McLendon, Loren

Encephalopathy is a disturbance in neurologic function. It is commonly attributed to infectious and inflammatory etiologies but encompasses a wide differential. Hypereosinophilic syndrome (HES) is a rare cause of encephalopathy that is associated with underlying infection or malignancy. We present a pediatric patient who experienced several days of worsening encephalopathy and neurological deterioration associated with influenza B infection and hypereosinophilia. A thorough workup ultimately revealed a diagnosis of HES secondary to B cell acute lymphoblastic leukemia (B-ALL). This case exemplifies the importance of maintaining a broad approach to the diagnostic evaluation, treatment, and management of encephalopathy.

Background: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an inflammatory disorder of the CNS with a variety of clinical manifestations, including cerebral edema. Case Summary: A 7-year-old boy presented with headaches, nausea, and somnolence. He was found to have cerebral edema that progressed to brainstem herniation. Invasive multimodality neuromonitoring was initiated to guide management of intracranial hypertension and cerebral hypoxia while he received empiric therapies for neuroinflammation. Workup revealed serum myelin oligodendrocyte glycoprotein antibodies. He survived with a favorable neurologic outcome. Conclusion: We describe a child who presented with cerebral edema and was ultimately diagnosed with MOGAD. Much of his management was guided using data from invasive multimodality neuromonitoring. Invasive multimodality neuromonitoring may have utility in managing life-threatening cerebral edema due to neuroinflammation.

Neurologic manifestations of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in pediatric patients have been reported in the acute and postinfectious stages of coronavirus disease 2019. Acute disseminated encephalomyelitis (ADEM) typically presents in children after a viral illness at a mean age of 3 to 7 years. A total of 60% to 90% of literature-reported pediatric patients with ADEM have minimal to no neurologic deficits at long-term follow-up. We present a 17-month-old developmentally typical girl with parental complaints of irritability, upper extremity weakness, and gait disturbance. She presented to the hospital afebrile and irritable with right-sided nasolabial fold flattening, neck stiffness, left upper extremity rigidity, right upper extremity paresis, bilateral lower extremity hyperreflexia, and truncal ataxia. During her hospital course, she became somnolent with autonomic instability and was transferred to intensive care. Contrasted brain MRI revealed diffuse patchy T2 hyperintensities without contrast enhancement. Nasopharyngeal SARS-CoV-2 polymerase chain reaction and serum antibody testing results were positive. Cerebral spinal fluid analysis was unremarkable. Respiratory viral panel and autoimmune encephalitis and demyelinating disorders panel results were negative. She was started on high-dose methylprednisolone and intravenous immunoglobulin, with improvement in mental status, focal deficits, and ambulation. After hospital discharge, she received inpatient rehabilitation for 2 weeks and at 2 month follow-up had a full neurologic recovery. We report the youngest case of postinfectious ADEM due to SARS-CoV-2 in a toddler. Early recognition of autoimmune and inflammatory complications of SARS-CoV-2 is vital for early aggressive immunomodulatory treatment and, consequently, improved morbidity in these patients.