Creutzfeldt-Jakob disease (CJD) is a very rare degenerative brain disease that affects one in 1 million people, that is just about six persons per year. A special form is variant CJE (vCJD), which can infect via beef from cattle affected by mad cow disease (bovine spongiform encephalopathy, BSE).
Creutzfeldt-Jakob disease has been known since the 1920s, but vCJD was first detected in Britain in the mid 1990s.
How do you get Creutzfeldt-Jakob disease?
CJD is caused by a so-called prion protein that is similar to a second protein that occurs naturally in the brain .However, the prion protein can not be broken down.When prion proteins enter the brain, the person’s normal proteins can also be converted to the prion proteins, which are deposited in the brain cells. The deposits will damage the brain cell function, and in the course of several years is formed large spongy (spongiform) holes in the brain.
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CJD can occur in several ways:
- Sporadic CJD: Due to an accidental mutation in a person’s genes. It represents approximately 75 percent of all cases and particularly affects older people.
- Inherited CJD: Occurs in certain families due to inherited mutations in the genes coding for the prion protein. Amounts to five to ten percent of all cases.
- Retained CJD: Some cases occurred earlier in transplantation of cornea or meninges, or after treatment with contaminated growth hormone formulation. Today used drugs without risk of infection.
- Variant CJD (vCJD): The most talked form since 1996 has affected approximately 160 people, predominantly in the UK. Variant CJD is contagious after consuming beef infected with mad cow disease.
What are the danger signs?
Sporadic CJD typically occurs after the age of 50 with progressive dementia . The coordination of movements deteriorates, personality changes, and the sight impaired or lost. Gradually lose the person speaking ability, and the body becomes stiff and motionless. The person dies within a few months.
vCJD affects younger, often in the 20-30 years of age. The first symptoms are psychological with a changed personality and behavior. Later develops character dementia and movement disorders. Death occurs within 18 months after the first symptoms.
What are prions?
Prions are not micro-organisms, proteins, under rare circumstances, transmitted from person to person as well as by certain foods.
In addition to CJD prions are involved in three other extremely rare disorders:
- Gerstmann-Straussler-Scheinker disease.
- Kuru who hit cannibals in Papua New Guinea who ate the brains.
- Familial fatal hypersomnia.
How is the disease diagnosed?
The typical clinical symptoms allows the suspicion, and EEG (measuring the brain’s electrical activity) , study of spinal fluid and MRI may provide additional clues. The diagnosis can usually only be made with confidence after the person’s death by examining a biopsy of the brain under the microscope.
How to treat the Creutzfeldt-Jakob disease?
CJD can not be cured but symptomatic treatment to relieve the patient’s condition as possible along the way.
Why is it called Creutzfeldt-Jakob disease?
In 1920 described the German neurologist Hans Gerhard Creutzfeldt a 23-year-old female patient died of progressive mental and neurological disorders. The following year, gave German Alfons Maria Jakob three patients with similar symptoms.
Name of disease “Creutzfeldt-Jakobsche Krankheit” was used for the first time in 1922. In 1954 introduced the concept of “slow virus” because it was erroneously believed that the symptoms caused by a virus that was several years to break out.
In 1957 described American, reminiscent of CJD among cannibals in New Guinea. He assumed also that it was about a slow virus and even received the Nobel Prize for this in 1976. The American Prusiner made in 1982 the theory of prions ago was awarded the Nobel Prize in 1998.
Prion diseases are found in many animal species, including cattle, elk, mink, cats, big cats and antelope. In all cases characterized diseases of the brain tissue is destroyed, forming holes in a sponge.
In the middle of the 1990s hit CJD surprisingly, younger people in the UK, which was unusual because the disease usually occurs in older people. This variant CJD (vCJD) was linked to mad cow disease, which in the same period of attack up to 200,000 cattle in the UK. The explanation was that meat and bone meal made from dead sheep and cattle until 1988 was used as a protein supplement in animal feed. This led the infection from the first animal that died of prion diseases on to a large number of animals in a vicious circle. A few cases of BSE detected in Denmark in 2000.
Subsequently, strict regulations for slaughter, which removes the brain, spinal cord and eyes, restrictions on the use of meat and bone meal as animal feed, BSE testing of cattle, as well as restrictions towards blood donation by blocking this pathway. Milk and dairy products contain no infection.