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A 9-year follow-up study of quantitative muscle strength changes in myotonic dystrophy type 1

Gagnon Cynthia, Petitclerc Émilie, Kierkegaard Marie, Mathieu Jean, Duchesne Élise et Hébert Luc J.. (2018). A 9-year follow-up study of quantitative muscle strength changes in myotonic dystrophy type 1. Journal of Neurology, 265, (7), p. 1698-1705.

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URL officielle: https://doi.org/10.1007/s00415-018-8898-4

Résumé

Myotonic dystrophy type 1 (DM1) is a neuromuscular disorder presenting with major muscle impairments. However, few studies have addressed muscle strength progression using quantitative methods. The aims of this study were to document muscle strength changes in eight muscle groups among adults with DM1 over a 9-year period, and to compare this progression between phenotypes (adult and late-onset) and sex. Patients with a genetic diagnosis of DM1 with the late-onset or the adult phenotype were recruited at baseline through the clinical registry of the Saguenay Neuromuscular Clinic. The maximum isometric muscle strength was measured at baseline and 9 years later using a standardized protocol of quantitative muscle testing. Muscle groups included were shoulder abductors, elbow flexors/extensors, wrist extensors, hip flexors, knee flexors/extensors, and ankle dorsiflexors. For the whole group, a mean loss of 24.5–52.8% was observed over the 9-year period for all muscle groups, except for hip flexors which remained stable. Generally, men were stronger and showed a significant greater rate of decline of muscle strength than women. The adult and late-onset phenotypes taken separately also showed a significant and similar decline over the 9-year period, except for the wrist and knee extensors where muscle strength of participants with the adult phenotype decreased faster than in the late-onset phenotype. The similar rate of decline of muscle strength loss observed between phenotypes highlights the need to develop interventions to prevent this decline, even for patients with the late-onset phenotype who are often considered as mildly impaired, and therefore neglected by the rehabilitation services.

Type de document:Article publié dans une revue avec comité d'évaluation
Volume:265
Numéro:7
Pages:p. 1698-1705
Version évaluée par les pairs:Oui
Date:2018
Sujets:Sciences de la santé
Sciences de la santé > Sciences de l'activité physique et réadaptation
Sciences de la santé > Sciences de l'activité physique et réadaptation > Physiothérapie
Département, module, service et unité de recherche:Départements et modules > Département des sciences de la santé > Unité d'enseignement en physiothérapie
Mots-clés:Myotonic dystrophy, muscle strength, quantitative muscle testing, Natural history study, Dystrophie myotonique, force musculaire, tests musculaires quantitatif
Déposé le:13 mars 2019 00:19
Dernière modification:13 mars 2019 00:19
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