The hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome has been known for a long time, but the first detailed electroclinical descriptions came from Henri Gastaut and the Marseilles school. The incidence of seizures occurring after prolonged febrile convulsions has progressively decreased over the past 30 years, due mostly to a better prevention of longlasting febrile seizures or status and to the widespread use of benzodiazepines since the 1960s; recently diagnosed cases are mostly of a symptomatic nature, and are associated with underlying brain lesions. The classical HHE syndrome is characterized by the succession of lateralized febrile seizures, focal cerebral edema and resulting various forms of focal atrophy, including hippocampal sclerosis but also other localizations and more diffuse epileptogenic lesions. Epilepsy begins between 1 and 20 years after the initial event, and seizures are not always of the typical, psychomotor type. Preventive treatment of longlasting febrile seizures is important, but there is no standard therapy for epilepsy in HHE syndrome, as many different clinical situations may be encountered in this context.
|Translated title of the contribution||Hemiconvulsion-hemiplegia-epilepsy syndrome (HHE)|
|Number of pages||8|
|Publication status||Published - Jun 1996|
ASJC Scopus subject areas
- Clinical Neurology