Respiration in Chronic Neuromuscular Diseases

Aim: Respiratory muscle weakness is the inevitable consequence of many neuromuscular disorders. Deterioration in respiratory function contributes to significant morbidity and is responsible for high mortality rates with these diseases. The aim of this review to summarize diagnostic and therapeutic approaches for respiratory problems in patients with neuromuscular disorders. Main Findings: Usually, chronic respiratory failure develops slowly over a period of several years. Initially, it presents with disordered breathing that is apparent only during sleep, followed by continuous progression to severe hypoventilation, cor pulmonale and eventually frank respiratory failure in end-stage disease. Early detection of respiratory compromise and preventive care are likely to improve outcome and quality of life for many patients. This has led to the development of proactive monitoring and treatment programmes that include regular assessment of pulmonary functions, respiratory strength and sleep studies, home mechanical ventilation, chest physiotherapy, early antibiotic treatment and nutritional intervention. In particular, noninvasive positive pressure ventilation is effective in resolving respiratory failure and has dramatically changed the management of these patients. Conclusion: Pulmonary functions and sleep related respiratory problems should be followed up regularly in patients with neuromuscular disorders. Noninvasive mechanical ventilation is the most important therapeutic option for the management of respiratory failure in these patients group.