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

Area of Science:

• Pediatric Neurology
• Infectious Diseases
• Epileptology

Background:

• Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome is a rare pediatric epilepsy characterized by prolonged focal status epilepticus, hemiplegia, and subsequent epilepsy.
• The pathophysiology involves hyperthermia, inflammation, and cytotoxic edema, but remains incompletely understood.
• Prognosis for HHE syndrome varies significantly, ranging from recovery to permanent neurological deficits.

Observation:

• A 2-year-old boy presented with superrefractory focal status epilepticus during acute COVID-19 and MIS-C.
• Neurological examination revealed right-sided hemiplegia.
• Brain MRI demonstrated left cerebral hemispheric edema, consistent with HHE syndrome.

Findings:

• This case represents the first documented instance of HHE syndrome associated with acute COVID-19 and MIS-C.
• The findings suggest a potential link between COVID-19, MIS-C, and the development of HHE syndrome.
• The clinical presentation included severe, prolonged seizures and unilateral cerebral edema.

Implications:

• This report expands the known triggers for HHE syndrome, suggesting infectious and inflammatory processes like COVID-19 and MIS-C may play a role.
• Further research is warranted to elucidate the mechanisms linking COVID-19/MIS-C to HHE syndrome.
• Understanding this association may inform diagnostic and therapeutic strategies for pediatric epilepsy in the context of viral infections.