Creutzfeldt-Jakob disease is a rare disease associated with brain damage due to the appearance of an abnormal prion protein in neurons and named for scientists who first described it. The most common pathology among people aged 65 - 70 years.
Causes of Creutzfeldt-Jakob syndrome
It has been scientifically established that Creutzfeldt-Jakob disease is an infectious pathology. It is known that in the nerve cells of the brain and some other cells of the human body there is a normal prion protein, whose functions remain unclear for today.
An abnormal infectious protein prion, penetrating the human body, enters the brain with blood, where it accumulates on neurons. Further, the pathological prion, in contact with the normal protein of the brain cells, causes a change in its structure, as a result of which the latter gradually transforms into a pathogenic form similar to the infectious prion. Abnormal prions form plaques and cause neuronal death.
Infection with pathogenic prions can occur in the following ways:
- when transplanting infected tissues (skin, cornea of the eye, hard shell of the brain);
- through an unqualified sterilized neurosurgical instrument;
- with blood transfusion;
- with the introduction of hormonal drugs (human gonadotropin, somatotropin);
- when eating meat of sick animals or carriers of infection (cows, goats, etc.).
Also, one of the factors causing the disease is the genetic predisposition associated with the mutation of genes. Some cases of the disease have an unknown origin.
Symptoms of Creutzfeldt-Jakob disease
Creutzfeldt-Jakob disease has a long incubation period, which is associated with the time for which penetration of infectious prions into brain tissue and pathogenic changes in normal prions occurs. How long these processes last depends on the method of infection. So, when infection of brain tissues with infected nonsurgical tools, the disease develops after 15 to 20 months, and when infected medicines are introduced, after 12 years.
Most of Creutzfeldt-Jakob disease has a gradual development. There are three stages of the disease, characterized by various symptoms:
1. The stage of prodromal symptoms:
- irritability;
- difficulty concentrating;
- headaches ;
- dizziness;
- sleep disorders;
- decreased visual acuity;
- changes in behavioral responses;
- sensation of causeless fear;
- abrupt hallucinatory experiences;
- unsteadiness of gait;
- violation of counting operations, reading.
2. Stage of unfolded clinical manifestations:
- spastic paralysis (of the lower or upper limbs, half of the body);
- disorder of coordination of voluntary movements;
- epileptic seizures ;
- muscle rigidity;
- tremor, athetosis;
- myoclonus;
- speech disorders;
- sensitivity disorders.
3. Terminal stage - characterized by deep dementia, in which patients are in a state of prostration, non-contact. There is a strong muscular atrophy, hyperkinesia, swallowing disorders, possible hyperthermia and epileptic seizures.
Treatment and outcome of Creutzfeldt-Jakob disease
This disease in all cases leads to death. The life expectancy of the majority of patients is no more than one year from the moment the onset of the disease begins. To date, approaches to specific treatment are in active development, and patients receive only symptomatic treatment.