Difficulty in recruiting patients

Facioscapulohumeral muscular dystrophy (FSHD) is an autosomal dominant disease characterized by progressive weakness and atrophy of specific skeletal muscles. One of the major problems of patients affected by FSHD is the limitation in performing daily activities induced by the progressive muscle weakness. This sedentary lifestyle can cause a "debilitative cycle," and neuromuscular deconditioning can even aggravate the muscular deficiencies. Recent studies have indicated the safety and the effectiveness of moderate aerobic training programs in patients with FSHD. However, these training programs have limited applicability in patients with more severe muscular weakness. Artificial strength training by means of neuromuscular electrical stimulation (NMES) appears to be a promising rehabilitation strategy for FSHD patients suffering from neuromuscular disorders. Therefore we propose to investigate the feasibility, safety, and effectiveness of NMES strength training to counteract quadriceps muscle weakness in patients affected by FSHD.

NMES will be delivered to both quadriceps by means of a Kneehab® XP device, which is an innovative and clinically-proven garment-based product designed specifically to treat quadriceps muscle atrophy. The main NMES current parameters are: 50-Hz frequency, 100-400 µs pulse duration, 5:10-second on:off time. Each session will last approximately 20 minutes and will be repeated 5 days/week for a total duration of the treatment program of 3 months.

Inclusion Criteria:

• Molecular and/or clinical diagnosis for FSHD
• Vignos scale ≤ 5: Able to walk without help
• Clinically characterized by quadriceps muscle weakness but with a maximal voluntary contraction (MVC) between 8 and 16 Kg
• Follow as part of their usual care in the Clinical Physiology Department of the University Hospital of Montpellier
• Free and informed consent of the patient
• Patient affiliated or beneficiary of a social security system

Exclusion Criteria:

• Patient with co-morbidity:pathologies heart, pathologies respiratory, metabolic and endocrine -pathologies, pathologies cancer.
• Patients treated with drugs could cause muscle side effects or interfere with muscle metabolism.
• Lesions or skin inflammation at the thighs that can prevent the application of NMES
• Claustrophobic subject
• Carrier of intracranial vascular clip, cardiac pacemaker, neurostimulator, cochlear implant, or intraocular metallic foreign
• Pregnant or nursing women
• Vulnerable Persons and protected Major or unable to consent
• Patient in exclusion period relative over another protocol, or for which the annual amount of maximum compensation of € 4,500 has been reached