Myotonic Dystrophy 1
Conditions
Brief summary
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.
Interventions
OTHERTraining 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).
Sponsors
Fondation du Grand défi Pierre Lavoie
Cynthia Gagnon
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
SUPPORTIVE_CARE
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
20 Years to 60 Years
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Change in maximal muscle strength in the knee extensors measured by quantified muscle testing | Before and after the 12-week training program | Changes in knee extensor maximal muscle strength evaluated by quantified muscle testing with a handheld dynamometer. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Changes in functional capacity measured by the UQAM-YMCA test | Before and after the 12-week training program | Functional capacity evaluated by 18 functional tests |
| Changes in sprint capacity measured by the Running-base anaerobic sprint test | Before and after the 12-week training program | Sprint capacity changes measured by the running-base anaerobic sprint test |
| Changes in fatigue measured by the Fatigue severity scale | Before and after the 12-week training program | Fatigue questionnaire: 9-item questionnaire rated on a scale from 1 to 7 for each item. A score a higher score means more fatigue. |
| Changes in daytime sleepiness measured by the Daytime sleepiness scale (Epworth Sleepiness Scale) | Before and after the 12-week training program | 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. |
| Changes in stumbles and falls measured by the Falls efficacy scale | Before and after the 12-week training program | 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. |
| Change in 6 minute walking test disance | Before and after the 12-week training program | Walking endurance change measured by the 6 minute walking test distance. |
| Changes in life habits measured by questionnaire | Before and after the 12-week training program | 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. |
| Changes in muscle biopsy-muscle fiber size | Before and after the 12-week training program for volunteers | To assess changes in muscle fiber size |
| Changes in muscle biopsy-muscle fiber typing | Before and after the 12-week training program for volunteers | To assess changes in muscle fiber typing |
| Changes in muscle biopsy-proportion of centrally nuclated fibers | Before and after the 12-week training program for volunteers | To assess changes in muscle centrally nucleated fibers proportion |
| Changes in muscle biopsy-leucocyte infiltration | Before and after the 12-week training program for volunteers | To assess changes in muscle fiber leucocyte infiltration |
| Changes in quality of life measured by the 36-Item Short Form Survey (SF-36) | Before and after the 12-week training program | 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. |
Countries
Canada
Outcome results
None listed