| Hippocampal sclerosis in the elderly: genetic and pathologic findings, some mimicking Alzheimer disease clinically. | |
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MedLine Citation:
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PMID: 21346515 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Hippocampal sclerosis (HpScl) in the elderly is often associated with neurodegeneration. METHODS: We studied the clinical and pathologic features of HpScl in 205 consecutive patients with dementia who came to autopsy from 1997 to 2008, focusing on associations with TAR DNA-binding protein 43 (TDP-43) pathology and allelic variants in the progranulin (GRN) and apolipoprotein E (APOE). RESULTS: Of the 205 dementia patients, 28 had HpScl (14%). TDP-43 pathology was more frequent in cases with HpScl compared with those without HpScl (89% vs. 24%). GRN rs5848 T-allele but not APOE ε4 was associated with HpScl. In cases of HpScl with TDP-43 pathology and age of onset after 75 years (n=11), 8 had Alzheimer disease (AD)-like amnestic syndrome, but most (6 of 8) had pathology not consistent with AD (Braak stage III or less), including 4 with frontotemporal lobar degeneration with TDP, 1 with diffuse Lewy body disease, and 1 with "pure HpScl." CONCLUSIONS: HpScl is common in an elderly cohort with dementia, occurring in 14% of the cases in this series, and 89% have TDP-43 pathology, often associated with a risk variant in GRN. Patients with HpScl who present after the age of 75 years often have presentations consistent with AD, but at autopsy have non-Alzheimer pathologies. Elderly patients with HpScl may be mistaken for AD. |
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Authors:
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Winnie C Pao; Dennis W Dickson; Julia E Crook; NiCole A Finch; Rosa Rademakers; Neill R Graff-Radford |
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Publication Detail:
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Type: Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Alzheimer disease and associated disorders Volume: 25 ISSN: 1546-4156 ISO Abbreviation: Alzheimer Dis Assoc Disord Publication Date: 2011 Oct |
Date Detail:
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Created Date: 2011-11-17 Completed Date: 2012-08-13 Revised Date: 2013-05-26 |
Medline Journal Info:
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Nlm Unique ID: 8704771 Medline TA: Alzheimer Dis Assoc Disord Country: United States |
Other Details:
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Languages: eng Pagination: 364-8 Citation Subset: IM |
Affiliation:
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Department of Neurology, Mayo Clinic, Jacksonville, FL, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Aged Aged, 80 and over Alzheimer Disease / diagnosis, genetics*, pathology* Diagnosis, Differential Female Hippocampus / pathology* Humans Longitudinal Studies Male Middle Aged Prospective Studies Sclerosis |
| Grant Support | |
ID/Acronym/Agency:
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P01AG03949/AG/NIA NIH HHS; P01AG17216/AG/NIA NIH HHS; P50 AG016574-12/AG/NIA NIH HHS; P50AG16574/AG/NIA NIH HHS |
| Comments/Corrections | |
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