一名患有凝血因子V莱顿突变的成年人出现脑室内出血的烟雾综合征。
Moyamoya syndrome with intraventricular hemorrhage in an adult with factor V Leiden mutation.
作者信息
Jabbour Rosette, Taher Ali, Shamseddine Ali, Atweh Samir F
机构信息
Department of Internal Medicine, American University of Beirut Medical Center, Beirut, Lebanon.
出版信息
Arch Neurol. 2005 Jul;62(7):1144-6. doi: 10.1001/archneur.62.7.1144.
OBJECTIVE
To report a case of proximal occlusion of 2 major cerebral vessels associated with moyamoya network circulation that manifested by spontaneous intraventricular hemorrhage.
DESIGN
Case report.
PATIENT AND RESULTS
A 36-year-old Syrian man presented with symptoms of sudden-onset headache, neck stiffness, and confusion. The computed tomography scan of his brain showed intraventricular bleeding, and the subsequent 4 vessel angiographies revealed occlusion of the left middle and anterior cerebral arteries with moyamoya appearance in the terminal branches. The coagulation profile showed the presence of heterozygous factor V Leiden mutation. The patient was treated conservatively until resolution of his blood clot, and later he was started on oral anticoagulation.
CONCLUSION
Factor V Leiden mutation may cause large cerebral vessel occlusion with moyamoya syndrome in adults.
目的
报告一例与烟雾状血管网循环相关的2条主要脑血管近端闭塞病例，该病例以自发性脑室内出血为表现。
设计
病例报告。
患者及结果
一名36岁的叙利亚男性出现突发头痛、颈部僵硬和意识模糊症状。其脑部计算机断层扫描显示脑室内出血，随后的4次血管造影显示左大脑中动脉和前动脉闭塞，终末分支呈烟雾状外观。凝血检查显示存在杂合子因子V莱顿突变。患者接受保守治疗直至血凝块溶解，之后开始口服抗凝治疗。
结论
因子V莱顿突变可能导致成人烟雾病综合征伴大脑大血管闭塞。
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