Evolving on Wheels: One-Year of Mobility Evolution in Adults with Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay

Introduction. Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a progressive neurological disorder that leads to significant motor impairments and eventual wheelchair use in adulthood. Although previous studies have described limited mobility and participation in adult wheelchair users with ARSACS, data remains scarce. This study aimed to describe mobility evolution over one year of adult manual wheelchair users with ARSACS. Methods. Longitudinal descriptive study including manual wheelchair users with genetically confirmed ARSACS (aged 18-59) recruited from two neuromuscular clinics in the province of Quebec (Canada). Participants completed validated questionnaires assessing wheelchair skills and self-efficacy, and wore two accelerometers for seven days to measure objective mobility (self-propulsion and non-propulsion). Data were collected at baseline and one year later, and analyzed using descriptive statistics and nonparametric repeated measures ANOVA. Results. Thirty-four adult manual wheelchair users with ARSACS participated in the study, with a mean age of 46.1 years (± 8.3 years) and moderate to severe disease severity at baseline. Participants reported low manual wheelchair skills, but high self-efficacy regarding acquired skills. Over one year, objective mobility measures showed a non-significant decrease in distance traveled, number of bouts and bout length. Differences in objective mobility measures according to age and sex were observed, but were non-statistically significant. Conclusion. This study represents a first step towards a better understanding of wheelchair mobility of adults with ARSACS. These findings have the potential to help improve their rehabilitation process. Keywords. ARSACS, manual wheelchair, actimetry, wheelchair skills, self-efficacy, mobility.