Sunday, August 25, 2013

Understanding Creutzfeldt-Jakob Disease

By Armand Zeiders

Neurodegenerative diseases are some of the most troubling to mankind, especially because there are no cures at present. Alzheimer's disease, for instance, is extremely distressing for the patient and the family and can linger for many years. Creutzfeldt-Jakob disease is another distressing, albeit very rare, neurological disease that has no cure. Unlike Alzheimer's, this disease kills its victims quickly, within just months after diagnosis.

There are several different types of CJD, and one type is known as familial or inherited CJD. Inherited cases of CJD account for only about 10 percent of the cases in general and are caused by a genetic mutation. The most common form of CJD is known as sporadic CJD and occurs in people with no family history or other risk factors.

Of the two rarest forms of CJD, the one most commonly known is acquired by patients who eat meat infected by bovine spongiform encephalopathy, which is better known as mad cow disease. Although rare, an outbreak in the United Kingdom was responsible for the deaths of 200 people in the U.K. and more than 40 more people from other countries. There were more than 200,000 cattle found affected with mad cow disease, and the U.K. government ended up euthanizing 4 million animals to eradicate the problem.

This disease can also be passed on from human to human, but only in extremely rare cases, known as iatrogenic CJD. This comes from infected corneal grafts or contaminated dural grafts or electrode implants. It also has been found to transmit from infected human growth hormone, although this is no longer an issue as the drug that contained the infected hormone has not been sold since 1985. Because of the problems in the United Kingdom, many blood donation agencies do not allow blood donations from anyone who spent an extended amount of time in the U.K. from the years 1980 through 1996.

Prions are considered by most scientists to be the cause of the disease, and a prion is a protein that has misfolded and then replicated itself many times. This tears holes in the tissue of the brain and it eventually looks a bit like a sponge. Common early symptoms include personality changes, especially in vCJD, and dementia is also a typical symptom. Anxiety, psychosis and paranoia are often symptoms, as well as problems with speech and coordination.

There are no medical treatments available for any type of CJD and no cure. Scientists currently are studying to find out whether the prion is the cause of the disease or whether the disease causes the prion to occur. To do this, custom protein expression and custom protein sequencing services are often purchased from biotechnology companies that specialize in these services.

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