Fukawa O, Aihara H, Wakasa H
No Shinkei Geka. 1982 Dec;10(12):1303-10.
About the middle cerebral artery occlusion with moyamoya phenomenon, we have reported the clinical and angiographical study of 10 cases. Now we present an autopsy case. A 53-year-old female was admitted to our hospital on May, 22, 1978, two hours after an apparent subarachnoid hemorrhage. Neurological examination revealed drowsiness, nuchal rigidity right hemiparesis and motor aphasia. Left carotid angiogram showed an occlusion of the left middle cerebral artery at its origin with moyamoya phenomenon, but did not demonstrate an occlusion and/or stenosis of the intracranial internal carotid artery. Furthermore right carotid and bilateral vertebral angiograms were normal. The hospital course was progressively uphill, and she did not have any focal neurological deficits at discharge. But she committed suicide with a poison (Paraquat) on August, 19, 1978. Histopathologic examination of the left middle cerebral artery revealed a stenosis, with a maximum diameter of 900 microns. In this portion the lumen was not be occluded, and arteriosclerotic and inframatric exchanges were not present. The elastica interna was reduplicated, frayed and at times discontinuous. The tunica media was very thin or disappeared. On the other hand, moyamoya vessels were found between distal and peripheral portion of the stenotic middle cerebral artery as a collateral circulation. These findings suggested that the stenotic arterial wall of the left middle cerebral artery might be a congenital hypoplasia.
关于伴有烟雾病现象的大脑中动脉闭塞，我们已报告了10例的临床和血管造影研究。现在我们呈现1例尸检病例。一名53岁女性于1978年5月22日蛛网膜下腔明显出血两小时后入院。神经学检查发现嗜睡、颈项强直、右侧偏瘫和运动性失语。左颈动脉血管造影显示左大脑中动脉起始处闭塞并伴有烟雾病现象，但未显示颅内颈内动脉闭塞和/或狭窄。此外，右颈动脉和双侧椎动脉血管造影均正常。住院过程呈渐进性好转，出院时她没有任何局灶性神经功能缺损。但她于1978年8月19日用百草枯自杀。左大脑中动脉的组织病理学检查显示有一处狭窄，最大直径为900微米。在该部位管腔未闭塞，也不存在动脉硬化和基质内改变。内弹性膜重复、磨损，有时不连续。中膜非常薄或消失。另一方面，在狭窄的大脑中动脉远端和周边部分之间发现了烟雾病血管作为侧支循环。这些发现提示左大脑中动脉狭窄的动脉壁可能是先天性发育不全。
