肌萎縮性側索硬化症之診斷及相關之復健議題：病例報告

肌萎縮性側索硬化症（amyotrophic lateral sclerosis）是一種病因未明的神經退化性疾病，它是一種少見卻不罕見的疾病，故臨床醫師對其診斷應有一定的認識。病患會表現上運動神經元合併下運動神經元症狀，當典型合併症狀發生於中老年男性，診斷不是那麼地困難。但在疾病早期，常發現只有上運動神經元或下運動神經元的症狀較明顯，且症狀可能只發生在某一肢體，因此早期診斷相當困難。本篇報告的個案首因左手麻木、無力來本院求診。病患曾先後被診斷為左側臂神經叢病變及頸部脊髓、神經根病變，由於症狀持續進展，病患於發病後一年接受頸椎手術。然而，右側上肢、左側下肢而後亦發生無力，根據臨床表現、理學檢查以及電生理檢查，病患最終診斷罹患肌萎縮性側索硬化症。我們回顧文獻，瞭解關於肌萎縮性側索硬化症診斷的重點、常見的誤診等。病患平均存活只有約三年，但有不可忽略的少數可存活更長的時間，同時，有限的存活並不排除病患享有更好的生活品質，於是復健治療佔有重要的角色。我們回顧關於肌萎縮性側索硬化症復健治療的文章，期望可提供病患更完整、全面的治療，提高病患的生活品質。

關鍵字

肌萎縮性側索硬化症；診斷；復健

並列摘要

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease of unknown etiology. Even though it is uncommon, there are cases seen. Thus it is important for clinicians to understand and catch the key points to its diagnosis. Patients with ALS would usually present with the combination of upper and lower motor neuron symptoms and signs. When the typical combination was found in several anatomic areas in an elderly man especially, the diagnosis is direct. We must also note that at early stage of ALS, the patient can present predominantly with either upper or lower motor neuron symptoms and signs confining only to one extremity, which makes an early diagnosis difficult. Our case came to our hospital with the chief complaint of numbness and weakness in his left hand. The initial diagnosis was brachial plexopathy but with revision to cervical myeloradiculopathy later. Cervical spine surgery was performed but weakness of right upper extremity and left lower extremity soon occurred progressively. From the finding of clinical presentation, physical examination and electrophysiologic study, a final diagnosis of ALS was made. We reviewed papers to discuss the key point to diagnosis of ALS and the usual misdiagnosis. The mean survival in ALS patients is about 3 years, but occasionally do we see patients survive past that. Patients with ALS not only have a limited life span, they often have poor quality of life. Thus it is important for a rehabilitation intervention to improve the quality of life.

並列關鍵字

amyotrophic lateral sclerosis ； diagnosis ； rehabilitation

國際替代計量

肌萎縮性側索硬化症之診斷及相關之復健議題：病例報告

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