title: Familial temporal lobe epilepsy: a clinically heterogeneous syndrome.
abstract: 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.
authors.affiliation: Department of Human Genetics, McGill University, Montreal, Quebec, Canada.
authors.forename: F
authors.initials: F
authors.lastname: Cendes
authors.forename: I
authors.initials: I
authors.lastname: Lopes-Cendes
authors.forename: E
authors.initials: E
authors.lastname: Andermann
authors.forename: F
authors.initials: F
authors.lastname: Andermann
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000927:Anticonvulsants / Q000627:therapeutic use; D002648:Child; D004828:Epilepsies, Partial / Q000235:genetics / Q000503:physiopathology; D017029:Epilepsy, Complex Partial / Q000235:genetics / Q000503:physiopathology; D004833:Epilepsy, Temporal Lobe / Q000188:drug therapy / Q000235:genetics* / Q000503:physiopathology; D004830:Epilepsy, Tonic-Clonic / Q000235:genetics / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D013577:Syndrome
chemical_list: D000927:Anticonvulsants