Abstract
The goal of this paper is to provide updated diagnostic criteria for the epilepsy syndromes that have a variable age of onset, based on expert consensus of the International League Against Epilepsy Nosology and Definitions Taskforce (2017–2021). We use language consistent with current accepted epilepsy and seizure classifications and incorporate knowledge from advances in genetics, electroencephalography, and imaging. Our aim in delineating the epilepsy syndromes that present at a variable age is to aid diagnosis and to guide investigations for etiology and treatments for these patients.
Original language | English (US) |
---|---|
Pages (from-to) | 1443-1474 |
Number of pages | 32 |
Journal | Epilepsia |
Volume | 63 |
Issue number | 6 |
DOIs | |
State | Published - Jun 2022 |
Keywords
- Rasmussen syndrome
- epilepsy with reading-induced seizures
- focal epilepsy syndromes
- mesial temporal lobe epilepsy with hippocampal sclerosis
- progressive myoclonus epilepsies
ASJC Scopus subject areas
- Neurology
- Clinical Neurology
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In: Epilepsia, Vol. 63, No. 6, 06.2022, p. 1443-1474.
Research output: Contribution to journal › Article › peer-review
}
TY - JOUR
T1 - International League Against Epilepsy classification and definition of epilepsy syndromes with onset at a variable age
T2 - position statement by the ILAE Task Force on Nosology and Definitions
AU - Riney, Kate
AU - Bogacz, Alicia
AU - Somerville, Ernest
AU - Hirsch, Edouard
AU - Nabbout, Rima
AU - Scheffer, Ingrid E.
AU - Zuberi, Sameer M.
AU - Alsaadi, Taoufik
AU - Jain, Satish
AU - French, Jacqueline
AU - Specchio, Nicola
AU - Trinka, Eugen
AU - Wiebe, Samuel
AU - Auvin, Stéphane
AU - Cabral-Lim, Leonor
AU - Naidoo, Ansuya
AU - Perucca, Emilio
AU - Moshé, Solomon L.
AU - Wirrell, Elaine C.
AU - Tinuper, Paolo
N1 - Funding Information: We gratefully acknowledge the contributions to this final article from wider members of the ILAE Nosology and Definitions Task Force (2017–2021), the ILAE Review Task Force (2021–2022), experts from around the world who contributed to our Delphi methodology, and the wider epilepsy community, who provided comments on a draft of this article posted on the ILAE’s website (April–July 2021). We specifically thank: ILAE Nosology and Definitions Task Force (2017–2021): Elissa Yozawitz, Helen Cross, Jo Wilmshurst, O. Carter Snead, Ronit Pressler, Sunao Kaneko, Paula Samia. ILAE Review Task Force (2021–2022): Chahnez Triki, Fatema Abdulla, Marilissa Guerreiro, Michael Sperling, Sam Gwer, Santiago Galicchio. Delphi experts (pediatric): Ana Carolina Coan (Brazil), Angelina Kakooza (Uganda), Charles Newton (Kenya), Eliane Roulet-Perez (Switzerland), Floor Jansen (Netherlands), Helen Yue-Hua Zhang (China), Kelly Knupp (USA), Loreto Rios (Chile), Lieven Lagae (Belgium), Lynette Sadlier (New Zealand), Mary Connolly (Canada), Mitsuhiro Kato (Japan), Raidah Al Baradi (Saudi Arabia), Renee Shellhaas (USA), Roberto Carraballo (Argentina), Thomas Bast (Germany), Vrajesh Udani (India). Delphi experts (adult): Ahmed Beydoun (Egypt), Barbara Jobst (USA), Birinus Adikaibe (Nigeria), Christian Bien (Germany), Daniel San Juan-Orta (Mexico), Danielle Andrade (Canada), Dong Zhou (China), John Dunne (Australia), Jose Serratosa (Spain), Kurupath Radakrishnan (India), Luis Carlos Mayor (Colombia), Meng-Han Tsai (Taiwan), Natela Okujava (Georgia), Reetta Kaiviainen (Finland), Sammy Ohene (Ghana), Sheryl Haut (USA), Silvia Kochen (Argentina). Funding Information: K.R. has received honoraria for educational symposia, advisory boards, and/or consultancy work from Eisai, LivaNova, Novartis, and UCB Australia. Her institution has supported clinical trials for Biogen Idec Research, DSLP, Eisai, Epigenyx Therapeutics, GW Research, Janssen‐Cilag, Marinus Pharmaceuticals, Medicure International, LivaNova, Neurocrine Biosciences, Noema Pharma, Novartis, SK Lifesciences, UCB Australia, UCB Biopharma, and Zogenix. E.S. reports research support from Eisai, UCB, Zynerba, Marinus, SK Life Sciences, 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 speaker’s fees from Eisai and the Epilepsy Consortium and consulting fees from Eisai, UCB, and Seqirus. E.H. has received honoraria from UCB, Eisai, LivaNova, Novartis, and GW Pharmaceuticals. 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 has received unrestricted research grants from Eisai, UCB, LivaNova, and GW Pharma and academic research grants from EJP‐RD (Horizons 2020) and IDEAL‐EPISTOP. 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. S.M.Z. has received research support from Epilepsy Research UK, Tenovus Foundation, Glasgow Children's Hospital Charity, and Scottish Government Digital Health & Care. His institution has undertaken commercial trials for GW Pharma, Zogenix, Stoke Therapeutics, Encoded Therapeutics, and Marinus Pharmaceuticals. He has received honoraria for educational symposia, advisory boards, and consultancy work from GW Pharma, UCB Pharma, Eisai, Zogenix, Arvelle Therapeutics, GRIN Therapeutics, Jaguar Gene Therapeutics, and Encoded Therapeutics. T.A. has received consultation fees from Eli Lilly, Lundbeck, Merck, Hikma, Novartis, and Sanofi, and research support from Novartis and Biogen. 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 Sciences, 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, and 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. 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.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 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. 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 the Cumming School of Medicine. His institution has received unrestricted educational grants from UCB Pharma, Eisai, and Sunovion. S.A. has served as a consultant for 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 an Associate Editor for . A.N. has received speaker or writer honoraria from Sanofi, Genzyme Sanofi, Ann Lake Publications, and Novartis. She has received research funding from the University of KwaZulu Natal and the KM Browse Scholarship. The unit at Greys Hospital or its members have received educational or travel support from National Bioproducts Institute, Boehringer Ingelheim, Allergan, and Equity Pharmaceuticals. E.P. has received speaker and/or consultancy fees from Angelini, Arvelle, Biogen, Biopas, Eisai, GW Pharma, Sanofi group of companies, SK Life Science, Takeda, UCB Pharma, Xenon Pharma, and Zogenix. He has also received royalties from Wiley, Elsevier, and Wolters Kluwer. S.L.M. is the Charles Frost Chair in Neurosurgery and Neurology and acknowledges grant support from the National Institutes of Health (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 an Associate Editor of and from for his work as an Associate Editor; and royalties from two books he coedited. E.C.W. has served as a paid consultant for Encoded Therapeutics and BioMarin. She is the Editor‐in‐Chief of Epilepsy.com. P.T. has received speaker's or consultancy fees from Arvelle, Eisai, GW Pharma, LivaNova, UCB Pharma, Xenon Pharma, and Zogenix. 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 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 - The goal of this paper is to provide updated diagnostic criteria for the epilepsy syndromes that have a variable age of onset, based on expert consensus of the International League Against Epilepsy Nosology and Definitions Taskforce (2017–2021). We use language consistent with current accepted epilepsy and seizure classifications and incorporate knowledge from advances in genetics, electroencephalography, and imaging. Our aim in delineating the epilepsy syndromes that present at a variable age is to aid diagnosis and to guide investigations for etiology and treatments for these patients.
AB - The goal of this paper is to provide updated diagnostic criteria for the epilepsy syndromes that have a variable age of onset, based on expert consensus of the International League Against Epilepsy Nosology and Definitions Taskforce (2017–2021). We use language consistent with current accepted epilepsy and seizure classifications and incorporate knowledge from advances in genetics, electroencephalography, and imaging. Our aim in delineating the epilepsy syndromes that present at a variable age is to aid diagnosis and to guide investigations for etiology and treatments for these patients.
KW - Rasmussen syndrome
KW - epilepsy with reading-induced seizures
KW - focal epilepsy syndromes
KW - mesial temporal lobe epilepsy with hippocampal sclerosis
KW - progressive myoclonus epilepsies
UR - http://www.scopus.com/inward/record.url?scp=85131271048&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85131271048&partnerID=8YFLogxK
U2 - 10.1111/epi.17240
DO - 10.1111/epi.17240
M3 - Article
C2 - 35503725
AN - SCOPUS:85131271048
SN - 0013-9580
VL - 63
SP - 1443
EP - 1474
JO - Epilepsia
JF - Epilepsia
IS - 6
ER -