AUTHOR=Fieldhouse Jay L. P. , van Paassen Dirk N. , van Engelen Marie-Paule E. , De Boer Sterre C. M. , Hartog Willem L. , Braak Simon , Schoonmade Linda J. , Schouws Sigfried N. T. M. , Krudop Welmoed A. , Oudega Mardien L. , Mutsaerts Henk J. M. M. , Teunissen Charlotte E. , Vijverberg Everard G. B. , Pijnenburg Yolande A. L. TITLE=The pursuit for markers of disease progression in behavioral variant frontotemporal dementia: a scoping review to optimize outcome measures for clinical trials JOURNAL=Frontiers in Aging Neuroscience VOLUME=16 YEAR=2024 URL=https://www.frontiersin.org/journals/aging-neuroscience/articles/10.3389/fnagi.2024.1382593 DOI=10.3389/fnagi.2024.1382593 ISSN=1663-4365 ABSTRACT=

Behavioral variant frontotemporal dementia (bvFTD) is a neurodegenerative disorder characterized by diverse and prominent changes in behavior and personality. One of the greatest challenges in bvFTD is to capture, measure and predict its disease progression, due to clinical, pathological and genetic heterogeneity. Availability of reliable outcome measures is pivotal for future clinical trials and disease monitoring. Detection of change should be objective, clinically meaningful and easily assessed, preferably associated with a biological process. The purpose of this scoping review is to examine the status of longitudinal studies in bvFTD, evaluate current assessment tools and propose potential progression markers. A systematic literature search (in PubMed and Embase.com) was performed. Literature on disease trajectories and longitudinal validity of frequently-used measures was organized in five domains: global functioning, behavior, (social) cognition, neuroimaging and fluid biomarkers. Evaluating current longitudinal data, we propose an adaptive battery, combining a set of sensitive clinical, neuroimaging and fluid markers, adjusted for genetic and sporadic variants, for adequate detection of disease progression in bvFTD.