【摘要】目的　探讨与Moyamoya病相关的眼部临床表现、发生机制及其临床意义。方法　回顾性分析37例有眼部症状的Moyamoya病患者的临床资料。结果　37例有眼部症状的Moyamoya病患者，单纯以眼部症状首发者8例，眼部症状与其它神经系统症状同时出现的29例；患者眼部症状主要表现为：一过性黑朦、偏盲、视力下降甚至丧失、视野缺损、瞳孔直接/间接对光反射消失、瞳孔大小异常、上睑下垂、眼球运动障碍、眼球疼痛。结论　Moyamoya病眼部症状多样，神经科医生和眼科医生均应掌握Moyamoya病的临床表现及其特点，对于有眼部症状的患者应及时做全面的检查，以免漏诊和误诊。

文献标识码：B

文章编号：1002-4379（2015）01-0026-02

DOI：10.13444/j.cnki.zgzyykzz.2015.01.007

作者单位：1湖北民族学院附属民大医院，恩施445000 2四川大学华西医院

通讯作者：杨华,

E-mail:yanghua8111@163.com

Clinical analysis of ocular presentations in moyamoya disease

ZHANG Chaogui, WAN Jincheng, QU

Changhua, et al. University Hospital of Hubei University for Nationalites, Enshi 445000, China

【Abstract】OBJECTIVE To explore the clinical manifestations and mechanism of ocular symptoms related to moyamoya disease. METHODS The clinical information was analyzed retrospectively in 37 moyamoya disease. RESULTS There were 8 patients examined to simply perform ocular manifestations, while 29 patients had ocular manifestations accompanied by other neurologic symptoms. These ocular signs included amaurosis fugax, hemianopia, decline even loss of the visual ability, visual field defect, pupil direct/indirect light reflex, pupil size abnormal, ptosis, ocular dyskinesia and eye pain. CONCLUSIONS The symptoms of moyamoya disease are multiple, which required neurologic physician and ophthalmologist be proficient in the onset characteristics and clinical manifestation. The patients must be diagnosed and treated as soon as possible to avoid misdiagnosis and missed diagnosis, especially for patients with ocular signs.

【Key words】moyamoya disease; ocular manifestations; clinical analysis

Moyamoya病（moyamoya disease，MMD），又称为脑底异常血管网病、烟雾病，是颈内动脉虹吸部、大脑前动脉、大脑中动脉起始部严重狭窄，小动脉代偿性增生、扩张，脑底部异常血管网形成为特征的脑血管疾病。Moyamoya病病因及发病机制尚未明确，目前认为该病可能与遗传因素，感染、炎症及免疫因素，细胞因子分泌异常、弹性蛋白堆积等因素有关 〔1〕。虽然目前对Moyamoya病的临床特点报道比较多，但是对Moyamoya病相关眼部症状的分析甚少，现回顾性分析37例有眼部症状Moyamoya病患者的临床资料，以期对其眼部症状发生的机制及其临床特点有一个比较全面的了解。

1　临床资料

1.1一般资料

2006年1月至2012年12月湖北民族学院附属民大医院及四川大学华西医院收住的37例有眼部症状的Moyamya病患者的临床资料。……