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Early-Onset Familial Alzheimer's Disease (EOFAD)

2012· review· en· 218 citations· W2058621419 on OpenAlex· 10.1017/s0317167100013949

Why is this work in the frame?

A frame that forgets how it found something cannot be audited. These are the routes that admitted this work.

Canadian affiliationAn author listed a Canadian institution. This is the only route the usual frame has.
Canadian funderA Canadian agency funded it. The work may carry no Canadian affiliation at all.
Canadian venueIt was published in a Canadian venue.

Full frame distilled prediction

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.

Candidate categories
Meta-epidemiology (narrow), Science and technology studies, Research integrity
Consensus categories
Science and technology studies
Domain
Candidate signal: noneConsensus signal: none
Study design
Candidate signal: ObservationalConsensus signal: none
Genre
Candidate signal: ReviewConsensus signal: Review
Teacher disagreement score
0.544
Threshold uncertainty score
1.000
Validation status
machine_predicted_unvalidated · codex-gemma-dda1882f352a

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0040.002
Meta-epidemiology (narrow)0.0010.001
Meta-epidemiology (broad)0.0020.002
Bibliometrics0.0020.002
Science and technology studies0.0030.010
Scholarly communication0.0010.001
Open science0.0030.000
Research integrity0.0010.003
Insufficient payload (model declined to judge)0.0000.000

Machine scores (provisional)

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

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.

Opus teacher head0.115
GPT teacher head0.358
Teacher spread
0.243 · how far apart the two teachers sit on this one work
Validation status
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Abstract

Early-onset familial Alzheimer's disease (EOFAD) is a condition characterized by early onset dementia (age at onset < 65 years) and a positive family history for dementia. To date, 230 mutations in presenilin (PS1, PS2) and amyloid precursor protein (APP) genes have been identified in EOFAD. The mutations within these three genes (PS1/PS2/APP) affect a common pathogenic pathway in APP synthesis and proteolysis, which lead to excessive production of amyloid β. Compared with sporadic Alzheimer's disease (AD), EOFAD has some distinctive features including early age at onset, positive familial history, a variety of non-cognitive neurological symptoms and signs, and a more aggressive course. There is marked phenotypic heterogeneity among different mutations of EOFAD. Studies in presymptomatic mutation carriers reveal biomarkers abnormalities. EOFAD diagnosis is based on clinical and family history, neurological symptoms and examination, biomarker features, as well as genotyping in some cases. New therapeutic agents targeting amyloid formation may benefit EOFAD individuals.

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.

The record

Venue
Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques
Topic
Alzheimer's disease research and treatments
Field
Medicine
Canadian institutions
Health Sciences CentreCentre for Addiction and Mental HealthUniversity of TorontoMontreal Neurological Institute and HospitalSunnybrook Health Science CentreMcGill University Health CentreUniversity of British ColumbiaMcGill UniversityDouglas Mental Health University Institute
Funders
Canadian Institutes of Health ResearchPfizer CanadaNational Natural Science Foundation of ChinaPfizerAlzheimer's Association
Keywords
PresenilinDementiaDiseaseBiomarkerFamily historyEarly-onset Alzheimer's diseaseAlzheimer's diseaseAge of onsetAmyloid precursor proteinMedicinePSEN1Amyloid (mycology)GeneticsOncologyBiologyInternal medicinePathology
Has abstract in OpenAlex
yes