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NCT Number: NCT02789059

Muscle Oxygenation in Effort in Neuromuscular Diseases

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.

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Key information

Conditions

Neuromuscular Diseases Amyotrophic Lateral Sclerosis Central Nervous System Diseases Congenital, Hereditary, and Neonatal Diseases and Abnormalities Genetic Diseases, Inborn Genetic Diseases, X-Linked Heredodegenerative Disorders, Nervous System Metabolic Diseases Motor Neuron Disease Muscular Diseases Muscular Disorders, Atrophic Muscular Dystrophies Muscular Dystrophy, Duchenne Musculoskeletal Diseases Myotonia Congenita Myotonic Disorders Nervous System Diseases Neurodegenerative Diseases Nutritional and Metabolic Diseases Proteostasis Deficiencies Spinal Cord Diseases TDP-43 Proteinopathies

Age range

18 year–70 year

Sex eligibility

Male

Study type

Interventional

Phase

Not applicable

Primary location

Hôpital Amiens Nord, Service de Neurologie, Amiens, France

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About this study

This study is a prospective physiological study in a pathological condition (neuromuscular diseases).

5 groups of voluntary participants will be investigated:

  • 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 .
  • 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

Who can participate

Healthy volunteers accepted: Yes

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

  • healthy subjects and
  • subjects affected by one of the fallowing neuromuscular diseases: Becker Muscular dystrophy Facioscapulohumeral dystrophy, Limb Girdle Muscular Dystrophy , Congenital Myopathy , Spinal Muscular Atrophy Charcot Marie Tooth Disease and Amyotrophic Lateral Sclerosis ,
  • able to walk
  • presenting a manual muscle testing of at Least 4/5 on the quadriceps according to the Medical research Council

Exclusion criteria

  • musculoskeletal pain of the quadriceps
  • other neurological disorders
  • Heart failure arrhythmia, uncontrolled hypertension, angina pectoris
  • dyspnoea >2 according to the NYHA
  • Peripheral artery disease
  • BMI >30kg.m-2.

Treatment and study plan

muscle oxygenation

Other

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 names: Muscle biopsy

Primary outcomes

  1. 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

  2. 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

Secondary outcomes

  1. 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

  2. Gas exchange

    Time frame: on the day of first evaluation Visit V1

    measurements of O2 and CO2 exchanges during the isokinetic effort

  3. 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.

  4. 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

  5. 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

  6. Brooke functional scales

    Time frame: on the day of first evaluation Visit V1

    the 1 to 10 Brooke score for upper limb functional assesment

  7. 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

  8. Mitochondrial phenotype

    Time frame: at V2 at least 1 week after V1

    Mitochondrial respiration (O2 consumption) of muscle fibers of the vastus lateralis

  9. Mitochondrial H2O2 production

    Time frame: at V2 at least 1 week after V1

    Mitochondrial H2O2 production of muscle fibers of the vastus lateralis

  10. 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

Sponsors and collaborators

Lead sponsor

University Hospital, Lille

Other

Registry information

Official study title

Muscle Oxygenation Modification During Effort in 4 Groups of Neuromuscular Diseases Compared to Healthy Controls, and Mitochondrial Function and Phenotype Assessment

Acronym: OXYNEMU

Important dates

Study start
2015
Primary completion
2017
Study completion
2017
First posted
Jun 2, 2016
Registry last updated
Apr 22, 2026

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

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This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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