Document Detail

Nonprogressive behavioural frontotemporal dementia: recent developments and clinical implications of the 'bvFTD phenocopy syndrome'.
MedLine Citation:
PMID:  20962637     Owner:  NLM     Status:  MEDLINE    
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.
Christopher M Kipps; John R Hodges; Michael Hornberger
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Current opinion in neurology     Volume:  23     ISSN:  1473-6551     ISO Abbreviation:  Curr. Opin. Neurol.     Publication Date:  2010 Dec 
Date Detail:
Created Date:  2010-11-05     Completed Date:  2011-09-06     Revised Date:  2014-02-20    
Medline Journal Info:
Nlm Unique ID:  9319162     Medline TA:  Curr Opin Neurol     Country:  England    
Other Details:
Languages:  eng     Pagination:  628-32     Citation Subset:  IM    
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MeSH Terms
Diagnosis, Differential
Diagnostic Imaging / methods*,  trends
Disease Progression
Frontotemporal Dementia / diagnosis*,  mortality,  psychology*
Genetics, Behavioral / methods,  trends
Neurodegenerative Diseases / diagnosis
Retrospective Studies
Grant Support
G9724461//Medical Research Council

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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