Developmental and Epileptic Encephalopathies
Developmental and Epileptic Encephalopathies (DEE) comprise a group of severe epileptic disorders characterized by both frequent, often drug-resistant seizures and significant developmental impairment. The term “encephalopathy” in this context refers to marked delays in developmental milestones or, in some cases, loss of previously acquired s****s.
DEE syndromes typically begin in the neonatal period, infancy, or early childhood and include: early myoclonic encephalopathy, Ohtahara syndrome, West syndrome, Dravet syndrome (severe myoclonic epilepsy of infancy), Lennox–Gastaut syndrome (LGS), Landau–Kleffner syndrome (LKS), epilepsy with continuous spike-and-wave during slow-wave sleep (ECSWS), Doose syndrome (myoclonic–atonic epilepsy), CDKL5 deficiency disorder (CDD), tuberous sclerosis complex (TSC), SCN8A-DEE, and KCNQ2-DEE.
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