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 skills.

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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