Nonprogressive behavioural frontotemporal dementia:Recent developments and clinical implications of the 'bvFTD phenocopy syndrome'

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Kipps, Christopher M., Hodges, John R. and Hornberger, Michael ORCID: https://orcid.org/0000-0002-2214-3788 (2010) Nonprogressive behavioural frontotemporal dementia:Recent developments and clinical implications of the 'bvFTD phenocopy syndrome'. Current Opinion in Neurology, 23 (6). pp. 628-632. ISSN 1350-7540

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Abstract

PURPOSE OF REVIEW: The clinical features of behavioural variant frontotemporal dementia (bvFTD) are well established; however, recent work has identified patients fulfilling diagnostic criteria for the disease who do not appear to progress clinically. This review describes means of distinguishing this group at an early stage from patients who are likely to deteriorate. RECENT FINDINGS: Despite indistinguishable clinical profiles, studies in a cohort of bvFTD patients showed a particularly good prognosis in a subgroup of predominantly male patients in whom initial structural imaging was normal. This could not be explained by differences in disease duration, and was confirmed by subsequent PET studies. Retrospective review of clinical data in these groups verified that the current clinical diagnostic criteria are both insensitive to true progressive bvFTD, particularly in the early stages, and also poorly specific. In contrast, measures of activity of daily living performance, executive function and tests of social cognition appear to have better discriminatory value for patients who show clear clinical progression, with many individual diagnoses verified by post mortem examination in this group. SUMMARY: It remains doubtful that the nonprogressive group have a neurodegenerative disease. The implication for the current clinical diagnostic criteria and their proposed revision is discussed.

Item Type:	Article
Uncontrolled Keywords:	diagnosis,frontotemporal dementia,imaging,neuropsychology,phenocopy
Faculty \ School:	Faculty of Medicine and Health Sciences > Norwich Medical School
UEA Research Groups:	Faculty of Medicine and Health Sciences > Research Groups > Mental Health Faculty of Medicine and Health Sciences > Research Centres > Lifespan Health
Related URLs:	http://www.scopus.com/inward/record.url?...
Depositing User:	Pure Connector
Date Deposited:	05 Jan 2016 10:01
Last Modified:	19 Oct 2023 01:36
URI:	https://ueaeprints.uea.ac.uk/id/eprint/55988
DOI:	10.1097/WCO.0b013e3283404309

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