Mad cow disease – Creutzfeldt-Jakob disease – Spastic psevdoskleroz

Creutzfeldt-Jakob Disease (Subacute Spongiform Encephalopathy; CJD)

Description of Creutzfeldt-Jakob disease

Spastic psevdoskleroz (BKJa) – rare, mortally, a degenerative brain disease, characterized by rapidly progressive dementia.

Creutzfeldt-Jakob disease can be categorized into different subtypes:

  • Sporadic Creutzfeldt-Jakob disease - also known as classical Creutzfeldt-Jakob disease; the most common type; generally affects people aged 50 years or more;
  • Familial Creutzfeldt-Jakob disease - a hereditary disease;
  • Iatrogenic Creutzfeldt-Jakob disease - the infection occurs through medical procedures, such as injections of growth hormone (growth hormone) or corneal transplants or dura mater of the patient donors;
  • A new variant of Creutzfeldt-Jakob disease (Variant Creutzfeldt-Jakob disease) - Occurs when the consumption of contaminated beef products, which can cause bovine spongiform encephalopathy;
    • Bovine spongiform encephalopathy, commonly known as mad cow disease. Variant Creutzfeldt-Jakob disease is different from other forms of Creutzfeldt-Jakob disease, since it affects young people and has a long period of development.

Causes of Creutzfeldt-Jakob disease

Research the causes of Creutzfeldt-Jakob disease found, that non-hereditary cases cause infectious proteins, called prions. Prions can transform normal protein molecules into abnormal, pathogenic molecule.

Risk factors for Creutzfeldt-Jakob disease

Factors, which increase the risk of Creutzfeldt-Jakob disease:

  • Age: 50-75 years;
  • Use of cadaveric growth hormone;
  • Corneal transplantation;
  • The transplantation of dura mater;
  • Family members with CJD – about 10% -15% of cases are inherited;
  • Consumption of beef products, produced in countries with epidemics of mad cow disease;
  • Medical profession, that work with the brain tissues;
  • Transfusion of blood from the patient CJD.

Symptoms of Creutzfeldt-Jakob disease

At the initial stage of CJD symptoms are not observed. As CJD progresses, symptoms, which may be, include:

  • Memory lapses;
  • Difficulty with concentration;
  • Difficulty with speech;
  • Loss of coordination;
  • Blurred vision;
  • Changes in behavior and mood;
  • Muscle spasms;
  • Convulsions;
  • Loss of mental and physical functions.

Depending on the type of CJD, the disease may last from 3-36 months or longer. CJD is almost always fatal.

Diagnosis of Creutzfeldt-Jakob disease

The doctor will ask about your symptoms and medical history, and perform a physical examination.

CJD is a difficult disease to diagnose. There are no tests, allowing to detect it. Tests, which may be used, to help diagnose:

  • Maybe, you want to record brain activity. This can be done with the electroencephalogram (EEG);
  • I need pictures of the brain, which are used to perform the following procedures:
    • Computed tomography of the head;
    • Magnetic resonance imaging (MRT);
  • Maybe, You will need to identify,how the brain uses glucose. This can be done by using positron emission tomography (PET);
  • There should be an analysis of body fluids. For this purpose:
    • Removing a sample of brain tissue for testing, Why is performed brain biopsy;
    • Blood tests;
    • Poyasnichnaya puncture.

Treatment of Creutzfeldt-Jakob disease

Creutzfeldt-Jakob disease is not curable. The goal of treatment is to relieve pain and symptoms of CJD.

Drug therapy may include:

  • Opiates to treat pain;
  • Anticonvulsants, to reduce neuromuscular problems.

Prevention of Creutzfeldt-Jakob disease

To avoid a new form of CJD, it is recommended not to eat beef, production in the, which may be bovine spongiform encephalopathy. Ways to prevent other forms of Creutzfeldt-Jakob disease does not exist.

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