Creutzfeldt-Jakob

Creutzfeldt-Jakob disease (CJD) is a rare brain disorder, fatal, caused by infectious protein particles called prions. Prions exist normally in humans, but moved to its three-dimensional shape, become pathogenic. The healthy proteins present such a conformation that allows them to function normally, but the deformed protein causes disease by "forcing" the other next to change the way healthy for unhealthy way, pathogenic. Thus, a prion "sick" induce more prions "sick", transforming healthy proteins.

The most common signs and symptoms of CJD is rapidly progressive dementia with mental decline, and jerky involuntary muscle movements called myoclonus. About 90% of patients with CJD die within 1 year of diagnosis.

Eighty-five to 95% of cases of CJD (called sporadic CJD) may originate from sporadic mutations when the DNA (deoxyribonucleic acid) of the normal brain protein is modified by it and causes a pathogenic prion. In some cases, the mutation is inherited (pass from parents to children), appearing most often in communities of Jews born in the Czech Republic, Slovakia, Chile and Libya. Given that the effects derived from this transformation occurs slowly, hereditary CJD, as the previous one, sporadic causes no events before adulthood.

Prions "sick" can also pass from one animal to a person or one person to another. Fortunately, it is difficult to acquire this type of disease. One form of CJD called "variant" shows a slower progression of brain injury, initially manifested by symptoms and psychiatric tends to affect young people. This "variant" relates to the intake of beef patient so-called "Bovine Spongiform Encephalopathy" (BSE), or "mad cow disease", which is also caused by prions.

In the 90s of the twentieth century have described the first cases of this "variant" of CJD in the UK, caused by eating beef infected by the disease. Since then, changes in the creation of cattle and processing practices helped to limit the number of new cases.

It is extremely rare that CJD from spreading from one person to another. However, in very rare cases, the "variant" was transmitted through blood transfusion and sporadic cases of CJD have resulted from a medical procedure (contaminated equipment) of infected tissue (such as corneas that are used for transplantation) or hormone injections extracted from tissues of humans infected with the disease.

Prevention

Most cases of CJD in sporadic cases is caused by mutations in the DNA of the prion protein, which can not be avoided, but fortunately rare.

Cases of CJD caused by medical procedures or by ingestion of infected animal flesh, are even rarer. The health care professionals minimize risk when handling body fluids and tissues of patients with extreme caution and use special sterilization methods to disinfect the equipment. Similarly, the industry of cattle breeding and processing industry beef took new precautions that helped make the risk of infection through ingestion of beef, very small.

Blood banks have also established additional restrictions on blood donors.

Treatment

You can not cure CJD, but these are some of its manifestations. For example analgesics for relieving pain and anticonvulsants such as valproic acid, clonazepam and to muscle spasms. In the future, perhaps, we may have a vaccine or a drug specifically targeted to the disease mechanism.