Along the subject of late night talk show subjects, Mad Cow Disease is anything but a laughing matter. It's a deadly transmittable brain disease in cattle, and has been a significant drain on the meat supply in the U.K. It was first recognized in 1986 and is now an epidemic in the farming industry, responsible for 180,000 cases in the last four years. With Mad Cow Disease cases appearing in France and other countries, concerns about transmissions to humans have caused an unprecedented controversy in Europe.

Understanding Mad Cow Disease

Mad Cow Disease, also known as Bovine Spongiform Encephalopathy (BSE), is a disease associated with a transmissible agent that affects the brain and spinal cord of the cattle. What exactly this transmissible agent is still not clearly known, but some scientists refer to it as a prion, a virus-like agent that can carry genetic information, although it acts like no bacteria or virus. A prion-like agent is responsible for kuru, a human spongiform encephalopathy that was at one time a serious epidemic in New Guinea. In these types of diseases, post-mortem autopsies reveal the brain to have a sponge-like appearance with the presence of tiny holes (hence the name, spongiform).

The source of Mad Cow Disease appears to be from cattle feed consisting of recycled animal protein known as ruminants. This has resulted in the banning of the use of ruminant proteins in animal feed preparation in 1998. The offending agent apparently is able to withstand heating and freezing temperatures, extremes normally used in pasteurization. The concerns for Mad Cow Disease in Europe have led to unprecendented slaughtering of cattle, fearful of the spread of this disease. To date, there is no cure for Mad Cow Disease and it's always fatal.

"The Mad "Human" Disease" - Cruetzfeldt-Jakob Disease

Mad Cow Disease represents a type of disease known as Transmissible Spongiform Encephalopathies (TSEs) found in animals. Other animals with this fatal disease include sheep's, minks and goats. There exists in humans TSEs as well, with the prototype disease known as Cruetzfeldt-Jakob Disease.

Cruetzfeldt-Jakob Disease and a number of variant diseases are thought to be due to prions. Just as Mad Cow disease, it's a fatal degenerative disease of the brain, resulting in muscle wasting and dementia. Both have long incubation periods, very contagious and are highly resistant to disinfection. Cruetzfeldt-Jakob Disease is extremely rare, with about one case per million of the population per year.

Can Humans Get Mad Cow Disease?

Scientists believed that there was a "species barrier" for humans getting the Mad Cow Disease. However in Britain it was noted that in 1999, younger people were dying from Cruetzfeldt-Jakob Disease, which normally does not affect anyone younger than 30 years of age. What they deduced was that a new variant of Cruetzfeldt-Jakob Disease had surfaced, with origins from beef infected with Mad Cow Disease. Britain has currently been embroiled with scandal on whether the public had been adequately informed on the dangers in eating British beef. To date, approximately 48 persons in Britain have been thought to be dead from this variant disease. The long incubation of mad cow disease (10 years) precludes scientists to determine if this is an isolated event, or if the country is in store for a terrible epidemic.

Mad Cow Disease has devastated the British cattle industry, resulting in the slaughter of 2.5 million cattle and an international boycott on exported beef from Britain. Obviously, there is still much more research needed to be completed on prion diseases, and the long incubation period ensures us that it may be a while before we get answers to many of the questions this disease might poses.

For Your Information: Creutzfeldt-Jakob Disease (CJD) and Mad Cow Disease. Health Canada Publication, 2000.

"Report: British Misled about Mad Cow Disease", Chicago Tribune, Oct. 26, 2000.