ReviewVariant Creutzfeldt-Jakob disease
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
References (67)
- et al.
Prion dementia without characteristic pathology
Lancet
(1990) - et al.
Genetic predisposition to iatrogenic Creutzfeldt-Jakob disease
Lancet
(1991) - et al.
Aminoacid polymorphism in human prion protein and age at death in inherited prion disease
Lancet
(1991) Scrapie and kuru
Lancet
(1959)- et al.
Molecular screening of sheep for bovine spongiform encephalopathy
Neurosci Lett
(1998) - et al.
A new variant of Creutzfeldt-Jakob disease in the UK
Lancet
(1996) - et al.
A new variant of prion disease
Lancet
(1996) - et al.
“Friendly fire” in medicine: hormones, homografts, and Creutzfeldt-Jakob disease
Lancet
(1992) - et al.
Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication
Cell
(1990) - et al.
Prion protein gene analysis in new variant cases of Creutzfeldt-Jakob disease
Lancet
(1996)