Evidence map›Paper›PMID 35731122›Full record

ArticleBrain : a journal of neurology2022

Right temporal degeneration and socioemotional semantics: semantic behavioural variant frontotemporal dementia.

Kyan Younes, Valentina Borghesani, Maxime Montembeault, Salvatore Spina, Maria Luisa Mandelli, Ariane E Welch, Elizabeth Weis, Patrick Callahan, Fanny M Elahi, Alice Y Hua and 15 more

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

Article in Brain : a journal of neurology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 89 papers, 3 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
89citing papers in PubMed, 3 pooled it
15.1field-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

89 citing papers in PubMed, 3 syntheses or guidelines pooled it, 135 citations in OpenAlex.

  1. The prevalence of frontotemporal dementia: a systematic review and meta-analysis.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2026
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  18. Multimodal semantic knowledge of emotion concepts in frontotemporal dementia.medRxiv : the preprint server for health sciences · 2026
    Article
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29 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

25 authors at 2 institutions in 1 country.

Kyan YounesMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.ORCID 0000-0002-9912-1616
Valentina BorghesaniMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.
Maxime MontembeaultMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.
Salvatore SpinaMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.ORCID 0000-0003-3570-9143
Maria Luisa MandelliMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.
Ariane E WelchMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.
Elizabeth WeisMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.
Patrick CallahanMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.
Fanny M ElahiMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.
Alice Y HuaMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.
David C PerryMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.
Anna KarydasMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.
Daniel GeschwindNeurogenetics Program, Department of Neurology and Semel Institute for Neuroscience and Human Behavior, David Geffen School of Medicine, University of California, Los Angeles, CA 90024, USA.ORCID 0000-0003-2896-3450
Eric HuangDepartment of Pathology, University of California, San Francisco, CA 94143, USA.ORCID 0000-0002-5381-3801
Lea T GrinbergMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.ORCID 0000-0002-6809-0618
Joel H KramerMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.
Adam L BoxerMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.
Gil D RabinoviciMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.
Howard J RosenMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.
William W SeeleyMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.
Zachary A MillerMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.ORCID 0000-0002-5991-3053
Bruce L MillerMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.
Virginia E SturmMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.
Katherine P RankinMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.
Maria Luisa Gorno-TempiniMemory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.
University of California, San Francisco · USUniversity of California, Los Angeles · US

Funding

TDP-43 Loss-of-Function: Biology to BiomarkersP01AG019724 · NIA · UNIVERSITY OF PENNSYLVANIA · PI Jennifer Merrilees · 2002 to 2026
$67.2M
Research Education ComponentP30AG062422 · NIA · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI Gil Dan Rabinovici · 2019 to 2026
$36.9M
PrPSc SPECIFIC INTERACTION WITH NOVEL PrP-Fc FUSION PROTEINSP50AG023501 · NIA · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI HUANG, YADONG · 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
Clinical Features and Neuropathological Basis of Sleep Wake Behavior in Alzheimer's and PSPR01AG060477 · NIA · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI Lea Tenenholz Grinberg, Thomas C Neylan · 2019 to 2026
$8.2M
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
Primary Progressive Aphasia: Cognition, Anatomy and ProgressionRF1NS050915 · NINDS · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI GORNO TEMPINI, MARIA LUISA · 2022 to 2022
$4.7M
Attention and Semantic Evaluation as Predictors of Empathy in Healthy Aging and Frontotemporal DementiaRF1AG029577 · NIA · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI RANKIN, KATHERINE P · 2019 to 2019
$4.0M
Diagnostic and prognostic certainty in behavioral variant frontotemporal dementiaR01AG062758 · NIA · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI PERRY, DAVID C · 2020 to 2025
$4.0M
Reward processing in genetic frontotemporal dementia and mood disordersR01AG059794 · NIA · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI PERRY, DAVID C · 2019 to 2023
$4.0M
NIA NIH HHS K08 AG052648NIA NIH HHS K24 AG045333NIA NIH HHS K24 AG053435NIA NIH HHS P01 AG019724NIA NIH HHS P30 AG062422NIA NIH HHS P50 AG023501NIA NIH HHS R01 AG029577NIA NIH HHS R01 AG038791NIA NIH HHS R01 AG052496NIA NIH HHS R01 AG059794NIA NIH HHS R01 AG060477NIA NIH HHS R01 AG062758NIA NIH HHS RF1 AG029577NIDCD NIH HHS K24 DC015544NINDS NIH HHS R01 NS050915NINDS NIH HHS RF1 NS050915NINDS NIH HHS U54 NS092089
6 · The paper itself

Abstract

Focal anterior temporal lobe degeneration often preferentially affects the left or right hemisphere. While patients with left-predominant anterior temporal lobe atrophy show severe anomia and verbal semantic deficits and meet criteria for semantic variant primary progressive aphasia and semantic dementia, patients with early right anterior temporal lobe atrophy are more difficult to diagnose as their symptoms are less well understood. Focal right anterior temporal lobe atrophy is associated with prominent emotional and behavioural changes, and patients often meet, or go on to meet, criteria for behavioural variant frontotemporal dementia. Uncertainty around early symptoms and absence of an overarching clinico-anatomical framework continue to hinder proper diagnosis and care of patients with right anterior temporal lobe disease. Here, we examine a large, well-characterized, longitudinal cohort of patients with right anterior temporal lobe-predominant degeneration and propose new criteria and nosology. We identified individuals from our database with a clinical diagnosis of behavioural variant frontotemporal dementia or semantic variant primary progressive aphasia and a structural MRI (n = 478). On the basis of neuroimaging criteria, we defined three patient groups: right anterior temporal lobe-predominant atrophy with relative sparing of the frontal lobes (n = 46), frontal-predominant atrophy with relative sparing of the right anterior temporal lobe (n = 79) and left-predominant anterior temporal lobe-predominant atrophy with relative sparing of the frontal lobes (n = 75). We compared the clinical, neuropsychological, genetic and pathological profiles of these groups. In the right anterior temporal lobe-predominant group, the earliest symptoms were loss of empathy (27%), person-specific semantic impairment (23%) and complex compulsions and rigid thought process (18%). On testing, this group exhibited greater impairments in Emotional Theory of Mind, recognition of famous people (from names and faces) and facial affect naming (despite preserved face perception) than the frontal- and left-predominant anterior temporal lobe-predominant groups. The clinical symptoms in the first 3 years of the disease alone were highly sensitive (81%) and specific (84%) differentiating right anterior temporal lobe-predominant from frontal-predominant groups. Frontotemporal lobar degeneration-transactive response DNA binding protein (84%) was the most common pathology of the right anterior temporal lobe-predominant group. Right anterior temporal lobe-predominant degeneration is characterized by early loss of empathy and person-specific knowledge, deficits that are caused by progressive decline in semantic memory for concepts of socioemotional relevance. Guided by our results, we outline new diagnostic criteria and propose the name, 'semantic behavioural variant frontotemporal dementia', which highlights the underlying cognitive mechanism and the predominant symptomatology. These diagnostic criteria will facilitate early identification and care of patients with early, focal right anterior temporal lobe degeneration as well as in vivo prediction of frontotemporal lobar degeneration-transactive response DNA binding protein pathology.

Indexed as

Aphasia, Primary ProgressiveFrontotemporal DementiaFrontotemporal Lobar DegenerationAtrophyDNA-Binding ProteinsHumansMagnetic Resonance ImagingNeuropsychological TestsSemanticsDNA-Binding Proteinsfrontotemporal dementiaFTLD-TDP type Closs of empathy and non-verbal semanticsperson-specific knowledgeright temporal lobe-predominant neurodegeneration

Identifiers

PMID35731122
PMCPMC10200288
OpenAlexW4283323937

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.