Study of Morphology and Functional Magnetic Resonance Imaging (MRI) Muscle Patients With Muscular Dystrophy Type FSHD Benefiting a Physical Training Introduced.

NCT ID: NCT01990976

Last Updated: 2015-07-28

Basic Information

• Recruitment Status: COMPLETED
• Total Enrollment: 15 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2012-05-31
• Study Completion Date: 2015-06-30

Brief Summary

The safety of guided practice of physical activity in myopathies is increasingly accepted, including muscular dystrophies. In facioscapulohumeral dystrophy (FSHD), one of the most common muscular dystrophy, the aerobic training showed its physiological and functional efficiency without affecting the quality of life of patients. The issue of exercise therapy extended to all neuromuscular diseases, as has been rigorously analyzed, shows that the use of a training program combining endurance exercise targeted exercises and strength is even more relevant.

To complete the multidimensional assessments be managed by each team ( physiological assessments, functional tissue and quality of life) it is relevant to continue , for a descriptive study quantitative and qualitative analyzes by muscle imaging and spectroscopy (MRI and Nuclear Resonance Imaging (NMR) spectroscopy ).

Detailed Description

These non-invasive assessments already in place at the time of the initial training (FSHD1 study, NCT01116570) will be repeated in the medium (12 months) and long term (30 months) in the same laboratory NMR AIM - CEA , Institute of Myology in Paris (P Carlier ). The data obtained from NMR repeated examinations and those obtained from biochemical analyzes and additional muscle histomorphometric (cellular aging estimation of involution fibroadipose ) on biopsies already programmed (initial, 6 months and 30 months, FSHD2 study, NCT01689480) will be respectively compared to initial time to follow the evolution food and energy capacity of trained muscles. The study of biopsies provide mechanistic arguments and morphometric specific cellular response to exercise, data will be advantageously complemented by NMR specialized examinations extended to all the muscles of the thigh (target of training ) and muscle quantification whole body MRI.

Conditions

Muscular Dystrophy; Facioscapulohumeral

Study Design

• Observational Model Type: COHORT
• Study Time Perspective: PROSPECTIVE

Study Groups

FSHD training

Only the patients who have participated to the FSHD1 study (NCT01116570) and the FSHD2 study (NCT01689480) can be included in this study.

MRI

Intervention Type: DEVICE

In Paris centre, a total body NMR imaging will be done with standard T2- and T1-weighted images.

Biopsy

Intervention Type: PROCEDURE

biopsy of the vastus lateralis muscle

Interventions

MRI

In Paris centre, a total body NMR imaging will be done with standard T2- and T1-weighted images.

Intervention Type: DEVICE

Biopsy

biopsy of the vastus lateralis muscle

Intervention Type: PROCEDURE

Eligibility Criteria

Inclusion Criteria

• Included in the FSHD1 and FSHD2 studies
• Social Security regimen affiliated
• Consent form signed

Exclusion Criteria

• Severe cardiac or respiratory insufficiency
• Cardiac pacemaker
• Morbid obesity (BMI upper to 35)
• Anti platelet therapy

• Minimum Eligible Age: 18 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Association Française contre les Myopathies (AFM), Paris

OTHER

Sponsor Role: collaborator

Ministry of Health, France

OTHER_GOV

Sponsor Role: collaborator

Centre Hospitalier Universitaire de Saint Etienne

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Principal Investigators

Léonard FEASSON, MD-PhD

Role: PRINCIPAL_INVESTIGATOR

CHU de Saint-Etienne

Locations

CHU de Grenbole

Grenoble, , France

CHU de Saint-Etienne

Saint-Etienne, , France

Countries

France

Other Identifiers

2012-A00430-43

Identifier Type: OTHER

Identifier Source: secondary_id

1208050

Identifier Type: -

Identifier Source: org_study_id