key: cord-0754759-8qdjjnxn authors: Baccarella, Alyssa; Linder, Alexandra; Spencer, Robert; Jonokuchi, Alexander; King, Paul Benjamin; Maldonado-Soto, Angel; Boneparth, Alexis; Hooe, Benjamin S.; Schweickert, Adam J.; Carlin, Rebecca; Kingery, Francesca; Vargas, Wendy S.; Sewell, Taylor B.; Silver, Wendy G. title: Increased intracranial pressure in the setting of Multisystem Inflammatory Syndrome in Children, associated with COVID-19 date: 2020-11-22 journal: Pediatr Neurol DOI: 10.1016/j.pediatrneurol.2020.11.008 sha: aa57ff1cd23e0b51eaa47aff0315d7f047863479 doc_id: 754759 cord_uid: 8qdjjnxn nan Multisystem inflammatory syndrome in children (MIS-C) is now an established sequalae of SARS-CoV-2 infection 1 ; however, pediatric neurologic manifestations are just beginning to be characterized 2 . We describe two children who presented with MIS-C and were found to have evidence of increased intracranial pressure (ICP). A previously healthy, fully vaccinated 9-year-old male presented to the emergency department with diplopia and worsening headache following a seven-day febrile illness with abdominal pain. He was diagnosed with MIS-C by the CDC diagnostic criteria 3 and admitted. Neurological examination revealed a right abducens palsy without papilledema. Antibodies to SARS-CoV-2 were present, whereas SARS-CoV-2 reverse A previously healthy, fully vaccinated 6-year-old male was seen in clinic for diplopia and was found to have a right abducens palsy and bilateral papilledema. He had been discharged from the hospital the previous day after a week-long admission to treat MIS-C, diagnosed as per the CDC criteria 3 . During this hospitalization, he demonstrated both SARS-CoV-2 RT-PCR nasopharyngeal swab and antibody positivity, and he required ICU admission for vasopressor support while being treated as per our institution's MIS-C protocol 4 . Prior to hospital discharge, he had several days of headache and was closing one eye while watching television, which had been attributed to failure to wear his glasses. However, upon re-presentation, and in light of his new focal neurological findings these were reinterpreted as early signs of increased ICP. As such, the patient was readmitted for further workup. MRI brain and orbits was significant for kinking and distention of the bilateral optic nerve sheaths with protrusion of the optic discs into the globes, consistent with increased ICP. Despite the papilledema, LP under sedation demonstrated a normal OP of 14 cm H 2 O; however, the LP was notably performed one week after the patient had been first observed with symptoms attributable to his abducens palsy. His CSF studies, including SARS-CoV-2 RT-PCR testing, were normal. The following morning, though his papilledema persisted, his diplopia had resolved and he was discharged home on a continued steroid taper. Five months post-presentation, his papilledema has resolved, and he is without recurrence of symptoms. We report two cases of increased ICP in the setting of MIS-C. Neither child was obese. At the time that Patient I was noted to have elevated OP on LP, he had not previously taken or received medications known to cause increased ICP. While Patient II did receive intravenous immunoglobulin and steroids (as part of his MIS-C treatment) prior to developing his neurologic symptoms, his CSF studies were normal, and steroids had only recently been initiated, making these medications unlikely causative factors for his increased ICP. It should be noted that these patients' laboratory studies revealed PCR and antibody positivity to other pathogens. However, there was no consistent infection pattern across the patients aside from SARS-CoV-2, and no other detected pathogen would explain the full constellation of each patient's symptoms. Therefore, it is unlikely that these other pathogens explain the patients' findings of increased ICP; rather, their detection likely reflects either prior or concurrent infection, as has been reported with SARS-CoV-2 infection 5 . Increased ICP has been noted as the initial manifestation of some systemic inflammatory disorders, including lupus, Sjogren's syndrome, and Kawasaki disease 6,7,8 . We posit that our patients' increased ICP similarly reflects systemic inflammation related to SARS-CoV-2 infection, resulting in central nervous system (CNS) effects. Given our patients' normal CSF studies, including negative SARS-CoV-2 RT-PCR testing, it is less likely that our patients experienced direct CNS infection with SARS-CoV-2. As more children present with MIS-C, we recommend that practitioners remain vigilant for signs and symptoms of increased ICP in these patients. J o u r n a l P r e -p r o o f Multisystem Inflammatory Syndrome in U.S. Children and Adolescents Neurologic and Radiographic Findings Associated with COVID-19 Infection in Children Centers for Disease Control and Prevention. Multisystem Inflammatory Syndrome in Children (MIS-C) Associated with Coronavirus Disease An Institutional Protocol for Evaluation, Management, and Follow-up of Multi-system Inflammatory Children Associated with Coronavirus Disease 2019 in a Children's Hospital in Rates of Co-infection Between SARS-CoV-2 and Other Respiratory Pathogens Pseudotumor cerebri associated with Sjögren's syndrome Bilateral Abducens Nerve Palsy due to Idiopathic Intracranial Hypertension as an Initial Manifestation of Systemic Lupus Erythematosus We thank the patients and their families for allowing us to publish their cases. We thank The authors report no conflicts of interest.