Hemiconvulsion-hemiplegia-epilepsy syndrome in South African children: insights from a retrospective case series.

INTRODUCTION: Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome is a recognized sequel of febrile partial status in children younger than 4 years.

OBJECTIVE: To describe the clinical features, neuroradiology and outcome in 8 South African children with HHE syndrome.

METHOD: A retrospective descriptive study of 8 consecutive cases of HHE syndrome presenting to tertiary hospitals in the Western Cape over a 2 year period.

RESULTS: The median age of onset of convulsive status was 16 months (range: 9-36 months). Gender distribution was equal. The duration of the initial episode of status exceeded 2 h in all children. All children were reported to have been developmentally normal prior to the onset of the first seizure and none previously suffered seizures or had a family history of febrile seizures and epilepsy. In 7 of the 8 cases the initial seizure was not associated with fever or preceding illness. Imaging demonstrated cerebral hemiatrophy in all and additional crossed cerebellar atrophy in 2 children. Moderate to severe intellectual disability ensued in the majority of children. The severity of the intellectual disability correlated with the degree of the motor deficit and occurred irrespective of the cerebral hemisphere involved.

CONCLUSION: In contrast to developed countries, HHE syndrome is still prevalent in South Africa. The neurological morbidity in South African children is significant and highlights the need for improved emergency care of status epilepticus.