|
Title
|
|
|
|
Hippocampal Sclerosis in the Elderly Genetic and Pathologic Findings, Some Mimicking Alzheimer Disease Clinically
| |
|
Author
|
|
|
|
| |
|
Abstract
|
|
|
|
| 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 epsilon 4 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. |
| |
|
Language
|
|
|
|
English
| |
|
Source (journal)
|
|
|
|
Alzheimer disease & associated disorders : an international journal. - Hagerstown, Md, 1987, currens
| |
|
Publication
|
|
|
|
Hagerstown, Md
:
2011
| |
|
ISSN
|
|
|
|
0893-0341
[print]
1546-4156
[online]
| |
|
DOI
|
|
|
|
10.1097/WAD.0B013E31820F8F50
| |
|
Volume/pages
|
|
|
|
25
:4
(2011)
, p. 364-368
| |
|
ISI
|
|
|
|
000297109900011
| |
|
Full text (Publisher's DOI)
|
|
|
|
| |
|