Abstract
Neuromuscular disorders can limit motor function and decrease independence. Intuitively, muscle strength affects physical function by powering movement, but joint contractures also limit physical function by limiting range of motion (ROM). In the COL6-related dystrophies (COL6-RDs) muscle weakness and progressive joint contractures occur together. Although goniometry is the current standard for measuring ROM, it is not a sensitive tool for longitudinal monitoring of contractures. Previous studies have adapted a 3D workspace as a functional measure with clinically meaningful results.
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