Creutzfeldt-Jakob Disease

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Video: Creutzfeldt-Jakob Disease

Video: Creutzfeldt-Jakob Disease
Video: Creutzfeldt -Jakob Disease (CJD) 2024, March
Creutzfeldt-Jakob Disease
Creutzfeldt-Jakob Disease
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What is Kreuzeld-Jacob disease

Kreuzeld-Jakob disease, also called subacute spongiform encephalopathy, is a neurological disease of a degenerative nature that affects the brain. It belongs to the group of prion diseases and is associated with the deposition of pathogenic prion protein in the brain.

The disease is sporadic, while the hereditary form is observed in only about 10% of cases. Kreuzeld-Jakob disease gained worldwide fame from its new variant, which appeared at the end of the last century and was transmitted from sick cattle to humans - the so-called. mad cow. It is incorrect to assume that Kreuzeld-Jakob disease and crazy cow are synonymous.

Causes of Kreuzeld-Jacob disease

The disease is caused by a protein without a nucleic acid called prim. The methods are defective normal proteins in the brain, which, once they enter or arise in a healthy person, begin to convert specific normal proteins into already pathogenic ones. The accumulation of receptions causes the death of brain cells and a clear manifestation of the disease. For this reason, once in a healthy person, the primate begins its reproduction.

Mad cow
Mad cow

The disease process affects the entire brain and this leads to the death of the person a few months after the onset of symptoms.

In most cases, the disease occurs accidentally / sporadically /, but it is possible to be inherited. The reception can be transferred and a person can become infected by hard brain transplantation, corneal transplantation or improperly sterilized electrode implants.

In the sporadic and hereditary forms, the disease occurs at a later age, sometimes even in the seventh decade of life. In very rare cases, the disease occurs before the age of 30. In the new form of the disease - the so-called. "Crazy cow" can make people sick at any age.

Classification of Kreuzeld-Jacob disease

The classification of the disease is based on the cause of its occurrence. 4 forms are known:

Sporadic form - occurs among people at random. It accounts for approximately 80% of all cases of the disease.

Family form - there is a proven heredity. It accounts for about 15% of cases.

Iatrogenic form - occurs after implant placement from a sick to a healthy person. It accounts for 5% of all cases.

New form - transmitted from sick cattle to humans, the most dangerous being the consumption of brain by an infected animal.

Symptoms of Kreuzeld-Jacob disease

The disease has a very rapid course of development. The first symptom is dementia, which leads to memory loss, hallucinations and various personality changes. The following symptoms appear later and develop more slowly.

Creutzfeldt-Jakob disease
Creutzfeldt-Jakob disease

These include various abnormalities - unconscious movements, inability to maintain balance, inarticulate speech, epileptic seizures, distraction and nervousness, convulsive arm movements, muscle spasms, loss of facial expression, already chronic dementia.

Diagnosis of Kreuzeld-Jacob disease

The diagnosis of Kreuzeld-Jakob disease is placed in the presence of rapidly developing senile dementia. The scanner, MRI and X-rays cannot give information about the lesions in the brain, in some cases only atrophy. The most useful test for detecting the disease is electroencephalography, which consists of measuring the electrical activity of the brain.

However, even changes in it are noticed only in the late stage of the disease. Taking brain tissue / biopsy / from a living or dead person establishes the exact changes in the brain cells, but it is difficult, risky for the living and expensive manipulation. It is the difficult diagnosis that is the reason for the untimely detection of Kreuzeld-Jakob disease.

Treatment of Kreuzeld-Jacob disease

To date, no cure has been found for this disease. Its rapid development, difficult diagnosis and pathological brain damage portend a fatal end. Some people die a few months after the first symptoms appear, while others survive for a year. This fatal disease is extremely rare, there are 1-2 cases per 1 million people worldwide.

The article is informative and does not replace a consultation with a doctor!

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