Continuous Measurement of the Activity for Clinical Evaluation at Home, for Non-ambulant Neuromuscular Patients

Study Overview
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Brief Summary
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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.

Detailed Description
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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.

Official Title
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Continuous Measurement of the Activity for Clinical Evaluation at Home, for Non-ambulant Neuromuscular Patients

Conditions
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Neuromuscular Disease

Intervention / Treatment
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* Other: Different standardized tasks with an accelerometer device


Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 10 Years
Maximum Age: 80 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Basic Science
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Intervention/Treatment |
| --- |
|Other: Different standardized tasks with an accelerometer device|Patients will perform tests like hand grip (MyoGrip), pinch (MyoPinch), Moviplate, box and block test of hand function, Minnesota Manual Dexterity Test. The tests will be performed with the accelerometer device worn on the wrist.|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Physical activity variables of upper limbs movement at home | A watch containing and inertial system will be worn by non-ambulant patients with neuromuscular diseases at home for 14 days. Physical activity variables will be deduced from these measurements. | each day for 14 days | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Physical activity variables of upper limbs movement in standardized setting | A watch containing and inertial system will be worn by non-ambulant patients with neuromuscular diseases during standardized tasks. Physical activity variables will be deduced from these measurements. | at baseline | 
| Physical activity variables of upper limbs movement in standardized setting | A watch containing and inertial system will be worn by non-ambulant patients with neuromuscular diseases during standardized tasks. Physical activity variables will be deduced from these measurements. | 14 days after baseline | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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accelerometry, neuromuscular disease