Electrostimulation of Shoulder Girdle and Quadriceps Muscles in Facioscapulohumeral Muscular Dystrophy Patients - Full Text View

Sponsored by:	Centre Hospitalier Universitaire de Nice
Information provided by:	Centre Hospitalier Universitaire de Nice
ClinicalTrials.gov Identifier:	NCT00821548

Purpose

The investigators evaluated clinical tolerance, biological tolerance, feasibility and efficacy of daily electrostimulation training of shoulder girdle and quadriceps muscles in 10 patients with facioscapulohumeral muscular dystrophy, the third most common inherited myopathy.

Condition	Intervention
Facioscapulohumeral Muscular Dystrophy	Procedure: electrostimulation

MedlinePlus related topics: Muscular Dystrophy

U.S. FDA Resources

Study Type:	Interventional
Study Design:	Treatment, Open Label, Uncontrolled, Single Group Assignment, Efficacy Study

Further study details as provided by Centre Hospitalier Universitaire de Nice:

Primary Outcome Measures:

Clinical tolerance evaluated by fatigue and pain analogic scale. Biological tolerance:evaluated by measuring CK values.The feasibility:evaluated by considering monthly average length of sessions, monthly score of participation to sessions [ Time Frame: daily, 1, 3 and 5 months ] [ Designated as safety issue: No ]

Secondary Outcome Measures:

Efficacy of electrostimulation training in FSHD patients will be evaluated comparing manual muscle testing, Barré test, quantitative muscle assessment, fatigue severity scale, 6 minutes walking test before the therapy [ Time Frame: after 3 months of therapy and at the end of the study, after 5 months. ] [ Designated as safety issue: No ]

Enrollment:	9
Study Start Date:	January 2008
Study Completion Date:	July 2008
Primary Completion Date:	July 2008 (Final data collection date for primary outcome measure)

Arms	Assigned Interventions
1: Experimental Electrostimulation of the muscles of the scapular belt and the femoris quadristocks	Procedure: electrostimulation Electrostimulation of shoulder girdle and quadriceps muscles in facioscapulohumeral muscular dystrophy patients

Detailed Description:

Autosomal dominant FSHD is characterized by selective pattern of muscle involvement. Weakness and atrophy typically involve facial and shoulder girdle muscles, and progressively anterior forearm and foreleg muscles and pelvic girdle muscles.

The physiopatholgical mechanism of this disease, due to a deletion of repeated units named D4Z4 located on 4q35, is still controversial. Up to date, no curative therapy is available for these patients. We proposed in the present study to test feasibility, clinical and biological tolerance and efficacy of shoulder muscle training by electrostimulation in a group of FSHD patients. 10 patients displaying classical FSHD phenotype participate to this study consisting in daily session of shoulder girdle and quadriceps muscles electrostimulation of 23 minutes for a period of 5 months.

We evaluated: clinical tolerance by daily pain and fatigue analogic scales, biological tolerance by measuring CK; feasibility: by measuring the monthly score of participation to sessions; the efficacy by manual muscle testing, quantitative muscle assessment, fatigue severity scale.

Eligibility

Ages Eligible for Study:	18 Years to 75 Years
Genders Eligible for Study:	Both
Accepts Healthy Volunteers:	No

Criteria

Inclusion Criteria:

18 to 75 years old patient
FSHD diagnosis confirmed by molecular biology
FSHD clinical phenotype including shoulder girdle and quadriceps involvement
Patient with Vignos scale ≤ 5 (patient able to walk)
Patient with health insurance
Patient that could be followed for at least 6 months

Exclusion Criteria:

Patient with monolateral or bilateral scapular omopexia
Patient that has already had electrostimulation training
Patients with CK values > 5 time normal values
Patient that has not signed informed consent
Patient with cancer, collagenopathies or others joint diseases
Pregnancy and breast-feeding
Patient on guardianship or trusteeship.
Patient actively participating to other biomedical researches or whose exclusion period is not ended
Patient with a formal contraindication to the protocol detected by inclusion examinations

Contacts and Locations

Please refer to this study by its ClinicalTrials.gov identifier: NCT00821548

Locations

France
CHU de Nice
Nice, France

Sponsors and Collaborators

Centre Hospitalier Universitaire de Nice

More Information

Responsible Party:	( Centre Hospitalier Universitaire de Nice - Estelle MARTINEZ )
Study ID Numbers:	07-CIR-01
Study First Received:	January 12, 2009
Last Updated:	January 12, 2009
ClinicalTrials.gov Identifier:	NCT00821548
Health Authority:	France: Afssaps - French Health Products Safety Agency

Keywords provided by Centre Hospitalier Universitaire de Nice:

facioscapulohumeral muscular dystrophy
electrostimulation
training

Study placed in the following topic categories:

Muscular Dystrophy, Facioscapulohumeral
Muscular Dystrophies
Muscular Diseases
Genetic Diseases, Inborn
Neuromuscular Diseases
Musculoskeletal Diseases

Muscular Disorders, Atrophic
Facioscapulohumeral muscular dystrophy 1a
Atrophy
Muscular dystrophy
Landouzy-Dejerine muscular dystrophy

Additional relevant MeSH terms:

Nervous System Diseases

ClinicalTrials.gov processed this record on January 16, 2009