ABSTRACT
Severe myoclonic epilepsy in infancy (SMEI) is an epileptic syndrome with distinctive electroclinical features described first by Dravet1 and subsequently by many authors.2-7 The occurrence in a significant number of patients of a similar electroclinical picture except for the absence of myoclonic seizures prompted the term ‘severe polymorphic epilepsy of infancy’.8,9 The Task Force on Classification of the International League Against Epilepsy (ILAE) has more recently recommended the name Dravet syndrome.10 The prevalence of Dravet syndrome has been estimated to be 1/20 0001/40 000 infants,11,12 and boys are more often affected than girls.
