Characterization of muscle strength and mobility in oculopharyngeal muscular dystrophy.


Journal

Muscle & nerve
ISSN: 1097-4598
Titre abrégé: Muscle Nerve
Pays: United States
ID NLM: 7803146

Informations de publication

Date de publication:
Nov 2023
Historique:
revised: 13 09 2023
received: 27 07 2022
accepted: 19 09 2023
medline: 22 11 2023
pubmed: 18 10 2023
entrez: 18 10 2023
Statut: ppublish

Résumé

Muscle weakness, and its association with mobility limitations, has received little study in oculopharyngeal muscular dystrophy (OPMD) using quantitative and standardized assessments. The objectives of this study were to (1) document upper and lower limb muscle strength, upper limb functions, fatigue, and mobility capacities; (2) compare them with reference values and across participant age groups; and (3) explore associations between muscle strength, fatigue, and mobility capacities among adults with OPMD. Thirty-four participants were included in this cross-sectional study. The following variables were assessed: quantitative maximal isometric muscle strength, grip and pinch strength, fatigue, walking speed, walking endurance, sit-to-stand, and stair ascent and descent capacities. Muscle strength was lower for older than younger participants for five muscle groups (P < .05). Walking endurance, sit-to-stand, stairs (ascent and descent), and strength of hip flexion, grip, and pinch were below 80% of reference values in participants ≥56 y old (55.3%-78.2%). Moderate to strong correlations were found between muscle strength and mobility capacities (ρ = 0.42-0.80, P < .05), and between fatigue and either muscle strength or mobility capacities (ρ = 0.42-0.75, P < .05). This study highlights the impact of OPMD on strength, endurance, and functional capacity, among others, with patients being well below reference values even before the age of 65 y. In addition to helping health professionals to offer better clinical guidance, these results will improve clinical trial readiness. The next steps will be to assess the metrological properties of outcome measures and continue to document the disease progression rate.

Identifiants

pubmed: 37849345
doi: 10.1002/mus.27984
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

841-849

Subventions

Organisme : Fonds de Recherche du Québec - Santé
ID : career-grant funding for C.G. (no. 31011)
Organisme : Programme de soutien au développement de la mission universitaire of the Centre intégré universitaire de santé et de services sociaux du Saguenay - Lac-St-Jean

Informations de copyright

© 2023 The Authors. Muscle & Nerve published by Wiley Periodicals LLC.

Références

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Auteurs

Jean-Denis Brisson (JD)

Centre de recherche du Centre hospitalier universitaire de Sherbrooke (CRCHUS), Université de Sherbrooke, Sherbrooke, Quebec, Canada.
Faculté de médecine et des sciences de la santé, Université de Sherbrooke, Sherbrooke, Quebec, Canada.
Groupe de recherche interdisciplinaire sur les maladies neuromusculaires (GRIMN), Centre intégré universitaire de santé et de services sociaux du Saguenay-Lac-St-Jean, Saguenay, Quebec, Canada.
Centre de recherche du Centre intégré universitaire du Saguenay-Lac-St-Jean, Clinique des maladies neuromusculaires, Saguenay, Quebec, Canada.

Bernard Brais (B)

Department of Neurology, McGill University, Montreal Neurological Institute, Montreal, Quebec, Canada.

Jean Mathieu (J)

Centre de recherche du Centre hospitalier universitaire de Sherbrooke (CRCHUS), Université de Sherbrooke, Sherbrooke, Quebec, Canada.
Faculté de médecine et des sciences de la santé, Université de Sherbrooke, Sherbrooke, Quebec, Canada.
Groupe de recherche interdisciplinaire sur les maladies neuromusculaires (GRIMN), Centre intégré universitaire de santé et de services sociaux du Saguenay-Lac-St-Jean, Saguenay, Quebec, Canada.
Centre de recherche du Centre intégré universitaire du Saguenay-Lac-St-Jean, Clinique des maladies neuromusculaires, Saguenay, Quebec, Canada.

Isabelle Lessard (I)

Centre de recherche du Centre hospitalier universitaire de Sherbrooke (CRCHUS), Université de Sherbrooke, Sherbrooke, Quebec, Canada.
Faculté de médecine et des sciences de la santé, Université de Sherbrooke, Sherbrooke, Quebec, Canada.
Groupe de recherche interdisciplinaire sur les maladies neuromusculaires (GRIMN), Centre intégré universitaire de santé et de services sociaux du Saguenay-Lac-St-Jean, Saguenay, Quebec, Canada.

Valérie Gagné-Ouellet (V)

Groupe de recherche interdisciplinaire sur les maladies neuromusculaires (GRIMN), Centre intégré universitaire de santé et de services sociaux du Saguenay-Lac-St-Jean, Saguenay, Quebec, Canada.

Isabelle Côté (I)

Groupe de recherche interdisciplinaire sur les maladies neuromusculaires (GRIMN), Centre intégré universitaire de santé et de services sociaux du Saguenay-Lac-St-Jean, Saguenay, Quebec, Canada.

Cynthia Gagnon (C)

Centre de recherche du Centre hospitalier universitaire de Sherbrooke (CRCHUS), Université de Sherbrooke, Sherbrooke, Quebec, Canada.
Faculté de médecine et des sciences de la santé, Université de Sherbrooke, Sherbrooke, Quebec, Canada.
Groupe de recherche interdisciplinaire sur les maladies neuromusculaires (GRIMN), Centre intégré universitaire de santé et de services sociaux du Saguenay-Lac-St-Jean, Saguenay, Quebec, Canada.

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