儿童烟雾病临床及影像学特点

Clinical and imagic characteristics of moyamoya disease in children

目的总结儿童烟雾病的临床及影像学特点。方法对17例烟雾病患儿的临床特点与辅助检查结果进行分析。结果发病年龄1岁3个月～12岁,其中4～10岁占82.4%。15例临床表现为肢体无力或偏瘫,其中12例以此为首发症状;感觉障碍6例;语言障碍6例;视物不清3例;头痛2例;癫疒间发作2例;不自主运动2例;吞咽困难1例;智力倒退1例。5例行头颅CT检查者均显示有缺血性改变。头颅MRI检查12例,11例显示缺血改变,包括梗死灶或软化灶,1例未见异常。17例头颅磁共振血管成像(MRA)检查,16例显示颈内动脉虹吸部末端和大脑前或中动脉狭窄或闭塞,脑基底部有异常血管网形成;1例未见脑基底部异常血管网。9例数字减影动脉造影(DSA)检查者中,8例与MRA检查结果一致;1例MRA未发现脑底血管网,DSA检查证实存在血管网异常。结论儿童烟雾病高发年龄为4～10岁,多表现为短暂性脑缺血发作,临床以肢体无力或偏瘫、感觉障碍、语言障碍为常见症状。MRA与DSA检查是诊断烟雾病的主要方法,两者结果相似,提示头颅MRA可作为儿童烟雾病的首选辅助检查。

Objectives To summarize the clinical and imagic characteristics of moyamoya disease in children. Methods The clinical characteristics and the results of laboratory examinations were reviewed in 17 children with moyamoya disease. Results The onset age ranged from 1 year and 3 months to 12 years （of which 4 yrs - 10 yrs accounted for 82.4%） . The clinical manifestations were different, including paresis or hemiplegia （15） , sensory disturbance （6） , dysphasia （6） , blurred vision （3） , headache （2） , epileptic seizures （2） , involuntary movement （2） , dysphagia （ 1 ） , and psychomotor going backwards （ 1 ）. Cranial CT scan showed ischemia in 5 cases. Cranial magnetic resonance imaging （MRI） scan showed ischemia in 11 of 12 cases, including infarction and malactic lesions. There was 1 case showed no abnormality on MRI. In 16 cases, magnetic resonance angiography （MRA） showed stenosis or occlusion at terminus of the siphon portions of internal carotid arteries or at proximal ends of anterior or middle cerebral arteries, as well as formation of abnormal vascular network at basal part. In one case, no basal vascular network showed on MRA, and it was confirmed by digital subtraction angiography （DSA） . In the 8 cases who performed DSA, the characteristics of 7 cases were consistent with MRA. Conclusions The peak onset age of children with moyamoya disease ranged from 4 yrs to 10 yrs. This disease manifested differently. Most patients presented as transient ischemic attack. Paresis or hemiplegia, sensory disturbance and dysphasia were the common symptoms. Cranial MRA and DSA are the dominating methods for diagnosis of moyamoya disease. MRA can be the first choice for detecting moyamoya disease.