CADASIL: familiäre Schlaganfälle und subkortikale vaskuläre Demenz

N. Peters; M. Dichgans

doi:10.1055/s-0038-1626277

Nervenheilkunde, Table of Contents

Nervenheilkunde 2004; 23(02): 86-89
DOI: 10.1055/s-0038-1626277

Original- und Übersichtsarbeiten - Original and Review Articles

Schattauer GmbH

CADASIL: familiäre Schlaganfälle und subkortikale vaskuläre Demenz

CADASIL: a monogenic condition causing stroke and subcortical ischemic vascular dementia

N. Peters

¹Neurologische Klinik und Poliklinik, Klinikum Großhadern, Ludwig-Maximilians-Universität München

,

M. Dichgans

¹Neurologische Klinik und Poliklinik, Klinikum Großhadern, Ludwig-Maximilians-Universität München

› Author Affiliations

Abstract

Zusammenfassung

CADASIL (zerebrale autosomal-dominante Arteriopathie mit subkortikalen Infarkten und Leukenzephalopathie) ist eine autosomal-dominant vererbte Mikroangiopathie, die sich klinisch durch eine Migräne mit Aura, rezidivierende ischämische Episoden und eine vaskuläre Demenz manifestiert. Ursache der Erkrankung sind Mutationen im Notch3-Gen. Diese Arbeit gibt einen Überblick über die molekularen Grundlagen, das Krankheitsbild und den derzeitigen Stand der diagnostischen und therapeutischen Möglichkeiten.

Summary

CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukencephalopathy) is an autosomal dominantly inherited microangiopathy, which is clinically characterized by migraine with aura, recurrent ischemic episodes and progressive cognitive deficits leading to subcortical dementia. This article summarizes current data on the molecular pathogenesis, clinical manifestations and diagnostic tools as well as therapeutic strategies in this condition.

Schlüsselwörter

CADASIL - Notch3 - Migräne - Schlaganfall - vaskuläre Demenz

Keywords

CADASIL - Notch3 - migraine - stroke - vascular dementia

Full Text

References

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