Creutzfeldt-Jakob disease is a very serious brain disease that occurs roughly to about 6,000 people per year throughout the world. Creutzfeldt-Jakob disease is more commonly known as CJD. There appear to be three general categories for classifying the means through which CJD may be acquired. First, the disease can occur sporadically, i.e. without apparent cause. Second, the disease can be inherited. Third, the disease can be transmitted through infection. CJD is named after Drs. Hans Gerhard Creutzfeldt and Alfons Jakob, who documented the first cases of this illness in the 1920’s.
CJD has also been experimental linked the variant forms of BSE, known as mad cow disease found in beef. The disease affects cows through food that has been contaminated by infected cows. The meat is then eaten and spread to humans. The incident was serious enough, that when discovered in the UK in 1986, led to a world ban on British beef. The incidence of BSE in the United Kingdom reached its peak of over 36,000 confirmed cases in one year in 1992, and has been steadily declining. There have been over 180,000 BSE cases worldwide, with the overwhelming majority, over 95%, occurring in the United Kingdom. The British government has taken steps to eliminate this threat, but recently a cow was found contaminated in Canada. This has lead to Canada beef being banned here in the United States.
Creutzfeldt - Jakob disease is caused by a protein called a "prion”, short for proteinaceous infectious particle, which has a distinctive shape. Each protein that gets near the abnormal one will take its shape and the disease spreads like the domino effect consuming the body. This directly affects the nerve cells in the brain that cause them to die. According to an article from ABC news, the disease seems to make tiny holes in the brain. The patient will have memory lose followed by depression, confusion, personality and behavioral changes. As the disease advances, the patient experiences a rapidly, progressive dementia and in most cases, involuntary and irregular jerking movements known as myoclonus. At the final stages, the patient will loose all mental and physical abilities. In the final days the patient will fall into a coma using dies from an infection like pneumonia.
Overall this disease is very hard to diagnose and even harder to treat. The disease hits so fast that by the time it is actually diagnosed it has done so much damage that possible treatments are useless. Scientist continue to research this issue and hope the drug quinacrine, an old antimalarial drug, will have promising results. As of now there is no known cure for this fatal disease.
Articles:
http://abcnews.go.com/sections/living/Healthology/HO_madcow.html
