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. Methods: 65 mutation carriers and 30 matched control subjects underwent a series of assessments including global cognitive scores (Mattis Dementia Rating Scale, MDRS; Mini-Mental State Examination, MMSE) the VaDAS-cog battery as well as specific tests for executive function and attention with measures…