The primary objective of the study is to perform 2-year follow up with motor parameters evolution using instrumental assessments in patients with limb girdle muscular dystrophie, and to identify which motor parameters are sensitive to change. The secondary objectives of the study are: * to describe the changes of the parameters obtained from instrumental evaluations in comparison with the changes obtained from clinical assessments. * to characterize the muscular impairments, the biomechanical gait disorders, the standing postural control disorders, the biomechanical upper limb disorders in spatial exploration, the limitation of upper limb capacities, the fatigue, the endurance, the patients' participation to their activities and their quality of life, in comparison with a healthy paired population. * to highlight the relationships between muscular assessment parameters, biomechanical gait parameters, standing posture control and upper limb spatial exploration. * to highlight the relationships between data from instrumental assessments and data from clinical assessments. * to highlight the relationships between instrumental assessments data and clinical assessments data on one side, and features of patients (age, sex, duration since diagnosis, type of LGMD, rehabilitation in follow-up, sportive practices...) on the other side.
This is a monocentric study in which 2 sub-groups of people will be enrolled: LGMD patients and healthy volunteers. Each enrolled LGMD patient will have 5 visits during 2 years, one baseline visit and four half-yearly visits. Each enrolled healthy volunteer will have one planned visit only. The duration of enrollments will last 20 months.
Study Type
OBSERVATIONAL
Enrollment
80
Laboratoire d'analyse du mouvement, Service d'exploration fonctionnelles, Hôpital Raymond Poincaré, APHP
Garche, France
RECRUITINGChange from baseline on maximal strength
Maximal strength and muscular activation of knee extensors will be evaluated by isometric test with an isokinetic dynamometer (Biodex Medical Systems Inc., Shirley, New York, USA).
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on peak hip flexion
Hip flexion peak during gait will be evaluated with tridimensional gait analysis with an optoelectronic system ((Optitrack system, Natural Point Inc. Corvallis, OR, USA)
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on speed of center of pression during standing postural control
Speed of center of pression will be measured by force platforms (AMTI, Advanced Mechanical Technology).
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on elbow peak extension during upper limb spatial exploration
Elbow peak extension will be measured biomechanically with an optoelectronic system during upper limb spatial exploration (Optitrack system, Natural Point Inc. Corvallis, OR, USA)
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on muscular parameters
Maximal strength during isometric tests of hip, knee flexors, ankle and maximal grip strength. (Biodex Medical Systems Inc., Shirley, New York, USA) Muscular fatigue
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Joint kinematic parameters: change from baseline on gait speed
Spatio-temporal from biomechanical gait analysis: an optoelectronic system (Optitrack system, Natural Point Inc. Corvallis, OR, USA) will be used. Spatio-temporal = speed (m/s)
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Joint kinematic parameters: change from baseline on step length
Spatio-temporal from biomechanical gait analysis: an optoelectronic system (Optitrack system, Natural Point Inc. Corvallis, OR, USA) will be used.
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Joint kinematic parameters: change from baseline on cadence
Spatio-temporal from biomechanical gait analysis: an optoelectronic system (Optitrack system, Natural Point Inc. Corvallis, OR, USA) will be used.
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Joint kinematic parameters: change from baseline on step width
Spatio-temporal from biomechanical gait analysis: an optoelectronic system (Optitrack system, Natural Point Inc. Corvallis, OR, USA) will be used.
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Joint kinematic parameters: change from baseline on % single support phase of gait cycle
Spatio-temporal from biomechanical gait analysis: an optoelectronic system (Optitrack system, Natural Point Inc. Corvallis, OR, USA) will be used.
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Joint kinematic parameters: change from baseline on % swing phase of gait cycle
Spatio-temporal from biomechanical gait analysis: an optoelectronic system (Optitrack system, Natural Point Inc. Corvallis, OR, USA) will be used.
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Joint kinematic parameters: change from baseline on % support phase of gait cycle
Spatio-temporal from biomechanical gait analysis: an optoelectronic system (Optitrack system, Natural Point Inc. Corvallis, OR, USA) will be used.
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on kinetic gait parameters
Kinetic parameters will be obtained from biomechanical gait analysis with AMTI force plateforms (Advanced Mechanical Technology, Waterton, MA, USA). This includes peak of ground reaction force in antero-posterior and vertical axis.
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on standing postural control parameters: COP displacements
Parameters of stabilization and postural orientation during postural control assessment on force platforms (AMTI, Advanced Mechanical Technology), Waterton, MA, USA). Parameters of stabilization include: Mean and maximal speed of center of pression (COP) displacements.
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on standing postural control parameters: elliptic surface
Parameters of stabilization and postural orientation during postural control assessment on force platforms (AMTI, Advanced Mechanical Technology), Waterton, MA, USA). Parameters of stabilization include: Elliptic surface covering 90% of COP positions.
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on standing postural control parameters: Amplitude of COP
Parameters of stabilization and postural orientation during postural control assessment on force platforms (AMTI, Advanced Mechanical Technology), Waterton, MA, USA). Parameters of stabilization include: Amplitude of COP shift in anteroposterior and mediolateral axis.
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on standing postural control parameters: Romberg quotient
Parameters of stabilization and postural orientation during postural control assessment on force platforms (AMTI, Advanced Mechanical Technology), Waterton, MA, USA). Parameters of stabilization include: Romberg quotient.
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on standing postural orientation parameters
Parameters of stabilization and postural orientation during postural control assessment on force platforms (AMTI, Advanced Mechanical Technology), Waterton, MA, USA). The orientation parameters include: mean position of COP in anteroposterior and mediolateral axis.
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on standing postural orientation parameters: limb loading ratio
Parameters of stabilization and postural orientation during postural control assessment on force platforms (AMTI, Advanced Mechanical Technology), Waterton, MA, USA). The orientation parameters include: limb loading ratio.
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on drinking task with left and right upper limb: velocity and movement time
The spatio-temporal parameters will be obtained from biomechanical analysis with an optoelectronic system (Optitrack system, Natural Point Inc. Corvallis, OR, USA) including: Velocity and movement time (Total movement time / Peak velocity/ Mean velocity)
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on drinking task with left and right upper limb: movement strategy
The spatio-temporal parameters will be obtained from biomechanical analysis with an optoelectronic system (Optitrack system, Natural Point Inc. Corvallis, OR, USA) including: Movement strategy (Time to peak velocity / time to first peak)
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on drinking task with left and right upper limb: smoothness and coordination movement
The spatio-temporal parameters will be obtained from biomechanical analysis with an optoelectronic system (Optitrack system, Natural Point Inc. Corvallis, OR, USA) including: Smoothness and coordination movement (number of movements units / interjoint coordination)
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on drinking task with left and right upper limb: trunk displacement
The spatio-temporal parameters will be obtained from biomechanical analysis with an optoelectronic system (Optitrack system, Natural Point Inc. Corvallis, OR, USA) including: Trunk displacement
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on drinking task with left and right upper limb: angular joint
The spatio-temporal parameters will be obtained from biomechanical analysis with an optoelectronic system (Optitrack system, Natural Point Inc. Corvallis, OR, USA) including: Angular joint
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on 6 minutes walking test distance
6 minutes walking test
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on the Berg Balance Scale score
The Berg Balance Scale
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on Brooke Upper Extremity Scale score
Brooke Upper Extremity Scale score for upper extremity capacities.
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on locomotor
Questionnaires Abiloco
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on upper limb capacities
Questionnaires Abilhand
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on patient occupations
Patient occupations assessed by COPM (Canadian Occupational Performance Measure). The measurement will be from 1 (not at all performant) to 10 (very performant) as score.
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on MRC (Medical Research Council) score on flexors and extensors of lower and upper limb
Testing MRC (Medical Research Council), a scale for muscle power. The muscle scale grades muscle power on a scale of 0 to 5 in relation to the maximum expected for that muscle.
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on Fatigue Severity Scale (FSS) score
Questionnaire Fatigue Severity Scale (FSS) for fatigue assessment
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on number of fall risks
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
Change from baseline on number of physiotherapy sessions per week
Time frame: at baseline, 6 months, 12 months, 18 months and 24 months
Change from baseline on number of sports practice per week
Time frame: at baseline, 6 months, 12 months, 18 months and 24 months
Change from baseline on Individualized Neuromuscular Quality of Life Questionnaire (INQoL)
Quality of life questionnaire: INQoL
Time frame: Assessed at 6 months, 12 months, 18 months and 24 months
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