Multicenter Study

. 2024 Aug;20(8):5647-5661.

doi: 10.1002/alz.14076. Epub 2024 Jul 10.

Clinical recognition of frontotemporal dementia with right anterior temporal predominance: A multicenter retrospective cohort study

Hulya Ulugut^{1

2}, Maxime Bertoux³, Kyan Younes^{2

4}, Maxime Montembeault^{2

5}, Giorgio G Fumagalli^{6

7}, Bedia Samanci⁸, Ignacio Illán-Gala^{9

10}, Gregory Kuchcinski³, Melanie Leroy³, Jennifer C Thompson^{11

12}, Christopher Kobylecki^{13

14}, Alexander F Santillo¹⁵, Elisabet Englund¹⁶, Maria Landqvist Waldö¹⁷, Lina Riedl¹⁸, Jan Van den Stock¹⁹, Mathieu Vandenbulcke¹⁹, Rik Vandenberghe²⁰, Robert Laforce Jr²¹, Simon Ducharme⁵, Peter S Pressman²², Paulo Caramelli²³, Leonardo Cruz de Souza²³, Leonel T Takada²⁴, Hakan Gurvit⁸, Oskar Hansson¹⁵, Janine Diehl-Schmid^{18

25}, Daniela Galimberti^{26

27}, Florence Pasquier³, Bruce L Miller², Philip Scheltens¹, Rik Ossenkoppele^{1

28}, Wiesje M van der Flier¹, Frederik Barkhof^{28

29}, Nick C Fox³⁰, Virginia E Sturm², Toji Miyagawa³¹, Jennifer L Whitwell³², Bradley Boeve³¹, Jonathan D Rohrer³⁰, Maria Luisa Gorno-Tempini^{2

33}, Keith A Josephs³¹, Julie Snowden^{11

12}, Jason D Warren³⁰, Katherine P Rankin², Yolande A L Pijnenburg¹; International rtvFTD Working Group

Affiliations

¹ Alzheimer Center Amsterdam, Department of Neurology, Amsterdam Neuroscience, Vrije Universiteit Amsterdam, Amsterdam UMC, De Boelelaan, Amsterdam, The Netherlands.
² Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, California, USA.
³ Lille Neuroscience & Cognition U1172, Univ. Lille, Inserm, CHU Lille, LiCEND & Labex DistALZ, Lille, France.
⁴ Stanford Neuroscience Health Center, Department of Neurology, Stanford University, Palo Alto, California, USA.
⁵ Department of Psychiatry, Douglas Mental Health University Institute, McGill University Health Centre, McGill University, Montreal, Quebec, Canada.
⁶ Department of Neurology, University of Milan, Milan, Italy.
⁷ Università degli Studi di Trento | UNITN·CIMEC - Center for Mind/Brain Sciences, Mattarello, Trentino, Italy.
⁸ Department of Neurology, Istanbul University, Fatih, Istanbul, Turkey.
⁹ Sant Pau Memory Unit, Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain.
¹⁰ Centro de Investigación en Red-Enfermedades Neurodegenerativas (CIBERNED), Madrid, Spain.
¹¹ Cerebral Function Unit, Greater Manchester Neuroscience Centre, Salford Royal NHS Foundation Trust, Salford, UK.
¹² Division of Neuroscience and Experimental Psychology, Faculty of Biology, Medicine and Health, University of Manchester, Salford, Manchester, UK.
¹³ Department of Neurology, Manchester Centre for Clinical Neurosciences NHS Foundation Trust, Salford, UK.
¹⁴ Division of Neuroscience, University of Manchester, Salford, Manchester, UK.
¹⁵ Clinical Memory Research Unit, Department of Clinical Sciences, Faculty of Medicine, Lund University, Lund, Sweden.
¹⁶ Division of Pathology, Department of Clinical Sciences, Lund University, Lund, Sweden.
¹⁷ Division of Clinical Sciences Helsingborg, Department of Clinical Sciences Lund, Lund University, Lund, Sweden.
¹⁸ School of Medicine, Department of Psychiatry and Psychotherapy, Technical University of Munich, Munich, Germany.
¹⁹ Neuropsychiatry, Department of Neurosciences, Leuven Brain Institute, Leuven, Belgium.
²⁰ Department of Neurology, University Hospital Leuven, Leuven, Belgium.
²¹ Clinique Interdisciplinaire de Mémoire (CIME), Département des Sciences Neurologiques, Laval University, Quebec City, Canada.
²² Anschutz Medical Campus, Behavioral Neurology Section, Department of Neurology, University of Colorado, Aurora, Colorado, USA.
²³ Behavioral and Cognitive Neurology Unit, Department of Internal Medicine, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil.
²⁴ Cognitive and Behavioral Unit, Hospital das Clinicas, Department of Neurology, University of São Paulo Medical School, Pacaembu, São Paulo, Brazil.
²⁵ Kbo-Inn-Salzach-Klinikum, Clinical Center for Psychiatry, Psychotherapy, Psychosomatic Medicine, Geriatrics and Neurology, Wasserburg/Inn, Germany.
²⁶ Department of Biomedical, Surgical and Dental Sciences, University of Milan, Milan, Italy.
²⁷ Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy.
²⁸ Alzheimer Center Amsterdam, Department of Radiology, Amsterdam Neuroscience, Vrije Universiteit Amsterdam, Amsterdam UMC, De Boelelaan, Amsterdam, The Netherlands.
²⁹ UCL Institutes of Neurology and Healthcare Engineering, University College London, London, UK.
³⁰ Dementia Research Centre, UCL Queen Square Institute of Neurology, London, UK.
³¹ Department of Neurology, Mayo Clinic, Rochester, Rochester, Minnesota, USA.
³² Department of Radiology, Mayo Clinic, Rochester, Rochester, Minnesota, USA.
³³ Dyslexia Center, University of California San Francisco, UCSF Weill Institute for Neurosciences, University of California, San Francisco, California, USA.

PMID: 38982845
PMCID: PMC11350044
DOI: 10.1002/alz.14076

Multicenter Study

Clinical recognition of frontotemporal dementia with right anterior temporal predominance: A multicenter retrospective cohort study

Hulya Ulugut et al. Alzheimers Dement. 2024 Aug.

. 2024 Aug;20(8):5647-5661.

doi: 10.1002/alz.14076. Epub 2024 Jul 10.

Authors

Affiliations

¹ Alzheimer Center Amsterdam, Department of Neurology, Amsterdam Neuroscience, Vrije Universiteit Amsterdam, Amsterdam UMC, De Boelelaan, Amsterdam, The Netherlands.
² Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, California, USA.
³ Lille Neuroscience & Cognition U1172, Univ. Lille, Inserm, CHU Lille, LiCEND & Labex DistALZ, Lille, France.
⁴ Stanford Neuroscience Health Center, Department of Neurology, Stanford University, Palo Alto, California, USA.
⁵ Department of Psychiatry, Douglas Mental Health University Institute, McGill University Health Centre, McGill University, Montreal, Quebec, Canada.
⁶ Department of Neurology, University of Milan, Milan, Italy.
⁷ Università degli Studi di Trento | UNITN·CIMEC - Center for Mind/Brain Sciences, Mattarello, Trentino, Italy.
⁸ Department of Neurology, Istanbul University, Fatih, Istanbul, Turkey.
⁹ Sant Pau Memory Unit, Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain.
¹⁰ Centro de Investigación en Red-Enfermedades Neurodegenerativas (CIBERNED), Madrid, Spain.
¹¹ Cerebral Function Unit, Greater Manchester Neuroscience Centre, Salford Royal NHS Foundation Trust, Salford, UK.
¹² Division of Neuroscience and Experimental Psychology, Faculty of Biology, Medicine and Health, University of Manchester, Salford, Manchester, UK.
¹³ Department of Neurology, Manchester Centre for Clinical Neurosciences NHS Foundation Trust, Salford, UK.
¹⁴ Division of Neuroscience, University of Manchester, Salford, Manchester, UK.
¹⁵ Clinical Memory Research Unit, Department of Clinical Sciences, Faculty of Medicine, Lund University, Lund, Sweden.
¹⁶ Division of Pathology, Department of Clinical Sciences, Lund University, Lund, Sweden.
¹⁷ Division of Clinical Sciences Helsingborg, Department of Clinical Sciences Lund, Lund University, Lund, Sweden.
¹⁸ School of Medicine, Department of Psychiatry and Psychotherapy, Technical University of Munich, Munich, Germany.
¹⁹ Neuropsychiatry, Department of Neurosciences, Leuven Brain Institute, Leuven, Belgium.
²⁰ Department of Neurology, University Hospital Leuven, Leuven, Belgium.
²¹ Clinique Interdisciplinaire de Mémoire (CIME), Département des Sciences Neurologiques, Laval University, Quebec City, Canada.
²² Anschutz Medical Campus, Behavioral Neurology Section, Department of Neurology, University of Colorado, Aurora, Colorado, USA.
²³ Behavioral and Cognitive Neurology Unit, Department of Internal Medicine, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil.
²⁴ Cognitive and Behavioral Unit, Hospital das Clinicas, Department of Neurology, University of São Paulo Medical School, Pacaembu, São Paulo, Brazil.
²⁵ Kbo-Inn-Salzach-Klinikum, Clinical Center for Psychiatry, Psychotherapy, Psychosomatic Medicine, Geriatrics and Neurology, Wasserburg/Inn, Germany.
²⁶ Department of Biomedical, Surgical and Dental Sciences, University of Milan, Milan, Italy.
²⁷ Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy.
²⁸ Alzheimer Center Amsterdam, Department of Radiology, Amsterdam Neuroscience, Vrije Universiteit Amsterdam, Amsterdam UMC, De Boelelaan, Amsterdam, The Netherlands.
²⁹ UCL Institutes of Neurology and Healthcare Engineering, University College London, London, UK.
³⁰ Dementia Research Centre, UCL Queen Square Institute of Neurology, London, UK.
³¹ Department of Neurology, Mayo Clinic, Rochester, Rochester, Minnesota, USA.
³² Department of Radiology, Mayo Clinic, Rochester, Rochester, Minnesota, USA.
³³ Dyslexia Center, University of California San Francisco, UCSF Weill Institute for Neurosciences, University of California, San Francisco, California, USA.

PMID: 38982845
PMCID: PMC11350044
DOI: 10.1002/alz.14076

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.

Keywords: emotion recognition; frontotemporal dementia; frontotemporal lobar degeneration; right anterior temporal lobe; semantic dementia; social cognition.

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Conflict of interest statement

The funders of the study had no role in study design, data collection, data analysis, data interpretation, or writing of the report. Oskar Hansson has acquired research support (for the institution) from ADx, AVID Radiopharmaceuticals, Biogen, Eli Lilly, Eisai, Fujirebio, GE Healthcare, Pfizer, and Roche. In the past 2 years, he has received consultancy/speaker fees from AC Immune, Amylyx, Alzpath, BioArctic, Biogen, Cerveau, Eisai, Eli Lilly, Fujirebio, Merck, Novartis, Novo Nordisk, Roche, Sanofi, and Siemens. Other authors declare no competing interest. Author disclosures are available in the supporting information.

Figures

**FIGURE 1**
Representation of the collected clinical data. The red line represents clinical symptoms, while the orange shadows depict associated cognitive test results available in the collected dataset. Cognitive tests and behavioral assessments were limited to smells, tastes, sounds, bodily sensations, objects, landmarks, mental rigidity, and preoccupations, which are denoted by a question mark (?) in the figure

**FIGURE 2**
Reported specific interests. One patient may have more than one symptom

See this image and copyright information in PMC

References

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1. Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain. 2011;134:2456‐2477. - PMC - PubMed
1. Gorno‐Tempini ML, Hillis AE, Weintraub S, et al. Classification of primary progressive aphasia and its variants. Neurology. 2011;76:1006‐1014. - PMC - PubMed
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Clinical recognition of frontotemporal dementia with right anterior temporal predominance: A multicenter retrospective cohort study

Affiliations

Clinical recognition of frontotemporal dementia with right anterior temporal predominance: A multicenter retrospective cohort study

Authors

Affiliations

Abstract

Conflict of interest statement

Figures

References

Publication types

MeSH terms

Grants and funding

LinkOut - more resources

Full Text Sources