Child Neurology: Hemiconvulsion-Hemiplegia-Epilepsy Syndrome in the Setting of COVID-19 Infection and Multisystem Inflammatory Syndrome

Abstract

Hemiconvulsion-Hemiplegia-Epilepsy (HHE) syndrome is a rare pediatric epilepsy syndrome characterized by prolonged focal febrile convulsive status epilepticus with unilateral hemispheric cerebral edema and followed by the subsequent development of hemiplegia, global atrophy of the affected hemisphere, and epilepsy. The pathophysiology of HHE syndrome remains poorly understood though is clearly multifactorial. Factors thus far implicated are hyperthermia, pro-inflammatory state, and cytotoxic edema from prolonged ictal activity. Prognosis is variable, from the resolution of hemiplegia and seizures to permanent hemiparesis and refractory epilepsy. We describe a 2-year-old boy who presented with super-refractory focal status epilepticus in the setting of acute Coronavirus Infectious Disease-2019 (COVID-19) and multisystem inflammatory syndrome in children (MIS-C). He had right-sided hemiplegia on neurological examination, and an MRI of the brain showed left cerebral hemispheric edema consistent with HHE syndrome. Our case represents the first report in the literature on HHE syndrome in the setting of acute COVID-19 and MIS-C.

Response to Letter to the Editor from Finsterer: “Encephalitis Associated With SARS-CoV-2 Infection in a Child With Chiari Malformation Type I”

“…However, we cannot exclude subclinical cerebral atrophy of another, as yet unidentified, etiology or cognitive impairment manifesting before encephalitis and independently from SARS-CoV-2 infection. In this sense, cerebral atrophy has been observed in children with COVID-19 (17)(18)(19).…”