Classification and Definition of Epilepsy Syndromes

The 2017 ILAE Classification of the Epilepsies defined three diagnostic levels including seizure type, epilepsy type and epilepsy syndrome. Epilepsy syndromes have been recognized as distinct electroclinical entities long before the first ILAE Classification of Epilepsies and Epilepsy Syndromes was proposed in 1985. However, a formally accepted ILAE classification of epilepsy syndromes was lacking, and the 2017-2021 Nosology and Definitions Task Force was charged with this endeavor.

The ILAE position papers are divided into (1) syndromes with onset in neonates and infants (up to age two years), (2) syndromes that onset in childhood, and (3) syndromes that may begin at a variable age (meaning in both pediatric and adult patients). Additionally, a fourth position paper on the idiopathic generalized epilepsies is included. For each syndrome, diagnostic electroclinical criteria, as well as expected results of other investigations (imaging, genetics), frequent co-morbidities, and natural history are provided.

A fifth paper outlines the methodology for this work with a list of syndromes. A translated list of syndromes is available in Arabic, Chinese, French, Greek, Italian, Japanese and Korean, with more languages coming soon.

ILAE anticipates that this work will facilitate clinical recognition of epilepsy syndromes, in order to hone high-yield diagnostic investigations, select optimal therapy, inform risk of comorbidities and provide more accurate prognosis regarding epilepsy outcome.

ILAE Classification and Definition of Epilepsy Syndromes

Introduction to the Epilepsy Syndrome Papers, Epilepsia (2022)
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ILAE Classification and Definition of Epilepsy Syndromes with Onset in Neonates and Infants: Position Statement by the ILAE Task Force on Nosology and Definitions, Epilepsia (2022)
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ILAE Classification and Definition of Epilepsy Syndromes with Onset in Childhood: Position Paper by the ILAE Task Force on Nosology and Definitions, Epilepsia (2022)
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ILAE Classification and Definition of Epilepsy Syndromes with Onset at a Variable Age: Position Statement by the ILAE Task Force on Nosology and Definitions, Epilepsia (2022)
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ILAE Definition of the Idiopathic Generalized Epilepsy Syndromes: Position Statement by the ILAE Task Force on Nosology and Definitions, Epilepsia (2022)
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Methodology for Classification and Definition of Epilepsy Syndromes with List of Syndromes: Report of the ILAE Task Force on Nosology and Definitions, Epilepsia (2022)
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