Bovine Spongiform Encephalopathy (BSE), commonly known as Mad-Cow Disease (MCD), is a fatal, neurodegenerative disease in cattle, that causes a spongy degeneration in the brain and spinal cord and also causes red eyes. BSE has a long incubation period, about 4 years, usually affecting adult cattle at a peak age onset of four to five years, all breeds being equally susceptible.[1] In the United Kingdom, the country worst affected, more than 179,000 cattle have been infected and 4.4 million slaughtered during the eradication programme.
Mad Cow Disease Causes
Prion disease may develop sporadically, for no apparent reason and with no pattern such as sporadic CJD. Cases can occur in men and women of all ages, but the average age is 62 years. The prevalence of sporadic CJD is about 1 case per million people each year throughout the world, even among vegetarians. Sporadic CJD is the most common type of human prion disease.
The exact cause of BSE is not known but it is generally accepted by the scientific community that the likely cause is infectious forms of a type of protein, prions, normally found in animals cause BSE. In cattle with BSE, these abnormal prions initially occur in the small intestines and tonsils, and are found in central nervous tissues, such as the brain and spinal cord, and other tissues of infected animals experiencing later stages of the disease.
Prions
Stanley Prusiner, a physician at the University of California at San Francisco, won the 1997 Nobel Prize for Medicine for his assertion that Transmissible Spongiform Encephalopathies are caused by misshaped proteins found in mammalian brains. He called these proteinaceous infectious agents prions.
Mad Cow Disease Symptoms
As the diseases progress, mental symptoms become more severe. Most people eventually lapse into a coma. Heart failure, respiratory failure, pneumonia or other infections are generally the cause of death. The disease usually runs its course in about seven months, although a few people may live up to one or two years after diagnosis.
What's Being Done?
If you're worried about mad cow disease, tell whoever buys the food in your household about how you feel. The type of protein that causes mad cow disease cannot be removed or destroyed when beef is processed or cooked. For this reason, the U.S. government has established several meat processing procedures to protect the public.
How is vCJD diagnosed?
There is no single test to diagnose variant Creutzfeldt-Jakob disease (vCJD). Doctors may suspect that a person has vCJD based on symptoms, medical history, and a review of where the person has lived. Imaging tests, such as an MRI, may be done to check for brain changes caused by vCJD.
'Mad Cow' treatment
Diseases such as mad cow disease (bovine spongiform encephalopathy or BSE), which are caused by faulty proteins called prions, could be treated by adding closely related, noninfectious prions from other species. This sort of therapy could increase the incubation period of the disease well past a human lifetime, according to a theoretical study by physicists at the University of California, Davis.
Behavior modification may be helpful, in some cases, for controlling unacceptable or dangerous behaviors. This consists of rewarding appropriate or positive behaviors and ignoring inappropriate behaviors (within the bounds of safety). Reality orientation, with repeated reinforcement of environmental and other cues, may help reduce disorientation.
