TY - JOUR
T1 - Clinical recognition of frontotemporal dementia with right anterior temporal predominance
T2 - A multicenter retrospective cohort study
AU - Ulugut, Hulya
AU - Bertoux, Maxime
AU - Younes, Kyan
AU - Montembeault, Maxime
AU - Fumagalli, Giorgio G.
AU - Samanci, Bedia
AU - Illán-Gala, Ignacio
AU - Kuchcinski, Gregory
AU - Leroy, Melanie
AU - Thompson, Jennifer C.
AU - Kobylecki, Christopher
AU - Santillo, Alexander F.
AU - Englund, Elisabet
AU - Waldö, Maria Landqvist
AU - Riedl, Lina
AU - van den Stock, Jan
AU - Vandenbulcke, Mathieu
AU - Vandenberghe, Rik
AU - Laforce Jr, Robert
AU - Ducharme, Simon
AU - Pressman, Peter S.
AU - Caramelli, Paulo
AU - de Souza, Leonardo Cruz
AU - Takada, Leonel T.
AU - Gurvit, Hakan
AU - Hansson, Oskar
AU - Diehl-Schmid, Janine
AU - Galimberti, Daniela
AU - Pasquier, Florence
AU - Miller, Bruce L.
AU - Scheltens, Philip
AU - Ossenkoppele, Rik
AU - van der Flier, Wiesje M.
AU - Barkhof, Frederik
AU - Fox, Nick C.
AU - Sturm, Virginia E.
AU - Miyagawa, Toji
AU - Whitwell, Jennifer L.
AU - Boeve, Bradley
AU - Rohrer, Jonathan D.
AU - International rtvFTD Working Group
AU - Gorno-Tempini, Maria Luisa
AU - Josephs, Keith A.
AU - Snowden, Julie
AU - Warren, Jason D.
AU - Rankin, Katherine P.
AU - Pijnenburg, Yolande A. L.
N1 - Publisher Copyright:
© 2024 The Author(s). Alzheimer's & Dementia published by Wiley Periodicals LLC on behalf of Alzheimer's Association.
PY - 2024/8
Y1 - 2024/8
N2 - 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.
AB - 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.
KW - emotion recognition
KW - frontotemporal dementia
KW - frontotemporal lobar degeneration
KW - right anterior temporal lobe
KW - semantic dementia
KW - social cognition
UR - https://www.scopus.com/pages/publications/85198107591
U2 - 10.1002/alz.14076
DO - 10.1002/alz.14076
M3 - Article
C2 - 38982845
SN - 1552-5260
VL - 20
SP - 5647
EP - 5661
JO - Alzheimer's and Dementia
JF - Alzheimer's and Dementia
IS - 8
ER -