Clinical trial NCT01990976: Study of Morphology and Functional Magnetic Resonance Imaging (MRI) Muscle Patients With Muscular Dystrophy Type FSHD Benefiting a Physical Training Introduced.
Official title: Study of Morphology and Functional Magnetic Resonance Imaging (MRI) Muscle Patients With Muscular Dystrophy Type FSHD Benefiting a Physical Training Introduced.
Study type: OBSERVATIONAL
Overall status: COMPLETED
Conditions: Muscular Dystrophy
Facioscapulohumeral
Keywords: Muscular Dystrophy
Facioscapulohumeral
Physical training
Magnetic Resonance Imaging
Muscle Biopsy
Lead sponsor: Centre Hospitalier Universitaire de Saint Etienne
Sponsor class: OTHER
Collaborators: name: Association Française contre les Myopathies (AFM), Paris
name: Ministry of Health, France
First posted: 2013-11-25
Enrollment: 15
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: Patient with facioscapulohumeral dystrophy and who have participated to the FSHD1 and FSHD2 studies

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.

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

Primary outcomes:
measure: Parameters of MRI
timeFrame: 30 months
Secondary outcomes:
measure: Muscle tissue parameters
timeFrame: 30 months
Other outcomes:
Locations:
city: Grenoble
country: France
facility: CHU de Grenbole
city: St Etienne
country: France
facility: CHU de Saint-Etienne
Study officials:
affiliation: CHU de Saint-Etienne
name: Léonard FEASSON, MD-PhD
role: PRINCIPAL_INVESTIGATOR
References: