Hippocampal sclerosis in the elderly: Genetic and pathologic findings, some mimicking Alzheimer disease clinically

Winnie C. Pao, Dennis W. Dickson, Julia E. Crook, Nicole A. Finch, Rosa Rademakers, Neill R. Graff-Radford

Research output: Contribution to journalArticlepeer-review

58 Scopus citations

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 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.

Original languageEnglish (US)
Pages (from-to)364-368
Number of pages5
JournalAlzheimer disease and associated disorders
Volume25
Issue number4
DOIs
StatePublished - Oct 2011

Keywords

  • Alzheimer disease
  • amnestic syndrome
  • hippocampal sclerosis

ASJC Scopus subject areas

  • Clinical Psychology
  • Gerontology
  • Geriatrics and Gerontology
  • Psychiatry and Mental health

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