Cervical Spinal Cord Degeneration in Spinocerebellar Ataxia Type 7
Bibliographic record
Abstract
BACKGROUND AND PURPOSE: Spinocerebellar ataxia type 7 is an autosomal dominant neurodegenerative disease caused by a cytosine-adenine-guanine (CAG) repeat expansion. Clinically, spinocerebellar ataxia type 7 is characterized by progressive cerebellar ataxia, pyramidal signs, and macular degeneration. In vivo MR imaging studies have shown extensive gray matter degeneration in the cerebellum and, to a lesser extent, in a range of cortical cerebral areas. The purpose of this study was to evaluate the impact of the disease in the spinal cord and its relationship with the patient's impairment. MATERIALS AND METHODS: Using a semiautomated procedure applied to MR imaging data, we analyzed spinal cord area and eccentricity in a cohort of 48 patients with spinocerebellar ataxia type 7 and compared them with matched healthy controls. The motor impairment in the patient group was evaluated using the Scale for Assessment and Rating of Ataxia. RESULTS: Our analysis showed a significantly smaller cord area ( t = 9.04, P < .001, d = 1.31) and greater eccentricity ( t = –2.25, P =. 02, d = 0.32) in the patient group. Similarly, smaller cord area was significantly correlated with a greater Scale for Assessment and Rating of Ataxia score ( r = –0.44, P = .001). A multiple regression model showed that the spinal cord area was strongly associated with longer CAG repetition expansions ( P = .002) and greater disease duration ( P = .020). CONCLUSIONS: Our findings indicate that cervical spinal cord changes are progressive and clinically relevant features of spinocerebellar ataxia type 7, and future investigation of these measures as candidate biomarkers is warranted. CAG : cytosine-adenine-guanine SARA : Scale for Assessment and Rating of Ataxia SCA7 : spinocerebellar ataxia type 7
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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 | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.001 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.003 | 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".