Effects and Acceptability of an Individualized Home-Based 10-Week Training Program in Adults with Myotonic Dystrophy Type 1

Article type: Research Article

Authors: Lessard, Isabelle^{a; b; e} | Gaboury, Sébastien^c | Gagnon, Cynthia^{b; d; e} | Bouchard, Kévin^c | Chapron, Kévin^c | Lavoie, Mélissa^{b; f} | Lapointe, Patrick^c | Duchesne, Elise^{a; b; e; *}

Affiliations: [a] Département des sciences de la santé, physiothérapie, Université du Québec à Chicoutimi, Québec, Canada | [b] Groupe de recherche interdisciplinaire sur les maladies neuromusculaires (GRIMN), Centre intégré universitaire de santé et de services sociaux du Saguenay–Lac-Saint-Jean, Québec, Canada | [c] Laboratoire d’Intelligence Ambiante pour la Reconnaissance d’Activités (LIARA), Université du Québec à Chicoutimi, Québec, Canada | [d] Faculté de médecine et des sciences de la santé, Université de Sherbrooke, Québec, Canada | [e] Centre de recherche Charles-Le Moyne –Saguenay-Lac-St-Jean sur les innovations en santé (CR-CSIS), Université de Sherbrooke, Québec, Canada | [f] Département des sciences de la santé, module des sciences infirmières, Université du Québec à Chicoutimi, Québec, Canada

Correspondence: [*] Correspondence to: Elise Duchesne, PT, PhD, Université du Québec à Chicoutimi, Département des sciences de la santé, 555, boulevard de l’Université, Chicoutimi (Québec) G7H 2B1, 418 545-5011 poste 6148, Québec, Canada. E-mail: [email protected].

Abstract: Background:Muscle weakness is a cardinal sign of myotonic dystrophy type 1, causing important functional mobility limitations and increasing the risk of falling. As a non-pharmacological, accessible and safe treatment for this population, strength training is an intervention of choice. Objective:To document the effects and acceptability of an individualized semi-supervised home-based exercise program on functional mobility, balance and lower limb strength, and to determine if an assistive training device has a significant impact on outcomes. Methods:This study used a pre-post test design and men with the adult form of DM1 were randomly assigned to the control or device group. The training program was performed three times a week for 10 weeks and included three exercises (sit-to-stand, squat, and alternated lunges). Outcome measures included maximal isometric muscle strength, 10-Meter Walk Test, Mini-BESTest, 30-Second Chair Stand Test and 6-minute walk test. Results:No outcome measures showed a significant difference, except for the strength of the knee flexors muscle group between the two assessments. All participants improved beyond the standard error of measurement in at least two outcome measures. The program and the device were well accepted and all participants reported many perceived improvements at the end of the program. Conclusions:Our results provide encouraging data on the effects and acceptability of a home-based training program for men with the adult form of DM1. These programs would reduce the financial burden on the health system while improving the clinical services offered to this population.

Keywords: Myotonic dystrophy, exercise, muscle strength, walking, postural balance, patient acceptance of health care

DOI: 10.3233/JND-200570

Journal: Journal of Neuromuscular Diseases, vol. 8, no. 1, pp. 137-149, 2021