Abstract Details

Title Longitudinal Upper Extremity Function in FSHD Monitored by Portable 3D Motion Capture Analyses

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P3 - Poster Session 3 (5:30 PM-6:30 PM)

Poster/Presentation
Number 11-006

Background ACTIVE-WSV has been validated in Duchenne muscular dystrophy as a marker of upper extremity function but has not been directly compared to a proprietary assessment currently used in FSHD trials (RWS). Validation of ACTIVE-WSV in FSHD patients as a biomarker of disease progression could facilitate FSHD research.

Objective 1) To score upper extremity muscular function in patients with facioscapulohumeral muscular dystrophy (FSHD) using an established automated joint tracking system (ACTIVE-Workspace Volume, WSV) comparing this to the popular Reachable Workspace (RWS) as an outcome measure in FSHD patients. 2) To develop a mobile body track system (ACTIVE-FSHD) able to capture joint range of motion xyz trajectories in this population.

Design/Methods We are performing a longitudinal pilot study with 10 FSHD patients and 5 controls assessed at baseline, 3, 6, 9, and 12 months using ACTIVE-WSV, RWS, and a tailored ACTIVE-FSHD platform. The system employs a Microsoft Azure Kinect camera and development kit programmed to capture movement of the following joints: Head, neck, chest, pelvis, clavicles, shoulders, elbows and wrists. Patients are seated 7 feet from the camera and asked to perform prespecified motor tasks during which joint movements are captured and tracked.

Results We will report the results of ACTIVE-WSV scoring compared to RWS and will correlate these measures to clinical status (Ricci and Lamperti scores, FSHD-RODS, MMT) and imaging by muscle MRI. We hypothesize that ACTIVE-WSV will show improved test-retest reliability and reduced ceiling effects compared to RWS by assessing maximal function and allowing movements of the trunk and core. Ongoing development of xyz trajectory analysis using developing ACTIVE-FSHD to characterize specific compensatory motor patterns during tasks will be presented.

Conclusions ACTIVE-WSV with automated analysis is a promising mobile platform for longitudinal assessment of disease progression in FSHD. ACTIVE-FSHD may allow analyses of specific motor patterns relevant to implementation of future FSHD clinical trials.

Authors/Disclosures
Eduardo Inacio Nascimento Andrade, MD (Umass Medical School) PRESENTER	Dr. Nascimento Andrade has nothing to disclose.
Ben Ridout (LittleSeed)	No disclosure on file
Jeffrey Penka (LittleSeed inc)	No disclosure on file
Jeff Stauffer (LittleSeed)	No disclosure on file
Linda P. Lowes, PT PhD	The institution of Ms. Lowes has received research support from Sarepta Therapeutics.
Lawrence J. Hayward, MD, PhD (UMass Chan Medical School)	Dr. Hayward has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Myocea, Inc. Dr. Hayward has received personal compensation in the range of $0-$499 for serving as a Consultant for HC Wainwright. Dr. Hayward has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Fulcrum Therapeutics. The institution of Dr. Hayward has received research support from Fulcrum Therapeutics. The institution of Dr. Hayward has received research support from Solve FSHD. The institution of Dr. Hayward has received research support from Myscular Dystrophy Association. The institution of Dr. Hayward has received research support from Swan Bio Therapeutics. The institution of Dr. Hayward has received research support from NIH.