TY - JOUR
T1 - International League Against Epilepsy classification and definition of epilepsy syndromes with onset in childhood
T2 - Position paper by the ILAE Task Force on Nosology and Definitions
AU - Specchio, Nicola
AU - Wirrell, Elaine C.
AU - Scheffer, Ingrid E.
AU - Nabbout, Rima
AU - Riney, Kate
AU - Samia, Pauline
AU - Guerreiro, Marilisa
AU - Gwer, Sam
AU - Zuberi, Sameer M.
AU - Wilmshurst, Jo M.
AU - Yozawitz, Elissa
AU - Pressler, Ronit
AU - Hirsch, Edouard
AU - Wiebe, Sam
AU - Cross, Helen J.
AU - Perucca, Emilio
AU - Moshé, Solomon L.
AU - Tinuper, Paolo
AU - Auvin, Stéphane
N1 - Publisher Copyright:
© 2022 The Authors. Epilepsia published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.
PY - 2022/6
Y1 - 2022/6
N2 - 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.
AB - 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.
KW - Landau–Kleffner syndrome
KW - Lennox–Gastaut syndrome
KW - Panayiotopoulos syndrome
KW - benign occipital epilepsy
KW - childhood epilepsy with centrotemporal spikes
KW - continuous spike-and-wave in sleep
KW - eyelid myoclonia
KW - febrile infection-related epilepsy syndrome
KW - hemiconvulsion–hemiplegia–epilepsy syndrome
KW - myoclonic absences
KW - myoclonic atonic
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U2 - 10.1111/epi.17241
DO - 10.1111/epi.17241
M3 - Article
C2 - 35503717
AN - SCOPUS:85129272393
SN - 0013-9580
VL - 63
SP - 1398
EP - 1442
JO - Epilepsia
JF - Epilepsia
IS - 6
ER -