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Neurology, Vol 50, Issue 2 554-557, Copyright © 1998 by American Academy of Neurology


ARTICLES

Familial temporal lobe epilepsy: a clinically heterogeneous syndrome

F Cendes, I Lopes-Cendes, E Andermann and F Andermann
Department of Human Genetics, McGill University, Montreal, Quebec, Canada.

We describe the clinical characteristics of a group of patients with familial temporal lobe epilepsy (TLE) in 11 kindreds with 36 affected individuals identified and investigated at the Montreal Neurological Hospital. Seizure types were simple partial (n = 20), complex partial (n = 29), and rare generalized tonic-clonic. Simple and complex partial seizures were infrequent or well controlled by anticonvulsant medication in 17 of 29 patients (59%) and without optimal response to medical therapy in 12 of 29 patients (41%). Pedigree analysis suggested autosomal dominant inheritance with incomplete penetrance. The syndrome of familial TLE has heterogeneous clinical manifestations and is not always benign.


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