title: Early-onset epileptic encephalopathies: Ohtahara syndrome and early myoclonic encephalopathy.
abstract: Ohtahara syndrome and early myoclonic encephalopathy are the earliest presenting of the epileptic encephalopathies. They are typically distinguished from each other according to specific clinical and etiologic criteria. Nonetheless, considerable overlap exists between the two syndromes in terms of clinical presentation, prognosis, and electroencephalographic signature. Newer understandings of underlying etiologies of these conditions may support the previously suggested concept that they represent a single spectrum of disease rather than two distinct disorders. We review both syndromes, with particular focus on the underlying genetics and pathophysiology and implications regarding the classification of these conditions.
authors.affiliation: Saul R. Korey Department of Neurology, Albert Einstein College of Medicine and Montefiore Medical Center, Bronx, New York, USA. jbeal@montefiore.org
authors.forename: Jules C
authors.initials: JC
authors.lastname: Beal
authors.forename: Koshi
authors.initials: K
authors.lastname: Cherian
authors.forename: Solomon L
authors.initials: SL
authors.lastname: Moshe
mesh_terms: D000367:Age Factors; D017668:Age of Onset; D000818:Animals; D004569:Electroencephalography / Q000379:methods; D004831:Epilepsies, Myoclonic / Q000453:epidemiology* / Q000235:genetics* / Q000503:physiopathology; D006801:Humans; D013577:Syndrome