Log In

Forgot Password?

OR

Not a member? Continue as a nonmember.

Become a Member

By becoming a member of the AAN, you can receive exclusive information to help you at every stage of your career. Benefits include:

Join Now See All Benefits

Loading... please wait

Abstract Details

Motor Performance and Lower Extremity Fat Fraction in Myotonic Dystrophies Type 1 and Type 2, and Controls
Neuromuscular and Clinical Neurophysiology (EMG)
P5 - Poster Session 5 (11:45 AM-12:45 PM)
10-014

DM1 and DM2 are dominantly inherited muscular dystrophy primarily characterized by progressive muscle weakness, myotonia, and multisystemic features. A potential biomarker of disease severity includes MFF (%) derived from magnetic resonance imaging (MRI). However, how lower extremity MFF compares between DM1 and DM2 has not been previously examined against controls.

To characterize lower extremity muscle fat fraction (MFF) in patients with myotonic dystrophy type 1 (DM1), myotonic dystrophy type 2 (DM2), and controls. Additionally, we examine the associations between MFF and disease-specific endpoints.

Lower extremity 3T MRIs were obtained using a two-point 3D Dixon volumetric interpolated breath-hold examination protocol. Muscle volumes (15mm thickness) were segmented from the proximal, middle, and distal levels of the thigh and calf in both legs. Lower extremity MFF was correlated with six-minute walk test (6MWT), grip strength, and 15-second step test using Spearman’s correlation (ρ). The sample consisted of seven patients with DM1(43% female), seven DM2 (86% female), and four controls (50% female).

Mean age was 50±13 years for DM1, 60±13 for DM2, and 56±8 for controls. Disease duration was 19±14 for DM1 and 18±14 years for DM2. Mean MFF of the thigh was 35.7% (range: 15.3%-79.9%) for DM1, 34.4% (17.8%-58.0%) for DM2, and 13.2% (10.5%-16.6%) for controls. Mean MFF of the calf was 32.9% (range: 17.9%-70.1%) for DM1, 27.3% (21.3%-34.6%) for DM2, and 12.6% (10.9%-14.9%) for controls. Higher MFF were strongly correlated with shorter distance of 6MWT (ρ=-0.74 for thigh and ρ=-0.64 for calf, p-value<0.01), weaker grip strength (ρ=-0.77 for thigh and calf, p-value<0.001), and less steps completed (ρ=-0.78 for thigh and ρ=-0.77 for calf, p-value<0.001). MFF were also moderately correlated with disease duration (p-value>0.5).

Higher muscle fat infiltration of the lower extremities is associated with poorer physical performance and grip strength in both DM1 and DM2 groups.
Authors/Disclosures
Diana Alejandra Madrid, MS (Wake Forest School of Medicine)
PRESENTER
Miss Madrid has received personal compensation in the range of $10,000-$49,999 for serving as a Grantee with Institute of Internation Education. Miss Madrid has received personal compensation in the range of $500-$4,999 for serving as a Early Investigator Travel Award with American Society for Bone and Mineral Research. Miss Madrid has received personal compensation in the range of $0-$499 for serving as a Travel Award with American Neurological Association.
Rebecca Knapp Ms. Knapp has nothing to disclose.
Paula R. Clemens, MD (Univ of Pittsburgh/ Dept of Neurology) The institution of Dr. Clemens has received research support from NS Pharma. The institution of Dr. Clemens has received research support from ReveraGen. The institution of Dr. Clemens has received research support from Amicus. The institution of Dr. Clemens has received research support from Sanofi. The institution of Dr. Clemens has received research support from Spark. The institution of Dr. Clemens has received research support from NIH. The institution of Dr. Clemens has received research support from MDA. The institution of Dr. Clemens has received research support from FDA.
Ashley A. Weaver, PhD (Wake Forest School of Medicine) Dr. Weaver has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Elemance, LLC. Dr. Weaver has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for KLS Martin. The institution of Dr. Weaver has received research support from KLS Martin. The institution of Dr. Weaver has received research support from Toyota. The institution of Dr. Weaver has received research support from NASA. The institution of Dr. Weaver has received research support from NHTSA. The institution of Dr. Weaver has received research support from NIH. The institution of Dr. Weaver has received research support from NSF. The institution of Dr. Weaver has received research support from RCCADS.
Araya Puwanant, MD (Wake Forest University School of Medicine) The institution of Dr. Puwanant has received research support from Muscular Dystrophy Association. The institution of Dr. Puwanant has received research support from NIH/NINDS. The institution of Dr. Puwanant has received research support from NIH/NINDS.