Autosomal recessive spastic ataxia of Charlevoix-Saguenay: upper extremity aptitudes, functional independence and social participation
Bibliographic record
Abstract
Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is an early-onset ataxia with pyramidal, cerebellar and distal neuropathic involvement. A cross-sectional study with 24 subjects was carried out to examine upper extremity aptitudes in ARSACS by comparing scores between two age groups (<40 and > or =40) and with reference values. Upper extremity aptitude measures included coordination, gross and fine dexterity, upper extremity strength, muscle tone, kinaesthetic sensitivity and global upper extremity performance. Functional aptitudes were evaluated with part of the Functional Independence Measure (FIM) and social participation was measured with the Assessment of Life Habits. Data showed significant differences between younger and older participants for coordination, gross and fine finger dexterity, pinch strength and global performance. Even the younger group had lower scores when compared with reference values. Functional aptitudes and social participation also showed differences between the two groups but the younger group had near normal performance as compared with reference values. This study demonstrated that upper extremity aptitudes related to coordination are affected early in the course of the disease. Functional independence and social participation showed a decrease performance later on and a high level of variability.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.008 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.000 |
| Science and technology studies | 0.000 | 0.001 |
| Scholarly communication | 0.000 | 0.001 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 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 teacher head, 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".