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Record W2087157508 · doi:10.1002/mds.25075

The incidence and prevalence of Huntington's disease: A systematic review and meta‐analysis

2012· review· en· W2087157508 on OpenAlex

Why this work is in the frame

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

affAt least one author lists a Canadian institution in the pinned OpenAlex snapshot.

Bibliographic record

VenueMovement Disorders · 2012
Typereview
Languageen
FieldNeuroscience
TopicGenetic Neurodegenerative Diseases
Canadian institutionsUniversity of TorontoUniversity of Calgary
Fundersnot available
KeywordsIncidence (geometry)Meta-analysisMedicineEpidemiologyChoreaConfidence intervalDementiaPrevalenceDemographyHuntington's diseaseDiseasePediatricsInternal medicine

Abstract

fetched live from OpenAlex

Huntington's disease (HD) is a rare, neurodegenerative disorder characterized by chorea, behavioral manifestations, and dementia. The aim of this study was to estimate the incidence and prevalence of HD through a systematic review of the literature. Medline and Embase databases were searched using terms specific to HD as well as studies of incidence, prevalence, and epidemiology. All studies reporting the incidence and/or prevalence of HD were included. Twenty original research articles were included. Eight studies examined incidence, and 17 studies examined prevalence. Meta-analysis of data from four incidence studies revealed an incidence of 0.38 per 100,000 per year (95% confidence interval [CI]: 0.16, 0.94). Lower incidence was reported in the Asian studies (n = 2), compared to the studies performed in Europe, North America, and Australia (n = 6). The worldwide service-based prevalence of HD, based on a meta-analysis (n = 13 studies), was 2.71 per 100,000 (95% CI: 1.55-4.72). Eleven studies were conducted in Europe, North American, and Australia, with an overall prevalence of 5.70 per 100,000 (95% CI: 4.42-7.35). Three studies were conducted in Asia, with an overall prevalence of 0.40 per 100,000 (95% CI: 0.26-0.61). Metaregression revealed a significantly lower prevalence of HD in Asia, compared to European, North American, and Australian populations. HD is a devastating neurodegenerative disorder with a higher prevalence in Europe, North America, and Australia than in Asia. The difference in prevalence of this genetic disorder can be largely explained by huntingtin gene haplotypes.

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.

Full frame distilled prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. 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.

metaresearch head score (Codex)0.001
metaresearch head score (Gemma)0.001
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesMeta-epidemiology (narrow)
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Systematic review · Consensus signal: none
GenreCandidate signal: Review · Consensus signal: Review
Teacher disagreement score0.973
Threshold uncertainty score1.000

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0010.001
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0030.001
Bibliometrics0.0000.001
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0010.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0000.000

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 head0.052
GPT teacher head0.321
Teacher spread0.269 · 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