Severe myoclonic epilepsy in infancy (SME) was described by Charlotte Dravet in 1978. In the Revised ILAE classification of epilepsies, the SMEI is named “Dravet syndrome” because of the lack of myoclonic seizures in many patients and is considered under Electroclinical syndromes [Berg et al., 2010]. Dravet syndrome is characterized by febrile and afebrile generalized and unilateral clonic or tonic clonic seizures that occur in the first year of life in an otherwise normal infant and are later associated with myoclonus, atypical absences, and partial seizures. All seizure types are resistant to antiepileptic drugs with developmental delay becomes apparent within the second year of life and is followed by definite cognitive impairment and personality disorders.

Epidemiology

• Incidence probably less than 1 per 40,000
• Males are more often affected than females, in a ratio of 2:1.

Clinical features

Details

• Editor(s): CNKE contributors
• Needs Update