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Abstract Details

Muscle Ultrasonography Changes over One Year in Duchenne and Becker Muscular Dystrophy
Muscle Disease/Neuromuscular Junction
P05 - (-)
086
BACKGROUND: Few biomarkers are available to monitor progression in DMD and BMD. Muscle US is a non-invasive option. Prior studies demonstrate increased muscle echogeneity and signal homogeneity in DMD and BMD, but little is known about how these findings change over time.
DESIGN/METHODS: Eleven patients with genetically confirmed DMD and BMD were recruited at the time of their routine clinic visits and underwent US imaging of the deltoid1, biceps brachii2, vastus lateralis3 and tibialis anterior4 at clinic visits one year apart. All muscles were imaged at 6MHz with gain and compression settings held constant. The thickest portion of each muscle was selected for imaging to ensure reproducibility. Muscle thickness and muscle echogeneity (calculated by grey scale analysis using Adobe Photoshop) were recorded at both visits. The standard deviation of echogeneity was used as a measure of signal homogeneity.
RESULTS: Over one year, trends toward increased muscle signal (Mean 95.81 to 104.121, 116.83 to 116.542, 123.55 to 126.793 and 117.84 to 120.784) and homogeneity (Mean 23.84 to 22.981, 27.76 to 24.302, 28.33 to 25.643 and 28.48 to 25.974) were seen in all muscles. Additionally, there was a trend toward reduced muscle thickness (Mean 136.18 to 133.731, 145.54 to 121.552, 164.00 to 142.273 and 133.64 to 116.1824), most prominent in the lower extremities.
CONCLUSIONS: This small pilot study provides evidence to support further investigation of muscle ultrasonography to monitor changes in Duchenne and Becker muscular dystrophy. A larger study also incorporating functional Duchenne and Becker muscular dystrophy measures is needed.
Authors/Disclosures
Carolina De Jesus-Acosta, MD
PRESENTER 		No disclosure on file
Lisa Hobson-Webb, MD, FAAN (Duke University Medical Center) Dr. Hobson-Webb has received personal compensation in the range of $500-$4,999 for serving as a Consultant for NMD Therapeutics. Dr. Hobson-Webb has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion . The institution of Dr. Hobson-Webb has received research support from Abcuro. The institution of Dr. Hobson-Webb has received research support from Avidity. Dr. Hobson-Webb has received personal compensation in the range of $500-$4,999 for serving as a 好色先生al Talk - not speaker bureau with Takeda. Dr. Hobson-Webb has received personal compensation in the range of $500-$4,999 for serving as a 好色先生al Talk - not speaker bureau with CSL Behring.
Vern C. Juel, MD, FAAN (Duke University Medical Center) Dr. Juel has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Juel has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Immunovant. The institution of Dr. Juel has received research support from argenx. The institution of Dr. Juel has received research support from Alexion. The institution of Dr. Juel has received research support from Janssen. The institution of Dr. Juel has received research support from NIH Rare Diseases Network.
Patrick Vermersch (CHR de Lille) Patrick Vermersch has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Patrick Vermersch has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Merck. Patrick Vermersch has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi. Patrick Vermersch has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Patrick Vermersch has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche. Patrick Vermersch has received personal compensation in the range of $500-$4,999 for serving as a Consultant for AB Science. Patrick Vermersch has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Imcyse. Patrick Vermersch has received personal compensation in the range of $500-$4,999 for serving as a Consultant for BMS-Celgene. Patrick Vermersch has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Ad Scientiam. Patrick Vermersch has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Patrick Vermersch has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Patrick Vermersch has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Patrick Vermersch has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi. Patrick Vermersch has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Teva. Patrick Vermersch has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for La revue des microbiotes. The institution of Patrick Vermersch has received research support from Roche. The institution of Patrick Vermersch has received research support from Sanofi.