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Irregular presence of abnormal prion protein in appendix in variant Creutzfeldt-Jakob disease
  1. S Joiner,
  2. J Linehan,
  3. S Brandner,
  4. J D F Wadsworth,
  5. J Collinge
  1. MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK
  1. Correspondence to:
 Professor John Collinge;

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We have investigated the presence of disease related prion protein (PrPSc) in appendix samples obtained at necropsy from four neuropathologically confirmed cases of variant Creutzfeldt-Jakob disease (vCJD). PrPSc was detected in only one vCJD appendix, at a level lower than found in a diagnostic tonsil biopsy sample obtained from the same patient. The single PrPSc positive appendix, but not the other samples, also showed abnormal prion protein immunohistochemistry. The finding that appendix samples from three of four cases of vCJD are devoid of detectable PrPSc questions the utility of screening archival appendicectomy tissues to estimate the prevalence of pre-clinical vCJD infection within the UK population.

The appearance of a novel human prion disease, variant Creutzfeldt-Jakob disease (vCJD), in the United Kingdom from 1995 onwards, and the experimental confirmation that this is caused by the same prion strain as that causing BSE in cattle, has raised the possibility that a major epidemic of vCJD will occur in the United Kingdom and other countries as a result of dietary or other exposure to BSE prions.1 The pathogenesis of vCJD differs significantly from that of other forms of CJD. Disease associated prion protein (PrPSc) is readily detectable in lymphoreticular tissues in vCJD and not in classic CJD.1–3 High levels of PrPSc are uniformly found in the central nervous system and lymphoreticular system of vCJD patients.2,3 The highest levels of PrPSc seen outside the central nervous system in vCJD are in tonsil (about 10% of that found in brain)2,3 and tonsil biopsy is used for ante-mortem diagnosis of vCJD.1–3 To date, positive prion protein immunohistochemistry has been reported in only a single appendix sample, although, importantly, this was …

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  • Competing interests: none declared