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Variant Creutzfeldt-Jakob Disease (vCJD): Clinical Presentation and Relationship to Bovine Spongiform Encephalopathy (BSE)

Variant Creutzfeldt-Jakob disease (vCJD), first described in 1996, is a rare and fatal human neurodegenerative disorder. The Centers for Disease Control and Prevention (CDC) states that strong scientific evidence exists to support that the agent responsible for BSE in cows is also responsible for vCJD in humans. Humans may contract the disease by consuming food that contains brain tissue from cattle infected with the bovine form of the disease. (The content of brain tissue may be higher in some food products than others. Contamination may also depend on the way the animal was slaughtered.) The resulting disease is known as variant CJD (or vCJD). 
The initial symptoms of vCJD are more psychiatric in nature, which often leads to a misdiagnosis. The initials symptoms include depression, withdrawal, anxiety, and trouble sleeping. After a few short months the infected individual will experience muscle spasms and a lack of muscle control. As the disease rapidly progresses, patients will generally experience visual deterioration, dementia, and muscle paralysis. In contrast to traditional forms of CJD, vCJD affects younger patients, the median age being 28 years and the median duration of illness approximately 13-14 months. The majority of vCJD cases have occurred in the United Kingdom. However, a very limited number of cases have also been reported in several European countries as well as Japan, Taiwan, Saudi Arabia, Canada, and the United States.