Creutzfeldt-Jakob disease (Mad Cow Disease)
- Posted on- Sep 29, 2015
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Mad cow disease is a neurological disease in which certain abnormal proteins known as prions induce the formation of lesions/plaques in the brain tissue of the infected animal. This disease is as old as 300 years, when it was reported in sheep as 'scrapie'. Other than cows and sheep, this disease can infect cats, deer, and elk as well. However, the bad news is that the infection is not restricted only to animal species. Mad cow disease, in humans, is known as the variant Creutzfeldt-Jakob disease (vCJD), or the new variant Creutzfeldt-Jakob disease (nvCJD), and there is strong evidence that it is contracted by eating beef that came from infected cows.
Causes of Creutzfeldt-Jakob disease
The classic Creutzfeldt-Jakob disease was described in the 1920s by German neurologist Hans Gerhard Creutzfeldt and Alfons Maria Jakob, giving the disease its name. Although it is often called the mad cow disease in human beings, this is a wrong association. The mad cow disease which is also known as the bovine spongiform encephalopathy (BSE), CJD, vCJD, and nvCJD are all types of transmissible spongiform encephalopathy (TSE), which is characterized by spongy appearance of the brain tissue of the infected organisms.
Creutzfeldt-Jakob disease can occur in three different ways. In over 80% of the cases, it occurs sporadically for no apparent reason. Almost 5% to 10% of the cases of CJD are hereditary, that are passed on as a result of gene mutation. A small percentage of classic CJD is contracted through contamination. Although some researchers dispute the role of the prion, till date, the prion theory is the most widely accepted cause of the TSE. However, greater characterization of this protein is required.
The disease in humans is believed to have been caused by consumption of infected cattle beef, carrying the agent of BSE. Besides the co-relation between the consumption of infected beef products, the fact that healthy cows when fed with tissue infected with BSE agent (prion) also contracted the disease has lent further ground to the theory of the disease being transmitted to human beings through infected bovine tissue.