Exome sequencing revealed a pathogenic mutation in GABRG2. Clinical features of family A were described previously.19 This large 5-generation French family included 9 individuals with seizures (table). All 9 patients presented simple FS in early childhood (age at onset range: 12 months to 3 years), 7 of 9 individuals subsequently developed focal epilepsy with EEG and clinical features strongly suggestive of TLE, and 1 patient had only generalized tonic-clonic seizures (GTCS). Brain MRI showed normal hippocampal architecture and volume on both sides in 5 patients. Interictal EEGs showed epileptiform abnormalities localized in the temporal lobe in 6 patients, and was normal in 1 patient (A/II-6) with FS only.
