Document Detail

Hippocampal sclerosis in the elderly: genetic and pathologic findings, some mimicking Alzheimer disease clinically.
MedLine Citation:
PMID:  21346515     Owner:  NLM     Status:  MEDLINE    
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.
Winnie C Pao; Dennis W Dickson; Julia E Crook; NiCole A Finch; Rosa Rademakers; Neill R Graff-Radford
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Alzheimer disease and associated disorders     Volume:  25     ISSN:  1546-4156     ISO Abbreviation:  Alzheimer Dis Assoc Disord     Publication Date:    2011 Oct-Dec
Date Detail:
Created Date:  2011-11-17     Completed Date:  2012-08-13     Revised Date:  2014-03-25    
Medline Journal Info:
Nlm Unique ID:  8704771     Medline TA:  Alzheimer Dis Assoc Disord     Country:  United States    
Other Details:
Languages:  eng     Pagination:  364-8     Citation Subset:  IM    
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MeSH Terms
Aged, 80 and over
Alzheimer Disease / diagnosis,  genetics*,  pathology*
Diagnosis, Differential
Hippocampus / pathology*
Longitudinal Studies
Middle Aged
Prospective Studies
Grant Support
P01 AG003949/AG/NIA NIH HHS; P01AG03949/AG/NIA NIH HHS; P01AG17216/AG/NIA NIH HHS; P50 AG016574/AG/NIA NIH HHS; P50 AG016574-12/AG/NIA NIH HHS; P50AG16574/AG/NIA NIH HHS

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