A Simple Guide to Variant Creutzfeldt-Jakob Disease, Diagnosis, Treatment and Related Conditions

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By Kenneth Kee

cover image of A Simple Guide to Variant Creutzfeldt-Jakob Disease, Diagnosis, Treatment and Related Conditions

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This book describes Variant Creutzfeldt-Jakob Disease, Diagnosis and Treatment and Related Diseases

Ever since the first TV and newspaper pictures of Mad Cow Disease with cows falling on their legs and foaming at the mouth appear in 1990s, many people in Singapore and worldwide have been fearful of taking beef.
Even the popular Bovril extract made of beef has its ingredients of beef changed to vegetables.

Bovine Spongiform Encephalopathy (BSE) or Mad Cow Disease is an infectious disease produced by prions that damage the brain tissues of cattle.

Bovine Spongiform Encephalopathy (BSE) indicates the changes in brain tissue of affected cows.

When humans consume the diseased tissue from cattle, they may form the human form of mad cow disease termed variant Creutzfeldt-Jakob disease (vCJD) or new variant Creutzfeldt-Jakob disease (nvCJD).

Creutzfeldt-Jakob disease in the classic type normally happens in older people either:
1. From an inherited tendency of the brain to change or
2. The disease happens naturally for no obvious reason.

The type diagnosed as happening from eating diseased cattle happens in younger people and has abnormal medical traits, with outstanding psychiatric or sensory symptoms at the time of medical manifestation and delayed start of neurological abnormalities.

These neurological abnormalities are:
1. Ataxia within weeks or months,
2. Dementia (with memory loss and confusion) and
3. Myoclonus late in the illness,
4. A period of illness of at least six months, and
5. A diffusely abnormal non-diagnostic electroencephalogram.

The transmissible causes that induce the disease in both cattle and humans are the atypical protein particles termed prions.

Prions are not the same as bacteria or viruses that produce other infectious diseases; rather, they are regarded as infectious abnormal proteins that are capable of inducing abnormal folding of specific normal cellular proteins.

The abnormal prions are present in the brain, spinal cord, eye (in the retina), and other tissues of the nervous system of affected animals or humans.

Also, prions can be discovered in the nervous system and in sites such as bone marrow, spleen, and lymph nodes.

Low levels of prions may also be present in blood.

CJD is a type of brain damage that causes a rapid decrease of movement and mental function.

Creutzfeldt-Jakob disease is very infrequent.

It happens in about 1 of every 1 million people.

Variant CJD produces less than 1% of all CJD cases.

It tends to affect younger people.

Over 220 people worldwide have had this disease.

Almost all cases happened in England and France.

Only 4 cases have been reported in the U.S., and in all 4 of these cases, there is evidence that indicates that the infection was acquired while abroad in Europe or the Middle East.

Variant CJD is produced by consuming infected meat.

The infection that produces the disease in cows is thought to be the same one that causes vCJD in humans.

CJD may be related to several other diseases caused by prions such as:
1. Chronic wasting disease (deer)
2. Kuru (mostly women in New Guinea who ate the brains of dead relatives as part of a funeral ritual)
3. Scrapie (sheep)
4. Other very rare inherited human diseases, such as Gerstmann-Straussler-Scheinker disease and fatal familial insomnia

All prion diseases are fatal.

The CJD disease can only be confirmed with a brain biopsy or autopsy.

There is no known cure for this disease.

The doctor will provide medicines to relieve symptoms

TABLE OF CONTENT

A Simple Guide to Variant Creutzfeldt-Jakob Disease, Diagnosis, Treatment and Related Conditions