Discontinuation of continuous positive airway pressure treatment in multiple system atrophy

Abstract Objective Continuous positive airway pressure (CPAP) is used for the management of sleep-disordered breathing in patients with multiple system atrophy (MSA). However, the long-term outcome after CPAP treatment is yet to be ascertained. Methods A retrospective study was performed to investigate the frequency and causes of CPAP treatment discontinuation, and to ascertain the determinations of CPAP treatment duration in Japanese patients diagnosed with probable MSA based upon the consensus diagnostic criteria, who were admitted to our hospital from 2001 to 2012. Results Twenty-nine consecutive patients treated with CPAP were analyzed. During the observation period, 19 patients (66%) discontinued CPAP treatment. The median CPAP treatment duration was 13.0 months (range, 1–53 months). The major causes for discontinuation were pulmonary infection, respiratory insufficiency of undetermined origin, and CPAP intolerance. On comparing the clinical characteristics of the groups subjected to short- and long-term CPAP treatment, floppy epiglottis was more frequently observed in the short-term group than in the long-term group (64% vs 15%; P = 0.015). Conclusion The CPAP treatment duration in MSA patients was not long, and floppy epiglottis may be a determinant of the duration of CPAP treatment.