Chapter 2 - Pulmonary pathophysiology of neuromuscular disease

Patients with neuromuscular weakness have a high risk of developing significant respiratory morbidity throughout life. Respiratory failure is recognized as the main cause of mortality in these conditions. Nevertheless, the prevalence, severity, and progression of respiratory impairment may vary significantly across specific neuromuscular conditions. This is due to the combination of skeletal muscle weakness, disease-specific respiratory muscle involvement, and the variable severity of comorbidities affecting respiratory function such as kyphoscoliosis, axial/truncal weakness, and bulbar involvement.