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Home Eurosurveillance Weekly Release  2006: Volume 11/ Issue 4 Article 4
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Eurosurveillance, Volume 11, Issue 4, 26 January 2006

Citation style for this article: Molesworth AM, Andrews NJ. Variant Creutzfeldt-Jakob disease in the United Kingdom and elsewhere: situation at the end of 2005. Euro Surveill. 2006;11(4):pii=2884. Available online:

Variant Creutzfeldt-Jakob disease in the United Kingdom and elsewhere: situation at the end of 2005

AM Molesworth (, NJ Andrews

Health Protection Agency Centre for Infections, London, United Kingdom

By the end of December 2005, a total of 159 cases of variant Creutzfeldt-Jakob disease (vCJD) had been reported in the United Kingdom, of which 153 have so far resulted in death. Elsewhere numbers remain small, with 15 cases in France, 4 in Ireland, 2 in the United States, and 1 each in Canada, Italy, Japan, the Netherlands, Portugal, Saudi Arabia and Spain [1]*.

In the UK, five deaths from vCJD were reported in 2005, four less than the previous year’s total of nine. Results from modelling the incidence of deaths indicate that the current epidemic wave reached a peak of 28 deaths in 2000, and has since declined (Figure). Extrapolating this trend gives an estimate of 2 deaths in the next 12 months (95% prediction interval 0 to 5). With 6 patients alive at the end of 2005, however, a prediction of 2 deaths is likely to be an underestimate [2].

Figure. vCJD deaths by year, and fitted quadratic model for incidence trend.

It is important to note that, to date, all vCJD cases have been methionine homozygote at codon 129 of the prion protein gene. Preclinical vCJD infection has, however, been reported in a heterozygous patient after blood transfusion from a donor who subsequently developed vCJD [3]. Although the initial epidemic wave is now in decline, it is possible that there will be further epidemics of cases in other genetic groups. There is also the possibility of continuing person to person transmission through certain forms of health care (for instance, in relation to surgery, blood transfusion or treatment with plasma products). It is essential, therefore, to maintain and promote active surveillance of CJD to investigate these possibilities.

This article was adapted by the authors from reference 2

*Correction. When this article was published, this sentence was linked to a reference to the website for The European and Allied Countries Collaborative Study Group of CJD (EUROCJD,, last updated 2 November 2005). The latest figures have been provided by personal communication with RG Will, National CJD Surveillance Unit, Edinburgh, UK, January 2006. This change was made on Friday 27 January.
Eurosurveillance Editorial Office

  1. Health Protection Agency. Variant Creutzfeldt-Jakob disease in the United Kingdom and elsewhere: situation at the end of 2005. CDR Weekly 2006; 16(4): news. (
  2. Andrews NG. Incidence of variant Creutzfeldt-Jakob disease onsets and deaths in the UK. January 1994 – December 2005 . Edinburgh: The National Creutzfeldt-Jakob Disease Surveillance Unit; 19 January 2006. (
  3. Peden AH, Head MW, Ritchie DL, Bell JE, Ironside JW. Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 2004;364:527-9.

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