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vCJD: the epidemic that never was

BMJ 2002; 325 doi: (Published 13 July 2002) Cite this as: BMJ 2002;325:102

New variant Creutzfeldt-Jakob disease: the critique that never was

  1. R G Will, consultant neurologist,
  2. R S G Knight, consultant neurologist,
  3. H J T Ward, consultant epidemiologist,
  4. J W Ironside, director
  1. National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Edinburgh EH4 2XU
  2. Department of Neurology, Leeds Teaching Hospital NHS Trust, Leeds LS9 7TF
  3. Burnley General Hospital, Burnley BB10 2PQ
  4. 29/8 Inverleith Place, Edinburgh EH3 5QD

    EDITOR—Venter's article on new variant Creutzfeldt-Jakob disease (vCJD) is intended to stimulate debate, which we hope will be better informed than the article itself.1

    Is variant Creutzfeldt-Jakob disease a new disease? Venters places great emphasis on Creutzfeldt's case, but this patient did not have Creutzfeldt-Jakob disease. The illness was characterised by gait disturbance, a relapsing and remitting course, nystagmus, and status epilepticus. These are not the clinical features of variant Creutzfeldt-Jakob disease and, crucially, the neuropathological appearances were “not characterisitic of Creutzfeldt-Jakob disease.”2

    In 1996 confidence in the novelty of variant Creutzfeldt-Jakob disease was based largely on the identification of a neuropathological phenotype that was distinct from that experienced in the United Kingdom from 1970. Since then archival tissues have been reviewed in many countries and no past cases with a similar neuropathological pattern have been found.3 Retrospective epidemiological surveys in England and Wales have not identified any missed cases of variant Creutzfeldt-Jakob disease from 1979-96.4 Current evidence strongly supports the hypothesis that variant Creutzfeldt-Jakob disease is indeed a new disease.

    Was variant Creutzfeldt-Jakob disease identified solely because of improved surveillance? There was a doubling in the annual death rates for sporadic Creutzfeldt-Jakob disease in the United Kingdom between the 1980s and the 1990s, but similar increases in the apparent death rates for sporadic Creutzfeldt-Jakob disease had occurred in other European countries.5 These countries have been subject to similar potential improvements in case identification, but variant Creutzfeldt-Jakob disease remains a disease occurring predominantly in the United Kingdom, despite significant numbers of young suspect cases being investigated in each country (figure).

    Death rates for sporadic Creutzfeldt-Jakob disease and total numbers of deaths from variant Creutzfeldt-Jakob disease by country

    Venters argues correctly that the curve for the epidemic of variant Creutzfeldt-Jakob disease does not parallel the number …

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