Efforts to duplicate the genetic and molecular breakthroughs of autosomal dominant frontal lobe epilepsy have lead to increased interest in familial temporal lobe epilepsy. In this report we describe three kindreds. The epilepsy syndrome described manifests after the teenage years and was generally mild and treatment responsive. The predominant seizure types were simple and complex partial seizures, typical of mesial temporal onset. Some family members had febrile convulsions only and others had epilepsy without preceding febrile convulsions. Three patients had both febrile convulsions and temporal lobe epilepsy. High-resolution quantitative and qualitative MRI was normal. The syndrome in these three kindreds is distinct from temporal lobe epilepsy due to mesial temporal sclerosis and febrile convulsions and is probably a form of idiopathic localization related epilepsy. Its relation to other familial temporal lobe epilepsy phenotypes is discussed.
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