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Quantitative Motor Assessment in Myotonic Dystrophy

Published online by Cambridge University Press:  16 December 2016

J. Mathieu ,
H. Boivin  and
C.L. Richards
J. Mathieu*
Affiliation:
Neuromuscular Clinic, Centre de réadaptation en déficience physique, Jonquiére, QC, Canada Community Genomic Medicine Center, Montreal University, Montreal, QC, Canada
H. Boivin
Affiliation:
Neuromuscular Clinic, Centre de réadaptation en déficience physique, Jonquiére, QC, Canada
C.L. Richards
Affiliation:
Laval University, Quebec, QC, Canada
*
Service de Neurologie, CHU de la Milétrie 86021, Poitiers, France
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Abstract:

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Objective:

To establish baseline data, using a quantitative motor evaluation protocol, prior to a prospective longitudinal study of the natural history of muscular involvement in myotonic dystrophy (DM).

Design/Methods:

We conducted a cross-sectional study using a protocol consisting of manual muscle testing (MMT), quantitative muscle testing (QMT), and timed functional testing (TFT) on 50 definite DM patients (27 men, 23 women), aged 16 to 67 years. The relationships between MMT, QMT and TFT scores and disease duration were examined using linear regression analysis.

Results:

The muscle weakness was symmetric and the neck flexors and the distal muscles of upper and lower extremities were weaker than proximal muscles. Using MMT scores, the average strength decline was 0.95% per year and was similar for men and women. The strength decline was significantly more rapid for distal muscles than for proximal muscles. Quantitative muscle testing scores documented a strength decline per year of disease duration of 1.2-1.6% for the hip flexors and of 2.0-3.0% for the hand grip flexors.

Conclusions:

We observed significant linear relationships between the scores generated by this protocol and disease duration. These data illustrate the distal to proximal progression of muscular involvement in DM, a pattern of progression well-recognized by the clinicians. The follow-up assessment of a large DM cohort in a longitudinal study will establish whether this quantitative protocol provides sensitive measures of the disease progression.

Résumé:

RÉSUMÉ:Objectif:

Établir des données de base au moyen d'un protocole d'évaluation motrice quantitative avant d'entreprendre une étude longitudinale prospective sur l'histoire naturelle de l'atteinte musculaire dans la dystrophie myotonique (DM).

Plan et Méthodes:

Nous avons effectué une étude transversale en utilisant un protocole comprenant une évaluation musculaire manuelle (ÉMM), une évaluation musculaire quantitative (ÉMQ) et une évaluation fonctionnelle chronométrée (ÉFC) chez 50 patients atteints de DM (27 hommes et 23 femmes), âgés de 16 à 67 ans. La relation entre les scores de l'ÉMM, l'ÉMQ et l'ÉFC et la durée de la maladie a été analysée au moyen de l'analyse de régression linéaire.

Résultats:

La faiblesse musculaire était symétrique et les fléchisseurs du cou et les muscles distaux des extrémités supérieures et inférieures étaient plus faibles que les muscles proximaux. Selon les scores de l'ÉMM, la diminution moyenne de la force était de 0,95% par année, tant chez les hommes que chez les femmes. La diminution de la force des muscles distaux était significativement plus rapide que celle des muscles proximaux. Les scores de l'ÉMQ ont montré une diminution de la force de 1,2 à 1,6% au niveau des fléchisseurs de la hanche et de 2,0 à 3,0% pour les fléchisseurs des doigts, par année de durée de la maladie.

Conclusions:

Nous avons observé une relation linéaire significative entre les scores générés par ce protocole et la durée de la maladie. Ces données illustrent la progression distale à proximale de l'atteinte musculaire dans la DM, ce qui est bien connu des cliniciens. L'étude longitudinale d'une grande cohorte de patients atteints de DM établira si on peut obtenir des mesures sensibles de la progression de la maladie au moyen de ce protocole quantitatif.

Type
Original Article
Information
Canadian Journal of Neurological Sciences , Volume 30 , Issue 2 , May 2003 , pp. 129 - 136
Copyright
Copyright © The Canadian Journal of Neurological 2003

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