Clinical Trial: Aerobic Training in Patients With Congenital Myopathies

Study Status: Completed
Recruit Status: Completed
Study Type: Interventional

Official Title: Aerobic Training in Patients With Congenital Myopathies

Brief Summary: Congenital myopathies are clinical and genetic heterogeneous disorders characterized by skeletal muscle weakness. Most patients experience muscle weakness and fatigue throughout their life. However, progression of symptoms is rare. There are no specific treatments for congenital myopathies. Training has been shown to benefit several other muscle diseases with weakness, but the defect in congenital myopathies involves contractile proteins of the sarcomere, why the effect of training is uncertain in these conditions. The investigators will therefore investigate the effect cycle-ergometer training for 30 minutes, three times weakly, for ten weeks in 15 patients with congenital myopathy. Another 5-10 congenital myopathy patients, who do not train, will serve as controls for the trained patients. The study starts and ends with a test day, where efficacy based on VO2max, performance in functional tests and a questionnaire will be assessed.

Detailed Summary: 10 weeks of cycle-ergometer training at home. Intensity at 70% of maximal VO2,max.
Sponsor: Rigshospitalet, Denmark

Current Primary Outcome: Efficacy based on VO2,max [ Time Frame: 10 weeks ]

Difference in VO2,max from baseline to end of intervention.


Original Primary Outcome: Same as current

Current Secondary Outcome:

  • Level of Creatine Kinase [ Time Frame: 10 weeks ]
    Marker for exercise-induced muscle damage. Taken week 0, 3 and 10.
  • Intensity in maximal Watt [ Time Frame: 10 weeks ]
    Changes in the maximal intensity (watt) from baseline VO2,max test to after the intervention.
  • 6 minute walk test [ Time Frame: 10 weeks ]
    Changes in the walking distance from baseline to after the intervention.
  • SF-36 questionnaire [ Time Frame: 10 weeks ]
    Changes in SF-36 questionnaire from baseline to after the intervention.
  • A timed five-repetitions-sit-to-stand-test [ Time Frame: 10 weeks ]
    Changes in time from baseline to after the intervention.
  • A timed 14-step-stair-test [ Time Frame: 10 weeks ]
    Changes in time from baseline to after the intervention.
  • Dynamometry [ Time Frame: 10 weeks ]
    Changes in external force production from baseline to after the intervention. Dynamometry measurements are done at hip flexion, dorsal foot flexion, plantar foot flexion, knee flexion, knee extension and elbow flexion.


Original Secondary Outcome: Same as current

Information By: Rigshospitalet, Denmark

Dates:
Date Received: November 28, 2013
Date Started: September 2013
Date Completion:
Last Updated: May 11, 2015
Last Verified: May 2015