Peter Stent was an experienced farmer, but had never seen such thing. A few days before 1984 Christmas, one of his cows from Pitsham Farm in South Downs, England began to lose weight, and could not keep balance and behave very strangely. When the vet arrived, the animal seemed that it had lost its mind. And when it died 6 weeks later, the farmer noticed the same symptoms on the other cows. Soon, nine cows died and no one could explain why.
During the same period, Alison Williams was 20 years old and lived in Caernarfon village in Nordh Wales. She was a brilliant and social student, who liked to swim and to navigate with boat on the nearby mountain lakes. But her personality was suddenly changed at the age of 22. She gave up on her studies and she went to live with her parents and brother. He started to prefer solitude spending hours looking out the window. In 1992 was diagnosed with nerves collaps, and in 1995, became paranoid and incontinence. With a month before she died, she lost her eyesight and the ability to speak. She spent her last 5 days in a coma.
I am convinced that we all heard such stories and noticed the links between them. Since the “mad cow” disease first emerged, or bovine spongiform encephalopathy, has so far killed more than 200,000 cows in England and Europe. And human variant, which the student Alison Williams contracted, made 94 victims. But these figures marks only the beginning. Once some animals have become ill, modern practices of cattle raise guarantee there’ll be millions. From England, the leftovers of the infected animals with bovine encephalopathy agent were exported worldwide, as flour that mixes with forage for cattle. In this way, the producing agent of the disease has reached 80 countries and over million of people, not only in Europe but also in Russia and Asia consumed the meat of animals that were fed with carcasses flour from England.
After a period of hesitation, the governments of Britain and Europe took drastic measures to stop the spread of disease. In England, the number of new cases is decreasing. Importing the cattle forage from England was banned in the US since 1988, but in many developing countries was nothing done to prevent the emergence of the disease. No one knows how many cattle around the globe are in the incubation stage nor how many people are contracting the disease. “We do not know how many victims will be in the coming years, “says specialist Dr. Frederick Saldmann from France. In fact, science itself is only now beginning to understand the disease. As transmissible spongiform encephalopathy disease occurs spontaneously in sheep, cattle, mink, deer and people. And once contracted, the disease can be spread.
Some transmissible spongiform encephalopathies forms are limited to a single species, while others ignore such things. But every form is fatal and has no cure destroying the brain, usually after a long period of latency causing symptoms ranging from dementia to psychosis and paralysis.
If the today accepted theory is fair, then the disease is not the product of germs, but prions, normal protein molecules which are infectious when they take an abnormal shape.
Prions are not “seen” by the immune system, so they are not attacked, are resistant to strong solvents and extreme temperatures. Can be frozen, boiled, kept in formaldehyde, carbolic acid or chloroform without losing its pathogenicity.
Prions history starts in 1730, when pastors in England and Europe have described the disease called “scrapie”, by which was meant a condition usually fatal to sheep, characterized by spastic contractions, excitability, severe itching, excessive thirst, emaciation, weakness and paralysis. As Peter Stent cows, sick sheeps became agitated, then, within 3 to 6 months were presenting seizure, paralysis, blindness and ultimately death. Scrapie is encountered even today in sheeps, but it seems that it can’t be transmitted in humans.
Dr. Carleton Gajdusek did not know anything about scrapie when reached to Papua New Guinea in the 50s. But there encountered something similar at the Fore indigenous, in the mountains of the East, especially in women and children. Indigenous called the disease kuru; had an evolution of about 16 months.
Instead of burying their dead, the indigenous ate them, especially the ones who deceased of kuru. The examination of the brain showed changes similar to Creutzfeldt-Jakob disease, a rare brain and fatal disease, discovered by the german scientists in 1920. In both states, the brain presents “vacuoles”, small cavities like those of a sponge. But while Creutzfeldt-Jakob disease was a biochemistry state, occurring slowly and randomly across the globe, kuru was spreading like a plague.