Managing breathing in neuromuscular weakness: Best practices

Background: Neuromuscular diseases (NMD) are a broad group of disorders that impair or damage peripheral nerves or muscles. Respiratory complications, including respiratory failure and aspiration risk, contribute to the morbidity and mortality of NMDs. Methods: Narrative literature review on the PubMed platform. Results: Some NMDs, particularly nonprogressive disorders, including diaphragm dysfunction, may be amenable to conservative management such as weight loss, positional therapy, and inspiratory muscle training. Noninvasive ventilatory support is an option for most NMDs and can improve quality of life and survival. For progressive disorders, failure of noninvasive support or difficulty managing bulbar symptoms, invasive ventilation may be considered. Mechanical insufflation-exsufflation and oscillatory devices that support expiratory function may reduce the risk of aspiration. Sialorrhea can also contribute to the aspiration risk, particularly in amyotrophic lateral sclerosis, with management options that include anticholinergic agents, botulinum toxin injection, or radiation therapy. Diaphragm plication is an option in nonprogressive disorders and asymmetric diaphragm impairment that has not recovered on its own. Diaphragm pacing is approved for patients with spinal cord injuries who are ventilator dependent and may be effective in various causes of unilateral or bilateral diaphragm impairments, although it may impart a survival disadvantage in amyotrophic lateral sclerosis. Diaphragm pacing may be combined with phrenic nerve reconstruction in select patients. Finally, emerging precision therapies directly address the fundamental pathophysiology of NMDs. Conclusion: Treatment of NMDs with conservative management, inspiratory and expiratory support, and surgical options can favorably impact survival and quality of life. Precision therapies have a demonstrated potential to favorably modify the course of several of those disorders.