CADASIL syndrome: A genetic form of vascular dementia

Mental disorders due to cerebral microvascular disease have been known for over 100 years. Recently, an autosomal dominant form of cerebral arteriopat hy (CADASIL) has been described in association with a Notch3 family gene on the short arm of chromosome 19. CADASIL causes subcortical lacunar infarct ion and dementia in over 80% of cases and depression in a large proportion of patients. Clinically, CADASIL may appear to be very similar to hypertens ive microvascular disease (Binswanger's disease), a condition that is seen in the elderly. This article reviews the clinical, pathologic, and genetic features of CADASIL. CADASIL is of interest to neurologists and psychiatris ts because it is the first syndrome of vascular dementia and depression wit h an identified gene. How the gene causes the widespread arteriopathy is no t yet known. Insights gained from the study of CADASIL should help us bette r understand its etiology, as well as the options for treatment of the more common forms of microvascular disease seen in the elderly.