Abstract
Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a rare degenerative movement disorder impacting balance and mobility. Rehabilitation helps to reduce disease severity and increase the quality of life of people with ARSACS. However, rehabilitation programs require many trips to dedicated facilities, posing a significant challenge for individuals living with ARSACS. This study aimed to develop a home-based training program specific for adults with ARSACS and to document its effects on ataxia severity, balance, mobility, and participation. This non-blinded and non-randomised interventional study used a pre-post design with a control phase. The initial level of training difficulty was tailored to each participant using a standardized assessment tool. Participants trained three times a week for 12 weeks. Outcome measures included the BERG Balance Scale, Ottawa Sitting Scale, 10-meter Walk Test, 30-second Chair Stand Test, 10-Steps Test, LIFE-H, and Scale for the Assessment and Rating of Ataxia. The retrospective acceptability of the program was also assessed using Sekhon's theoretical framework of acceptability. Fourteen participants (eight walkers, 50% men) completed the program (self-reported attendance rate rate: 75-100%) and seven dropped out. All outcome measures remained stable during the control phase. Ataxia severity and participation significantly improved after the 12-week home-based training program. At an individual level, clinical improvements in standing and sitting balance were noted for almost half of the participants, particularly for non-walkers. The eight participants who assisted to the focus group reported that the program was acceptable. This study indicates that tailored home-based training is safe, acceptable, and helps to reduce ataxia severity and participation restriction for adults with ARSACS.