Creutzfeldt-Jakob disease (CJD) is a rapidly progressive and invariably fatal brain disease that is caused by a prion, an infectious protein particle. You will find two major forms of CJD, a so-called classic form that is most common in adults between the ages of forty-five and sixty-five, and variant CJD (vCJD), which primarily affects younger adults in their mid- to late twenties who live in Europe, nearly entirely in England.
Classic CJD is the most typical of the transmissible spongiform encephalopathies (TSEs) found in humans. These diseases are known as transmissible simply because individuals (and animals) can get them through blood transfusions, tissue transplantation, or eating infected food materials. Spongiform means that the brain of a individual or animal that has died from a TSE has microscopic holes in it at autopsy and a generally spongy texture when examined under a microscope. Encephalopathy is the medical term for a illness of the brain that causes changes in the function of the brain. CJD is named for the two German doctors who first described it, Hans Gerhard Creutzfeldt (1885-1964) and Alfons Maria Jakob (1884-1931).
CJD is related to several other rare prion diseases, including kuru, a disease that was epidemic in Papua New Guinea in the mid-twentieth century; and fatal familial insomnia (FFI), an inherited illness that has been identified in only twenty-eight families around the world. Other prion diseases infect animals; these include so-called mad cow disease and scrapie, a disease discovered in sheep and goats. Some think that mad cow illness is a form of scrapie and may have started in the early 1980s, when the remains of dead sheep had been used as a protein supplement in cattle feed.
The importance of mad cow illness is its relationship to variant CJD (occasionally known as new variant CJD), the other major human form of TSE. Variant CJD is thought to result from eating meat from a cow infected with mad cow illness just as mad cow disease resulted from feeding protein derived from infected sheep or cattle to cattle. This type of feeding procedure is no longer utilized.
Classic CJD and variant CJD are both very rare diseases. Classic CJD occurs in about one individual in every 1 million per year around the world. There are about 200 cases reported each year in the United States. Variant CJD (vCJD) was first reported in the United Kingdom in 1995. Of the about 200 known or suspected cases of vCJD, 164 were reported in the United Kingdom, twenty-one in France, four in Ireland, and 3 in the United States. Of the 3 instances reported in the United States, two of the patients had been born in the United Kingdom and lived there for some years; the third had grown up in Saudi Arabia. As of 2008, there have been no instances of vCJD that have originated in the United States. CJD is thought to be equally typical in both sexes and all races.
CJD is thought to be caused by a prion, an infectious protein particle that causes comparable normally folded protein molecules in the brain to refold themselves into abnormal shapes. The misfolded proteins accumulate in the brain tissue, interfere with the functioning of the brain cells, and eventually trigger the death of the brain tissue. Classic CJD is thought to have an incubation period as long as twenty to forty years. A individual can develop CJD in one of 3 methods:
The main difference between classic CJD and variant CJD is the age group affected. Most persons with classic CJD are middle-aged or older adults, whereas the average age of persons with variant CJD is twentyeight years. It's also thought that variant CJD might have a shorter incubation period than classic CJD, perhaps only eleven or twelve years in length. The symptoms of CJD include psychiatric as well as physical symptoms:
A definite diagnosis of CJD could be made only after the patient has died along with a sample of brain tissue is examined. Prior to death, nevertheless, doctors can make a tentative diagnosis by first of all ruling out certain other diseases that can cause similar symptoms. They can give the patient a spinal tap in order to rule out some other diseases. A CT scan can be performed to rule out stroke. The most important diagnostic tests are an electroencephalogram, or EEG, which measures brain waves; and a magnetic resonance imaging test, or MRI. Patients with CJD will have a specific kind of abnormal brain wave pattern that isn't found in any other disorder. MRI images often reveal patterns of damage to brain tissue that are characteristic of CJD.
There's no recognized treatment that will cure CJD. Researchers have tried many different medications, including steroids, antibiotics, antiviral drugs used to treat AIDS, and a number of other experimental drugs. Treatment consists of making the patient as comfortable as feasible. Morphine and similar drugs could be given to relieve pain. Some other anti-seizure drugs might be given to stop seizures. The patient can be turned frequently to prevent bedsores and be given fluids and nutrition intravenously.
CJD is a rapidly progressing illness with a fatal outcome. Most people develop dementia within six months of the onset of symptoms and turn out to be totally unable to take care of their fundamental physical functions shortly afterward. Most of those with classical CJD die within seven months; however, a couple of patients live as long as one to two years after diagnosis. Death is usually caused by infection, heart failure, or respiratory failure. Those with variant CJD might survive longer, perhaps a year or two.
There is no known way to stop genetic transmission of classic CJD, as patients generally are old enough to marry and start a family long before the symptoms of the illness appear. There is also no recognized method to stop the sporadic form of the illness. Some public health measures, however, have been put into location:
Researchers continue to look for a cure for CJD. One of the main problems is the nature of prions themselves; unlike bacteria or viruses, they're extremely difficult to kill and don't contain any genetic material (DNA or RNA). A better understanding of the nature of prions might lead eventually to an efficient treatment for CJD.
Our website is not responsible for the information contained by this article. Webworldarticles.com is a free articles resource thus practically any visitor can submit an article. However if you notice any copyrighted material, please contact us and we will remove the article(s) in discussion right away.
This article was sent to us by:
Alexis Kellgen at
01142011
1. Carbon Monoxide Poisoning: causes and fast treatment
All articles in this directory are property of their respective authors. Additionally, read our Privacy Policy
© 2010 WebWorldarticles.com - All Rights Reserved. Partners: Gunblade Saga