Bovine Spongiform Encephalopathy

BSE (Mad Cow Disease) is a disease affecting cattle characterized by early dementia, bizarre behaviour and uncontrollability which often results in the animal having to be killed or killing itself. The name Mad Cow Disease has been applied to affected animals. The recent scares about BSE and disease in human dates back to the first outbreak of BSE in Britain in 1986. BSE has never been reported in Australia.

It was discovered that BSE was similar to other animal diseases including Scrapie (sheep) and Transmissible Mink Encephalopathy (rodents) and was probably caused by an infected protein, or Prion. What alarmed scientists was the potential for the prion to be passed between species, and potentially to man. Cattle with BSE had be shown to have eaten feed containing sheep’s brains from animals affected by scrapie. It was therefore reasoned that such degenerative brain disorders might be passed on to humans who ate infected animals.

Tradition amongst New Guinea Highlanders includes the eating of the brains of revered tribesmen, and a dementia known as Kuru was seen as a result. Under the microscope, the brains of affected humans and animals developed large holes, like a sponge, and this was manifested as dementia whilst the person or animal was still alive. This microscopic picture was also seen in the rare human disorder Creutzfelt Jakob Disease, or CJD, a pre-senile dementia described in the 1940's.

Since Kuru had been demonstrated to be transmissible between humans, and since the microscopic appearance of CJD was so similar, it was felt that CJD might also be passed on in this fashion. Unfortunately this was found to be the case: some 7 or so Australian recipients of donated brain tissue and hormones used to promote growth in under-developed children have contracted CJB from the donor tissue.

Further investigation is now underway to examine the relationship between CJD and BSE, with the conjecture that the Prion and its effects can be passed from animal to man after meat from affected animals is eaten.

Toll-free Australian BSE Information line 1800 02 06 12

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