Neuromyelitis Optica (Devic's Disease) Report of One Case

視神經脊髓炎（Devic 氏病）：一病例報告

柯芳蓉(Fang-Jong Ko) ； 江季勛(Chi-Hsiun Chiang) ； 鐘育志(Yuh-Jyh Jong) ； 張中興(Chung-Hsing Chang)

《Acta Paediatrica Sinica》 30卷6期 (1989/12) Pp. 428-431

摘要

Neuromyelitis Optica又名Devic氏病，定義爲視神經炎及橫貫性脊髓炎兩者同時急性發生的症候群。一般認爲是多發性硬化症的一種，但臨床表現上可能只有一次發作而沒有以後反覆出現及惡化的趨勢。高雄醫學院小兒科經歷一12歲女孩，症狀開始于下肢酸痛，漸漸變成下肢無力，約36小時後下肢完全癱瘓，並有尿瀦留，便秘及視力模糊等症狀。神經學檢查顯示：意識嗜睡構語模糊，視力銳减，只對光有反應，視野縮小，辨色力差，瞳孔等大，對光反射正常。眼底檢查在兩側顳葉稍顯蒼白，但受視乳頭水腫。眼球運動有疼痛感及障礙，幷有複視。其他腦神經檢查均正常。肌力檢查：下肢Gr.O，上肢Gr.3－4；深腱反射均消失；感覺檢查：T5以下對痛、溫度、振動、輕撫及位置感均消失。脊髓液內細胞中等度上升，蛋白質112mg/dl，糖分57mg/dl, IgG:14.lmg/dl, IgA:2. 85mg/dl, IgM:2.37mg/di，並無發現單株抗體。血清病毒抗體Anti-EB病毒價效大於1:320。神經電學檢查：腦波呈連續性慢波，四肢神經傳導速度(NCV)正常，體感覺誘發電位(SSEP)呈現中樞傳導時間延長，視覺誘發電位(VEP)兩眼均呈現Latency延長，Amplitude下降的情形。脊髓造影斷層掃描及腦斷層掃描均無異常。病人在Devic氏病的診斷下給予支持療法及prednisolone治療，視神經炎進步遠較橫貫性神經炎迅速，病人於急性發作後20天出院，視力及視覺誘發電位已接近正常，但仍有下肢無力及左下腿感覺异常。六個月後追踪檢查，視力完全正常，肌力爲Gr.5，無感覺异常，但仍偶有尿失禁現象。在最近兩年內病人並無疾病再發或惡化的情形。

並列摘要

Neuromyelitis optica also named Devic's disease is an acute combined optic neuritis and transverse myelitis. It is thought to be a variant of multiple sclerosis, but its clinical presentation probably has only one attack without further recurrence and exacerbation. We present a 12-year-old girl who suffered from sudden onset of lower extremeties weakness, sensory loss and blurred vision after a prodromal URI symptom. CSF examination showed mild pleocytosis, elevated immunoglobulins, mild elevation of protein concentration. No oligoclonal band was detected. Serum virology showed high titer of anti-EB virus antibody. Visual evoke potential showed prolong of latency and decreased amplitude of both eyes. After prednisolone treatment, her visual accuity began to improve on the 7th hospital day and motor function improved on the 11th hospital day. Two years later, she has normal visual accuity, normal motor function and shows no evidence of disease recurrence.

並列關鍵字

neuromyelitis optica ； Devic's disease ； multiple sclerosis