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

• DOI: 10.1111/epi.17241
• Publication Date: 2022-05-03T16:50:06Z
• Authors: Nicola Specchio; Elaine C. Wirrell; Ingrid E. Scheffer; Rima Nabbout; Kate Riney; Pauline Samia; Marilisa Guerreiro; Sam Gwer; Sameer M. Zuberi; Jo M. Wilmshurst; Elissa Yozawitz; Ronit Pressler; Edouard Hirsch; Samuel Wiebe; Helen J. Cross; Emilio Perucca; Solomon L. Moshé; Paolo Tinuper; Stéphane Auvin
• Keywords: Epilepsy syndromes; Generalized epilepsy; Semiology; Childhood absence epilepsy; Seizure types

Abstract The 2017 International League Against Epilepsy classification has defined a three‐tier system with epilepsy syndrome identification at the third level. Although cannot be determined in all children epilepsy, of specific provides guidance on management and prognosis. In this paper, we describe childhood onset syndromes, most which have both mandatory seizure type(s) interictal electroencephalographic (EEG) features. Based Classification Seizures Epilepsies, some names been updated using terms directly describing semiology. syndromes beginning divided into three categories: (1) self‐limited focal epilepsies, comprising four syndromes: centrotemporal spikes, autonomic seizures, occipital visual photosensitive lobe epilepsy; (2) generalized absence myoclonic absence, eyelid myoclonia; (3) developmental and/or epileptic encephalopathies, five myoclonic–atonic Lennox–Gastaut syndrome, encephalopathy spike‐and‐wave activation sleep, hemiconvulsion–hemiplegia–epilepsy febrile infection‐related syndrome. We define each, highlighting seizure(s), EEG features, phenotypic variations, findings from key investigations.