ABSTRACT
BACKGROUND: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited arteriopathy caused by mutation in the Notch 3 gene. Cognitive impairment, which is the second most frequent clinical manifestation, worsens with recurrent stroke. Comparison studies between the pre- and poststroke phases in CADASIL have not yet been performed in Asia. Here we describe the clinical characteristics of both the preand poststroke phases and identified the risk factors for stroke in CADASIL. METHODS: Fifty-three consecutive patients were investigated. Patients were divided into two groups depending on the presence (poststroke group; n=31) or absence (prestroke group; n=22) of clinically overt stroke. All patients underwent an MRI scan with the same protocol. Cognition was assessed by applying detailed neuropsychological tests. RESULTS: The poststroke group exhibited an increase in lacunae and cerebral microbleeds. The memory scores on the Alzheimer's Dementia Assessment Scale cognitive subscale and the number of correct Stroop color-naming scores were lower in the poststroke group than in the prestroke group. Hypertension was more prevalent in the poststroke group, while chronic headache was more prevalent in the prestroke group. CONCLUSIONS: These findings suggest that cerebral microbleeds as well as lacunae predict the risk of clinically overt stroke, which leads to a worsening of frontal-lobe function in CADASIL.