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Limits of Postural Stability in Myotonic Dystrophy Compared to Normative Values

Limits of Postural Stability in Myotonic Dystrophy Compared to Normative Values. Mary Pautler, MPH University of Utah, Salt Lake City UT. Prevalence estimated at 1/8,000 Dominantly inherited, multisystem disorder Characterization : Progressive muscle weakness Myotonia

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Limits of Postural Stability in Myotonic Dystrophy Compared to Normative Values

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  1. Limits of Postural Stability in Myotonic Dystrophy Compared to Normative Values • Mary Pautler, MPH • University of Utah, Salt Lake City UT

  2. Prevalence estimated at 1/8,000 • Dominantly inherited, multisystem disorder • Characterization: • Progressive muscle weakness • Myotonia • Early onset cataracts • -Increased risk for falls • Limitations with mobility and gait impact quality of life Myotonic Dystrophy Type 1 (DM1)

  3. Study Design • Aim 1: Compare Limits of Stability via maximum excursion (MXE) scores for DM1 versus normative values. • Aim 2: Compare MXE scores at baseline and 3-months. • Study Visits • Baseline and 3-months • Inclusion Criteria • Age 18-60 • Genetically confirmed DM1 • Onset of symptoms after age 12 • Exclusion Criteria • Mexiletine, Coumadin or other anticoagulants • Other neuromuscular disorder • Significant trauma within one month

  4. Limits of Stability • Voluntary maximum displacement of center of gravity while maintaining a fixed base of support. • Maximum Excursion (MXE): • Measure of maximum movement potential • Illustrates limits of stability • Reduced MXE values indicate: • Reduced limits of stability • Decreased stability during weight shifting • Increased fall risk Balance and Posture, Andrew L. McDonough

  5. Anterior Computerized Posturography Posterior • NeuroCom Balance Manager System NeurCom Clinical Interpretation Guide, Computerized Dynamic Posturography

  6. Anterior Limit of Stability TracingDM1 participant Posterior

  7. Participant Demographics

  8. Results: Aim 1 • Maximum Excursion (MXE): Comparison of DM1 to Normative Values

  9. Results: Aim 2 • Maximum Excursion (MXE): Comparison of Baseline to 3-Month Visit

  10. MXE vs DF Strength (r = 0.65, p=0.004) MXE vs mDGI (r = 0.60, p=0.008) Results • MXE vs Age (r = -0.64, p=0.004)

  11. Summary • Limits of stability are significantly reduced in DM1 as compared to normal values. • No change in stability limits over 3-months. • Age, dorsiflexion strength and dynamic balance correlate with stability limits. • Evaluate additional contributing factors. • Consider limits of stability for measuring disease progression.

  12. CONTRIBUTORS / SUPPORT • Heather Hayes, PT, DPT, NCS, PhD • Man Hung, PhD • Jerry Bounsanga • Yushan Gu • Deanna DiBella, PT, MPT • Evan Pucillo, PT, DPT • Caren Trujillo, RN, MSN • Missy Dixon, PhD, MS • Russell Butterfield, MD, PhD • Nicholas Johnson, MD

  13. Horak, F. B. (2006). Postural orientation and equilibrium: what do we need to know about neural control of balance to prevent falls? Age Ageing, 35 Suppl 2, ii7-ii11

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