A novel GABRG2 mutation associated with febrile seizures.

Audenaert, D; Schwartz, E; Claeys, K G; Claes, L; Deprez, L; Suls, A; Van Dyck, T; Lagae, L et al. · Neurology · 2006

case_series · Level IV

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Abstract

Mutations in the gene encoding the gamma2 subunit of the gamma-aminobutyric acid type A receptor (GABRG2) have been reported to cause childhood absence epilepsy (CAE), febrile seizures (FS), and generalized epilepsy with FS plus (GEFS+). The authors analyzed GABRG2 in 47 unrelated patients with CAE, FS, and GEFS+ and identified a novel mutation that cosegregated with FS. Electrophysiologic studies demonstrated altered current desensitization and reduced benzodiazepine enhancement in mutant receptors.

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