Sleep-Disordered Breathing in Neuromuscular Disease: Diagnostic and Therapeutic Challenges

Normal sleep related rapid eye movement sleep atonia, reduced lung volumes, reduced chemosensitivity, and impaired airway dilator activity, become significant vulnerabilities in the setting of neuromuscular disease. In that context, the compounding effects of respiratory muscle weakness and disease-specific features that promote upper airway collapse or cause dilated cardiomyopathy, contribute to various sleep-disordered breathing events. The reduction in lung volumes with neuromuscular disease is further compromised by sleep and the supine position, exaggerating the tendency for upper airway collapse and desaturation with sleep-disordered breathing events. The most commonly identified events are diaphragmatic /pseudo-central, due to a decrease in the rib cage contribution to the tidal volume during phasic REM sleep. Obstructive and central sleep apneas are also common. Non-invasive ventilation can improve survival and quality of sleep but should be used with caution in the context of dilated cardiomyopathy or significant bulbar symptoms. Non-invasive ventilation can also trigger sleep-disordered breathing events including ineffective triggering, auto-triggering, central sleep apnea and glottic closure, which compromise the potential benefits of the intervention by increasing arousals, reducing adherence, and impairing sleep architecture. Polysomnography plays an important diagnostic and therapeutic role by correctly categorizing sleep-disordered events, identifying sleep-disordered breathing triggered by non-invasive ventilation, and improving non-invasive ventilation settings. Optimal management may require dedicated hypoventilation protocols and a technical staff versed in identification and troubleshooting respiratory events.

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