Respiratory therapies for Amyotrophic Lateral Sclerosis: A state of the art review

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative condition noteworthy for upper and lower motor neuron death. Involvement of respiratory motor neuron pools leads to progressive pathology. These impairments include decreases in neural activation and muscle coordination, progressive airway obstruction, weakened airway defenses, restrictive lung disease, increased risk of pulmonary infections, and weakness and atrophy of respiratory muscles. These neural, airway, pulmonary, and neuromuscular changes deteriorate integrated respiratory-related functions including sleep, cough, swallowing, and breathing. Ultimately, respiratory complications account for a large portion of morbidity and mortality in ALS. This state-of-the-art review highlights applications of respiratory therapies for ALS, including lung volume recruitment, mechanical insufflation-exsufflation, non-invasive ventilation, and respiratory strength training. Therapeutic acute intermittent hypoxia, an emerging therapeutic tool for inducing respiratory plasticity will also be introduced. A focus on emerging evidence and future work underscores the common goal to continue to improve survival for patients living with ALS.

Automated summary

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by motor neuron death. Respiratory motor neuron impairments lead to various respiratory complications, ultimately accounting for a significant portion of morbidity and mortality in ALS. This review discusses respiratory therapies for ALS, including lung volume recruitment, mechanical insufflation-exsufflation, non-invasive ventilation, and respiratory strength training. It also introduces therapeutic acute intermittent hypoxia as a potential treatment to induce respiratory plasticity. The focus on emerging evidence and future work aims to improve survival for ALS patients.