Abstract：
In amyotrophic lateral sclerosis (ALS), Guillain-Barré syndrome (GBS), and neuromuscular junction disorders, three mechanisms may lead, singly or together, to respiratory emergencies and increase the disease burden and mortality: (i) reduced strength of diaphragm and accessory muscles; (ii) oropharyngeal dysfunction with possible aspiration of saliva/bronchial secretions/drink/food; and (iii) inefficient cough due to weakness of abdominal muscles. Breathing deficits may occur at onset or more often along the chronic course of the disease. Symptoms and signs are dyspnea on minor exertion, orthopnea, nocturnal awakenings, excessive daytime sleepiness, fatigue, morning headache, poor concentration, and difficulty in clearing bronchial secretions. The \"20/30/40 rule\" has been proposed to early identify GBS patients at risk for respiratory failure. The mechanical in-exsufflator is a device that assists ALS patients in clearing bronchial secretions. Noninvasive ventilation is a safe and helpful support, especially in ALS, but has some contraindications. Myasthenic crisis is a clinical challenge and is associated with substantial morbidity including prolonged mechanical ventilation and 5%-12% mortality. Emergency room physicians and consultant pulmonologists and neurologists must know such respiratory risks, be able to recognize early signs, and treat properly.
摘要：
在肌萎缩侧索硬化症（ALS）中，格林-巴利综合征（GBS）和神经肌肉接头疾病，三种机制可能导致，单独或一起，呼吸急症和增加疾病负担和死亡率:(i)膈肌和副肌的强度降低;(ii)口咽功能障碍,可能吸入唾液/支气管分泌物/饮料/食物;和(iii)由于腹肌无力引起的低效咳嗽。呼吸缺陷可在发病时或在疾病的慢性过程中更频繁地发生。症状和体征是轻微劳累时呼吸困难，端坐呼吸,夜间觉醒，白天过度嗜睡，疲劳,早上头痛,浓度差,很难清除支气管分泌物。已提出“20/30/40规则”以早期识别有呼吸衰竭风险的GBS患者。机械吹出器是一种帮助ALS患者清除支气管分泌物的装置。无创通气是一种安全和有益的支持，特别是在ALS中，但是有一些禁忌症。肌无力危象是一项临床挑战，与大量的发病率有关，包括长时间的机械通气和5％-12％的死亡率。急诊室医生和咨询肺科医师和神经科医师必须知道这种呼吸风险，能够识别早期迹象，并适当对待。
