Accumulation of prion protein in tonsil and appendix: review of tissue samples

BMJ 2002; 325 doi: (Published 21 September 2002) Cite this as: BMJ 2002;325:633
  1. David A Hilton (, neuropathologista,
  2. Azra C Ghani, research fellowb,
  3. Lisa Conyers, research techniciana,
  4. Philip Edwards, biomedical scientista,
  5. Linda McCardle, biomedical scientistc,
  6. Mark Penney, research techniciana,
  7. Diane Ritchie, research assistantc,
  8. James W Ironside, professor of clinical neuropathologyc
  1. a Department of Histopathology, Derriford Hospital, Plymouth PL6 8DH
  2. b Department of Infectious Disease Epidemiology, Faculty of Medicine, Imperial College of Science, Technology and Medicine, London SW7 2AZ
  3. c National CJD Surveillance Unit, University of Edinburgh, Edinburgh EH4 2XU
  1. Correspondence to: D A Hilton
  • Accepted 30 July 2002

Variant Creutzfeldt-Jakob disease is almost certainly caused by the bovine spongiform encephalopathy agent, and although the disease is rare (115 deaths to date) there is uncertainty about future numbers of cases.1 The lack of a conventional immune response and the inability to detect abnormal prion protein in blood has hampered the development of a blood test.1 Lymphoreticular accumulation of prion protein has been used as a preclinical test for scrapie (the form of the disease in sheep and goats) and is a consistent feature of variant Creutzfeldt-Jakob disease, occurring before the onset of symptoms.24 We screened large numbers of specimens from appendicectomies and tonsillectomies for the presence of prion protein in lymphoreticular tissue to determine the number of people with preclinical variant Creutzfeldt-Jakob disease.

Methods and results

We retrieved appendix and tonsil samples removed between 1995 and 1999 from patients aged 10-50 from histopathology departments across the United Kingdom. The samples were anonymised before testing. We also examined appendix samples …

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