Muscle Oxygenation in Effort in Neuromuscular Diseases (OXYNEMU)
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|ClinicalTrials.gov Identifier: NCT02789059|
Recruitment Status : Recruiting
First Posted : June 2, 2016
Last Update Posted : May 19, 2017
Previous studies showed modifications of muscle oxygenation parameters in muscular dystrophies du to an impairment or an absence of dystrophin.
Our study aim at assessing muscle oxygenation during effort in different neuromuscular diseases (muscular dystrophies related and not related to dystrophin, non dystrophic myopathies and motor neuron diseases) compared to a group of healthy controls. Patients and controls are invited to perform an inframaximal , standardized effort of the knee extensors by the mean of an isokinetic dynamometer. Muscle oxygenation parameters are assessed through a Near Infrared Spectroscopy (NIRS) Device.
In patients affected by dystrophin related myopathies, a muscle biopsy will be performed in order to analyse mitochondrial oxygenation parameters and mitochondrial phenotype.
Our Hypothesis is that muscle oxygenation is impaired in dystrophin related muscular dystrophies compared to other neuromuscular diseases and healthy controls because of lack of muscle capillary vessels dilatation during effort and impairment of mitochondrial function.
|Condition or disease||Intervention/treatment||Phase|
|Neuromuscular Diseases||Other: muscle oxygenation||Not Applicable|
This study is a prospective physiological study in a pathological condition (neuromuscular diseases).
5 groups of voluntary participants will be investigated:
1. 20 subjects affected by Dystrophin related muscular dystrophy (Becker Muscular Dystrophy ) 2. Subjects affected by muscular dystrophy not related to dystrophin impairment : 20 subjects affected by facioscapulohumeral dystrophy and 20 subjects affected by Limb Girdle Muscular dystrophy 3. 20 subjects affected by non-dystrophic myopathies (Congenital myopathies ) 4. 20 subjects affected by motor diseases: amyotrophic lateral sclerosis(ALS), Charcot Marie Tooth disease, Spinal Muscular Atrophy .
5. 20 healthy controls Objective: asses the muscular oxygenation modifications during a standardized effort and compare the variables between groups. Analyze the mitochondrial function and phenotype in the BMD group compared to healthy controls Outcome : Muscle Oxygenation by Near Infrared Spectroscopy parameters; oxygen consumption, Muscle Function Measure, Vignos and Brooke score, Borg scale, 6 minutes walk test, mitochondrial phenotype, mitochondrial oxygenation 3 visits : 1- inclusion 2- standardized effort protocol 3- for Becker dystrophy patients and voluntary controls, muscle biopsy of the Vastus Lateralis
|Study Type :||Interventional (Clinical Trial)|
|Estimated Enrollment :||170 participants|
|Intervention Model:||Single Group Assignment|
|Masking:||None (Open Label)|
|Official Title:||Muscle Oxygenation Modification During Effort in 4 Groups of Neuromuscular Diseases Compared to Healthy Controls, and Mitochondrial Function and Phenotype Assessment|
|Actual Study Start Date :||July 2, 2015|
|Estimated Primary Completion Date :||July 2017|
|Estimated Study Completion Date :||July 2017|
Experimental: muscle oxygenation
assesment of muscle oxygenation and gas exchanges
Other: muscle oxygenation
Subjects will be invited to perform an isokinetic effort of the knee extensors against an isokinetic dynamometer. During this effort, muscle oxygenation and oxygen consumption will be assessed with a near infrared spectroscopy device and a gas exchange measurement device. Patients affected by Becker muscular dystrophy and healthy controls will be invited to have a muscle biopsy of the vastus lateralis.
Other Name: Muscle biopsy
- muscle oxygenation [ Time Frame: on the day of first evaluation Visit V1 ]level of deoxyhemoglobin assessed with the NIRS device during the isokinetic effort of the knee extensors
- Muscle oxygenation [ Time Frame: on the day of first evaluation Visit V1 ]kinetics of the deoxyhemoglobin assessed with the NIRS device during the isokinetic effort of the knee extensors
- maximal isokinetic strength of the knee extensors [ Time Frame: on the day of first evaluation Visit V1 ]measurements of the maximal moment during a maximal effort of the knee extensors with an isokinetic dynamometer
- Gas exchange [ Time Frame: on the day of first evaluation Visit V1 ]measurements of O2 and CO2 exchanges during the isokinetic effort
- MFM score [ Time Frame: on the day of first evaluation Visit V1 ]Motor Function Measure score (in %) assessment. quantitative scale that makes it possible to measure the functional motor abilities of a person affected by a neuromuscular disease.
- 6 Minutes Walking Test (MWT) [ Time Frame: on the day of first evaluation Visit V1 ]assessment of the time performed during a 6 minutes walking test
- Vignos functional scales [ Time Frame: on the day of first evaluation Visit V1 ]assesment of the 1 to 6 Vignos score for the lower limb functional assesment
- Brooke functional scales [ Time Frame: on the day of first evaluation Visit V1 ]the 1 to 10 Brooke score for upper limb functional assesment
- Medical Research Council Muscle testing [ Time Frame: on the day of first evaluation Visit V1 ]Assesment of the Medical Research Council 1 to 5 Muscle testing score of the quadriceps muscles
- Mitochondrial phenotype [ Time Frame: at V2 at least 1 week after V1 ]Mitochondrial respiration (O2 consumption) of muscle fibers of the vastus lateralis
- Mitochondrial H2O2 production [ Time Frame: at V2 at least 1 week after V1 ]Mitochondrial H2O2 production of muscle fibers of the vastus lateralis
- kinetic of Muscle oxygenation [ Time Frame: on the day of first evaluation Visit V1 ]kinetic of level of deoxyhemoglobin during the isokinetic effort of the extensors of the knee
To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.
Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT02789059
|Contact: Vincent Tiffreau, MD||vincent.tiffereau@CHRU-LILLE.FR|
|Contact: Nicolas Olivier, PhDfirstname.lastname@example.org|
|Hôpital Amiens Nord, Service de Neurologie||Not yet recruiting|
|Contact: Philippe MERLE, MD,PhD email@example.com|
|Principal Investigator: Philippe MERLE, MD,PhD|
|CHRU de Lille, Hôpital Swyngedhauw||Recruiting|
|Contact: Vincent TIFFEREAU, MD firstname.lastname@example.org|
|Principal Investigator: Vincent TIFFREAU, MD|
|Sub-Investigator: Arnaud LACOUR, MD|
|Hôpital Sébastopol, CHU de Reims||Not yet recruiting|
|Contact: François BOYER, MD,PhD email@example.com|
|Principal Investigator: François BOYER, MD,PhD|
|Principal Investigator:||Vincent Tiffreau, MD||CHRU de LILLE|