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Age at symptom onset and death and disease duration in genetic frontotemporal dementia: an international retrospective cohort study
Katrina Moore, Jennifer Nicholas, Murray Grossman, Corey T. McMillan, David J. Irwin, Lauren Massimo, Vivianna M Van Deerlin, Jason D. Warren, Nick C. Fox, Martin N. Rossor, Simon Mead, Martina Bocchetta, Bradley F. Boeve, David S. Knopman, Neill R. Graff‐Radford, Leah K. Forsberg, Rosa Rademakers, Zbigniew K. Wszołek, John C. van Swieten, Lize C. Jiskoot, Lieke Meeter, Elise G.P. Dopper, Janne M. Papma, Julie S. Snowden, Jennifer A. Saxon, Matthew Jones, Stuart Pickering‐Brown, Isabelle Le Ber, Agnès Camuzat, Alexis Brice, Paola Caroppo, Roberta Ghidoni, Michela Pievani, Luisa Benussi, Giuliano Binetti, Bradford C. Dickerson, Diane Lucente, Samantha Krivensky, Caroline Graff, Linn Öijerstedt, Marie Fallström, Håkan Thonberg, Nupur Ghoshal, John C. Morris, Barbara Borroni, Alberto Benussi, Alessandro Padovani, Daniela Galimberti, Elio Scarpini, Giorgio Fumagalli, Ian R. Mackenzie, Ging‐Yuek Robin Hsiung, Pheth Sengdy, Adam L. Boxer, Howie Rosen, Joanne Taylor, Matthis Synofzik, Carlo Wilke, Patricia Sulzer, John R. Hodges, Glenda M. Halliday, John B. Kwok, Raquel Sánchez‐Valle, Albert Lladó, Sergi Borrego‐Écija, Isabel Santana, Maria Rosário Almeida, Miguel Tábuas‐Pereira, Fermín Moreno, Myriam Barandiarán, Begoña Indakoetxea, Johannes Levin, Adrian Danek, James B. Rowe, Thomas Cope, Markus Otto, Sarah Anderl‐Straub, Alexandre de Mendonça, Carolina Maruta, Mario Masellis, Sandra E. Black, Philippe Couratier, Géraldine Lautrette, Edward D. Huey, Sandro Sorbi, Benedetta Nacmias, Robert Laforce, Marie-Pier L Tremblay, Rik Vandenberghe, Philip Van Damme, Emily Rogalskı, Sandra Weıntraub, Alexander Gerhard, Chiadi U. Onyike, Simon Ducharme, Sokratis G. Papageorgiou, Adeline Su Lyn Ng, Amy Brodtmann, Elizabeth Finger, Rita Guerreiro, José Brás, Jonathan D. Rohrer, Carolin Heller, Rhian S. Convery, Ione Woollacott, Rachelle Shafei, Jonathan Graff-Radford, David T. Jones, Christina Dheel, Rodolfo Savica, Maria I. Lapid, Matt Baker, Julie A. Fields, Ralitza Gavrilova, Kimiko Domoto-Reilly, Jackie M. Poos, Emma L. van der Ende, Jessica Panman, Laura Donker Kaat, Harro Seelaar, Anna Richardson, Giovanni B. Frisoni, Anna Mega, Silvia Fostinelli, Huei-Hsin Chiang, Antonella Alberici, Andrea Arighi, Chiara Fenoglio, Hilary W. Heuer, Bruce L. Miller, Anna Karydas, Jamie Fong, Maria João Leitão, Beatriz Santiago, Diana Duro, Carlos Ferreira, Alazne Gabilondo, María de Arriba, Mikel Tainta, Miren Zulaica, Catarina B. Ferreira, Elisa Semler, Albert Ludolph, G. Bernhard Landwehrmeyer, Alexander E. Volk, Gabriel Miltenberger, Ana Verdelho, Sònia Afonso, Maria Carmela Tartaglia, Morris Freedman, Ekaterina Rogaeva, Camilla Ferrari, Irene Piaceri, Valentina Bessi, Gemma Lombardi, Frédéric St‐Onge, Marie-Claire Doré, Rose Bruffaerts, Mathieu Vandenbulcke, Jan Van den Stock, Marsel Mesulam, Eileen H. Bigio, Christos Koros, John Papatriantafyllou, Christos Κroupis, Leonidas Stefanis, Christien Shoesmith, Erik Robertson, Eliana Marisa Ramos, Daniel H. Geschwind
Bibliographic record
VenueThe Lancet Neurology · 2019
Typearticle
Languageen
FieldMedicine
TopicAmyotrophic Lateral Sclerosis Research
Canadian institutionsWestern UniversityUniversité LavalHôpital de l'Enfant-JésusUniversity of TorontoMcGill University Health CentreSunnybrook Health Science CentreHealth Sciences CentreUniversity of British Columbia
FundersJanssen PharmaceuticalsNational Institute of Neurological Disorders and StrokeNational Institute on AgingUCLH Biomedical Research CentreNational Health and Medical Research CouncilMedical Research CouncilGE HealthcareAlzheimer's Disease Research Center, Emory UniversityGenentechCentre Hospitalier Universitaire de NîmesInstitut de FranceHjärnfondenDemensfondenH. Lundbeck A/SStockholm läns landstingCentre Hospitalier Universitaire de NiceFédération pour la Recherche sur le CerveauRocheStockholms Läns LandstingBiogenAgence Nationale de la RechercheBundesministerium für Bildung und ForschungGuarantors of BrainNational Institute for Health and Care ResearchAssociation France ParkinsonStichting DioraphteWellcome TrustOntario Ministry of Economic Development and InnovationPfizerEU Joint Programme – Neurodegenerative Disease ResearchAstex PharmaceuticalsWeston Brain InstituteAlzheimerfondenKarolinska InstitutetZonMwNational Institutes of HealthAlzheimer's Drug Discovery FoundationTauRx PharmaceuticalsUCBMinistero della SaluteAssistance Publique - Hôpitaux de ParisAbbVieFondation Brain CanadaVetenskapsrådetCytokineticsNational Medical Research CouncilAlzheimer NederlandInstituto de Salud Carlos IIIUK Dementia Research InstituteOntario Brain InstituteMinistero dello Sviluppo EconomicoHeart and Stroke Foundation of CanadaNederlandse Organisatie voor Wetenschappelijk OnderzoekNovartisFondation Roger de SpoelberchEli Lilly and CompanyAmgenAssociation for Frontotemporal DegenerationAlexion PharmaceuticalsCanadian Institutes of Health ResearchAlzheimer's Society
KeywordsC9orf72Frontotemporal dementiaAge of onsetDementiaRetrospective cohort studyMedicineDiseaseCohortInternal medicineOncologyPediatrics
Abstract
No abstract in any covered source. Its absence is recorded, not treated as a negative.
No abstract. This is not a gap in this database; OpenAlex has none either. 23.3% of the frame is in this state, and the screen finds HALF as much metaresearch here, so the absence is a measured bias rather than a missing field.
How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot 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)0.003
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 score0.022
Threshold uncertainty score0.044
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.003 |
| Meta-epidemiology (narrow) | 0.001 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.002 |
| Bibliometrics | 0.002 | 0.004 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.002 | 0.002 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.002 | 0.002 |
| Insufficient payload (model declined to judge) | 0.002 | 0.001 |
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 head0.025
GPT teacher head0.305
Teacher spread0.280 · 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 itClassification
machine, unvalidatedMachine 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
Citations311
Published2019
Admission routes2
Has abstractno