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Review article: Biomedical intelligence

Vol. 145 No. 4546 (2015)

The end of the BSE saga: do we still need surveillance for human prion diseases?

  • Herbert Budka
  • Robert G. Will
DOI
https://doi.org/10.4414/smw.2015.14212
Cite this as:
Swiss Med Wkly. 2015;145:w14212
Published
01.11.2015

Summary

The epidemics of classical bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) related to BSE-infected food are coming to an end. The decline in concern about these diseases may invite complacency and questions whether surveillance for human prion diseases is still necessary. This article reviews the main points of surveillance and why it is still needed: animal sources for human prion infection other than BSE cannot be excluded; the potentially increasing circulation of prions between humans by blood, blood products and medical procedures; the prevalence of vCJD prion carriers in the UK; and the scientific study of prion diseases as paradigm for other neurodegenerative diseases with “prion-like” spread of pathological proteins. We conclude that continuation of detailed surveillance of human prion disorders would be prudent in view of all these points that deserve clarification.

References

  1. Budka H. Editorial: The European Response to BSE: A Success Story. EFSA Journal 2011;9(9):e991 http://www.efsa.europa.eu/en/efsajournal/pub/e991.htm.
  2. WHO: Public health issues and clinical and neurological characteristics of the new variant of Creutzfeldt-Jakob disease and other human and animal transmissible spongiform encephalopathies: Memorandum from two WHO meetings. Bull WHO. 1996;74(5):453–63.
  3. WHO: WHO manual for surveillance of human transmissible spongiform encephalopathies including variant Creutzfeldt-Jakob disease. WHO Communicable Disease Surveillance and Response, WHO Geneva 2003.
  4. European Food Safety Authority (EFSA), European Centre for Disease Prevention and Control (ECDC): Joint Scientific Opinion on any possible epidemiological or molecular association between TSEs in animals and humans. EFSA Journal 2011;9(1):1945 [1111 pp.] http://www.efsa.europa.eu/en/efsajournal/doc/1945.pdf.
  5. Green AJE, Ramljak S, Müller WEG, Knight RSG, Schröder HC. 14-3-3 in the cerebrospinal fluid of patients with variant and sporadic Creutzfeldt-Jakob disease measured using capture assay able to detect low levels of 14-3-3 protein. Neurosci Lett. 2002;324(1):57–60.
  6. McGuire LI, Peden AH, Orrú CD, Wilham JM, Appleford NE, Mallinson G, et al. Real time quaking-induced conversion analysis of cerebrospinal fluid in sporadic Creutzfeldt-Jakob disease. Ann Neurol. 2012;72(2):278–85.
  7. Budka H. Neuropathology of prion diseases. Br Med Bull. 2003;66(1):121–30.
  8. Parchi P, de Boni L, Saverioni D, Cohen ML, Ferrer I, Gambetti P, et al. Consensus classification of human prion disease histotypes allows reliable identification of molecular subtypes: an inter-rater study among surveillance centres in Europe and USA. Acta Neuropathol. 2012;124(4):517–29.
  9. Parchi P, Giese A, Capellari S, Brown P, Schulz-Schaeffer W, Windl O, et al. Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol. 1999;46:224–33.
  10. Polymenidou M, Moos R, Scott M, Sigurdson C, Shi Y-z, Yajima B, et al. The POM Monoclonals: A Comprehensive Set of Antibodies to Non-Overlapping Prion Protein Epitopes. PLoS ONE 2008;3(12):e3872.
  11. Zou W-Q, Puoti G, Xiao X, Yuan J, Qing L, Cali I, et al. Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein. Ann Neurol. 2011;68(2):162–72.
  12. Kovacs GG, Peden A, Weis S, Höftberger R, Berghoff A, Yull H, et al. Rapidly progressive dementia with thalamic degeneration and peculiar cortical prion protein immunoreactivity, but absence of proteinase K resistant PrP: a new disease entity? Acta Neuropathol Commun. 2013;1(1):72.
  13. Gelpi E, Colom-Cadena M, Budka H. Molecular Genetics of Creutzfeldt-Jakob Disease and Gerstmann- Sträussler-Scheinker Disease. In: eLS. John Wiley & Sons, LTD: Chichester. DOI: 10.1002/9780470015902.a0024442; 2015: 1–11.
  14. Kovacs GG, Puopolo M, Ladogana A, Pocchiari M, Budka H, van Duijn C, et al. Genetic prion disease: the EUROCJD experience. Hum Genet. 2005;118(2):166–74.
  15. Parchi P, Castellani R, Capellari S, Ghetti B, Young K, Chen SG, et al. Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol. 1996,39:767–78.
  16. Ladogana A, Puopolo M, Croes EA, Budka H, Jarius C, Collins S, Klug GM, et al. Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada. Neurology. 2005;64(9):1586–91.
  17. Klug GM, Wand H, Simpson M, Boyd A, Law M, Masters CL, et al. Intensity of human prion disease surveillance predicts observed disease incidence. J Neurol Neurosurg Psychiatry. 2013;84:1372–7.
  18. Gelpi E, Heinzl H, Höftberger R, Unterberger U, Ströbel T, Voigtländer T, et al. Creutzfeldt-Jakob Disease in Austria: An Autopsy-Controlled Study. Neuroepidemiology. 2008;30(4):215–21.
  19. Pocchiari M, Puopolo M, Croes EA, Budka H, Gelpi E, Collins S, et al. Predictors of survival in sporadic Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies. Brain. 2004;127(10):2348–59.
  20. Alcalde-Cabero E, Almazán-Isla J, Brandel J-P, Breithaupt M, et al. Health professions and risk of sporadic Creutzfeldt–Jakob disease, 1965 to 2010. Euro Surveill. 2012;17(15):13–22.
  21. Mahillo-Fernandez I, de Pedro-Cuesta J, Bleda MJ, Cruz M, Mølbak K, Laursen H, et al. Surgery and Risk of Sporadic Creutzfeldt-Jakob Disease in Denmark and Sweden: Registry-Based Case-Control Studies. Neuroepidemiology. 2008;31(4):229–40.
  22. Dorsey K, Zou S, Schonberger LB, Sullivan M, Kessler D, Notari E, et al. Lack of evidence of transfusion transmission of Creutzfeldt-Jakob disease in a US surveillance study. Transfusion. 2009;49(5):977–84.
  23. de Pedro Cuesta J, Ruiz Tovar M, Ward H, Calero M, Smith A, Verduras CA, et al. Sensitivity to Biases of Case-Control Studies on Medical Procedures, Particularly Surgery and Blood Transfusion, and Risk of Creutzfeldt-Jakob Disease. Neuroepidemiology. 2012;39(1):1–18.
  24. Chen C-C, Wang Y-H. Estimation of the Exposure of the UK Population to the Bovine Spongiform Encephalopathy Agent through Dietary Intake During the Period 1980 to 1996. PLoS ONE 2014;9(4):e94020.
  25. Ward HJT, Everington D, Cousens SN, Smith-Bathgate B, Leitch M, Cooper S, et al. Risk factors for variant Creutzfeldt-Jakob disease: A case-control study. Ann Neurol. 2006;59(1):111–20.
  26. Gill ON, Spencer Y, Richard-Loendt A, Kelly C, Dabaghian R, Boyes L, et al. Prevalent abnormal prion protein in human appendixes after bovine spongiform encephalopathy epizootic: large scale survey. Br Med J. 2013;347:f5675.
  27. Hewitt PE, Llewelyn CA, Mackenzie J, Will RG. Creutzfeldt-Jakob disease and blood transfusion: results of the UK Transfusion Medicine Epidemiological Review study. Vox Sanguinis. 2006;91(3):221–30.
  28. Brown P, Brandel J-P, Sato T, Nakamura Y, MacKenzie J, Will RG, et al. Iatrogenic Creutzfeldt-Jakob Disease, Final Assessment. Emerging Infectious Diseases. 2012;18(6):901–7.
  29. Ladogana A, Puopolo M, Poleggi A, Almonti S, Mellina V, Equestre M, et al. High incidence of genetic human transmissible spongiform encephalopathies in Italy. Neurology. 2005;64(9):1592–7.
  30. Alpers MP. The epidemiology of kuru: monitoring the epidemic from its peak to its end. Philosophical Transactions of the Royal Society B: Biological Sciences. 2008;363(1510):3707–13.
  31. Asante EA, Smidak M, Grimshaw A, Houghton R, Tomlinson A, Jeelani A, et al. A naturally occurring variant of the human prion protein completely prevents prion disease. Nature. 2015;522(7557):478–81.
  32. Cassard H, Torres J-M, Lacroux C, Douet J-Y, Benestad SL, Lantier Fdr, et al. Evidence for zoonotic potential of ovine scrapie prions. Nat Commun. 2014;5:5821.
  33. Comoy EE, Mikol J, Luccantoni-Freire S, Correia E, Lescoutra-Etchegaray N, Durand Vr, et al. Transmission of scrapie prions to primate after an extended silent incubation period. Sci Rep. 2015;5:11573.

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