20 women with myotonic dystrophy type 1 (DM1) will complete a 12-week lower-limb strength training program. The training program consist 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 will be closely supervise and take place twice a week. It is hypothesize that the training program will induce muscular hypertrophy despite the genetic defect. The training program should also have positive effects on function. The participants will be evaluated at baseline, week 6, week 12, month 6 and month 9 to quantify the effects of the training program and if these effects will be maintained over time.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
SUPPORTIVE_CARE
Masking
NONE
Enrollment
12
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 will be perform between 6 and 8 maximal repetitions.
Groupe de recherche interdisciplinaire sur les maladies neuromusculaires
Saguenay, Quebec, Canada
Changes in maximal isometric muscle strength of the knee extensors
Changes in maximal isometric muscle strength of the knee extensors measure by quantify muscle testing using a handheld dynamometer
Time frame: At baseline, week 6, week 12, month 3 and month 6
Changes in maximal isometric muscle strength of the knee flexors
Changes in maximal isometric muscle strength of the knee flexors measure by quantify muscle testing using a handheld dynamometer
Time frame: At baseline and week 12
Changes in maximal isometric muscle strength of the hip flexors
Changes in maximal isometric muscle strength of the hip flexors measure by quantify muscle testing using a handheld dynamometer
Time frame: 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 measure by quantify muscle testing using a handheld dynamometer
Time frame: At baseline and week 12
Changes in maximal isometric muscle strength of the hip abductors
Changes in maximal isometric muscle strength of the hip abductors measure by quantify muscle testing using a handheld dynamometer
Time frame: 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 measure by quantify muscle testing using a handheld dynamometer
Time frame: At baseline and week 12
Changes in maximal isometric grip strength
Changes in maximal isometric muscle strength of prehension measure by quantify muscle testing using a hand grip dynamometer
Time frame: 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
Time frame: 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
Time frame: 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
Time frame: 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
Time frame: 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
Time frame: 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
Time frame: 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
Time frame: 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.
Time frame: 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.
Time frame: 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.
Time frame: 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.
Time frame: At baseline, week 12, month 6 and month 9
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.
Time frame: 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
Time frame: 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)
Time frame: 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)
Time frame: 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)
Time frame: 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
Time frame: 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)
Time frame: At baseline and week 12
Changes in the time to perform of the Timed Up and Go
Changes in the time to perform of the Timed Up and Go
Time frame: At baseline, week 6, week 12, month 6 and month 9
Changes in the time to perform the Timed stair test
Changes in the time to perform the Timed stair test
Time frame: At baseline, week 6, week 12, month 6 and month 9
Change in the score of the Brief Pain Inventory
Change in the score of the Brief Pain Inventory. The Brief Pain Inventory is a self-administer test that asses the dimensions of pain such as intensity of pain, functional incapacities, social and family repercussions, and psychological distress.
Time frame: At baseline, week 6, week 12, month 6 and month 9
Self-perceived changes in daily life
Self-perceived changes in daily life
Time frame: At week 12
Change in the number of falls
Number of falls in the last week and in the last month
Time frame: At baseline, week 12, month 6 ans month 9
Change in score of the DM1-Activ questionnaire
Change in score of the DM1-Activ questionnaire. This questionnaire asses activity and social participation with DM1 population. It contains 20 items with a maximal socre of 40 points, a lower score represent a bigger impact of the disease on activities and social participation.
Time frame: At baseline, week 6, week 12, month 6 ans month 9
Change in blood : biochemical markers (CK, Protein C reactive, leukocytes, cytokines, etc.)
Change in blood : biochemical markers (CK, Protein C reactive, leukocytes, cytokines, etc.)
Time frame: At baseline and week 12
Change in urine sample : exercise biomarkers (-Omics markers)
Change in urine sample : exercise biomarkers (-Omics markers)
Time frame: At baseline and week 12
Change in saliva sample : exercise biomarkers (-Omics markers)
Change in saliva sample : exercise biomarkers (-Omics markers)
Time frame: At baseline and week 12
Change of the center of pressure (COP) measure in static posturography : open eyes bipodal position
Change of the center of pressure (COP) in static posturography : open eyes bipodal position
Time frame: At baseline, week 6, week 12, 6 months and 9 months
Change of the center of pressure (COP) measure in static posturography : closed eyes bipodal position
Change of the center of pressure (COP) in static posturography : closed eyes bipodal position
Time frame: At baseline, week 6, week 12, 6 months and 9 months
Change of the center of pressure (COP) measure in static posturography : open eyes semi-tandem position
Change of the center of pressure (COP) in static posturography : open eyes semi-tandem position
Time frame: At baseline, week 6, week 12, 6 months and 9 months
Change of the center of pressure (COP) measure in static posturography : open eyes tandem position
Change of the center of pressure (COP) in static posturography : open eyes tandem position
Time frame: At baseline, week 6, week 12, 6 months and 9 months
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