[CADASIL and CARASIL].

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL) is clinically characterized by migraines with aura, recurrent ischemic strokes, cognitive and behaviour impairments and dementia and shows typical histological lesions in the muscular arteries. The disease is linked to mutations in NOTCH3, a gene located in chromosome 19. On the other hand, cerebral autosomal recessive arteriopathy with subcortical infarcts and leucoencephalopathy (CARASIL) is a poorly understood disease mainly described in the Japanese literature. This is also a hereditary vascular disease but the affected gene still is not identified. The disease clinically associates recurrent ischemic strokes with bone lesions and alopecia. None of these conditions are related with hypertension. This paper reviews the genetic, clinical, and pathological aspects of both diseases.