Article
Fatal Cerebral Edema With Status Epilepticus in Children With Dravet Syndrome: Report of 5 Cases.
Pediatrics - 1 Apr 2017
Myers Kenneth A, McMahon Jacinta M, Mandelstam Simone A, Mackay Mark T, Kalnins Renate M, Leventer Richard J, Scheffer Ingrid E
Abstract excerpt
Dravet syndrome (DS) is a well-recognized developmental and epileptic encephalopathy associated with SCN1A mutations and 15% mortality by 20 years. Although over half of cases succumb to sudden unexpected death in epilepsy, the cause of death in the remainder is poorly defined. We describe the clinical, radiologic, and pathologic characteristics of a cohort of children with DS and SCN1A mutations who developed...
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