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[双胞胎中的烟雾病]

[Moyamoya disease in twins].

作者信息

Kawamura S, Hadeishi H, Suzuki A, Yasui N

出版信息

No To Shinkei. 1987 Feb;39(2):119-25.

PMID:3828146
Abstract

The authors described twins of ten-year-old girls who developed Moyamoya disease at the same time. The family history showed no abnormality. They were born in 1973 under the cesarean section for the early rupture of the membranes and weak pains. The first case experienced tonic convulsions, and abnormal findings on the EEG were pointed out on May, 1979. Seven months later, CT scan was performed in another hospital, which showed the findings of brain atrophy. She experienced absence attacks on January, 1980, and had the first admission. She was diagnosed as epilepsy. Right carotid angiography (CAG) was performed, which showed no abnormal findings. On August, 1983, she complained transient ischemic attacks (TIA) of right hemiweakness and sometimes loss of consciousness for 5 to 10 minutes. She had the second admission on September, 1984. Growing status and neuro-psychological findings were normal. The EEG seemed almost normal, but she complained a TIA on right upper extremity two minutes after the hyperventilation, so the examination was stopped. CT findings was the same as that performed in 1979. Bilateral CAG showed marked stenosis of the internal carotid arteries (ICA) at the siphon and basal moyamoya vessels. Vertebral angiography had no sterno -occlusive lesions. Left encephalo-duro-arterio-synangiosis (EDAS) was performed, and six weeks later, right EDAS. Postoperatively, TIAs disappeared and she had a good clinical course. The second case had a similar history as described in case 1, except the absence of epileptic attacks. EEG abnormality was pointed out, and she also had the diagnosis of epilepsy.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

作者描述了一对同时患上烟雾病的10岁双胞胎女孩。家族史无异常。她们于1973年因胎膜早破和宫缩乏力行剖宫产出生。首例患儿于1979年5月出现强直性惊厥,脑电图检查发现异常。7个月后,在另一家医院进行了CT扫描,显示脑萎缩。她于1980年1月出现失神发作,并首次入院。被诊断为癫痫。右侧颈动脉血管造影(CAG)未发现异常。1983年8月,她出现右侧半身无力的短暂性脑缺血发作(TIA),有时意识丧失5至10分钟。1984年9月她第二次入院。生长发育状况及神经心理学检查结果正常。脑电图看似基本正常,但过度换气两分钟后她诉说右上肢体出现TIA,因此检查终止。CT检查结果与1979年相同。双侧CAG显示虹吸部颈内动脉(ICA)明显狭窄及基底烟雾状血管。椎动脉血管造影未发现闭塞性病变。先行左侧脑硬膜动脉血管吻合术(EDAS),六周后行右侧EDAS。术后,TIA消失,临床过程良好。第二例患儿病史与病例1相似,但无癫痫发作。脑电图检查发现异常,她也被诊断为癫痫。(摘要截选至250字)

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