Acta Neuropathologica

, Volume 84, Issue 1, pp 15–23 | Cite as

Gerstmann-Sträussler syndrome — A variant type: amyloid plaques and Alzheimer's neurofibrillary tangles in cerebral cortex

  • Naoji Amano
  • Saburo Yagishita
  • Susumu Yokoi
  • Yoji Itoh
  • Jun Kinoshita
  • Toshio Mizutani
  • Takeshi Matsuishi
Regular Papers


This report presents a variant of Gerstmann-Sträussler syndrome (GSS). A 53-year-old female had developed slowly progressive dementia and atactic gait since the age of 45. No myoclonic jerks and periodic synchronous discharges were observed throughout the illness. The neuropathological study revealed that many amyloid plaques and widespread Alzheimer's neurofibrillary tangles (NFTs) appeared in the cerebral cortex. Characteristically, the plaques reacted with anti-prion protein and none of them reacted with anti-β protein, and they were made of many components, including amyloid cores, macrophages laden with lipid granules and/or degenerated neurites. Neuropil threads were seen mainly in amyloid plaques. Moreover, plaques appeared which were confluent and laminar in arrangement in the fifth and sixth cortical layers and had a close relationship to the neuronal loss. There was no spongiform change in the cerebral cortex or cerebellum. The cerebellum was almost intact except for a few amyloid plaques. Ultrastructurally, some of the plaques simulated kuru plaques and others had many degenerated neurites possessing paired helical filaments and other accumulated organelles. GSS has been proposed to include cases with progressive ataxia, dementia and massive multifocal plaques in the brain with or without cerebral spongiform changes. The case presented here is a very peculiar case of GSS. Recently, similar cases have been reported in some large families, diagnosed as familial Alzheimer's disease. These cases may be a telencephalic form with numerous NFTs of GSS.

Key words

Gerstmann-Sträussler syndrome Variant Amyloid plaques Alzheimer's neurofibrillary tangles Prion protein 


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Copyright information

© Springer-Verlag 1992

Authors and Affiliations

  • Naoji Amano
    • 1
    • 2
  • Saburo Yagishita
    • 1
    • 2
  • Susumu Yokoi
    • 1
    • 2
  • Yoji Itoh
    • 1
    • 2
  • Jun Kinoshita
    • 3
  • Toshio Mizutani
    • 4
  • Takeshi Matsuishi
    • 5
  1. 1.Division of Neuro-psychiatryThe Kanagawa Rehabilitation CenterAtsugi, KanagawaJapan
  2. 2.Division of PathologyThe Kanagawa Rehabilitation CenterAtsugi, KanagawaJapan
  3. 3.Takanedai HospitalHiratsuka, KanagawaJapan
  4. 4.Department of NeuropathologyTokyo Metropolitan Institute of GerontologyTokyoJapan
  5. 5.Faculty of EducationYokohama National UniversityYokohama, KanagawaJapan

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