烟雾病的临床分析

Clinical analysis of moyamoya disease

目的分析moyamoya病的临床及影像学特征以提高对该病的诊断水平。方法选择2005年9月-2011年10月中国医科大学附属盛京医院收治的17例moyamoya病患者的资料进行回顾性分析,总结其临床及影像学特点,结合文献进行归纳。结果 17例moyamoya病患者,男8例,女9例;儿童组21个月至15岁,8例;成人组34-53岁,9例。数字减影血管造影(digital subtraction angiography,DSA)检查4例,磁共振成像(magnetic resonance imaging,MRI)+磁共振血管造影(magnetic resonance angiography,MRA)检查10例,CT血管成像(computerized tomography angiography,CTA)检查2例,DSA+MRA检查1例。3例单侧血管典型改变,8例双侧颈内动脉虹吸段狭窄或闭塞,10例双侧大脑前动脉起始部狭窄或闭塞,12例双侧大脑中动脉起始部狭窄或闭塞,2例双侧大脑后动脉起始部狭窄或闭塞,17例颅底大量纤细紊乱血管形成。结论Moyamoya病发病率低。发病年龄分布呈两组高峰。儿童患者大多表现为脑缺血。成人患者一部分表现为颅内出血,一部分则表现为脑缺血。典型的MRA提示双侧颈内动脉虹吸段和/或大脑前动脉及大脑中动脉起始段狭窄或闭塞,侧支血管形成脑底异常血管网时可确诊。对于缺乏常见危险因素的中青年患者或多发性脑梗塞的儿童患者,应及时行头MRI+MRA或DSA检查,除外moyamoya病。

【Objective】 To conclude and analyze the clinical and imaging characteristics of moyamoya disease,so as to improve the diagnosis level【Methods】We choose 17 patients of moyamoya disease in Shengjing hospital from September 2005 to Octorber 2011.The clinical data were analyzed retrospectively,including clinical manifestation,imaging features as well as associated literature.【Resluts】 17 patients with moyamoya disease are divided into 2 groups：children group（age ranged from 21 months to 15 years old） and adults group（age ranged from 34 to 54 years old）.4 patients were diagnosed by DSA;10 patients were diagnosed by MRI＋MRA;2 patients were diagnosed by CTA;1 patient was diagnosed by DSA＋MRA.The imaging features including：3 patients with typical unilateral involved;8 patients with bilateral stenosis or occlusion at the terminal portion of internal carotid artery;10 patients with bilateral stenosis or occlusion at the proximal portion of the anterior cerebral artery;12 patients with bilateral stenosis or occlusion at the proximal portion of middle cerebral artery;2 patients with bilateral stenosis or occlusion at the proximal portion of posterior cerebral artery;17 patients with abnormal vascular network in the basilar region.【Conclusion】Moyamoya disease is one of main cause of stroke in children and young adults.Most children patients presented as acute cerebral infarction or transient ischemic attack,while half patients in adults group attacked as acute cerebral hemorrhage.The typical MRA shows stenosis or occlusion at the terminal portion of internal carotid artery,and/or stenosis or occlusion at the proximal portion of the anterior cerebral artery and the middle cerebral artery.When CT/MRI shows more serious lesions,the moyamoya disease should be excluded.