ILAE definition of the Idiopathic Generalized Epilepsy Syndromes: Position statement by the ILAE Task Force on Nosology and Definitions

Edouard Hirsch¹, Jacqueline French², Ingrid E Scheffer³, Alicia Bogacz⁴, Taoufik Alsaadi⁵, Michael R Sperling⁶, Fatema Abdulla⁷, Sameer M Zuberi⁸, Eugen Trinka^{9

10}, Nicola Specchio¹¹, Ernest Somerville¹², Pauline Samia¹³, Kate Riney^{14

15}, Rima Nabbout¹⁶, Satish Jain¹⁷, Jo M Wilmshurst¹⁸, Stephane Auvin^{19

20}, Samuel Wiebe²¹, Emilio Perucca^{22

23}, Solomon L Moshé²⁴, Paolo Tinuper^{25

26}, Elaine C Wirrell²⁷

Affiliations

¹ Francis Rohmer Neurology Epilepsy Units, National Institute of Health and Medical Research 1258, Federation of Translational Medicine of Strasbourg, Strasbourg University, Strasbourg, France.
² New York University Grossman School of Medicine and NYU Langone Health, New York, New York, USA.
³ Austin Health and Royal Children's Hospital, Florey Institute, Murdoch Children's Research Institute, University of Melbourne, Melbourne, Victoria, Australia.
⁴ Institute of Neurology, Clinical Hospital, Faculty of Medicine, University of the Republic, Montevideo, Uruguay.
⁵ Department of Neurology, American Center for Psychiatry and Neurology, Abu Dhabi, United Arab Emirates.
⁶ Department of Neurology, Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, Pennsylvania, USA.
⁷ Salmaniya Medical Complex-Government Hospital, Manama, Bahrain.
⁸ Paediatric Neurosciences Research Group, Royal Hospital for Children and Institute of Health & Wellbeing, University of Glasgow, member of EpiCARE, Glasgow, UK.
⁹ Department of Neurology and Neuroscience Institute, Christian Doppler University Hospital, Paracelsus Medical University, Center for Cognitive Neuroscience, member of EpiCARE, Salzburg, Austria.
¹⁰ Department of Public Health, Health Services Research, and Health Technology Assessment, University for Health Sciences, Medical Informatics, and Technology, Hall in Tirol, Austria.
¹¹ Rare and Complex Epilepsy Unit, Department of Neuroscience, Bambino Gesù Children's Hospital, Scientific Institute for Research and Health Care, member of EpiCARE, Rome, Italy.
¹² Prince of Wales Hospital, University of New South Wales, Sydney, New South Wales, Australia.
¹³ Department of Pediatrics and Child Health, Aga Khan University, East Africa, Nairobi, Kenya.
¹⁴ Neurosciences Unit, Queensland Children's Hospital, South Brisbane, Queensland, Australia.
¹⁵ Faculty of Medicine, University of Queensland, Brisbane, Queensland, Australia.
¹⁶ Reference Center for Rare Epilepsies, Department of Pediatric Neurology, Necker-Enfants Malades 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.
¹⁷ Indian Epilepsy Center, New Delhi, India.
¹⁸ Department of Paediatric Neurology, Red Cross War Memorial Children's Hospital, Neuroscience Institute, University of Cape Town, Cape Town, South Africa.
¹⁹ Pediatric Neurology, Public Hospital Network of Paris, Robert Debré Hospital, NeuroDiderot, National Institute of Health and Medical Research, Department Medico-Universitaire, Innovation Robert-Debré, University of Paris, Paris, France.
²⁰ University Institute of France, Paris, France.
²¹ Department of Clinical Neurosciences, University of Calgary, Calgary, Alberta, Canada.
²² Department of Neuroscience, Monash University, Melbourne, Victoria, Australia.
²³ Department of Medicine, Austin Health, University of Melbourne, Heidelberg, Victoria, Australia.
²⁴ Isabelle Rapin Division of Child Neurology, Saul R. Korey Department of Neurology, and Departments of Neuroscience and Pediatrics, Albert Einstein College of Medicine and Montefiore Medical Center, Bronx, New York, USA.
²⁵ Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy.
²⁶ Institute of Neurological Sciences, Scientific Institute for Research and Health Care, member of EpiCARE, Bologna, Italy.
²⁷ Divisions of Child and Adolescent Neurology and Epilepsy, Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.

PMID: 35503716
DOI: 10.1111/epi.17236

Free article

ILAE definition of the Idiopathic Generalized Epilepsy Syndromes: Position statement by the ILAE Task Force on Nosology and Definitions

Edouard Hirsch et al. Epilepsia. 2022 Jun.

Free article

. 2022 Jun;63(6):1475-1499.

doi: 10.1111/epi.17236. Epub 2022 May 3.

Authors

Affiliations

¹ Francis Rohmer Neurology Epilepsy Units, National Institute of Health and Medical Research 1258, Federation of Translational Medicine of Strasbourg, Strasbourg University, Strasbourg, France.
² New York University Grossman School of Medicine and NYU Langone Health, New York, New York, USA.
³ Austin Health and Royal Children's Hospital, Florey Institute, Murdoch Children's Research Institute, University of Melbourne, Melbourne, Victoria, Australia.
⁴ Institute of Neurology, Clinical Hospital, Faculty of Medicine, University of the Republic, Montevideo, Uruguay.
⁵ Department of Neurology, American Center for Psychiatry and Neurology, Abu Dhabi, United Arab Emirates.
⁶ Department of Neurology, Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, Pennsylvania, USA.
⁷ Salmaniya Medical Complex-Government Hospital, Manama, Bahrain.
⁸ Paediatric Neurosciences Research Group, Royal Hospital for Children and Institute of Health & Wellbeing, University of Glasgow, member of EpiCARE, Glasgow, UK.
⁹ Department of Neurology and Neuroscience Institute, Christian Doppler University Hospital, Paracelsus Medical University, Center for Cognitive Neuroscience, member of EpiCARE, Salzburg, Austria.
¹⁰ Department of Public Health, Health Services Research, and Health Technology Assessment, University for Health Sciences, Medical Informatics, and Technology, Hall in Tirol, Austria.
¹¹ Rare and Complex Epilepsy Unit, Department of Neuroscience, Bambino Gesù Children's Hospital, Scientific Institute for Research and Health Care, member of EpiCARE, Rome, Italy.
¹² Prince of Wales Hospital, University of New South Wales, Sydney, New South Wales, Australia.
¹³ Department of Pediatrics and Child Health, Aga Khan University, East Africa, Nairobi, Kenya.
¹⁴ Neurosciences Unit, Queensland Children's Hospital, South Brisbane, Queensland, Australia.
¹⁵ Faculty of Medicine, University of Queensland, Brisbane, Queensland, Australia.
¹⁶ Reference Center for Rare Epilepsies, Department of Pediatric Neurology, Necker-Enfants Malades 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.
¹⁷ Indian Epilepsy Center, New Delhi, India.
¹⁸ Department of Paediatric Neurology, Red Cross War Memorial Children's Hospital, Neuroscience Institute, University of Cape Town, Cape Town, South Africa.
¹⁹ Pediatric Neurology, Public Hospital Network of Paris, Robert Debré Hospital, NeuroDiderot, National Institute of Health and Medical Research, Department Medico-Universitaire, Innovation Robert-Debré, University of Paris, Paris, France.
²⁰ University Institute of France, Paris, France.
²¹ Department of Clinical Neurosciences, University of Calgary, Calgary, Alberta, Canada.
²² Department of Neuroscience, Monash University, Melbourne, Victoria, Australia.
²³ Department of Medicine, Austin Health, University of Melbourne, Heidelberg, Victoria, Australia.
²⁴ Isabelle Rapin Division of Child Neurology, Saul R. Korey Department of Neurology, and Departments of Neuroscience and Pediatrics, Albert Einstein College of Medicine and Montefiore Medical Center, Bronx, New York, USA.
²⁵ Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy.
²⁶ Institute of Neurological Sciences, Scientific Institute for Research and Health Care, member of EpiCARE, Bologna, Italy.
²⁷ Divisions of Child and Adolescent Neurology and Epilepsy, Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.

PMID: 35503716
DOI: 10.1111/epi.17236

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.

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.

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References

REFERENCES

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ILAE definition of the Idiopathic Generalized Epilepsy Syndromes: Position statement by the ILAE Task Force on Nosology and Definitions

Affiliations

ILAE definition of the Idiopathic Generalized Epilepsy Syndromes: Position statement by the ILAE Task Force on Nosology and Definitions

Authors

Affiliations

Abstract

References

REFERENCES

MeSH terms

Substances

Supplementary concepts

LinkOut - more resources

Full Text Sources

Other Literature Sources