Effects of a Multiple Component Training Program on Muscles in Adults With Myotonic Dystrophy Type 1

Effects of a Multiple Component Training Program on Muscles, Maximal Muscle Strength, Endurance and Functional Performance in Adults With Myotonic Dystrophy Type 1: A Pilot Study

A strength and endurance training program was conducted in adults with myotonic dystrophy type 1 (DM1). Participants underwent a 12-week/18-session supervised training program consisted of 6 exercises: elbow flexion/extension, shoulder horizontal adduction, leg press, and knee flexion/extension. To offer a complete training program aimed at improving function it was divided: the first 6 weeks were dedicated to strength-training, whereas the following weeks focused on endurance-training. To evaluate the effects of the training program, participants completed maximal strength, endurance and functional evaluations. Volunteers could also add a muscle biopsy at the beginning and the end of the program to evaluate physiological parameters.

Study Overview

• Status: Completed
• Conditions: Myotonic Dystrophy 1
• Intervention / Treatment: Other: Training program

• Study Type: Interventional
• Enrollment (Actual): 11
• Phase: Not Applicable

Contacts and Locations

Study Locations

• Canada
  • Quebec
    • Saguenay, Quebec, Canada, G7X 7X2
      • Groupe de recherche interdisciplinaire sur les maladies neuromusculaires

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 20 years to 60 years (Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

Description

Inclusion Criteria:

• Adults with DM1
• Walk without technical aid
• Muscle impairment rating scale (MIRS) of 3 or 4

Exclusion Criteria:

• Severe musculoskeletal disorders
• Any medical contraindication to physical exercise
• Any medical contraindication to muscle biopsies for the biopsy volunteers

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Supportive Care
• Allocation: N/A
• Interventional Model: Single Group Assignment
• Masking: None (Open Label)

Number of Arms

1

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: Training program; 12-week strength and endurance training program	Other: Training program; 12-week, 18-session training program. To offer a complete training program aimed at improving function it was divided : the first 6 weeks were dedicated to strength-training (2 sets of 6 repetitions at 80% of 1-RM), whereas the following weeks focused on endurance-training (1 set of 25 repetitions at 40% of 1-RM).

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Change in maximal muscle strength in the knee extensors measured by quantified muscle testing	Changes in knee extensor maximal muscle strength evaluated by quantified muscle testing with a handheld dynamometer.	Before and after the 12-week training program

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Change in 6 minute walking test disance	Walking endurance change measured by the 6 minute walking test distance.	Before and after the 12-week training program
Changes in functional capacity measured by the UQAM-YMCA test	Functional capacity evaluated by 18 functional tests	Before and after the 12-week training program
Changes in sprint capacity measured by the Running-base anaerobic sprint test	Sprint capacity changes measured by the running-base anaerobic sprint test	Before and after the 12-week training program
Changes in fatigue measured by the Fatigue severity scale	Fatigue questionnaire: 9-item questionnaire rated on a scale from 1 to 7 for each item. A score a higher score means more fatigue.	Before and after the 12-week training program
Changes in daytime sleepiness measured by the Daytime sleepiness scale (Epworth Sleepiness Scale)	8-item questionnaire, rated on a scale from 0 to 3 for each item, the higher the score, the more the patient has daytime sleepiness.	Before and after the 12-week training program
Changes in stumbles and falls measured by the Falls efficacy scale	10-item questionnaire about stumbles and falls. All items are rated on a 10-point scale. A higher score means a higher fear of falling. A score over 70 means a significant fear of falling.	Before and after the 12-week training program
Changes in quality of life measured by the 36-Item Short Form Survey (SF-36)	36-item quality of life questionnaire. The final score is calculated on a total of 100 where the lower the score, the more disability. A score of 100 means no disability.	Before and after the 12-week training program
Changes in life habits measured by questionnaire	Life habits questionnaire Assessment of life habits is a 77-item questionnaire with a scale from 0 to 9 for each item. A higher score means no disability and a low score means more disability.	Before and after the 12-week training program
Changes in muscle biopsy-muscle fiber size	To assess changes in muscle fiber size	Before and after the 12-week training program for volunteers
Changes in muscle biopsy-muscle fiber typing	To assess changes in muscle fiber typing	Before and after the 12-week training program for volunteers
Changes in muscle biopsy-proportion of centrally nuclated fibers	To assess changes in muscle centrally nucleated fibers proportion	Before and after the 12-week training program for volunteers
Changes in muscle biopsy-leucocyte infiltration	To assess changes in muscle fiber leucocyte infiltration	Before and after the 12-week training program for volunteers

Collaborators and Investigators

• Sponsor: Cynthia Gagnon
• Collaborators: Fondation du Grand défi Pierre Lavoie
• Investigators: Principal Investigator: Cynthia Gagnon, Ph.D., Université de Sherbrooke

Publications and helpful links

General Publications

• Roussel MP, Morin M, Girardin M, Fortin AM, Leone M, Mathieu J, Gagnon C, Duchesne E. Training program-induced skeletal muscle adaptations in two men with myotonic dystrophy type 1. BMC Res Notes. 2019 Aug 20;12(1):526. doi: 10.1186/s13104-019-4554-z.

Study record dates

Study Major Dates

• Study Start (Actual): July 23, 2013
• Primary Completion (Actual): December 13, 2013
• Study Completion (Actual): December 13, 2013

Study Registration Dates

• First Submitted: June 21, 2019
• First Submitted That Met QC Criteria: June 26, 2019
• First Posted (Actual): June 28, 2019

Study Record Updates

• Last Update Posted (Actual): June 28, 2019
• Last Update Submitted That Met QC Criteria: June 26, 2019
• Last Verified: June 1, 2019

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Nervous System Diseases; Genetic Diseases, Inborn; Musculoskeletal Diseases; Muscular Diseases; Neuromuscular Diseases; Neurodegenerative Diseases; Muscular Disorders, Atrophic; Heredodegenerative Disorders, Nervous System; Muscular Dystrophies; Myotonic Disorders; Myotonic Dystrophy
• Other Study ID Numbers: 2013-008

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: No

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No