Kuru is a neurodegenerative disease belonging to spongif.webporm encephalopathies. It is caused by infectious prions. It was spread among the tribes of Papua New Guinea, today, fortunately, it is practically no longer present. Fortunately - because so far no cure for prion diseases has been invented.

Kuru diseasewas discovered in the 1950s. It is also called cannibal disease because it took a heavy toll among the Fore people of the Eastern Mountains of Papua New Guinea. They cultivated ritual cannibalism.

Another word for kuru is laughing death - due to the characteristic symptoms of the disease, incl. violent, uncontrollable laughter.

The deceased's brain was gouged out, then boiled and eaten. Another way to "extend the life" of tribe members was to rub the brain of a deceased person into the skin of his relatives, especially women and children - the most privileged. In both cases, dangerous prions could enter the human body, scratches on the skin or damaged mucosa of the digestive system were enough. The kuru epidemic left 90 percent of the population of these primitive settlements extinct. It was not until the 1950s, when cannibalism was officially banned, that the incidence of the disease began to gradually decline. Kuru was discovered and described by Daniel Carleton Gajdusek in 1957, and 20 years later he was awarded the Nobel Prize for it. He showed that it is an infectious disease, transmitted by a factor associated with cannibalism.

What is kuru?

Kuru is a central nervous system disease caused by pathogenic prions and the first well-described human spongif.webporm encephalopathy. Prions cause destructive changes in the brain and loss of nerve cells. The disease is difficult to diagnose as it may take up to 40 years to develop, and the initial symptoms are non-specific.

What are prions?

These mutant proteins are normally found in the envelopes of nerve cells and white blood cells (lymphocytes). They should be broken down by cellular enzymes and proteases. However, a mutation may occur that leads to these proteins being converted into forms that are not broken down by proteases. Then they accumulate in the nervous tissue and cause its slow destruction, giving a characteristic image of spongy changes.

Prions are unconventional factorsinfectious, neither bacteria nor viruses. They are still unknown to scientists and have aroused their surprise and fascination for years. They do not contain nucleic acid, they do not metabolize, but they behave like a protein life form. In addition, they have the ability to duplicate themselves.

Infected nervous tissues of animals may be a source of prion infection.

Priony was discovered by the American biochemist Stanley B. Prusiner in the 1980s. In 1997, he was awarded the Nobel Prize for research in which he proved that these proteins are infectious.

It is also possible to become infected during medical procedures, such as corneal transplantation, implantation of dura mater or brain electrodes, as a result of using contaminated surgical instruments. It is true that prions of different species have a different structure, but there is a probability of breaking the interspecific barrier. For example, it is suspected that cows contracted BSE (mad cow disease) for the first time after consuming feed that was made from scrapie-infected sheep's brains. Similarly, cats - the feline variant of BSE, acquired by eating contaminated food.

Worth knowing

Diseases caused by prionsIn humans:

  • Kuru's disease
  • Creutzfeldt-Jakob disease
  • Fatal Familial Insomnia (FFI)
  • Gerstmann-Straussler-Scheinker syndrome (GSS)

In animals:

  • scrapie
  • mad cow disease (BSE)

Disease symptoms kuru

As mentioned, the disease takes a long time, ranging from several months to 40 years. Usually - several months. During this time, the patient develops:

  • incoherent movement (cerebellar ataxia)
  • strong trembling (kuru means trembling)
  • involuntary movements
  • urinary incontinence and faecal incontinence
  • rapid mood changes - uncontrolled bouts of crying or laughing
  • withdrawal

Kuru usually has three phases. The first, introductory, is like the flu. The patient complains of headaches and pain in the eyes, pain in the joints and limbs in general, fever, sore throat, but also weight loss. Additionally, there are mobility problems, limb tremors, loss of balance and characteristic involuntary and rhythmic eye movements.

In the second phase, the patient can no longer move by himself or even sit without support. The above-mentioned symptoms intensify and the person slowly becomes completely dependent.

The final phase of the kuru is waiting for death. The sick person is completely immobilized, does not hold urine or feces, food with difficulty (and finally at all)moves into the stomach. He forcibly laughs or cries with throws. Dementia (dementia) is almost never seen in kuru, so you are fully aware. Death occurs as a result of total organism destruction, starvation or aspiration pneumonia.

Kuru treatment

Unfortunately, no effective cure for prion diseases has been invented so far. However, intensive research in this direction is ongoing. Various methods are tested, including pharmacotherapy, nanotechnology, vaccine or protein therapy.

Prevention of prion diseases

Prion diseases do not spread by droplets, as is commonly the case with viral or bacterial diseases. So we have a good chance of preventing infection. You can take care of it in a number of ways:

  • compliance with basic hygiene rules
  • avoiding eating meat of unknown origin (e.g. from markets, without veterinary certificates)
  • not using meat or bone-and-meat meals in animal feed
  • because there is a risk of infection of the patient during surgery, the method of prophylaxis of prion diseases is to quarantine the equipment used for operations within the brain, tonsils or appendix until the result is a biopsy of these organs for prion carrier
  • examination of medical preparations for possible infection. For example, in 2000, the British Ministry of He alth ordered the removal of the oral poliomyelitis vaccine from circulation, which was produced on the basis of serum from calves suspected of being infected.

Category:

Neurology