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
