Clinical Trial: 1 Year MRI Followup in Facioscapulohumeral Muscular Dystrophy

Study Status: Completed
Recruit Status: Completed
Study Type: Observational

Official Title: Disease Progression in Facioscapulohumeral Muscular Dystrophy - 1 Year MRI Follow-up

Brief Summary: 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.

Detailed Summary:

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.


Sponsor: Grete Andersen, MD

Current Primary Outcome: Fat infiltration [ Time Frame: 1 year ]

Change in the distribution of fat infiltration in back, thigh and calves muscles seen at the MRI images.


Original Primary Outcome: Same as current

Current Secondary Outcome:

  • Muscle structure [ Time Frame: 1 year ]
    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.
  • Muscle strength [ Time Frame: 1 year ]
    Changes in muscle strength between 1st. and 2nd MRI-scan.
  • Back pain [ Time Frame: 1 year ]
    Changes in self-assessed back-pain between 1st. and 2nd scan.
  • Muscle function [ Time Frame: 1 year ]
    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.


Original Secondary Outcome: Same as current

Information By: Rigshospitalet, Denmark

Dates:
Date Received: June 6, 2014
Date Started: May 2014
Date Completion:
Last Updated: May 13, 2015
Last Verified: May 2015