Hippocampal Sclerosis of Aging Can Be Segmental: Two Cases and Review of the Literature

J Neuropathol Exp Neurol. 2015 Jul;74(7):642-52. doi: 10.1097/NEN.0000000000000204.

Abstract

Hippocampal sclerosis of aging (HS-Aging) is a neurodegenerative disease that mimics Alzheimer disease (AD) clinically and has a prevalence rivaling AD in advanced age. Whereas clinical biomarkers are not yet optimized, HS-Aging has distinctive pathological features that distinguish it from other diseases with "hippocampal sclerosis" pathology, such as epilepsy, cerebrovascular perturbations, and frontotemporal lobar degeneration. By definition, HS-Aging brains show neuronal cell loss and gliosis in the hippocampal formation out of proportion to AD-type pathology; it is strongly associated with aberrant TDP-43 pathology and arteriolosclerosis. Here, we describe 2 cases of "segmental" HS-Aging in which "sclerosis" in the hippocampus was evident only in a subset of brain sections by hematoxylin and eosin (H&E) stain. In these cases, TDP-43 pathology was more widespread on immunostained sections than the neuronal cell loss and gliosis seen in H&E stains. The 2 patients were cognitively intact at baseline and were tracked longitudinally over a decade using cognitive studies with at least 1 neuroimaging scan. We discuss the relevant HS-Aging literature, which indicates the need for a clearer consensus-based delineation of "hippocampal sclerosis" and TDP-43 pathologies in aged subjects.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Aged
  • Aged, 80 and over
  • Aging / pathology*
  • DNA-Binding Proteins / metabolism
  • Female
  • Hippocampus / pathology*
  • Humans
  • Male
  • Neurodegenerative Diseases / complications*
  • Neurodegenerative Diseases / pathology
  • Sclerosis / etiology
  • Sclerosis / pathology

Substances

  • DNA-Binding Proteins