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Variant Creutzfeldt-Jakob disease in the United Kingdom: a countrywide or local risk?
  1. Anna M Molesworth1,
  2. Simon N Cousens2,
  3. Gill O Noel1,
  4. Hester J T Ward3
  5. on behalf of the local investigation teams
  1. 1Health Protection Agency Centre for Infections, London, UK
  2. 2London School of Hygiene and Tropical Medicine, London, UK
  3. 3National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Edinburgh, UK
  1. Correspondence to Hester J T Ward, Medical Director, Information Services Division, NHS National Services Scotland, Gyle Square, Edinburgh, EH12 9EB, UK; hester.ward{at}


Background The aim of this study was to identify factors that may have augmented local risks for variant Creutzfeldt-Jakob disease (vCJD).

Methods A descriptive study was conducted of local investigations of UK cases of vCJD, who had lived close together at some point since 1980. The main outcome measures were domestic, educational, occupational, healthcare associated, social and recreational links between cases; common dietary, iatrogenic and other possible routes of exposure to vCJD infection; and locally elevated vCJD risk.

Results A cluster of five cases of vCJD in a rural area in North Leicestershire was investigated in 2000 (p=0.004). A further 12 investigations of geographically associated cases of vCJD have been undertaken in the UK. In nine of the 12 locations, some or all of the local cases had consumed beef purchased from the same local retail outlets or provided by a common supplier of school meals, or had some aspect of their medical‑dental care in common. In only three of these locations were circumstances identified where the local risk of transmission might have been elevated. In none of the locations was there strong evidence to exclude chance as a likely explanation for the local occurrence of these vCJD cases.

Conclusion Although it is possible that in some parts of the UK local factors may have increased the risk of acquiring vCJD, most cases that were geographically close to each other are most likely due to the same factors that gave rise to the large majority of other vCJD cases in the UK.

  • Creutzfeldt-Jakob disease
  • epidemiology
  • cluster
  • geographical distribution
  • neuroepidemiology

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  • Funding No direct funding was involved in this study. The National Creutzfeldt-Jakob Disease Surveillance Unit is funded by the Department of Health and the Scottish Government.

  • Competing interests None.

  • Ethics approval Lothian MREC approved risk factor data retrieval as part of DH funded ongoing case‑control study by NCJDSU. Consent was obtained from families to share information with local investigation teams to carry out each ‘local’ investigation.

  • Provenance and peer review Not commissioned; externally peer reviewed.