Bovine spongiform encephalopathy (BSE) is the scientific name for “Mad Cow Disease”. This is a fatal disease that affects the brain and central nervous system of cows. The human form of mad cow disease is known as Creutzfeldt – Jakob disease (CJD). Even though the idea of becoming sick and dying from eating a sausage or a hamburger is pretty scary thought it should be noted that Creutzfeldt – Jakob disease is very rare and difficult to contract.
It’s so rare that as of November 2006 there were only 200 individuals worldwide diagnosed with mad cow disease, including 164 people in the United Kingdom, 21 in France, 4 in the Republic of Ireland, the 3 in the US, 2 in the Netherlands, and 1 each in Canada, Italy, Japan, Portugal, Saudi Arabia, and Spain. (Kugler) The disease first appeared in cows in Great Brittan in 1985 (Sheen, 2005). An eight year old cow trembled, lost weight, and showed aggressive behavior towards the farmer and other cows.
No one could find what was wrong with her and without any sign of infection, it was impossible to determine what was causing the cow’s illness (Sheen, 2005). By 1987, 420 cows had died of the disease. Scientists then performed autopsies on the cattle. They discovered that all the cows had holes in their brain. The holes were spread although out the brain giving it the appearance of a sponge. These holes would eliminate brain cells and neurons, making it impossible to for messages to be transmitted to the body. (Sheen, 2005).
The suspected cause was a prion transmitted in meat and bone meal products fed to cattle there, the government banned the practice of feeding such products that may contain diseased tissue to animals in 1988. By then, however, infected cattle had already entered the human food supply. (Wisniewski, 2005) In both humans and cows the symptoms can take from a few months to 30 or 40 years to submerge but the results are always fatal. (NINDS, 2009) At the longest death will occur within a year of the occurring symptoms.
Creutzfeldt – Jakob disease is most likely transmitted through eating meat products infected with BSE from cows. (Sheen, 2005) The disease is not bacteria, fungi, or a virus, but a type of protein called prions. First a prion will enter a brain cell, and change normal proteins into prions as well. A cell cannot break down prions so the prions continue to reproduce and build as plaque. The cell then dies and the prions release to infect more cells. (Sheen, 2005) This process continues until the organism eventually dies.
Prions are also near indestructible. Symptoms first start as psychological problems such as anxiety, depressioin, or a short temper. Then it gets worse to more physical problems such as clumsiness or the victim making sudden jerking movements. The illness will get so bad that eventually a once healthy person who runs and works everyday will become as helpless as an infant. Basic tasks like eating and urinating becomes a difficult task. The first teenage victim in Brittan was Stephen Churchill.
In the fall of 1994, 18 year old Stephen Churchill began acting strangely. His father said “he became quiet, withdrawn, and depressed. ” (Sheen, 2005). Based on these symptoms he was diagnosed with depression antidepressant medication, which had no effect on him. It eventually got worse. One day Stephen and his mother went to the grocery store and they drank tea and ate donuts. When they left to go home Stephen’s mother asked if he liked it but he had already forgot what he had eaten 15 minutes later.
Making matters worse Stephen started to have hallucinations. One day he was watching Baywatch and he felt as though he was drowning. Medication to stop hallucinations was prescribed and it still had no effect on Stephen. By January 1995 Stephen was unable to walk or speak clearly. He began to jerk and tremble and could not feed himself. Stephen’s parents were told that his problem was a neurological problem and that death was inevitable. Indeed, Stephen lost more and more muscle control.
In May, 1995, eighteen months after exhibiting symptoms, Stephen passed away. (Sheen, 2005) Doctors now know that Stephen’s death and others was caused by simply eating one bad piece of meat. No cure is known on how to stop mad cow disease. More research needs to be done on how it can be stopped, but due to the fact that it’s such a rare disease it’s very difficult to research. This report is not to dismay people from eating beef but just to educate those about the disease and it’ s effects on the world.