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Bovine spongiform encephalopathy and Creutzfeldt-Jakob disease: facts and uncertainties underlying the causal link between animal and human diseases

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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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Authors and Affiliations

  1. Mario Negri Institute of Pharmacological Research, Via Eritrea 62, I-20157, Milan, Italy

    E. Beghi

  2. Neurological Clinic, University of Milan-Bicocca, Monza, Italy

    E. Beghi, C. Ferrarese & L. Frattola

  3. Neurological Clinic, University of Genoa, Italy

    C. Gandolfo & G. L. Mancardi

  4. Neurological Clinic, Borgo Roma Policlinico, Verona, Italy

    N. Rizzuto

  5. Faculty of Veterinarian Medicine, University of Milan, Italy

    G. Poli

  6. Division of Neurology, G. Salvini Hospital Garbagnate, Milanese (MI), Italy

    M. C. Tonini

  7. Neurological Clinic 2, Politecnico Universitario G. Martini, Messina, Italy

    G. Vita & C. Messina

  8. Neurological Clinic, Ospedale Maggiore della Carità, Novara, Italy

    M. Leone

  9. Department of Epidemiology, Harvard University, School of Public Health Boston, Massachusetts, USA

    G. Logroscino

  10. Neurological Clinic, University of Ferrara, Italy

    E. Granieri

  11. Institute of Neuropsychiatry, University of Palermo, Italy

    G. Salemi

  12. Department of Neuroscience, University of Palermo, Italy

    G. Savettieri

  13. IRCSS Don Gnocchi Foundation, Milan, Italy

    L. Frattola

  14. Istituto Zooprofilattico, Turin, Italy

    G. Ru

Authors
  1. E. Beghi
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  2. C. Gandolfo
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  3. C. Ferrarese
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  4. N. Rizzuto
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  5. G. Poli
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  6. M. C. Tonini
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  7. G. Vita
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  8. M. Leone
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  9. G. Logroscino
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  10. E. Granieri
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  11. G. Salemi
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  12. G. Savettieri
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  13. L. Frattola
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  14. G. Ru
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  15. G. L. Mancardi
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  16. C. Messina
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Corresponding author

Correspondence to E. Beghi.

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Beghi, E., Gandolfo, C., Ferrarese, C. et al. Bovine spongiform encephalopathy and Creutzfeldt-Jakob disease: facts and uncertainties underlying the causal link between animal and human diseases. Neurol Sci 25, 122–129 (2004). https://doi.org/10.1007/s10072-004-0249-9

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  • DOI: https://doi.org/10.1007/s10072-004-0249-9

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