tetano
Editor, Senior Moderator
Neurology
. 2022 Aug 25;10.1212/WNL.0000000000201226.
doi: 10.1212/WNL.0000000000201226. Online ahead of print.
Child Neurology: Hemiconvulsion-Hemiplegia-Epilepsy Syndrome in the Setting of COVID-19 Infection and Multisystem Inflammatory Syndrome
Paul Gong[SUP] 1 [/SUP], Cemal Karakas[SUP] 2 3 [/SUP], Bethanie Morgan[SUP] 2 3 [/SUP]
Affiliations
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.
. 2022 Aug 25;10.1212/WNL.0000000000201226.
doi: 10.1212/WNL.0000000000201226. Online ahead of print.
Child Neurology: Hemiconvulsion-Hemiplegia-Epilepsy Syndrome in the Setting of COVID-19 Infection and Multisystem Inflammatory Syndrome
Paul Gong[SUP] 1 [/SUP], Cemal Karakas[SUP] 2 3 [/SUP], Bethanie Morgan[SUP] 2 3 [/SUP]
Affiliations
- PMID: 36008144
- DOI: 10.1212/WNL.0000000000201226
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.