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Thursday, 6 September, 2001, 15:07 GMT 16:07 UK
Q&A: What is CJD?
vCJD incidence graph
Variant CJD is still very rare
By BBC News Online's Ivan Noble

What is Creutzfeldt-Jakob Disease?

Creutzfeldt-Jakob Disease (CJD) is a rare and invariably fatal neurodegenerative disease. The brains of CJD sufferers deteriorate rapidly, resulting in dementia and death.

How long has it been around?

The disease was first described by two German neurologists in the 1920s. It is similar to the sheep disease scrapie, which has been known for over 300 years and is endemic in the UK.

How common is it?

Around a hundred people have died in the UK from the new variant form of the disease since 1994. The originally described form of the disease occurs sporadically in roughly one in a million people each year.

What is the difference between CJD and variant CJD?

Variant CJD is the new form of the disease which researchers suspect may be linked to consumption of BSE-infected meat.

The variant form of the disease tends to affect people who are younger than typical sufferers of the sporadic form.

Variant CJD sufferers studied in the UK had an average age of around 27 years, while sporadic CJD sufferers tend to be between 50 and 75 years old.

Variant CJD tends to progress more slowly than the originally known form.

What causes CJD?

The cause of CJD is a "matter of some controversy" according to the UK Creutzfeldt-Jakob Disease Surveillance Unit.

Research points to the involvement of prions - a kind of protein found in healthy brains.

The theory is that if one abnormal prion gets into the brain it will start a chain reaction, turning other prions bad, leading to brain damage and eventual death.

The problem is knowing where that first abnormal prion comes from.

It could be that in very rare cases, a prion spontaneously turns bad. This might account for the one-in-a-million sporadic cases of CJD.

It is also believed that some people inherit a genetic problem which makes them up to a hundred times more susceptible to CJD, though at one in 10,000, the risk for such people is still very low.

And in the case of variant CJD, the suspected but by no means convicted culprit is BSE-infected meat.

There are still problems with the prion theory and scientists are not fully agreed on what lies behind CJD or how it actually causes damage in the brain.

Is there a cure?

No, says the UK Creutzfeldt-Jakob Disease Surveillance Unit.

"Creutzfeldt-Jakob disease and other human prion diseases are invariably fatal and there is currently no available treatment for the underlying disease process," the unit says.

But, it points out, research in the US suggests that quinacrine and chlorpromazine, two drugs commonly used to treat malaria and mental illness, may, in combination, act to slow the progress of the disease and deal with some of its symptoms.

Source: UK Creutzfeldt-Jakob Disease Surveillance Unit





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