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

Mariska M.H.P. Janssen, Jaap Harlaar, Bart Koopman, Imelda J.M. De Groot

Research output: Contribution to journalArticleAcademicpeer-review

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.

Original languageEnglish
Article number45
JournalJournal of NeuroEngineering and Rehabilitation
Volume14
Issue number1
DOIs
Publication statusPublished - 2017

Cite this

@article{c321acabaf5f42a6bc0ebc2105d05bdf,
title = "Dynamic arm study: Quantitative description of upper extremity function and activity of boys and men with duchenne muscular dystrophy",
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.",
keywords = "3D motion analysis, Duchenne muscular dystrophy, Muscle torque, Muscle ultrasound, Surface electromyography, Upper limb",
author = "Janssen, {Mariska M.H.P.} and Jaap Harlaar and Bart Koopman and {De Groot}, {Imelda J.M.}",
year = "2017",
doi = "10.1186/s12984-017-0259-5",
language = "English",
volume = "14",
journal = "Journal of NeuroEngineering and Rehabilitation",
issn = "1743-0003",
publisher = "BioMed Central",
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Dynamic arm study : Quantitative description of upper extremity function and activity of boys and men with duchenne muscular dystrophy. / Janssen, Mariska M.H.P.; Harlaar, Jaap; Koopman, Bart; De Groot, Imelda J.M.

In: Journal of NeuroEngineering and Rehabilitation, Vol. 14, No. 1, 45, 2017.

Research output: Contribution to journalArticleAcademicpeer-review

TY - JOUR

T1 - Dynamic arm study

T2 - Quantitative description of upper extremity function and activity of boys and men with duchenne muscular dystrophy

AU - Janssen, Mariska M.H.P.

AU - Harlaar, Jaap

AU - Koopman, Bart

AU - De Groot, Imelda J.M.

PY - 2017

Y1 - 2017

N2 - 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.

AB - 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.

KW - 3D motion analysis

KW - Duchenne muscular dystrophy

KW - Muscle torque

KW - Muscle ultrasound

KW - Surface electromyography

KW - Upper limb

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U2 - 10.1186/s12984-017-0259-5

DO - 10.1186/s12984-017-0259-5

M3 - Article

VL - 14

JO - Journal of NeuroEngineering and Rehabilitation

JF - Journal of NeuroEngineering and Rehabilitation

SN - 1743-0003

IS - 1

M1 - 45

ER -