Mad Cow Disease
Mad Cow Disease, or bovine spongiform encephalopathy (BSE), is a disease in cattle which is likely to have originated in the UK. It is fatal and usually affects cattle between two and eight years of age. It has recently come into focus as its variant -- a rare brain disorder called Variant Creutzfeld-Jakob Disease (VCJD), has been recently found to affect humans too. Between 1995 and August 2006, 195 people were afflicted by this rare brain disorder. 162 cases were reported in the UK, 20 in France, 4 in Ireland, 2 in the United States, and 1 each in Canada, Italy, Japan, the Netherlands, Portugal, Saudi Arabia, and Spain.
 Link Between BSE in Cows and vCJD in Humans
No one realized the magnitude of the threat to public health when the first cases of Mad Cow Disease were identified in the 1980s. But by 1990 BSE was responsible for the death of 10,000 cattle. Only in 1996 when a link between BSE in cows and vCJD in humans was discovered that the true threat of Mad Cow Disease was realized.
The cause of this disease is attributed to a prion which spreads between cows via the animal byproducts in their feed. Only one piece of infected brain or spinal cord tissue about the size of a single grain of rice (10 milligrams) is enough to transmit BSE from one cow to another. Feed  describes what is ideal feed for cows and how they are fed in profit-driven farms.
The prion is a "self-replicating" protein, rather than a bacterium or virus which can enter the brain, central nervous system tissue, and the distal ileum (portion of the small intestine). The disease makes a portion of the brain sponge-like.
 Organic Beef as an Alternative
Mad cow scare has increased the popularity of organic farming. This is because of a consumer perception that mass-produced meat is more vulnerable to a fatal, brain-destroying disease that scientists believe is linked to a similar illness in humans.
It is estimated that sale of eco products have gone up 60 percent in the European Union due mainly to diminishing consumer confidence in the governments’ ability to ensure safe foods. What is holding back further growth of the market is the high cost of organic food. Read the advice of the Consumers Union  on meat labels that do and don’t help reduce risk of exposure to mad cow disease. Similar information can be found in Eco Labels 
 How BSE Works
BSE spreads following contact with the brain or other nervous-system tissues from an infected individual. Contact can be from food or contaminated food by-products or from instruments that had contacts with the diseased nervous tissue. The infectious agent can lie dormant for as long as 10 to 15 years after entering the brain. When activated, the agent kills the brain cells and runs its course in less than one year, ultimately causing death.
Symptoms among BSE infected cows are loss of weight, abnormal behavior (skittishness), paralysis and ultimately death. Humans afflicted with nvCJD first show signs psychiatric problems (paranoia) and other problems related with the senses. Other symptoms are muscle coordination problems, muscle spasms, problems with hearing, vision and memory loss. Humans too may finally go into coma and die.
Here are some facts about the agent that causes BSE:
• It is extremely minute, even smaller than a virus
• It can’t be killed by cooking or freezing – It needs much higher temperatures to die than what is required in cooking or sterilizing.
• Disinfectants don't work - Normal chemicals used for disinfecting surfaces for bacteria and viruses are not effective.
 What are Prions?
• Prions are proteins that can cause disease.
• Prions aren't alive, so can’t be killed. Proteins can be inactivated by de-naturing them (e.g., extreme heat, certain chemical agents), but these same processes usually destroy food. So there isn't an effective method to decontaminate beef.
• Prions naturally occur in the body, so they are not recognized as foreign and don't stimulate the immune system. They have the potential to cause disease, but won't automatically harm you.
• Disease-causing prions may physically contact normal prions, altering them so that they too can cause disease. The mechanism of prion action is not well understood.
 Beef Safety
• No correct information is available as to how much beef has to be consumed to cause infection.
• Nerve tissue (e.g., brain) and various ground meat products and by-products carry the infectious agents.
• Muscle tissue (meat) may carry the infectious agent.
• Rendering or processing foods can (with difficulty) destroy prions.
• Normal cooking will not destroy prions.
 What Causes Mad Cow Disease and Variant Creutzfeldt-Jakob Disease (vCJD)?
There are many kinds of proteins in animal cells. According to scientists, cow disease is triggered when there is a change in some of the proteins in animal cells caused by prions. There is no clear evidence as to how prions cause these changes. Nor is there evidence that these abnormal proteins are found in muscle meat (such as steak) or in milk.
Following slaughter, certain parts of the cow are used for human food and others for animal feed. If the animal feed is from an infected cow other cows can catch the infection. On rare occasions, people can develop vCJD if they eat the brain or spinal cord tissue of infected cattle.
 What are the symptoms of vCJD?
With Variant Creutzfeldt-Jakob disease (vCJD) the brain to deteriorates and results in death. Symptoms include:
• Dementia and psychotic behavior.
• With the progress of the disease a person is no longer able to walk as coordination problems develop
How is vCJD diagnosed?
No single test can diagnose variant Creutzfeldt-Jakob disease (vCJD). It is done mostly on symptoms, by studying the patient’s medical history, and a review of where the person has lived. Brain changes caused by vCJD can be mapped by imaging tests, such as an MRI.
The disease still cannot be detected by blood tests, though research is under way. A diagnosis of vCJD can now only be confirmed with a brain biopsy
How is vCJD treated?
There is no cure for vCJD.
 How to Protect Oneself from Mad Cow Disease
- Parts of the cow that are likely to carry the infection are the brain, ground products, which go to make hot dogs, bologna, or certain luncheon meats. Avoid eating items made from such infected parts.
- Avoid processed meat from an unknown source.
- Mad Cow Disease affects nervous tissue. It is better to avoid eating any part of an infected cow until it is known which part of the nervous system has been affected.
Eating all beef is not unsafe. Eating steaks, roasts, or burgers known to have been made from uninfected herds is perfectly safe. In processed meat products, however, it is difficult to ascertain the origin of the meat.
Is Cow's Milk a Source of BSE?
According to scientific research, BSE cannot be transmitted in cow's milk, even if the milk comes from a cow with BSE.
The World Health Organization (WHO) has stated that tests on milk from BSE-infected animals have not shown any BSE infectivity. Hence it is safe to drink milk and milk products, even in countries with a high incidence of BSE
When and How did BSE in Cattle Occur?
BSE in cattle was first reported in 1986 in the UK. The exact origins of BSE remain uncertain but it is thought that cattle initially may have become infected when given feed contaminated with scrapie-infected sheep meat-and-bone meal (MBM). The scientific evidence suggests that the U.K. BSE outbreak in cattle then was expanded by feeding BSE-contaminated cattle protein (MBM) to calves.
 Which Countries Have Reported Cases of BSE?
These countries are: Albania, Austria, Belgium, Bosnia-Herzegovina, Bulgaria, Croatia, Czech Republic, Denmark, Federal Republic of Yugoslavia, Finland, France, Germany, Greece, Hungary, Ireland, Israel, Italy, Liechtenstein, Luxembourg, former Yugoslavia Republic of Macedonia, The Netherlands, Norway, Oman, Poland, Portugal, Romania, Slovak Republic, Slovenia, Spain, Sweden, Switzerland, Japan, and United Kingdom (Great Britain including Northern Ireland and the Falkland Islands).
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