Effects of a 12-week Strength Training Program in Men With Myotonic Dystrophy Type 1

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.

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: 30 years to 65 years (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: Male

Description

Inclusion Criteria:

• DM1 diagnosis must be confirmed by genetic analysis;
• Male gender, aged between 30 and 65 years old;
• Be able to walk without assistance;
• Consent of the neurologist must be given to participate in this study;
• Must reside in the Saguenay-Lac-St-Jean region;
• Subjects must be able to give their consent freely and voluntarily.

Exclusion Criteria:

• Patients with any other form of muscular dystrophy are excluded;
• Any contraindication for strenuous exercise or muscle biopsy.

Study Plan

How is the study designed?

Design Details

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

Number of Arms

1

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: Training program	Other: Training program; 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.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	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

Secondary Outcome Measures

Outcome Measure	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	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

Collaborators and Investigators

• Sponsor: Élise Duchesne
• Collaborators: Fondation du Grand défi Pierre Lavoie
• Investigators: Principal Investigator: Elise Duchesne, Ph.D., Université du Québec à Chicoutimi

Study record dates

Study Major Dates

• Study Start (Actual): July 1, 2017
• Primary Completion (Actual): June 22, 2018
• Study Completion (Actual): June 22, 2018

Study Registration Dates

• First Submitted: July 9, 2019
• First Submitted That Met QC Criteria: July 9, 2019
• First Posted (Actual): July 15, 2019

Study Record Updates

• Last Update Posted (Actual): December 15, 2021
• Last Update Submitted That Met QC Criteria: December 6, 2021
• Last Verified: December 1, 2021

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: 2017-005

Drug and device information, study documents

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