Disease of Creutzfeldt-Jakob, why it occurs and how it manifests itself

Creutzfeldt-Jakob disease, also called corticostrioscopic degeneration syndrome or spastic pseudosclerosis, refers to the fatal and extremely dangerous diseases associated with the progression of the dystrophic changes in the CNS structures in a short time. In this process, the cerebral cortex, the basal ganglia, and the spinal cord are affected. This pathology was first discovered by two German scientists, and was named by their names.

To all the fault of the protein

Creutzfeldt-Jakob disease develops as a result of metabolic disorders with a gradual accumulation of prion protein body in certain places of the central nervous system.

A similar kind of protein exists in the body of perfectly healthy people, but in patients with pseudospas- tic sclerosis, it undergoes changes that determine violations.

At the heart of pathogenesis lies the ingress of pathological protein into the body, and its ability to transform the normal form of protein formations into anomalous. This transformation takes place at a high speed and in the form of a geometric progression.

Diseases are fairly rare. A mutation that promotes its development is noted in one or two people per one million of the population.

Causes of infection

A mutagenic protein in the body can manifest itself in the following cases:

1. Sometimes a person could get sick during transplant operations of an infected organ( cornea, structures of the spinal cord or hard shell of the brain) from one person to another, or as a result of blood transfusion.
2. In a number of cases, the reason is insufficient processing of the instrument during neurosurgical interventions.
3. The pathological form of the protein can penetrate into the human body from the outside, along with the meat of sick animals by cow rabies( spongiform encephalitis).
4. Sometimes there is a hereditary transfer from parents to children. Transmitted by autosomal dominant type, according to statistics, this reason is revealed in 15% of all cases.
5. There have been cases of the development of the disease when used for the treatment of medicines made from the collection of material from animal corpses.

Occasionally reported cases of sporadic disease, without apparent cause. In this case, the transformation of the protein is spontaneous.

How does the disease go?

If you get into the body for a while, the disease does not manifest itself, because the body is able to compensate up to a certain point. But gradually, with the accumulation in neurons of formations that change their functions, triggering the process of apoptosis, their death occurs and the clinical picture evolves in severe neurological symptoms.

The death of such a patient occurs within 3-4 years, and sometimes after the appearance of the first symptoms is only a few weeks. Unfortunately, at the moment there is no such method or means that would be able to cure the syndrome of corticostriospinal degeneration.

The first manifestations of the pathological condition occur in 40-50 years, according to unknown reasons until now science.

In 40% of patients subacute course is noted, with progressive cognitive dysfunction. In 40% there are violations of the function of the cerebellum. The Creutzfeldt-Jakob disease in 20% of cases has a mixed pattern of disorders.

Symptomatology consists in the disorder of behavioral reactions, changes in the work of higher cortical structures, cerebellar disorders, the emergence of signs of pyramidal and extrapyramidal disorders.

The most common manifestations are as follows:

• violation or slowing down of thinking, reduced ability to concentrate;
• emotional lability;
• various, including visual hallucinations;
• is sometimes brought to the fore by visual disturbances or even the development of complete blindness;
• myoclonia, tremor, parkinsonism;
• in almost all cases there are epileptic seizures.

In the sporadic variant of the disease( 85-95% of cases), the development of dementia, up to total marasmic abnormalities, is characteristic.

Diagnosis Awareness Methods

The physician can prescribe the Creutzfeldt-Jakob disease in case of rapid development of the clinical picture of dementia in an elderly patient along with the onset of ataxia and convulsive syndrome, the rapid progression of the disease

. In an encephalogram study, such a patient may have typical( for two or three-phase acutewave).When taking an analysis of the cerebrospinal fluid, no pathological changes are detected.

How to alleviate the condition of

As already noted, at the moment the medicine does not have tools to influence the cause of the development of this disease.

Treatment is symptomatic, it is aimed at some relief of the patient's condition. The attempt to use standard antiviral therapy in therapeutic practice did not bring a proper result.

Prevention

To prevent the onset of this disease, employees who work in laboratories and examine tissues and body fluids in patients with a presumed diagnosis of Creutzfeldt-Jakob disease should work in special gloves to eliminate direct contact.

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