Muscle Oxygenation in Effort in Neuromuscular Diseases

NCT02789059 | Completed

• 2 other identifiers: 2013_292014-A01157-40
• Study Type: interventional
• Target: 17
• Locations: 1 country
• Sites: 3

Brief Summary

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.

Conditions

Neuromuscular Diseases

Keywords

Becker muscular dystrophy; Facioscapulohumeral dystrophy; congenital myopathy; motor neurone diseases

Outcome Measures

Primary Outcomes (2)

• muscle oxygenation

  level of deoxyhemoglobin assessed with the NIRS device during the isokinetic effort of the knee extensors

  on the day of first evaluation Visit V1

• Muscle oxygenation

  kinetics of the deoxyhemoglobin assessed with the NIRS device during the isokinetic effort of the knee extensors

  on the day of first evaluation Visit V1

Secondary Outcomes (10)

• maximal isokinetic strength of the knee extensors

  on the day of first evaluation Visit V1

• Gas exchange

  on the day of first evaluation Visit V1

• MFM score

  on the day of first evaluation Visit V1

• 6 Minutes Walking Test (MWT)

  on the day of first evaluation Visit V1

• Vignos functional scales

  on the day of first evaluation Visit V1

Study Arms (1)

muscle oxygenation

EXPERIMENTAL

assesment of muscle oxygenation and gas exchanges

Other: muscle oxygenation

Interventions

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.

Also known as: Muscle biopsy

muscle oxygenation

Eligibility Criteria

• Age: 18 Years - 70 Years
• Sex: male
• Healthy Volunteers: Yes
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• 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

You may not qualify if:

• 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.

Sponsors & Collaborators

• University Hospital, Lille: lead

Study Sites (3)

Hôpital Amiens Nord, Service de Neurologie

Amiens, France

Location

CHRU de Lille, Hôpital Swyngedhauw

Lille, France

Location

Hôpital Sébastopol, CHU de Reims

Reims, France

Location

Related Publications (2)

• Allart E, Olivier N, Hovart H, Thevenon A, Tiffreau V. Evaluation of muscle oxygenation by near-infrared spectroscopy in patients with Becker muscular dystrophy. Neuromuscul Disord. 2012 Aug;22(8):720-7. doi: 10.1016/j.nmd.2012.04.011. Epub 2012 Jun 8.

  PMID: 22683375 BACKGROUND

• Olivier N, Boissiere J, Allart E, Mucci P, Thevenon A, Daussin F, Tiffreau V. Evaluation of muscle oxygenation by near infrared spectroscopy in patients with facioscapulohumeral muscular dystrophy. Neuromuscul Disord. 2016 Jan;26(1):47-55. doi: 10.1016/j.nmd.2015.10.004. Epub 2015 Nov 11.

  PMID: 26608622 RESULT

MeSH Terms

Conditions

Neuromuscular Diseases; Muscular Dystrophy, Duchenne; Myotonia Congenita; Amyotrophic Lateral Sclerosis

Condition Hierarchy (Ancestors)

Nervous System Diseases; Muscular Dystrophies; Muscular Disorders, Atrophic; Muscular Diseases; Musculoskeletal Diseases; Genetic Diseases, X-Linked; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Myotonic Disorders; Heredodegenerative Disorders, Nervous System; Neurodegenerative Diseases; Spinal Cord Diseases; Central Nervous System Diseases; Motor Neuron Disease; TDP-43 Proteinopathies; Proteostasis Deficiencies; Metabolic Diseases; Nutritional and Metabolic Diseases

Study Officials

• Vincent Tiffreau, MD

  CHRU de LILLE

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NA
• Masking: NONE
• Purpose: DIAGNOSTIC
• Intervention Model: SINGLE GROUP
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: May 24, 2016
• First Posted: June 2, 2016
• Study Start: July 2, 2015
• Primary Completion: December 1, 2017
• Study Completion: December 1, 2017
• Last Updated: April 22, 2026

Record last verified: 2025-12

Locations

FR (3)