伴有皮层下梗死和白质脑病的常染色体显性遗传的脑动脉病(CADASIL)临床分析

Clinical Analysis of Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy

目的分析CADASIL的临床特点,以便早期诊治。方法对3例确诊为CADASIL的患者进行回顾性研究。结果 CADASIL表现为皮质下缺血事件,并导致进行性痴呆伴假性球麻痹、严重情感障碍,而最早的临床表现可为有先兆的偏头痛。结论当患者有TIA或卒中、严重情感障碍、伴先兆的偏头痛、痴呆等表现,MRI或CT显示皮质下白质、基底节广泛异常改变时,要考虑CADASIL的诊断。

Objective To analyze the clinical characteristics of CADASIL for early diagnosis and treatment. Methods 3 cases of diagnosed with CADASIL patients were retrospectively studied. Results CADASIL manifestations of subcortical ischemic events, and causse of dementia patients with pseudobulbar palsy, severe mood disorders, and the early clinical manifestations can be for migraine with aura. Conclusion When patients with TIA or stroke, severe mood disorders, aura of migraine, dementia, MRI or CT showed subeortical white matter and basal ganglia widely abnormal changes, it is necessary to consider the CADASIL diagnosis.