Document Detail

Clinicopathological correlates in frontotemporal dementia.
MedLine Citation:
PMID:  15349867     Owner:  NLM     Status:  MEDLINE    
The term frontotemporal dementia (FTD) encompasses a range of clinical syndromes that are believed not to map reliably onto the spectrum of recognized pathologies. This study reexamines the relationships between clinical and pathological subtypes of FTD in a large series from two centers (n = 61). Clinical subtypes defined were behavioral variant FTD (n = 26), language variants (semantic dementia, n = 9; and progressive nonfluent aphasia, n = 8), and motor variants (corticobasal degeneration, n = 9; and motor neuron disease, n = 9), although most cases presented with a combination of behavioral and language problems. Unexpectedly, some behavioral cases (n = 5) had marked amnesia at presentation. The pathological subtypes were those with tau-immunopositive inclusions (with Pick bodies, n = 20; or without, n = 11), those with ubiquitin immunopositive inclusions (n = 16), and those lacking distinctive histology (n = 14). Behavioral symptoms and semantic dementia were associated with a range of pathologies. In contrast, other clinical phenotypes had relatively uniform underlying pathologies: motor neuron disease predicted ubiquitinated inclusions, parkinsonism and apraxia predicted corticobasal pathology, and nonfluent aphasia predicted Pick bodies. Therefore, the pathological substrate can be predicted in a significant proportion of FTD patients, which has important implications for studies targeting mechanistic treatments.
John R Hodges; R Rhys Davies; John H Xuereb; Barney Casey; Melissa Broe; Thomas H Bak; Jillian J Kril; Glenda M Halliday
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Annals of neurology     Volume:  56     ISSN:  0364-5134     ISO Abbreviation:  Ann. Neurol.     Publication Date:  2004 Sep 
Date Detail:
Created Date:  2004-09-06     Completed Date:  2004-10-06     Revised Date:  2014-02-19    
Medline Journal Info:
Nlm Unique ID:  7707449     Medline TA:  Ann Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  399-406     Citation Subset:  IM    
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MeSH Terms
Aged, 80 and over
Brain / pathology*
Dementia / diagnosis,  pathology*,  psychology
Diagnosis, Differential
Middle Aged
Reproducibility of Results
Retrospective Studies
tau Proteins / analysis
Grant Support
G9724461//Medical Research Council
Reg. No./Substance:
0/tau Proteins

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

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