Clinical presentation of CADASIL: data from the Spanish study. Diagnostic criteria proposed

CADASIL is an autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy leading to dementia. Histological studies are characte ristic and genetical studies have demonstrated mutations on Notch 3 gene (1 9p.13.1). No large series are still available and therefore, we collected t he data of CADASIL in Spain to found the clinical spectrum of the disase. Data were obtained from 4 Centers and our results are consistent with other reports, being brain infarct (94%), dementia (72%), cerebral transiet isch emic attacks (61%), and migraine (44%) the most frequent clinical manifesta tions. All cases had an autosomal dominant inheritance and the mean age of onset was in the fourth decade. This study allow us to propose clinical criteria for CADASIL (possible, pro bable, definite).