多發性進行性顱內血管阻塞－MOYAMOYA疾病和其症候群

MULTIPLE PROGRESSIVE INTRA-CRANIAL ARTERIAL OCCLUSION - MOYAMOYA DISEASE AND ITS SYNDROME

郭萬佑(Wan-Yuo Guo) ； 鄧木火(Michael Mu-Huo Teng) ； 陳信實(Sin-Sue Chen) ； 張遵(Wu-Chung Shen) ； 沈戊忠(Chien-Fan Yang) ； 楊建芳(Tsuen Chang)

《中華放射線醫學雜誌》 12卷3期 (1987/09) Pp. 249-255

摘要

使民國69年到76年台北榮民總醫院及台中分院共有13例經血管攝影發現是多發性顱內血管狹窄或阻塞的病例。其中8例呈兩側內顫動脈末端阻塞符合moyamoya的診斷標準。兩例只有單側內頸動脈末端發生阻塞，可能是因為moyamoya疾病引起；另外3例則分別因動脈硬化，星狀細胞瘤，系統性紅斑狼瘡引起血管血栓或阻塞，我們分析這些病例的各項臨床資料與放射線檢查表徵，並將之文獻上的報告做比較。雖然在台灣地區。moyamoya疾病的年發生率報告只有0.018/100,000，但實際病倒數可能高於此數，這有賴於醫師的提高警覺及各項放射線檢查的配合才能證實，對於電腦斷層攝影發現可疑的病例，進一步血管攝影是必要的診斷步驟，而數位化贅影去除血管攝影的應用，除了可以減少血管攝影的危臨性以外，更可方便地應用於病例的追蹤檢查或發掘更多的病例。而臨床上可能引起moyamoya血管的各種原因的排除，更是診斷moyamoya疾病的重要步驟。

並列摘要

From 1980 to 1987, there were thirteen cases of multiple progressive intra-cranial arterial occlusive disease in our hospitals. Eight of them were diagnosed as moyamoya disease who had fullfilled the criteria. Two cases with unilateral involvement were propable cases of moyamaya disease. The other three cases with mimetic angiographic pictures were due to atherosclerosis, astrocytoma, and systemic lupus erythematosus respectively. The clinical data and radiographic pictures were analysed. In comparison with other series in the literature and the differential diagnosis are discussed in details.