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.
|Study Design:||Endpoint Classification: Efficacy Study
Intervention Model: Single Group Assignment
Masking: Open Label
Primary Purpose: Treatment
- 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 ]
- 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 ]
|Study Start Date:||January 2008|
|Study Completion Date:||July 2008|
|Primary Completion Date:||July 2008 (Final data collection date for primary outcome measure)|
Electrostimulation of the muscles of the scapular belt and the femoris quadristocks
Electrostimulation of shoulder girdle and quadriceps muscles in facioscapulohumeral muscular dystrophy patients
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.