The Effect of Aerobic Exercise, on Fitness and Functional Muscle Strength, in Patients With Muscular Dystrophy

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details. Identifier: NCT01895283
Recruitment Status : Completed
First Posted : July 10, 2013
Last Update Posted : April 6, 2016
Information provided by (Responsible Party):
Christoffer Rasmus Vissing, Rigshospitalet, Denmark

Brief Summary:

Muscular dystrophy is a group of disorders that are characterized by progressive muscle weakening and loss of muscle mass, caused by defects in muscle proteins. Muscular dystrophy is almost always inherited disorders, and so far, no curative treatments exist.

Previous studies have shown that endurance training significantly improves fitness and self-assessed muscle function in a variety of muscular dystrophies. In this study, we wish to investigate whether patients with Bethlem myopathy (a specific form of muscular dystrophy) also benefit from endurance training.

The study consists of two test days, a 10-week training period and five blood tests.

Patients will be required to train, three times per week, for 10 weeks, on a bike ergometer with a specific training intensity, under pulse rate monitoring.

The training period will be flanked by two test days, where we will determine, and compare the patients' muscle strength and fitness level, from before to after the training program. The patients conditioning level will be determined from a 15 minute cycle ergometer test and patients will undergo three functional tests to determine their functional muscle strength.

We will as a safety measure analyze blood tests before, during and after the training program for the muscle enzyme creatine kinase (an indicator of muscle damage) and through adverse effects as reported by patients during weekly telephone-consultations with the Principal investigator.

We anticipate, that Bethlem myopathy patients will have a similar rise in fitness level and functional muscle strength, as that seen in patients with other forms of muscular dystrophy, who undertake a similar training program.

Condition or disease Intervention/treatment Phase
Bethlem Myopathy Behavioral: Moderate-intensity training Not Applicable

Study Type : Interventional  (Clinical Trial)
Actual Enrollment : 7 participants
Intervention Model: Single Group Assignment
Masking: None (Open Label)
Primary Purpose: Prevention
Official Title: The Effect of a Bike Ergometer Training Program in Patients With Bethlem Myopathy
Study Start Date : September 2013
Actual Primary Completion Date : December 2014
Actual Study Completion Date : January 2015

Arm Intervention/treatment
Experimental: Moderate-intensity training
Regular supervised moderate-intensity training on a bike ergometer, three times per week, for 30 minutes a total of 10 weeks.
Behavioral: Moderate-intensity training
Training at 70% of VO2max, for 1/2 hour, three times weekly, for 10 weeks, on a bike ergometer.

Primary Outcome Measures :
  1. Maximal oxygen uptake (VO2max) [ Time Frame: after 10 week bike ergometer training program ]
    VO2max will be determined during an incremental bike test to exhaustion. VO2max is a physiological measure of physical fitness. VO2max relies on a persons ability to supply and consume oxygen ("energy") in muscle tissue.

Secondary Outcome Measures :
  1. Functional muscle strength [ Time Frame: After completion of a 10 week training program ]
    Functional muscle strength will be determined by performance in three functional tests: a five-repetition-sit-to-stand-test, a 6 minute walk test and a 14 step stair test.

  2. Muscle strength determined by dynamometry [ Time Frame: After completion of a 10 week training program ]
  3. Self-assessed improvements in muscle function [ Time Frame: After completion of a 10 week training program ]
  4. Self-assessed improvements in incidence of problems that are related to inactivity (in patients who are confined to a wheel-chair) [ Time Frame: After a 10 week training program ]

Other Outcome Measures:
  1. Safety [ Time Frame: During a 10 week training program ]
    The safety of undertaking a 10-week training program will be determined by analyzing reports of adverse effects faced during the training program, and by interpretation of Creatine Kinase levels measured before, during and after the training program.

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Ages Eligible for Study:   16 Years to 90 Years   (Child, Adult, Older Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   Yes

Inclusion Criteria:

  • Patients with Bethlem myopathy

Exclusion Criteria:

  • Patients with disorders that would confound the interpretation of the effect from training (cardiomyopathy, pregnant women, etc.)
  • Patients who are deemed mentally unfit for cooperation with the training program

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its identifier (NCT number): NCT01895283

Rigshospitalet, Neuromuscular Research Unit, section 3342
Copenhagen, Denmark, 2100
Sponsors and Collaborators
Rigshospitalet, Denmark
Principal Investigator: Christoffer R Vissing, BsC Rigshospitalet, Neuromuscular Research Unit

Responsible Party: Christoffer Rasmus Vissing, BsC, Rigshospitalet, Denmark Identifier: NCT01895283     History of Changes
Other Study ID Numbers: H-2-2013-066
First Posted: July 10, 2013    Key Record Dates
Last Update Posted: April 6, 2016
Last Verified: April 2016

Keywords provided by Christoffer Rasmus Vissing, Rigshospitalet, Denmark:
Aerobic training
Bethlem myopathy

Additional relevant MeSH terms:
Muscular Diseases
Muscular Dystrophies
Musculoskeletal Diseases
Neuromuscular Diseases
Nervous System Diseases
Joint Diseases
Muscular Disorders, Atrophic
Genetic Diseases, Inborn