TY - CHAP M1 - Book, Section TI - Severe Myoclonic Epilepsy of Infancy (Dravet Syndrome) A1 - Dravet, Charlotte A2 - Duchowny, Michael A2 - Cross, J. Helen A2 - Arzimanoglou, Alexis PY - 2017 T2 - Pediatric Epilepsy 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. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/10/04 UR - neurology.mhmedical.com/content.aspx?aid=1138410062 ER -