Chen S T, Liu Y H, Hsu C Y, Hogan E L, Ryu S J
Department of Neurology, Chang Gung Memorial Hospital, Taipei, Taiwan.
Stroke. 1988 Jan;19(1):53-9. doi: 10.1161/01.str.19.1.53.
Moyamoya disease occurring in Chinese has been inadequately described. Here we report 13 cases of this disease identified by review of 3,200 cerebral angiograms performed between August 1979 and March 1986. Nine were males and four were females; there were 12 adults (aged 34-51 years) and one child (aged 11 years). All had hemorrhagic strokes with one exception, a patient with an occipital infarction. Intraventricular hemorrhage was noted by computed tomography in 10; five of these emanated from the caudate nucleus. A localized hematoma without intraventricular hemorrhage was found in two. All 13 angiograms had smokelike basal anastomoses with various degrees of stenosis or occlusion of the anterior portion of the circle of Willis; the involvement was bilateral in 12 and unilateral in one. Aneurysms were found in two patients, one in the anterior communicating artery and the other in the left anterior choroidal artery. Eleven patients recovered from the initial stroke, but two died with recurrent hemorrhage. This series differs from the series reported in Japan by the predominance of adult males. The high incidence of intraventricular hemorrhage and intracerebral hematoma is not in keeping with the previous Chinese series, in which subarachnoid hemorrhage was suspected to be the major clinical manifestation.
关于中国人群中发生的烟雾病，相关描述尚不充分。在此，我们报告通过回顾1979年8月至1986年3月期间进行的3200例脑血管造影而确诊的13例烟雾病病例。其中男性9例，女性4例；成人12例（年龄34 - 51岁），儿童1例（年龄11岁）。除1例枕叶梗死患者外，其余均为出血性卒中。计算机断层扫描显示10例存在脑室内出血，其中5例出血源自尾状核。2例发现有局限性血肿但无脑室内出血。13例脑血管造影均显示有烟雾状基底吻合， Willis环前部存在不同程度的狭窄或闭塞；12例为双侧受累，1例为单侧受累。2例患者发现有动脉瘤，1例位于前交通动脉，另1例位于左侧脉络膜前动脉。11例患者从初次卒中恢复，但2例因反复出血死亡。本系列与日本报告的系列不同之处在于成年男性占主导。脑室内出血和脑内血肿的高发生率与之前的中国系列报道不符，之前的报道怀疑蛛网膜下腔出血是主要临床表现。
