60
Participants
Start Date
December 15, 2023
Primary Completion Date
January 31, 2024
Study Completion Date
January 31, 2024
Strength force measurment
The grip and pinch strength of the patient will be evaluate using dedicated devices (MyoGrip and MyoPinch)
Time tests
Lower and upper limb capacities of the patients will be measured during timed tests
Motor scales
Patient's motor functional abilities will be evaluated using specific motor scales (MFM32, RULM)
Questionnaires
Patients and caregivers quality of life will be measured with different questionnaires (SMA-FRS, QOL-gNMD, SMAIS, PREM)
Accelerometry
Patients physical activity will be measured at home using accelerometer sensors
MNR
Sub-group of patients will perform an NMR imaging to evaluate the intramuscular fatty infiltration in thighs and muscle volume in thighs
Bio-impedance analysis
The patients' muscular and fatty volume will be evaluated using BIA technic (compared to MNR)
CHRU de Tours, Tours
CHU de Nantes, Nantes
CHU d'Angers, Angers
CHU de Reims, Reims
CHU de Lille, Lille
Institute of Myology, Paris
Roche Pharma AG
INDUSTRY
Institut de Myologie, France
OTHER