MétaCan
Menu
Back to cohort
Record W4416366173 · doi:10.1177/13872877251393414

Neurodevelopmental effects of genetic frontotemporal dementia mutations revealed by total intracranial volume differences

2025· article· en· W4416366173 on OpenAlexafffund
Isis So, Arabella Bouzigues, Lucy L. Russell, Phoebe H. Foster, Eve Ferry‐Bolder, John C. van Swieten, Lize C Jiskoot, Harro Seelaar, Raquel Sánchez‐Valle, Robert Laforce, Caroline Graff, Daniela Galimberti, Rik Vandenberghe, Alexandre de Mendonça, Pietro Tiraboschi, Isabel Santana, Alexander Gerhard, Johannes Levin, Sandro Sorbi, Markus Otto, Florence Pasquier, Simon Ducharme, Christopher Butler, Isabelle Le Ber, Maria Carmela Tartaglia, Mario Masellis, James B. Rowe, Matthis Synofzik, Fermín Moreno, Barbara Borroni, Tyler Kolander, Carly Mester, Danielle Brushaber, Kejal Kantarci, Hilary W. Heuer, Leah K. Forsberg, Jonathan D. Rohrer, Bradley F. Boeve, Adam L. Boxer, Howard J. Rosen, Elizabeth Finger

Bibliographic record

VenueJournal of Alzheimer s Disease · 2025
Typearticle
Languageen
FieldMedicine
TopicAmyotrophic Lateral Sclerosis Research
Canadian institutionsOccupational Cancer Research CentreDouglas Mental Health University InstituteUniversité LavalParkwood InstituteSunnybrook Health Science CentreLawson Health Research InstituteWestern University
FundersJanssen PharmaceuticalsNIHR Cambridge Biomedical Research CentreInstituto de Salud Carlos IIIMedical Research CouncilCanadian Institutes of Health ResearchGenentechTau ConsortiumBayer VitalCanada First Research Excellence FundStichting DioraphteHjärnfondenVetenskapsrådetEisaiMinistero della SaluteKarolinska InstitutetAlzheimer's Drug Discovery FoundationWeston Brain InstituteZonMwWellcome TrustUniversity College LondonUniversity of CambridgeAvid RadiopharmaceuticalsBundesministerium für Bildung und ForschungNational Institute for Health and Care ResearchEU Joint Programme – Neurodegenerative Disease ResearchSunovionAlzheimer’s Research UKNational Institutes of HealthAssociation for Frontotemporal DegenerationRegeneron PharmaceuticalsAlzheimer's AssociationAstex PharmaceuticalsDeutsche ForschungsgemeinschaftMcGill UniversityEli Lilly and CompanyAlzheimer NederlandBiogenIonis Pharmaceuticals
KeywordsFrontotemporal dementiaNeuroimagingCohortDementiaCohort studyMutationBrain size

Abstract

fetched live from OpenAlex

Background Converging evidence hints at neurodevelopmental effects in people at risk of genetic frontotemporal dementia (FTD). Objective We investigated total intracranial volume (TIV), a neuroimaging marker of neurodevelopment, and years of education differences between adult mutation carriers and familial non-mutation carriers, as measures of the structural and functional neurodevelopmental effects of FTD-causing genetic mutations. Methods This cross-sectional cohort study, facilitated through the FTD Prevention Initiative (FPI), included 902 adult pathogenic mutation carriers of GRN , MAPT , or C9orf72 , and 532 familial non-carriers. ANCOVAs were computed to compare TIV and education between groups per gene. Pearson's correlations were used to examine associations between TIV and education. Results Mutation carriers (mean ± SD age = 50.0 ± 13.2 years, sex = 55% female, n ( GRN ) = 298, n( MAPT ) = 187, n ( C9orf72 ) = 417) were compared to familial non-carriers (age = 48.0 ± 12.9 years, sex = 58% female, n ( GRN ) = 201, n( MAPT ) = 114), n ( C9orf72 ) = 217). Consistent with prior findings in young adults, GRN carriers showed larger TIV, on average by 20531 mm 3 , compared to familial non-carriers (95% CI [85.4, 40977], p = 0.049, η 2 p = 0.008). Larger TIV correlated with higher years of education in GRN carriers (95% CI [0.01, 0.24], r (295) = 0.12, p = 0.03) and GRN non-carriers (95% CI [0.08, 0.34], r(198) = 0.21, p = 0.002). MAPT carriers demonstrated smaller TIV than non-carriers, on average by 29896 mm 3 (95% CI [–58248, −1545], p = 0.039, η 2 p = 0.02). Models with C9orf72 and education as outcome variables did not reveal significant differences. Conclusions In support of the neurodevelopmental hypothesis of FTD, GRN and MAPT mutations are linked to structural neurodevelopmental changes in TIV. Further research is needed to identify mechanisms underlying neurodevelopmental influences of FTD mutations and ascertain their suitability as intervention targets.

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 score0.004
Threshold uncertainty score0.009

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 studies0.0000.000
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.000
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 head0.011
GPT teacher head0.273
Teacher spread0.262 · 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

Citations0
Published2025
Admission routes2
Has abstractyes

Explore more

Same venueJournal of Alzheimer s DiseaseSame topicAmyotrophic Lateral Sclerosis ResearchFrench-language works237,207