HRB Clinician Scientist, Trinity College and Beaumont Hospital, Dublin, Ireland.
J Neurol. 2011 Mar;258(3):359-65. doi: 10.1007/s00415-010-5830-y. Epub 2010 Nov 17.
Respiratory insufficiency is a frequent feature of ALS and is present in almost all cases at some stage of the illness. It is the commonest cause of death in ALS. FVC is used as important endpoint in many clinical trials, and in decision-making events for patients with ALS, although there are limitations to its predictive utility. There are multiple causes of respiratory muscle failure, all of which act to produce a progressive decline in pulmonary function. Diaphragmatic fatigue and weakness, coupled with respiratory muscle weakness, lead to reduced lung compliance and atelectasis. Increased secretions increase the risk of aspiration pneumonia, which further compromises respiratory function. Bulbar dysfunction can lead to nutritional deficiency, which in turn increases the fatigue of respiratory muscles. Early recognition of respiratory decline and symptomatic intervention, including non-invasive ventilation can significantly enhance both quality of life and life expectancy in ALS. Patients with respiratory failure should be advised to consider an advance directive to avoid emergency mechanical ventilation.
呼吸功能不全是 ALS 的常见特征，几乎所有病例在疾病的某个阶段都会出现。它是 ALS 最常见的死因。FVC 是许多临床试验中的重要终点，也是 ALS 患者决策事件的重要指标，尽管它的预测效果有一定的局限性。呼吸肌衰竭有多种原因，所有这些原因都会导致肺功能逐渐下降。膈肌疲劳和无力，加上呼吸肌无力，导致肺顺应性降低和肺不张。分泌物增加会增加吸入性肺炎的风险，从而进一步损害呼吸功能。延髓功能障碍可导致营养缺乏，进而增加呼吸肌疲劳。早期识别呼吸功能下降并进行症状干预，包括无创通气，可显著提高 ALS 患者的生活质量和预期寿命。对于呼吸衰竭的患者，应建议他们考虑预先指示，以避免紧急机械通气。
