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, Rima Nabbout, Ingrid E. Scheffer, Taoufik Alsaadi, Alicia Bogacz, Jacqueline A. French, Edouard Hirsch, Satish Jain, Sunao Kaneko, Kate Riney, Pauline Samia, O. Carter Snead, Ernest Somerville, Nicola Specchio, Eugen Trinka, Sameer M. Zuberi, Simona Balestrini, Samuel Wiebe, J. Helen Cross, Emilio PeruccaSolomon L. Moshé, Paolo Tinuper

Research output: Contribution to journalArticlepeer-review

14 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)1333-1348
Number of pages16
JournalEpilepsia
Volume63
Issue number6
DOIs
StatePublished - Jun 2022

Keywords

  • developmental and epileptic encephalopathy
  • electroencephalogram
  • focal epilepsy
  • idiopathic generalized epilepsy
  • semiology

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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