Skip to main content
Book cover

Pathologie pp 164–168Cite as

Übertragbare spongiforme Enzephalopathien

  • Chapter
  • 88 Accesses

Zusammenfassung

Dieser Gruppe von Erkrankungen des ZNS, die bei Menschen und Haustieren vorkommen, ist eine ungewöhnlich lange Inkubationszeit und dann ein meist rascher tödlicher Verlauf gemeinsam. Die bekanntesten hierzu gehörigen Krankheiten, die im folgenden besprochen werden sollen, sind Kuru, die Jakob-Creutzfeldt-Erkrankung (CJD) und die Gerstmann-Sträussler-Scheinker-Krankheit (GSS) beim Menschen sowie Scrapie und die bovine spongiforme Enzephalopathie (BSE) bei Haustieren. BSE sowie weitere Formen dieser Krankheitsgruppe bei Tieren wie u. a. bei Nerzen (transmissible Mink Encephalopathy TME) u. a. werden auf Verfüttern von mit Scrapie infizierten Kadavern zurückgeführt. Sie sind unter den international tiblichen Abkürzungen aufgeftihrt in.

This is a preview of subscription content, log in via an institution.

Buying options

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   54.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD   69.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Literatur

  1. Bastian FO (1991) Creutzfeldt-lakob disease and other transmissible spongiform encephalopathies. Mosby Year Book, St. Louis, 256pp

    Google Scholar 

  2. Gajdusek DC (1990) Subacute spongiform encephalopathies: Transmissible cerebral amyloidoses caused by unconventional viruses. In: Fields BN, Knipe DM et al. (eds) Virology, 2nd ed. Raven, New York, pp2289–2324

    Google Scholar 

  3. Bell JE, Ironside JW (1993) How to tackle a possible CreutzfeldtJakob disease necropsy. J Clin Pathol 46: 193–197

    Article  PubMed  CAS  Google Scholar 

  4. Berger JR, David NJ (1993) Creutzfeldt-Jakob disease in a physician: A review of the disorder in health care workers. Neurology 43.205–206

    PubMed  CAS  Google Scholar 

  5. Boellaard JW, Schlote W, Tateishi J (1989) Neuronal autophagy in experimental Creutzfeldt-Jakob disease. Acta Neuropathol 82: 410–418

    Article  Google Scholar 

  6. Brown P (1988) Human growth hormone therapy and Creutzfeldt- Jakob disease: A drama in three acts. Pediatrics 81: 85–92

    PubMed  CAS  Google Scholar 

  7. Brown P, Cervenáková L, Goldfarb LG et al. (1994) Iatrogenic Creutzfeldt-Jakob disease: An example of the interplay between ancient genes and modern medicine. Neurology 44, 291–293

    PubMed  CAS  Google Scholar 

  8. Brown P, Goldfarb LG, Brown WT et al. (1991) Clinical and molecular genetic study of a large German kindred with Gerstmann-Sträussler-Scheinker syndrome. Neurology 41: 375–379

    PubMed  CAS  Google Scholar 

  9. Brown P, Kaur P, Sulima MP et al. (1993) Real and imagined clinicopathologicallimits of Éprion dementiaÉ. Lancet 341: 127–129

    Article  PubMed  CAS  Google Scholar 

  10. Brown P, Wolff A, Gajdusek DC (1990) A simple and effective method for inactivating virus infectivity in formalin-fixed tissue samples from patients with Creutzfeldt-Jakob disease. Neurology 40:887–890

    PubMed  CAS  Google Scholar 

  11. Büeler H, Aguzzi A, Sailer A et al. (1993) Mice devoid of PrP are resistent to scrapie. Cell 73: 1339–1347

    Article  PubMed  Google Scholar 

  12. Diringer H (1990) Unkonventionelle Viruskrankheiten. Bundesgesundhcitsblatt 33, 187–194

    Google Scholar 

  13. Diringer H (1990) Durchbrechen von Speziesbarrieren mit unkonventionellen Viren. Bundesgesundheitsblatt 33: 435–440

    Google Scholar 

  14. Gajdusek DC (1977) Unconventional viruses and the origin and disappearance of kuru. Science 197: 943–960

    Article  PubMed  CAS  Google Scholar 

  15. Ghetti B, Tagliavini F, Masters CL et al. (1989) Gerstmann Sträussler- Scheinker disease. II. Neurofibrillary tangles and plaques with PrP-amyloid coexist in an affected family. Neurology 39,1453–1461

    PubMed  CAS  Google Scholar 

  16. Goldfarb L, Mitrova E, Brown P, Toh BH, Gajdusek DC (1990) Mutation in codon 200 of the scrapie amyloid protein gene in two clusters of Creutzfeldt-Jakob disease in Slowakia. Lancet 336: 514–515

    Article  PubMed  CAS  Google Scholar 

  17. Hsiao K, Scott M, Foster D et al. (1990) Spontaneous neurodegeneration in transgenenic mice with mutant prion protein. Science 250:1587–1590

    Article  PubMed  CAS  Google Scholar 

  18. Hsiao K, Dlouhy SR, Farlow MR et al. (1992) Mutant prion proteins in Gerstmann-Straussler-Scheinker disease with neurofibrillary tangles. Nature genetics 1, 68–71

    Article  PubMed  CAS  Google Scholar 

  19. Keohane C, Peatfield R, Duchen LW (1985) Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease) with amyloid angiopathy. J Neurol Neurosurg Psychiatry 48: 1175–1178

    Article  PubMed  CAS  Google Scholar 

  20. Manetto V, Medori R, Cortelli P et al. (1992) Fatal familial insomania: Clinical and pathological study in five new cases. Neurology 42, 312–319

    PubMed  CAS  Google Scholar 

  21. Merz PA, Somerville RA, Wisniewski HM (1983) Scrapie-associated fibrils in Creutzfeldt-Jakob disease. Nature 306: 474–476

    Article  PubMed  CAS  Google Scholar 

  22. Mitteilungen des Berufsverbandes Deutscher Pathologen (1993) Umgang mit Gewebe von Patienten mit Verdacht auf Creutzfeldt- Jakob‘scher Krankheit (Stand 1.3. 1992) Der Pathologe 14, 355

    Google Scholar 

  23. Monari L, Chen SG, Brown P et al. (1994) Fatal familial insomnia and familial Creutzfeldt-Jakob disease: Different prion proteins determined by a DNA polymorphism. Proc natl Acad Sci USA 91,2839–2842

    Article  PubMed  CAS  Google Scholar 

  24. Prusiner SB (1982) Novel proteinaceous infectious particles cause scrapie. Science 216: 136–144

    Article  PubMed  CAS  Google Scholar 

  25. Prusiner SB (1987) The biology of prion transmission and replication. VIII. Oral transmission. In: Prusiner SB, McKinley MP (eds) Prions. Novel infectious pathogens causing scrapie and Creutzfeldt-Jakob disease, Acad Press San Diego, New York, Berkeley, Boston, London, Sidney, Tokyo, Toronto, pp 93–97

    Google Scholar 

  26. Rappaport E (1987) Iatrogenic Creutzfeldt-Jakob disease. Neurology 37: 1520–1522

    PubMed  CAS  Google Scholar 

  27. Tateishi J, Kitamoto T, Doh-ura K, Boellaard JW, Peiffer J (1992) Creutzfeldt-Jakob disease with amyloid angiopathy: Diagnosis by immunological analyses and transmission experiments. Acta Neuropathol 83: 559–563

    Article  PubMed  CAS  Google Scholar 

  28. Titford M, Bastian FO (1989) Handling Creutzfeldt-Jakob disease tissues in the histology laboratory. J Histotechn 12: 214–217

    Google Scholar 

  29. Uysal A, Kaaden O-R (1993) Zum Umgang mit unkonventionellen Erregern. Pathologe 14, 351–354

    PubMed  CAS  Google Scholar 

  30. Wells GAH, Wilesmith JW, McGill IS (1991) Bovine spongiform encephalopathy: A neuropathological perspective. Brain Pathol 1:69–78

    Article  PubMed  CAS  Google Scholar 

Download references

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 1995 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Boellaard, J.W. (1995). Übertragbare spongiforme Enzephalopathien. In: Remmele, W., Peiffer, J., Schröder, J.M. (eds) Pathologie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-85179-7_11

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-85179-7_11

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-85180-3

  • Online ISBN: 978-3-642-85179-7

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics