Elsevier

The Lancet

Volume 354, Issue 9175, 24 July 1999, Pages 317-323
The Lancet

Review
Variant Creutzfeldt-Jakob disease

https://doi.org/10.1016/S0140-6736(99)05128-4Get rights and content

Summary

It is clear that the prion strain causing bovine spongiform encephalopathy (BSE) in cattle has infected human beings, manifesting itself as a novel human prion disease, variant Creutzfeldt-Jakob disease (CjD). Studies of the incubation periods seen in previous epidemics of human prion disease and of the effect of transmission barriers limiting spread of these diseases between species, suggest that the early variant CJD cases may have been exposed during the preclinical phase of the BSE epidemic. It must therefore be considered that many cases may follow from later exposure in an epidemic that would be expected to evolve over decades. Since the number of people currently incubating this disease is unknown, there are concerns that prions might be transmitted iatrogenically via blood transfusion, tissue donation, and, since prions resist routine sterilisation, contamination of surgical instruments. Such risks remain unquantified. Although variant CJD can be diagnosed during life by tonsil biopsy, a prion-specific blood test is needed to assess and manage this potential threat to public health. The theoretical possibility that BSE prions might have transferred to other species and continue to present a risk to human health cannot be excluded at present.

Section snippets

Has the BSE agent infected human beings?

The transmissibility of the prototypic prion disease, scrapie, between sheep (and goats) was shown in 1936 by experimental inoculation,20 and the neuropathological similarities between scrapie and the human disease kuru led to the suggestion that kuru may also be transmissible.21 Kuru and then CJD were transmitted to primates in the 1960s.22, 23 Transmissible mink encephalopathy and chronic wasting disease of mule deer and elk were desribed from the 1940s onwards. The appearance of BSE in UK

Scale of the epidemic

Factors that are important in determining the probability of BSE transmission to an individual include dose, route of exposure, genetic susceptibility, and the height of the species barrier between cattle and human beings. The latter is expected to be different for individuals with different PRNP genotypes. In addition, the species barrier varies with prion-strain type, although BSE is thought to be caused by a single strain. To date, however, only nine cattle brains, from more than 170 000

Remaining routes of transmission of BSE and variant CJD

The substantial extension of measures to limit dietary exposure to BSE prions in March, 1996, especially the 30-month rule (whereby only animals younger than this age can be used for human foodstuffs), allied with the continued decline in the UK BSE epidemic, should have ensured that any cattle BSE entering the human diet is kept to a minimum, if significant at all, compared with earlier exposure. It is still theoretically possible, however, that BSE could have been transmitted to other

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