Electrostimulation of Shoulder Girdle and Quadriceps Muscles in Facioscapulohumeral Muscular Dystrophy Patients

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.

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.

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