全身性紅斑狼瘡併發毛毛樣血管症候群之臨床表現及復健治療：病例報告

Clinical Features and Rehabilitation of Systemic Lupus Erythematosus Complicated with Moyamoya Syndrome: A Case Report

10.6315/2008.36(4)06

連偉志(Wei-Chih Lien)；陳若佟(Jo-Tong Chen)；林書正(Shu-Cheng Lin)；郭乃文(Nai-Wen Guo)；官大紳(Da-Shen Kuan)

全身性紅斑狼瘡 ； 毛毛樣血管症候群 ； 復健 ； systemic lupus erythematosus ； moyamoya syndrome ； rehabilitation

台灣復健醫學雜誌

36卷4期（2008 / 12 / 01）

241 - 250

繁體中文

毛毛樣血管症(moyamoya disease)是一個罕見的腦血管病變，其主要特徵是兩側內頸動脈遠端及前、中大腦動脈近端發生漸進式狹窄阻塞，並因補償作用於基底核區發展出廣泛性網狀側枝循環，於腦血管攝影檢查之下看起來就像“煙霧”一般。毛毛樣血管症候群是指毛毛樣血管病變表現在患有其他疾病的病人身上。本文報告一位二十六歲全身性紅斑狼瘡的女性病人，於發生多次缺血性腦中風之後，經腦血管攝影檢查證實病患兩側內頸動脈遠端狹窄併毛毛樣血管病變。病患因左側肢體癱瘓、左側中樞型顏面神經麻痺、假性延髓麻痺致嚴重吞嚥及構音困難轉至復健科病房。復健內容包括了口腔動作訓練、構音訓練、吞嚥訓練、平衡訓練、姿態訓練、肌力訓練、步行訓練和日常生活訓練等。本文報告病患的臨床表現及其藥物治療與復健治療，並針對全身性紅斑狼瘡合併毛毛樣血管症候群的相關文獻作回顧與討論。

Moyamoya disease is an uncommon clinical entity characterized by progressive narrowing and occlusion of the bilateral distal internal carotid arteries, as well as the proximal parts of their major branches, namely, the middle and anterior cerebral arteries. Concomitant development of prominent collateral vascularity in the basal regions of the brain results in the typical angiographic appearance of moyamoya, which literally means a wavering puff of smoke. Moyamoya syndrome refers to findings of moyamoya vasculopathy in the context of known systemic disease. We present a 26-year-old woman with systemic lupus erythematosus who had several episodes of ischemic stroke. Her cerebral angiograms revealed a typical moyamoya phenomenon. She was transferred to the rehabilitation ward because of weakness of her left limbs, left central facial palsy, pseudobulbar palsy, severe dysarthria and dysphagia. Her rehabilitation program included speech therapy, strengthening and oral motor exercises, as well as training in swallowing, balance, posture, ambulation, and activities of daily living. We describe the clinical features of systemic lupus erythematosus complicated with moyamoya syndrome and review the literature.