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Mad Cow Disease

Mad cow disease or more accurately BSE is a fatal neurodegenerative disease found in cattle that results in the deterioration of the brain and spinal chord. The incubation period may be as much a four years and affects adult cattle; all breeds are also prone to getting the disease. As the disease progresses the animal will exhibit strange behaviours including inability to stand because of irreversible brain degeneration. The disease appeared fairly recently and has been determined to have begun because cattle were being fed the remains of other infected animals mainly sheep and other cattle. However, the true origin of the disease has still not yet been discovered.

The disease is also transmissible to humans in the form new variant Creutzfeldt-Jakob disease or vCJD. It is transmited to humans when infected meat from cattle is consumed. Unfortunately cooking the meat does not necessarily destroy the prion as it is able to survive in relatively high temperatures. It has been responsible for almost two hundred deaths worldwide with the majority of deaths occuring in Britain.

Causes

Creutzfeldt-Jakob disease is a prion disease meaning that it is caused by an infection with a prion (a misfolded protein) which is neither bacterial or viral in origin. The disease also has a genetic link which makes up for an estimated 5-10% of CJD cases. A mutation of the gene that codes for the protein PRNP may lead to the development of the disease in individuals with this genetic variant.Mad Cow Disease 300x200 Mad Cow Disease

Symptoms

Dementia is usually the initial symptom to materialize with the disease subsequently leading to personality changes, hallucinations and pronounced memory loss. Victims may also develop speech problems, ataxia or loss of coordination and balance, seizures, stiff posture, alterations in gait (how one walks or runs), and Myoclonus or jerky movements.

The disease may have varying duration periods, however, the sporadic forms of the disease tend to have relatively shorter incubation periods and cause more rapid degeneration of the brain and spinal cord tissue.

The symptoms that will manifest are as a result of nerve cell death related to the accumulation of prions. It has been observed that brain tissue removed from persons afflicted with the disease will contain many tiny holes where nerve cells have died when viewed under a microscope. The brain tissue will be sponge like because of the progressive deterioration.

Treatment

There is no cure for vCDJ and it is always fatal within months or years after the onset of symptoms. Treatment may be given to alleviate some of the associated symptoms but will not slow or prevent the progress of the disease.

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