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British Medical Bulletin 66:255-265 (2003)
© 2003 The British Council

Acquired prion disease: iatrogenic CJD, variant CJD, kuru

Robert G Will

National CJD Surveillance Unit, Department of Clinical Neuroscience, Western General Hospital, Edinburgh, UK

Human prion diseases can be classified as sporadic, hereditary or acquired. The cause of sporadic Creutzfeldt-Jakob disease (CJD) is unknown, hereditary cases are associated with mutations of the prion protein gene (PRNP) and acquired forms are caused by the transmission of infection from human to human or, as a zoonosis, from cattle to human. Although acquired forms of human prion disease are rare, the transmission of a fatal and untreatable neurological disorder has had major implications for public health and public policy.


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