The use of muscle strength assessed with handheld dynamometers as a non-invasive biological marker in myotonic dystrophy type 1 patients: a multicenter study

<p>Abstract</p> <p>Background</p> <p>Myotonic dystrophy type 1 (DM1) is a multisystem disorder that demonstrates variable symptoms and rates of progression. Muscle weakness is considered one of the main problems with a clinical picture that is characterized by distal we...

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Main Authors: Vial Christophe, Saulnier Joanne, Remec Jean-François, Hébert Luc J, Puymirat Jack
Format: Article
Language:English
Published: BMC 2010-04-01
Series:BMC Musculoskeletal Disorders
Online Access:http://www.biomedcentral.com/1471-2474/11/72
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spelling doaj-8f55a4ee012a426f81b233aba8d7eabb2020-11-24T21:53:27ZengBMCBMC Musculoskeletal Disorders1471-24742010-04-011117210.1186/1471-2474-11-72The use of muscle strength assessed with handheld dynamometers as a non-invasive biological marker in myotonic dystrophy type 1 patients: a multicenter studyVial ChristopheSaulnier JoanneRemec Jean-FrançoisHébert Luc JPuymirat Jack<p>Abstract</p> <p>Background</p> <p>Myotonic dystrophy type 1 (DM1) is a multisystem disorder that demonstrates variable symptoms and rates of progression. Muscle weakness is considered one of the main problems with a clinical picture that is characterized by distal weakness of the limbs progressing to proximal weakness. The main objective of this study was to characterize the maximal strength of ankle eversion and dorsiflexion in DM1 patients. Manual and handheld dynamometer (HHD) muscle testing were also compared.</p> <p>Methods</p> <p>The maximal strength of 22 patients from Quebec (mean age = 41,1 ± 13,8) and 24 from Lyon (mean age = 41,6 ± 10,2) were compared to 16 matched controls.</p> <p>Results</p> <p>With the use of HHD, an excellent reproducibility of the torque measurements was obtained for both centers in eversion (R<sup>2 </sup>= 0,94/Quebec; 0,89/Lyon) and dorsiflexion (R<sup>2 </sup>= 0,96/Quebec; 0,90/Lyon). The differences between 3 groups of DM1 (mild, moderate, severe) and between them and controls were all statistically significant (p < 0,001). No statistical differences between sites were observed (p > 0.05). The degree of muscle strength decline in dorsiflexion (eversion) were 60% (47%), 77% (71%), and 87% (83%) for DM1 with mild, moderate, and severe impairments, respectively. The smallest mean difference between all DM1 patients taking together was 2.3 Nm, a difference about twice than the standard error of measurement. There was a strong relationship between eversion and dorsiflexion strength profiles (R<sup>2 </sup>= 0,87;Quebec/0,80;Lyon). Using a 10-point scale, manual muscle testing could not discriminate between the 3 groups of DM1 patients.</p> <p>Conclusions</p> <p>The HHD protocol showed discriminative properties suitable for multicentre therapeutic trial. The present results confirmed the capacity of quantitative muscle testing to discriminate between healthy and DM1 patients with different levels of impairments. This study is a preliminary step for the implementation of a valid, reliable and responsive clinical outcome for the measurement of muscle impairments with this population.</p> http://www.biomedcentral.com/1471-2474/11/72
collection DOAJ
language English
format Article
sources DOAJ
author Vial Christophe
Saulnier Joanne
Remec Jean-François
Hébert Luc J
Puymirat Jack
spellingShingle Vial Christophe
Saulnier Joanne
Remec Jean-François
Hébert Luc J
Puymirat Jack
The use of muscle strength assessed with handheld dynamometers as a non-invasive biological marker in myotonic dystrophy type 1 patients: a multicenter study
BMC Musculoskeletal Disorders
author_facet Vial Christophe
Saulnier Joanne
Remec Jean-François
Hébert Luc J
Puymirat Jack
author_sort Vial Christophe
title The use of muscle strength assessed with handheld dynamometers as a non-invasive biological marker in myotonic dystrophy type 1 patients: a multicenter study
title_short The use of muscle strength assessed with handheld dynamometers as a non-invasive biological marker in myotonic dystrophy type 1 patients: a multicenter study
title_full The use of muscle strength assessed with handheld dynamometers as a non-invasive biological marker in myotonic dystrophy type 1 patients: a multicenter study
title_fullStr The use of muscle strength assessed with handheld dynamometers as a non-invasive biological marker in myotonic dystrophy type 1 patients: a multicenter study
title_full_unstemmed The use of muscle strength assessed with handheld dynamometers as a non-invasive biological marker in myotonic dystrophy type 1 patients: a multicenter study
title_sort use of muscle strength assessed with handheld dynamometers as a non-invasive biological marker in myotonic dystrophy type 1 patients: a multicenter study
publisher BMC
series BMC Musculoskeletal Disorders
issn 1471-2474
publishDate 2010-04-01
description <p>Abstract</p> <p>Background</p> <p>Myotonic dystrophy type 1 (DM1) is a multisystem disorder that demonstrates variable symptoms and rates of progression. Muscle weakness is considered one of the main problems with a clinical picture that is characterized by distal weakness of the limbs progressing to proximal weakness. The main objective of this study was to characterize the maximal strength of ankle eversion and dorsiflexion in DM1 patients. Manual and handheld dynamometer (HHD) muscle testing were also compared.</p> <p>Methods</p> <p>The maximal strength of 22 patients from Quebec (mean age = 41,1 ± 13,8) and 24 from Lyon (mean age = 41,6 ± 10,2) were compared to 16 matched controls.</p> <p>Results</p> <p>With the use of HHD, an excellent reproducibility of the torque measurements was obtained for both centers in eversion (R<sup>2 </sup>= 0,94/Quebec; 0,89/Lyon) and dorsiflexion (R<sup>2 </sup>= 0,96/Quebec; 0,90/Lyon). The differences between 3 groups of DM1 (mild, moderate, severe) and between them and controls were all statistically significant (p < 0,001). No statistical differences between sites were observed (p > 0.05). The degree of muscle strength decline in dorsiflexion (eversion) were 60% (47%), 77% (71%), and 87% (83%) for DM1 with mild, moderate, and severe impairments, respectively. The smallest mean difference between all DM1 patients taking together was 2.3 Nm, a difference about twice than the standard error of measurement. There was a strong relationship between eversion and dorsiflexion strength profiles (R<sup>2 </sup>= 0,87;Quebec/0,80;Lyon). Using a 10-point scale, manual muscle testing could not discriminate between the 3 groups of DM1 patients.</p> <p>Conclusions</p> <p>The HHD protocol showed discriminative properties suitable for multicentre therapeutic trial. The present results confirmed the capacity of quantitative muscle testing to discriminate between healthy and DM1 patients with different levels of impairments. This study is a preliminary step for the implementation of a valid, reliable and responsive clinical outcome for the measurement of muscle impairments with this population.</p>
url http://www.biomedcentral.com/1471-2474/11/72
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