Evidence map›Paper›PMID 29053860›Full record

ArticleBrain : a journal of neurology2017

Clinicopathological correlations in behavioural variant frontotemporal dementia.

David C Perry, Jesse A Brown, Katherine L Possin, Samir Datta, Andrew Trujillo, Anneliese Radke, Anna Karydas, John Kornak, Ana C Sias, Gil D Rabinovici and 28 more

Open access · bronzeAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Brain : a journal of neurology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 190 papers, 6 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
190citing papers in PubMed, 6 pooled it
16.7field-weighted citation impact, top 1% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

190 citing papers in PubMed, 6 syntheses or guidelines pooled it, 313 citations in OpenAlex.

  1. Pooled it
  2. Pooled it
  3. Pooled it
  4. Pooled it
  5. Pooled it
  6. Pooled it
  7. Trial
  8. Trial
  9. Review
  10. Article
  11. Article
  12. Article
  13. Multimodal semantic knowledge of emotion concepts in frontotemporal dementia.medRxiv : the preprint server for health sciences · 2026
    Article
  14. Article
  15. Article
  16. Article
  17. Article
  18. Article
  19. Italian intersocietal recommendations for restructuring the diagnostic-therapeutic pathway for the implementation and appropriate use of anti-amyloid monoclonal antibodies in Alzheimer's disease.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2025
    Review
  20. Article

130 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

38 authors at 7 institutions in 4 countries.

David C PerryDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Jesse A BrownDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Katherine L PossinDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Samir DattaDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Andrew TrujilloDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Anneliese RadkeDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Anna KarydasDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
John KornakDepartment of Epidemiology and Biostatistics, University of California, San Francisco, San Francisco, CA, USA.
Ana C SiasDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Gil D RabinoviciDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Maria Luisa Gorno-TempiniDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Adam L BoxerDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Mary De MayDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Katherine P RankinDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Virginia E SturmDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Suzee E LeeDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Brandy R MatthewsDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Aimee W KaoDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Keith A VosselDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Maria Carmela TartagliaDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Zachary A MillerDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Sang Won SeoDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Manu SidhuDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Stephanie E GausDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Alissa L NanaDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Jose Norberto S VargasDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Ji-Hye L HwangDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Rik OssenkoppeleDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Alainna B BrownDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Eric J HuangDepartment of Pathology and Laboratory Medicine, University of California, San Francisco, San Francisco, CA, USA.
Giovanni CoppolaNeurogenetics program, Department of Neurology, and Semel Institute for Neuroscience and Human Behaviour, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA, USA.
Howard J RosenDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Daniel GeschwindNeurogenetics program, Department of Neurology, and Semel Institute for Neuroscience and Human Behaviour, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA, USA.
John Q TrojanowskiCenter for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Lea T GrinbergDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Joel H KramerDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
Bruce L MillerDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
William W SeeleyDepartment of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.
University of California, San Francisco · USUniversity of California, Los Angeles · USInstitute for Neurodegenerative Disorders · USSungkyunkwan University · KRUniversity of California, Davis · USUniversity of Toronto · CAUniversity of Washington · US

Funding

National Centralized Repository for Alzheimer's Disease and Related Dementias (NCRAD)U24AG021886 · NIA · INDIANA UNIV-PURDUE UNIV AT INDIANAPOLIS · PI TATIANA M. FOROUD · 2002 to 2026
$119.8M
TDP-43 Loss-of-Function: Biology to BiomarkersP01AG019724 · NIA · UNIVERSITY OF PENNSYLVANIA · PI Jennifer Merrilees · 2002 to 2026
$67.2M
Transgenic Mouse Models of Tau PathologiesP01AG017586 · NIA · UNIVERSITY OF PENNSYLVANIA · PI LEE, VIRGINIA M · 2000 to 2021
$37.3M
PSYCHOSOCIAL COREP30AG008051 · NIA · NEW YORK UNIVERSITY SCHOOL OF MEDICINE · PI SHAO, YONGZHAO · 1990 to 2019
$36.1M
UPenn ADCC Biomarker CoreP30AG010124 · NIA · UNIVERSITY OF PENNSYLVANIA · PI XIE, SHARON XIANGWEN · 1991 to 2020
$35.1M
PrPSc SPECIFIC INTERACTION WITH NOVEL PrP-Fc FUSION PROTEINSP50AG023501 · NIA · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI MILLER, BRUCE L · 2004 to 2018
$27.1M
The Four Repeat Tauopathy Neuroimaging InitiativeR01AG038791 · NIA · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI BOXER, ADAM L. · 2010 to 2020
$18.1M
Progressive Aphasia Cognition Anatomy and ProgressionR01NS050915 · NINDS · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI MARIA LUISA GORNO TEMPINI · 2004 to 2026
$11.0M
The Frontotemporal Lobar Degeneration Neuroimaging InitiativeR01AG032306 · NIA · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI ROSEN, HOWARD J · 2009 to 2013
$10.0M
Emotion network dysfunction and decline in early frontotemporal dementiaR01AG052496 · NIA · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI Virginia Emily Sturm · 2017 to 2026
$6.9M
Training - The Frontotemporal Lobar Degeneration Clinical Research ConsortiumU54NS092089 · NINDS · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI BOXER, ADAM L. · 2014 to 2018
$6.4M
Decision-Making in Genetic FTDR01AG022983 · NIA · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI CHIONG, WINSTON · 2004 to 2021
$6.0M
NIA NIH HHS K01 AG055698NIA NIH HHS K23 AG038357NIA NIH HHS K23 AG039414NIA NIH HHS K23 AG045289NIA NIH HHS K24 AG045333NIA NIH HHS P01 AG017586NIA NIH HHS P01 AG019724NIA NIH HHS P30 AG008051NIA NIH HHS P30 AG010124NIA NIH HHS P50 AG023501NIA NIH HHS R01 AG022983NIA NIH HHS R01 AG032306NIA NIH HHS R01 AG038791NIA NIH HHS R01 AG052496NIA NIH HHS RF1 AG029577NIA NIH HHS U01 AG052943NIA NIH HHS U24 AG021886NIDCD NIH HHS K24 DC015544NINDS NIH HHS R01 NS050915NINDS NIH HHS R01 NS095257NINDS NIH HHS U54 NS092089
6 · The paper itself

Abstract

Accurately predicting the underlying neuropathological diagnosis in patients with behavioural variant frontotemporal dementia (bvFTD) poses a daunting challenge for clinicians but will be critical for the success of disease-modifying therapies. We sought to improve pathological prediction by exploring clinicopathological correlations in a large bvFTD cohort. Among 438 patients in whom bvFTD was either the top or an alternative possible clinical diagnosis, 117 had available autopsy data, including 98 with a primary pathological diagnosis of frontotemporal lobar degeneration (FTLD), 15 with Alzheimer's disease, and four with amyotrophic lateral sclerosis who lacked neurodegenerative disease-related pathology outside of the motor system. Patients with FTLD were distributed between FTLD-tau (34 patients: 10 corticobasal degeneration, nine progressive supranuclear palsy, eight Pick's disease, three frontotemporal dementia with parkinsonism associated with chromosome 17, three unclassifiable tauopathy, and one argyrophilic grain disease); FTLD-TDP (55 patients: nine type A including one with motor neuron disease, 27 type B including 21 with motor neuron disease, eight type C with right temporal lobe presentations, and 11 unclassifiable including eight with motor neuron disease), FTLD-FUS (eight patients), and one patient with FTLD-ubiquitin proteasome system positive inclusions (FTLD-UPS) that stained negatively for tau, TDP-43, and FUS. Alzheimer's disease was uncommon (6%) among patients whose only top diagnosis during follow-up was bvFTD. Seventy-nine per cent of FTLD-tau, 86% of FTLD-TDP, and 88% of FTLD-FUS met at least 'possible' bvFTD diagnostic criteria at first presentation. The frequency of the six core bvFTD diagnostic features was similar in FTLD-tau and FTLD-TDP, suggesting that these features alone cannot be used to separate patients by major molecular class. Voxel-based morphometry revealed that nearly all pathological subgroups and even individual patients share atrophy in anterior cingulate, frontoinsula, striatum, and amygdala, indicating that degeneration of these regions is intimately linked to the behavioural syndrome produced by these diverse aetiologies. In addition to these unifying features, symptom profiles also differed among pathological subtypes, suggesting distinct anatomical vulnerabilities and informing a clinician's prediction of pathological diagnosis. Data-driven classification into one of the 10 most common pathological diagnoses was most accurate (up to 60.2%) when using a combination of known predictive factors (genetic mutations, motor features, or striking atrophy patterns) and the results of a discriminant function analysis that incorporated clinical, neuroimaging, and neuropsychological data.

Indexed as

AdultAgedAged, 80 and overAlzheimer DiseaseAmyotrophic Lateral SclerosisAutopsyBrainFemaleFrontotemporal DementiaFrontotemporal Lobar DegenerationHumansMagnetic Resonance ImagingMaleMiddle AgedOrgan SizePick Disease of the BrainAlzheimer’s diseasecorticobasal degenerationfrontotemporal dementiafrontotemporal lobar degenerationPick’s disease

Identifiers

PMID29053860
PMCPMC5841140
OpenAlexW2760832975

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.