Klassifikation von Epilepsiesyndromen

Im Jahr 2022 wurden von der Nosology and Definitions Task Force der Internationalen Liga gegen Epilepsie (ILAE) in einer Serie von Positionspapieren erstmals von der ILAE anerkannte Definitionen von Epilepsiesyndromen vorgestellt, die in der vorliegenden Übersichtsarbeit kurz zusammengefasst werden sollen [1–6].

Abstract

Epilepsy syndromes are defined as a characteristic cluster of clinical and EEG features, often supported by specific etiologic findings (structural, genetic, metabolic, immune or infectious). Epilepsy syndromes are grouped according to the age at epilepsy onset in epilepsy syndromes with neonatal and infantile onset, epilepsy syndromes with childhood onset and epilepsy syndromes with variable age at onset. According to seizure types in each age group syndromes are further subdivided into generalized, focal or combined focal and generalized. Developmental and epileptic encephalopathies (DEE) and syndromes with progressive neurological deterioration represent an additional separate category in each age group. Idiopathic generalized epilepsies are combined in a separate group irrespective of age at onset. For each syndrome the following information is summarized: epidemiology, clinical context, natural history, seizure types, EEG, neuroimaging and genetic findings, other relevant laboratory studies and differential diagnosis. For each syndrome mandatory and exclusionary criteria as well as alerts are presented. The term syndrome in evolution should be used for syndromes in which some mandatory signs are still missing early in the epilepsy course, but appear with further disease progression. Etiology-specific syndromes are defined as syndromes in which a specific etiology is associated with a clearly defined, relatively uniform and distinct clinical phenotype as well as with consistent findings on EEG, neuroimaging and genetics in most affected individuals.

Schlüsselwörter

Anfallsformen - EEG - Epilepsiebeginn - Ätiologie

Key word

seizure type - EEG - epilepsy onset - etiology

muscle cramps - spasm - electromyography

Publication History

Article published online:
07 September 2023

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