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Friday, May 21, 1999 Published at 10:16 GMT 11:16 UK


Health

CJD deaths could be on the rise

A scientist checks tissue samples for CJD

An expert in the human form of mad cow disease says that a recent increase in the number of deaths could mark the start of a gradual climb in the death rate.

Dr Marc Turner, senior lecturer in tranfusion medicine at the University of Edinburgh, told a conference that he expected more new variant Creutzfeld-Jakob Disease (nv-CJD) cases in future months.

"Whether this increase in deaths is simply going to represent the peak and will die away, or is the beginning of a longer slow climb, I don't know," he said.

A screening test for the human form of mad cow disease in blood is at least two years away, he warned the conference, which is debating the possible risk of transmitting the disease through blood transfusions.

He said that the millions spent by the government on precautionary measures in the meantime was justified.

Dr Turner said: "I personally don't think we are gong too far. It could take five years or more to establish concrete proof, and a lot of people could be exposed by then. There is huge moral pressure"

The NHS currently spends £95m a year to make sure that new variant Creutzfeld-Jakob Disease can't be caught through blood transfusions, even though there is no proof that it can be transmitted this way.

Evidence of danger

Other scientists say there is evidence which suggests nv-CJD can in theory be passed to humans via blood and blood products.

Dr James Ironside, a consultant neuropathologist at the CJD Surveillance Centre in Edinburgh, said the differences between CJD and the newly-discovered nv-CJD were giving cause for concern.

New variant CJD first came to light in 1996, and to date, 40 cases have been confirmed in the UK.


[ image: Human brain tissue]
Human brain tissue
In original CJD, the agent thought to carry the infection, the prion, was found only in the brain and central nervous system of patients.

However, in nv-CJD there is evidence that it may have travelled widely around the body into areas like the tonsils and spleen, increasing the risk that it may be carried in blood or blood plasma.

Dr Ironside said: "It's this difference that is focusing concerns on the risks or additional risks to blood and blood products.

"We don't have any direct evidence to say blood is infectious - we are waiting for the results of animal experiments, but these take time.

"So the question is, do we wait, or do we do something in the meantime?"

Younger victims

It differs from the standard form of CJD in that the average age of sufferers is considerably younger - and it generally takes longer for the patient to die after symptoms are first noticed.

Sufferers exhibit symptoms of dementia, with memory loss, confusion and physical unsteadiness as the brain is attacked by the disease.

There is no cure for the disease, and until recently, the diagnosis can only be confirmed after death by analysis of brain tissue. Research has shown that in many cases, the patients were initially thought to have psychiatric disorders.

However, the development of a new test using living tonsil tissue could mean far earlier diagnosis.

CJD is the human form of the cow disease, Bovine Spongiform Encephalopathy, although little is known about links between the outbreak of BSE in cattle and the number of cases of CJD in the human population.

Some families whose relatives died of nv-CJD are suing the government for compensation, claiming the population should have been protected from meat from cattle which had died from BSE.

However, scientists led by Alan Ebringer, professor of immunology at King's College, London, claimed this week that CJD is in fact caused by the body's own reaction to a bacterium found commonly in contaminated water and the soil.

This, they say, would explain such oddities as the fact that one of the victims of nvCJD was a vegetarian.



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