Gerstmann-Sträussler-Scheinker Disease

Bernardino Ghetti, Fabrizio Tagliavini, Gabor G. Kovacs, Pedro Piccardo

Research output: Chapter in Book/Report/Conference proceedingChapter

22 Scopus citations

Abstract

This chapter describes the clinical and neuropathological phenotypes associated with Gerstmann-Sträussler-Scheinker disease and prion protein cerebral amyloid angiopathy. The main sections are devoted to the clinical, neuropathological, biochemical and molecular genetic characteristics of this disorder. The clinical phenotypes may vary in presentation and age at onset. One table shows the genetic mutations associated with Gerstmann-Sträussler-Scheinker disease and prion protein cerebral amyloid angiopathy; another shows the main neuropathological and biochemical characteristics associated with each mutation.

Original languageEnglish (US)
Title of host publicationNeurodegeneration
Subtitle of host publicationThe Molecular Pathology of Dementia and Movement Disorders: Second Edition
PublisherWiley-Blackwell
Pages364-377
Number of pages14
ISBN (Print)9781405196932
DOIs
StatePublished - Sep 21 2011

Keywords

  • Amyloid
  • Neuritic plaques
  • Neurofibrillary tangles
  • Prion disease
  • Prion protein
  • PRNP
  • Spongiform encephalopathy
  • Tau protein
  • Transmissibility

ASJC Scopus subject areas

  • Medicine(all)

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