Benign neonatal familial convulsions: a model of idiopathic epilepsy.

Benign neonatal familial convulsions have been recognized as a distinctive epileptic syndrome since 1964. This rare epileptic syndrome was classified in the category of idiopathic generalized epilepsies. Recently, mutations o f potassium channel genes (KCNQ2, KCNQ3) were identified as responsible for this autosomic dominant epileptic syndrome. Generalized tonico-clonic seiz ures start at the second or third day after birth in children with no prena tal or perinatal pathological history. Interictal EEG is normal. This epile psy is age-dependent: less than ten percent of children present seizures la ter in life. Despite their rarity, BNFC represent a useful model to underst and the pathophysiology of idiopathic age dependant epilepsies.