1. Clinical recognition of frontotemporal dementia with right anterior temporal predominance: A multicenter retrospective cohort study
- Author
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Ulugut, Hulya, Bertoux, Maxime, Younes, Kyan, Montembeault, Maxime, Fumagalli, Giorgio G, Samanci, Bedia, Illán‐Gala, Ignacio, Kuchcinski, Gregory, Leroy, Melanie, Thompson, Jennifer C, Kobylecki, Christopher, Santillo, Alexander F, Englund, Elisabet, Waldö, Maria Landqvist, Riedl, Lina, Van den Stock, Jan, Vandenbulcke, Mathieu, Vandenberghe, Rik, Laforce, Robert, Ducharme, Simon, Pressman, Peter S, Caramelli, Paulo, de Souza, Leonardo Cruz, Takada, Leonel T, Gurvit, Hakan, Hansson, Oskar, Diehl‐Schmid, Janine, Galimberti, Daniela, Pasquier, Florence, Miller, Bruce L, Scheltens, Philip, Ossenkoppele, Rik, van der Flier, Wiesje M, Barkhof, Frederik, Fox, Nick C, Sturm, Virginia E, Miyagawa, Toji, Whitwell, Jennifer L, Boeve, Bradley, Rohrer, Jonathan D, Gorno‐Tempini, Maria Luisa, Josephs, Keith A, Snowden, Julie, Warren, Jason D, Rankin, Katherine P, Pijnenburg, Yolande AL, and Group, International rtvFTD Working
- Subjects
Biological Psychology ,Biomedical and Clinical Sciences ,Psychology ,Acquired Cognitive Impairment ,Dementia ,Neurosciences ,Alzheimer's Disease Related Dementias (ADRD) ,Aging ,Neurodegenerative ,Clinical Research ,Mental Health ,Basic Behavioral and Social Science ,Frontotemporal Dementia (FTD) ,Rare Diseases ,Alzheimer's Disease including Alzheimer's Disease Related Dementias (AD/ADRD) ,Brain Disorders ,Behavioral and Social Science ,Mental health ,Neurological ,Good Health and Well Being ,Humans ,Male ,Frontotemporal Dementia ,Retrospective Studies ,Female ,Temporal Lobe ,Aged ,Middle Aged ,Neuropsychological Tests ,Atrophy ,emotion recognition ,frontotemporal dementia ,frontotemporal lobar degeneration ,right anterior temporal lobe ,semantic dementia ,social cognition ,International rtvFTD Working Group ,Clinical Sciences ,Geriatrics ,Clinical sciences ,Biological psychology - Abstract
IntroductionAlthough 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.MethodsRetrospective clinical data from 18 centers across 12 countries yielded 360 FTD patients with predominant RATL atrophy through initial neuroimaging assessments.ResultsCommon 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.DiscussionThis 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.HighlightsThis 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.
- Published
- 2024