Nav1.1 mutations cause febrile seizures associated with afebrile partial seizures.

Sugawara, T; Mazaki-Miyazaki, E; Ito, M; Nagafuji, H; Fukuma, G; Mitsudome, A; Wada, K; Kaneko, S et al. · Neurology · 2001

case_series · Level IV

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Abstract

Recent evidence has suggested that the neuronal voltage-gated sodium channel alpha(1)-subunit gene (Na(v)1.1: SCN1A) is responsible for generalized epilepsy with febrile seizures plus (GEFS+2). Here the authors report two novel disease mutations of Na(v)1.1 in patients with febrile seizures associated with afebrile partial seizures. One is a Val1428Ala substitution in the pore-forming region, and the other is Ala1685Val in the transmembrane helix. These results support the previous findings and contribute to the reliable diagnosis of epilepsy.

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