Abstract
The diagnostic hallmarks of hippocampal sclerosis (HS) are severe volume loss of the hippocampus, severe neuronal loss, and reactive gliosis involving primarily two especially vulnerable fields, CA1 and the subiculum. Occasionally, HS may be the only neuropathological change detected in older individuals with dementia and is known as pure HS. In the majority of cases, HS occurs in the setting of other degenerative changes, usually Alzheimer’s disease (AD). In these cases, it is classified as combined HS. Although a clinical profile for HS has been identified, its similarities with AD make the diagnosis during life quite challenging; thus, the diagnosis is often made postmortem. The pathogenesis of HS is not completely understood, but the strong association with transactive response DNA-binding protein 43 (TDP-43), in approximately 90 %, and the recent discovery of genetic risk factors are important contributions to a better understanding of the disease process.
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This article is part of the Topical Collection on Dementia
Juliana R. Dutra and Etty P. Cortés contributed equally to this work.
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Dutra, J.R., Cortés, E.P. & Vonsattel, J.P.G. Update on Hippocampal Sclerosis. Curr Neurol Neurosci Rep 15, 67 (2015). https://doi.org/10.1007/s11910-015-0592-7
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DOI: https://doi.org/10.1007/s11910-015-0592-7