A new, more dangerous form of mad cow disease has been discovered in the US

Recently the head of the World OrganizationHealth (WHO) announced the threat of a new pandemic, even more deadly than COVID-19. At a forum in Geneva, experts urged scientists to pay close attention to a number of diseases with pandemic potential. These include Zika, Ebola and SARS, but new, previously unknown diseases are on the rise. So, a mutation of one of the variants of bird flu has already led to the death of an infected man in China, and in the USA a new type of Creutzfeldt-Jakob disease, better known as "mad cow disease", has been discovered. The causative agent of the disease are prions - proteins that are abnormal in their structure, which literally turn the brain of infected animals and people into a sponge. Prions are considered the most dangerous infectious agents in the world, as they are extremely contagious, and there is no cure. We tell what is known about the atypical case of "mad cow disease" and why it causes concern to scientists.

Bovine spongiform encephalopathy(BSE) is extremely rare in the US. However, in May, the country's health authorities identified an atypical case in one cow at a slaughterhouse in South Carolina.

Spongiform transmissible encephalopathies – group of neurodegenerative diseases cthe formation of spongiform encephalopathy, which is characterized by damage to the central nervous system, muscular, lymphatic and other systems with a 100% fatal outcome.

Content

• 1 Prions are unusual proteins
• 2 Spongiform encephalopathies and COVID-19
• 3 Creutzfeldt-Jakob disease
• 4 A new variant of Creutzfeldt-Jakob disease

Prions are unusual proteins

Among the many diseases known to science,transmissible spongiform encephalopathies - diseases caused by prions, occupy a special place. These abnormal proteins, firstly, do not contain genetic material, and secondly, they have 100% lethality. Interestingly, before the discovery of prions by Stanley Prusiner in 1982, many neurodegenerative diseases were considered viral.

Essentially, prions are tiny proteincompounds that fold in a special way and damage healthy brain cells. As a result of their activities, dementia rapidly progresses in infected people, and death is painful. At the same time, it is impossible to determine the moment of infection due to the long incubation period, and after the onset of symptoms, there is no more salvation.

Prion diseases turn the brain into spongy tissue and are the most terrible in the world

The prion protein is resistant to radiation, chemicals and high temperatures, and its function is still unknown.

Researchers first encountered prions in1950 while studying a strange disease that afflicted women and children in the tribes of Papua New Guinea. After analyzing the brain samples of the victims, scientists found that brain tissue looked like a sponge - there were holes in place of the cells.

This condition was indicative of a decreasethe number of brain cells, which is characteristic of encephalopathy (the same pattern was observed in people and animals who died from Creutzfeldt-Jakob disease (CJD / CJD).

The cause of the disease was a special ritualprocess - when someone from the tribe died, his brain was removed and eaten. And since the brain was considered a healthy delicacy, it was most often given to women and children. The symptoms of the disease now known as kuru included tremors, incoordination, and dementia. Most of those infected died within 24 months, gradually ceasing to speak and respond to others.

An epidemic infectious disease called kuru was first identified in the 1950s in the Fore tribe of Papua New Guinea.

Fortunately, a total ban on ritual cannibalism has virtually eradicated this mysterious disease.

Spongiform encephalopathies and COVID-19

Although prion diseasesrare, they are so dangerous that they led to a three-month moratorium on further prion research in France after two INRAE ​​employees contracted mad cow disease. How exactly this happened is currently unknown. However, as well as the exact causes of the development of neurodegenerative diseases.

It is believed that infection with prion agents is more oftenmost occurs when contaminated meat is eaten or when the infection enters directly into the brain, skin and muscle tissue. As for Creutzfeldt-Jakob disease (CJD), all its forms, as a rule, are transmitted through close contact with an infected medulla.

Prions are also pathogens ina wide range of animals, including chimpanzees and deer. Read more about the death of deer in the US, Canada and Europe from Chronic Wasting Disease in this article, do not miss it!

Scientists from around the world are increasingly reporting an increase in cases of prion diseases.

Scientists are concerned, however, not onlyreported "classic" cases. The fact is that prions were talked about during the COVID-19 pandemic: the results of the studies showed that Prion diseases are one of the complications of coronavirus infection. Some experts even believe that covid threatens humanity with a prion pandemic.

The strange connection of mad cow disease (mostcommon prion disease in the world) and coronavirus is explained by the fact that when the spike S-protein enters the cells, it leads to a cascade of systemic inflammatory mediators and provokes the body to produce proteins with an abnormal structure.

So, in 2021, researchers from the United States reported aboutthe first case of the simultaneous course of Creutzfeldt-Jakob disease and COVID-19, and doctors from St. Petersburg reported an increase in cases of prion diseases against the background of the pandemic. Fortunately, there are still few such patients in the world, but in the future the situation may change.

Changes in the brain during CJD

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Creutzfeldt-Jakob disease

Since mad cow disease is the mostcommon prion disease on Earth, its course and pathogenesis are well studied. So, animals and people infected with CJD refuse food, lose control over the body and cannot navigate in space (which, however, is not surprising, because prions turn brain tissue into a sponge).

CJD is most commonly diagnosed in cattle.livestock on farms, and the first case of mad cow disease was reported in the UK in the mid-1990s. Then thousands of cows suffered from an outbreak of prion disease. Note that sick animals have difficulty walking and may behave aggressively. Death occurs within two weeks or six months, while clinical signs of infection may take three to six years to appear.

See also: Deadly diseases that are considered worse than cancer

In the past, outbreaks of CJD in the US, Canada, Israel, Europe, and Japan have resulted in food supply disruptions.

Today there is two main types of CJD - classic and atypical, but, according to the dataUSDA, neither form is contagious. Animals become ill with the classic form of the disease by feeding on feeds containing meat or bone meal of cows with CJD. For this reason, the use of certain bovine tissues in feed for all animals is prohibited.

As of May 2022, worldwide there were 233 cases of mad cow disease in humans. Scientists believe that the infection occurred due to the consumption of food prepared from infected animals.

Fortunately, “you can’t get CJD from eating milk or dairy products, even if they come from a sick cow,” experts point out.

Mad cow disease poses a serious threat to global public health

This is interesting: The most rare and unusual diseases in the world

A new variant of Creutzfeldt-Jakob disease

On May 5, the United States Department of Agriculture (USDA) announced the discovery an extremely rare form of L-type CJD from a 5-year-old cow at a slaughterhouse in South Carolina. This is the first reported case in the country since 2018. According to experts, a variant form of mad cow disease (vCJD) different from classical (CJD) and potentially dangerous for people.

An atypical variant of vCJD occurs in a largeolder cattle. According to the USDA, the atypical form occurs rarely and spontaneously and is not associated with feed or ingestion of infected foods. Most likely, genetic and environmental factors contribute to the emergence of vCJD, but scientists do not have an exact explanation.

The registered case is a single one, andprecautions have prevented intrusions into food service and animal feed supply systems, APHIS and South Carolina and Tennessee veterinary officials said.

BSE was found in a five-year-old cow in South Carolina, according to a USDA statement.

Despite assurances from the FDA that there is no threatthe spread of atypical CJD, the South Korean authorities have requested epidemiological information from the US government about this outbreak and are considering taking further action regarding regulations and the results of the epidemiological investigation. According to Business Korea, it has raised the spot check rate for U.S. beef from 3 percent to 10 percent effective May 22.

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Earlier in May, the first in more than 10a case of bovine spongiform encephalopathy on a farm in the Netherlands. The exact type of spongiform encephalopathy identified is currently unknown. In February 2023, an outbreak of mad cow disease was confirmed in Brazil, which led to a halt in beef exports to China.

Over the past two decades, only seven cases of CJD have been detected in the United States.

It seems that CJD is actively walking around the planet.At the same time, own forms of this most common prion disease are found in sheep, goats, deer and other animals. But among domestic animals, only cats can get sick with their own kind of this spongiform encephalopathy. Well, let's hope that neither the coronavirus nor new varieties of CJD will become a threat to public health in the future.