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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; Illan-Gala, Ignacio; ... Pijnenburg, Yolande AL; + view all (2024) Clinical recognition of frontotemporal dementia with right anterior temporal predominance: A multicenter retrospective cohort study. Alzheimer's & Dementia: The Journal of the Alzheimer's Association , 20 (8) pp. 5647-5661. 10.1002/alz.14076. Green open access

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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.

Type: Article
Title: Clinical recognition of frontotemporal dementia with right anterior temporal predominance: A multicenter retrospective cohort study
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1002/alz.14076
Publisher version: https://doi.org/10.1002/alz.14076
Language: English
Additional information: © 2024 The Author(s). Alzheimer's & Dementia published by Wiley Periodicals LLC on behalf of Alzheimer's Association. This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
Keywords: Emotion recognition, frontotemporal dementia, frontotemporal lobar degeneration, right anterior temporal lobe, semantic dementia, social cognition
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Brain Repair and Rehabilitation
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Neurodegenerative Diseases
URI: https://discovery.ucl.ac.uk/id/eprint/10197056
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