Background and purpose: To assess the long-term acceptance of non-invasive nasal continuous positive air pressure (CPAP) in multiple system atrophy (MSA) patients with polysomnographic (PSG)-confirmed sleep apneas and/or nocturnal stridor.
Patients and methods: Sleep-related breathing disorders were investigated by PSG in 22 MSA patients in whom stridor and sleep-related respiratory disturbances were clinically suspected. Patients in whom the first PSG disclosed either a sleep apnea/hypopnea index (AHI)> or =10 or stridor with or without apneas underwent a second PSG for CPAP titration.
Results: Three patients presented with an obstructive sleep apnea syndrome without stridor, whereas 15 patients presented stridor occurring alone or accompanied by apneas. Twelve patients pursued CPAP. Two severely disabled patients died a few days after CPAP initiation, and five discontinued CPAP because of discomfort. One patient died after 17 months of follow-up. Since the onset of CPAP, the four remaining patients reported more efficacious sleep and improved daytime alertness. These patients had significantly less severe disease at the time of CPAP initiation. Age, disease duration, the presence of sleep complaints, excessive daytime somnolence (EDS) and AHI did not account for CPAP compliance.
Conclusion: The severity of motor impairment at the initiation of treatment appears to be the most significant limiting factor for CPAP long-term acceptance.