Abstract
The epidemic of bovine spongiform encephalopathy (BSE) in the United Kingdom, which began in 1986 and has affected nearly 200,000 cattle, is waning to a conclusion, but leaves in its wake an outbreak of human Creutzfeldt-Jakob disease, most probably resulting from the consumption of beef products contaminated by central nervous system tissue. Although averaging only 10-15 cases a year since its first appearance in 1994, its future magnitude and geographic distribution (in countries that have imported infected British cattle or cattle products, or have endogenous BSE) cannot yet be predicted. The possibility that large numbers of apparently healthy persons might be incubating the disease raises concerns about iatrogenic transmissions through instrumentation (surgery and medical diagnostic procedures) and blood and organ donations. Government agencies in many countries continue to implement new measures to minimize this risk.
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- Bateman D., Hilton D., Love S., Zeidler M., Beck J., Collinge J. Sporadic Creutzfeldt-Jakob disease in a 18-year-old in the UK. Lancet. 1995 Oct 28;346(8983):1155–1156. doi: 10.1016/s0140-6736(95)91828-0. [DOI] [PubMed] [Google Scholar]
- Britton T. C., al-Sarraj S., Shaw C., Campbell T., Collinge J. Sporadic Creutzfeldt-Jakob disease in a 16-year-old in the UK. Lancet. 1995 Oct 28;346(8983):1155–1155. doi: 10.1016/s0140-6736(95)91827-2. [DOI] [PubMed] [Google Scholar]
- Brown P., Bradley R. 1755 and all that: a historical primer of transmissible spongiform encephalopathy. BMJ. 1998 Dec 19;317(7174):1688–1692. doi: 10.1136/bmj.317.7174.1688. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Brown P. Can Creutzfeldt-Jakob disease be transmitted by transfusion? Curr Opin Hematol. 1995 Nov;2(6):472–477. doi: 10.1097/00062752-199502060-00012. [DOI] [PubMed] [Google Scholar]
- Brown P., Cervenáková L., McShane L. M., Barber P., Rubenstein R., Drohan W. N. Further studies of blood infectivity in an experimental model of transmissible spongiform encephalopathy, with an explanation of why blood components do not transmit Creutzfeldt-Jakob disease in humans. Transfusion. 1999 Nov-Dec;39(11-12):1169–1178. doi: 10.1046/j.1537-2995.1999.39111169.x. [DOI] [PubMed] [Google Scholar]
- Brown P., Gajdusek D. C. Survival of scrapie virus after 3 years' interment. Lancet. 1991 Feb 2;337(8736):269–270. doi: 10.1016/0140-6736(91)90873-n. [DOI] [PubMed] [Google Scholar]
- Brown P., Preece M., Brandel J. P., Sato T., McShane L., Zerr I., Fletcher A., Will R. G., Pocchiari M., Cashman N. R. Iatrogenic Creutzfeldt-Jakob disease at the millennium. Neurology. 2000 Oct 24;55(8):1075–1081. doi: 10.1212/wnl.55.8.1075. [DOI] [PubMed] [Google Scholar]
- Brown P. The risk of bovine spongiform encephalopathy ('mad cow disease') to human health. JAMA. 1997 Sep 24;278(12):1008–1011. [PubMed] [Google Scholar]
- Bruce M. E., Will R. G., Ironside J. W., McConnell I., Drummond D., Suttie A., McCardle L., Chree A., Hope J., Birkett C. Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature. 1997 Oct 2;389(6650):498–501. doi: 10.1038/39057. [DOI] [PubMed] [Google Scholar]
- Cervenáková L., Goldfarb L. G., Garruto R., Lee H. S., Gajdusek D. C., Brown P. Phenotype-genotype studies in kuru: implications for new variant Creutzfeldt-Jakob disease. Proc Natl Acad Sci U S A. 1998 Oct 27;95(22):13239–13241. doi: 10.1073/pnas.95.22.13239. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Collee J. G., Bradley R. BSE: a decade on--Part 2. Lancet. 1997 Mar 8;349(9053):715–721. doi: 10.1016/S0140-6736(96)08496-6. [DOI] [PubMed] [Google Scholar]
- Collee J. G., Bradley R. BSE: a decade on--Part I. Lancet. 1997 Mar 1;349(9052):636–641. doi: 10.1016/S0140-6736(96)01310-4. [DOI] [PubMed] [Google Scholar]
- Collinge J., Sidle K. C., Meads J., Ironside J., Hill A. F. Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature. 1996 Oct 24;383(6602):685–690. doi: 10.1038/383685a0. [DOI] [PubMed] [Google Scholar]
- Cousens S. N., Vynnycky E., Zeidler M., Will R. G., Smith P. G. Predicting the CJD epidemic in humans. Nature. 1997 Jan 16;385(6613):197–198. doi: 10.1038/385197a0. [DOI] [PubMed] [Google Scholar]
- Fleetwood A. J., Furley C. W. Spongiform encephalopathy in an eland. Vet Rec. 1990 Apr 21;126(16):408–409. [PubMed] [Google Scholar]
- Foster J. D., Hope J., McConnell I., Bruce M., Fraser H. Transmission of bovine spongiform encephalopathy to sheep, goats, and mice. Ann N Y Acad Sci. 1994 Jun 6;724:300–303. doi: 10.1111/j.1749-6632.1994.tb38920.x. [DOI] [PubMed] [Google Scholar]
- Ghani A. C., Ferguson N. M., Donnelly C. A., Anderson R. M. Predicted vCJD mortality in Great Britain. Nature. 2000 Aug 10;406(6796):583–584. doi: 10.1038/35020688. [DOI] [PubMed] [Google Scholar]
- Ghani A. C., Ferguson N. M., Donnelly C. A., Hagenaars T. J., Anderson R. M. Epidemiological determinants of the pattern and magnitude of the vCJD epidemic in Great Britain. Proc Biol Sci. 1998 Dec 22;265(1413):2443–2452. doi: 10.1098/rspb.1998.0596. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Holt T. A., Phillips J. Bovine spongiform encephalopathy. Br Med J (Clin Res Ed) 1988 Jun 4;296(6636):1581–1582. doi: 10.1136/bmj.296.6636.1581. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Huillard d'Aignaux J., Costagliola D., Maccario J., Billette de Villemeur T., Brandel J. P., Deslys J. P., Hauw J. J., Chaussain J. L., Agid Y., Dormont D. Incubation period of Creutzfeldt-Jakob disease in human growth hormone recipients in France. Neurology. 1999 Oct 12;53(6):1197–1201. doi: 10.1212/wnl.53.6.1197. [DOI] [PubMed] [Google Scholar]
- Jeffrey M., Wells G. A. Spongiform encephalopathy in a nyala (Tragelaphus angasi). Vet Pathol. 1988 Sep;25(5):398–399. doi: 10.1177/030098588802500514. [DOI] [PubMed] [Google Scholar]
- Kimberlin R. H. Bovine spongiform encephalopathy. Taking stock of the issues. Nature. 1990 Jun 28;345(6278):763–764. doi: 10.1038/345763a0. [DOI] [PubMed] [Google Scholar]
- Kimberlin R. H., Cole S., Walker C. A. Temporary and permanent modifications to a single strain of mouse scrapie on transmission to rats and hamsters. J Gen Virol. 1987 Jul;68(Pt 7):1875–1881. doi: 10.1099/0022-1317-68-7-1875. [DOI] [PubMed] [Google Scholar]
- Kimberlin R. H., Walker C. A. Competition between strains of scrapie depends on the blocking agent being infectious. Intervirology. 1985;23(2):74–81. doi: 10.1159/000149588. [DOI] [PubMed] [Google Scholar]
- Kimberlin R. H., Walker C. A., Fraser H. The genomic identity of different strains of mouse scrapie is expressed in hamsters and preserved on reisolation in mice. J Gen Virol. 1989 Aug;70(Pt 8):2017–2025. doi: 10.1099/0022-1317-70-8-2017. [DOI] [PubMed] [Google Scholar]
- Kirkwood J. K., Wells G. A., Wilesmith J. W., Cunningham A. A., Jackson S. I. Spongiform encephalopathy in an arabian oryx (Oryx leucoryx) and a greater kudu (Tragelaphus strepsiceros) Vet Rec. 1990 Oct 27;127(17):418–420. [PubMed] [Google Scholar]
- Kulczycki J., Jedrzejowska H., Gajkowski K., Tarnowska-Dziduszko E., Lojkowska W. Creutzfeldt-Jakob disease in young people. Eur J Epidemiol. 1991 Sep;7(5):501–504. doi: 10.1007/BF00143129. [DOI] [PubMed] [Google Scholar]
- Manuelidis L. Decontamination of Creutzfeldt-Jakob disease and other transmissible agents. J Neurovirol. 1997 Feb;3(1):62–65. doi: 10.3109/13550289709015793. [DOI] [PubMed] [Google Scholar]
- O'Rourke K. I., Baszler T. V., Besser T. E., Miller J. M., Cutlip R. C., Wells G. A., Ryder S. J., Parish S. M., Hamir A. N., Cockett N. E. Preclinical diagnosis of scrapie by immunohistochemistry of third eyelid lymphoid tissue. J Clin Microbiol. 2000 Sep;38(9):3254–3259. doi: 10.1128/jcm.38.9.3254-3259.2000. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Pocchiari M., Peano S., Conz A., Eshkol A., Maillard F., Brown P., Gibbs C. J., Jr, Xi Y. G., Tenham-Fisher E., Macchi G. Combination ultrafiltration and 6 M urea treatment of human growth hormone effectively minimizes risk from potential Creutzfeldt-Jakob disease virus contamination. Horm Res. 1991;35(3-4):161–166. doi: 10.1159/000181894. [DOI] [PubMed] [Google Scholar]
- Roels S., Vanopdenbosch E., Langeveld J. P., Schreuder B. E. Immunohistochemical evaluation of tonsillar tissue for preclinical screening of scrapie based on surveillance in Belgium. Vet Rec. 1999 Oct 30;145(18):524–525. doi: 10.1136/vr.145.18.524. [DOI] [PubMed] [Google Scholar]
- Schmerr M. J., Jenny A. L., Bulgin M. S., Miller J. M., Hamir A. N., Cutlip R. C., Goodwin K. R. Use of capillary electrophoresis and fluorescent labeled peptides to detect the abnormal prion protein in the blood of animals that are infected with a transmissible spongiform encephalopathy. J Chromatogr A. 1999 Aug 20;853(1-2):207–214. doi: 10.1016/s0021-9673(99)00514-2. [DOI] [PubMed] [Google Scholar]
- Scott M. R., Will R., Ironside J., Nguyen H. O., Tremblay P., DeArmond S. J., Prusiner S. B. Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans. Proc Natl Acad Sci U S A. 1999 Dec 21;96(26):15137–15142. doi: 10.1073/pnas.96.26.15137. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Taylor D. M. Bovine spongiform encephalopathy and human health. Vet Rec. 1989 Oct 14;125(16):413–415. doi: 10.1136/vr.125.16.413. [DOI] [PubMed] [Google Scholar]
- Wells G. A., Scott A. C., Johnson C. T., Gunning R. F., Hancock R. D., Jeffrey M., Dawson M., Bradley R. A novel progressive spongiform encephalopathy in cattle. Vet Rec. 1987 Oct 31;121(18):419–420. doi: 10.1136/vr.121.18.419. [DOI] [PubMed] [Google Scholar]
- Will R. G., Ironside J. W., Zeidler M., Cousens S. N., Estibeiro K., Alperovitch A., Poser S., Pocchiari M., Hofman A., Smith P. G. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet. 1996 Apr 6;347(9006):921–925. doi: 10.1016/s0140-6736(96)91412-9. [DOI] [PubMed] [Google Scholar]
- Willoughby K., Kelly D. F., Lyon D. G., Wells G. A. Spongiform encephalopathy in a captive puma (Felis concolor). Vet Rec. 1992 Nov 7;131(19):431–434. doi: 10.1136/vr.131.19.431. [DOI] [PubMed] [Google Scholar]