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 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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This post is Copyright: Hulya Ulugut,
Maxime Bertoux,
Kyan Younes,
Maxime Montembeault,
Giorgio G. Fumagalli,
Bedia Samanci,
Ignacio Illán‐Gala,
Gregory Kuchcinski,
Melanie Leroy,
Jennifer C. Thompson,
Christopher Kobylecki,
Alexander F Santillo,
Elisabet Englund,
Maria Landqvist Waldö,
Lina Riedl,
Jan Van den Stock,
Mathieu Vandenbulcke,
Rik Vandenberghe,
Robert Laforce Jr,
Simon Ducharme,
Peter S. Pressman,
Paulo Caramelli,
Leonardo Cruz de Souza,
Leonel T. Takada,
Hakan Gurvit,
Oskar Hansson,
Janine Diehl‐Schmid,
Daniela Galimberti,
Florence Pasquier,
Bruce L. Miller,
Philip Scheltens,
Rik Ossenkoppele,
Wiesje M. van der Flier,
Frederik Barkhof,
Nick C. Fox,
Virginia E. Sturm,
Toji Miyagawa,
Jennifer L. Whitwell,
Bradley Boeve,
Jonathan D. Rohrer,
Maria Luisa Gorno‐Tempini,
Keith A. Josephs,
Julie Snowden,
Jason D. Warren,
Katherine P. Rankin,
Yolande A. L. Pijnenburg,
International rtvFTD Working Group | July 10, 2024