Amyotrophic lateral sclerosis (ALS) is a rare, neurodegenerative motor neuron disease that affects voluntary muscle movement. Often, difficulty in coughing, breathing, and swallowing are sequela associated with the condition, and the presence of bulbar muscle predominant weakness results in deleterious effects on airway clearance and secretion management. This narrative review will provide practical guidance for clinicians treating this population. Cough insufficiency in this population typically manifests as a prolonged, slow, weak cough effort that impedes the clearability of secretions and airway protection. Dystussia and dysphagia frequently occur simultaneously in bulbar dysfunction, subsequently impacting respiratory health. Measures of respiratory strength should be obtained and monitored every 3-6 months, preferably in a multidisciplinary clinic setting. Cough augmentation, whether manual or mechanical techniques, should be sought as early in the disease progression as possible to adequately control secretions in the proximal airways. This airway clearance strategy can aid in the prevention and treatment of respiratory tract infections (RTIs), which can pose a significant clinical hurdle to those with ALS. The use of mechanical insufflation-exsufflation may be complicated by severe bulbar dysfunction rendering this technique ineffective. Though peripheral airway clearance strategies, such as high-frequency chest-wall compression, have the advantage of being less impacted by bulbar dysfunction, it is only recommended this modality be used in conjunction with, versus in lieu of, proximal strategies. Salivary secretion management includes the use of anticholinergics, botulinum toxin, and radiation therapy depending on severity and desire for relief.

中文翻译：

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肌萎缩侧索硬化症的气道清除策略和分泌物管理。

肌萎缩侧索硬化症 (ALS) 是一种罕见的神经退行性运动神经元疾病，会影响随意肌肉运动。通常，咳嗽、呼吸和吞咽困难是与该病症相关的后遗症，并且球肌主要无力的存在会对气道清除和分泌管理产生有害影响。这篇叙述性综述将为治疗这一人群的临床医生提供实用指导。该人群的咳嗽不足通常表现为长时间、缓慢、微弱的咳嗽，妨碍分泌物的清除和气道保护。延髓功能障碍经常会同时出现发音困难和吞咽困难，从而影响呼吸系统健康。应每 3-6 个月进行一次呼吸强度测量并进行监测，最好是在多学科诊所环境中进行。无论是手动还是机械技术，都应在疾病进展的早期寻求增强咳嗽的方法，以充分控制近端气道的分泌物。这种气道清除策略有助于预防和治疗呼吸道感染 (RTI)，这种感染可能给 ALS 患者带来重大的临床障碍。机械吹气-吹气的使用可能因严重的延髓功能障碍而变得复杂，导致该技术无效。尽管外周气道清除策略（例如高频胸壁压迫）具有受延髓功能障碍影响较小的优点，但仅建议将这种方式与近端策略结合使用，而不是代替近端策略。唾液分泌管理包括根据严重程度和缓解愿望使用抗胆碱能药物、肉毒杆菌毒素和放射治疗。