Abstract
In 2017, the International League Against Epilepsy (ILAE) Classification of Epilepsies described the “genetic generalized epilepsies” (GGEs), which contained the “idiopathic generalized epilepsies” (IGEs). The goal of this paper is to delineate the four syndromes comprising the IGEs, namely childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy, and epilepsy with generalized tonic–clonic seizures alone. We provide updated diagnostic criteria for these IGE syndromes determined by the expert consensus opinion of the ILAE’s Task Force on Nosology and Definitions (2017–2021) and international external experts outside our Task Force. We incorporate current knowledge from recent advances in genetic, imaging, and electroencephalographic studies, together with current terminology and classification of seizures and epilepsies. Patients that do not fulfill criteria for one of these syndromes, but that have one, or a combination, of the following generalized seizure types: absence, myoclonic, tonic-clonic and myoclonic-tonic-clonic seizures, with 2.5–5.5 Hz generalized spike-wave should be classified as having GGE. Recognizing these four IGE syndromes as a special grouping among the GGEs is helpful, as they carry prognostic and therapeutic implications.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 1475-1499 |
| Number of pages | 25 |
| Journal | Epilepsia |
| Volume | 63 |
| Issue number | 6 |
| DOIs | |
| State | Published - Jun 2022 |
Keywords
- absence seizures
- childhood absence epilepsy
- generalized tonic–clonic seizures
- generalized tonic–clonic seizures alone
- genetic generalized epilepsy
- juvenile absence epilepsy
- juvenile myoclonic epilepsy
- myoclonic seizures
ASJC Scopus subject areas
- Neurology
- Clinical Neurology
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In: Epilepsia, Vol. 63, No. 6, 06.2022, p. 1475-1499.
Research output: Contribution to journal › Article › peer-review
}
TY - JOUR
T1 - ILAE definition of the Idiopathic Generalized Epilepsy Syndromes
T2 - Position statement by the ILAE Task Force on Nosology and Definitions
AU - Hirsch, Edouard
AU - French, Jacqueline
AU - Scheffer, Ingrid E.
AU - Bogacz, Alicia
AU - Alsaadi, Taoufik
AU - Sperling, Michael R.
AU - Abdulla, Fatema
AU - Zuberi, Sameer M.
AU - Trinka, Eugen
AU - Specchio, Nicola
AU - Somerville, Ernest
AU - Samia, Pauline
AU - Riney, Kate
AU - Nabbout, Rima
AU - Jain, Satish
AU - Wilmshurst, Jo M.
AU - Auvin, Stephane
AU - Wiebe, Samuel
AU - Perucca, Emilio
AU - Moshé, Solomon L
AU - Tinuper, Paolo
AU - Wirrell, Elaine C.
AU - Adikaibe, Drs Birinus
AU - Al Baradi, Raidah
AU - Andrade, Danielle
AU - Bast, Thomas
AU - Beydoun, Ahmed
AU - Bien, Christian
AU - Caraballo, Roberto
AU - Carolina Coan, Ana
AU - Connolly, Mary
AU - Dunne, John
AU - Haut, Sheryl
AU - Jansen, Floor
AU - Jobst, Barbara
AU - Kalviainen, Reetta
AU - Kakooza, Angela
AU - Kato, Mitsuhiro
AU - Knupp, Kelly
AU - Kochen, Silvia
AU - Lagae, Lieven
AU - Carlos Mayor, Luis
AU - Okujava, Natela
AU - Radakishnan, Kurupath
AU - Roulet-Perez, Eliane
AU - Rios, Loreto
AU - Sadleir, Lynette
AU - Juan-Orta, Daniel San
AU - Serratosa, Jose
AU - Shellhaas, Renee
AU - Tsai, Meng Han
AU - Udani, Vrajesh
AU - Yue-Hua Zhang, Helen
AU - Zhou, Dong
N1 - Funding Information: E.H. has received honoraria from UCB, Eisai, LivaNova, Novartis, and GW Pharmaceuticals. J.F. receives NYU salary support from the Epilepsy Foundation and for consulting work and/or attending scientific advisory boards on behalf of the Epilepsy Study Consortium for Adamas, Aeonian/Aeovian, Anavex, Arkin Holdings, Arvelle Therapeutics, Athenen Therapeutics/Carnot Pharma, Baergic Bio, Biogen, BioXcel Therapeutics, Cavion, Cerebral Therapeutics, Cerevel, Crossject, CuroNZ, Eisai, Eliem Therapeutics, Encoded Therapeutics, Engage Therapeutics, Engrail, Epiminder, Equilibre BioPharmaceuticals, Fortress Biotech, Greenwich Biosciences, GW Pharma, Janssen Pharmaceutica, Knopp Biosciences, Lundbeck, Marinus, Mend Neuroscience, Merck, NeuCyte, Neurocrine, Otsuka Pharmaceutical Development, Ovid Therapeutics, Passage Bio, Praxis, Redpin, Sage, SK Life Science, Sofinnova, Stoke, Supernus, Synergia Medical, Takeda, UCB, West Therapeutic Development, Xenon, Xeris, Zogenix, and Zynerba. J.F. has also received research support from the Epilepsy Research Foundation, Epilepsy Study Consortium (funded by Andrews Foundation, Eisai, Engage, Lundbeck, Pfizer, SK Life Science, Sunovion, UCB, Vogelstein Foundation), Epilepsy Study Consortium/Epilepsy Foundation (funded by UCB, Engage, Neurelis, SK Life Science), GW/One8 Foundation/FACES, and NINDS. She is on the editorial board of and . She is Chief Medical/Innovation Officer for the Epilepsy Foundation, for which NYU receives salary support. She has received travel reimbursement related to research, advisory meetings, or presentation of results at scientific meetings from the Epilepsy Study Consortium, the Epilepsy Foundation, Arvelle Therapeutics, Biogen, Cerevel, Engage, Lundbeck, NeuCyte, Otsuka, Sage, UCB, Xenon, and Zogenix. I.E.S. has served on scientific advisory boards for UCB, Eisai, GlaxoSmithKline, BioMarin, Nutricia, Rogcon, Chiesi, Encoded Therapeutics and Xenon Pharmaceuticals; has received speaker honoraria from GlaxoSmithKline, UCB, BioMarin, Biocodex, and Eisai; has received funding for travel from UCB, Biocodex, GlaxoSmithKline, Biomarin, and Eisai; has served as an investigator for Zogenix, Zynerba, Ultragenyx, GW Pharma, UCB, Eisai, Anavex Life Sciences, Ovid Therapeutics, Epigenyx, Encoded Therapeutics, and Marinus; and has consulted for Zynerba Pharmaceuticals, Atheneum Partners, Ovid Therapeutics, Care Beyond Diagnosis, Epilepsy Consortium, and UCB. M.R.S. has received compensation for speaking at CME programs from Medscape, Projects for Knowledge, International Medical Press, Eisai, and UCB Pharma. He is an advisor for scientific publications for Neurelis. He has consulted for Medtronic and Johnson & Johnson. He has received research support from Eisai, Medtronic, Neurelis, SK Life Science, Takeda, Xenon, Cerevel, UCB Pharma, Janssen, and Engage Pharmaceuticals. He has received royalties from Oxford University Press and Cambridge University Press. S.M.Z. has received research support from Epilepsy Research UK, Tenovus Foundation, Glasgow Children's Hospital Charity, and the Scottish government's Technology Enabled Care. He has received honoraria for educational symposia, advisory boards, and consultancy work from GW Pharma, Zogenix, Arvelle Therapeutics, and Encoded Therapeutics. E.T. reports personal fees from EVER Pharma, Marinus, Argenix, Arvelle, Angelini, Medtronic, Bial–Portela & Cª, NewBridge, GL Pharma, GlaxoSmithKline, Hikma, Boehringer Ingelheim, LivaNova, Eisai, UCB, Biogen, Genzyme Sanofi, GW Pharmaceuticals, and Actavis; his institution has received grants from Biogen, UCB Pharma, Eisai, Red Bull, Merck, Bayer, the European Union, FWF Österreichischer Fond zur Wissenschaftsforderung, Bundesministerium für Wissenschaft und Forschung, and Jubiläumsfond der Österreichischen Nationalbank outside the submitted work. N.S. has served on scientific advisory boards for GW Pharma, BioMarin, Arvelle, Marinus, and Takeda; has received speaker honoraria from Eisai, Biomarin, LivaNova, and Sanofi; and has served as an investigator for Zogenix, Marinus, Biomarin, UCB, and Roche. E.S. reports research support from Eisai, UCB, Zynerba, Marinus, SK Life Science, Upsher Smith, Cerevel, National Health and Medical Research Council of Australia, and Australian Research Council. He has received support for educational activities from Sanofi, UCB, and ILAE. He reports speakers fees from Eisai and the Epilepsy Consortium and consulting fees from Eisai, UCB, and Seqirus. K.R. has received speaker honoraria, advisory board payments, and/or research funding from UCB, Eisai, Novartis, Zogenix, SK Lifesciences, AFT Pharmaceuticals, LivaNova, Queensland Genomic Health Alliance, Department of Health (Australia), Medicure International, Novartis, and Janssen‐Cilag. R.N. has served as principal investigator in clinical trials for Novartis, Nutricia, Eisai, UCB, GW Pharma, and LivaNova. She has received consulting fees from Biogene, BioMarin, GW Pharma, Zogenix, Novartis, Nutricia, Stoke, Ionis, Targeon, and Takeda and honoraria from Nutricia, Biocodex, Zogenix, GW Pharma, Advicennes, and Eisai. She received unrestricted research grants from Eisai, UCB, LivaNova, and GW Pharma and academic research grants from EJP‐RD (Horizons 2020) and IDEAL‐EPISTOP. T.A. has received consultation fees from Eli Lilly, Lundbeck, Merck, Hikma, Novartis, and Sanofi, and research support from Novartis and Biogen. J.M.W. has received an honorarium for activities as Associate Editor for . S.A. has served as a consultant or received honoraria for lectures from Biocodex, Biomarin, Eisai, GW Pharma, Neuraxpharm, Nutricia, UCB Pharma, Xenon, and Zogenix. He has been an investigator for clinical trials for Eisai, UCB Pharma, and Zogenix. He is Associate Editor for . S.W. has received research support from the Canadian Institutes of Health Research and Alberta Innovates Health Solutions. He chairs the Clinical Research Unit at the University of Calgary, which receives support from Cumming School of Medicine. His institution has received unrestricted educational grants from UCB Pharma, Eisai, and Sunovion. E.P. received speaker and/or consultancy fees from Angelini, Arvelle, Biogen, Biopas, Eisai, GW Pharma, the Sanofi group of companies, SK Life Science, Takeda, UCB Pharma, Xenon Pharma, and Zogenix and royalties from Wiley, Elsevier, and Wolters Kluwers. S.L.M. is the Charles Frost Chair in Neurosurgery and Neurology and acknowledges grant support from the NIH (U54 NS100064 and NS43209), US Department of Defense (W81XWH‐18–1–0612), Heffer Family and Segal Family Foundations, and Abbe Goldstein/Joshua Lurie and Laurie Marsh/Dan Levitz families. S.L.M. is serving as Associate Editor of . He is on the editorial board of , , , , and . He receives compensation from Elsevier for his work as Associate Editor of and from for his work as Associate Editor; and royalties from two books he coedited. P.T. has received speaker's or consultancy fees from Arvelle, Eisai, GW Pharma, LivaNova, UCB Pharma, Xenon Pharma, and Zogenix. E.C.W. has served as a paid consultant for Encoded Therapeutics and Biomarin. She is the Editor‐in‐Chief of Epilepsy.com. None of the other authors has any conflict of interest to disclose. We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this report is consistent with those guidelines. Lancet Neurology Neurology Today Epilepsia Epilepsia Neurobiology of Disease Brain and Development Pediatric Neurology Annals of Neurology MedLink Physiological Research Neurobiology of Disease MedLink 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 - In 2017, the International League Against Epilepsy (ILAE) Classification of Epilepsies described the “genetic generalized epilepsies” (GGEs), which contained the “idiopathic generalized epilepsies” (IGEs). The goal of this paper is to delineate the four syndromes comprising the IGEs, namely childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy, and epilepsy with generalized tonic–clonic seizures alone. We provide updated diagnostic criteria for these IGE syndromes determined by the expert consensus opinion of the ILAE’s Task Force on Nosology and Definitions (2017–2021) and international external experts outside our Task Force. We incorporate current knowledge from recent advances in genetic, imaging, and electroencephalographic studies, together with current terminology and classification of seizures and epilepsies. Patients that do not fulfill criteria for one of these syndromes, but that have one, or a combination, of the following generalized seizure types: absence, myoclonic, tonic-clonic and myoclonic-tonic-clonic seizures, with 2.5–5.5 Hz generalized spike-wave should be classified as having GGE. Recognizing these four IGE syndromes as a special grouping among the GGEs is helpful, as they carry prognostic and therapeutic implications.
AB - In 2017, the International League Against Epilepsy (ILAE) Classification of Epilepsies described the “genetic generalized epilepsies” (GGEs), which contained the “idiopathic generalized epilepsies” (IGEs). The goal of this paper is to delineate the four syndromes comprising the IGEs, namely childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy, and epilepsy with generalized tonic–clonic seizures alone. We provide updated diagnostic criteria for these IGE syndromes determined by the expert consensus opinion of the ILAE’s Task Force on Nosology and Definitions (2017–2021) and international external experts outside our Task Force. We incorporate current knowledge from recent advances in genetic, imaging, and electroencephalographic studies, together with current terminology and classification of seizures and epilepsies. Patients that do not fulfill criteria for one of these syndromes, but that have one, or a combination, of the following generalized seizure types: absence, myoclonic, tonic-clonic and myoclonic-tonic-clonic seizures, with 2.5–5.5 Hz generalized spike-wave should be classified as having GGE. Recognizing these four IGE syndromes as a special grouping among the GGEs is helpful, as they carry prognostic and therapeutic implications.
KW - absence seizures
KW - childhood absence epilepsy
KW - generalized tonic–clonic seizures
KW - generalized tonic–clonic seizures alone
KW - genetic generalized epilepsy
KW - juvenile absence epilepsy
KW - juvenile myoclonic epilepsy
KW - myoclonic seizures
UR - http://www.scopus.com/inward/record.url?scp=85129250406&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85129250406&partnerID=8YFLogxK
U2 - 10.1111/epi.17236
DO - 10.1111/epi.17236
M3 - Article
C2 - 35503716
AN - SCOPUS:85129250406
SN - 0013-9580
VL - 63
SP - 1475
EP - 1499
JO - Epilepsia
JF - Epilepsia
IS - 6
ER -