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


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.

SUBMITTER: Ighodaro ET 

PROVIDER: S-EPMC4539239 | biostudies-literature | 2015 Jul

REPOSITORIES: biostudies-literature

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

Ighodaro Eseosa T ET   Jicha Gregory A GA   Schmitt Frederick A FA   Neltner Janna H JH   Abner Erin L EL   Kryscio Richard J RJ   Smith Charles D CD   Duplessis Taylor T   Anderson Sonya S   Patel Ela E   Bachstetter Adam A   Van Eldik Linda J LJ   Nelson Peter T PT  

Journal of neuropathology and experimental neurology 20150701 7


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 gliosi  ...[more]

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