What is cjd disease

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Early signs and symptoms typically include:. As the disease progresses, mental symptoms worsen. Most people eventually fall into a coma. Heart failure, lung respiratory failure, pneumonia or other infections are generally the cause of death, which usually occurs within a year. In people with the rarer vCJD , psychiatric symptoms may be more apparent in the beginning.

In many cases, dementia — the loss of the ability to think, reason and remember — develops later in the illness. Prions are proteins that occur naturally in the brains of animals and people.

Normally, the proteins are harmless, but when they're misshapen, they can cause devastating illnesses such as BSE disease in cattle and Creutzfeldt-Jakob disease in humans. Creutzfeldt-Jakob disease and its variants belong to a broad group of human and animal diseases known as transmissible spongiform encephalopathies TSEs.

The name derives from the spongy holes, visible under a microscope, that develop in affected brain tissue. The cause of Creutzfeldt-Jakob disease and other TSEs appears to be abnormal versions of a kind of protein called a prion.

Normally these proteins are produced in our bodies and are harmless. But when they're misshapen, they become infectious and can harm normal biological processes. The risk of CJD is low. The disease can't be spread through coughing or sneezing, touching, or sexual contact. CJD can develop in three ways:.

By contamination. A small number of people have developed CJD after being exposed to infected human tissue during a medical procedure, such as a cornea or skin transplant. Also, because standard cleaning methods don't destroy abnormal prions, a few people have developed CJD after undergoing brain surgery with contaminated instruments. A small number of people have also developed the disease from eating contaminated beef. Variant CJD is linked primarily to eating beef infected with mad cow disease bovine spongiform encephalopathy, or BSE.

In an autosomal dominant disorder, the mutated gene is a dominant gene located on one of the nonsex chromosomes autosomes. You need only one mutated gene to be affected by this type of disorder. Most cases of Creutzfeldt-Jakob disease occur for unknown reasons, and no risk factors can be identified.

However, a few factors seem to be associated with different kinds of CJD :. Exposure to contaminated tissue. People who've received infected manufactured human growth hormone, or who've had transplants of the infected tissues that cover the brain dura mater , may be at risk of iatrogenic CJD. The risk of getting vCJD from eating contaminated beef is very low.

In general, if countries are effectively implementing public health measures, the risk is virtually nonexistent. Chronic wasting disease CWD is a prion disease that affects deer, elk, reindeer and moose. There is no cure for the disease. It progresses quickly and every case is fatal. A person usually dies within 1 year after symptoms appear. There are different types of CJD. It can develop sporadically, without any identifiable pattern. It can be inherited and may be transmitted.

A person can acquire vCJD after eating the meat of a cow that has bovine spongiform encephalopathy, commonly called mad cow disease. However, developing vCJD in this way is rare. CJD is caused by a prion, a misfolded protein that can transmit its malformation to healthy variants of the same protein. Examples in other animals include scrapie, in sheep and goats, and bovine spongiform encephalopathy, in cattle.

Laboratory tests indicate that transmission can occur between animals, but this does not necessarily mean that CJD is transmissible between humans.

However, transmission may be possible, for example, through transfusions or transplants of infected blood or tissues. CJD has a long incubation period. Symptoms may take decades to appear. Symptoms emerge as the disease destroys brain cells. The symptomatic period lasts 4—5 months on average, and the disease is usually fatal within 1 year.

The hallmark symptoms of CJD are a rapid progression of dementia and myoclonus — spasmodic, involuntary movement of muscle groups. As the disease progresses, problems with coordination and muscle control worsen.

Over time, the person will lose their vision and their ability to move and speak. Eventually, they will enter a coma. Autopsies of brain tissue have revealed that CJD leads to certain changes that do not occur with other causes of dementia. A prion is a type of protein with an abnormal structure, and it passes this abnormality on to other proteins. This damages brain tissue and causes the characteristic symptoms of CJD. Prions have a long incubation period and are difficult to target.

CJD may be inherited or acquired. In some cases, it develops sporadically, without an identifiable pattern.