Congenital Myotonia
Conditions
Keywords
Congenital myotonia, Myotonia congenita, Thomsons disease, Becker type, Training, Exercise
Brief summary
Investigators aimed to investigate whether training can increase fitness levels in patients with myotonia, and thereby reduce the symptom of myotonia.
Detailed description
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.
Interventions
OTHERTraining
30 minutes of home based pulse watch regulated cycle-ergometer exercise, three times a week at 75% of maximal oxygen consumption.
Sponsors
Grete Andersen, MD
Study design
Allocation
NON_RANDOMIZED
Intervention model
PARALLEL
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to 75 Years
Healthy volunteers
No
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.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Changes in myotonia | Week 0 and week 10 | 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. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Changes in fitness | baseline and after week 10 | 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. |
| Changes in creatine Kinase | baseline, week 2, week 4, week 7 and week 10 | Creatine Kinase (CK) is measure in plasma to follow muscle injury during the trial. |
| Changes in other myotonia | baseline and after week 10 | 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. |
Countries
Denmark
Outcome results
None listed