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Review
. 2022 Mar;21(3):258-272.
doi: 10.1016/S1474-4422(21)00341-0.

Advances and controversies in frontotemporal dementia: diagnosis, biomarkers, and therapeutic considerations

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Review

Advances and controversies in frontotemporal dementia: diagnosis, biomarkers, and therapeutic considerations

Bradley F Boeve et al. Lancet Neurol. 2022 Mar.

Abstract

Frontotemporal dementia comprises a group of clinical syndromes that are characterised by progressive changes in behaviour, executive function, or language. The term frontotemporal lobar degeneration encompasses the neurodegenerative diseases that give rise to these clinical syndromes and involve proteinopathies associated with frontotemporal network dysfunction. Improvements in clinical, genetic, and molecular characterisation have provided new insights into frontotemporal dementia and frontotemporal lobar degeneration, with a much broader range of signs and symptoms at presentation than has been previously considered. Accurate and early diagnosis of frontotemporal dementia is now a possibility due to development of neuropsychological measures with a special focus on social cognition. Advances in plasma and CSF biomarkers, and innovations in structural and functional imaging, will prove useful for future clinical trials in people with frontotemporal dementia.

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Conflict of interest statement

Declaration of interests BFB has served as an investigator for clinical trials sponsored by Alector, Biogen, and EIP Pharma; serves on the scientific advisory board for the Tau Consortium; and receives funding from the US National Institutes of Health (NIH), the Mangurian Foundation, and the Little Family Foundation. ALB receives research support from the NIH, the Rainwater Charitable Foundation, the Association for Frontotemporal Degeneration, Bluefield Project to Cure Frontotemporal Dementia, the Alzheimer's Drug Discovery Foundation, and the Alzheimer's Association; has served as a consultant for Alector, Applied Genetic Technologies Corporation, Arkuda, Arvinas, AZTherapies, GlaxoSmithKline, Oligomerix, Ono, Regeneron, Roche, Samumed, Stealth, Third Rock, Transposon, TrueBinding, and Wave; and has received research support from Biogen, Eisai, and Regeneron. FK receives funding from the Australian National Health and Medical Research Council. YP receives funding from the Dioraphte Foundation, Health Holland, and ZonMW. JDR has served on the medical advisory board for Alector, Wave Life Sciences, Arkuda Therapeutics, and Prevail Therapeutics; receives funding from the UK Medical Research Council, UK National Institute for Health Research, Association for Frontotemporal Degeneration, Alzheimer's Association, and the Bluefield Project.

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