Benzo-Iglesias María Jesús, Rocamora-Pérez Patricia, Valverde-Martínez María de Los Ángeles, García-Luengo Amelia Victoria, Benzo-Iglesias Pedro Miguel, López-Liria Remedios
Health Research Centre, Humanidades-498 Research Group, Department of Nursing, Physiotherapy and Medicine, University of Almería, Almería, Spain.
Health Research Centre, Humanidades-498 Research Group, Department of Nursing, Physiotherapy and Medicine, University of Almería, Carretera de Sacramento s/n, La Cañada de San Urbano, Almería 04120, Spain.
Ther Adv Respir Dis. 2025 Jan-Dec;19:17534666251346095. doi: 10.1177/17534666251346095. Epub 2025 Jun 9.
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that affects motor neurons, resulting in muscle weakness, loss of function, and ultimately death due to respiratory failure. Due to the lethal prognosis of ALS, respiratory muscle training has been proposed as a potentially beneficial intervention.
To systematically review the efficacy of respiratory muscle training on lung function and respiratory muscle strength in ALS patients.
A systematic review and meta-analysis of randomized controlled trials.
Articles published in PubMed, PEDro, Scopus, and Web of Science databases up to July 2024. The Preferred Reporting Items for Systematic reviews and Meta-Analyses 2020 statement guideline was followed. Included studies had (1) ALS patients, (2) respiratory muscle training, (3) physical exercise, usual care or no intervention were provided as a comparison group, (4) assessments of lung function, respiratory muscle strength, quality of life, survival, fatigue, and functional capacity outcome measures, and (5) a randomized controlled trial design. Methodological quality was analyzed using the PEDro scale, and risk of bias with the Cochrane Collaboration Risk of Bias Tool. Meta-analyses were performed with Review Manager software.
Five randomized controlled trials with 170 participants were included. The results showed that respiratory muscle training improved muscle strength, particularly maximum expiratory and inspiratory pressures. One study suggested inspiratory muscle training as a survival predictor in ALS patients. No significant effects were observed in forced vital capacity or quality of life. No adverse effects were reported.
Respiratory muscle training improves ventilatory function, particularly respiratory muscle strength, in people with ALS. While evidence is limited, it shows promise as an adjuvant therapy to enhance quality of life and survival. It has been registered in the PROSPERO (CRD42024568235).
肌萎缩侧索硬化症（ALS）是一种进行性神经退行性疾病，会影响运动神经元，导致肌肉无力、功能丧失，最终因呼吸衰竭而死亡。由于ALS的致命预后，呼吸肌训练已被提议作为一种可能有益的干预措施。
系统评价呼吸肌训练对ALS患者肺功能和呼吸肌力量的疗效。
对随机对照试验进行系统评价和荟萃分析。
检索截至2024年7月在PubMed、PEDro、Scopus和Web of Science数据库中发表的文章。遵循系统评价和荟萃分析的首选报告项目2020声明指南。纳入的研究需满足：（1）ALS患者；（2）呼吸肌训练；（3）将体育锻炼、常规护理或不进行干预作为对照组；（4）评估肺功能、呼吸肌力量、生活质量、生存率、疲劳和功能能力结局指标；（5）随机对照试验设计。使用PEDro量表分析方法学质量，使用Cochrane协作偏倚风险工具分析偏倚风险。使用Review Manager软件进行荟萃分析。
纳入了5项随机对照试验，共170名参与者。结果表明，呼吸肌训练可提高肌肉力量，尤其是最大呼气和吸气压力。一项研究表明吸气肌训练是ALS患者生存的预测指标。在用力肺活量或生活质量方面未观察到显著影响。未报告不良反应。
呼吸肌训练可改善ALS患者的通气功能，尤其是呼吸肌力量。虽然证据有限，但它有望作为辅助治疗提高生活质量和生存率。该研究已在PROSPERO（CRD42024568235）注册。
