Effect of Aerobic Training in Patients With Oculopharyngeal Muscular Dystrophy

Study Overview
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Brief Summary
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The investigators want to investigate if patients with Oculopharyngeal Muscular Dystrophy can improve fitness by home-training on a cycle ergometer 30 minute, 3 times a week for 10 weeks. Participants will be evaluated on maximal oxygen consumption and maximal workload measured by an incremental test at baseline and at the end of the exercise period.

Official Title
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Effect of Aerobic Training in Patients With Oculopharyngeal Muscular Dystrophy

Conditions
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Oculopharyngeal Muscular Dystrophy

Intervention / Treatment
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* Behavioral: Exercise


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Danish patients with Oculopharyngeal muscular dystrophy Exclusion Criteria: Patients who are too week to train on an cycle-ergometer for 10 weeks Patients with other health issues wich confound the interpretation of the efficacy.

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 75 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Supportive Care
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Excercise<br>10 weeks of home training on a cycle-ergometer. Exercise 30 minutes every other day or at least three times a week. | Behavioral: Exercise<br>* Ten weeks of home-training on a cycle ergometer 3 times a week for 30 minutes.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Efficacy based on VO2max | Difference in VO2max measured before and after intervention | 10 weeks | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| SF-36 Questionaire | Changes in self-rated health from baseline to end of intervention | 10 weeks | 
| 6 minute walk test | Changes in walking distance from baseline to end of intervention | 10 weeks | 
| Intensity in maximal load (Watt) | Changes in maximal load (watt) in the VO2max-test from baseline to end of intervention. | 10 weeks | 
| Level of plasma creatine kinase | Marker for exercise-induced muscle damage. Taken week 0,3 and 10. | 10 weeks | 
| Level of plasma myoglobin | Marker for excercise-induced muscle damage, taken week 0, 3 and 10. | 10 weeks | 
| 6 minute walk test | Changes in walking distance in the test from baseline to end of intervention. | 10 weeks | 
| A five-time-repetition-sit-to-stand-test | Changes in time of five repetitions from baseline to end of intervention | 10 weeks | 
| A 14-step-stair-test | Changes in time completing the test from baseline to end of intervention | 10 weeks | 
| Dynamometry | Changes in external force production from baseline to end of intervention. Dynamometry are measured on hip flexion, dorsal foot flexion, plantar foot flexion, knee flexion, knee extension, and elbow flexion. | 10 weeks |