Bovine spongiform encephalopathy and Creutzfeldt-Jakob disease: facts and uncertainties underlying the causal link between animal and human diseases |
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| Authors: | E. Beghi C. Gandolfo C. Ferrarese N. Rizzuto G. Poli M. C. Tonini G. Vita M. Leone G. Logroscino E. Granieri G. Salemi G. Savettieri L. Frattola G. Ru G. L. Mancardi C. Messina |
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| Affiliation: | (1) Mario Negri Institute of Pharmacological Research, Via Eritrea 62, I-20157 Milan, Italy;(2) Neurological Clinic, University of Milan-Bicocca, Monza, Italy;(3) Neurological Clinic, University of Genoa, Italy;(4) Neurological Clinic, Borgo Roma Policlinico, Verona, Italy;(5) Faculty of Veterinarian Medicine, University of Milan, Italy;(6) Division of Neurology, G. Salvini Hospital Garbagnate, Milanese (MI), Italy;(7) Neurological Clinic 2, Politecnico Universitario G. Martini, Messina, Italy;(8) Neurological Clinic, Ospedale Maggiore della Carità, Novara, Italy;(9) Department of Epidemiology, Harvard University, School of Public Health Boston, Massachusetts, USA;(10) Neurological Clinic, University of Ferrara, Italy;(11) Institute of Neuropsychiatry, University of Palermo, Italy;(12) Department of Neuroscience, University of Palermo, Italy;(13) IRCSS Don Gnocchi Foundation, Milan, Italy;(14) Istituto Zooprofilattico, Turin, Italy |
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| Abstract: | Abstract. Following an outbreak of bovine spongiform encephalopathy (BSE) in dairy cows in the United Kingdom (UK), 153 definite and probable human cases of new variant Creutzfeldt-Jakob disease (nvCJD) have been reported, almost exclusively in the UK. Although exposure to the BSE agent is the most plausible interpretation for the occurrence of nvCJD, the causal link between the BSE prion and nvCJD is still debated. This review discusses the pros and cons of nvCJD as a separate nosographic entity, the scientific basis for a correlation between BSE and nvCJD, the validity of the current diagnostic criteria for CJD and nvCJD, the contribution of epidemiology to the detection of a causal relation between BSE and nvCJD, and the present and future directions of the epidemiological research on BSE, CJD and nvCJD. |
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| Keywords: | Bovine spongiform encephalopathy Creutzfeldt-Jakob disease Epidemiology |
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