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
Tax calculation will be finalised at checkout
Purchases are for personal use only
Learn about institutional subscriptionsPreview
Unable to display preview. Download preview PDF.
Literatur
Bastian FO (1991) Creutzfeldt-lakob disease and other transmissible spongiform encephalopathies. Mosby Year Book, St. Louis, 256pp
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
Bell JE, Ironside JW (1993) How to tackle a possible CreutzfeldtJakob disease necropsy. J Clin Pathol 46: 193–197
Berger JR, David NJ (1993) Creutzfeldt-Jakob disease in a physician: A review of the disorder in health care workers. Neurology 43.205–206
Boellaard JW, Schlote W, Tateishi J (1989) Neuronal autophagy in experimental Creutzfeldt-Jakob disease. Acta Neuropathol 82: 410–418
Brown P (1988) Human growth hormone therapy and Creutzfeldt- Jakob disease: A drama in three acts. Pediatrics 81: 85–92
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
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
Brown P, Kaur P, Sulima MP et al. (1993) Real and imagined clinicopathologicallimits of Éprion dementiaÉ. Lancet 341: 127–129
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
Büeler H, Aguzzi A, Sailer A et al. (1993) Mice devoid of PrP are resistent to scrapie. Cell 73: 1339–1347
Diringer H (1990) Unkonventionelle Viruskrankheiten. Bundesgesundhcitsblatt 33, 187–194
Diringer H (1990) Durchbrechen von Speziesbarrieren mit unkonventionellen Viren. Bundesgesundheitsblatt 33: 435–440
Gajdusek DC (1977) Unconventional viruses and the origin and disappearance of kuru. Science 197: 943–960
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
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
Hsiao K, Scott M, Foster D et al. (1990) Spontaneous neurodegeneration in transgenenic mice with mutant prion protein. Science 250:1587–1590
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
Keohane C, Peatfield R, Duchen LW (1985) Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease) with amyloid angiopathy. J Neurol Neurosurg Psychiatry 48: 1175–1178
Manetto V, Medori R, Cortelli P et al. (1992) Fatal familial insomania: Clinical and pathological study in five new cases. Neurology 42, 312–319
Merz PA, Somerville RA, Wisniewski HM (1983) Scrapie-associated fibrils in Creutzfeldt-Jakob disease. Nature 306: 474–476
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
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
Prusiner SB (1982) Novel proteinaceous infectious particles cause scrapie. Science 216: 136–144
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
Rappaport E (1987) Iatrogenic Creutzfeldt-Jakob disease. Neurology 37: 1520–1522
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
Titford M, Bastian FO (1989) Handling Creutzfeldt-Jakob disease tissues in the histology laboratory. J Histotechn 12: 214–217
Uysal A, Kaaden O-R (1993) Zum Umgang mit unkonventionellen Erregern. Pathologe 14, 351–354
Wells GAH, Wilesmith JW, McGill IS (1991) Bovine spongiform encephalopathy: A neuropathological perspective. Brain Pathol 1:69–78
Editor information
Editors and Affiliations
Rights 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