急性發炎性去髓鞘多發性神經病變：病例報告

急性發炎性去髓鞘多發性神經病變(acute inflammatory demyelinating polyradiculoneuropathy, AIDP)也就是所謂的居楊-巴賀症候群(Guillain-Barre syndrome, GBS)，是最常見引起急性全身性癱瘓現象的疾病。一般認為是一種後天性、對稱型的多發性神經病變，最先開始影響下肢，肌肉牽張反射為消失或嚴重減退。診斷的依據包括患者臨床的表現，電生理學的檢查及腦脊髓液上的變化。典型的症狀包括開始於腳趾或手指的指尖，發生四肢尤其是下肢之上行性無力的現象，甚至影響到呼吸肌、臉部或口咽部的肌肉。疼痛亦相當常見。脊髓穿刺常可發現脊髓液中有蛋白質濃度增加的現象。治療上以血漿置換及靜脈輸入免疫球蛋白為主，並輔以支持療法及復健計劃。對於影響呼吸肌的病人，則需要更詳密的呼吸照護。本報告在此提出兩位患者診斷及復健治療的經驗，藉此對AIDP這個症候群做一個討論與文獻回顧。

關鍵字

居楊-巴賀症候群；急性發炎性去髓鞘多發性神經病變

並列摘要

Acute inflammatory demyelinating polyradiculoneuropathy (AIDP), also known as Guillain-Barre syndrome (GBS)，the most common illness that causes acute and generalized paralyzing condition in the post-polio era, is diagnosed dependent on the base of clinical manifestations with supportive evidence of electrophysiologic study and cerebrospinal fluid examination. GBS often begins with the distal part of four limbs followed by a progressive course as ascending weakness and may even involve respiratory , facial or oropharyngeal muscles. Spinal fluid examination often reveals elevated protein concentration without associated increase in cells. Plasma exchange has become the gold standard of GBS treatment; in patients contraindicated for plasma exchange, intravenous immune globulin therapy has the same efficacy and safety. Rehabilitation program is crucial for patients’ functional achievement and comprehensive pulmonary care is encouraged in patients with respiratory insufficiency. We propose the experiences of two cases’ management and have a literature review and discussion of this special syndrome.

並列關鍵字

Guillain-Barre syndrome ； acute inflammatory demyelinating polyradiculoneuropathy

國際替代計量

急性發炎性去髓鞘多發性神經病變：病例報告

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