Compliance and Factors Associated with Sleep Disordered Breathing in Myotonic Dystrophy Type 1 (S19.005)

OBJECTIVE: To assess prevalence, compliance, and associated patient factors with sleep disordered breathing (SDB) in Myotonic Dystrophy Type 1 (DM1). BACKGROUND: DM1 has a high prevalence of SDB, which can lead to further morbidity and mortality. DESIGN/METHODS: Analysis from a U.S. prospective, observational registry of clinically- and genetically-verified DM1 adult patients (age at entry: 42±12 yrs; follow-up: 9.5±3.2 yrs). Patients were surveyed with a study-designed sleep questionnaire, the Epworth Sleepiness Scale (ESS), the SF-36 Health Survey, and the Berlin Questionnaire for Sleep Apnea. RESULTS: 93 (64.6%) of 144 surveyed patients completed the assessment. 41 (44.1%) of 93 had been diagnosed with SDB, while 49 (52.7%) of 93 underwent a sleep study. 20 (48.8%) of the 41 diagnosed with SDB reported compliance with PAP. Patients diagnosed with SDB were weaker at study entry (muscular impairment rating 3.1±0.9 vs. 2.6±1.0, P=0.04), were more likely to have classical DM1 than either congenital- or senile-onset (50.1% vs. 20.0%, P=0.01), had ESS>10 (61.0% vs. 29.4%, P=0.002), and had a Berlin score indicating high risk (72.4% vs. 31.3%, P CONCLUSIONS: SDB is common in DM1, is associated with more severe muscle weakness, and occurs primarily in patients with a classical presentation. There is no relationship between BMI and SDB in DM1. Compliance rates with PAP treatment are comparable to non-DM1 patients and there are no clinical factors useful to determine patients destined to be non-compliant. Disclosure: Dr. Mazda has nothing to disclose. Dr. Bodkin has nothing to disclose. Dr. Mehta has nothing to disclose. Dr. Graham has nothing to disclose. Dr. Pascuzzi has nothing to disclose. Dr. Groh has nothing to disclose.