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
2022-03
Authors
Gwer, Sam
Spechio, Nicola
Wirrell2, Elaine C.
Scheffer, Ingrid E.
Nabbout, Rima
Moshé, Solomon L.
Tinuper, Paolo
Auvin, Stéphane
Perucca, Emilio
Cross, Helen J
Journal Title
Journal ISSN
Volume Title
Publisher
Wiley online library
Abstract
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
Description
Article
Keywords
benign occipital epilepsy, childhood epilepsy with centrotemporal spikes, continuous spike-and- wave in sleep eyelid myoclonia, febrile infection-related epilepsy syndrome, hemiconvulsion–hemiplegia– epilepsy syndrome, Landau–Kleffner syndrome, Lennox–Gastaut syndrome, myoclonic absences, myoclonic atonic, Panayiotopoulos syndrome
Citation
Specchio, N., Wirrell, E. C., Scheffer, I. E., Nabbout, R., Riney, K., Samia, P., ... & Auvin, S. (2022). 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. Epilepsia, 63(6), 1398-1442.