Hippocampal lesions in dominantly inherited Alzheimer’s disease

Robert G. Struble, Ronald J. Polinsky, John C. Hedreen, Linda E. Nee, Peter Frommelt, Robert G. Feldman, Donald L. Price

Research output: Contribution to journalArticlepeer-review

Abstract

We compared hippocampal lesions in three pedigrees of Familial Alzheimer’s Disease (FAD). In these pedigrees, the disease is inherited as an autosomal dominant disorder and has been linked to DNA markers on chromosome 21. In eight cases of FAD (four from one pedigree and two each from two others) we quantified neurofibrillary tangles (NFT) and senile plaques (SP) in hippocampal subdivisions CA1-4, subiculum, presubiculum, and dentate gyms. We observed consistent patterns of the distribution of lesions: The highest density of NFT and SP was present in CA1-2; virtually no SP or NFT were present in presubiculum; SP diameter was consistently greatest in CA4. We found no overall differences among pedigrees in total densities of NFT and SP, but statistical analyses disclosed that an uncommon type of SP was disproportionately present in two pedigrees. This type of SP was usually restricted to CA4, had a marked amyloid core devoid of argyrophilic neurites. These studies also disclosed inter- and intrafamilial heterogeneity of lesion distribution (including congophilic angiopathy and cerebellar plaques) in these three pedigrees.

Original languageEnglish (US)
Pages (from-to)82-94
Number of pages13
JournalJournal of neuropathology and experimental neurology
Volume50
Issue number1
DOIs
StatePublished - Jan 1991

Keywords

  • Alzheimer’s disease, familial
  • Hippocampus
  • Neurofibrillary tangles
  • Senile plaques

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

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