Neurological Sciences

, Volume 25, Issue 3, pp 122–129 | Cite as

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

  • 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
REVIEW

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.

Key words

Bovine spongiform encephalopathy Creutzfeldt-Jakob disease Epidemiology 

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Copyright information

© Springer-Verlag Italia 2004

Authors and Affiliations

  • E. Beghi
    • 1
    • 2
  • C. Gandolfo
    • 3
  • C. Ferrarese
    • 2
  • N. Rizzuto
    • 4
  • G. Poli
    • 5
  • M. C. Tonini
    • 6
  • G. Vita
    • 7
  • M. Leone
    • 8
  • G. Logroscino
    • 9
  • E. Granieri
    • 10
  • G. Salemi
    • 11
  • G. Savettieri
    • 12
  • L. Frattola
    • 2
    • 13
  • G. Ru
    • 14
  • G. L. Mancardi
    • 3
  • C. Messina
    • 7
  1. 1.Mario Negri Institute of Pharmacological ResearchMilanItaly
  2. 2.Neurological ClinicUniversity of Milan-BicoccaMonzaItaly
  3. 3.Neurological ClinicUniversity of GenoaItaly
  4. 4.Neurological ClinicBorgo Roma PoliclinicoVeronaItaly
  5. 5.Faculty of Veterinarian MedicineUniversity of MilanItaly
  6. 6.Division of NeurologyG. Salvini Hospital GarbagnateMilanese (MI)Italy
  7. 7.Neurological Clinic 2Politecnico Universitario G. MartiniMessinaItaly
  8. 8.Neurological ClinicOspedale Maggiore della CaritàNovaraItaly
  9. 9.Department of EpidemiologyHarvard University, School of Public Health BostonMassachusettsUSA
  10. 10.Neurological ClinicUniversity of FerraraItaly
  11. 11.Institute of NeuropsychiatryUniversity of PalermoItaly
  12. 12.Department of NeuroscienceUniversity of PalermoItaly
  13. 13.IRCSS Don Gnocchi FoundationMilanItaly
  14. 14.Istituto ZooprofilatticoTurinItaly

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