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

NCT02165358 | Completed

• 1 other identifier: H-2-2014-035 (MRI BMD/LGMD2I)
• Study Type: observational
• Target: 38
• Locations: 1 country
• Sites: 1

Brief Summary

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.

Conditions

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

Keywords

BMD; LGMD2I; Calf enlargement; Muscular dystrophy; MRI

Outcome Measures

Primary Outcomes (1)

• Calves muscle quality

  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 Outcomes (1)

• MRI T1 scan of the tongue

  1 day

Study Arms (2)

Patients

Patients verified with either becker muscular dystrophy or limb-girdle muscular dystrophy type 2I

Controls

Healthy controls matched for age and gender.

Eligibility Criteria

• Age: 18 Years - 80 Years
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

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

You may qualify if:

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

You may not qualify if:

• 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

Sponsors & Collaborators

• Rigshospitalet, Denmark: lead

Study Sites (1)

Neuromuscular Research Unit, Rigshospitalet

Copenhagen, 2100, Denmark

Location

MeSH Terms

Conditions

Muscular Dystrophy, Duchenne; Muscular Dystrophy, Limb-Girdle, Type 2I; Muscular Dystrophies

Condition Hierarchy (Ancestors)

Muscular Disorders, Atrophic; Muscular Diseases; Musculoskeletal Diseases; Neuromuscular Diseases; Nervous System Diseases; Genetic Diseases, X-Linked; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Officials

• Nicoline Løkken, Bsc

  Neuromuscular Research Unit, Rigshospitalet, Copenhagen

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: CASE CONTROL
• Time Perspective: CROSS SECTIONAL
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Bsc

Study Record Dates

• First Submitted: June 11, 2014
• First Posted: June 17, 2014
• Study Start: June 1, 2014
• Primary Completion: January 1, 2015
• Study Completion: January 1, 2015
• Last Updated: November 11, 2016

Record last verified: 2016-11

Locations

DK (1)