition is one of extreme rarity, and in many of the cases leads to complete paralysis, with all its com- plications, and to death in a relatively short period of time. A confusion can, therefore, exist only in the first few days of the disease, and under such cir- cumstances the suddenness of the onset would argue strongly in favor of hemorrhage. Spinal caries, with the resulting paralysis, may occasionally present symp- toms similar to those of spinal tumor. In both, pressure symptoms are pres- ent, but in the case of caries of the bone the symptoms are not nearly so intense, as a rule, as in those due to the presence of neoplasm. The chief reliance in differential diagnosis must, however, be placed upon the detection of bone trouble in the case of spinal caries, although, as we have seen, such evidence does not always precede the onset of pressure symptoms. The more rapid course of the symptoms in spinal tumor, and the negative results of treatment, will also help to differentiate the two conditions. In caries of the vertebras, symptoms pointing to the ventral (anterior) surface are a little more frequent than in tumor. As between pachymengitis cervicalis and tumor of the cervical region, there are but few points of differential diagnosis, and the diagnosis will have to rest very largely upon the excessive rigidity, the very typical distribution of the paralysis in the hand, and the more acute pain in cases of pachymenin- gitis than in those of tumor. In tumor, moreover, the symptoms are more apt to be unilateral for a time, while those of pachymeningitis are, as a rule, bilateral and symmetrical. Some of these same points of differential diagnosis will help us also to distinguish between transverse myelitis and tumor of the cord, the chief points of difference being these : In myelitis the onset is much more rapid than in tumor, there is less pain, and the entire cross-section of the cord shows symptoms of involvement very much more promptly in myelitis than in the majority of cases of neoplasm. A doubt as to which of these two conditions is present can exist in the first few days or weeks only ; after this the general course of the symptoms will determine the character of the morbid process. Syringo-myelia is closely related to tumor of the cord ; there is doubt, in fact, whether some, if not many, of the cases of syringo-myelia do not repre- sent primary neoplasm of the cord, with secondary enlargement of the central canal and a breaking down of the spinal tissues. But the gliosis which may or may not be the starting-point of a syringo-myelia, takes an extremely chronic course, and it is, therefore, by this fact alone, as well as by others, that the two diseases can be sufficiently distinguished from one another. In syringo-myelia the symptoms are often strictly bilateral, or symmetrical, and the disturbances of sensation (the loss of pain and temperature sense, while the tactile and muscular sense are preserved) are very typical of syringo-my- elia and are rarely met with in cases of spinal tumor. In syringo-myelia there is also evidence in many cases of a slowly ascending or descending morbid process and such slow ascent or descent is not common in cases of tumor. The differential diagnosis is one of great importance, for the one TUMORS OF THE SPINAL CORD AND ITS MENINGES. 37 1 disease is compatible with prolongation of life, while the other is rapidly de- structive. (See end of this chapter.) The symptoms of neuritis may for a time obscure those due to tumor. In cases which I have observed the first symptoms that appeared were those of a neuritis involving various branches of the brachial plexus. These were due to compression of the spinal roots, and are naturally present in those cases in which the tumor begins either in the meninges or near the surface of the cord. The early appearance of complete paralysis and the develop- ment of other symptoms, particularly those pointing to interference with the vesical and rectal reflexes, will argue in favor of neoplasm and not of a sim- ple neuritis. The rapid progress of the symptoms in the case of tumor, the entire absence of fever in most of them, will also point to tumor rather than to neuritis. The presence of sensory and paralytic symptoms in both halves of the body, or in the lower as well as upper extremities, without involvement of the bladder or rectum, may be considered as pointing to a multiple neuritis rather than to tumor. A localized specific meningitis may at times simulate tumors of the cord, and, as was indicated before, the frequent association of gummatous de- posits with such specific meningo-myelitis may present unusual difficulties of differential diagnosis. In one case under my observation for a period of several months, it was impossible to differentiate accurately between these two conditions ; and unfortunately the result of treatment is not to be de- pended upon, for even in cases of a distinctly specific character the symptoms do not always yield to antisyphilitic treatment. Traumatic injuries of the cord may present symptoms resembling those of tumor. But the history of the case, and the extreme painfulness over the spinous processes, will prevent the possibility of confusion between the two. y Prognosis. — In spite of some few surgical successes tumor of the cord is an extremely grave disease. Spinal growths are secondary to malignant disease in other parts of the organism and help to intensify the gravity of the dis- ease elsewhere. In the case of tubercle, sarcomata, and car- cinomata the prognosis is absolutely bad, while it is only a little better in cases of gummata. The duration of the disease varies according to the site of the tumor and according to the morbid character of the new growth. Tumors of the cervical region are, on the whole, more rapidly fatal than those in the dorsal or lumbar region. Tubercles, carcinomata, and sarcomata, take a more rapid course, as a rule, than the other forms of neoplasm. Treatment. — Tumors of the spinal cord and its me- ninges cannot be sensibly affected bv drugs. There is a 372 THE NERVOUS DISEASES QF CHILDREN. possible exception in the case of gummata ; yet, as was intimated above, the effect of specific treatment in these cases is, to say the least, extremely doubtful. I would ad- vise, however, that every case of tumor of the cord be given the benefit of the doubt, and that active specific treatment by iodides and mercurial inunctions be attempted. If these drugs avail little, no other drug need be exhibited. Much has been expected of surgical treatment ; but, as in the case of cerebral tumors, our hopes in this direction have not been realized. Extra-medullary growths are the only ones that we can expect the surgeon to remove. Suc- cessful cases of this description have been reported by Gowers, Horsley, Laqueur, and others.* The cases most favorable for operation are those which occur near the sur- face of the cord or in the meninges, and those in which the operation is done at a relatively early day, before the neo- plasm has invaded the cord or has been followed by sec- ondary myelitis and secondary degenerations. The case published by Laqueur, in which the tumor was found out- side of the dura mater and compressing the cauda equina, was an exceptionally fortunate one, for the tumor was easily found and the effects of compression of the cord were not very marked. The question arises whether extirpation of the tumor should be attempted in cases of a tubercular nature and in those in which gumma is suspected. In tu*bercle of the cord, even the successful removal would in all probability prolong life but very little, so that surgical interference is scarcely warranted ; and yet in a case that is otherwise fa- vorable for operation there seems to me to be sufficient rea- son to interfere, if there are no other symptoms of general tuberculosis. I am in favor of removing gummata if the tumor has resisted, as it generally does, every form of treat- ment, provided such treatment has been carried out relig- iously for a period of from three to four weeks without any sign of improvement. I consider Horsley's advice entirely sound, that if a tumor has resisted every form of treatment for a sufficient length of time, and if the conditions are * Since the above was written, Starr has reported several cases in which the neo- plasm was removed successfully ; but not a single case can be said to have been cured. TUMORS OF THE SPINAL CORD AND ITS MENINGES. 373 favorable for surgical interference, the proper surgical pro- cedures should be resorted to, and that, too, without much delay, for delay is far more serious under these circum stances than prompt and proper surgical interference would be. SYRINCxOMYELIA AND GLIOSIS OF THE CORD. Syringomyelia, or, more properly speaking, " myelosyringosis," is a form of disease that is rare enough in the adult, and still rarer in children. Its symptoms resemble somewhat those of tumor of the cord, of amyotrophic lat- eral sclerosis and of progressive muscular atrophy. As the name indicates, the disease is due to cavity formation in the spinal cord. This may be the result of a congenital abnormality of structure, or it may be due to a glioma- tous process starting in the vicinity of the central canal, and causing a de- struction of tissue. (See Hoffman's articles.) We need not discuss whether the one or the other mode of origin is the more frequent, and on this point opinions still vary. The cavity is, as a rule, largest in the cervical segments, but may be continued up into the medulla and downward into the dorsal and lumbar segments. Symptoms. — Less than fifteen years ago syringomyelia was considered a mere curiosity without any special practical importance ; but the investigations of Schultze and of Kahler have enabled us to recognize the disease by a very definite set of symptoms. The symptoms will naturally vary according to the extent of the cavity ; but as the cavity is generally most developed, and has its beginning in the cervical, region, the symptoms are first observed in the upper extremity and generally around the shoulder. We may divide the symptoms into three groups : The first group consists of trophic disturbances, affecting the skin, its subcutaneous tissues, and the bones. Glossiness of skin, particularly of the fingers, deep fissures, felons — which are sometimes painless — and phalangeal necrosis, with marked distor- tion of the fingers, are the most frequent. Patients also complain of burning, prickling feelings, and of a sensation of numbness. These various disturb- ances are associated at an early date with the second group of symptoms, con- sisting of partial disturbances of sensation. There is either a diminution or a complete abolition of the sense of pain and of temperature, while the sense of touch and of muscular innervation remains absolutely normal. The third group of symptoms includes a progressive atrophy of the mus- cles, beginning in the small muscles of the hand, and gradually involving the forearm, the arm, and the shoulder muscles. The resemblancce to the Aran- Duchenne type of progressive muscular atrophy is very great (see Chapter XXIII.), and it is only by the association of this progressive muscular atrophy with dissociated disturbances of sensation, and with the trophic symptoms described above, that we can differentiate between syringomyelia and progres- sive muscular atrophy, as well as amyotrophic lateral sclerosis. In progressive muscular atrophy of the Aran-Duchenne type, there are no marked disturb- ances in the skin and subcutaneous tissue ; and in amyotrophic lateral sole- 374 THE NERVOUS DISEASES OF CHILDREN rosis there are no disturbances of sensation, and no marked trophic symptoms,, but in this disease and in syringomyelia the reflexes may be exaggerated. In syringomyelia other symptoms occur, which will depend largely upon the extent of the cavity formation. If the cavity encroaches very largely upon the anterior gray matter, there will be a large amount of flaccid atrophy and paralysis, the electric reactions will be disturbed, and the reflexes will be Fig. 89. — Section through Seventh Cervical Segment, showing Gliosis of Cord. (Herter.)* diminished in the parts governed by the affected region. Fibrillary tremor is present in some cases. All these symptoms are generally most pronounced in the upper extremities, since the cavity is greatest in the cervical region, and if the lateral columns in the cervical region are involved, we may expect a spastic paralysis of the lower extremities with increased reflexes, rigidity, and con- tractures. Cutaneous reflexes are, at times, normal, at other times diminished or entirely absent. Hyperidrosis or hypidrosis (unilateral or bilateral) have been reported. In the cervical cases a narrowing of the palpebral fissure and * This and the following figure are reproductions of specimens prepared and pho- tographed by Dr. Herter, and kindly furnished me for use in this book. TUMORS OF THE SPINAL CORD AND ITS MENINGES. 375 contraction or dilatation of the pupils must be added to the possible symptoms of this curious disease. If the affection extends into the medulla and pons, disturbed sensation in the area supplied by the trigeminus is present, together with atrophy and paralysis of the tongue, hoarseness, difficulty of deglutition, and disturbances of the senses of taste and hearing. Other cranial nerve nuclei may be involved ; nystagmus may be present ; polyuria and increased salivation have also been noticed. Pains and rigidity in the upper portion of FlG. 90.— Section through Part of Fourth Dorsal Segment. The central canal is in- vaded by glia cells and surrounded by groups of cells and nuclei resembling those of the ependyma. (Herter.) the spinal column are not infrequent, and scoliosis or a combination of scoli- osis and kyphosis may also be present. Vesical and rectal disturbances are at times superadded. Syringomyelia is practically a disease of adolescence and of adult life, but some of its symptoms can at times be traced back to the second decade of life. The earliest case which I have seen was in a woman of twenty, in whom the first symptoms began at the age of fifteen. It is interesting to note, however, in this connection, that a disease which is intimately related 376 THE NERVOUS DISEASES OF CHILDREN. (at least in its pathology) to syringomyelia, has been observed by Dr. Herter in a child one year of age. Through his kindness I am enabled to insert a summary of his (unpublished) case, which he reported to the New York Neu- rological Society. Three months before the child was examined the right arm and hand began to be weak, and the head drooped forward. The examination brought out the following points : Complete loss of powe^and atrophy of muscles in both arms and shoulders ; the muscles were flabby ; there was slight move- ment of hands and fingers ; rigidity of neck ; the knee-jerks were increased, especially on the right side ; ankle clonus was elicited on both sides ; there was an irregular rise of temperature, ranging between ioo° and 105 F. daily. The child died two weeks after examination. The post-mortem examination revealed considerable enlargement of the right half of the medulla oblongata and of the cervical cord extending to the eighth cervical segment. There was some enlargement from this point to the sixth dorsal ; below this the cord appeared to be normal. In the upper cer- vical segments the nervous structures of the spinal cord were replaced by a new growth ; in the lower cervical region the pressure effects of the new growth were evident. At about the level of the seventh cervical segment the central canal w T as lined with an unbroken layer of columnar ependymal cells, except at the posterior wall, which was broken through by a mass of larger glia cells, which partially filled the canal. (Fig. 88.) The glioma in- vaded nearly the entire cross-section of the cord, but undoubtedly originated in the vicinity of the central canal. The case is not unlike one reported by K. Miura ; Sokoloff described a glioma of the medulla, with cavity formation, in a boy, aged five years. BIBLIOGRAPHY. Glaser : Arch, fur Psychiatrie, vol. xvi., p. 89. Gowers and Horsley : Med. Chirurg. Trans., vol. lxxi., 1888. Hayem : Arch, de Physiologie Norm., etc., 1873. Herter : Journal of Nervous and Mental Disease, 1890, p. 631. Hoffman (Syringomyelia) : Volkmann's series, No. 20, 1891 ; also Zeitschr. f. Nervenheilk. , vol. iii., 1892. (Contains literature.) Kolitz : Wiener Med. Blatter, 1885, pp. 41-44. (On Tumors of Spinal Cord in Children.) Laqueur: Neurologisches Centralblatt, 1891. Lloyd : In American Text-Book of Diseases of Children. Mills and Lloyd : In Pepper's System of Medicine, vol. v., p. 1091. M. Miura : Virchow's Arch., vol. cxvii., p. 435. K. Miura : Ziegler's Beitrage, vol. xi. Sachs: Journal of Nervous and Mental Disease, vol. xiii., No. 11, 1886. Schultze, Richard : Arch. f. Psychiatrie, 1885, vol. xvi., p. 592. Sokoloff: Deutsch. Arch. f. kl. Med., vol. xli., 1887. Thorburn : Surgery of Spinal Cord, Case xlix., p. 168. Walter Vought : New York Medical Journal, June n, 1892. Williams : Deutsches Archiv. f. kl. Medicin, vol. xxv., 1880. CHAPTER XXII. HEREDITARY OR FAMILY DISEASES OF THE SPINAL CORD. Hereditary or family affections of the spinal cord con- stitute an important group of diseases. They are the re- sult of defective development, or possibly of arrest of de- velopment, of various parts and systems of the spinal cord. Of these disorders hereditary ataxy, or Friedreich's disease, is the best known. A few years ago Nonne and Marie de- scribed a type of disease which bears a close resemblance to hereditary ataxy, and for which Marie proposed the term Hereditary cerebellar ataxy (Heredo-ataxie cerebelleuse). In Friedreich's disease the morbid process is restricted to the posterior columns or to the posterior and lateral col- umns ; in Marie's disease there is in all probability a defec- tive development of the cerebellum and of the tracts con- nected with it. Congenital or hereditary defect of the pyramidal tracts also leads to another distinct group of dis- eases which we may term Hereditary spastic paralysis ; and Hoffman has still more recently described an hereditary form of progressive muscular atrophy of spinal origin in which the chief defect was found in the anterior gray mat- ter of the cord. These hereditary diseases suggest a clinical relationship to syphilitic disease of the spinal cord (which may be inherited, though not hereditary) and to some forms of cerebral birth palsies. Moreover, some of these hered- itary affections are distinctly cerebro-spinal, both as regards the distribution of the lesions and the character of the symp- toms. It is no easy task to separate these various forms from one another and to bring order out of chaos. The family form of progressive muscular atrophy will be con- sidered to much greater advantage in connection with the other forms of progressive muscular disease. 378 THE NERVOUS DISEASES OF CHILDREN HEREDITARY ATAXY. — FRIEDREICH'S DISEASE. Hereditary ataxy, or Friedreich's disease, as it is called in honor of the physician who gave the first satisfactory ac- count of it, is a family af- fection. Several genera- tions are often affected by it ; but naturally we may find every now and then that the disease occurs in one or several mem- bers of a family without any history of a similar trouble in the patient's ancestry. It is not nec- essary, therefore, to speak of such cases as non- hereditary forms of Fried- reich's ataxia, as an Eng- lish writer has recently done, for the disease must invariably have a starting- point, and the patients under observation may indeed be the first of a long series that are to follow. The children who have come under my spe- cial notice in three dif- ferent families gave no history of any similar trouble in preceding gen- erations. The marked tenden- cy to the occurrence of this disease in families is shown, as Gowers states, by the occurrence of sixty-five cases in nineteen families, giving an average of rather more than three to each fam- Fig. 91. -Case of Hereditary Ataxy (Fried- reich's Disease). HEREDITARY DISEASES OF THE SPINAL CORD. 379 ily. The number has varied from two to as many as eight in a single family. Both sexes are about equally liable, but sometimes a special predilection is shown in favor of one or the other sex. In one family of nineteen, two males were affected with the disease and seventeen females escaped. All the cases which I have observed were in boys, the sis- ters of these escaping entire- ly unharmed. The disease comes on, as a rule, very early in life, most of the symptoms being fully developed before the age of fourteen. In the family referred to by Everett Smith, of Boston, the symp- toms of ataxic paraplegia were observed in the father at the age of sixty-six, but it is questionable whether his was a typical hereditary ataxy, and it is safer to adhere to the belief that Friedreich's disease will always appear early in life. If several mem- bers of one family are affected the disease is developed in all at about the same age. Symptoms. — The symp- tomatology of hereditary at- axy is now as firmly estab- lished as that of almost any spinal disease. Friedreich described most of the symp- toms in 1861. The French school, including Charcot, Vulpian, Brissaud, and Ma- rie, have given careful study to this disease. In England. Carpenter, Gowers, Ormerod, and Bury described cases of true hereditary ataxy ; while in this country the chief con- tributions to this subject have been made by Smith. Ham- mond, Seguin, Dana, Church, Sanger Brown, and [nglis. Fig. 92. — Same Patient as in Fig. 89. Marked atrophy of the muscles about the shoulder girdles (infra-spinati, su- pra-spinati, rhomboids, and others). 380 THE NERVOUS DISEASES OF CHILDREN. At a very early age children suffering from Friedreich's disease exhibit peculiarities in walking and standing. The child walks with its legs widely apart, in an uncertain, hes- itating fashion, reminding one very strongly of a combined ataxic and cerebellar gait. There are in addition in some pa- tients oscillatory movements of the head, which remind one a little of multiple sclerosis, and a little of such movements as we sometimes see in cases of senile degeneration. When the patient is asked to stand still, with his feet closely ap- proximated, he soon begins to stagger, as tabic patients do, and as soon as his eyes are closed falls to the ground un- less properly supported. Marie states that Romberg's symptom is ordinarily absent, but it has been most distinct- ly present in those subjects whom I have had occasion to examine. In addition to these disturbances in gait and in standing we also find a coarse tremor present in many of the cases, and in some a condition which is not so distinctly tremulous as it is ataxic and awkward. If the patient is asked to take hold of a pencil, to raise a glass of water to his lips, or to attempt to write, he seizes upon the object that he is to hold in a distinctly ataxic way and uses the hands awkwardly, sometimes after the fashion of extremely choreic movements. It is this combination of tremor, of awkward movements, and of choreic manifestations that gives to these children their characteristic aspect. There has been some discussion as to the occurrence of actual pa- ralysis ; in those whom I have examined there was in the first few years only a very slight loss of muscular power both in the upper and in the lower extremities. In ad- vanced stages the grasp is almost nil, and the lower extrem- ities may be so thoroughly paralyzed that it is impossible for the patient to lift a leg or even to move a toe, but in this respect the disease evidently varies very much. Sensation may be interfered with to a slight degree, but not nearly so markedly as in the tabes dorsalis of the adult. Lightning pains are infrequent, although Charcot insists that in some instances these occur quite as typically as in tabes. Anaesthesia and analgesia are rarely present. Several French authors have described cases in which hemianes- thesia occurred ; but Marie is inclined to attribute this to the HEREDITARY DISEASES OF THE SPINA I CORD. 38 1 association of hysteria with Friedreich's disease. The mus- cular sense is rarely affected, and in the persons whom I have examined for the purpose of determining the presence or absence of this sense, I have found it invariably present, even in those in whom there was marked inco-ordination of the upper extremities. Reflexes. — The superficial reflexes are, as a rule, pre- served ; but the deep reflexes are generally absent. It is this absence of the deep reflexes, particularly of the knee- jerks, that brings out in some subjects the very closest re- semblance between Friedreich's disease and tabes dorsalis. In cases in which the reflexes are exaggerated there is good reason to doubt whether the disease corresponds to that described by Friedreich ; * but there is no reason why in those forms in which the affection involves both the posterior and lateral columns the knee-jerk should not occasionally be augmented, and it is probably in view of such mixed types that Gowers has spoken of a form of hereditary ataxic para- plegia under the heading of Friedreich's disease. It is one thing to maintain a type as originally described by its dis- coverer, and quite another thing to deny that any departure from such a typical series of symptoms implies an entirely different order of disease. Difficulties of micturition and of defecation occasionally occur, but are not so constant an accompaniment of Fried- reich's disease as they are of tabes ; in the earlier stages of the disease, at all events, the vesical and rectal reflexes re- main intact. Trophic and vasomotor disturbances are also rare, if we except a general discoloration of the skin and coldness of feet and hands. Marie and other French authors refer to the occurrence of a special form of club-foot which they have observed in these cases of hereditary ataxy. Marie states that the foot is shortened ; that the anterior portion is particularly broad if viewed laterally ; the foot is in the condition of a pes cavus. The toes are hyperextended and have a claw shape. This deformity of the toes is said to have been observed as one of the early symptoms bv parents in whose families this special disease has been hereditary. * Some of these may belong to the Heredo-ataxie cerebelleuse of Marie. 382 THE NERVOUS DISEASES OF CHILDREN. Muscular atrophy constitutes an important symptom of this disease. It is most distinctly visible in the shoulder and pelvic girdles. It was so prominent a feature in one of my patients that on first examination I was inclined to re- gard the trouble as one of an hereditary form of progressive muscular atrophy, until the examination of the brother, who was in a more advanced stage of the disease, proved to me beyond the possibility of a doubt that both cases were typ- ical of Friedreich's disease. In the boy represented in Fig. 90, the excessive atrophy must be attributed to an addi- tional involvement of the gray matter of the cord. Fig. 93. — Deformity of the Feet in a Case of Friedreich's Disease ; Hyperextension of the Toes and Club Foot. (Marie.) We have now to consider a further set of symptoms which seem to me to prove the cerebro-spinal character of the disease. N}'stagmus occurs in many of these cases, but, as a rule, does not appear until several years after the onset of the first symptoms. The nystagmus can often be elicited only upon extreme use of the ocular muscles. In this con- nection it may be well to remember that nystagmus upon extreme movements occurs in not a few healthy individuals, and that the presence of such movements in a child of a family affected with Friedreich's disease need not neces- sarily indicate the development of this serious trouble. Ocular palsies occur, but are rare. The Argyll-Robertson HEREDITARY DISEASES OF THE SPINAL CORD. 383 pupil is as regularly absent in Friedreich's disease as it is present in cases of tabes. Optic atrophy and amblyopia have not been described in these cases. All the other senses remain entirely unaffected. Among cerebral disorders occurring in the course of Friedreich's disease, vertigo and dizziness are extremely frequent. These may in part be due to the oscillatory movements of the head. In the earlier years of the disease the intelligence is not affected, except that the children can- not be educated at schools as other children are, and there- fore remain backward. As the disease progresses a distinct defect of intelligence is noticeable, and a condition of semi- idiocy may be the result. The patients appear, however, far more idiotic than they really are, and this appearance is caused by the peculiar stupid expression of face, and above all by the disturbances in speech, to which we shall now refer. The speech reminds one a little of multiple sclerosis in that it is slightly scanning, but it is at times hesitating and jerky, at other times slow, deliberate, and awkward. On the whole, I find Marie's simile a good one, who com- pares it with the cerebellar gait, since it is, as he says, awk- ward, uncertain, and vacillating. All these symptoms are not developed until after the lapse of years. At first the peculiar gait and position are noticeable, later on the inco- ordination of the upper extremities and the difficulties of speech become more marked ; then, after the lapse of a longer or shorter period of time, the patients become help- less and may remain bedridden or confined to their chairs for a period of ten and even twenty years. The gradual development of cerebral symptoms denotes the continuous progress of the disease. In the following histories of two brothers the chief symptoms of Fried- reich's disease are well illustrated : Case I. — H. S , twenty-three years of age, second child.* Family History : Parents healthy ; not related to one another ; no neu- rotic taint in the ancestry. Thirteen children, all well and normal in every respect except this patient and his younger brother (Case il.). An older brother and two younger sisters of the patient and his parents were examined * I am particularly indebted to Dr. L. Stieglitz, who visited this patient at his home in the country ; the patient's brother (Case II.) was examined at my clinic. 384 THE NERVOUS DISEASES OF CHILDREN. with regard to speech, gait, and ocular muscles ; no anomalies discovered in them ; speech fluent. Patient was well till his eighth year, when he was knocked down by a cow ; but was not injured. First symptoms came on a year later ; the waddling gait of the boy attracted attention ; his gait grew steadily worse, he often " stumbled over himself " and fell to the ground ; if he attempted to run he would reel as though intoxicated. His mother believes his speech began to change when he was thirteen years old ; it then became slow, monot- onous, somewhat jerky, less distinct than it used to be. About three years ago he suffered for many months from severe shooting pains in both legs ; the pains gradually left him ; the last two years he has been free from pain. During this period other symptoms were developed ; bladder trouble set in and bowels were constipated ; patient could not retain his water and wet his bed, especially at night, for several weeks. For the past few years the patient has been perfectly helpless. Examination (July 3, 1893) : Patient sits in a chair and is utterly helpless. Expression of face idiotic ; intelligence, however, but very little below the average. He is not able to move his legs, feet, or toes. He moves his arms when asked to do so in a distinctly ataxic, awkward fashion. Muscles of arms very well developed ; grasp strong ; no weakness discoverable in any of the groups of muscles in the upper extremities. The first interosseous space on either side depressed. Electrical reaction (faradic current) of all the nerves and muscles of the arms unimpaired. No disturbance of sensation in either arm. The legs seem to consist of nothing but skin and bones. Cir- cumference of calf (both legs) 23 ctm. (about ten inches). Both feet large, deformed (pes equino-varus), blue and slightly swollen, reminding one of the blue swelling of lead palsy. Slight movement of the thighs at the hip- joints, otherwise complete paraplegia ; with a strong faradic current response is quick ; contractions of all of the muscles of the thigh and legs were elicited on direct and indirect excitation. Knee-jerks are absent, plantar reflex present. Sensation for touch and of temperature slightly impaired, especially in the feet. Pupils react promptly to light. Vision apparently unimpaired. Very marked ataxic nystagmus. Speech very slow, monotonous, at times jerky. Case II. — L. S (Figs. 89, 90), twelve years of age ; ninth child. Per- fectly well till seven years old, when mother first remarked that he waddled in his walk ; he could walk quite a distance at that time without feeling fatigued ; had great difficulty in going up and down stairs ; became slowly but steadily worse. About two years ago his speech began to change ; he began to speak more slowly, and in a monotonous tone. Went to school for two years and did well. No pains ; no bladder trouble. His mother says he never lets things drop out of his hands, when he once has hold of them, but in trying to grasp a glass or cup he would often knock against it and throw it over before getting a firm grip upon it. The last few months the boy has been suffering from malaria. Examination (spring of 1893): Ill-nourished, emaciated boy; shy, and when addressed appears to be stupid, but his intelligence is quite up to the HEREDITARY DISEASES OF THE SPINAL CORD. 385 average. The first interosseous space of both hands, the fossae infra- and supra-spinatae much depressed ; winged scapulae. Entire muscular system poorly developed. Motor Sphere : The boy's gait is highly ataxic (cerebellar-ataxic), his legs are spread wide apart, both in standing and in walking ; staggers as though intoxicated, stumbles over his own feet, but manages to cover ground fairly well. Romberg's symptom. Knee-jerks absent. Muscles of limbs, of back, and thorax thin and atrophic ; grip fairly good ; the power of his muscles much greater than their volume would lead one to expect. No true paresis in any single group of muscles. There is distinct weakness with atrophy in the muscles of the shoulder, the deltoid, pectoralis, infra- and supra-spinati, and the rhomboids ; the lower half of trapezius is atrophic, the upper portion fairly well preserved. With a strong current (faradic) all but the most atrophic muscles were made to contract on direct and indirect excitation ; the rhomboids and the muscles of the shoulder-blade responded feebly to the strongest currents. Galvanic formula not reversed, but excitability much diminished ; ataxy of arms and legs, and no disturbance of sensation. Slight nystagmus upon extreme lateral movements of eyeballs. Speech slow, monotonous — almost scanning. One year after this examination the boy could scarcely walk ; is now in a hospital suffering from mitral insufficiency ; is extremely emaciated ; intelli- gence not markedly impaired. Differential Diagnosis. — The symptoms of heredi- tary ataxy are so distinct that one would suppose confusion with other diseases a sheer impossibility, yet mistakes do frequently occur. The disease bears closest clinical re- semblance to tabes. The age of the patient and the occur- rence of the disease in families will serve to distinguish it from tabes dorsalis, but if a person in advanced age is ex- amined for the first time, special attention must be directed to the following points : Tabes. Argyll-Robertson pupil present. No nystagmus. No tremor of the head. No peculiarities of speech. Inco-ordination of upper extremities rare. Lightning pains and various crises. Ataxic gait. No defect of intelligence. 25 Friedreich's Disease. Argyll-Robertson pupil absent. Nystagmus present in later years. Tremor of the head present. Speech altered. Inco-ordination the rule. No such pains and crises, as a rule. Ataxic-cerebellar gait. Intelligence defective in later years. 386 THE NERVOUS DISEASES OE CHILDREN. Iii the above parallel columns the symptoms which both have in common have been omitted, such as absence of re- flexes and Romberg's symptom. Multiple sclerosis can be distinguished easily from Friedreich's disease by the excessive exaggeration of the reflexes, by the intention tremor, by the marked spasticity of the gait, by the occurrence of ocular palsies, all of which rarely occur in cases of Friedreich's disease. Confusion might arise from consideration of the similarity in speech and the occurrence of nystagmus. Marie includes in the consideration of the differential diagnosis ordinary chorea, but it seems to me that no one but a very obtuse person could be misled by the occasional occurrence of awkward and choreic movements in Friedreich's disease, for in no other respect are the two diseases at all similar. Confusion may more readily occur with cerebellar diseases, and Schultze has recently, in a satirical vein, in answer to Senator, called attention to the fact that, after all, cases that would seem to be of the cerebellar order are altogether different from the type which Friedreich described. I can ap- prove of this remark of Schultze, for I have myself seen a case of defective cerebellar development * in which there was a superficial resemblance be- tween them and cases of Friedreich's disease, and yet a careful considera- tion of the conditions was sufficient to distinguish between the two sets of diseases. Pathological Anatomy. — The morbid anatomy of Friedreich's disease has not, as yet, been satisfactorily de- termined. Few autopsies have been made, and in some of the cases which have been examined post mortem, the symp- toms have varied so much from the type, as laid down by Friedreich, that the results of the examination have helped to obscure rather than to clear up the doubts regarding the true anatomical changes of the disease. A number of au- thors have called attention to the attenuation of the entire cord in cases of hereditary ataxia, the diameter of the cord being but three-fourths or two-thirds of the normal. This attenuation is most marked in the dorsal region ; but whether it is due to a disappearance of nerve-fibres, to the retraction of sclerotic tissue, or whether it is simply a de- fect of development, has not been clearly made out. Any * See Chapter on Conditions due to Defective Development of the Brain. HEREDITARY DISEASES OF THE SPINA I CORD. 387 one of these three causes may be operative in some cases, or a co-operation of all three is a possibility in others. The one fact that is absolutely indisputable is that on micro- scopical examination a sclerosis of the spinal cord is found, involving at different levels, or at one and the same level, various systems of the cord. This sclerosis affects most frequently the posterior columns or the lateral columns, or both together, and hence the symptoms vary between those of a pure posterior spinal sclerosis and those due to a Fig. 94. — Section through Middle Dorsal Region of a Case of Friedreich's Disease. (After Blocq et Marinesco, from Marie's treatise.) A, small degenerated area, prob- ably Gowers' tract ; B, degeneration in lateral columns ; C, direct cerebellar tract ; D, column of Burdach ; E, column of Goll — both degenerated ; G, a strand of healthy fibres. postero-lateral sclerosis, resembling the symptoms of the ataxic paraplegia of the adult. Taking up the lesions of Friedreich's disease in detail, we may note the following distribution : 1. In the posterior columns, in a number of the cases, the columns of Goll are sclerosed throughout their entire length from the lower part of the spinal cord to the calamus scriptorius. The column of Burdach is also in- volved throughout the greater part of its course; but, according to Marie, the intensity of this sclerosis varies in different levels of the cord. The external portion of the column of Burdach, near the posterior horns, is generally ex- empt from sclerosis ; in the cervical region the sclerosis of the column of Burdach begins to diminish, and disappears altogether in the vicinity of the medulla oblongata. 388 THE NERVOUS DISEASES OF CHILDREN. 2. The direct cerebellar tract is involved from its beginning in the lower dorsal region to the upper cervical region. Toward this latter part it dimin- ishes considerably, but is most marked in the upper dorsal segments. Sev- eral observers have noted that the lesion extends beyond the direct cerebellar tract, and involves the antero -lateral tract, or tract of Gowers ; and Marie goes to the extent of stating that he thinks this involvement of the antero- lateral tract an almost constant feature of Friedreich's disease. 3. As for the lateral columns, the opinions of authors differ somewhat. Marie is not willing to allow the regular involvement of the lateral columns, while he concedes that the diseased fibres occupy the position of the crossed pyramidal tract. He does not believe that they represent the fibres of that Fig. 95. — Section through a Cervical Segment from a Case of Friedreich's Disease. (Schultze.) Degenerated areas in posterior and lateral columns and in anterior col- umn (left half of figure). Posterior root fibres also degenerated. tract. His reasons for maintaining this are, first, that the lesion of the lateral columns in Friedreich's disease diminishes considerably from below upward to the level of the lower portion of the medulla oblongata, the very reverse of which would occur if these fibres were part of the pyramidal tract. Secondly, that on a transverse section of the spinal cord the localization of the lesion of the lateral column does not correspond exactly to the site of the lesion of the pyramidal tract proper. Third, nothing in the clinical appearances of Fried- reich's disease reminds one of the symptoms which are a constant accom- paniment of changes in the pyramidal tract. The only explanation which Marie is able to give for the fibres that are affected in the lateral region is that they are probably fibres which connect the direct cerebellar tract with the antero-lateral tract of Gowers. Of the reasons advanced by Marie, the first seems to me to be the only one that will bear close scrutiny ; the second is scarcely susceptible of proof, and as for the third, it is sufficient to state HEREDITARY DISEASES OF THE SPINAL CORD. 389 that the disease attacks both the posterior columns and the pyramidal fibres at various levels. 4. A difference of opinion also exists as regards the marginal zone of Lissauer, as many authors claim to have found these zones entirely intact, while others claim that an affection of these fibres constitutes a regular feat- ure of Friedreich's disease. At all events the affection of Lissauer's tract is not nearly so constant or so early a feature of the disease as it is in the spinal ataxia in the adult. The morbid anatomy of Friedreich's disease is not exhausted with this statement regarding the behavior of the white fibres. The gray matter is affected as well. The columns of Clarke exhibit a loss of fibres very much like that in tabes, as well as a considerable diminution of their cells, which are also smaller and devoid of their long-cell processes. In the posterior horns there, is a diminution of volume, and also a decided diminution in the number of cells. The anterior horns have been found altered by some, Friedreich being among them, and the cells may be atrophied. There is no reason to doubt this occurrence in view of a considerable atrophy of the muscles which occurs in some of these cases, as in one of my own. Some changes have been noticed in the ependyma of the central canal, but these are not sufficiently constant to be considered a regular morbid feature of Friedreich's disease. Some have also insisted that the spinal meninges are involved, while others dispute such involvement. If they are affected at all it is in the vicinity of the posterior columns. Little more can be said of the be- havior of the posterior roots, although Blocq and Marinesco insist that they are as frequently altered in Friedreich's disease as they are in ordinary tabes. The peripheral nerves are supposed to be entirely normal in Friedreich's disease, and to this fact Dejerine and others attributed the entire absence of fulgurating pains in hereditary ataxy, while they are universally present in tabes dorsalis. The question remains as to the nature of the morbid proc- ess which gives rise to Friedreich's disease? A number of views have been propounded. Some have claimed it to be a mere arrest of normal development, while others regard the morbid process as a true sclerosis. Dejerine and Letulle, on the strength of certain peculiar fibres which they have found, and which they consider similar to fibres discovered bv them in the cortex of epileptic patients, are inclined to re- gard the sclerosis of hereditary ataxia as a sclerosis of the neuroglia. They insist that neither the connective-tissue septa nor the blood-vessels are at all altered, and that the scle- rosis is therefore very different from the vascular sclerosis which so often involves the pyramidal tracts and the direct cerebellar tract ; but later investigators, among them Wei- gert, have disproved these views of Dejerine and Letulle. 390 THE NERVOUS DISEASES OF CHILDREN. They regard the sclerosis of Friedreich's disease as in no- wise different from the ordinary sclerosis excepting in this, that it has been developed at an unusually early period. Weigert, in his recent studies on the neuroglia, concludes that in the ordinary spinal sclerosis, as it occurs in tabes, in multiple cerebro-spinal sclerosis, and in amyotrophic lateral sclerosis, the proliferation of the neuroglia is a much more marked feature than in hereditary ataxy. The refutation of this theory of Dejerine and Letulle does not, how- ever, help us in the recognition of any true theory of the disease, and for the present we must confess that we do not know why or how the sclerosis of Friedreich's disease is developed. Prognosis. — The prognosis of Friedreich's disease is in- variably grave. In the course of four to six years the patient becomes crippled, unable to move about except in a chair, awkward in speech, and more or less demented. Unfortu- nately the disease does not lead to a fatal issue within a reasonable period of time, some of the patients attaining the age of forty years and more. As for treatment, no special method need be recom- mended. The progress of the disease cannot be stayed, the relief of pain constituting the only reason for active inter- ference on the part of the physician. The ordinary anti- neuralgic remedies may be employed to this end. In addi- tion, suspension, electrical treatment, massage, and the like, may be ordered, in the discretion of the physician. HEREDITARY ATAXY (CEREBELLAR TYPE ; TYPE NONNE-MARIE). Under the title " Heredo-ataxie Cerebelleuse " Marie collected a number of cases which bear a very close resemblance to Friedreich's disease, yet pre- sent several characteristic and different symptoms. Titubation, Romberg's symptom, tremor of the head and of the extremities, choreiform, awkward movements, particularly of the arms and hands, and nystagmus, are symp- toms common to Friedreich's disease, and to the form we are now describing. The cerebellar form of hereditary ataxy comes on, as a rule, at the age of pu- berty or later. The knee-jerks, instead of being diminished, are increased. There are marked ocular symptoms, the pupils fail to react to light and dur- ing accommodation. There is diplopia in some cases, color-blindness in others. Contraction of the visual field has been observed, and amblyopia, due to atrophy of the optic nerve, is a typical symptom. There are also marked HEREDITARY DISEASES OE THE SPINAL CORD. 39 1 disturbances of sensation. The deformities of the foot and kyphosis, noted in Friedreich's disease, are never present in the cerebellar type. This symptomatology, as developed by Marie, reveals a very striking re- semblance between these cases of hereditary cerebellar ataxy and a form of family disease described by Nonne. In all probability Nonne's and Marie's descriptions refer to the same type. While the latter deserves credit for naming the disease, it seems to me just that Nonne's name should be con- nected with this type. There is also a very close resemblance between this hereditary cerebellar ataxy and some of the cases which have been described under the heading of " Hereditary Spastic Paralysis." Senator's views regard- ing Friedreich's disease may in part be explained by the recognition of this spe- cial order of disease, and there would surely be a possibility of confounding this type of hereditary ataxy with the cerebellar type of multiple sclerosis which Charcot has described. It is too early to report definitely upon the pathology of hereditary cere- bellar ataxy. If we are correct in considering the cases described by Marie and Nonne as representatives of the same type, .the post-mortem findings in one of Nonne's cases will give some clew to the pathology of this variety of disease. In Nonne's case the cerebellum was small, and there was a simple diminution of tissue without sclerosis or degeneration. The spinal cord was much attenuated throughout its entire length. Under the heading of " He- reditary Ataxy and Atrophy of the Cerebellum " Menzel described a condition in which there was a combined systemic disease of the spinal cord, and in addition atrophy of the cerebellum, pons, and medulla. Two French authors, Royet and Collet, have reported a case which was diagnosticated during life as multiple sclerosis, in which there was found on post-mortem examination a lesion of the cerebellum and of the cerebellar tracts in the pons. It is possible that some of the cases which have been regarded as a form of he- reditary multiple sclerosis may come under this same heading. I recognize that there is great danger in multiplying types of disease. The form de- scribed by Marie and Nonne should in justice to these two authors be given due consideration ; but I believe it better for the present to regard it as a variety of hereditary ataxy.* HEREDITARY SPASTIC PARALYSIS (SPINAL, CEREBRAL OR CEREBRO-SPINAL TYPES). During the past few years a number of family affections have been described by Homen, Newmark, Pelizaeus, Strum- pell, Freud, Erb, and myself, in all of which a spastic rigidity, or a spastic paralysis affecting the lower extremi- ties chiefly, has been the most prominent symptom. In some cases the spastic symptoms were evidently of purely * The cases reported by Dr. Sanger Brown also resemble this type. 392 THE NERVOUS DISEASES OF CHILDREN. spinal origin ; in other cases they were associated with dis- tinct cerebral symptoms. A classification of all of these various diseases according to the morbid conditions under- lying them, is not yet possible; it is sufficient for the pres- ent to discuss them from the etiological point of view. I have chosen, therefore, to designate them as hereditary spastic paralysis, and to subdivide them, according to their more prominent symptoms, into a spinal, and a cerebral or a cerebro-spinal variety. After adopting a subdivision of this kind, the pathology of each type will be more readily made out ; and it is quite probable that the various types will be found to represent a mere difference in the topographical distribution of le- sions and symptoms, while the morbid process, an arrest of development, or an early degeneration, is common to all. The spinal type of hereditary spastic paralysis is well illustrated by Newmark's cases. In view of the impor- tance of the subject a short summary of a few of his cases will be given. A girl, aged fifteen, tall and intelligent ; normal birth and labor ; no con- vulsions. Did not kick with the legs as other children do in bed. Began to walk at eighteen months. Gait peculiar from the first, and always remained typical of spastic paraplegia. Lower extremities flexed at the hips and knees ; adduction of thighs and pes equinus. No wasting of muscles, which react well to both currents. Knee-jerks and adductor reflex exaggerated ; no ankle clonus (probably on account of extreme contracture). No disturb- ance in the upper extremities, but all tendon reflexes exaggerated in them. Jaw-jerk present. Intelligence, sensations, and sphincters intact. No nys- tagmus, no strabismus. Girl is able to use a tricycle. Speech is normal. A brother, aged five, exhibits very similar symptoms. No history of syphilis in the family. There were no other cases of similar nature in the family, ex- cept one of spastic diplegia in a first cousin ; but this diplegia was evidently the result of difficult labor. In the second family the father is said to be a tall, healthy man of thirty- eight, who denies syphilitic infection or alcoholic habit. Has never known of any affection similar to that observed among his children. His reflexes are normal. Mother of children, aged thirty-seven, has always been healthy. Her knee-jerks and Achilles tendon reflex quite active. Lively reflexes in the upper extremities and jaw-jerk distinct ; no ankle clonus. No blood re- lationship between man and wife. They have had eleven children, eight of whom are living. The oldest living child is a boy, aged sixteen, who was well until about a year and a half before he was examined. At that HEREDITARY DISEASES OF THE SPINA I CORD. 2>93 time a stiffness appeared in the legs, especially on rising in the morning. His condition gradually developed into a mild form of spastic paraplegia, with typical gait and position of the extremities. Knee-jerks much exag- gerated ; no patellar clonus nor ankle clonus. Plantar, cremasteric, and abdominal reflexes very lively. Abdominal muscles said to be rigid. Up- per limbs normal ; reflexes much increased. Jaw-jerk well marked. In a second brother, aged fourteen, the symptoms had become more pronounced, and had set in at the age of seven and a half years. The legs were stiff and walking was difficult ; had been compelled to use crutches. There were con- tractures in the knee-joints ; there was adductor spasm and pes equinus, furthermore, an exaggeration of the knee-jerks. Ankle clonus was present, and the plantar and cremasteric reflexes were also very lively. A third brother developed exactly the same symptoms at the age of nine years, after typho-malarial fever. Though these three cases are the most pronounced ones in the family, one sister exhibits increased reflexes ; a boy, eight years of age, is said to be stiff in the knees, with exaggeration of the patellar re- flex ; another child, aged six, is said to have a lazy, dragging walk, with ex- cessive increase of knee-jerks ; a child, aged three and one half, has active knee-jerk and jaw-jerk. That there is a tendency to spastic paralysis in this family there is no doubt ; but the fact that instruments were used at the birth of several children, including the one whose symptoms were most pronounced, makes it probable that " heredity " alone was not responsible for the multiplicity of cases. For the same reason Schultze's patients cannot be included under this heading; there was a history of difficult labor in all his cases. The symptoms of this spinal type (spastic paraplegia, with rigidity or contractures, increased reflexes) are distinctly due to interference with the pyramidal tracts in the lateral colunjns of the cord. Many years ago Striimpell described the cases of two brothers, who pre- sented the typical features of a spastic spinal palsy. In one of these cases a post-mortem examination was made, by which it was determined that the symptoms were due to a primary systematic degeneration of both pyramidal tracts in the lateral columns, together with a slight affec- tion of the cerebellar tracts and the columns of Goll. More recently Striimpell has described another case, very much like the other two, in which he made the diagnosis of he- reditary spastic palsy. In the family of this patient the grandfather was said to have suffered from a palsy of the 394 THE NERVOUS DISEASES OF CHILDREN. legs, the father had a peculiar gait, and people who knew both the father and the present patient said that one walked like the other. Two uncles were said to have the same walk. The mother of the patient and other brothers and sisters were entirely healthy. The patient did military service from his twenty-first to his thirty-third year, and was not inconvenienced in any way, except that he occasionally noticed a peculiar feeling in the legs. At the age of about thirty-five his first symptoms began. His legs became stiff and he soon had to resort to a stick in walking. The exam- ination of the patient revealed a typical spinal spastic paralysis, with exaggeration of the reflexes, rigidity of the joints, normal sensation, and a spastic gait. The vesical and rectal reflexes were not interfered with. All the symptoms steadily increased, but no new ones were developed. In addition to its interest as a form of hereditary spinal disease the type also deserves some special recognition from the fact that, from first to last, the symptoms have been such as are due exclusively to disease of the lateral columns. Strumpell refers to Bernhardt's patients, and claims that they are entirely identical with those described by him. Four out of six brothers presented the phenomena of spastic spinal palsy ; three of these four developed their symptoms at the age of thirty, and all the morbid signs progressed in a remarkably slow fashion. I can agree with Strumpell in believing that these diseases are entirely similar to those de- scribed by him, although in one case of Bernhardt the symp- toms tended toward a cerebro-spinal rather than a purely spinal type. The forms described by Tooth and Philip should be placed in the same category. Spastic spinal paralysis (spastic paraplegia) occurs in children of families in which there is no history of a similar affection in the same or past genera- tions. In February, 1893, a girl, four years of age, was brought to my clinic, who was afflicted with a typical spastic paraplegia ; paresis and rigidity of both lower extremities ; very rigid gait, increased knee-jerks on both sides, • slight double ankle clonus — these reflexes being a little more marked on the right side than on the left side ; increased reflexes in the upper extremities, but no weakness. Electrical reaction of all muscles and nerves normal ; no dis- turbances of sensation ; no impairment of vesical and rectal reflexes ; no nystagmus. Intelligence good. The birth of the child (first-born) had been entirely normal and at full term ; no instruments were used ; the child was HEREDITARY DISEASES OF THE SPINAL CORD. 395 supposed to be perfectly well, except that it did not begin to walk until it was three years old, and its walk was unusually stiff and awkward ; it dragged its feet. Three younger children are perfectly healthy. Excepting the early age of onset there is no distinction in clinical symptoms between such cases as these and the spastic spinal paralysis of the adult. It is best not to speculate regarding the morbid pathol- ogy of this type. The symptoms must be attributed to the pyramidal tracts of the lateral columns, but whether the disease in them is primary or secondary it is too early to state. It is probable that by disease, or by arrest of de- velopment, the entire pyramidal tract, and not merely the spinal portion of it, is involved. In view of Strumpell's inves- tigations there is no longer any doubt about the occurrence of a primary degeneration of the lateral columns develop- ing later in life, but due in all probability to abnormal con- genital conditions. There is some reason, therefore, to be- lieve that the question of the occurrence of a primary lateral sclerosis will be affirmatively answered through these stud- ies on hereditary spastic paralysis. Diagnosis. — The diagnosis of these diseases need not be given in further detail. The history of a case exhibiting the symptoms of spastic paraplegia of the lower extremities, with increase of the reflexes, with rigidities and contrac- tures, without involvement of the vesical or rectal reflexes, without atrophy, without disturbances of speech and nys- tagmus, would be sufficient to place the case in this cate- gory, provided the hereditary character of the affection could be established. The disease should be carefully dif- ferentiated from the spastic cerebral palsies of childhood.* The diplegias and paraplegias occurring as a result of dirh- * While this chapter was passing through the printer's hands, a reprint of Erb's ar- ticle on Hereditary Spastic Paralysis, reached me. Erb thinks that some of the congenital diplegias and paraplegias which Freud, myself, and others have attributed to cerebral lesions may at times be due to spinal lesions. I am willing to concede the possibility of this ; but I think that such spinal affections are, after all, rare ; and there is the one fact, to which I have alluded in various writings, that in forty-five per cent, of all the cases of infantile spastic paraplegia there is marked idiocy. This asso< militates against the diagnosis of a purely spinal disease. I have reviewed the his- tories of all my patients with supposed cerebral paraplegia andean find none which I am willing to attribute to a spinal lesion. The child reported on page 394, though suf- fering from paraplegia, was recognized as probably of spinal origin ; and such an origin may be suspected in types of spastic paraplegia, without idiocy coming on several years after a normal birth. 396 THE XERVOUS DISEASES OF CHILDREN. culty during labor (^Little's disease), would be most easily confounded with this hereditary form. A careful inquiry into the history of labor, the determination of the exact age at which the trouble began, the frequent occurrence of con- vulsions and of defective mental development, in birth palsies, will help to differentiate between these two sets of cases. If spastic paraplegia is developed in a child, or in a youth, that has not been preceded by convulsions, or is not associated with defective mental development, the case would come more properly under the head of hereditary spastic paraplegia, and we must remember that such cases may occur without any history of similar disease in the same or preceding generations.* The prognosis of hereditary spastic spinal paralysis is a grave one as regards recovery from the disease, but not unfavorable as regards the duration of life, for the majority of patients thus far examined have been well advanced in years. In the way of treatment, nothing can be attempted except to apply the usual methods of massage and elec- tricity, or possibly to attempt to improve contractured limbs by various tenotomies. The Cerebral Type. — Cases of this character were de- scribed by Freud and myself several years ago in studies on the cerebral palsies of childhood. I am a little loath to re- move them from that category of diseases, but believe that they should be referred to in this connection for the sake of pointing out their exact clinical relation to the other forms mentioned in this chapter. Under the title of " Arrested Cerebral Development " I published my first case of this description in 1887, without suspecting the hereditary element; in 1892 I recorded the occurrence of a similar affection in a sister of my first case, and in that year was able to refer to another family * Marie and some oiher French authors deny the existence of a primary spinal spastic paralysis in the adult, and believe that if such a condition does exist, it is to be traced back to the early years of life. They describe as Tabes dorsal spasmodique of children, a group of symptoms which German and American authors have included un- der the term, Congenital Spastic Diplegia and Paraplegia. The French view would take all of these cases out of the category of cerebral diseases, and for this there is as yet no warrant. Marie (p. 101) states that "Tabes dorsal spasmodique" is never hereditary ; and attributes the condition to defective development of the pyramidal tract. This tract is surely a cerebro-spinal affair. HEREDITARY DISEASES OF THE SPINAL CORD. 397 in which four children were affected with this same dis- ease. The striking features of these cases are as follows: A child of apparently normal physical and cranial devel- opment thrives well until about the age of four or five months, then it begins to show marked retrogression ; does not take notice of things as other children do, cannot be induced to play ; does not recognize the nurse or mother. Its vision is defective and leads at an early day to blind- *ti Fig. 96. — Photograph of Brain of Author's First Case of the " Cerebral Type " of Hereditary Spastic Paralysis. The histological condition was described as an "agenesis corticalis. " In the above figure, the confluence of fissures and the ex- posure of the Island of Reil are the signs of a low order of cerebral development. Through the hardening process, the conditions have been accentuated a little, but they were present in the fresh specimen. At x, and in the frontal lobes, sections had been removed for histological examination. Other letters refer to fissures. ness. Nystagmus is present. The child utters a few sounds, but does not exhibit the least sign of intelligence. The symptoms continue in this way for a period of one year, or at the utmost two. The child's physical condition grad- ually deteriorates, and at the end of the specified period of time, after complete emaciation and marasmus, the child dies. Convulsions are never present. These cases appear to me to deserve special attention 398 THE NERVOUS DISEASES OF CHILDREN. Among the large number of cases of idiocy that I have seen, and which I have attempted to analyze from a clinical and anatomical point of view, they stand out as a distinct group, and they are also distinct from the other spastic palsies. The disease has been noticed by ophthalmologists, and has recently been well described by Mr. Kingdon, of England, who has included among his list the report of one of my patients. My first case was examined by Dr. Knapp, when the child was about three months old. He reported vibratory nystagmus ; pupils contracted as is usual with children at this age. Media clear, optic-nerve discs pale. Fovea centralis of a cherry-red color, and surrounded by an intense grayish-white opacity. Dr. Knapp considered this condition to be due to a developmental defect ; ultimately there was complete optic atrophy. Discs as white as paper. In all of the children reported by various authors the fact of heredity has come out with unusual distinctness, several diseases of the same order occur- ring in successive generations, and in single families. The disease may be characterized as hereditary spastic paralysis, with idiocy and amaurosis. Freud has reported several interesting cases under the title of " Family Forms of Cerebral Diplegia." Two of the children were bright at the ages of six and five years respectively, but they presented from earliest childhood the following symptoms : Nystagmus, atrophy of the optic nerve, convergent strabismus, awkwardness in speech and in the use of the arms ; tremor of the arms and a spastic paraplegia of the lower extremities ; no convulsions. In these boys the disease is evidently present in its lightest and most favor- able form. The improvement in vision as the one child grew older seems evidence of this, but the resemblance of these forms to those described by me is shown by a third child that died at the age of ten months ; it was paralyzed from birth, and idiotic ; whether it was blind also is not stated. The father thought the child had died of rickets (more probably of maras- mus). Pelizaeus has described a family disease of which the chief symptoms were : Nystagmus, mental imbecility, disturbances of speech (Bradylalia), awkwardness of hands, spastic paraplegia of the lower extremities. The disease attacked male members of several generations, but was transmitted through healthy mothers. All of those who were attacked showed some symptoms in childhood, and several died young. Pelizaeus was inclined to the diagnosis of multiple sclerosis ; but his cases evidently constitute a late form of hereditary spastic paralysis.* The morbid anatomy of this cerebral type of hereditary spastic palsy is not fully known. In two of the children ob- * The cases of Home'n were probably of a syphilitic nature. The cases reported by Krafft-Ebing were instances of Little's disease, with a distinct history of premature delivery and other abnormal birth conditions. HEREDITARY DISEASES OF THE SPINAL CORD. 399 served by myself (two sisters), the chief changes consisted in a complete arrest in the development of the cortical cells, a condition to which the term agenesis corticalis seemed applicable.* The external configuration of the brain also proved it to be of a low order of development. Whether agenesis corticalis represents the entire morbid condition is doubtful, to say the least. Unfortunately I was not able to examine the spinal cord in either one of my cases. In all probability evidences of the arrest of devel- opment will be found in the cortex, in the pyramidal tracts, and in other parts of the central nervous system. (Fig. 96.) / / Cervical. Dorsal. Fig. 97. — Section through Cervical and Dorsal Segments in a Case of Meningo-En- cephalitis. Probable defective development of pyramidal tracts in the lateral col- umns ; no trace of a defect in the direct pyramidal tract. (Weigert stain.) These cases must be differentiated from the congenital diplegias (Little's disease), due to premature birth and ab- normal labor. This can *be done best by referring to the history of normal labor, to the absence of convulsions, and to the visual troubles. But a cerebral condition — a meningo- encephalitis — due to difficulties at birth, may be associated with a defective development of the pyramidal tract. This is the interpretation which I now give to the following case, which puzzled me for a long time, and presented a number of anomalous features.f The patient, a boy, one year of age at the time of death, was born of a healthy mother (primipara) after a dry labor of forty-eight hours. The child * For a fuller description of the anatomical condition, the reader is referred to the chapter on Diseases due to Defective Development of the Brain. tThis case was published in full in the New York Medical Journal for May a, [89] 400 THE NERVOUS DISEASES OF CHILDREN. was asphyxiated. From the first day to the age of six and a half months it passed through innumerable convulsive seizures of a very extreme order. (See Fig. 38.) It presented in addition : Retarded mental development ; spastic : t-._V;:-- - - "'■-, -^P '■;-." '—"' ' t^ ■rr ♦^•T-^i- ■&*" 3^<" f « 1 yy^^' ?-V«£'>. B2 a/ 4A vfe ■--4?-; Fig. 98.— Section through Motor Cortex in Case of Spastic Paraplegia, 0, space be- tween the cortex and pia ; b, blood-vessels in transverse and longitudinal sections, showing infiltration of their walls ; c, altered cells with pericellular spaces ; p, the pia, thickened and infiltrated, sending projection downward between two convolutions. paraplegia (upper extremities less paralyzed, but somewhat rigid) ; increased deep reflexes (patellar, clonus, etc.), and convergent strabismus. The epileptic seizures were lessened by treatment, but otherwise the condition remained unchanged. The child died of an acute infectious disease of a doubtful character. (So reported from the Babies' Hospital.) HEREDITARY DISEASES OE THE SPINAL CORD. 401 On post-mortem examination I found distinct evidences of a wide-spread chronic meningo-encephalitis (probably due to meningeal hemorrhage at birth, see Fig. 98), and in ad- dition, what I supposed at the time was a secondary de- generation in the pyramidal tracts. But on re-examination of my specimens I was struck by the fact that there was relatively less sclerotic tissue in the diseased areas than in ordinary cases of secondary degeneration, and that the nerve-fibres were present in large numbers, but all of them were degenerated. The anterior pyramidal tracts, more- over, were not involved. (Fig. 97.) This degeneration or defect I now believe to have been primary and congenital A variation in the development of the pyramidal tracts in the lateral columns may account for the relative immunity of the upper extremities, al- though the cortical areas for both upper and lower ex- tremities were equally diseased. I do not wish to infer, however, that all cases of congenital spastic paraplegia are due to a spinal rather than a cerebral process. Some of them may be explained in this way ; but I cannot adopt the views of Marie and other French authors until further evidence of a purely spinal origin shall have been furnished in a number of cases. In all cases of hereditary spastic paralysis the prog- nosis is grave as regards recovery, or normal development of mind and body ; the probable duration of life can be gauged by the severity of the symptoms. The cases asso- ciated with idiocy and blindness die between the ages of one and three years. Treatment is purely symptomatic. As a preliminary division of the diseases discussed in this chapter, the following is submitted : HEREDITARY AND FAMILY AFFECTIONS. . . j i. Friedreich's Disease. Hereditary Ataxy, -j ^ Hereditary Cer ebellar Ataxy (Type, Nonne-Marie). f Spinal Type. Cases of Newmark, Bernhardt. Strum- Hereditary Spastic 1 pell, Erb, Tooth, and others. Paralysis, ) Cerebral or Cerebro-spinal Type. Cases of Pelizaeus, [ Sachs, Freud. Hereditary Progressive Muscular Atrophy.* * (Described in the following chapter. See page 409. ) 26 402 THE NERVOUS DISEASES 0E CHILDREN, BIBLIOGRAPHY. FRIEDREICH'S DISEASE. Besold : Deutsche Zeitschr. f. Nervenheilkunde, Bd. V., p. 157. Brown, Sanger : Brain, vol. xv., p. 250. Bury, Judson S. : Brain, July, 1886, vol. ix., p. 145 (full literature). Dejerine et Letulle : Semaine Medicale, March 12, 1890. Erb : Neurologisches Centralblatt, 1890, No. 12, p. 378. Friedreich : Virchow's Archiv, Bd. LXVIII. and Bd. LXX. ; also Arch. f. Psych., Bd. VII., 1876. Griffith : Transactions of College of Physicians, Philadelphia, 1888. Hammond : Journal of Nervous and Mental Disease, 1882. Kahler u. Pick : Arch. f. Psych., Bd. VIII., 1878. Ladame : Brain, vol. xii., p. 467. Marie : Maladies de la Moelle, p. 381, 1892. Ormerod : Brain, vol. vii., p. 105 ; vol. xv., p. 268. Remak : Berl. kl. Wochenschr., 1885, No. 7. Rook and Dana : Journal of Nervous and Mental Disease, 1890, p. 173. Schultze, F. : Zeitschr. f. Nervenheilkunde, Bd. V. ; Virch. Arch., Bd. LXXIX. Seeligmuller : Arch. f. Psychiatric Bd. X., 1880, October, 1885. Seguin, E. C. : New York Medical Record, 1885. Senator : Berl. kl. Wochenschr., 1893, No. 21. Smith, W. E. : Boston Medical and Surgical Journal, 1885. HEREDITARY CEREBELLAR ATAXY. Brissaudet Londe : Revue Neurologique, No. 5, March 15, 1894. Marie: Semaine Medicale, 1893, p. 444. Menzel: Hereditary Ataxy and Cerebellar Atrophy, Arch. f. Psych., Bd. XXII., p. 160. Nonne: Arch. f. Psych., Bd. XXII. HEREDITARY SPASTIC PARALYSIS. Bernhardt: Virchow's Arch., Bd. CXXVI., 1891. Bloch: Archiv f. Psychiatric Bd. XII. Erb : Zeitschr. f. Nervenheilkunde, Bd. VI., December, 1894. Freud: Zur Kenntniss der Cerebralen Diplegien. Leipzig u. Wein, 1893, p. 143; also, Neurolog. Centralbl, 1893, Nos. 15 and 16. Homen : Arch. f. Psych., 1892, Bd. XXIV., p. 191. Krafft-Ebing : Wiener kl. Wochenschr., 1892, No. 47. Marie : Les Maladies de la Moelle. Paris, 1892, pp. 87-107, Pelizaeus : Arch. f. Psychiatric Bd. XVI. , p. 698. Philip : Brain, vol. viii. } 1886. HEREDITARY DISEASES OE THE SPINAL CORD. 403 Sachs : Journal of Nervous and Mental Disease, 1887, p. 541 ; 1892, p. 603 ; Volkman's Vortag, Nos. 46 and 47, p. 467. Seeligmiiller : Deutsche Med. Wochenschrift, 1876, Nos. 16 and 17, Arch. f. Psychiatrie, Bd. X., p. 222, 1880. Striimpell : Arch. f. Psych., Bd. XVII. ; Deutsche Zeitschr. f. Nervenheil- kunde, Bd. IV. Tooth : St. Bartholomew's Hospital Reports, xxvii. ; see also Neurolog. Cen- tralbl., 1802, No. 8. CHAPTER XXIII. PROGRESSIVE MUSCULAR ATROPHIES. In this chapter we intend to discuss all those diseases which are characterized by a progressive weakness and atrophy of certain groups of muscles. We have nothing to do with muscular atrophy, whether progressive or not, which follows after acute disease of the brain, of the spinal cord, or of the peripheral nerves. The term "progressive muscular atrophy " was formerly given to a single type of disease with which we shall become more intimately ac- quainted, and for a time all cases resembling this one type were designated in the same way. It became evident, how- ever, that this one term was altogether too general. While it was a convenient clinical designation for an entire group of diseases, it did not sufficiently describe a number of other forms which were closely allied to the chief type. In order to avoid confusion, it would be well if we could dismiss the term progressive muscular atrophy altogether, for in many of the cases hypertrophy as well as atrophy is present for a long period of time. The word dystrophy, which might be used to designate both conditions, has, un- fortunately, been restricted to the cases of primary or idio- pathic muscular wasting ; we cannot, therefore, apply it to the spinal forms. Moreover, a number of diseases were included under the heading of progressive muscular atrophy which we now recognize to have been cases of amyotrophic lateral sclerosis, of syringomyelia, and possibly of spinal syphilis. The chief question at the present time regarding the various forms of progressive muscular atrophy is, whether in a given case the disease is of spinal or muscular origin. PROGRESSIVE MUSCULAR ATROPHIES. 405 Amyotrophy . Neural Atrophy Those forms of progressive muscular atrophy due to a spinal lesion we call amyotrophies, and to those due to disease of the muscular system only we give the name myopathies. We shall see that there is some reason, too, to constitute a third type, which we might designate as a neural form of progressive muscular atrophy. (Fig. 99.) The study of this entire subject began many years ago with the establishment of two distinct diseases — the first was the typical " progressive muscu- lar atrophy," as described by Aran and Duchenne ; the sec- ond, pseudo-hypertrophic mus- cular paralysis. Since that time at least six different forms of progressive muscular wast- ing have been described, and the attempt has been made in each case to prove the relation of the special form, either to the Aran-Duchenne type, or to the type of muscular pseudo- hypertrophy. The Aran-Du- chenne type has become the chief exponent of progressive amyotrophies ; while muscular pseudo-hypertrophy has been considered the most pro- nounced form of primary myo- pathies. The various types of progressive muscular disease have been established very largely in accordance with the mere topographical distribution of atrophy or hyper- trophy. Though convenient for clinical designation, such a distinction is not sufficient for a rational classification of these various diseases. It must be our aim to find the cardinal symptoms which will help us to differentiate at once, and easily, between those cases of progressive mus- cular disease due to spinal-cord lesions and those primary dystrophies, which represent a disease of the muscular system. Myopathy Fig. 99. — A Diagram designed to show the Site of the Morbid Lesion in the Several Groups of Progressive Mus- cular Atrophy. 406 THE NERVOUS DISEASES OF CHILDREN. The following are the cardinal symptoms present in the majority of cases belonging to the two large groups of cases : Progressive Amyotrophies. Progressive Myopathies. Onset late in life ; rarely in early Onset in early life. childhood. Not hereditary, as a rule. Generally hereditary (family trouble). Wasting first in the upper extremi- Wasting or hypertrophy begins in ties (leg type rare). the lower extremities. Hypertrophy does not occur. Hypertrophy frequent. Fibrillary twitchings. No fibrillary twitchings. Reaction of degeneration often pres- Reaction of degeneration rare (quan- ent in affected muscles. titative, not qualitative, electrical changes). These points of differential diagnosis will hold good in the majority of cases. But cases of spinal progressive muscular disease occur in which there is a strong history of an hereditary or family affection. Fibrillary twitch- ing has been seen in some cases that appeared to be typical myopathies, and the electrical reactions have been found considerably altered in similar cases, so that of all these cardinal symptoms there are, after all, only a very few which are invariably present in one or the other form of progressive mus- cular disease, and it is wiser, therefore, to be guided by the general agree- ment of symptoms rather than by any one single symptom.* This confusion of types of disease and of symptoms, need not cause surprise, if we remem- ber that the ganglion cells of the spinal cord, the peripheral nerves, and the muscles, constitute a physiological unit (Neuron). With these prefatory remarks we may proceed to the consideration of that class of cases which for a very long- time were supposed to be the only representatives of what was formerly called "progressive muscular atrophy." This is a spinal-cord affection which, as a rule, begins late in life. It might, therefore, be considered out of place to treat of this disease in a work on the nervous diseases of children ; but the entire subject of muscular diseases cannot be prop- erly understood unless we can recognize this special type, and, furthermore, cases of this type have of late years been described by Hoffmann and others, in children. * The hereditary cases reported by Werdnig are particularly interesting in this re- spect. PROGRESSIVE MUSCULAR ATROPHIES. 407 PROGRESSIVE AMYOTROPHY. — " PROGRESSIVE MUSCULAR ATROPHY." (TYPE, ARAN-DUCHENNE.) This form begins, in the majority of the cases, with an atrophy and a corresponding weakness in the small muscles of the hand (thenar and hypothenar). The atrophy extends slowly from muscle to muscle (" atrophie individuelle "), be- ginning as a rule with the adductor pollicis. It involves by degrees the opponens pollicis and the deep muscles of the thenar. From these it gradually extends to the muscles of the hypothenar, the interossei, the flexors and extensors in the forearm. At this point the disease may remain sta- tionary, or it may spread to the flexors in the upper arm, to the deltoid, the triceps, and finally to the muscles of the trunk, the shoulders, and the back. Duchenne recognized the fact that the atrophy may, in exceptional cases, begin in the trunk, in the shoulders, or in the legs. Some of these would now, no doubt, be considered under a different head- ing, but I have myself seen cases which could in nowise be distinguished from the typical Aran-Duchenne disease which began in the muscles of the thighs. If the disease begins in the upper extremities, the legs are, as a rule, not affected until very late in the course of the disease ; but there are exceptions to this rule. I have observed several cases in which the atrophy in the lower extremities began almost simultaneously with that in the upper extremities. The atrophied muscles in this form exhibit fibrillary con- tractions, and, as a rule, present marked changes in electri- cal contractility. These changes are not so pronounced as in cases of acute or subacute anterior poliomyelitis, and yet after the disease has lasted for a considerable length of time a typical reaction of degeneration will be found present in most of the wasted muscles. In the majority of cases thus far recorded the disease has not been distinctly hereditary, although series of such cases with distinct hereditary ten- dencies have been published by Naunyn, Eichhorst, Ham- mond, Osier, and others * * The famous Weathersby family, reported by Hammond, and the Fair family, of Vermont, described by Osier, may possibly represent other types of progressive muscular atrophy, which we shall consider later on. 408 THE NERVOUS DISEASES OF CHILDREN. As the disease progresses the wasting becomes more and more extreme ; the patient is no longer able to get about, becomes bedridden, and after mairy years of annoyance, if not of suffering, dies of some intercurrent disease, or from extension of the process upward into the region of the me- dulla, with consequent paralysis of the vital centres. The symptoms of the disease were well described by Duchenne, and very little has been added since his day to the clinical characterization of this special form. But a very warm discussion was waged for a long time regarding the origin of the disease, some maintaining its spinal origin, others believing it to be a peripheral disease. There was some reason for this difference of opinion, for the cases upon which the older authors based their views were in part due to spinal disease, and in part due to nerve or muscular lesions. The microscopical studies of Charcot and Joffroy, of Lockhart Clarke, of Hayem, and others, proved beyond a doubt that this special form of progressive muscular atrophy was due to changes in the spinal cord. The chief changes found are these : A sclerotic and pigmentary atro- phy of the ganglion cells of the anterior horns, inflamma- tory changes in the neuroglia, increased size of the blood- vessels, and proliferation of the cellular elements. In fresh preparations granular corpuscles are found, and according to the degree and stage of the disease the anterior horns may be very much reduced in all diameters, and the gan- glion cells either atrophied or entirely lost. The anterior nerve-roots are affected secondarily to the lesion of the gray substance. The nerve-fibres are not all destroyed, a num- ber of them remaining intact. Those that are destroyed exhibit the appearances of simple atrophy, a point to which Charcot alludes as distinguishing these cases from infantile spinal paralysis. According to these pathological findings we must sup- pose that an inflammation spreads slowly from the ganglion cells of the anterior horns along the anterior nerve-roots without destroying as many of these fibres as is the case in infantile poliomyelitis. The atrophic changes in the mus- cles are the direct result of irritation, which begins in the cells of the anterior horns, and is propagated thence through PROGRESSIVE MUSCULAR ATROPHIES. 4O9 normal or only half-wasted nerve-roots, to the peripheral muscular fibre. Positive as these anatomical findings seem to be, it is somewhat surprising to learn how few reliable post-mortem examinations have been made in these cases proving the correctness of these views. The cases of Pier- rot -Troissier, of Striimpell and of Hoffmann, are among the few which have been so carefully examined as to have placed the spinal origin of this special type of progressive muscular atrophy beyond question. In these cases the an- terior gray matter was the only part affected, and alone re- sponsible for the wide-spread muscular atrophy. There is no need in this treatise to give a typical history of a case of spinal progressive muscular atrophy, as it oc- curs in the adult. I have stated above that these cases are, as a rule, not hereditary, and upon this absence of the factor of inheritance the differential diagnosis was formerly fre- quently based between a progressive amyotrophy and a pro- gressive myopathy. But this point of differential diagnosis has been rudely shaken by the interesting article which Hoffmann published only recently concerning the occur- rence of chronic spinal muscular atrophy in children on an hereditary basis. I will endeavor to summarize one of his cases of HEREDITARY PROGRESSIVE MUSCULAR ATROPHY. (HOFFMANN.) A girl, four years of age ; the birth of the child was entirely normal ; when nine months of age was able to stand ; early abnormal development of adipose tissue. Gradually the child became so weak that it could not stand, could not sit upright in bed, could not turn around without assistance. For a long time it was able to move its feet and its arms. The motor dis- turbances increased gradually, and the child lost its superfluous fat and be- came thoroughly emaciated, particularly in the trunk and the extremities. The face remained full. The sphincters were at no time involved. Mental development was good. Speech was normal ; no convulsions ; no strabismus ; no difficulties in mastication or deglutition. The child was able to turn its head, but could not lift it from the pillow. There was no evidence of any hypertrophy or pseudo-hypertrophy in any of the muscles, but there was pa- resis and atrophy of the deep muscles of the neck, of the sterno-eleido-mas- toid, of the trapezius, of most of the shoulder-muscles, of the latissimus dorsi, of the serrati, the pectoral and deltoids, and of the flexor muscles in the arm. The biceps and brachialis anticus were very thin, the supinator longus a little stronger than these. The triceps was thin and weak. The extensors and 410 THE NERVOUS DISEASES OF CHILDREN. flexors in the forearm were also atrophic and paretic. The thenar and hypo- thenar eminences were thin, flaccid, and weak, as were also the individual in- terossei. The paralysis was in proportion to the atrophy of these muscles. The tendon reflexes were entirely wanting in the upper extremities. The mechanical excitability of the muscles was diminished. The nerve-trunks were neither thickened nor sensitive on pressure. The paresis and atrophy of both upper extremities were entirely symmetrical. There were no fibrillary movements. There were no trophic or vasomotor disturbances of the skin in the upper extremities. Sensation was entirely normal. There was marked diminution of electrical excitability in the median and ulnar nerves, and com- plete reaction of degeneration in the biceps. The muscles of the back and abdomen were very paretic, the long mus- cles of the spine much diminished in volume and power. There was lor- dosis of the lumbar region of the spinal column. The gluteal muscles, and all the muscles of the thigh, were very atrophic and almost completely para- lyzed. The muscles of the leg were also atrophic and paretic. Movements of the toes tolerably good. A progressive diminution in the volume of the calf and thigh muscles was noted at periods six months apart. No indica- tion of any deep reflexes in the lower extremities. The faradic excitability of the nerves in the lower extremities was distinctly diminished. No sensory disturbances, no fibrillary or fascicular or choreic movements of muscles. The paralysis was symmetrical and flaccid. The joints were like those of infantile spinal palsy. The child died of an intercurrent pulmonary trouble about one year after the first examination. The report of the autopsy included the following points : The lumbar portion of the spinal cord less in volume than under normal conditions. Very marked atrophy of the anterior spinal-cord roots throughout the entire spinal cord as high up as the medulla. On microscopical examination the chief changes found were in the anterior gray matter and in the anterior nerve-roots, from the lower portion of the medulla through the whole spinal cord. There was a distinct atrophy and diminution in number of the gan- glion cells of the anterior horns throughout the entire spinal cord. This was more marked in the lumbar than in the cervical portion. There was also very marked atrophy of the anterior roots and a similar affection in the peripheral nerves and the nerve-filaments in the muscles, as well as a very marked atrophy of the muscles supplied by these nerves. In addition to these chief changes there were also slight and symmetrical changes in the motor tracts of the spinal cord, particularly in the crossed pyramidal tracts and in the lateral columns as well as in the direct pyramidal tracts. Medulla oblongata was not involved. This case is so similar, from a clinical and anatomical point of view, to the typical cases of the Aran-Duchenne type that the close relationship be- tween these forms cannot be doubted. It remains to add that an entirely similar affection was reported in the case of a brother of the first child, in whom the disease began at about the same age and behaved in very much the same way. The parents of these two children have raised a family of fifteen ; several of these have died of convulsions, others are afflicted with PROGRESSIVE MUSCULAR ATROPHIES. 4 1 I distinct lipomatosis, and the parents invariably accepted the occurrence of this excessive accumulation of fat as an evil omen. Abortive and hereditary forms of progressive muscular atrophy of the spinal type have come under my notice. The cases are those of a physician, living in Canada, and his daughter, about twelve years of age ; in both of them there was distinct atrophy of the interossei of both hands, flattening of the thenar, and slight extension of the atrophy to muscles of the forearm. " Weak hands " have been characteristic of the family, but the disease does not appear seriously to invade other parts. The father (physician) has had this trouble for years, and is now compelled to give up surgical work. PROGRESSIVE NEURAL MUSCULAR ATROPHY — PROGRESSIVE NEUROTIC MUSCULAR ATROPHY — THE PERONEAL FORM, OR LEG TYPE, OF PROGRESSIVE MUSCULAR ATROPHY.* In this form of progressive muscular wasting the dis- ease begins, in the majority of cases, in the lower extrem- ities. At first the extensor muscles of the toes show a slight weakness. The small muscles of the feet may be- come involved, and then the atrophy spreads very much after the fashion of the spreading of the atrophy in the spinal cases, from muscle to muscle, until the entire leg is considerably atrophied and weakened. As a result of this weakening, deformities of the foot may arise ; pes equinus or pes equino-varus is a frequent result. In other cases a distinct club-foot is developed, and inasmuch as the affec- tion may spread quite rapidly from one side to the other, a progressive form of wasting in both lower extremities, including possibly the development of double club-foot, is extremely suggestive of this " leg type " of progressive muscular atrophy. The disease, as Hoffmann has shown, in rare instances attacks the upper extremities first and then involves the lower. Hoffmann has objected to the use of the designation " leg type," but since in this form the legs are involved at a very early stage, whether the disease begins in them or in the upper extremities, it seems proper for the present to retain this designation. The atrophy in the upper extremities may involve the small muscles of the hand, the extensor and flexor muscles of the forearm or the * This disease is also known as the type of Charcot-Marie-Tooth. Hoffmann sug- gested the term "progressive neurotic," Bernhardt, the term " progressive neural,'* muscular atrophy. 412 THE NERVOUS DISEASES OF CHILDREN. arm, and may cause a wasting of the muscles about the shoulder-girdle. I have seen the infra- and supra-spinati especially wasted in several of these cases. The atrophy in the upper extremities is, as a rule, not so distinct nor so early a symptom as in the cases of the Aran-Duchenne type. Fig. ioo. — Two Brothers afflicted with the Peroneal Form of Progressive Muscular Atrophy, Eight Months and One Year respectively after First Operation. Sensory changes are generally present, and serve as an important point of differentiation between this special form of atrophy and a spinal amyotrophy. The various forms of sensation may be slightly altered, or, in some cases, tac- tile sensation and temperature sense may remain normal, while the pain sense may be more distinctly involved. Par- amnesias may be present in addition to the objective changes in sensation. The reflexes in the lower extremities are either diminished or lost ; the exact state of the reflexes PROGRESSIVE MUSCULAR ATROPHIES. 413 depending somewhat upon the stage of the disease at the time the patient is examined. The electrical reactions in the atrophied muscles are, as a rule, altered. The changes are not so extreme as in the cases of spinal amyotrophy, nor are they as mild as in the primary muscular dystrophies. The reactions are diminished quantitatively, and altered also as regards the quality of contractions. A case has not yet been reported in which the muscles of the face were involved, and there were no changes in sensation, and none in electrical reactions in any of the muscles or nerves of the neck and head. The symptomatology of this rare form will be best elucidated by an extract from the account given of the disease as it occurred in two brothers which was reported in a paper published by the author in the year 1890. These cases are all the more interesting as they are the only ones, to my knowledge, which for a time were successfully treated by surgical measures. The family history is very meagre. The father, a Bavarian, is dead ; cause of death unknown. Mother, living and healthy, thirty- two years of age. The two patients were the only children. Both boys were born healthy. Each showed disturbances in the use of the legs at a very early day, and at the age of five years both had acquired double club-foot. When the younger brother was first admitted to the hospital, in 1887, he was compelled to use crutches. According to the hospital records there was marked shortening of the Achilles tendon and plantar fascia of both feet. Foot arched (pes cavus) ; when at rest inner side does not touch the floor. Measurements : Right calf, 7 inches ; left calf, 7 inches. Dr. Gibney performed double achillotomy. Separation of ends ij£ inch on the left and almost the same on the right side. Feet were flexed dorsally to about eighty degrees, and plaster-of-Paris splints were supplied. He was dis- charged four months after the operation, with a note that the patient walks quite well, soles flat on the ground, toes slightly inverted. He returned to the hospital in 1888 with paralytic limp and with a position of the feet as rep- resented in the accompanying figure. Double achillotomy was again per- formed, division of plantar fascia? was made, and there followed application of Thomas's tarsoclast and plaster splints with the results as shown in Figs. 101 and 102. Two months after the operation the feet were in typical cal- caneus position, when using his shoes without apparatus ; standing squarely on the soles of the feet he shows disposition to roll feet inward. He could voluntarily flex the ankle-joint a little beyond ninety degrees, but in so doing the toes were hyperextended. He walks very much as children do with a peroneal type of poliomyelitis. Marked disposition to pes varus. My own examination elicited the following points : The boy was of stouter and shorter stature than his brother. 4H THE NERVOUS DISEASES OF CHILDREN. Intelligence good. His broad chest and fat stomach are in curious contrast to his spindle-shaped extremities. Circum- ference of chest, 26 inches. Right arm, 6^ inches ; left arm, 63^ inches. Right forearm, 6^ inches ; left forearm, 6]^ inches. Grasp of both hands very weak. A general emaci- ation of all parts of upper ex- tremities. Very distinct atro- phy of infraspinatus. In the legs general atrophy is very well marked. The right thigh, four inches above pa- tella, 1 1 inches ; left thigh, 10^ inches. Right calf, at^ greatest circumference, 8 inches ; left calf, 8^ inches. The boy walks with a slightly waddling gait and has great difficulty in climbing the stairs. He can raise toes slight- ly on the left side, less well on the right side. Can raise left leg on tiptoe, but cannot do this with the right leg. In attempting to raise the whole body on tiptoe, falls forward. Sensation : Tactile sensation normal as determined by cot- ton, pin test, and the writing of numbers on the skin. Tem- perature sense normal. Pain sense exaggerated. Muscu- lar sense normal. Plantar re- flexes present and knee-jerk about normal. Slight lividity of legs, not so marked, however, as in the case of his brother. Fig. ioi. (Same patient as in Fig. 102.) The electrical examination : In the upper extremities the faradic response in the median and ulnar nerves was decidedly diminished. In the median nerve first KCC with 13 MA; ACC not at 20 MA. Galvanic current: In the right leg no reactions could be obtained by excitation of the nerves with currents used. In the extensor hallucis longus the first KCC and ACC were PROGRESSIVE MUSCULAR ATROPHIES. 415 obtained with a current of 14 MA. The tibialis anticus did not respond to currents of 20 MA. The anterior thigh muscles and posterior thigh muscles respond to strong currents of 16 MA, without reversal of formula. In the Jeft leg, the extensor hallucis, first KCC with 16 MA ; first ACC with 18 MA. No contractions could be obtained by direct excitation of the tibialis anticus, with currents up to 20 MA ; on excitation of the extensor digitorum communis there is a slight movement of the small toe. The electrical examination, there- fore, shows that the reaction of de- generation is present in its typical form in most of the muscles below the patella, the galvanic excitability of the peroneal nerve being entirely lost. It also shows changes in elec- trical behavior in nerves of the upper extremities, since the responses of the ulnar and median nerves were markedly diminished. Comparing the histories of the two brothers, it was noted that they resemble each other very closely as regards the first appearance of the symptoms and the manner in Avhich the disease spread from muscle to mus- cle ; but there were also certain differences, such as the more marked electrical changes in the younger brother and the greater in- volvement of the upper ex- tremities in him, than in the older boy. In the younger brother the disease was more fully developed in every re- spect than in his older broth- er ; but such variations as occurred were within a reason- able limit and will serve to show to what extent variations may occur in persons undoubtedly suffering from the same type of disease. Fig. 102. — The Vounger of the Two Brothers (see Fig. 100) after Second Operation, showing Correction of De- formity of Feet, Marked Atrophy of Legs, and Incipient Atrophy of Muscles above Elbow and around the Shoulder Girdle. 41 6 THE NERVOUS DISEASES OF CHILDREN. In addition to the symptoms which these boys have exhibited, it is inter- esting to note that Vizioli has reported the occurrence of amaurosis in a similar case, due to an optic-nerve atrophy. This symptom would support the argument in favor of the nerve origin of the disease. Furthermore we may insist on the fact that hypertrophy has not been recorded in any case, and that fibrillary movements seem to occur in some. Thomson and Bruce have reported an interesting case of a progressive muscular atrophy in a child ; but they nave not attempted to classify it under any special type. The disease began in the lower extremities, and gradually extended to the upper, involving both upper extremities and the muscles of the back. The gradual changes are well represented in Figs. 103-105. The child exhibited some disturbances of sensation (hyperalgesia) ; marked paresis in neck, back, and abdomen — most marked in loins, buttocks, and legs ; least marked in shoulders and arms ; no hypertrophy of any muscles. Electrical reactions at first little altered ; later on faradic excitability consid- erably impaired ; more markedly in the legs than in the arms. The report of the galvanic responses is too uncertain to permit of positive inferences ; the disease was steadily progressive. The case is all the more unique, as the authors found a spinal lesion and only very slight changes in the peripheral nerves.* Etiology. — Nothing more need be said upon this head than may be inferred from the previous histories. The disease is evidently a family affection, sometimes beginning at a very early age, as in my own cases, or appearing as late as the age of twenty, as in the one case described by Charcot-Marie. Whether the cases described by Osier as occurring in the Farr family of Vermont, which set in as late as the age of forty-six in some of the subjects, belong to this category or not is questionable, as the cases were reported before this special type of progressive muscular atrophy was known. In both my cases a thorough drench- ing of the skin by exposure to wet was mentioned, but I can- not attribute any further importance to this fact than that it may have helped to hasten a disease which was latent in the systems of the boys. Diagnosis. — The diagnosis of this "leg type," or neural form, of progressive muscular atrophy rests upon the recog- nition of the early beginning of the disease and of its hered- itary or family character. Moreover, the paralysis begin- ning in the leg muscles, and spreading to the upper extrem- *The case appears to bear a close resemblance to the peroneal form, as well as to an hereditary spinal form. PROGRESSIVE MUSCULAR ATROPHIES. 417 - 3 "-^^^Bi ■P$ ; *M|; p& ; v*'M" i%>" ■ ? ^li mm? s/ MM P^, ,^^*** 1 ^%^ * -***||g^'*\ ^fe^ ta^..... 1 Hi "3 S-. Pi tH) o