Evolving on Wheels: One-Year of Mobility Evolution in Adults with Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay
Bibliographic record
Abstract
Abstract 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. Highlights - Manual wheelchair users reported low wheelchair skills, but high self-efficacy - Total distance traveled, number of bouts, and bout length decreased over one year - Proportion of self-propelled objective mobility decreased over one year - Differences according to age and sex were observed, but were non-significant
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.002 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.001 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.001 | 0.000 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.000 | 0.001 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.001 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".