Abstract Details

Title Compliance and Factors Associated with Sleep Disordered Breathing in Myotonic Dystrophy Type 1

Topic Sleep

Presentation(s) S19 - (-)

Poster/Presentation
Number 005

Objective

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<0.001). There was a trend for SDB to be more common in males than females (53.3% vs. 35.4%, P=0.08). There was no difference in SF-36 scores, body mass index (BMI), cytosine-thymine-guanine (CTG) repeat length, or DM1-related cardiac complications in those with SDB. No clinical factors differentiated those with SDB who were compliant or non-compliant with PAP.

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.

Authors/Disclosures
Monica E. Mazda, MD PRESENTER	No disclosure on file
Cynthia Bodkin, MD, FAAN (Indiana University)	Dr. Bodkin has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. The institution of Dr. Bodkin has received research support from Massachusetts General Hospital. The institution of Dr. Bodkin has received research support from Alector LL. The institution of Dr. Bodkin has received research support from Atlantic research group. The institution of Dr. Bodkin has received research support from Amicus Therapeutics. The institution of Dr. Bodkin has received research support from Alexion. The institution of Dr. Bodkin has received research support from Anelixis. The institution of Dr. Bodkin has received research support from Medicinova INC. The institution of Dr. Bodkin has received research support from Ra Pharmaceuticals Inc.
	No disclosure on file
Taylor Graham, MD	Dr. Graham has nothing to disclose.
Robert M. Pascuzzi, MD, FAAN (Indiana Univ Schl of Medicine)	Dr. Pascuzzi has nothing to disclose.
David Vaillancourt	David Vaillancourt has received personal compensation for serving as an employee of Automated Imaging Diagnostics. David Vaillancourt has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Wiley. The institution of David Vaillancourt has received research support from NIH. David Vaillancourt has received intellectual property interests from a discovery or technology relating to health care.
William J. Groh, MD (Medical University of South Carolina)	No disclosure on file
	No disclosure on file

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