Dravet syndrome (DS) is a rare but severe encephalopathy with onset of seizures in a normal infant before the age of one year in a fever context. Infants develop prolonged febrile unilateral, asymmetric or apparently generalized seizures. From the second year of life on, psychomotor delays appear and refractory epilepsy develops, with several seizure types. There is no epileptic spasm, and tonic seizures are infrequent in this syndrome. No characteristic MRI change has been described. Molecular genetic analysis has shown mutations in the SCN1A (sodium channel) gene in over 80 % of cases [1].
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