Article

Child Neurology: Hemiconvulsion-hemiplegia-epilepsy syndrome

Department of Neurology, Cincinnati Childrens Hospital Medical Center, Cincinnati, OH, USA.
Neurology (Impact Factor: 8.3). 07/2012; 79(1):e1-4. DOI: 10.1212/WNL.0b013e31825dce5f
Source: PubMed

ABSTRACT Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome is an uncommon outcome of prolonged focal 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 followed by chronic atrophy. Intractable epilepsy may develop at a time remote from the initial presentation. 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 accurate prognosis regarding the subsequent development of epilepsy.

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