伴皮层下梗死和白质脑病的常染色体显性遗传性脑动脉病(cerebral autosomal dominant arteriopathywith subcortical infarcts and leukoencephalopathy,CA-DASIL)是由第19号染色体上NOTCH3基因突变导致的遗传性脑动脉病[1,2]。该病主要以伴有先兆的偏头痛、反复缺血性卒中、进行性或阶梯性进展的皮质下痴呆及运动和精神障碍等为主要临床表现[1,3]。现总结我科诊治的2个CADASIL家系患者的临床资 Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CA-DASIL) with subcortical infarction and leukoencephalopathy is a genetic disorder of the cerebral artery caused by a mutation in the NOTCH3 gene on chromosome 19 [1 ,2]. The disease is predominantly characterized by migraine headaches, recurrent ischemic stroke, progressive or progressive subcortical dementia, and motor and psychiatric disorders [1,3]. Now summarize the clinical data of 2 CADASIL pedigrees treated in our department