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Clinical recognition of frontotemporal dementia with right anterior temporal predominance : A multicenter retrospective cohort study

Ulugut, Hulya ; Bertoux, Maxime ; Younes, Kyan ; Montembeault, Maxime ; Fumagalli, Giorgio G. ; Samanci, Bedia ; Illán-Gala, Ignacio ; Kuchcinski, Gregory LU ; Leroy, Melanie and Thompson, Jennifer C. , et al. (2024) In Alzheimer's and Dementia
Abstract

INTRODUCTION: Although frontotemporal dementia (FTD) with right anterior temporal lobe (RATL) predominance has been recognized, a uniform description of the syndrome is still missing. This multicenter study aims to establish a cohesive clinical phenotype. METHODS: Retrospective clinical data from 18 centers across 12 countries yielded 360 FTD patients with predominant RATL atrophy through initial neuroimaging assessments. RESULTS: Common symptoms included mental rigidity/preoccupations (78%), disinhibition/socially inappropriate behavior (74%), naming/word-finding difficulties (70%), memory deficits (67%), apathy (65%), loss of empathy (65%), and face-recognition deficits (60%). Real-life examples unveiled impairments regarding... (More)

INTRODUCTION: Although frontotemporal dementia (FTD) with right anterior temporal lobe (RATL) predominance has been recognized, a uniform description of the syndrome is still missing. This multicenter study aims to establish a cohesive clinical phenotype. METHODS: Retrospective clinical data from 18 centers across 12 countries yielded 360 FTD patients with predominant RATL atrophy through initial neuroimaging assessments. RESULTS: Common symptoms included mental rigidity/preoccupations (78%), disinhibition/socially inappropriate behavior (74%), naming/word-finding difficulties (70%), memory deficits (67%), apathy (65%), loss of empathy (65%), and face-recognition deficits (60%). Real-life examples unveiled impairments regarding landmarks, smells, sounds, tastes, and bodily sensations (74%). Cognitive test scores indicated deficits in emotion, people, social interactions, and visual semantics however, lacked objective assessments for mental rigidity and preoccupations. DISCUSSION: This study cumulates the largest RATL cohort unveiling unique RATL symptoms subdued in prior diagnostic guidelines. Our novel approach, combining real-life examples with cognitive tests, offers clinicians a comprehensive toolkit for managing these patients. Highlights: This project is the first international collaboration and largest reported cohort. Further efforts are warranted for precise nomenclature reflecting neural mechanisms. Our results will serve as a clinical guideline for early and accurate diagnoses.

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author collaboration
organization
publishing date
type
Contribution to journal
publication status
in press
subject
keywords
emotion recognition, frontotemporal dementia, frontotemporal lobar degeneration, right anterior temporal lobe, semantic dementia, social cognition
in
Alzheimer's and Dementia
publisher
Wiley
external identifiers
  • pmid:38982845
  • scopus:85198107591
ISSN
1552-5260
DOI
10.1002/alz.14076
language
English
LU publication?
yes
additional info
Publisher Copyright: © 2024 The Author(s). Alzheimer's & Dementia published by Wiley Periodicals LLC on behalf of Alzheimer's Association.
id
5de9a97d-a659-4da2-98d1-1abb7dbf3d2a
date added to LUP
2024-07-22 16:17:50
date last changed
2024-07-23 10:56:17
@article{5de9a97d-a659-4da2-98d1-1abb7dbf3d2a,
  abstract     = {{<p>INTRODUCTION: Although frontotemporal dementia (FTD) with right anterior temporal lobe (RATL) predominance has been recognized, a uniform description of the syndrome is still missing. This multicenter study aims to establish a cohesive clinical phenotype. METHODS: Retrospective clinical data from 18 centers across 12 countries yielded 360 FTD patients with predominant RATL atrophy through initial neuroimaging assessments. RESULTS: Common symptoms included mental rigidity/preoccupations (78%), disinhibition/socially inappropriate behavior (74%), naming/word-finding difficulties (70%), memory deficits (67%), apathy (65%), loss of empathy (65%), and face-recognition deficits (60%). Real-life examples unveiled impairments regarding landmarks, smells, sounds, tastes, and bodily sensations (74%). Cognitive test scores indicated deficits in emotion, people, social interactions, and visual semantics however, lacked objective assessments for mental rigidity and preoccupations. DISCUSSION: This study cumulates the largest RATL cohort unveiling unique RATL symptoms subdued in prior diagnostic guidelines. Our novel approach, combining real-life examples with cognitive tests, offers clinicians a comprehensive toolkit for managing these patients. Highlights: This project is the first international collaboration and largest reported cohort. Further efforts are warranted for precise nomenclature reflecting neural mechanisms. Our results will serve as a clinical guideline for early and accurate diagnoses.</p>}},
  author       = {{Ulugut, Hulya and Bertoux, Maxime and Younes, Kyan and Montembeault, Maxime and Fumagalli, Giorgio G. and Samanci, Bedia and Illán-Gala, Ignacio and Kuchcinski, Gregory and Leroy, Melanie and Thompson, Jennifer C. and Kobylecki, Christopher and Santillo, Alexander F. and Englund, Elisabet and Waldö, Maria Landqvist and Riedl, Lina and Van den Stock, Jan and Vandenbulcke, Mathieu and Vandenberghe, Rik and Laforce, Robert and Ducharme, Simon and Pressman, Peter S. and Caramelli, Paulo and de Souza, Leonardo Cruz and Takada, Leonel T. and Gurvit, Hakan and Hansson, Oskar and Diehl-Schmid, Janine and Galimberti, Daniela and Pasquier, Florence and Miller, Bruce L. and Scheltens, Philip and Ossenkoppele, Rik and van der Flier, Wiesje M. and Barkhof, Frederik and Fox, Nick C. and Sturm, Virginia E. and Miyagawa, Toji and Whitwell, Jennifer L. and Boeve, Bradley and Rohrer, Jonathan D. and Gorno-Tempini, Maria Luisa and Josephs, Keith A. and Snowden, Julie and Warren, Jason D. and Rankin, Katherine P. and Pijnenburg, Yolande A.L.}},
  issn         = {{1552-5260}},
  keywords     = {{emotion recognition; frontotemporal dementia; frontotemporal lobar degeneration; right anterior temporal lobe; semantic dementia; social cognition}},
  language     = {{eng}},
  publisher    = {{Wiley}},
  series       = {{Alzheimer's and Dementia}},
  title        = {{Clinical recognition of frontotemporal dementia with right anterior temporal predominance : A multicenter retrospective cohort study}},
  url          = {{http://dx.doi.org/10.1002/alz.14076}},
  doi          = {{10.1002/alz.14076}},
  year         = {{2024}},
}