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Clinical Trial Summary

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.


Clinical Trial 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. ;


Study Design

Endpoint Classification: Efficacy Study, Intervention Model: Single Group Assignment, Masking: Open Label, Primary Purpose: Treatment


Related Conditions & MeSH terms

  • Facioscapulohumeral Muscular Dystrophy
  • Muscular Dystrophies
  • Muscular Dystrophy, Facioscapulohumeral

NCT number NCT00821548
Study type Interventional
Source Centre Hospitalier Universitaire de Nice
Contact
Status Completed
Phase N/A
Start date January 2008
Completion date July 2008

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