肌萎縮性側索硬化症(amyotrophic lateral sclerosis; ALS)是一種進行性的神經肌肉疾病,發生原因未明。病人大多數死於呼吸肌衰弱所導致的肺部功能下降,目前醫學界除了從基礎研究的方法試圖尋找ALS的治療方法外,還有許多的論文在探討如何延緩該疾病的過程並增進此類病人的生活品質。本文由呼吸治療的角度切入,從ALS的病理生理學及其對呼吸系統的影響開始,再分別介紹目前常用於監測此類病患的呼吸功能的工具,還有通氣輔助設備在此疾病中扮演的角色。
Amyotrophic lateral sclerosis (ALS) is a relentless, progressive neuromuscular disorder that is inevitably fatal. Because no therapeutic drug is available, treatment is primarily symptomatic, including respiration, nutrition, pain, cognitive disorder, and palliative care. In this article, we briefly review the respiratory-related pathophysiology, methods of respiratory system monitoring, and roles of mechanical insufflation-exsufflation and invasive/non-invasive assisted ventilation in ALS.