Creutzfeldt-Jakob disease (spongiform degeneration of the brain)

Proven content

Creutzfeldt and Jakob disease (belongs to the so-called spongiform encephalopathies, characterized by the destruction of the gray matter of the cerebral cortex, consisting in the formation of vacuoles in its vicinity, ie vesicular particles filled with protein. The symptoms are accompanied by symptoms in the form of disturbances in sensation and coordination of movements.

Creutzfeldt and Jakob disease

In the course of this disease, the brain begins to resemble a sponge over time. Increasing losses of brain tissue cause tremors in the body, then disturbances in balance and coordination of movements, psychomotor impairment and, finally, inevitable death. Spongiform encephalopathies are transmitted to susceptible animals by tissue implantation from infected individuals. Creutzfeldt-Jakob disease occurs in four different forms, which differ mainly in etiology: familial form; sporadic form; iatrogenic form; form of the so-called variant of Creutzfeldt-Jakob disease. The common feature of these varieties is the puckering of the brain and the atrophy of neurons. Creutzfeldt-Jakob disease is difficult to diagnose as it has a varied clinical course. However, thanks to the development of medicine in the field of neurology, diagnostic possibilities are expanded.

Creutzfeldt and Jakob disease – causes

The infectious factor contributing to the development of this disease is a protein smaller than the virus – prion. Prion protein occurs in humans and animals. It is a component of the envelope of nerve cells and leukocytes (white blood cells). Prion is a common and pathogenic (scrapie) protein. Both the first and the second form have the same chemical composition, they differ only in the spatial arrangement of the amino acids from which they are made.

Pathogenic variety – scrapie – has a remarkable property. When it comes into contact with normal proteins, it transforms them into similar ones, which triggers a series of events: the changed proteins, like dominoes, transform into an avalanche of subsequent and subsequent ones, etc. When the amount of pathogenic protein is large enough, brain cells disappear.

Formerly, a certain type of prion resided in a given animal organism of one species, and the routes of their spread were very limited. Currently, as a result of food production on an industrial scale and a large number of secondary infections of animals (by feeding them with meat and bone meal (from different species of animals), prion varieties have been developed that are very resistant and have a predisposition to transmit the infection to various species of mammals.

For a disease to be transmitted to humans, only a small fragment (a few amino acids) of an animal prion has to be identical to that of a human. It is assumed that in humans, prion disease may develop:

1. on the effect of the spontaneous change of prion proteins from non-pathogenic to pathogenic form; it is genetically determined,
2. as an infection with BSE prion from diseased animals by eating contaminated products; most often it is beef; the muscle itself does not show the presence of the infectious agent (prion); rather, the source of the infection is meat contaminated with nervous tissue during the slaughter of the cows; The main source, however, is mechanically recovered meat that has been contaminated with brain or spinal cord tissues, e.g. in dumplings, sausages, dumplings, as well as in milk and gelatin,
3. as a result of an infection related to medical activities (corneal transplant, dura mater of the brain, after the use of growth hormone obtained from human pituitary glands).

Creutzfeldt-Jakob disease occurs all over the world.

Symptoms of Creutzfeldt and Jakob disease

Initially, people with Creutzfeldt’s and Jakob’s disease have impaired sensation and coordination, or experience confusion and inadequate behavior with worsening symptoms. Over time, he becomes fully dull and coma.

It is worth emphasizing that XNUMX% elimination of the disease in animals eliminates the source of the infectious agent, which is tantamount to the elimination of the risk to human health.

In humans, the group of spongiform encephalopathies, in addition to Creutzfeldt and Jakob disease, includes kuru disease, defined from one of its symptoms as “laughing death”. This disease, formerly only found in Papua New Guinea, was related to the cultivation of ritual cannibalism. Well, as a sign of respect for the dead, their brains were eaten. After variously long periods, even 40 years, a slow process of dying began. It led to imbalances, forced crying and laughter, tremors, and finally emaciation and death.

Other ailments in this group include Gerstmann-Strussler syndromecharacterized by a loss of coordination, and fatal family insomniawhere, after a period of difficulty falling asleep, there is progressive dementia.

Among the animals there are those known for two hundred years scrapie in sheep and goats and BSE, commonly referred to as mad cow disease.

Creutzfeldt and Jakob disease – types and their characteristics

1. Iatrogenic Creutzfeldt-Jakob form – this variant of the disease is currently very rarely diagnosed. The reason for its formation is the inadvertent transmission of the disease to humans during, for example, corneal transplantation. The most common symptoms of this form are cerebellar symptoms and dementia (rarely or slightly severe). So far, this disease has not been reported in Poland.

2. Creutzfeldt-Jakob family character – accounts for about 15 percent all cases of spongiform degeneration of the brain. This form of the disease is caused by mutations in the PrP gene. The most common symptoms include: nystagmus, gait disturbance and intention tremor.

3. Variant of Creutzfeldt-Jakob disease – the cause is the passing of bovine spongiform encephalopathy (BSE) to humans as a result of eating, for example, raw beef. This form mainly affects young people who experience delusions and hallucinations. They are also often agitated and aggressive.

4. Sporadic form of Creutzfeldt-Jakob disease – it is the most common form of Creutzfeldt-Jakob disease. It is characterized by symptoms in the form of dementia, which soon become accompanied by disturbances in balance and consciousness. Some patients complain of feeling disturbed on one side of the body. Occasionally seizures may occur and symptom of Parinaudthat is, the inability to look up.

Creutzfeldt and Jakob disease – diagnosis

The following tests are used in the diagnosis of spongiform degeneration of the brain:

1. magnetic resonance imaging – helpful in visualizing characteristic symptoms;
2. electroencephalography – a study necessary to make a proper diagnosis;
3. examination of the cerebrospinal fluid – in most patients it allows for an accurate diagnosis, it is a sensitive examination;
4. Computed tomography – its performance is necessary for diagnosis, although it is usually without deviations.

In the diagnosis of Creutzfeldt and Jakob disease, it is also important to differentiate the disease with other ailments, such as:

1. central nervous system infections;
2. symptoms that indicate a brain tumor;
3. Wernicki-Korsakoff syndrome;
4. vascular incidents;
5. poisoning with tricyclic antidepressants;
6. vascular ailments;
7. Parkinson’s disease;
8. Alzheimer’s disease.

Treatment of the disease

Spongiform degeneration of the brain is an incurable disease and an irreversible process. There are no preparations that would be able to eliminate prions from the body and stop the development of the disease.

Prognosis

The disease develops rapidly in most patients and only 5% of patients live with this condition for more than 2 years.

Also read: 8 diseases for which there is still no cure

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