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[儿童纤维肌发育不良伴脑梗死。病例报告]

[Fibromuscular dysplasia with cerebral infarction in children. Case report].

作者信息

Takenaka K, Shimizu K, Ando T, Sakai N, Yamada H

出版信息

Neurol Med Chir (Tokyo). 1989 Oct;29(10):912-6. doi: 10.2176/nmc.29.912.

Abstract

A 15-year-old boy suddenly developed a headache, followed by progressive weakness of the right extremities. A computed tomographic scan obtained on admission showed a low-density area in the left putamen extending to the internal capsule. No neurological deficits were observed other than slight dysarthria and right hemiparesis. Left carotid angiography showed the characteristic "string of beads" deformity of fibromuscular dysplasia (FMD) extracranially and complete occlusion of the internal carotid artery at the C2 portion. The right carotid artery and both vertebral arteries were normal. Anastomosis of the left superficial temporal and middle cerebral arteries was performed 1 month after the onset. The patient recovered quickly from the hemiplegia and was discharged with no neurological deficits. Angiograms obtained 3 months later showed not only patency of the bypass, but also recanalization of the intracranial internal carotid artery. Cervico-cephalic FMD mainly affects middle-aged women, and is uncommon in children; only nine childhood cases have been reported. This disorder may play an important role in the genesis of strokes among children.

摘要

一名15岁男孩突然出现头痛,随后右侧肢体进行性无力。入院时的计算机断层扫描显示左侧壳核有一低密度区延伸至内囊。除轻微构音障碍和右侧偏瘫外,未观察到其他神经功能缺损。左侧颈动脉血管造影显示颅外纤维肌发育不良(FMD)的典型“串珠样”畸形,以及颈2段颈内动脉完全闭塞。右侧颈动脉和双侧椎动脉正常。发病1个月后进行了左侧颞浅动脉和大脑中动脉吻合术。患者偏瘫恢复迅速,出院时无神经功能缺损。3个月后获得的血管造影显示不仅搭桥血管通畅,而且颅内颈内动脉再通。颈脑型FMD主要影响中年女性,在儿童中并不常见;仅报道过9例儿童病例。这种疾病可能在儿童中风的发生中起重要作用。

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