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Introduction: Myotonic Dystrophy(DM) is a progressive neurological condition. Respiratory failure is the leading cause of mortality in DM. Compliance with positive airway pressure therapy (PAP) remains poor in patients with DM due to lack of perceived benefit. Clinical course suggests central dysregulation of control of breathing contributes to alveolar hypoventilation independent and in addition to respiratory muscle weakness. Aim: We looked to investigate impact of PAP on carbon dioxide (CO2) levels and its correlation with compliance. Methods: Records of 70 patients with DM were retrospectively analysed. The variables included spirometry, respiratory muscle assessment, excessive daytime sleepiness (EDS), ODI and CO2 levels at the time of initiation of PAP therapy. Follow up (FU) visits were arranged. Results: 45 patients were initiated on PAP therapy (Table 1).62% (n=28) of patients complained of EDS with mean CO2 of 6.02 kPa at initiation.FU CO2 did not show significant improvement (mean 5.98kpa). Compliance was poor with 53% (n=24) using less than 4 hours and was comparable in NIV and CPAP groups (59% and 63%). Mortality was higher in NIV group (41%) as compared to CPAP group (9%) Discussion: EDS in patients with DM is independent of CO2 levels indicating underlying neurological dysfunction. In our cohort, patients with near normal CO2 levels had poorer compliance.
Irshad et al. (Thu,) studied this question.