Abstract
A variant form of Creutzfeldt-Jakob disease (vCJD) has had major impact in Europe during the last decade. In this article, we review the aetiology of vCJD and its relation with bovine spongiform encephalopathy. Further, treatment of the disease, the strategies focusing on prevention of transmission, future prospects, and the limitations encountered in epidemiological research of vCJD are discussed.
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References
Masters CL, Harris JO, Gajdusek DC, et al. Creutzfeldt-Jakobdisease: Patterns of worldwide occurrence and the significance of familial and sporadic clustering. Ann Neurol 1979; 5: 177–188.
Will RG, Ironside JW, Zeidler M, et al. A new variant of Creutzfeldt-Jakobdisease in the UK. Lancet 1996; 347: 921–925.
Prusiner SB, Hsiao KK. Human prion diseases. Ann Neurol 1994; 35: 385–395.
DeArmond SJ, Bouzamondo E. Fundamentals of prion biology and diseases. Toxicology 2002; 181-182: 9–16.
Pan KM, Baldwin M, Nguyen J, et al. Conversion of ahelices into b-sheets features in the formation of the scrapie prion protein. Proc Natl Acad Sci USA 1993; 90: 10962–10966.
Prusiner SB, Scott M, Foster D, et al. Transgenetic studies implicate interaction between homologous PrP isoforms in scrapie prion replication. Cell 1990; 63: 673–686.
Hill AF, Desbruslais M, Joiner S, et al. The same prion strain causes vCJD and BSE. Nature 1997; 389: 448–450.
Bruce ME, Will RG, Ironside JW, et al. Transmissions to mice indicate that ‘new variant’ CJD is caused by the BSE agent. Nature 1997; 389: 498–501.
Wells GAH, Scott AC, Johnson CT, et al. A novel progressive spongiform encephalopathy in cattle. Vet Rec 1987; 121: 419–420.
Taylor DM. Current perspectives on bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease. Clin Microbiol Infect 2002; 8: 332–339.
Wilesmith JW, Wells GAJ, Cranwell MP, Ryan JBM. Bovine spongiform encephalopathy: Epidemiological studies. Vet Rec 1988; 123: 638–644.
Almond JW, Brown P, Gore SM, et al. Creutzfeldt-Jakobdisease and bovine spongiform encephalopathy: Any connection? Br Med J 1995; 311: 1415–1421.
Spencer MD, Knight RSG, Will RG. First hundred cases of variant Creutzfeldt-Jakobdisease retrospective case note review of early psychiatric and neurological features. Br Med J 2002; 324: 1479–1482.
Collie DA, Sellar RJ, Zeidler M, Colchester AC, Knight R, Will RG. MRI of Creutzfeldt-Jakobdisease: Imaging features and recommended MRI protocol. Clin Radiol 2001; 9: 726–739.
Will RG, Zeidler M, Stewart GE, et al. Diagnosis of new variant Creutzfeldt-Jakobdisease. Ann Neurol 2000; 47: 575–582.
Ironside JW. Pathology of variant Creutzfeldt-Jakob disease. Arch Virol Suppl 2000; 16: 143–151.
Masullo C, Macchi G, Xi YG, Pocchiari M. Failure to ameliorate Creutzfeldt-Jakobdisease with amphotericin B therapy. J Infect Dis 1992; 165: 784–785.
Ladogana A, Casaccia P, Ingrosso L, et al. Sulphate polyanions prolong the incubation period of scrapie-infected hamsters. J Gen Virol 1992; 73: 661–665.
Korth C, May BC, Cohen FE, Prusiner SB. Acridine and phenothiazine derivatives as pharmacotherapeutics for prion disease. Proc Natl Acad Sci USA 2001; 98: 9836–9841.
Collins J, Lewis V, Brazier M, Hill AF, Fletcher A, Masters CL. Quinacrine does not prolong survival in a murine Creutzfeldt-Jakobdisease model. Ann Neurol 2002; 52: 503–506.
Iversen L. Amyloid diseases: Small drugs lead the attack. Nature 2002; 417: 231–233.
White AR, Enever P, Tayebi M, et al. Monoclonal antibodies inhibit prion replication and delay the development of prion disease. Nature 2003; 422: 80–83.
Hill AF, Butterworth RJ, Joiner, et al. Investigation of variant Creutzfeldt-Jakobdisease and other human prion diseases with tonsil biopsy samples. Lancet 1999; 353: 183–189.
Houston F, Foster JD, Chong A, Hunter N, Bostock CJ. Transmission of BSE by blood transfusion in sheep. Lancet 2000; 356: 999–1000.
Andrews NJ, Farrington CP, Cousens SN, et al. Incidence of variant Creutzfeldt-Jakobdisease in the UK. Lancet 2000; 356: 481–482.
Andrews NJ, Farrington CP, Ward HJT, et al. Deaths from variant Creutzfeldt-Jakobdisease in the UK. Lancet 2003; 361: 751–752.
Valleron AJ, Boelle PY, Will R, Cesbron JY. Estimation of epidemic size and incubation time based on age characteristics of vCJD in the United Kingdom. Science 2001; 294: 1726–1728.
d'Aignaux JN, Cousens SN, Smith PG. Predictability of the UK variant Creutzfeldt-Jakobdisease epidemic. Science 2001; 294: 1729–1731.
Ferguson NM, Ghani AC, Donnelly CA, Hagenaars TJ, Anderson RM. Estimating the human health risk from possible BSE infection of the British sheep flock. Nature 2002; 415: 420–424.
Jackson GS, Beck JA, Navarrete C, et al. HLA_DQ7 antigen and resistance to vCJD. Nature 2001; 414: 269–270.
Pepys MB, Bybee A, Booth DR, et al. MHC typing in variant Creutzfeldt-Jakobdisease. Lancet 2003; 361: 487–489.
Glatzel M, Rogivue C, Ghani A, et al. Incidence of Creutzfeldt-Jakobdisease in Switzerland. Lancet 2002; 360: 139–141.
Asante EA, Linehan JM, Desbruslais M, et al. BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO J 2002; 21: 6358–6366.
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Croes, E.A., van Duijn, C.M. REVIEW: Variant Creutzfeldt-Jakob disease. Eur J Epidemiol 18, 473–477 (2003). https://doi.org/10.1023/A:1024683119263
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DOI: https://doi.org/10.1023/A:1024683119263