Background. Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a progressive disorder where upper and lower extremities motor performances may bring participation restriction. Purpose. To document participation in adults with ARSAC and explore associations with motor performances. Method. Twenty-eight participants took part in the study. Participation was assessed using the LIFE-H. Motor performance was assessed using several outcomes including the SARA, LEMOCOT, Berg Balance Scale, 10-Meter Walk Test, and Finger-to-nose Test. Findings. Participation was significantly lower in the wheelchair user subgroup. Also, for 29 activities out of 77, at least 15% of participants reported severely disrupted participation. Participation was correlated with upper and lower limbs coordination, walking ability, balance, disease severity, and fine dexterity (Spearman r = .41–0.85, p < .03). Implications. Results showed significant participation restrictions and suggest that interventions aiming to improve or compensate upper and lower limbs functions could help to decrease disease burden.

背景。Charlevoix-Saguenay 的常染色体隐性痉挛性共济失调 (ARSACS) 是一种进行性疾病，其上肢和下肢运动表现可能会带来参与限制。目的。记录成人 ARSAC 的参与情况，并探索与运动表现的关联。方法。28 名参与者参加了这项研究。使用 LIFE-H 评估参与度。运动表现使用多种结果进行评估，包括 SARA、LEMOCOT、Berg 平衡量表、10 米步行测试和指鼻测试。发现。轮椅使用者亚组的参与度显着降低。此外，在 77 项活动中的 29 项活动中，至少 15% 的参与者报告说参与严重中断。参与与上肢和下肢协调、行走能力、平衡、疾病严重程度和良好的灵活性相关（Spearman r  = .41–0.85，p  < .03）。影响。结果显示出显着的参与限制，并表明旨在改善或补偿上肢和下肢功能的干预措施有助于减轻疾病负担。