The pattern of cognitive performance in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)

Background and purpose: small vessel disease (SVD) is a major cause of vascular dementia. CADASIL is a monogenic variant of SVD caused by mutations in NOTCH3. Mutation carriers almost invariably develop cognitive deficits. The current study determines the characteristics of cognitive abnormalities seen in CADASIL.