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Record W4395669077 · doi:10.1002/alz.13818

α‐Synuclein seed amplification assay detects Lewy body co‐pathology in autosomal dominant Alzheimer's disease late in the disease course and dependent on Lewy pathology burden

2024· article· en· W4395669077 on OpenAlexfundno aff
Johannes Levin, Simone Baiardi, Corinne Quadalti, Marcello Rossi, Angela Mammana, Jonathan Vöglein, Alexander Bernhardt, Richard J. Perrin, Mathias Jucker, Oliver Preische, Anna Hofmann, Günter U. Höglinger, Nigel J. Cairns, Erin Franklin, Patricio Chrem, Carlos Cruchaga, Sarah Berman, Jasmeer P. Chhatwal, Alisha Daniels, Gregory S. Day, Natalie S. Ryan, Alison Goate, Brian A. Gordon, Edward D. Huey, Laura Ibáñez, Celeste M. Karch, Jae‐Hong Lee, Jorge J. Llibre‐Guerra, Francisco Lopera, Colin L. Masters, John C. Morris, James M. Noble, Alan E. Renton, Jee Hoon Roh, Matthew P. Frosch, C. Dirk Keene, Catriona McLean, Raquel Sánchez‐Valle, Peter R. Schofield, Charlene Supnet, Chengjie Xiong, Armin Giese, Oskar Hansson, Randall J. Bateman, Eric McDade

Bibliographic record

VenueAlzheimer s & Dementia · 2024
Typearticle
Languageen
FieldMedicine
TopicParkinson's Disease Mechanisms and Treatments
Canadian institutionsnot available
FundersInstituto de Salud Carlos IIICanadian Institutes of Health ResearchDeutsche ForschungsgemeinschaftDeutsches Zentrum für Neurodegenerative ErkrankungenNational Institute of Mental HealthMinistero della SaluteBundesministerium für Bildung und ForschungKorea Dementia Research CenterJapan Agency for Medical Research and DevelopmentFondation Brain CanadaWashington University in St. LouisFleniNational Institute on AgingAlzheimer's Association
KeywordsPathologyDiseaseLewy bodyLewy body diseaseAlzheimer's diseaseMedicineAmyloid (mycology)SynucleinAlpha-synucleinDementia with Lewy bodiesDementiaParkinson's disease

Abstract

fetched live from OpenAlex

INTRODUCTION: Amyloid beta and tau pathology are the hallmarks of sporadic Alzheimer's disease (AD) and autosomal dominant AD (ADAD). However, Lewy body pathology (LBP) is found in ≈ 50% of AD and ADAD brains. METHODS: Using an α-synuclein seed amplification assay (SAA) in cerebrospinal fluid (CSF) from asymptomatic (n = 26) and symptomatic (n = 27) ADAD mutation carriers, including 12 with known neuropathology, we investigated the timing of occurrence and prevalence of SAA positive reactivity in ADAD in vivo. RESULTS: No asymptomatic participant and only 11% (3/27) of the symptomatic patients tested SAA positive. Neuropathology revealed LBP in 10/12 cases, primarily affecting the amygdala or the olfactory areas. In the latter group, only the individual with diffuse LBP reaching the neocortex showed α-synuclein seeding activity in CSF in vivo. DISCUSSION: Results suggest that in ADAD LBP occurs later than AD pathology and often as amygdala- or olfactory-predominant LBP, for which CSF α-synuclein SAA has low sensitivity. HIGHLIGHTS: Cerebrospinal fluid (CSF) real-time quaking-induced conversion (RT-QuIC) detects misfolded α-synuclein in ≈ 10% of symptomatic autosomal dominant Alzheimer's disease (ADAD) patients. CSF RT-QuIC does not detect α-synuclein seeding activity in asymptomatic mutation carriers. Lewy body pathology (LBP) in ADAD mainly occurs as olfactory only or amygdala-predominant variants. LBP develops late in the disease course in ADAD. CSF α-synuclein RT-QuIC has low sensitivity for focal, low-burden LBP.

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)0.000
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: none
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.001
Threshold uncertainty score0.004

Distilled classifier scores by category (both heads)

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

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 head0.021
GPT teacher head0.294
Teacher spread0.273 · 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

Citations38
Published2024
Admission routes1
Has abstractyes

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