title: Update on Hippocampal Sclerosis.
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.
authors: Division of Aging and Dementia, Department of Neurology, Columbia University Medical Center, 622 West 168th Street, PH-19, Room 121, New York, NY, 10032, USA, jd3109@cumc.columbia.edu.
authors: Juliana R
authors: JR
authors: Dutra
authors: Etty P
authors: EP
authors: Cortés
authors: Jean Paul G
authors: JP
authors: Vonsattel
mesh_terms: D004351:Drug Resistance; D006624:Hippocampus / Q000473:pathology*; D006801:Humans; D009483:Neuropsychological Tests; D012307:Risk Factors; D012598:Sclerosis / Q000188:drug therapy / Q000473:pathology* / Q000523:psychology