Encyclopedia

Creutzfeldt-Jakob disease

Description

Creutzfeldt-Jakob Disease /CJD/ is a degenerative brain disease that leads to dementia, and if left untreated progresses very quickly and can lead to and unto death.

The symptoms of the disease sometimes closely resemble those of other brain disorders leading to dementia such as Alzheimer’s disease.

This degenerative brain disease came to public attention in the 1990s when several people in the UK developed a variant of the disease after eating meat from diseased cattle. However, the classic form of the disease is not associated with contaminated beef.

Although serious, the disease is rare, especially the form that is caused by eating infected beef. Worldwide, every year 1 case of the disease is detected per 1 million people, most often in elderly people.

What are the symptoms?

The disease is marked by a rapid deterioration of the mental state of the sufferer, usually this happens within a few months. Initial signs and symptoms most often are:

• Negative personality changes;
• Anxiety;
• Depression;
• Gradual loss of memory;
• Decreased ability to perform more complex mental activities.
• Blurred vision;< br/> • Insomnia;
• Difficulty speaking;
• Difficulty swallowing;
• Sudden sharp movements;

As the disease progresses, the negative mental changes deepen. Subsequently, most people fall into a coma. Heart failure, respiratory failure, pneumonia or other infections are usually the cause of death.

The disease in most cases develops within 7 months and reaches its final phase also within this period. However, many cases have been described in which after the diagnosis, the patients continue to live another 1 or 2 years.

In people with rarer forms of the disease, mental symptoms may be more pronounced at the beginning with dementia – a gradual loss of mental abilities. And memory loss and speech disorders develop later as the disease progresses.

This variety of the disease usually affects people at a younger age than the classical one, and lasts longer, usually 12-14 months.

What are the causes?

BCL and its variants belong to a large group of diseases known as transmissible spongiform encephalopathies. The name derives from the porous openings visible under a microscope that develop in the affected brain tissue.

The cause of the disease, as for other diseases in the group, is the infectious form of the PrPSc protein, called a prion. Normally this protein, when in its normal form PrPC is not harmful to body tissues, but when in its infectious form it wreaks havoc on normal biological processes.

Creutzfeldt-Jakob Disease Treatment

There is no effective treatment for the disease or any of its variants. A number of drugs have been tested including steroids, antibiotics and antiviral drugs but have not had a positive effect on the sufferers.

Medications interleukins help slow down the disease. For this reason, doctors focus their efforts on alleviating pain and other symptoms in order to provide as much comfort as possible to the sick. Medicines are administered to limit aggressive behavior.

Prevention

Hospitals and other medical facilities implement policies to prevent iatrogenic BCD. These measures are expressed in:

• Exclusive use only of synthetic human growth hormone, not the kind secreted by the human pituitary gland.
Destruction of surgical instruments used in surgery on the brain or nerve tissue of someone with proven or suspected Creutzfeldt-Jakob disease.
• The use of single-use instruments to perform lumbar punctures.< /p>

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