Human prion diseases, atypical

Keywords Animal Atypical Atypical/Nor98 scrapie BSE-H BSE-L Human Prion disease Prion strain Prion type... 

One explanation that has been proposed for the differences in occurrence of human and animal prion diseases is age-distribution. The farm animal population at highest risk for development of sporadic or familial prion disease is the elderly segment, which is normally quite small in production animals. The likelihood of discovery is further reduced by low disease incidence and challenging diagnostics. Thus, large-scale surveillance programs, with particular scrutiny of the populations at risk, have proved vital in the discovery of atypical ruminant prion diseases, as detailed in this chapter. 

There has been great concern over the large-scale outbreak of BSE that occurred in the UK from 1988 as a result of feeding cattle with supplements prepared fiom sheep and cattle offal. Brain extracts firm BSE cattle have transmitted the disease to mice, sheep, cattle, pigs and monkeys. Studies of 12 recent cases of atypical CJD in the UK have provided evidence that the bovine prions have infected humans through the consumption of contaminated beef... 

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