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Muscle MRI in Becker Muscular Dystrophy and in Limb-girdle Muscular Dystrophy Type 2I

This study has been completed.
Sponsor:
ClinicalTrials.gov Identifier:
NCT02165358
First Posted: June 17, 2014
Last Update Posted: November 11, 2016
The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details.
Information provided by (Responsible Party):
Nicoline Løkken, Rigshospitalet, Denmark
  Purpose

The purpose of this study is to investigate the paradoxical muscle enlargement in the calves and tongue seen in patients affected by Becker muscular dystrophy and Limb-girdle muscular dystrophy type 2I.

The enlarged calves' muscle quality will be assessed primarily on the basis of the muscle structure on MRI and based on a calculation of muscle strength per cross-sectional area.The findings will be compared with results from non-affected controls.

Additionally we want to describe the tongue muscle appearance on T1-weighted MRI.


Condition
Becker Muscular Dystrophy Limb-Girdle Muscular Dystrophy Type 2I

Study Type: Observational
Study Design: Observational Model: Case Control
Time Perspective: Cross-Sectional
Official Title: Muscle MRI Study of Patients With Becker Muscular Dystrophy and Limb-girdle Muscular Dystrophy Type 2I

Resource links provided by NLM:


Further study details as provided by Nicoline Løkken, Rigshospitalet, Denmark:

Primary Outcome Measures:
  • Calves muscle quality [ Time Frame: 1 day ]

    the investigators will describe muscle quality by:

    1. MRI scans of the calves comprising of a T1-weighted MRI scan, a 3-point Dixon scan to measure the fraction of fat in the muscle, a short TI inversion recovery (STIR) scan, and a diffusion tensor imaging (DTI) scan to see the muscle fiber organization.

      The MRI scans will further be used to measure the fat free muscle cross-sectional area of the muscle groups responsible for plantar flexion and dorsi flexion respectively.

    2. Biodex muscle strength measurements: Isokinetic muscle dynamometry measurements on plantar flexion and dorsal flexion over the ankle.
    3. Calculating of muscle strength per muscle cross-sectional area


Secondary Outcome Measures:
  • MRI T1 scan of the tongue [ Time Frame: 1 day ]
    MRI T1 scan of the tongue to investigate muscle volume


Enrollment: 38
Study Start Date: June 2014
Study Completion Date: January 2015
Primary Completion Date: January 2015 (Final data collection date for primary outcome measure)
Groups/Cohorts
Patients
Patients verified with either becker muscular dystrophy or limb-girdle muscular dystrophy type 2I
Controls
Healthy controls matched for age and gender.

Detailed Description:

The purpose of this study is to investigate the paradoxical muscle enlargement seen in patients affected by Becker muscular dystrophy and Limb-girdle muscular dystrophy type 2I.

Becker and limb-girdle muscular dystrophies are both hereditary muscular disorders. Both diseases appear clinically with shrinkage and muscle weakness of the muscles around the shoulder girdle and pelvis, while muscle hypertrophy of the calves and tongue is seen.

The quality of the enlarged calves has to the investigators knowledge not previously been described, and their hypothesis is that the normal correlation between muscle strength and muscle cross-section is absent in the calf muscles. Thus the investigators hypothesis suggests that there is no actual hypertrophy of the calf muscles, but rather a pseudohypertrofy with reduced force per cross-sectional area. With a picture of increased fat infiltration and disorganized muscle fibers.

The enlarged calves' muscle quality will be assessed primarily on the basis of the muscle structure on MRI (assessed on both T1 imaging, 3-point Dixon scans, and diffusion tension imaging), and based on a calculation of muscle strength per cross-sectional area. Isokinetic muscle dynamometry (Biodex-4) will be used to measure muscle strength. The findings will be compared with results from non-affected controls.

Additionally the investigators want to describe the tongue muscle appearance on T1-weighted MRI.

  Eligibility

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Ages Eligible for Study:   18 Years to 80 Years   (Adult, Senior)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   Yes
Sampling Method:   Non-Probability Sample
Study Population
Danish patients genetically verified with either becker muscular dystrophy or limb-girdle muscular dystrophy type 2I
Criteria

Inclusion Criteria:

  • Danish patients genetically verified with either becker muscular dystrophy or limb-girdle muscular dystrophy type 2I
  • calves enlargement

Exclusion Criteria:

  • Patients who have metal implants in the body, for instance surgical clips, cochlear implants, pacemaker etc.
  • pregnant or breast-feeding patients
  • patients who are claustrophobic
  • atrophic calves
  Contacts and Locations
Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT02165358


Locations
Denmark
Neuromuscular Research Unit, Rigshospitalet
Copenhagen, Denmark, 2100
Sponsors and Collaborators
Rigshospitalet, Denmark
Investigators
Principal Investigator: Nicoline Løkken, Bsc Neuromuscular Research Unit, Rigshospitalet, Copenhagen
  More Information

Responsible Party: Nicoline Løkken, Bsc, Rigshospitalet, Denmark
ClinicalTrials.gov Identifier: NCT02165358     History of Changes
Other Study ID Numbers: H-2-2014-035 (MRI BMD/LGMD2I)
First Submitted: June 11, 2014
First Posted: June 17, 2014
Last Update Posted: November 11, 2016
Last Verified: November 2016

Keywords provided by Nicoline Løkken, Rigshospitalet, Denmark:
BMD
LGMD2I
Calf enlargement
Muscular dystrophy
MRI

Additional relevant MeSH terms:
Muscular Dystrophies
Muscular Dystrophy, Duchenne
Muscular Dystrophies, Limb-Girdle
Muscular Disorders, Atrophic
Muscular Diseases
Musculoskeletal Diseases
Neuromuscular Diseases
Nervous System Diseases
Genetic Diseases, Inborn
Genetic Diseases, X-Linked