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Neuromuscul Disord. 2019 Jul;29(7):503-513. doi: 10.1016/j.nmd.2019.05.006. Epub 2019 May 23.

Longitudinal study of upper extremity reachable workspace in fascioscapulohumeral muscular dystrophy.

Author information

1
Department of Physical Medicine and Rehabilitation, University of California at Irvine School of Medicine, Irvine, CA, USA; Long Beach Veterans Affairs (VA) Medical Center, Spinal Cord Injury/Disorder Center, Long Beach, CA, USA.
2
Department of Physical Medicine and Rehabilitation, University of California at Irvine School of Medicine, Irvine, CA, USA.
3
Department of Electrical Engineering and Computer Science, University of California at Berkeley College of Engineering, Berkeley, CA, USA.
4
Department of Physical Medicine and Rehabilitation, University of California at Davis School of Medicine, Sacramento, CA, USA.
5
Department of Physical Medicine and Rehabilitation, University of California at Irvine School of Medicine, Irvine, CA, USA; Long Beach Veterans Affairs (VA) Medical Center, Geriatrics, Rehabilitation Medicine and Extended Care, Long Beach, CA, USA. Electronic address: jayjhan@uci.edu.

Abstract

Facioscapulohumeral Dystrophy (FSHD) results in slowly progressive strength impairment, especially the upper extremities. Recent discoveries regarding pathophysiology have led to exciting novel therapeutic strategies. To further facilitate drug development, improved FSHD outcome measures that are functionally-relevant and sensitive to longitudinal change will be critical. Recently, a motion sensor (Kinect)-based upper extremity outcome called 'reachable workspace' that provides a quantitative reconstruction of an individual's reachability was developed. In this study, changes in reachable workspace were tracked upwards for five-years in 18 FSHD subjects. Results show -1.63 %/year decline in total reachable workspace (p = 0.144); with most notable decline in the above-the-shoulder level quadrants (upper-lateral Q3: -9.5 %/year, p < 0.001 and upper-medial Q1: -6.8 %/ year, p = 0.063) with no significant changes in the lower quadrants (Q2, Q4). Reachable workspace declined more significantly if the subjects were challenged with 500 g wrist weights: total reachable workspace: -1.82 %/year, p = 0.039; Q1: -7.20 %/year, p = 0.041; Q3: -8.09 %/year, p = 0.001. Importantly, reachable workspace outcome was also able to distinguish subgroups in FSHD: mildly- and severely-affected with essentially unchanging reachability over years, and moderately-affected who demonstrate the most detectable changes longitudinally. The study demonstrates utility for measuring declines in upper quadrant reachability, and provides enrichment/stratification of FSHD populations most likely to show treatment effects in clinical trials.

KEYWORDS:

Facioscapulohumeral muscular dystrophy; Kinect; Longitudinal; Reachable workspace; Upper extremity function

PMID:
31345604
DOI:
10.1016/j.nmd.2019.05.006

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