Kortikale Dysplasien als Ursache fokaler Epilepsien

Zusammenfassung

Fokale kortikale Dysplasien werden zunehmend als Ursache fokaler Epilepsien erkannt. Sie verursachen oft schon im frühen Kindesalter sich manifestierende, schwere und meist medikamentös therapierefraktäre Epilepsien, die im Kindesalter nicht selten zu einer Beeinträchtigung der kognitiven Entwicklung führen können. Die Weiterentwicklung bildgebender Verfahren ermöglicht es in zunehmendem Maße, fokale kortikale Dysplasien bei bislang als „kryptogen” eingestuften Epilepsien nachzuweisen und hierdurch den Weg zu einer Erfolg versprechenden epilepsiechirurgischen Therapie zu eröffnen. Während weniger als ein Viertel der Patienten eine Anfallskontrolle durch Antiepileptika erzielt, wird an spezialisierten Zentren heute operativ eine vollständige Anfallskontrolle bei ca. 60 % der Patienten erreicht. In der vorliegenden Übersicht werden histologische, klinische, EEG- und MR-Charakteristika fokaler kortikaler Dysplasien und die derzeitigen therapeutischen Optionen dargestellt.

Abstract

Focal cortical dysplasias are an increasingly recognized etiology of focal epilepsy. Onset often is during early childhood, seizures may be clinically severe and often pharmacoresistant and often lead to impairment of psychomotor development. Progress in imaging techniques allows to increasingly identify focal cortical dysplasias in formerly „cryptogenic” epilepsies which opens the way for successful surgical therapy. Whereas seizure control is achieved in less than 25 % of patients by current antiepileptic drugs, epilepsy surgery in specialized centers leads to seizure freedom in up to 60 % of patients. In this review, histological, clinical, EEG- and MR characteristics of focal cortical dysplasias as well as therapeutic options are reported.

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