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Record W4283369459 · doi:10.1017/cjn.2022.95

A.5 Neuropathology of eight cases of the New Brunswick cluster of Neurological Syndrome of Unknown Cause (NSUC)

2022· article· en· W4283369459 on OpenAlexaffvenueabout
GH Jansen, AK Miller, AS Easton, JM Woulfe

Bibliographic record

VenueCanadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques · 2022
Typearticle
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicPrion Diseases and Protein Misfolding
Canadian institutionsUniversity of Winnipeg
Fundersnot available
KeywordsNeuropathologyPathologyLewy body diseaseDiseaseMedicineLewy bodyCluster (spacecraft)Anatomical pathologyPediatricsDementiaDementia with Lewy bodiesImmunohistochemistry

Abstract

fetched live from OpenAlex

Background: In March 2021, at a press conference, the presence of a cluster of patients, claimed to have a novel neurological syndrome, was announced in New Brunswick. These patients were suggested to have symptoms reminiscent of CJD. The onset of disease was between 2015 and 2021. The size of this cluster has been reported as approximately 50 cases. Further news publications have suggested that various environmental factors were causing this disease. Methods: Between 2019 and 2021 eight patients have died in this cluster. Their neuropathological findings are reported here. Results: There was one case of metastatic carcinoma, one case of FTLD-TDP43, one case of neocortical Lewy body pathology, one case of neocortical Lewy body pathology and AD, 2 cases of AD with vascular pathology, one case of mainly vascular pathology, and one case without significant pathology (consistent with patient’s history). In all these patients no evidence for a prion disease was found, nor novel pathology. Conclusions: We suggest that these 8 patients represent a group of misclassified clinical diagnoses. Classical probability theorem based statistical evaluation shows that this group of deceased patients is representative for the entire cluster at a p=0.0001 level, which would suggest that the entire cluster is based on misdiagnoses.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame machine prediction

Teacher imitation

Not 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.

metaresearch head score (Codex)0.000
metaresearch head score (Gemma)0.001
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Case report · Consensus signal: none
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.904
Threshold uncertainty score0.192

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0000.001
Meta-epidemiology (narrow)0.0010.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0020.001
Science and technology studies0.0020.001
Scholarly communication0.0000.000
Open science0.0010.001
Research integrity0.0010.000
Insufficient payload (model declined to judge)0.0020.000

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.

Opus teacher head0.022
GPT teacher head0.253
Teacher spread0.231 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designCase report
Domainnot available
GenreEmpirical

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".

Quick stats

Citations0
Published2022
Admission routes3
Has abstractyes

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