Einzelne Epilepsiesyndrome

Zusammenfassung

Die Internationale Liga gegen Epilepsie (ILAE) hat in einer Serie von Positionspapieren erstmals von der ILAE anerkannte Definitionen von Epilepsiesyndromen zusammenfassend dargestellt. Entsprechend dem Alter bei Erkrankungsbeginn werden Epilepsiesyndrome eingeteilt in Epilepsiesyndrome mit Erkrankungsbeginn im Neugeborenen- und Kleinkindalter (≤2 Jahre), Epilepsiesyndrome mit Erkrankungsbeginn in der Kindheit (2–12 Jahre) sowie Epilepsiesyndrome mit einem variablen Alter zu Erkrankungsbeginn (≤18 Jahre und≥19 Jahre). In jeder Altersgruppe erfolgt gemäß den Anfallsformen eine weitere Unterteilung in generalisierte, fokale sowie kombiniert generalisierte und fokale Syndrome. Entwicklungsbedingte und epileptische Enzephalopathien (developmental and epileptic encephalopathies – DEE) sowie Syndrome mit progressiver neurologischer Verschlechterung werden als separate Kategorien in allen Altersgruppen abgehandelt. Ätiologie-spezifische Epilepsiesyndrome sind Syndrome, bei denen eine spezifische Ätiologie bei den meisten betroffenen Individuen mit einem klar definierten, relativ uniformen und umschriebenem klinischen Phänotyp sowie mit konsistenten Befunden im EEG, in der Bildgebung und/oder in der Genetik assoziiert ist und werden ebenso in allen Altersgruppen vorgestellt. Die idiopathischen generalisierten Epilepsien werden unabhängig vom Alter bei Erkrankungsbeginn als eigene Kategorie zusammengefasst.

Abstract

The International League against Epilepsy (ILAE) presented in a series of position papers for the first time definitions of epilepsy syndromes acknowledged by the ILAE. Epilepsy syndromes are grouped according to the age at epilepsy onset in epilepsy syndromes with neonatal and infantile onset (≤2 years), epilepsy syndromes with childhood onset (2–12 years) and epilepsy syndromes with variable age at onset (≤18 years and ≥ 19 years). According to seizure types in each age group syndromes are further subdivided into generalized, focal or combined focal and generalized syndromes. Developmental and epileptic encephalopathies (DEE) and syndromes with progressive neurological deterioration represent additional separate categories in each age group. 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/or genetics in most affected individuals and are presented in each age group. Idiopathic generalized epilepsies are summarized in a separate group irrespective of age at onset.

Schlüsselwörter

Alter bei Epilepsiebeginn - Anfallsform - EEG - Bildgebung - Ätiologie

Key words

age at epilepsy onset - seizure type - EEG - neuroimaging - etiology

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