Child Neurology: Hemiconvulsion-hemiplegia-epilepsy syndrome

Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome is an uncommon outcome of prolonged fo-cal status epilepticus in childhood. The prolonged focal motor seizure usually occurs during the course of a febrile illness and is followed by hemiplegia ipsilateral to the side of convulsions. This is accompanied by radiologic evidence of acute cytotoxic edema in the affected hemisphere fol-lowed by chronic atrophy. Intractable epilepsy may develop at a time remote from the initial pre-sentation. The clinical features of HHE syndrome were first described more than 5 decades ago but its pathophysiology remains poorly understood and the long-term cognitive outcomes are unclear. Early recognition of the syndrome may help provide patients and families with an accu-rate prognosis regarding the subsequent development of epilepsy. Neurology ® 2012;79:e1–e4 GLOSSARY FIRES fever-induced refractory epileptic encephalopathy in school-aged children; HH hemiconvulsion-hemiplegia; HHE hemiconvulsion-hemiplegia-epilepsy; HHS hemiconvulsion-hemiplegia syndrome; MR magnetic resonance; NORSE new-onset refractory status epilepticus. CLINICAL CASE, PART I A 21-month-old boy, former 25-week premature infant, with a history of bilatera