The Creutzfeldt-Jakob disease was described by two German scientists, whose surnames were called the disease, as early as the beginning of the 20th century. Although since that time has passed more than a century, a cure for this disease has never been found. Scientists were able to identify the source of the disease - a hostile prion, but could not learn how to deal with it.
Creutzfeldt-Jakob disease - what is it?
Creutzfeldt-Jakob prion disease develops as a consequence of a gene mutation caused by a hostile human organism, the protein prion. It is believed that the source of this protein is cattle, but new research hypothesizes that the disease occurs spontaneously and without an external cause. Researchers believe that KBH disease (mad cow disease) is progressing, and cases of new types of disease are noted. In the 1990s, cases of the subspecies of this disease were recorded, which was called mad cow disease.
Previously, the disease affected people older than 65 years, but now there are cases of damage to younger people. Prion virus affects the brain, as a result of which cognitive processes and character begin to suffer in man. The development of the lesion leads to an increase in symptoms, speech disorders, seizures and paresis of the limbs. The peak of the disease is coma and death. After infection, a person lives no more than two years. The average life expectancy for prion damage is 8 months.
Creutzfeldt-Jakob disease - causative agent
The virus of cow rabies is caused by a mutant prion protein. Prions exist in the human body, but have a different structure. The hostile protein that comes from outside does not die in the human body, but comes from the blood stream to the brain. There he begins to interact with human prions, which leads to a change in their structure. The infectious prion creates plaques on neurons, after which the neuron perishes.
Creutzfeldt-Jakob disease - the way of infection
Scientists distinguish such ways of infection of Creutzfeldt-Jakob disease:
- transplantation of infected bovine tissues infected with infectious prions;
- transfusion of contaminated blood;
- transmission of infection through non-sterile contaminated surgical instruments;
- eating badly processed infected meat of cows, sheep, goats.
Disease of Creutzfeldt-Jakob - causes
The etiological cause of Creutzfeldt-Jakob disease is not exactly established. Although the version of the ingress of an alien prion from the outside (often from an animal) is generally accepted, there are other theories. One of the theories is the assumption that the human prion, changed for some reason, begins to change neighboring prions, which leads to the defeat of different structures of the brain.
Mutagenic prions with Creutzfeldt-Jakob disease start to work against the host organism. They prevent the cell from performing its function, blocking the processes occurring in it. As a result of the prions, the cell dies. Around dead cells, inflammatory processes develop, in which highly active enzymes participate. These substances interfere with the work of healthy cells, thereby increasing the damage to the structures of the brain.
Creutzfeldt-Jakob disease - symptoms
Cow rabies in people whose symptoms depend on the location of the lesion at the initial stage manifests itself by such signs:
- distraction, irritability;
- headaches, dizziness ;
- problems with sleep;
- deterioration of vision;
- apathy, lack of initiative;
- memory impairment ;
- decreased libido;
- episodic euphoria or fear;
- delirium or hallucinations;
- problems with speech;
- paresis of limbs, unsteadiness while moving.
In the second stage, mad cow disease, the symptoms of which increase, is manifested by such signs:
- epileptic seizures ;
- paralysis;
- loss of muscle control, tremor;
- dementia;
- severe speech impairment;
- psychiatric disorders.
The terminal stage is characterized by the following symptoms:
- state of prostration;
- loss of control of pelvic organs;
- impaired swallowing;
- complete paralysis;
- coma.
Kreutzfeldt-Jakob disease - diagnosis
To clarify the diagnosis requires a complete clinical picture, confirmed by instrumental data. In this case, the doctor, when collecting an anamnesis, finds out in which zone the patient lives, whether there were contacts with cattle. It is important to find out all the symptoms that the patient has addressed. Particular attention is paid to problems with vision, mental and motor abilities.
Instrumental data include the results of such surveys:
- EEG (electroencephalogram) - it will be reduced activity with periodic or pseudoperiodic acute waves.
- PET of the brain.
- Creutzfeldt-Jakob disease, MRI in which the T2-mode is performed, is detected in the examination by the so-called "honeycomb symptom" - areas with elevated signals.
- Lumbar puncture for the study of cerebrospinal fluid.
- Stereotoxic biopsy of the brain, which allows to detect an infectious protein.
Creutzfeldt-Jakob disease - treatment
Since there is still no exact cause of the disease, no medicine has been found against it. Immunization of cows and humans did not bring the desired results. Do not act on hostile prions and antiviral drugs. The researchers were able to understand how to prolong the life of infected cells, but this is only a small step in the search for an effective drug. At the moment, mad cow disease in humans is treated only symptomatically. The patient is prescribed anticonvulsant and antiepileptic drugs.