Rapidly Progressive Creutzfeldt-Jakob Disease VV1 Subtype
Bibliographic record
Abstract
Creutzfeldt-Jakob disease (CJD) is rare transmissible spongiform encephalopathy caused by abnormal, host-encoded prion proteins that accumulate in the central nervous tissue. CJD occurs in 3 forms: sporadic, familial and acquired (iatrogenic and variant). Six different molecular subtypes (MM1, MM2, MV1, MV2, VV1, and VV2) of sporadic CJD (sCJD) have been identified by genotype at codon 129. The VV1 type represents one of the rarest subtyp es. In contrast to the classic sCJD types, these patients are characterized by young age at onset, and slowly progressive dementia. Our case illustrates that sCJD VV1 subtype can have a rapid progression. A 50 year old Caucasian presented with memory loss and personality changes. Magnetic resonance imaging of the brain showed increased signal intensity in the grey mater of cerebral cortex. Brain biopsy confirmed the diagnosis of sCJD VV1 subtype. Currently there is no effective treatment and patient succumbed to death within 3 months of presentation. doi:10.4021/jmc221w
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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 | 0.000 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.000 |
| Science and technology studies | 0.001 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.001 | 0.000 |
| Insufficient payload (model declined to judge) | 0.001 | 0.001 |
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".