Biomechanical and Morphological Changes in Dystrophic Muscle

Study Overview
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Brief Summary
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The loss of ability to walk in many children with DMD (Duchenne muscular Dystrophy) is a pejorative event. Biomechanical and morphological unknowledge about the loss of the walk ability in children with DMD is an obstacle in reeducative, pharmacological or surgical therapeutic targets.

Detailed Description
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The loss of ability to walk in many children with DMD (Duchenne muscular Dystrophy) is a pejorative event. Biomechanical and morphological unknowledge about the loss of the walk ability in children with DMD is an obstacle in reeducative, pharmacological or surgical therapeutic targets. We suppose that there are muscular characteristics and predictive parameters of the loss of walk ability. The identification of these potential therapeutic targets would improve the surveillance and the clinical care but would also guide future clinical and fundamental trials too.

Official Title
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Determining Biomechanical and Morphological Factors That Affect Children With Duchenne Muscular Dystrophy (DMD) Who Loss of the Ability to Walk

Conditions
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Duchenne Dystrophy Muscular

Intervention / Treatment
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* Other: No drug and no placebo were used in this study


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Young man ou woman (5 to 17 years old) with Duchenne Muscular Dystrophy (confirmed by immunohistochimy on the muscular biopsy and/or mutation in the dystrophin confirmed by molecular biology) Time more than 7 secondes to test of 10 m and/or distance less than 330 m to walk test of 6 minutes. These values are recent markers to include children with a strong risk of loss of walking ability in 2 years. Parental inform sign consent and / or child inform consent Exclusion Criteria: Recent orthopaedic surgery of lower limbs (6 months) Other chronic disease associated, which have an impact on the walking Cognitive Deficiency or behavior disorders limiting the understanding of the study Children who can benefit ATU (translarna ® or other) during the study All MRI contradications : pacemaker or neurosensory stimulator or implantable defibrillator, neurosurgical valves, cochlear implant or ferromagnetic implants near nervous structures, brace, metallic prostheses, not cooperative or agitated patients, patient claustrophobic, pregnant woman.

Ages Eligible for Study
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Minimum Age: 5 Years
Maximum Age: 17 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Other
Allocation: Non-Randomized
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Other: Duchenne muscular dystrophy children<br>No drug and no placebo were used in this study. For 2h30 (time) Measurement of leg strength using a dynamometer Measurement of Motor Function Walk test 6 minutes Walk test 10 meters Walk analysis: 3D recording of walking Muscle MRI | Other: No drug and no placebo were used in this study<br>* measures of muscular strength of legs, motor function, walk testing and analysis (3D video recording), RMI<br>* Other names: Several parameters must be performed.;|
| Other: Healthy children<br>No drug and no placebo were used in this study. For 2h30 (time) Measurement of leg strength using a dynamometer Measurement of Motor Function Walk test 6 minutes Walk test 10 meters Walk analysis: 3D recording of walking Muscle MRI | Other: No drug and no placebo were used in this study<br>* measures of muscular strength of legs, motor function, walk testing and analysis (3D video recording), RMI<br>* Other names: Several parameters must be performed.;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Determine biomechanical and morphological predictive factors of the loss of the walk ability of the children with DMD | Determine the biomechanical factors (muscular atrophy, muscular strength, muscular shrinkage) and morphological (greasy infiltration, contractile portion, muscular geometry) predictive of the loss of the walk ability of the children with DMD | 24 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Identify the muscles wasting and their implication in the loss of strength and the walking ability | Identify the muscles wasting and their implication in the loss of strength and walking. This will be made possible thanks to the predictive analysis and the evolutionary analysis of the MRI and strength before and after loss of walking ability | 24 years | 
| Biomechanical evolutionary data collected during the last 2 years of walking | Biomechanical evolutionary data collected during the last 2 years of walking by the repetition of the analysis of the walking until the loss of the walking ability. | 24 years | 
| Establish the relationship between the parameters of walking and the scrawny body morphological anomalies | Establish the relationship between the parameters of walking and the scrawny body morphological anomalies (muscular Atrophy, greasy infiltration, contractile muscular portion, three-dimensional morphological parameters) by doing correlations analysis | 24 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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DMD, morphological predictive factors, Mechanical