Conceptual framework for the definition of preclinical and prodromal frontotemporal dementia

Alberto Benussi, Antonella Alberici, GENFI Consortium (-investigators), Kiran Samra, Lucy L. Russell, Caroline V. Greaves, Martina Bocchetta, Simon Ducharme, Elizabeth Finger, Giorgio Fumagalli, Daniela Galimberti, Lize C. Jiskoot, Isabelle Le Ber, Mario Masellis, Benedetta Nacmias, James B. Rowe, Raquel Sanchez-Valle, Harro Seelaar, Matthis Synofzik, Jonathan D. Rohrer*Barbara Borroni, Sónia Afonso, Maria Rosario Almeida, Sarah Anderl-Straub, Christin Andersson, Anna Antonell, Silvana Archetti, Andrea Arighi, Mircea Balasa, Myriam Barandiaran, Nuria Bargalló, Robart Bartha, Benjamin Bender, Maxime Bertoux, Anne Bertrand, Valentina Bessi, Sandra Black, Sergi Borrego-Ecija, Arabella Bouzigues, Jose Bras, Alexis Brice, Rose Bruffaerts, Chris R. Butler, Agnès Camuzat, Marta Cañada, Valentina Cantoni, Lieke Meeter, Rick van Minkelen, Jessica Panman, Janne M. Papma, John C. van Swieten

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

33 Citations (Scopus)
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Abstract

The presymptomatic stages of frontotemporal dementia (FTD) are still poorly defined and encompass a long accrual of progressive biological (preclinical) and then clinical (prodromal) changes, antedating the onset of dementia. The heterogeneity of clinical presentations and the different neuropathological phenotypes have prevented a prior clear description of either preclinical or prodromal FTD. Recent advances in therapeutic approaches, at least in monogenic disease, demand a proper definition of these predementia stages. It has become clear that a consensus lexicon is needed to comprehensively describe the stages that anticipate dementia. The goal of the present work is to review existing literature on the preclinical and prodromal phases of FTD, providing recommendations to address the unmet questions, therefore laying out a strategy for operationalizing and better characterizing these presymptomatic disease stages.

Original languageEnglish
Pages (from-to)1408-1423
Number of pages16
JournalAlzheimer's and Dementia
Volume18
Issue number7
DOIs
Publication statusPublished - 1 Jul 2022

Bibliographical note

ACKNOWLEDGMENTS
This project received support from the JPND grant “GENFI-prox” to M.S, J.D.R., and B.B. M.B. is supported by a Fellowship award from the Alzheimer's Society, UK (AS-JF-19a-004-517). M.B.’s work is also supported by the UK Dementia Research Institute, which receives its funding from DRI Ltd, funded by the UK Medical Research Council, Alzheimer's Society, and Alzheimer's Research UK. J.D.R. is supported by an MRC Clinician Scientist Fellowship (MR/M008525/1) and has received funding from the NIHR Rare Disease Translational Research Collaboration (BRC149/NS/MH).

Publisher Copyright:
© 2021 The Authors. Alzheimer's & Dementia published by Wiley Periodicals LLC on behalf of Alzheimer's Association.

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