僵直人症候群合併胸腺瘤經術後造成急性呼吸衰竭之病例報告

Experience of Respiratory Care for Stiff Person Syndrome

僵直人症候群（Stiff person syndrome; SPS）是一種極為罕見的神經系統疾病，其特徵為漸進式的波動性肌肉僵硬（progressive fluctuating muscular rigidity）及疼痛性的痙攣（spasms）。一般情形下，很少會發生急性呼吸衰竭（Acute respiratory failure）。本文描述一名52歲的女性因為經常性跌倒入院，且血清中檢測到大量的抗谷氨酸脫羧酶抗体（Anti-glutamic acid decarboxylase antibodies; anti-GAD antibodies），診斷為僵直人症候群。同時胸部電腦斷層掃描發現有胸腺瘤，經胸腺切除術後在加護病房期間突然全身癲癇發作、眼神向上凝視、發紺和低血氧導致急性呼吸衰竭。由於罕見病，其病理生理學尚不清楚。呼吸衰竭發生的機制可能為(1)呼吸暫停肌肉僵硬和陣發性肌肉痙攣，或(2)陣發性自主神經功能亢進。因此應考慮在SPS期間需持續監測可能進展至急性呼吸衰竭和猝死，作為急症的訊號。

Stiff person syndrome (SPS) is an extremely rare neurological disease characterized by progressive fluctuating muscular rigidity and spasms .In this case, acute respiratory failure is rarely reported. A 52-year-old woman was admitted to the hospital because of frequent falls. High levels of anti-glutamic acid decarboxylase antibodies (anti-GAD antibodies) in her serum were detected and she was diagnosed with Stiff person syndrome. Meanwhile, the chest computer tomography showed a thymoma. During the intensive care unit after the thymectomy, there was a sudden whole body seizure, staring eyes, cyanosis, and hypoxia leading to acute respiratory failure. Due to its rareness, its pathophysiology is unclear. The two suggested mechanisms are (1) apnea muscle stiffness and paroxysmal muscle spasms, or (2) paroxysmal autonomic hyperfunction. It should be noted that continuous monitoring during SPS may progress to acute respiratory failure and sudden death as an acute signal.