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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\u2010limited focal epilepsies, comprising four syndromes: self\u2010limited epilepsy with centrotemporal spikes, self\u2010limited 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\u2013atonic seizures, Lennox\u2013Gastaut syndrome, developmental and\/or epileptic encephalopathy with spike\u2010and\u2010wave activation in sleep, hemiconvulsion\u2013hemiplegia\u2013epilepsy syndrome, and febrile infection\u2010related epilepsy syndrome. We define each, highlighting the mandatory seizure(s), EEG features, phenotypic variations, and findings from key investigations.<\/jats:p>","DOI":"10.1111\/epi.17241","type":"journal-article","created":{"date-parts":[[2022,5,3]],"date-time":"2022-05-03T12:50:06Z","timestamp":1651582206000},"page":"1398-1442","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":983,"title":["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"],"prefix":"10.1111","volume":"63","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8120-0287","authenticated-orcid":false,"given":"Nicola","family":"Specchio","sequence":"first","affiliation":[{"name":"Rare and Complex Epilepsy Unit Department of Neuroscience Bambino Ges\u00f9 Children\u2019s Hospital, Scientific Institute for Research and Health Care Full Member of EpiCARE Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3015-8282","authenticated-orcid":false,"given":"Elaine C.","family":"Wirrell","sequence":"additional","affiliation":[{"name":"Divisions of Child and Adolescent Neurology and Epilepsy Department of Neurology Mayo Clinic Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2311-2174","authenticated-orcid":false,"given":"Ingrid E.","family":"Scheffer","sequence":"additional","affiliation":[{"name":"Austin Health and Royal Children\u2019s Hospital Florey Institute Murdoch Children\u2019s Research Institute University of Melbourne Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5877-4074","authenticated-orcid":false,"given":"Rima","family":"Nabbout","sequence":"additional","affiliation":[{"name":"Reference Center for Rare Epilepsies Department of Pediatric Neurology Necker\u2013Sick Children Hospital, Public Hospital Network of Paris member of EpiCARE Imagine Institute, National Institute of Health and Medical Research, Mixed Unit of Research 1163 University of Paris Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1122-3555","authenticated-orcid":false,"given":"Kate","family":"Riney","sequence":"additional","affiliation":[{"name":"Neurosciences Unit Queensland Children's Hospital South Brisbane Queensland Australia"},{"name":"Faculty of Medicine University of Queensland South Brisbane Queensland Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7427-0439","authenticated-orcid":false,"given":"Pauline","family":"Samia","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Child Health Aga Khan University Nairobi Kenya"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marilisa","family":"Guerreiro","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Campinas Campinas Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sam","family":"Gwer","sequence":"additional","affiliation":[{"name":"School of Medicine Kenyatta University, and Afya Research Africa Nairobi Kenya"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sameer M.","family":"Zuberi","sequence":"additional","affiliation":[{"name":"Paediatric Neurosciences Research Group Royal Hospital for Children and Institute of Health &amp; Wellbeing member of EpiCARE University of Glasgow Glasgow UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7328-1796","authenticated-orcid":false,"given":"Jo M.","family":"Wilmshurst","sequence":"additional","affiliation":[{"name":"Department of Paediatric Neurology Red Cross War Memorial Children\u2019s Hospital Neuroscience Institute University of Cape Town Cape Town South Africa"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8230-8364","authenticated-orcid":false,"given":"Elissa","family":"Yozawitz","sequence":"additional","affiliation":[{"name":"Isabelle Rapin Division of Child Neurology of the Saul R. Korey Department of Neurology Montefiore Medical Center Bronx New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2905-6839","authenticated-orcid":false,"given":"Ronit","family":"Pressler","sequence":"additional","affiliation":[{"name":"Programme of Developmental Neurosciences University College London National Institute for Health Research Biomedical Research Centre Great Ormond Street Institute of Child Health Department of Clinical Neurophysiology Great Ormond Street Hospital for Children London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0833-8850","authenticated-orcid":false,"given":"Edouard","family":"Hirsch","sequence":"additional","affiliation":[{"name":"Neurology Epilepsy Units \u201cFrancis Rohmer\u201d INSERM 1258, FMTS, Strasbourg University Strasbourg France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1061-9099","authenticated-orcid":false,"given":"Samuel","family":"Wiebe","sequence":"additional","affiliation":[{"name":"Department of Clinical Neurosciences University of Calgary Calgary Alberta Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7345-4829","authenticated-orcid":false,"given":"Helen J.","family":"Cross","sequence":"additional","affiliation":[{"name":"Programme of Developmental Neurosciences University College London National Institute for Health Research Biomedical Research Centre Great Ormond Street Institute of Child Health Great Ormond Street Hospital for Children and Young Epilepsy Lingfield London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8703-223X","authenticated-orcid":false,"given":"Emilio","family":"Perucca","sequence":"additional","affiliation":[{"name":"Department of Neuroscience Monash University Melbourne Victoria Australia"},{"name":"Department of Medicine Austin Health University of Melbourne Heidelberg Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9427-9476","authenticated-orcid":false,"given":"Solomon L.","family":"Mosh\u00e9","sequence":"additional","affiliation":[{"name":"Isabelle Rapin Division of Child Neurology Saul R. 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