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

2022-06

Wirrell, Elaine C

Nabbout, Rima

Scheffer, Ingrid E

Alsaadi, Taoufik

Bogacz, Alicia

French, Jacqueline A

Hirsch, Edouard

Jain, Satish

Kaneko, Sunao

Riney, Kate

Samia, Pauline

Snead, O Carter

Somerville, Ernest

Specchio, Nicola

Trinka, Eugen

Zuberi, Sameer M

Balestrini, Simona

Wiebe, Samuel

Cross, J Helen

Perucca, Emilio

Moshé, Solomon L

Tinuper, Paolo

developmental and epileptic encephalopathy

electroencephalogram

focal epilepsy

idiopathic generalized epilepsy

semiology

Journal Article

https://orcid.org/0000-0003-3015-8282

https://orcid.org/0000-0001-5877-4074

https://orcid.org/0000-0002-2311-2174

https://orcid.org/0000-0002-7513-5706

https://orcid.org/0000-0003-2242-8027

https://orcid.org/0000-0003-0833-8850

https://orcid.org/0000-0002-1122-3555

https://orcid.org/0000-0002-7427-0439

https://orcid.org/0000-0001-8789-1122

https://orcid.org/0000-0002-8120-0287

https://orcid.org/0000-0002-5950-2692

https://orcid.org/0000-0001-5639-1969

https://orcid.org/0000-0002-1061-9099

https://orcid.org/0000-0001-7345-4829

https://orcid.org/0000-0001-8703-223X

https://orcid.org/0000-0001-9427-9476

10.1111/epi.17237

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.

link

Epilepsia 2022; 63(6): 1333-1348

Epilepsia