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| Name | Class |
|---|---|
| Fondation du Grand défi Pierre Lavoie | UNKNOWN |
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Eleven men with myotonic dystrophy type 1 (DM1) underwent a 12-week lower-limb strength training program. The training program consisted of 3 series of 6 to 8 maximal repetitions of 5 different exercises: Leg extension, leg press, hip abduction, squat and plantar flexion. Training sessions were closely supervised and took place twice a week. It is hypothesised that the training program will induce muscular hypertrophy despite the genetic defect. The training program should also have positive effects on function. The participants were evaluated at baseline, week 6, week 12, month 6 and month 9 to see the effects of the training program and if these effects are maintained over time.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Training program | Experimental |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Training program | Other | 12-week strength training program of the lower limbs consisting of 5 different exercises: Leg extension, leg press, hip abduction, squat and plantar flexion. All exercises were performed between 6 and 8 maximal repetitions. |
| Measure | Description | Time Frame |
|---|---|---|
| Changes in maximal isometric muscle strength of the knee extensors | Changes in maximal isometric muscle strength of the knee extensors measured by quantified muscle testing using a handheld dynamometer | At baseline, week 6, week 12, month 6 and month 9 |
| Measure | Description | Time Frame |
|---|---|---|
| Changes in maximal isometric muscle strength of the knee flexors | Changes in maximal isometric muscle strength of the knee flexors measured by quantified muscle testing using a handheld dynamometer | At baseline and week 12 |
| Changes in maximal isometric muscle strength of the hip flexors |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Elise Duchesne, Ph.D. | Université du Québec à Chicoutimi | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Groupe de recherche interdisciplinaire sur les maladies neuromusculaires | Saguenay | Quebec | G7X 7X2 | Canada |
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| ID | Term |
|---|---|
| D009223 | Myotonic Dystrophy |
| ID | Term |
|---|---|
| D009136 | Muscular Dystrophies |
| D020966 | Muscular Disorders, Atrophic |
| D009135 | Muscular Diseases |
| D009140 | Musculoskeletal Diseases |
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Participants had to complete a 12-week strength training program and return to their normal activities for the following 6 months after the end of the training program
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Most outcomes where not masked, however the outcome assessor was masked for all muscle biopsy analysis: while analysing the muscle biopsy outcomes, the outcome assessor did not know if it was from baseline or from after the training program.
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Changes in maximal isometric muscle strength of the hip flexors measured by quantified muscle testing using a handheld dynamometer |
| At baseline and week 12 |
| Changes in maximal isometric muscle strength of the hip extensors | Changes in maximal isometric muscle strength of the hip extensors measured by quantified muscle testing using a handheld dynamometer | At baseline and week 12 |
| Changes in maximal isometric muscle strength of the ankle dorsiflexors | Changes in maximal isometric muscle strength of the ankle dorsiflexors measured by quantified muscle testing using a handheld dynamometer | At baseline and week 12 |
| Changes in 1-repetition maximum strength of the leg extension exercise | Changes in 1-repetition maximum strength of the leg extension exercise | At baseline, week 6 and week 12 |
| Changes in 1-repetition maximum strength of the leg press exercise | Changes in 1-repetition maximum strength of the leg press exercise | At baseline, week 6 and week 12 |
| Changes in 1-repetition maximum strength of the hip abduction exercise | Changes in 1-repetition maximum strength of the hip abduction exercise | At baseline, week 6 and week 12 |
| Changes in 1-repetition maximum strength of the squat exercise | Changes in 1-repetition maximum strength of the squat exercise | At baseline, week 6 and week 12 |
| Changes in comfortable walking speed in the 10-meter walk test | Changes in comfortable walking speed in the 10-meter walk test | At baseline, week 6, week 12, month 6 and month 9 |
| Changes in maximal walking speed in the 10-meter walk test | Changes in maximal walking speed in the 10-meter walk test | At baseline, week 6, week 12, month 6 and month 9 |
| Changes in the number of repetitions in the 30-seconds sit-to-stand test | Changes in the number of repetitions in the 30-seconds sit-to-stand test | At baseline, week 6, week 12, month 6 and month 9 |
| Changes in the score of the lower extremity functional scale | Changes in the score of the lower extremity functional scale (LEFS). The LEFS is an 80-point questionnaire with 20 items scored from 0 to 4. A score of 80 means no disfunction while a score of 0 means maximal disfunction. | At baseline, week 12, month 6 and month 9 |
| Changes in the score of the myotonic dystrophy health index | Changes in the score of the myotonic dystrophy health index (MDHI). The MDHI is a 114-item questionnaire scored from 0 to 100, where 0 means no disability and 100 means maximal disability. | At baseline, week 6, week 12, month 6 and month 9 |
| Changes in the score of the Fatigue and Daytime Sleepiness Scale | Changes in the score of the Fatigue and Daytime Sleepiness Scale (FDSS). The FDSS is a 12-item questionnaire where all questions are scored from 0 to 2. A higher score means more daytime sleepiness and fatigue. | At baseline, week 6, week 12, month 6 and month 9 |
| Changes in the Marin apathy scale | Changes in the Marin apathy scale. The Marin apathy scale is scored by the clinician where he interviews the subject and then scores an 18-item list on a scale of 1 to 4. A high score means more apathy. | At baseline, week 12, month 6 and month 9 |
| Changes in the Well-Being Manifestations Measure Scale | Changes in the Well-Being Manifestations Measure Scale (WBMMS). The WBMMS is a 25-item questionnaire where each question is scored on a scale from 1 to 5. A high score means high well-being. | At baseline, week 6, week 12 and month 6 |
| Changes in the Hospital Anxiety and Depression Scale | Changes in the Hospital Anxiety and Depression Scale (HADS). The HADS is a 14-item questionnaire with a scale from 0 to 3 for each item. A high score means high depression and anxiety. | At baseline, week 6, week 12 and month 6 |
| Changes in muscle biopsy of the vastus lateralis: muscle fiber typing | Changes in muscle biopsy of the vastus lateralis: muscle fiber typing | At baseline and week 12 |
| Changes in muscle biopsy of the vastus lateralis: muscle fiber size | Changes in muscle biopsy of the vastus lateralis: muscle fiber size (smallest diameter of the fiber) | At baseline and week 12 |
| Changes in muscle biopsy of the vastus lateralis: muscle proteomics | Changes in muscle biopsy of the vastus lateralis: muscle proteomics (protein expression) | At baseline and week 12 |
| Changes in muscle biopsy of the vastus lateralis: muscle transcriptomics | Changes in muscle biopsy of the vastus lateralis: muscle transcriptomics (RNA expression analysis) | At baseline and week 12 |
| Changes in muscle biopsy of the vastus lateralis: mitochondrial function | Changes in muscle biopsy of the vastus lateralis: mitochondrial function. Analysis of mitochondrial markers and enzymes | At baseline and week 12 |
| Changes in muscle biopsy of the vastus lateralis: Nuclear foci | Changes in muscle biopsy of the vastus lateralis: Nuclear foci (changes in accumulation of nuclear foci) | At baseline and week 12 |
| D020967 | Myotonic Disorders |
| D020271 | Heredodegenerative Disorders, Nervous System |
| D019636 | Neurodegenerative Diseases |
| D009422 | Nervous System Diseases |
| D009468 | Neuromuscular Diseases |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |