Aerobic Training in Patients With Congenital Myopathies

Rigshospitalet

Status: Completed

Conditions: Congenital Myopathy

Treatments: Other: Exercise on a cycle-ergometer

Study type: Interventional

Funder types: Other

Identifiers

NCT02020187

H2-2013-066

Details and patient eligibility

About

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.

Full description

10 weeks of cycle-ergometer training at home. Intensity at 70% of maximal VO2,max.

Enrollment: 16 patients

Sex: All

Ages: 18 to 75 years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• Danish patients with gene verified congenital myopathy

Exclusion criteria

• Patients who are to week to train at a cycle-ergometer for 10 weeks
• Patients who have other health issues, which can disturb the interpretation of the efficacy

Trial design

Primary purpose: Supportive Care

Allocation: Non-Randomized

Interventional model: Single Group Assignment

Masking: None (Open label)

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16 participants in 2 patient groups

Exercise

Experimental group

Description:

10 weeks of home training on a cycle-ergometer. Exercise 30 minutes every other day or at least three times a week.

Treatment:

Other: Exercise on a cycle-ergometer

Controls

No Intervention group

Description:

Controls with diagnosed congenital myopathy. Subjects are tested two times on a cycle ergometer. There will be ten weeks between the tests. In between tests the subjects are living life as usual without any interventions.