Abstract Details

Title Resting Blood Pressure Patterns in Adults With Myotonic Dystrophy Type One and Type Two

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P8 - Poster Session 8 (11:45 AM-12:45 PM)

Poster/Presentation
Number 9-004

Objective The aim of this study is to characterize resting blood pressure (BP) patterns in adults with myotonic dystrophy type 1 (DM1) and type 2 (DM2) and to assess whether values differ from contemporary U.S. population norms.

Background Myotonic Dystrophy is the most common adult-onset muscular dystrophy and is characterized by muscle weakness and multisystem involvement, including cardiac and autonomic dysfunction. Previous studies have suggested that adults with DM1 may exhibit lower resting BP, potentially related to autonomic impairment or other systemic factors; however findings have been limited by small sample sizes. Population-level comparisons to contemporary reference data are lacking.

Design/Methods A retrospective chart review was conducted of all adults attending the myotonic dystrophy clinic between January 2020 and December 2023. Eligible participants were aged 18-100 years with a clinical or genetically confirmed diagnosis of DM1 or DM2 and at least one valid BP measurement obtained using a calibrated automatic sphygmomanometer. Participants with an undetermined diagnosis or taking antihypertensive medications were excluded. Mean systolic (SBP) and diastolic (DBP) blood pressures were stratified by sex, diagnosis, and age group (18–39, 40–59, and ≥ 60 years) and descriptively compared with National Health and Nutrition Examination Survey (NHANES) 2021–2023 data.

Results Seventy-seven participants met eligibility criteria (mean age 46 years; 53% male). DM1 accounted for 88% (n=68) and DM2 for 12% (n=9). Mean BP in DM1 (SBP 122.5 mmHg, DBP 79.2 mmHg) was comparable to NHANES adults (SBP 119.5 mmHg, DBP 75.7 mmHg), while DM2 participants exhibited higher values (SBP 141.3 mmHg DBP 85.8 mmHg). No subgroup demonstrated systemic hypotension.

Conclusions

Adults with DM1 display normotensive BP profiles comparable to population averages, whereas DM2 participants show mild elevations. These results suggest that systemic hypotension is not a consistent feature of myotonic dystrophy. Future studies incorporating longitudinal follow-up and physiological assessments are warranted to further characterize BP regulation patterns.

Authors/Disclosures
Jennifer Argudo PRESENTER	Jennifer Argudo has nothing to disclose.
Matthew Vasquez, MD (University of Florida - Neurology)	Dr. Vasquez has nothing to disclose.
S H. Subramony, MBBS, FAAN (University of Florida)	Dr. Subramony has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Avidity. Dr. Subramony has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Dyne. Dr. Subramony has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Lupin. Dr. Subramony has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Fallonmedica. Dr. Subramony has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Dr. Subramony has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amicus. Dr. Subramony has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Fulcrum. The institution of Dr. Subramony has received research support from Reata. The institution of Dr. Subramony has received research support from Retrotope. The institution of Dr. Subramony has received research support from Acceleron. The institution of Dr. Subramony has received research support from Biohaven. The institution of Dr. Subramony has received research support from Pharnext. The institution of Dr. Subramony has received research support from Fulcrum. The institution of Dr. Subramony has received research support from National Ataxia Foundation. The institution of Dr. Subramony has received research support from Friedreich Ataxia Research Alliance. The institution of Dr. Subramony has received research support from Muscular Dystrophy Association. The institution of Dr. Subramony has received research support from Univ of Rochester, MDA. The institution of Dr. Subramony has received research support from Virginia Commonwealth Univ (FDA, Wyck Foundation)). The institution of Dr. Subramony has received research support from Children's Hospital, Philadelphia (FDA). The institution of Dr. Subramony has received research support from Houston Methodist (NIH). The institution of Dr. Subramony has received research support from NIHR01 AR076060-01A1 . The institution of Dr. Subramony has received research support from NIH2R42HD089804-04 . The institution of Dr. Subramony has received research support from NIH R01 AR056973 . The institution of Dr. Subramony has received research support from FSHD Society. The institution of Dr. Subramony has received research support from AAVANTI BIO. The institution of Dr. Subramony has received research support from COHAV FL State Dept of Health. The institution of Dr. Subramony has received research support from Avidity. The institution of Dr. Subramony has received research support from PTC. The institution of Dr. Subramony has received research support from Biohaven. The institution of Dr. Subramony has received research support from Fulcrum. The institution of Dr. Subramony has received research support from Vertex. The institution of Dr. Subramony has received research support from Arthrex.

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