Document Detail


TDP-43 pathology in primary progressive aphasia and frontotemporal dementia with pathologic Alzheimer disease.
MedLine Citation:
PMID:  20361198     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The clinical syndrome of primary progressive aphasia (PPA) can be associated with a variety of neuropathologic diagnoses at autopsy. Thirty percent of cases have Alzheimer disease (AD) pathology, most often in the usual distribution, which defies principles of brain-behavior organization, in that aphasia is not symptomatic of limbic disease. The present study investigated whether concomitant TDP-43 pathology could resolve the lack of clinico-anatomic concordance. In this paper, 16 cases of clinical PPA and 10 cases of primarily non-aphasic frontotemporal dementia (FTD), all with AD pathology, were investigated to determine whether their atypical clinical phenotypes reflected the presence of additional TDP-43 pathology. A comparison group consisted of 27 cases of pathologic AD with the typical amnestic clinical phenotype of probable AD. Concomitant TDP-43 pathology was discovered in only three of the FTD and PPA but in more than half of the typical amnestic clinical phenotypes. Hippocampal sclerosis (HS) was closely associated with TDP-43 pathology when all groups were combined for analysis. Therefore, the clinical phenotypes of PPA and FTD in cases with pathologic AD are only rarely associated with TDP-43 proteinopathy. Furthermore, medial temporal TDP-43 pathology is more tightly linked to HS than to clinical phenotype. These findings challenge the current notions about clinicopathologic correlation, especially about the role of multiple pathologies.
Authors:
Eileen H Bigio; Manjari Mishra; Kimmo J Hatanpaa; Charles L White; Nancy Johnson; Alfred Rademaker; Bing Bing Weitner; Han-Xiang Deng; Steven D Dubner; Sandra Weintraub; Marsel Mesulam
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2010-04-02
Journal Detail:
Title:  Acta neuropathologica     Volume:  120     ISSN:  1432-0533     ISO Abbreviation:  Acta Neuropathol.     Publication Date:  2010 Jul 
Date Detail:
Created Date:  2010-06-21     Completed Date:  2010-10-14     Revised Date:  2014-09-14    
Medline Journal Info:
Nlm Unique ID:  0412041     Medline TA:  Acta Neuropathol     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  43-54     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Aged
Alzheimer Disease / genetics,  metabolism,  pathology*
Aphasia, Primary Progressive / genetics,  metabolism,  pathology*
Apolipoprotein E4 / genetics,  metabolism
Brain / metabolism,  pathology*
DNA-Binding Proteins / metabolism
Female
Frontotemporal Dementia / genetics,  metabolism,  pathology*
Gliosis / genetics,  metabolism,  pathology
Hippocampus / metabolism,  pathology
Humans
Male
Middle Aged
Neurons / metabolism,  pathology
Organ Size
Phenotype
Sclerosis / genetics,  metabolism,  pathology
TDP-43 Proteinopathies / genetics,  metabolism,  pathology*
Temporal Lobe / metabolism,  pathology
Grant Support
ID/Acronym/Agency:
AG12300/AG/NIA NIH HHS; AG13854/AG/NIA NIH HHS; P30 AG012300/AG/NIA NIH HHS; P30 AG013854-149003/AG/NIA NIH HHS; R01 DC008552/DC/NIDCD NIH HHS; R01 DC008552-04/DC/NIDCD NIH HHS
Chemical
Reg. No./Substance:
0/Apolipoprotein E4; 0/DNA-Binding Proteins; 0/protein TDP-43
Comments/Corrections

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