Clinical recognition of frontotemporal dementia with right anterior temporal predominance: A multicenter retrospective cohort study. Ulugut, H., Bertoux, M., Younes, K., Montembeault, M., Fumagalli, G. G, Samanci, B., Illán-Gala, I., Kuchcinski, G., Leroy, M., Thompson, J. C, Kobylecki, C., Santillo, A. F, Englund, E., Waldö, M. L., Riedl, L., Van den Stock, J., Vandenbulcke, M., Vandenberghe, R., Laforce, R. J., Ducharme, S., Pressman, P. S, Caramelli, P., de Souza, L. C., Takada, L. T, Gurvit, H., Hansson, O., Diehl-Schmid, J., Galimberti, D., Pasquier, F., Miller, B. L, Scheltens, P., Ossenkoppele, R., van der Flier, W. M, Barkhof, F., Fox, N. C, Sturm, V. E, Miyagawa, T., Whitwell, J. L, Boeve, B., Rohrer, J. D, Gorno-Tempini, M. L., Josephs, K. A, Snowden, J., Warren, J. D, Rankin, K. P, & Pijnenburg, Y. A L Alzheimer's & dementia : the journal of the Alzheimer's Association, 20(8):5647–5661, aug, 2024.
doi  abstract   bibtex   
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.
@article{Ulugut2024,
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.},
author = {Ulugut, Hulya and Bertoux, Maxime and Younes, Kyan and Montembeault, Maxime and Fumagalli, Giorgio G and Samanci, Bedia and Ill{\'{a}}n-Gala, Ignacio and Kuchcinski, Gregory and Leroy, Melanie and Thompson, Jennifer C and Kobylecki, Christopher and Santillo, Alexander F and Englund, Elisabet and Wald{\"{o}}, Maria Landqvist and Riedl, Lina and {Van den Stock}, Jan and Vandenbulcke, Mathieu and Vandenberghe, Rik and Laforce, Robert Jr 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},
doi = {10.1002/alz.14076},
institution = {International rtvFTD Working Group},
issn = {1552-5279 (Electronic)},
journal = {Alzheimer's & dementia : the journal of the Alzheimer's Association},
keywords = {Aged,Atrophy,Female,Frontotemporal Dementia,Humans,Male,Middle Aged,Neuropsychological Tests,Retrospective Studies,Temporal Lobe,diagnosis,diagnostic imaging,pathology,statistics & numerical data},
language = {eng},
month = {aug},
number = {8},
pages = {5647--5661},
pmid = {38982845},
title = {{Clinical recognition of frontotemporal dementia with right anterior temporal  predominance: A multicenter retrospective cohort study.}},
volume = {20},
year = {2024}
}

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