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目的研究ｍｏｙａｍｏｙａ病的临床及病理特点。方法分析经脑血管造影（５５例）及剖检（１５例）证实的ｍｏｙａｍｏｙａ病的临床、ＣＴ及病理资料。结果７０例平均年龄２９．２５岁。出血组５５例，平均年龄３３．１３岁，以原发性脑室出血（２１例）、脑叶出血（１３例）及ＳＡＨ（９例）为多，出血原因为异网血管、动脉瘤破裂及梗塞性出血；缺血组１５例，平均年龄１８．４岁，８０％病灶多发，８６．７％为２次以上发病，８０％有两侧症状及体征。结论儿童及青壮年发生脑血管病，血压不高，反复出现脑梗塞或出现脑室出血、脑叶出血或ＳＡＨ等要考虑ｍｏｙａｍｏｙａ病的可能
Objective To study the clinical and pathological features of moyamoya disease. Methods The clinical, CT and pathological data of moyamoya disease confirmed by cerebral angiography (55 cases) and autopsy (15 cases) were analyzed. Results The average age of 70 cases was 29.25 years old. Bleeding group 55 cases, average age 33.13 years old, with primary ventricular hemorrhage (21 cases), lobar hemorrhage (13 cases) and SAH (9 cases) were more bleeding due to abnormal blood vessels, aneurysm rupture and 15 cases of ischemic group, the average age of 18.4 years old, 80% of multiple lesions, 86.7% for more than 2 times the incidence, 80% have bilateral symptoms and signs. Conclusions The incidence of cerebrovascular disease in children and young adults is not high, and recurrent cerebral infarction or ventricular hemorrhage, cerebral lobar hemorrhage or SAH may consider the possibility of moyamoya disease