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Record W3037390891 · doi:10.1002/mds.28171

Parkinson's Disease, <scp><i>NOTCH3</i></scp> Genetic Variants, and White Matter Hyperintensities

2020· article· en· W3037390891 on OpenAlexafffundabout
Joel Ramirez, Allison A. Dilliott, Malcolm A. Binns, David P. Breen, Emily C. Evans, Derek Beaton, Paula McLaughlin, Donna Kwan, Melissa F. Holmes, Miracle Ozzoude, Christopher J.M. Scott, Stephen C. Strother, Sean Symons, Richard H. Swartz, David A. Grimes, Mandar Jog, Mario Masellis, Sandra E. Black, Anne Joutel, Connie Marras, Ekaterina Rogaeva, Robert A. Hegele, Anthony E. Lang

Bibliographic record

VenueMovement Disorders · 2020
Typearticle
Languageen
FieldMedicine
TopicCerebrovascular and genetic disorders
Canadian institutionsToronto Western HospitalOccupational Cancer Research CentreOttawa HospitalHealth Sciences CentreQueen's UniversityNova Scotia Health AuthorityCanadian Celiac AssociationUniversity of OttawaOntario Brain InstituteBaycrest HospitalDalhousie UniversityUniversity of TorontoWestern UniversitySunnybrook Health Science Centre
FundersWellcome TrustOntario Brain Institute
KeywordsHyperintensityWhite matterParkinson's diseaseDiseaseMedicineMagnetic resonance imagingPsychologyCohortCognitive declinePopulationNeuroscienceCardiologyInternal medicineDementiaRadiology

Abstract

fetched live from OpenAlex

BACKGROUND: White matter hyperintensities (WMH) on magnetic resonance imaging may influence clinical presentation in patients with Parkinson's disease (PD), although their significance and pathophysiological origins remain unresolved. Studies examining WMH have identified pathogenic variants in NOTCH3 as an underlying cause of inherited forms of cerebral small vessel disease. METHODS: We examined NOTCH3 variants, WMH volumes, and clinical correlates in 139 PD patients in the Ontario Neurodegenerative Disease Research Initiative cohort. RESULTS: We identified 13 PD patients (~9%) with rare (<1% of general population), nonsynonymous NOTCH3 variants. Bayesian linear modeling demonstrated a doubling of WMH between variant negative and positive patients (3.1 vs. 6.9 mL), with large effect sizes for periventricular WMH (d = 0.8) and lacunes (d = 1.2). Negative correlations were observed between WMH and global cognition (r = -0.2). CONCLUSION: The NOTCH3 rare variants in PD may significantly contribute to increased WMH burden, which in turn may negatively influence cognition. © 2020 International Parkinson and Movement Disorder Society.

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)0.001
metaresearch head score (Gemma)0.002
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.075
Threshold uncertainty score0.149

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0010.002
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0010.001
Science and technology studies0.0000.001
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0030.000

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 head0.007
GPT teacher head0.195
Teacher spread0.188 · 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 designObservational
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

Citations28
Published2020
Admission routes3
Has abstractyes

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