An investigation of disease progression in adult danish patients with facioscapulohumeral muscular dystrophy. The disease progression is followed for a year with two test-days, including functional muscle tests and a MRI scan of muscles in the back and legs.
A cohort of adult danish patients with facioscapulohumeral muscular dystrophy is invited to perform a MRI scan to describe the relation between backpain and disease affection in back muscles. After one year they are re-invited to perform another MRI scan to follow the disease progression. The MRI scan is done to measure the quantity and quality of the muscles in the back, thigh and calf. Further, investigators investigate the muscle function by measuring of muscle strength in legs and in the back together with three functional tests. A walking test (6MWT), a stair test (14SST), and a chair test (5-TSTST). Self-assessment of back pain is measured by a standardized questionnaire about backpain.
Study Type
OBSERVATIONAL
Enrollment
45
MRI scan of muscles in the back and legs. Including T1, DIXON and STIR images.
Neuromuscular Research Unit, Department of Neurology, Rigshospitalet
Copenhagen, Denmark
Fat infiltration
Change in the distribution of fat infiltration in back, thigh and calves muscles seen at the MRI images.
Time frame: 1 year
Muscle structure
Muscle structure is evaluated with a four-point semi-quantitative visual scale. Two observers evaluate images independently. Changes in evaluation between 1st and 2nd scan are the outcome measure.
Time frame: 1 year
Muscle strength
Changes in muscle strength between 1st. and 2nd MRI-scan.
Time frame: 1 year
Back pain
Changes in self-assessed back-pain between 1st. and 2nd scan.
Time frame: 1 year
Muscle function
Changes in muscle function between 1st and 2nd MRI-scan, measured by a 6 minute walk test, a 14-step-stair test and a 5-times-sit-to-stand-test.
Time frame: 1 year
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