International League against Epilepsy Classification And Definition of Epilepsy Syndromes with Onset in Childhood: Position Paper by the ILAE Task Force on Nosology and Definitions

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Date
2022-03Author
Gwer, Sam
Spechio, Nicola
Wirrell2, Elaine C.
Scheffer, Ingrid E.
Nabbout, Rima
Moshé, Solomon L.
Tinuper, Paolo
Auvin, Stéphane
Perucca, Emilio
Cross, Helen J
Samuel, Wiebe
Pressler, S. W.
Yozawitz, Elissa
Samia, Pauline
Wilmshurst, Jo M.
Hirsch, Edouard
Guerreiro, Marilisa
Zuberi, Sameer M.
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The 2017 International League Against Epilepsy classification has defined a three-tier
system with epilepsy syndrome identification at the third level. Although a syndrome
cannot be determined in all children with epilepsy, identification of a specific syndrome
provides guidance on management and prognosis. In this paper, we describe
the childhood onset epilepsy syndromes, most of which have both mandatory seizure
type(s) and interictal electroencephalographic (EEG) features. Based on the 2017
Classification of Seizures and Epilepsies, some syndrome names have been updated
using terms directly describing the seizure semiology. Epilepsy syndromes beginning
in childhood have been divided into three categories: (1) self-limited
focal epilepsies,
comprising four syndromes: self-limited
epilepsy with centrotemporal spikes, self-limited
epilepsy with autonomic seizures, childhood occipital visual epilepsy, and
photosensitive occipital lobe epilepsy; (2) generalized epilepsies, comprising three syndromes:
childhood absence epilepsy, epilepsy with myoclonic absence, and epilepsy
with eyelid myoclonia; and (3) developmental and/or epileptic encephalopathies,
comprising five syndromes: epilepsy with myoclonic–atonic
seizures, Lennox–Gastaut
syndrome, developmental and/or epileptic encephalopathy with spike-and-
wave
activation
in sleep, hemiconvulsion–hemiplegia–
epilepsy
syndrome, and febrile infection-related
epilepsy syndrome. We define each, highlighting the mandatory seizure(s),
EEG features, phenotypic variations, and findings from key investigations
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