G07 Differences between Huntington's disease and spinocerebellar ataxia types 1 and 2 on cognitive and behavioural profile
Bibliographic record
Abstract
Background Huntington9s disease (HD) patients show progressive, mostly frontal-striatal-type, cognitive deterioration, and heterogeneous psychiatric symptoms. In a previous study, we found that negative psychiatric symptoms were common in HD. Like HD, spinocerebellar ataxias (SCAs) are adult onset dominantly transmitted triplet expansion neurodegenerative diseases that affect cognition and behaviour, although these aspects have not been exhaustively explored. Aims To compare cognitive and psychiatric impairment in HD and the two most common SCA forms in Italy (types 1 and 2). Methods We examined 15 HD, 15 SCA1 and 15 SCA2 patients of similar age and education. We assessed functional invalidity with the Total Functional Capacity scale. To assess cognitive state we used: Raven Progressive Matrices for non-verbal deductive reasoning; the Short Tale Test for long term verbal memory; the Phonemic Fluency Test for strategic search for words; the Visual Search Test for focused attention; and the Benton Visual Orientation Line Test for orientation and spatial perception. We examined anxiety and depression with the Hamilton Rating Scales for Anxiety and Depression. The Scales for the Assessment of Positive and Negative (SANS) Psychiatric Symptoms were also used to assess schizophrenia-like symptoms. Results HD patients did worse than SCA1 patients on most cognitive tests, except focused attention, where the two groups performed similarly. SCA2 patients had similar compromise to HD patients on most cognitive tests except verbal fluency, where HD did worse. The three groups did not differ on anxiety, depression or SAPS score but HD patients had a higher Scales for the Assessment of Positive and Negative score. Conclusions Although cognitive impairment is greatest in HD, the impairment pattern in all three diseases is consistent with frontal-striatal dysfunction. However, the greater prominence of negative psychiatric symptoms in HD constitutes a clear difference compared with the similarly invalidating SCAs, also characterised by frontal-striatal dysfunction. The difference may be due to differing involvement of the frontal-striatal and cerebellar-striatal circuits in these conditions.
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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 | 0.000 |
| Science and technology studies | 0.000 | 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) | 0.002 | 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".