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[烟雾病患儿的临床表现及神经影像学特征]

[Clinical manifestations and neuroimaging characteristics of children with moyamoya disease].

作者信息

Li Jie, Liu Rui, Li Zhu-Yi, Wu Da-Fang, Ma Xi-Juan, Miao Jian-Ting

机构信息

Department of Neurology,Tangdu Hospital, Fourth Military Medical University, Xi'an 710038, China.

出版信息

Zhongguo Dang Dai Er Ke Za Zhi. 2010 Aug;12(8):637-40.

Abstract

OBJECTIVE

To study the clinical manifestations and neuroimaging characteristics of pediatric moyamoya disease.

METHODS

The clinical data of 17 children with moyamoya disease were retrospectively studied.

RESULTS

The onset age was between 3 and 14 years. The main clinical manifestations included motor weakness of extremities or hemiplegia, sensory disturbance and headache. Cranial CT or/and MRI examinations predominately showed cerebral infarct. Magnetic resonance angiography (MRA) and digital subtraction angiography (DSA) showed stenosis or occlusion at the terminus of the siphon portions of internal carotid arteries and proximal portions of anterior or middle cerebral arteries, and abnormal vascular networks at the base of brain.

CONCLUSIONS

Cerebral ischemia is main clinical manifestations in children with moyamoya disease, presenting motor weakness of extremities or hemiplegia, sensory disturbance and headache. DSA is essential to the diagnosis of the disease.

摘要

目的

研究儿童烟雾病的临床表现及神经影像学特征。

方法

回顾性分析17例儿童烟雾病的临床资料。

结果

发病年龄3~14岁。主要临床表现为肢体运动无力或偏瘫、感觉障碍及头痛。头颅CT或/和MRI检查主要表现为脑梗死。磁共振血管造影(MRA)及数字减影血管造影(DSA)显示颈内动脉虹吸段末端及大脑前、中动脉近端狭窄或闭塞,脑底部异常血管网形成。

结论

儿童烟雾病主要临床表现为脑缺血,表现为肢体运动无力或偏瘫、感觉障碍及头痛。DSA对该病的诊断至关重要。

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