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

Edouard Hirsch, Jacqueline French, Ingrid E. Scheffer, Alicia Bogacz, Taoufik Alsaadi, Michael R. Sperling, Fatema Abdulla, Sameer M. Zuberi, Eugen Trinka, Nicola Specchio, Ernest Somerville, Pauline Samia, Kate Riney, Rima Nabbout, Satish Jain, Jo M. Wilmshurst, Stephane Auvin, Samuel Wiebe, Emilio Perucca, Solomon L MoshéPaolo Tinuper, Elaine C. Wirrell, Drs Birinus Adikaibe, Raidah Al Baradi, Danielle Andrade, Thomas Bast, Ahmed Beydoun, Christian Bien, Roberto Caraballo, Ana Carolina Coan, Mary Connolly, John Dunne, Sheryl Haut, Floor Jansen, Barbara Jobst, Reetta Kalviainen, Angela Kakooza, Mitsuhiro Kato, Kelly Knupp, Silvia Kochen, Lieven Lagae, Luis Carlos Mayor, Natela Okujava, Kurupath Radakishnan, Eliane Roulet-Perez, Loreto Rios, Lynette Sadleir, Daniel San Juan-Orta, Jose Serratosa, Renee Shellhaas, Meng Han Tsai, Vrajesh Udani, Helen Yue-Hua Zhang, Dong Zhou

Research output: Contribution to journalArticlepeer-review

2 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)1475-1499
Number of pages25
JournalEpilepsia
Volume63
Issue number6
DOIs
StatePublished - Jun 2022

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

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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