Matsuki Y, Kawakami M, Ishizuka T, Kawaguchi Y, Hidaka T, Suzuki K, Nakamura H
National Defense Medical College, Saitama, Japan.
Scand J Rheumatol. 1997;26(5):392-4. doi: 10.3109/03009749709065707.
Moyamoya disease is a rare clinical entity, diagnosed by cerebral angiography and characterized by occlusion of the internal carotid artery system and the development of collateral arteries. A 30-year-old woman with systemic lupus erythematosus and Sjögren's syndrome recurrently presented transient right homonymous hemianopsia. Cerebral angiography showed occlusion of the left posterior cerebral artery associated with the development of collateral circulation ("moyamoya vessels"). In a young adult, as in this case, the unilaterality of the lesion and the presentation of transient ischemic attacks rather than subarachnoid hemorrhage are rare features for Moyamoya disease. Antiphospholipid syndrome was absent.
烟雾病是一种罕见的临床病症，通过脑血管造影诊断，其特征为颈内动脉系统闭塞并伴有侧支动脉形成。一名患有系统性红斑狼疮和干燥综合征的30岁女性反复出现短暂性右侧同向性偏盲。脑血管造影显示左侧大脑后动脉闭塞并伴有侧支循环（“烟雾血管”）形成。在本例这样的年轻成年人中，病变的单侧性以及短暂性脑缺血发作而非蛛网膜下腔出血的表现是烟雾病的罕见特征。该患者不存在抗磷脂综合征。
