TY - JOUR
T1 - Hippocampal Sclerosis of Aging Can Be Segmental
T2 - Two Cases and Review of the Literature
AU - Ighodaro, Eseosa T.
AU - Jicha, Gregory A.
AU - Schmitt, Frederick A.
AU - Neltner, Janna H.
AU - Abner, Erin L.
AU - Kryscio, Richard J.
AU - Smith, Charles D.
AU - Duplessis, Taylor
AU - Anderson, Sonya
AU - Patel, Ela
AU - Bachstetter, Adam
AU - Van Eldik, Linda J.
AU - Nelson, Peter T.
N1 - Publisher Copyright:
Copyright © 2015 by the American Association of Neuropathologists, Inc.
PY - 2015/7/29
Y1 - 2015/7/29
N2 - 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.
AB - 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.
KW - Biomarkers
KW - Case study
KW - Dementia
KW - Frontotemporal lobar degeneration (FTLD)
KW - Hippocampal sclerosis
KW - Neuropathology
KW - Oldest-old
UR - http://www.scopus.com/inward/record.url?scp=84934292543&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84934292543&partnerID=8YFLogxK
U2 - 10.1097/NEN.0000000000000204
DO - 10.1097/NEN.0000000000000204
M3 - Review article
C2 - 26083567
AN - SCOPUS:84934292543
SN - 0022-3069
VL - 74
SP - 642
EP - 652
JO - Journal of Neuropathology and Experimental Neurology
JF - Journal of Neuropathology and Experimental Neurology
IS - 7
ER -