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Creutzfeldt-Jakob disease (CJD), or spongif.webporm degeneration of the brain, is a disease whose incidence is not high - it affects 1 person in a million annually. Nevertheless, it is scary because it leads to progressive degradation of the brain. Mad cow disease is thought to cause a variant of Creutzfeldt-Jakob disease (vCJD) in humans. What are the causes and symptoms of Creutzfeldt-Jakob disease? Is it possible to cure her?

Creutzfeldt-Jakob disease , otherwisespongif.webporm degeneration of the brainorspongif.webporm encephalopathy , is fatal a neurodegenerative disease of the brain. Its essence is the degeneration of the gray matter of the cerebral cortex, i.e. clusters of nerve cells. Within it, vacuoles are formed, i.e. vesicular particles filled with the infectious protein (so-called prions). They lead to the atrophy of the nervous tissue, and then to the brain's spongiosis - the brain literally starts to look like a sponge. Creutzfeldt-Jakob disease is the most common prion disease .¹

Creutzfeldt-Jakob disease (CJD) - what are prions?

The causes of brain cell degradation areprions(short for "infectious protein") pathogenic protein molecules that cause nervous system disorders, both in animals and in animals people.

Prions are common in humans and animals - they are a component of the cells of the immune system and nerve cells. There are two forms of prions: normal (cPrP) and pathogenic (scPrP). Both have identical chemical compositions, the only difference being the spatial arrangement of the structural amino acids.

The normal form is safe for humans, while the pathogenic form transforms it into such infectious forms as it comes into contact with a he althy protein. Then, the structurally changed amino acids on a domino basis modify the composition of other he althy proteins. This way, infectious prions can replicate and infect other cells in the body.

Infectious prions also have another property: they are an integral part of the cell membrane, making them "invisible" to cells of the immune system, which explains why the immune system of an infected organism fails to defend itself: it simply cannot distinguish between normal proteinsprions from their pathogenic forms.

Prion diseases include: ²

in humans:

  • Creutzfeldt-Jakob disease (CJD)
  • kuru
  • Gerstmann-Sträussler-Scheinker Disease (GSS)
  • fatal familial insomnia (FFI)

in animals:

  • scrapie (unused Polish name: scrapie) in sheep, goats and mouflons
  • transmissiblemink encephalopathy (TME)
  • Bovine Spongif.webporm Encephalopathy,BSE )
  • chronic wasting disease

Variant Creutzfeldt-Jakob Disease (vCJD) is a new disease caused by prion diseases caused by the passage of the bovine spongif.webporm encephalopathy (BSE) factor .² You can contract it by eating meat from BSE-sick cattle.

Creutzfeldt-Jakob Disease (CJD) - Causes

Creutzfeldt-Jakob disease is divided into 4 basic forms:

  • sporadic
  • family
  • iatrogenic
  • CJD variant

The most common cause of the disease are sporadic, randomly occurring gene mutations, as a result of which, instead of properly functioning prions, pathogenic ones are produced in the body. This is called the sporadic, classic form of the disease (sCJD), which is the most common form of CJD (it accounts for about 90% of all CJD cases).

Creutzfeldt-Jakob disease may also be the result of spontaneous genetically determined changes related to the mutation of the PRNP gene encoding the PrP prion protein (fCJD is about 5% of cases).

The other forms of the disease are acquired, most likely through the oral route (a new variant of this disease - vCJD) or through the bloodstream - the iatrogenic form - jCJD - caused by medical procedures.

Creutzfeldt-Jakob Disease (CJD) - How can I get infected?

To transmit a disease to humans, a fragment of a few infectious prions identical to humans is enough. This can occur, for example, through the ingestion, through the consumption of beef contaminated with the nervous tissue of animals infected with the pathogenic prion. Meat is usually infected during the slaughter of cows, which is why it is most often beef.

The source of infection can also be mechanically separated beef, contaminated with contaminated brain or spinal cord tissues, which can then be found, for example, in sausages or dumplings. It is worth knowing that it is much more dangerous thanof pure beef there are various types of offal, e.g. hamburgers, frankfurters, sausages, brains, pates. Beef gelatin can also be dangerous.

You can drink milk from cows without fear. Studies so far show that prions "don't like" milk. Dairy products such as kefirs, cheese, etc. are similarly safe.

You can also eat pork without fear. Pigs do not get diseases caused by prions. Just like poultry.

The body fluids of an infected person can also be the route of infection, e.g. during an injury, as well as as a result of infection related to medical activities (transplants, transfusions, treatment with growth hormone obtained from the human pituitary gland).

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Creutzfeldt-Jakob Disease (CJD) - symptoms

U approx. 30 percent patients experience heraldic symptoms. Unfortunately, they are not specific as they include mood changes, lack of appetite and related weight loss, anxiety, sleep disturbances, and cognitive disturbances.

The actual symptoms of the disease depend on the size of the brain defect.

initially appear
  • disorders of sensation, balance and motor coordination
  • body trembling
  • paresis of lower limbs first, then upper limbs, leading to psychomotor impairment

However, according to the World He alth Organization, the most important diagnostic criterion necessary for the diagnosis of Creutzfeldt-Jakob disease is rapidly progressing dementia. It occurs within weeks or months of first symptoms and inevitably leads to coma and then death.

It should be noted that in the course of Creutzfeldt-Jakob disease, psychopathological symptoms may occur, which without thorough diagnosis can be easily confused with psychiatric disease entities. This is the result of research by American scientists whose research results were published in "The Journal of Neuropsychiatry & Clinical Neurosciences". In the analysis of 126 cases of sCJD, the presence of psychiatric symptoms was found in the form of:

  • depressed
  • anxiety
  • psychosis
  • behavioral disorders
  • sleep disorders

u 80 percent patients in the first 100 days of the disease, and in 26%. among them they were the initial symptoms.

Creutzfeldt-Jakob disease(CJD) - diagnostics

To confirm the diagnosis, an electroencephalographic (EEG) recording is required. Imaging tests are also necessary, e.g. computed tomography, which reveals features of brain atrophy.

Creutzfeldt-Jakob Disease (CJD) - treatment

Draining the brain is an irreversible process. There is also no medicine that eliminates pathogenic prions from the body, and thus stops the progression of the disease.

Creutzfeldt-Jakob disease (CJD) - prognosis

In most cases, spongif.webporm degeneration of the brain develops suddenly and quickly - only 5%. survives for more than 2 years.

Source:

1. Sikorska B., Creutzfeldt-Jakob disease and its variants, "News Neurologiczne" 2011, 11 (1), pp. 29-37

2. James W. Ironside, Creutzfeldt-Jakob Disease Variant: The Current State of Knowledge, "Neurological News" 2011, 11 (1), pp. 44-48

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Neurology