Relations Between Myotonia and Fitness

Study Overview
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Brief Summary
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Investigators aimed to investigate whether training can increase fitness levels in patients with myotonia, and thereby reduce the symptom of myotonia.

Detailed Description
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Investigators aimed to investigate whether a 10-week bicycle exercise program can increase fitness levels in patients with myotonia, and thereby reduce the symptom of myotonia in everyday life. The effect on myotonia is assessed by a standardized myotonia scale with six statements concerning self-rated myotonia, and a number of performance tests that objectively describes the degree of myotonia. The effect of the training program is assessed by an incremental test, measuring maximal oxygen uptake, maximal heart rate and maximal workload. The muscle enzyme creatine kinase (CK) is measured in plasma to follow muscle injury and safety during the training.

Official Title
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Relations Between Fitness Status and the Severity of Myotonia in Patients With Congenital Myotonia

Conditions
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Congenital Myotonia

Intervention / Treatment
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* Other: Training


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Diagnosed with either Myotonia congenita or Paramyotonia congenita. Patients who have symptoms of myotonia while they are walking stairs. Exclusion Criteria: Pregnant or breastfeeding women. Physical or mental condition, which prevent participating in the study protocol or which could influence the results. Participating in other studies, which could influence the results.

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: Treatment
Allocation: Non-Randomized
Intervention Model: Parallel Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Training<br>Participants exercise 3 times a week, 30 minute, on an ergometer bike. | Other: Training<br>* 30 minutes of home based pulse watch regulated cycle-ergometer exercise, three times a week at 75% of maximal oxygen consumption.<br>|
| No Intervention: Control<br>Participants is tested with the 4 objective myotonia test and measurements of self-assessed myotonia by the Myotonia Behavior Scale is collected. | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Changes in myotonia | Changes in myotonia is assessed before and after 10 weeks of exercise training. Myotonia is measured as self-assessment of myotonia using the Myotonia Behavior Scale every day in a week before training start and every day in a week before training finish. Furthermore, myotonia is measured as changes in time climbing a 14 steps stair before and after the 10 weeks training period. | Week 0 and week 10 | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Changes in fitness | An incremental test is performed at baseline and in the end of 10 weeks training. The primary outcome is change between the two tests in maximal oxygen consumption and work load. | baseline and after week 10 | 
| Changes in creatine Kinase | Creatine Kinase (CK) is measure in plasma to follow muscle injury during the trial. | baseline, week 2, week 4, week 7 and week 10 | 
| Changes in other myotonia | Myotonia assessed by eye-open-close test, hand open-close test, and by up-and-go test. The three performance tests describe objectively the degree of myotonia. The outcome measure is changes between before and after training. | baseline and after week 10 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Congenital myotonia, Myotonia congenita, Thomsons disease, Becker type, Training, Exercise