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

QU Communicable Disease Report Weekly 2006 Jan 26; 16(4)

IA http://www.hpa.org.uk/cdr/archives/archive06/News/news0406.htm#vcjd

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VT A cumulative total of 159 cases of variant Creutzfeldt-Jakob disease (vCJD) had been reported in the UK by the end of December 2005, of whom 153 had died. Outside the UK, the numbers remain small with 15 cases in France, four in the Republic of Ireland, two in the United States, and one each in Canada, Italy, Japan, the Netherlands, Portugal, Saudi Arabia, and Spain (1).
Recent data published by the National CJD surveillance unit shows that, in the United Kingdom (UK), a total of five deaths from vCJD were reported in 2005, four less than the previous year's total of nine (2). 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 two deaths in the next 12 months (95% prediction interval 0 to 5). With six cases alive at the end of 2005, however, a prediction of two deaths is likely to be an underestimate.
Figure vCJD deaths by year and fitted quadratic model for the 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 healthcare (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.
References
1. The European and Allied Countries Collaborative Study Group of CJD (EUROCJD) [online].The European and allied countries collaborative study group of cjd (eurocjd). [cited 26 Janaury 2006]. Available at: .
2.The National CJD surveillance unit [online]. Incidence Of variant Creutzfeldt-Jakob Disease onsets and deaths in the UK - January 1994 - December 2005. Available at: .
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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