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

November 10, 2016 updated by: Nicoline Løkken, Rigshospitalet, Denmark

Muscle MRI Study of Patients With Becker Muscular Dystrophy and Limb-girdle Muscular Dystrophy Type 2I

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.

Study Overview

Status

Completed

Conditions

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.

Study Type

Observational

Enrollment (Actual)

38

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Locations

  • Denmark
      • Copenhagen, Denmark, 2100
        • Neuromuscular Research Unit, Rigshospitalet

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

16 years to 78 years (Adult, Older Adult)

Accepts Healthy Volunteers

No

Genders Eligible for Study

All

Sampling Method

Non-Probability Sample

Study Population

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

Description

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

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

  • Observational Models: Case-Control
  • Time Perspectives: Cross-Sectional

Cohorts and Interventions

Group / Cohort
Controls
Healthy controls matched for age and gender.
Patients
Patients verified with either becker muscular dystrophy or limb-girdle muscular dystrophy type 2I

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Measure Description
Time Frame
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
1 day

Secondary Outcome Measures

Outcome Measure
Measure Description
Time Frame
MRI T1 scan of the tongue
Time Frame: 1 day
MRI T1 scan of the tongue to investigate muscle volume
1 day

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

Rigshospitalet, Denmark

Investigators

  • Principal Investigator: Nicoline Løkken, Bsc, Neuromuscular Research Unit, Rigshospitalet, Copenhagen

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start

June 1, 2014

Primary Completion (Actual)

January 1, 2015

Study Completion (Actual)

January 1, 2015

Study Registration Dates

First Submitted

June 11, 2014

First Submitted That Met QC Criteria

June 16, 2014

First Posted (Estimate)

June 17, 2014

Study Record Updates

Last Update Posted (Estimate)

November 11, 2016

Last Update Submitted That Met QC Criteria

November 10, 2016

Last Verified

November 1, 2016

More Information

Terms related to this study

Keywords

Additional Relevant MeSH Terms

Other Study ID Numbers

  • H-2-2014-035 (MRI BMD/LGMD2I)

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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