Mad Cow Disease

Tuesday, November 16, 2021 1:20:32 PM

Mad Cow Disease



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The word BSE is short but it stands for a disease with a long name, bovine spongiform encephalopathy. BSE is a progressive neurologic disease of cows. Progressive means that it gets worse over time. Most scientists think that BSE is caused by a protein called a prion. For reasons that are not completely understood, the normal prion protein changes into an abnormal prion protein that is harmful. The body of a sick cow does not even know the abnormal prion is there.

A common sign of BSE in cows is incoordination. A sick cow has trouble walking and getting up. It usually takes four to six years from the time a cow is infected with the abnormal prion to when it first shows symptoms of BSE. This is called the incubation period. During the incubation period, there is no way to tell that a cow has BSE by looking at it. Once a cow starts to show symptoms, it gets sicker and sicker until it dies, usually within two weeks to six months. There is no treatment for BSE and no vaccine to prevent it. Currently, there is no reliable way to test for BSE in a live cow. After a cow dies, scientists can tell if it had BSE by looking at its brain tissue under a microscope and seeing the spongy appearance.

Scientists can also tell if a cow had BSE by using test kits that can detect the abnormal prion in the brain. Brain from a cow sick with BSE, as seen under a microscope using special stains. The large white spaces are like the "holes" of a sponge. Most cases of Creutzfeldt-Jakob disease occur for unknown reasons, and no risk factors can be identified. However, a few factors seem to be associated with different kinds of CJD :. Exposure to contaminated tissue. People who've received infected manufactured human growth hormone, or who've had transplants of the infected tissues that cover the brain dura mater , may be at risk of iatrogenic CJD.

The risk of getting vCJD from eating contaminated beef is very low. In general, if countries are effectively implementing public health measures, the risk is virtually nonexistent. Chronic wasting disease CWD is a prion disease that affects deer, elk, reindeer and moose. It has been found in some areas of North America. To date, no documented cases of CWD have caused disease in humans. Creutzfeldt-Jakob disease greatly affects the brain and body. CJD usually progresses quickly. Over time, people with CJD withdraw from friends and family and eventually lose the ability to recognize or relate to them.

They also lose the ability to care for themselves and many eventually slip into a coma. The disease is always fatal. If you have a family history of neurological disease, you may benefit from talking with a genetics counselor. He or she can help you sort through the risks associated with your situation. Hospitals and other medical institutions follow clear policies to prevent iatrogenic CJD. These measures have included:. This includes people who:. The U. Only four cases have been reported in the U. According to the U. Centers for Disease Control and Prevention CDC , strong evidence suggests that these cases were acquired in other countries outside of the U.

In the United Kingdom, where the majority of vCJD cases have occurred, fewer than cases have been reported. CJD incidence peaked in the U. A very small number of other vCJD cases also have been reported in other countries worldwide. Nonetheless, the CDC recommends that hunters strongly consider having deer and elk tested before eating the meat in areas where CWD is known to be present. In addition, hunters should avoid shooting or handling meat from deer or elk that appear sick or are found dead. Most countries have taken steps to prevent BSE-infected tissue from entering the food supply, including:. Our patients tell us that the quality of their interactions, our attention to detail and the efficiency of their visits mean health care like they've never experienced.

Eventually, clusters of brain cells are killed and deposits of misfolded prion protein called plaques may appear in the brain. Prion infections also cause small holes to develop in the brain, so it becomes sponge-like. The damage to the brain causes the mental and physical impairment associated with CJD, and eventually leads to death. Prions can survive in nerve tissue, such as the brain or spinal cord, for a very long time, even after death. The different types of CJD are all caused by a build-up of prions in the brain. But the reason why this happens is different for each type.

It's not known what triggers sporadic CJD, but it may be that a normal prion protein spontaneously changes into a prion, or a normal gene spontaneously changes into a faulty gene that produces prions. Sporadic CJD is more likely to occur in people who have specific versions of the prion protein gene. At present, nothing else has been identified that increases the risk of developing sporadic CJD. In , a government inquiry concluded that the prion was spread through cattle that were fed meat-and-bone mix containing traces of infected brains or spinal cords.

The prion then ended up in processed meat products, such as beef burgers, and entered the human food chain. Strict controls have been in place since to prevent BSE entering the human food chain, and the use of meat-and-bone mix has been made illegal. Almost all definite cases of vCJD occurred in people with a specific version MM of the prion protein gene, which affects how the body makes a number of amino acids. It's estimated up to 4 in 10 of the UK population have this version of the gene. There have been no confirmed deaths from to Some experts believe that the food controls have worked and further cases of vCJD will continue to decline, but this doesn't rule out the possibility that other cases may be identified in the future.

It's also possible for vCJD to be transmitted by blood transfusion , although this is very rare and measures have been put in place to reduce the risk of it happening.