1887

Abstract

Creutzfeldt-Jakob disease (CJD) belongs to a group of human and animal diseases, distinguished from each other by differences in their clinical and neuropathological presentation or aetiology, but known collectively as the prion diseases. Prion diseases are unique amongst transmissible diseases in that the transmissible ‘agent’ does not appear to contain any nucleic acid and the only protein known to be associated with infectivity is host-coded. Prion disease is endemic in certain species, notably humans and sheep. It has also appeared in epidemic form in humans: for example, amongst tribal peoples of Papua New Guinea, where the disease is known as kuru, and in Western societies as a consequence of iatrogenic misadventure. Epidemic prion disease also occurs in animals: for example, in cattle [as the current epidemic of BSE (bovine spongiform encephalopathy) in Britain (Wells & Wilesmith, 1995)] and in farmed mink (Marsh, 1992).

Loading

Article metrics loading...

/content/journal/jgv/10.1099/0022-1317-77-12-2895
1996-12-01
2022-08-17
Loading full text...

Full text loading...

/deliver/fulltext/jgv/77/12/JV0770122895.html?itemId=/content/journal/jgv/10.1099/0022-1317-77-12-2895&mimeType=html&fmt=ahah

References

  1. Almond J. W., Brown P., Gore S. M., Hofman A., Wientjens D. P. W. M., Ridley R. M., Baker H. F., Roberts G. W., Tyler K. L. 1995; Creutzfeldt–Jakob disease and bovine spongiform encephalopathy: any connection? (Commentaries). British Medical Journal 311:1415–1421
    [Google Scholar]
  2. Alpers M. 1987; Epidemiological and clinical aspects of kuru. In Prions: Novel Infectious Pathogens causing Scrapie and Creutzfeldt–Jakob Disease pp 451–465 Edited by Prusiner S. B., McKinley M. P. San Diego: Academic Press;
    [Google Scholar]
  3. Bahmanyar S., Williams E. S., Johnson F. B., Young S., Gajdusek D. C. 1985; Amyloid plaques in spongiform encephalopathy in mule deer. Journal of Comparative Pathology 95:1–5
    [Google Scholar]
  4. Baker H. F., Ridley R. M. 1992; The genetics and transmissibility of human spongiform encephalopathy. Neurodegeneration 1:3–16
    [Google Scholar]
  5. Baker H. F., Ridley R. M., Crow T. J. 1985a; Experimental transmission of an autosomal dominant spongiform encephalopathy: does the infectious agent originate in the human genome?. British Medical Journal 291:299–302
    [Google Scholar]
  6. Baker H. F., Ridley R. M., Crow T. J. 1985b; Creutzfeldt–Jakob disease. British Medical Journal 291:822–823
    [Google Scholar]
  7. Baker H. F., Ridley R. M., Wells G. A. 1993; Experimental transmission of BSE and scrapie to the common marmoset. Veterinary Record 132:403–406
    [Google Scholar]
  8. Brandner S., Isenmann S., Raeber A., Fischer M., Sailer A., Kobayashi Y., Marino S., Weissmann C., Aguzzi A. 1996; Normal host prion protein necessary for scrapie-induced neurotoxicity. Nature 379:339–343
    [Google Scholar]
  9. Brown P. 1996; Environmental causes of human spongiform encephalopathy. In Prion Diseases pp 139–154 Edited by Baker H. F., Ridley R. M. Totowa, N]: Humana Press;
    [Google Scholar]
  10. Brown P., Cathala F., Sadowsky D. 1983; Correlation between population density and the frequency of Creutzfeldt–Jakob disease in France. Journal of the Neurological Sciences 60:169–176
    [Google Scholar]
  11. Brown P., Cathala F., Raubertas R. F., Gajdusek D. C., Catalgne P. 1987; The epidemiology of Creutzfeldt–Jakob disease: conclusion of a 15 year investigation in France and review of the world literature. Neurology 37:895–904
    [Google Scholar]
  12. Brown P., Gibbs C. J. Jr, Rodgers-Johnson P., Asher D. M., Sulima M. P., Bacote A., Goldfarb L. G., Gajdusek D. C. 1994; Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease. Annals of Neurology 35:513–529
    [Google Scholar]
  13. Brown D. R., Schmidt B., Kretzschmar H. A. 1996; Role of microglia and host prion protein in neurotoxicity of a prion protein fragment. Nature 380:345–347
    [Google Scholar]
  14. Bruce M. E. 1981; Serial studies on the development of cerebral amyloidosis and vacuolar degeneration in murine scrapie. Journal of Comparative Pathology 91:589–597
    [Google Scholar]
  15. Bruce M. E., Chree A., McConnell I., Foster J., Pearson G., Fraser H. 1994; Transmission of bovine spongiform encephalopathy and scrapie to mice: strain variation and the species barrier. Philosophical Transactions of the Royal Society Series B Biological Sciences 343:405–411
    [Google Scholar]
  16. Büeler H., Aguzzi A., Sailer A., Greiner R. A., Autenried P., Aguet M., Weissmann C. 1993; Mice devoid of PrP are resistant to scrapie. Cell 73:1339–1347
    [Google Scholar]
  17. Butler D. 1996; Did UK ‘dump’ contaminated feed after ban?. Nature 381:544–545
    [Google Scholar]
  18. Cathala F., Baron H. 1987; Clinical aspects of Creutzfeldt–Jakob disease. In Prions: Novel Infectious Pathogens causing Scrapie and Creutzfeldt–Jakob Disease pp 467–509 Edited by Prusiner S. B., McKinley M. P. San Diego: Academic Press;
    [Google Scholar]
  19. Chazot G., Broussolle E., Lapras C., Blättler T., Aguzzi A., Kopp N. 1996; New variant of Creutzfeldt–Jakob disease in a 26-year-old French man. Lancet 347:1181
    [Google Scholar]
  20. Collinge J., Brown J., Hardy J., Mullan M., Rossor M. N., Baker H. F., Crow T. J., Lofthouse R., Poulter M., Ridley R., Owen F., Bennett C., Dunn G., Harding A. E., Quinn N., Doshi B., Roberts G. W., Honavar M., Janota I., Lantos P. L. 1992; Inherited prion disease with 144 base pair gene insertion. 2. Clinical and pathological features. Brain 115:687–710
    [Google Scholar]
  21. Collinge J., Palmer M. S., Sidle K. C. L., Hill A. F., Gowland I., Meads J., Asante E., Bradley R., Doey L. J., Lantos P. L. 1995; Unaltered susceptibility to BSE in transgenic mice expressing human prion protein. Nature 378:779–783
    [Google Scholar]
  22. Cousens S. N., Harries-Jones R., Knight R., Will R. G., Smith P. G., Matthews W. B. 1990; Geographical distribution of cases of Creutzfeldt–Jakob disease in England and Wales 1970–84. Journal of Neurology, Neurosurgery and Psychiatry 53:459–465
    [Google Scholar]
  23. Delasnerie-Laupretre N., Poser S., Pocchiari M., Wientjens D. P. W. M., Will R. 1995; Creutzfeldt–Jakob disease in Europe. Lancet 346:898
    [Google Scholar]
  24. Dickinson A. G. 1976; Scrapie in sheep and goats. In Slow Virus Diseases of Animals and Man pp 209–241 Edited by Kimberlin R. H. Amsterdam: North Holland Publishing Co;
    [Google Scholar]
  25. Dickinson A. G., Fraser H. 1979; An assessment of the genetics of scrapie in sheep and mice. In Slow Transmissible Diseases of the Nervous System vol 1 pp 367–385 Edited by Prusiner S. B., Hadlow W. J. New York: Academic Press;
    [Google Scholar]
  26. Dickinson A. G., Stamp J. T., Renwick C. C. 1974; Maternal and lateral transmission of scrapie in sheep. Journal of Comparative Pathology 84:19–25
    [Google Scholar]
  27. Fraser H. 1979; The pathogenesis and pathology of scrapie. In Aspects of Slow and Persistent Virus Infections pp 30–58 Edited by Tyrrell D. A. J. The Hague: Martinus Nijhoff;
    [Google Scholar]
  28. Gabizon R., Kahana E., Hsiao K., Prusiner S. B., Meiner Z. 1992; Inherited prion disease in Libyan Jews. In Prion Diseases of Humans and Animals pp 168–179 Edited by Prusiner S. B., Collinge J., Powell J., Anderton B. Chichester: Ellis Horwood;
    [Google Scholar]
  29. Gajdusek D. C., Gibbs C. J., Alpers M. 1966; Experimental transmission of a kuru-like syndrome to chimpanzees. Nature 209:794–796
    [Google Scholar]
  30. Gibbs C. J., Gajdusek D. C., Asher D. M., Alpers M. P., Beck E., Daniel P. M., Matthews W. B. 1968; Creutzfeldt–Jakob disease (spongiform encephalopathy): transmission to the chimpanzee. Science 161:388–389
    [Google Scholar]
  31. Glasse R., Lindenbaum S. 1992; Fieldwork in the South Fore: the process of ethnographic inquiry. In Prion Diseases of Humans and Animals pp 77–91 Edited by Prusiner S. B. Collinge J., Powell J., Anderton B. Chichester: Ellis Horwood;
    [Google Scholar]
  32. Goldfarb L. G., Mitrova E., Brown P., Toh B. K., Gajdusek D. C. 1990; Mutation in codon 200 of scrapie amyloid protein gene in two clusters of Creutzfeldt–Jakob disease in Slovakia [letter]. Lancet 336:514–515
    [Google Scholar]
  33. Hayward P. A. R., Bell J. E., Ironside J. W. 1994; Prion protein immunocytochemistry: reliable protocols for the investigation of Creutzfeldt–Jakob disease. Neuropathology and Applied Neurobiology 20:375–383
    [Google Scholar]
  34. Herzberg L., Herzberg R. N., Gibbs C. J., Sullivan W., Amyx H., Gajdusek D. C. 1974; Creutzfeldt–Jakob disease: hypothesis for high incidence in Libyan Jews in Israel. Science 186:848
    [Google Scholar]
  35. Hourrigan J. L., Klingsporn A., Clark W. W., de Camp M. 1979; Epidemiology of scrapie in the United States. In Slow Transmissible Diseases of the Nervous System vol 1 pp 331–356 Edited by Prusiner S. B., Hadlow W. J. New York: Academic Press;
    [Google Scholar]
  36. Hunter N., Foster J. D., Hope J. 1992; Natural scrapie in British sheep: breeds, ages and PrP gene polymorphisms. Veterinary Record 130:389–392
    [Google Scholar]
  37. Hunter N., Foster J. D., Goldmann W., Stear M. J., Hope J., Bostock C. 1996; Natural scrapie in a closed flock of Cheviot sheep occurs only in specific PrP genotypes. Archives of Virology 141:809–824
    [Google Scholar]
  38. Ironside J. W. 1996; Neuropathological diagnosis of human prion disease–morphological studies. In Prion Diseases pp 35–57 Edited by Baker H. F., Ridley R. M. Totowa, NJ: Humana Press;
    [Google Scholar]
  39. Klitzman R. L., Alpers M. P., Gajdusek D. C. 1984; The natural incubation period of kuru and the episodes of transmission in these clusters of patients. Neuroepidemiology 3:3–20
    [Google Scholar]
  40. Kocisko D. A., Come J. H., Priola S. A., Chesebro B., Raymond G. J., Lansbury P. T., St Caughey B. 1994; Cell-free formation of protease-resistant prion protein. Nature 370:471–474
    [Google Scholar]
  41. Kulczycki J., Jedrzejowska H., Gajkowski K., Tarnowska-Dziduszko E., Lojkowska W. 1991; Creutzfeldt–Jakob disease in young people. European Journal of Epidemiology 7:501–504
    [Google Scholar]
  42. Lasmézas C. I., Deslys J. P., Demainay R., Adjou K. T., Lamoury F., Dormont D., Robain O., Ironside J., Hauw J. J. 1996; BSE transmission to macaques. Nature 381:743–744
    [Google Scholar]
  43. McKeith I. G., Perry R. H., Fairbairn A. F., Jabeen S., Perry E. K. 1992; Operational criteria for senile dementia of Lewy body type. Psychological Medicine 22:911–922
    [Google Scholar]
  44. Marsh R. F. 1992; Transmissible mink encephalopathy. In Prion Diseases of Animals and Humans pp 300–307 Edited by Prusiner S. B., Collinge J., Powell J., Anderton B. Chichester: Ellis Horwood;
    [Google Scholar]
  45. Masters C. L., Harris J. O., Gajdusek D. C., Gibbs C. J. Jr, Bernoulli C., Asher D. M. 1978; Creutzfeldt–Jakob disease: patterns of worldwide occurrence and the significance of familial and sporadic clustering. Annals of Neurology 5:177–188
    [Google Scholar]
  46. Masters C. L., Gajdusek D. C., Gibbs C. J. 1981a; Creutzfeldt–Jakob disease virus isolations from the Gerstmann-Straussler syndrome. Brain 104:559–588
    [Google Scholar]
  47. Masters C. L., Gajdusek D. C., Gibbs C. J. 1981b; The familial occurrence of Creutzfeldt–Jakob disease and Alzheimer’s disease. Brain 104:535–558
    [Google Scholar]
  48. Owen F., Poulter M., Collinge J., Crow T. J. 1990; Codon 129 changes in the prion protein gene in Caucasians. American Journal of Human Genetics 46:1215–1216
    [Google Scholar]
  49. Pan K., Baldwin M., Nguyen J., Gasset M., Serban A., Groth D., Mehlhom I., Huang Z., Fletterick R. J., Cohen F. E., Prusiner S. B. 1993; Conversion of α-helices into β-sheets features in the formation of the scrapie prion proteins. Proceedings of the National Academy of Sciences, USA 90:10962–10966
    [Google Scholar]
  50. Parry H. B. 1983 Scrapie Disease in Sheep; Historical, Clinical, Epidemiological and Practical Aspects of the Natural Disease London: Academic Press;
    [Google Scholar]
  51. Poulter M., Baker H. F., Frith C. D., Leach M., Lofthouse R., Ridley R. M., Shah T., Owen F., Collinge J., Brown G., Hardy J. A., Mullan M. J., Harding A. E., Bennet C., Doshi R., Crow T. J. 1992; Inherited prion disease with 144 base pair gene insertion. 1. Genealogical and molecular studies. Brain 115:675–685
    [Google Scholar]
  52. Raubertas R. F., Brown P., Cathala F., Brown I. 1989; The question of clustering of Creutzfeldt–Jakob disease. American Journal of Epidemiology 129:146–154
    [Google Scholar]
  53. Ridley R. M. 1994; Perceptions of prion disease. Journal of Clinical Pathology 47:876–879
    [Google Scholar]
  54. Ridley R. M., Baker H. F. 1995; The myth of maternal transmission of spongiform encephalopathy. British Medical Journal 311:1071–1075
    [Google Scholar]
  55. Ridley R. M., Baker H. F. 1996; To what extent is strain variation evidence for an independent genome in the agent of the transmissible spongiform encephalopathies?. Neurodegeneration 5: (in press)
    [Google Scholar]
  56. SEAC 1995 Transmissible Spongiform Encephalopathies–a Summary of Present Knowledge and Research (Spongiform Encephalopathy Advisory Committee) London: HMSO ISBN 0-11-242-9874;
    [Google Scholar]
  57. Telling G. C., Scott M., Mastrianni J., Gabizon R., Torchia M., Cohen F. E., Dearmond S. J., Prusiner S. B. 1995; Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein. Cell 83:79–90
    [Google Scholar]
  58. Wells G. A. H., Wilesmith J. W. 1995; The neuropathology and epidemiology of bovine spongiform encephalopathy. Brain Pathology 5:91–103
    [Google Scholar]
  59. Wells G. A. H., Sayers A. R., Wilesmith J. W. 1995; Clinical and epidemiological correlates of the neurohistology of cases of histologically unconfirmed, clinically suspect bovine spongiform encephalopathy. Veterinary Record 136:211–216
    [Google Scholar]
  60. Will R. G. 1996; Surveillance of prion diseases in humans. In Prion Diseases pp 119–137 Edited by Baker H. F., Ridley R. M. Totowa, NJ: Humana Press;
    [Google Scholar]
  61. Will R. G., Ironside J. W., Zeidler M., Cousens S. N., Estibeiro K., Alperovitch A., Poser S., Pocchiari M., Hofman A., Smith P. G. 1996; A new variant of Creutzfeldt–Jakob disease in the UK. Lancet 347:921–925
    [Google Scholar]
http://instance.metastore.ingenta.com/content/journal/jgv/10.1099/0022-1317-77-12-2895
Loading

Most cited this month Most Cited RSS feed

This is a required field
Please enter a valid email address
Approval was a Success
Invalid data
An Error Occurred
Approval was partially successful, following selected items could not be processed due to error