Biomechanical and Morphological Changes in Dystrophic Muscle

University Hospital, Brest50 enrolled

Overview

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.

Study Type

INTERVENTIONAL

Allocation

NON_RANDOMIZED

Purpose

OTHER

Masking

NONE

Enrollment

Conditions

Duchenne Dystrophy Muscular

Interventions

No drug and no placebo were used in this studyOTHER

measures of muscular strength of legs, motor function, walk testing and analysis (3D video recording), RMI

Eligibility

Sex: ALLMin age: 5 YearsMax age: 17 YearsHealthy volunteers:

Medical Language ↔ Plain English

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.

Locations (10)

CHRU

Brest, Brittanny, France

RECRUITING

CHRU

Lille, North, France

NOT_YET_RECRUITING

CHRU

Angers, France

Outcomes

Primary Outcomes

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

Time frame: 24 years

Secondary Outcomes

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

Time frame: 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.

Time frame: 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

Time frame: 24 years

Central Contacts

Sylvain BROCHARD, Dr

CONTACT

02 98 22 31 52 sylvain.brochard@chu-brest.fr

Data from ClinicalTrials.gov

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