目的　探讨成人烟雾病的的临床、头颅CT和脑血管造影 (DSA)影像学特征。方法　对 21例病例的临床、头颅CT和DSA表现进行综合分析。结果　本组 80%的患者首发症状是以出血为主,主要集中在大于 40岁年龄组,头颅CT扫描示脑室系统出血、脑实质出血及蛛网膜下腔出血;30岁以下以缺血性表现为主,头部CT扫描多未见异常密度灶,仅少数可见低密度灶或软化灶。21例DSA表现为ICA末端狭窄或闭塞,脑底烟雾状血管网以及侧枝循环血管网形成。结论　DSA检查是确诊烟雾病的主要方法,临床上脑室出血、蛛网膜下腔出血以及无明确病因的脑实质出血或脑缺血发作均应常规行DSA检查。 Objective To investigate the clinical, cranial CT and cerebral angiography (DSA) features of adult moyamoya disease. Methods The clinical, CT and DSA manifestations of 21 cases were analyzed comprehensively. Results The first symptom of 80% of patients in this group was mainly hemorrhage, mainly in the group of over 40 years old. The head CT scan showed ventricular system hemorrhage, intraparenchymal hemorrhage and subarachnoid hemorrhage. Under the age of 30, Mainly, the head CT scan more than no abnormal density, only a few visible low-density lesions or softening stove. 21 cases of DSA showed ICA terminal stenosis or occlusion, cerebellum smoke vascular network and collateral circulation vascular network formation. Conclusions DSA is the main method for the diagnosis of moyamoya disease. DSA should be routinely performed in patients with clinical ventricular hemorrhage, subarachnoid hemorrhage and cerebral parenchymal hemorrhage or cerebral ischemic attack without clear etiology.