Moyamoya疾病：三病例報告

Moyamoya Disease: Report of Three Cases

吳克恭(Keh-Gong Wu) ； 遲景上(Ching-Shiang Chi) ； 張開屏(Kai-Ping Chang) ； 黃棣棟(Tai-Tong Wong) ； 湯仁彬(Ren-Bin Tang) ； 黃碧桃(Betau Hwang)

《Acta Paediatrica Sinica》 29卷1期 (1988/02) Pp. 64-71

摘要

Moyamoya疾病爲一種罕見的進行性腦血管阻塞性疾病，併同毛細血管擴張。常發生在年輕女孩。日本人病例報告比較多。本文報告3例經內頸動腦血管攝影證實的病例，一位男童、兩位女童，年齡分別爲4、5、6歲。三病例均以突發性、反覆性和間歇性的右半身偏癱伴隨不等程度的運動性失語症爲主要臨床症狀。其中男孩一例經小兒神經外科手術做兩側顳淺動脈和中大腦動脈分枝的血管繞道手術及腦肌瓣血管吻合術後，情況穩定，繼續在門診追蹤檢查。

並列摘要

Moyamoya disease is a rare type of cerebrovascular occlusive disorder in childhood. It is characterized by progressive stenosis or occlusion of the supraclinoid portion of the internal carotid arteries, associated with basal telangiectasias that give the condition its name. The so-called ”Moyamoya” is a Japanese term for a hazy appearance just like a puff of cigarette smoke drifting in the air. This disease appears bilaterally n most cases, and occurs predominantly in Japanese children, with a higher incidnce in girls. This paper presents three cases of Chinese children, one boy and two girls with the age of 4, 5 and 6 years respectively. Sudden onset of repetitive and intermittent right side hemiplegia, associated with varied degree of motor aphasia are the predominant clinical features in all three cases. They are recognized as the rare Moyamoya diseases by the confirmation of carotid angiographies. The boy had received bilateral superficial artery-middle cerebral artery bypass (STA-MCA bypass) and encephalo-myo-synangiosis with clinical improvement and was followed up regularly.

並列關鍵字

Moyamoya disease ； cerebrovascular occlusive disorder ； superficial temporal artery-middle cerebral artery bypass (STA-MCA bypass) ； encephalo-myo-synangiosis