Creutzfeldt-Jakob: A Fatal Disease

By Armand Zeiders


There are many serious degenerative neurological diseases, and like many other brain diseases, Creutzfeldt-Jakob disease is one that is not only incurable, but also fatal. CJD is an extremely rare disorder with fewer than 200 cases each year. From the time of diagnosis, death usually occurs in less than one year.

There are actually four variants of CJD, and the most common type is called sporadic CJD, and more than 80% of those with CJD have this particular form. In addition, these patients have no family history of the disease and no specific risk factors for CJD. About 10 percent of cases are inherited, which is called familial CJD or fCJD.

The remainder of cases, which are quite rare, fall into two categories. Variant Creutzfeldt-Jakob disease is acquired by eating food products that are contaminated by bovine spongiform encephalopathy, which is more commonly known as mad cow disease. This was a widespread problem in the United Kingdom, and the government destroyed more than 4 million cattle. About 200,000 cattle tested positive for mad cow disease, and more than 200 people died after eating tainted meat. While cooking meat at a high enough temperature can kill many bacteria, it has no effect on mad cow disease, and tainted meat is dangerous at any temperature.

Human to human transmission does not occur from casual contact nor is it airborne. However, coming into contact with parts of the brain infected with CJD can cause transmission of the disease, which is called iatrogenic CJD. This could be caused by receiving infected immunoglobulin or corneal grafts, as well as dural grafts. A few cases were linked to a drug containing infected human growth hormone, but this product was removed from the market in the 1980s. Blood transmission of vCJD also is possible and because of this, the American Red Cross and other organizations have forbidden individuals who spent three or more months in the United Kingdom during the 1980s through 1996 from donating blood.

No one knows why this disease occurs, but they do known that the disease develops because of misfolded proteins known as prions. The prions reproduce many times and eventually begin creating holes in the brain's tissue, and the tissue becomes sponge like. The first symptoms of CJD are often problems with memory, hallucinations and personality changes. The latter is especially true of the vCJD related to mad cow disease, and patients suffer from several mood changes as well as a heightened sense of touch which causes a great deal of pain.

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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By Armand Zeiders


There are many serious degenerative neurological diseases, and like many other brain diseases, Creutzfeldt-Jakob disease is one that is not only incurable, but also fatal. CJD is an extremely rare disorder with fewer than 200 cases each year. From the time of diagnosis, death usually occurs in less than one year.

There are actually four variants of CJD, and the most common type is called sporadic CJD, and more than 80% of those with CJD have this particular form. In addition, these patients have no family history of the disease and no specific risk factors for CJD. About 10 percent of cases are inherited, which is called familial CJD or fCJD.

The remainder of cases, which are quite rare, fall into two categories. Variant Creutzfeldt-Jakob disease is acquired by eating food products that are contaminated by bovine spongiform encephalopathy, which is more commonly known as mad cow disease. This was a widespread problem in the United Kingdom, and the government destroyed more than 4 million cattle. About 200,000 cattle tested positive for mad cow disease, and more than 200 people died after eating tainted meat. While cooking meat at a high enough temperature can kill many bacteria, it has no effect on mad cow disease, and tainted meat is dangerous at any temperature.

Human to human transmission does not occur from casual contact nor is it airborne. However, coming into contact with parts of the brain infected with CJD can cause transmission of the disease, which is called iatrogenic CJD. This could be caused by receiving infected immunoglobulin or corneal grafts, as well as dural grafts. A few cases were linked to a drug containing infected human growth hormone, but this product was removed from the market in the 1980s. Blood transmission of vCJD also is possible and because of this, the American Red Cross and other organizations have forbidden individuals who spent three or more months in the United Kingdom during the 1980s through 1996 from donating blood.

No one knows why this disease occurs, but they do known that the disease develops because of misfolded proteins known as prions. The prions reproduce many times and eventually begin creating holes in the brain's tissue, and the tissue becomes sponge like. The first symptoms of CJD are often problems with memory, hallucinations and personality changes. The latter is especially true of the vCJD related to mad cow disease, and patients suffer from several mood changes as well as a heightened sense of touch which causes a great deal of pain.

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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