Prion disease: advances in diagnosis and treatment
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Keywords

Prion disease
Transmissible spongiform encephalopathies
BSE
vCJD

Abstract

Steve Dealler is a medical microbiologist with Morecambe Bay Hospitals NHS Trust. His work on on the diagnosis, epidemiology and potential treatment of transmissible spongiform encephalopathies has brought him inter-national recognition. He has been at the forefront of work on the epidemiology of human food containing the vector for bovine spongiform encephalopathy (BSE), and the development of prophylaxis against variant Creutzfeldt-Jakob disease (vCJD). He is currently working on a potential treatment, pentosan polysulphate. Here he describes the current state of knowledge in the battle against this devastating disease and the political inertia that frustrated earlier attempts to prevent the epidemic.

https://doi.org/10.48037/mbmj.v4i10.877
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References

Collins SJ, Masters VA, Lawson CL. Transmissible spongiform encephalopathies. Lancet 2004;363:51-61. Available from: https://doi.org/10.1016/S0140-6736(03)15171-9

Miller MW, Williams ES. Chronic wasting disease of cervids. Curr Top Microbial lmmunol 2004;284:193-214. Available from: https://doi.org/10.1007/978-3-662-08441-0_8

Kovac GG, Voigtlander T, Gelpi E, Budka H. Rationale for diagnosing human prion disease. World J Biol Psychiatry 2004;5:83-91. Available from: https://doi.org/10.1080/15622970410029916

Kubler E, Oesch B, Raeber AJ. Diagnosis of prion diseases. Br Med Bull 2003;66:267-79. Available from: https://doi.org/10.1093/bmb/66.1.267

Korth C, Kaneko K, Groth D, et al. Abbreviated incubation times for human prions in mice expressing a chimeric mouse-human prion protein transgene. Proc Natl Acad Sci USA 2003;100:4784-9. Available from: https://doi.org/10.1073/pnas.2627989100

Liberski PP, Jeffrey M. Tubulovesicular structures: what are they really? Microsc Res Tech 2000;50:46-57. Available from: https://doi.org/10.1002/1097-0029(20000701)50:1<46::AID-JEMT8>3.0.CO;2-D

O'Rourke Kl, Zhuang D, Lyda A, et al. Abundant PrP(CWD) in tonsil from mule deer with preclinical chronic wasting disease. J Vet Diagn Invest 2003;15:320-3. Available from: https://doi.org/10.1177/104063870301500403

Collie DA, Summers DM, Sellar RJ, et al. Diagnosing variant Creutzfeldt-Jakob disease with the pulvinar sign: MR imaging findings in 86 neuropathologically confirmed case . Am J Neuroradiol 2003;24:1560-9

Wu D, Stowell CP. Prion diseases: recent developments toward diagnostic tests. Am J Clin Pathol 2003;120Suppl:S46-52. Available from: https://doi.org/10.1309/R5AR3MV7HNJ9D2AN

Green AJ. Use of 14-3-3 in the diagnosis of Creutzfeldt-Jakob disease. Biochem Soc Trans 2002;30:382-6. Available from: https://doi.org/10.1042/bst0300382

Pomfrett CJ, Glover DG, Bollen BG, Pollard BJ. Perturbation of heart rate variability in cattle fed BSE-infected material. Vet Rec 2004;154:687-91. Available from: https://doi.org/10.1136/vr.154.22.687

Atkinson PH. Glycosylation of prion strains in transmissible spongiform encephalopathies. Aust Vet J 2004;82:292-9. Available from: https://doi.org/10.1111/j.1751-0813.2004.tb12709.x

Dealler SF, Rainov N, Pomfrett C. Variant CJD case treated by intraventricular PPS: symptomatic seen without disease progression. International Prion Conference. Munich. 2003