| TDP-43 pathology in primary progressive aphasia and frontotemporal dementia with pathologic Alzheimer disease. | |
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MedLine Citation:
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PMID: 20361198 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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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:
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Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't Date: 2010-04-02 |
Journal Detail:
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Title: Acta neuropathologica Volume: 120 ISSN: 1432-0533 ISO Abbreviation: Acta Neuropathol. Publication Date: 2010 Jul |
Date Detail:
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Created Date: 2010-06-21 Completed Date: 2010-10-14 Revised Date: 2011-09-26 |
Medline Journal Info:
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Nlm Unique ID: 0412041 Medline TA: Acta Neuropathol Country: Germany |
Other Details:
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Languages: eng Pagination: 43-54 Citation Subset: IM |
Affiliation:
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Cognitive Neurology and Alzheimer Disease Center, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA. e-bigio@northwestern.edu |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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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:
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AG12300/AG/NIA NIH HHS; AG13854/AG/NIA NIH HHS; P30 AG012300/AG/NIA NIH HHS; P30 AG013854-149003/AG/NIA NIH HHS; R01 DC008552-04/DC/NIDCD NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Apolipoprotein E4; 0/DNA-Binding Proteins; 0/protein TDP-43 |
| Comments/Corrections | |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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