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Assessing risk is the business of prion disease research

American Society for Clinical Investigation
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BSE 4/20/2010 1 All About BSE The word BSE is short but it stands for a disease with a long name, bovine spongiform encephalopathy. "Bovine" means that the disease affects cows, "spongiform" refers to the way the brain from a sick cow looks spongy under a microscope, and "encephalopathy" indicates that it is a disease of the brain. What Is BSE? BSE is a progressive neurologic disease of cows. Progressive means that it gets worse over time. Neurologic means that it damages a cow’s central nervous system (brain and spinal cord). What Causes BSE? 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. Without knowing it is there, the cow’s body cannot fight off the disease. What Are The Symptoms Of BSE? A common symptom of BSE is incoordination. A sick cow has trouble walking and getting up. A sick cow may also act very nervous or violent. 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. 4/20/2010 2 Currently, there is no reliable way to test for BSE in a live cow. After a cow has died, scientists can tell if it had BSE by looking a

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