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

Study Overview
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Brief Summary
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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.

Official Title
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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

Conditions
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Myotonic Dystrophy 1

Intervention / Treatment
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* Other: Training program


Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 20 Years
Maximum Age: 60 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Supportive Care
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Training program<br>12-week strength and endurance training program | Other: Training program<br>* 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).<br>|

What is the study measuring?
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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 |