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Published in: Journal of NeuroEngineering and Rehabilitation 1/2017

Open Access 01-12-2017 | Research

Dynamic arm study: quantitative description of upper extremity function and activity of boys and men with duchenne muscular dystrophy

Authors: Mariska M. H. P. Janssen, Jaap Harlaar, Bart Koopman, Imelda J. M. de Groot

Published in: Journal of NeuroEngineering and Rehabilitation | Issue 1/2017

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Abstract

Background

Therapeutic management of upper extremity (UE) function of boys and men with Duchenne Muscular Dystrophy (DMD) requires sensitive and objective assessment. Therefore, we aimed to measure physiologic UE function of healthy subjects and DMD patients in different disease stages, and to evaluate the relation between these physiologic measures and functional UE scales.

Methods

Twenty-three DMD patients and twenty healthy controls (7–23 years) participated in this explorative case–control study. Maximal muscle torque, maximal and normalized surface electromyography (sEMG) amplitudes, muscle thickness, echogenicity and maximal passive and active joint angles were measured. At activity level, Brooke upper extremity rating scale and the Performance of Upper Limb (PUL) scale were used.

Results

Outcome measures related to proximal UE function could discriminate between disease stages. Increased normalized sEMG amplitudes were found in patients, even in early disease stages. Maximal active joint angles showed the strongest relation to Brooke scale (R 2  = 0.88) and PUL scale (R 2  = 0.85).

Conclusions

The decline of muscle functions precedes the decline in performance of UE activities, and therefore may play a role in early detection of UE limitations. Increased sEMG levels demonstrate that DMD patients use more of their muscle capacity compared to healthy subjects, to perform daily activities. This might result in increased fatigability. Active maximal joint angles are highly related to functional scales, so preserving the ability to use the full range of motion is important for the performance of daily activities. Close monitoring of active joint angles could therefore help in starting interventions that minimize functional UE decline in DMD patients timely.
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Metadata
Title
Dynamic arm study: quantitative description of upper extremity function and activity of boys and men with duchenne muscular dystrophy
Authors
Mariska M. H. P. Janssen
Jaap Harlaar
Bart Koopman
Imelda J. M. de Groot
Publication date
01-12-2017
Publisher
BioMed Central
Published in
Journal of NeuroEngineering and Rehabilitation / Issue 1/2017
Electronic ISSN: 1743-0003
DOI
https://doi.org/10.1186/s12984-017-0259-5

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