Hippocampal Sclerosis in Frontotemporal Dementia: When Vascular Pathology Meets Neurodegeneration

Anne Sieben, Tim Van Langenhove, Yannick Vermeiren, Helena Gossye, Marleen Praet, Dimitri Vanhauwaert, Céline Cousaert, Sebastiaan Engelborghs, Robrecht Raedt, Paul Boon, Patrick Santens, Peter Paul De Deyn, Ken R Bracke, Katia De Meulemeester, Christine Van Broeckhoven, Jean-Jacques Martin, Maria Bjerke

Research output: Contribution to journalArticle

Abstract

Hippocampal sclerosis (HS) is a common neuropathological finding and has been associated with advanced age, TDP-43 proteinopathy, and cerebrovascular pathology. We analyzed neuropathological data of an autopsy cohort of early-onset frontotemporal dementia patients. The study aimed to determine whether in this cohort HS was related to TDP-43 proteinopathy and whether additional factors could be identified. We examined the relationship between HS, proteinopathies in frontotemporal cortices and hippocampus, Alzheimer disease, cerebrovascular changes, and age. We confirmed a strong association between HS and hippocampal TDP-43, whereas there was a weaker association between HS and frontotemporal lobar degeneration with TDP-43 pathology (FTLD-TDP). Nearly all of the FTLD-TDP cases had TDP-43 pathology in the hippocampus. HS was present in all FTLD-TDP type D cases, in 50% of the FTLD-TDP A cohort and in 6% of the FTLD-TDP B cohort. Our data also showed a significant association between HS and vascular changes. We reviewed the literature on HS and discuss possible pathophysiological mechanisms between TDP-43 pathology, cerebrovascular disease, and HS. Additionally, we introduced a quantitative neuronal cell count in CA1 to objectify the semiquantitative visual appreciation of HS.

Original languageEnglish
Pages (from-to)313-324
Number of pages12
JournalJournal of neuropathology and experimental neurology
Volume80
Issue number4
Early online date26 Feb 2021
DOIs
Publication statusPublished - 22 Mar 2021

Bibliographical note

© 2021 American Association of Neuropathologists, Inc. All rights reserved.

Keywords

  • Cerebrovascular disease
  • FTLD-TDP
  • Frontotemporal dementia
  • Frontotemporal lobar degeneration
  • Hippocampal sclerosis
  • TDP-43

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