RT Book, Section A1 Dravet, Charlotte A2 Duchowny, Michael A2 Cross, J. Helen A2 Arzimanoglou, Alexis SR Print(0) ID 1138410062 T1 Severe Myoclonic Epilepsy of Infancy (Dravet Syndrome) T2 Pediatric Epilepsy YR 2017 FD 2017 PB McGraw-Hill Education PP New York, NY SN 9780071496216 LK neurology.mhmedical.com/content.aspx?aid=1138410062 RD 2024/03/28 AB Recognized as a syndrome by the International League Against Epilepsy in the last classification,1 severe myoclonic epilepsy of infancy (SMEI) has been placed among the "epileptic encephalopathies", defined as conditions in which the epileptiform abnormalities are believed to contribute to a progressive disturbance in cerebral function, whether seizures are generalized, localized, symptomatic, idiopathic or cryptogenic.2 Since 20013 its etiology is regarded to be genetic as the majority of affected patients carry a mutation in the sodium-channel gene SCN1A. However, it remains unproven whether the cognitive decline observed in the first stages of the disease is a consequence of the epilepsy. In this new scheme, SMEI is renamed "Dravet syndrome" because of the lack of myoclonic seizures in many patients.