48 participants (24 women and 24 men) with autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) will participate in 2 phases : control phase (12-week usual care) and intervention phase (12-week home-based rehabilitation program). The participants will be evaluated at baseline, week 12 (end of control phase) and week 24 (end of intervention phase) to quantify the effects of an individualized home-based rehabilitation program. Participants will also participate on a focus group at the end of the program to evaluate the acceptability of the program and the perceived changes.
Co-creation phase: Preceding the intervention, a co-creation phase involving healthcare professionals (3), patient-partners (2), and ARSACS researchers (3) will be conducted to develop the exercises for the rehabilitation program and the evaluation scale to determine the level of difficulty of each exercise assigned to participants. Intervention phase: Random sampling stratified by gender and level of indoor mobility (unassisted walking, assisted walking, wheelchair) from the Neuromuscular Clinic's registry of 48 participants (24 women and 24 men) will be conducted. * Control phase: All participants will be asked to maintain their usual activities for 12 weeks. * Intervention phase: All participants recruited to the project will complete the home-based rehabilitation program assigned to them for 12 weeks, unsupervised, 20 minutes 3 times a week. Follow-up calls by a physiotherapist will be conducted at weeks 2, 4, 6, 8, 10 and 12.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
SUPPORTIVE_CARE
Masking
NONE
Enrollment
48
X
Groupe de recherche interdisciplinaire sur les maladies neuromusculaires
Saguenay, Quebec, Canada
RECRUITINGChange in sitting balance
Measured with the Ottawa sitting scale, score range 0-40, a higher score indicates a better outcome
Time frame: Baseline, week 12, week 24
Change in balance
Measured with the Berg Balance Scale, score range 0-56, a higher score indicates better balance
Time frame: Baseline, week 12, week 24
Change in walking speed
Change in the walking speed, measured with the time to walk 10 meters at self-selected and maximum speed
Time frame: Baseline, week 12, week 24
Change in the balance confidence
Measured with the modified Activities-specific Balance Confidence (ABC) Scale, score range 0-100, 0 indicates no confidence and 100 is full confidence
Time frame: Baseline, week 12, week 24
Change in the number of falls
Number of self-reported falls in the last month
Time frame: Baseline, week 12, week 24
Change in the lower limb coordination
Number of touched targets in a 30 second period with each foot, measured with the Lower Extremity MOtor COordination Test (LEMOCOT)
Time frame: Baseline, week 12, week 24
Change in the number of sit-to-stand performed in 30 seconds
Number of complete sit-to-stand performed in 30 seconds without the help of arms
Time frame: Baseline, week 12, week 24
This platform is for informational purposes only and does not constitute medical advice. Always consult a qualified healthcare professional.
Change in the time required to ascent 10 stairs
Time required to ascent 10 stairs, measured with the 10 Stairs Ascent
Time frame: Baseline, week 12, week 24
Change in the time required to descent 10 stairs
Time required to descent 10 stairs, measured with the 10 Stairs Descent
Time frame: Baseline, week 12, week 24
Change in the life space
Measured with the Life Space Assessment (LSA), score range 0-120, a higher score indicates higher level of mobility in the next five areas: outside the bedroom, outside the house, in the neighborhood, outside of the neighborhood but in town, and outside town during the past four weeks
Time frame: Baseline, week 12, week 24
Change in the upper limb coordination
Number of finger touched targets in a 20 second period of each side, measured with the Standardized Finger-Nose Test (SFNT)
Time frame: Baseline, week 12, week 24
Change in social participation
The performance in activities of daily living, measured with the Assessment of Life Habits (LIFE-H), score range 0-9
Time frame: Baseline, week 12, week 24
Change in the Scale for the assessement and rating of ataxia (SARA)
Measured with the Scale for the assessment and rating of ataxia (SARA), score range 0-40, higher score indicates higher ataxia severity
Time frame: Baseline, week 12, week 24
Measure of the usability of the program and hardware
Measured with the French System Usability Scale (F-SUS), original score range 0-40 converted to 0-100, a SUS score above a 68 would be considered above average and anything below 68 is below average
Time frame: Week 24
Change in sleep components
The sleep components are measured with an actigraphy watch
Time frame: Week 12, week 24
Change in mobility components
The changes in mobility components are measured with an actigraphy watch
Time frame: Week 12, week 24
Change in the adductor hip muscle group spasticity
Change in the adductor hip muscle group spasticity, measured with the Modified Ashworth Scale
Time frame: Baseline, week 12, week 24
Change in the knee extensor muscle group spasticity
Change in the knee extensor muscle group spasticity, measured with the Modified Ashworth Scale
Time frame: Baseline, week 12, week 24
Change in the knee flexor muscle group spasticity
Change in the knee flexor muscle group spasticity, measured with the Modified Ashworth Scale
Time frame: Baseline, week 12, week 24
Change in the plantar flexor muscle group spasticity
Change in the plantar flexor muscle group spasticity, measured with the Modified Ashworth Scale
Time frame: Baseline, week 12, week 24
Sum of lower limb muscle spasticity
Sum of the spasticity results obtained for the adductor hip, knee extensor, knee flexor and plantar flexor muscle groups
Time frame: Baseline, week 12, week 24