Abstract
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary microangiopathy characterized by a genetic predisposition to small arteries of the brain. It is produced by a mutation in the NOTCH3 gene and concerns adults. The symptomatology is diversified including migraines with or without aura, subcortical ischemic events, and cognitive impairment. The diagnosis of CADASIL is suspected by neuroimaging and confirmed by genetic testing. Treatment of the disease remains preventive. We report a case of CADASIL manifesting as status migrainosus with persistent aura.