Facioscapulohumeral Muscular Dystrophy Clinical Trial
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.
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Endpoint Classification: Efficacy Study, Intervention Model: Single Group Assignment, Masking: Open Label, Primary Purpose: Treatment
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