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

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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.

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Ighodaro, E. T., Jicha, G. A., Schmitt, F. A., Neltner, J. H., Abner, E. L., Kryscio, R. J., … Nelson, P. T. (2015, July 29). Hippocampal Sclerosis of Aging Can Be Segmental: Two Cases and Review of the Literature. Journal of Neuropathology and Experimental Neurology. Lippincott Williams and Wilkins. https://doi.org/10.1097/NEN.0000000000000204

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