Methodology for classification and definition of epilepsy syndromes with list of syndromes: Report of the ILAE Task Force on Nosology and Definitions

doi:10.1111/epi.17237

. 2022 Jun;63(6):1333-1348.

doi: 10.1111/epi.17237. Epub 2022 May 3.

Methodology for classification and definition of epilepsy syndromes with list of syndromes: Report of the ILAE Task Force on Nosology and Definitions

Elaine C Wirrell¹, Rima Nabbout^{2

3}, Ingrid E Scheffer⁴, Taoufik Alsaadi⁵, Alicia Bogacz⁶, Jacqueline A French⁷, Edouard Hirsch⁸, Satish Jain⁹, Sunao Kaneko¹⁰, Kate Riney^{11

12}, Pauline Samia¹³, O Carter Snead¹⁴, Ernest Somerville¹⁵, Nicola Specchio¹⁶, Eugen Trinka^{17

18

19}, Sameer M Zuberi^{20

21}, Simona Balestrini^{22

23

24}, Samuel Wiebe²⁵, J Helen Cross^{26

27}, Emilio Perucca^{28

29}, Solomon L Moshé³⁰, Paolo Tinuper^{31

32}

Affiliations

¹ Divisions of Child and Adolescent Neurology and Epilepsy, Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
² Reference Center for Rare Epilepsies, Department of Pediatric Neurology, Necker-Sick Children Hospital, Public Hospital Network of Paris, member of EpiCARE, Paris, France.
³ Imagine Institute, National Institute of Health and Medical Research, Mixed Unit of Research 1163, University of Paris, Paris, France.
⁴ Austin Health and Royal Children's Hospital, Florey Institute, Murdoch Children's Research Institute, University of Melbourne, Melbourne, Victoria, Australia.
⁵ Department of Neurology, American Center for Psychiatry and Neurology, Abu Dhabi, United Arab Emirates.
⁶ Faculty of Medicine, Clinics Hospital, Institute of Neurology, University of the Republic, Montevideo, Uruguay.
⁷ New York University Grossman School of Medicine and NYU Langone Health, New York, New York, USA.
⁸ Francis Rohmer Neurology Epilepsy Unit, National Institute of Health and Medical Research 1258, Federation of Translational Medicine of Strasbourg, Strasbourg University, Strasbourg, France.
⁹ Indian Epilepsy Center, New Delhi, India.
¹⁰ North Tohoku Epilepsy Center, Minato Hospital, Hachinohe, Japan.
¹¹ Neurosciences Unit, Queensland Children's Hospital, South Brisbane, Queensland, Australia.
¹² Faculty of Medicine, University of Queensland, Brisbane, Queensland, Australia.
¹³ Department of Pediatrics and Child Health, Aga Khan University, Nairobi, Kenya.
¹⁴ Department Pediatrics [Neurology], Faculty of Medicine, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
¹⁵ Prince of Wales Hospital and University of New South Wales, Sydney, New South Wales, Australia.
¹⁶ 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.
¹⁷ Department of Neurology, Christian Doppler University Hospital, Paracelsus Medical University, Salzburg, Austria.
¹⁸ Center for Cognitive Neuroscience, member of EpiCARE, Salzburg, Austria.
¹⁹ Neuroscience Institute, Christian Doppler University Hospital, Paracelsus Medical University, Salzburg, Austria.
²⁰ Paediatric Neurosciences Research Group, Royal Hospital for Children and Institute of Health & Wellbeing, University of Glasgow, Glasgow, UK.
²¹ Collaborating Centre of European Reference Network EpiCARE, Glasgow, UK.
²² Neuroscience Department, Meyer Children's Hospital-University of Florence, Florence, Italy.
²³ Department of Clinical and Experimental Epilepsy, University College London Queen Square Institute of Neurology, London, UK.
²⁴ Chalfont Centre for Epilepsy, Buckinghamshire, UK.
²⁵ Department of Clinical Neurosciences, University of Calgary, Calgary, Alberta, Canada.
²⁶ 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, London, UK.
²⁷ Young Epilepsy Lingfield, Lingfield, UK.
²⁸ Department of Neurosciences, Monash University, Melbourne, Victoria, Australia.
²⁹ Department of Medicine, Austin Health, University of Melbourne, Melbourne, Victoria, Australia.
³⁰ 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.
³¹ Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy.
³² Institute of Neurological Sciences, Scientific Institute for Research and Health Care, Bologna, Italy.

PMID: 35503715
DOI: 10.1111/epi.17237

Free article

Methodology for classification and definition of epilepsy syndromes with list of syndromes: Report of the ILAE Task Force on Nosology and Definitions

Elaine C Wirrell et al. Epilepsia. 2022 Jun.

Free article

. 2022 Jun;63(6):1333-1348.

doi: 10.1111/epi.17237. Epub 2022 May 3.

Authors

Affiliations

¹ Divisions of Child and Adolescent Neurology and Epilepsy, Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
² Reference Center for Rare Epilepsies, Department of Pediatric Neurology, Necker-Sick Children Hospital, Public Hospital Network of Paris, member of EpiCARE, Paris, France.
³ Imagine Institute, National Institute of Health and Medical Research, Mixed Unit of Research 1163, University of Paris, Paris, France.
⁴ Austin Health and Royal Children's Hospital, Florey Institute, Murdoch Children's Research Institute, University of Melbourne, Melbourne, Victoria, Australia.
⁵ Department of Neurology, American Center for Psychiatry and Neurology, Abu Dhabi, United Arab Emirates.
⁶ Faculty of Medicine, Clinics Hospital, Institute of Neurology, University of the Republic, Montevideo, Uruguay.
⁷ New York University Grossman School of Medicine and NYU Langone Health, New York, New York, USA.
⁸ Francis Rohmer Neurology Epilepsy Unit, National Institute of Health and Medical Research 1258, Federation of Translational Medicine of Strasbourg, Strasbourg University, Strasbourg, France.
⁹ Indian Epilepsy Center, New Delhi, India.
¹⁰ North Tohoku Epilepsy Center, Minato Hospital, Hachinohe, Japan.
¹¹ Neurosciences Unit, Queensland Children's Hospital, South Brisbane, Queensland, Australia.
¹² Faculty of Medicine, University of Queensland, Brisbane, Queensland, Australia.
¹³ Department of Pediatrics and Child Health, Aga Khan University, Nairobi, Kenya.
¹⁴ Department Pediatrics [Neurology], Faculty of Medicine, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
¹⁵ Prince of Wales Hospital and University of New South Wales, Sydney, New South Wales, Australia.
¹⁶ 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.
¹⁷ Department of Neurology, Christian Doppler University Hospital, Paracelsus Medical University, Salzburg, Austria.
¹⁸ Center for Cognitive Neuroscience, member of EpiCARE, Salzburg, Austria.
¹⁹ Neuroscience Institute, Christian Doppler University Hospital, Paracelsus Medical University, Salzburg, Austria.
²⁰ Paediatric Neurosciences Research Group, Royal Hospital for Children and Institute of Health & Wellbeing, University of Glasgow, Glasgow, UK.
²¹ Collaborating Centre of European Reference Network EpiCARE, Glasgow, UK.
²² Neuroscience Department, Meyer Children's Hospital-University of Florence, Florence, Italy.
²³ Department of Clinical and Experimental Epilepsy, University College London Queen Square Institute of Neurology, London, UK.
²⁴ Chalfont Centre for Epilepsy, Buckinghamshire, UK.
²⁵ Department of Clinical Neurosciences, University of Calgary, Calgary, Alberta, Canada.
²⁶ 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, London, UK.
²⁷ Young Epilepsy Lingfield, Lingfield, UK.
²⁸ Department of Neurosciences, Monash University, Melbourne, Victoria, Australia.
²⁹ Department of Medicine, Austin Health, University of Melbourne, Melbourne, Victoria, Australia.
³⁰ 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.
³¹ Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy.
³² Institute of Neurological Sciences, Scientific Institute for Research and Health Care, Bologna, Italy.

PMID: 35503715
DOI: 10.1111/epi.17237

Abstract

Epilepsy syndromes have been recognized for >50 years, as distinct electroclinical phenotypes with therapeutic and prognostic implications. Nonetheless, no formally accepted International League Against Epilepsy (ILAE) classification of epilepsy syndromes has existed. The ILAE Task Force on Nosology and Definitions was established to reach consensus regarding which entities fulfilled criteria for an epilepsy syndrome and to provide definitions for each syndrome. We defined an epilepsy syndrome as "a characteristic cluster of clinical and electroencephalographic features, often supported by specific etiological findings (structural, genetic, metabolic, immune, and infectious)." The diagnosis of a syndrome in an individual with epilepsy frequently carries prognostic and treatment implications. Syndromes often have age-dependent presentations and a range of specific comorbidities. This paper describes the guiding principles and process for syndrome identification in both children and adults, and the template of clinical data included for each syndrome. We divided syndromes into typical age at onset, and further characterized them based on seizure and epilepsy types and association with developmental and/or epileptic encephalopathy or progressive neurological deterioration. Definitions for each specific syndrome are contained within the corresponding position papers.

Keywords: developmental and epileptic encephalopathy; electroencephalogram; focal epilepsy; idiopathic generalized epilepsy; semiology.

PubMed Disclaimer

Cited by

Pathogenicity Prediction of GABA_A Receptor Missense Variants.
Wang YJ, Vu GH, Mu TW. Wang YJ, et al. bioRxiv [Preprint]. 2023 Nov 16:2023.11.14.567135. doi: 10.1101/2023.11.14.567135. bioRxiv. 2023. PMID: 38014242 Free PMC article. Preprint.
[A multicentre epilepsy registry in Mexico].
Reséndiz-Aparicio JC, Ruiz-García M, Castro-Martínez E. Reséndiz-Aparicio JC, et al. Rev Neurol. 2024 Jan 1;78(1):9-15. doi: 10.33588/rn.7801.2023296. Rev Neurol. 2024. PMID: 38112652 Free PMC article. Spanish.
Off-label use of cannabidiol in genetic epileptic and developmental encephalopathies: A case report.
Mannini E, Misirocchi F, Lazzari S, Balella G, Bottignole D, Frapporti M, Zinno L, Florindo I, Parrino L, Mutti C. Mannini E, et al. Epilepsy Behav Rep. 2024 Jun 20;27:100687. doi: 10.1016/j.ebr.2024.100687. eCollection 2024. Epilepsy Behav Rep. 2024. PMID: 39040437 Free PMC article.
Duration of epileptic seizure types: A data-driven approach.
Meritam Larsen P, Wüstenhagen S, Terney D, Gardella E, Aurlien H, Beniczky S. Meritam Larsen P, et al. Epilepsia. 2023 Feb;64(2):469-478. doi: 10.1111/epi.17492. Epub 2023 Jan 3. Epilepsia. 2023. PMID: 36597206 Free PMC article.
Vagus nerve stimulation for treating developmental and epileptic encephalopathy in young children.
Geng G, Hu W, Meng Y, Zhang H, Zhang H, Chen C, Zhang Y, Gao Z, Liu Y, Shi J. Geng G, et al. Front Neurol. 2023 Oct 20;14:1191831. doi: 10.3389/fneur.2023.1191831. eCollection 2023. Front Neurol. 2023. PMID: 37928141 Free PMC article.

See all "Cited by" articles

References

REFERENCES

1. West WJ. On a peculiar form of infantile convulsions. Lancet. 1841;35:724-5.
1. Gibbs FA, Gibbs EL. Epilepsy. Atlas of electroencephalography. Cambridge, MA: Addison-Wesley; 1952.
1. Lennox WG, Davis JP. Clinical correlates of the fast and the slow spike-wave electroencephalogram. Pediatrics. 1950;5:626-44.
1. Gastaut H, Roger J, Soulayrol R, Saint-Jean M, Tassinari CA, Regis H, et al. Epileptic encephalopathy of children with diffuse slow spikes and waves (alias "petit mal variant") or Lennox syndrome. Ann Pediatr. 1966;13:489-99.
1. Tissot DM. Traité de l'épilepsie. Faisant le tonne troisième du traité des nerf & de leur maladies. Paris, France: Lausanne et P.F. Didot, le jeune; 1770.

MeSH terms

Actions
Actions
Actions
Actions
Actions
Actions
Actions

LinkOut - more resources

Full Text Sources
Other Literature Sources
- The Lens - Patent Citations Database
Medical
- MedlinePlus Consumer Health Information
- MedlinePlus Health Information

[1] West WJ. On a peculiar form of infantile convulsions. Lancet. 1841;35:724-5.

[2] West WJ. On a peculiar form of infantile convulsions. Lancet. 1841;35:724-5.

[3] Gibbs FA, Gibbs EL. Epilepsy. Atlas of electroencephalography. Cambridge, MA: Addison-Wesley; 1952.

[4] Gibbs FA, Gibbs EL. Epilepsy. Atlas of electroencephalography. Cambridge, MA: Addison-Wesley; 1952.

[5] Lennox WG, Davis JP. Clinical correlates of the fast and the slow spike-wave electroencephalogram. Pediatrics. 1950;5:626-44.

[6] Lennox WG, Davis JP. Clinical correlates of the fast and the slow spike-wave electroencephalogram. Pediatrics. 1950;5:626-44.

[7] Gastaut H, Roger J, Soulayrol R, Saint-Jean M, Tassinari CA, Regis H, et al. Epileptic encephalopathy of children with diffuse slow spikes and waves (alias "petit mal variant") or Lennox syndrome. Ann Pediatr. 1966;13:489-99.

[8] Gastaut H, Roger J, Soulayrol R, Saint-Jean M, Tassinari CA, Regis H, et al. Epileptic encephalopathy of children with diffuse slow spikes and waves (alias "petit mal variant") or Lennox syndrome. Ann Pediatr. 1966;13:489-99.

[9] Tissot DM. Traité de l'épilepsie. Faisant le tonne troisième du traité des nerf & de leur maladies. Paris, France: Lausanne et P.F. Didot, le jeune; 1770.

[10] Tissot DM. Traité de l'épilepsie. Faisant le tonne troisième du traité des nerf & de leur maladies. Paris, France: Lausanne et P.F. Didot, le jeune; 1770.

Save citation to file

Email citation

Add to Collections

Add to My Bibliography

Your saved search

Create a file for external citation management software

Your RSS Feed

Methodology for classification and definition of epilepsy syndromes with list of syndromes: Report of the ILAE Task Force on Nosology and Definitions

Affiliations

Methodology for classification and definition of epilepsy syndromes with list of syndromes: Report of the ILAE Task Force on Nosology and Definitions

Authors

Affiliations

Abstract

Similar articles

Cited by

References

REFERENCES

MeSH terms

LinkOut - more resources

Full Text Sources

Other Literature Sources

Medical

Abstract

Similar articles

Cited by

References

REFERENCES

MeSH terms

Related information

LinkOut - more resources

Full Text Sources

Other Literature Sources

Medical