Validation of a measure specifically suitable to evaluate the residual function of upper limbs of non-ambulant neuromuscular patients, especially with Duchenne muscular dystrophy and spinal muscular atrophy.

This approach is of fundamental importance for clinical monitoring and preparing the future therapeutic trials.

The main objective is the continuous measurement of muscle activity in a standardized setting and in the non ambulatory patient's natural environment. This measure uses an innovative technology based on accelerometer and magneto-inertial sensors. The system is intended to measure the physical activity of patients from the extraction of data recorded by the sensors in the three space axes.

The non ambulatory patients will be included and evaluated at baseline and 14 days later. The patients wear the accelerometer device during some standard evaluations and also at home continuously for 14 days.

With this protocol the investigators want to try to find the best variable that characterizes the movement of the upper limbs of non-ambulant neuromuscular patients.

The aim of Pre-Acti is to propose a primary outcome measure that may be used in clinical monitoring of patients.

Inclusion Criteria:

• age over 10 years
• neuromuscular disease documented by genetic testing or by muscle biopsy
• not able to walk 10 meters without support
• capable of sitting upright in a wheelchair for at least 3 hours
• subject affiliated to a social security system
• subject who signed an informed consent

Exclusion Criteria:

• severe intellectual impairment limiting the comprehension of the demanded tasks
• acute neurologic, inflammatory, infectious, endocrine, orthopedic disease in the month preceding the inclusion
• surgery scheduled within 3 weeks after enrollment
• surgery at upper limbs in the three months preceding the inclusion
• pregnant or nursing women