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Friday, 17 March, 2000, 12:10 GMT
Living CJD victims diagnosed
CJD brain
CJD causes sponginess in the brain tissue
Scientists can for the first time diagnose somebody suffering from the brain disease CJD before they die.

Until now it has only been possible to confirm that somebody was suffering from the human form of mad cow disease after they died when a brain biopsy could be carried out.

But doctors now say that they have sufficient techniques to be able to say that a person is "probably" suffering from CJD.

Deputy Chief Medical Officer Dr Pat Troop has ordered that living, "probable" CJD victims should now be included in official statistics for the prevalence of the disease from April.

Being able to count and record the number of living sufferers will allow us to make more accurate predictions about the future of this disease

Dr Pat Troop, Deputy Chief Medical Officer
Dr Troop was acting on the advice of the Spongiform Encephalopathy Advisory Committee (SEAC), the Government's expert body on BSE and CJD.

Interim figures show that there are 12 living patients in the UK with probable CJD and three other people who have died but whose relatives were still awaiting confirmation that the disease was the cause of death.

Dr Troop said: "Being able to count and record the number of living sufferers will allow us to make more accurate predictions about the future of this disease.

"This is now possible because diagnostic tests have been very accurate in identifying `probable' CJD in severely neurologically impaired patients.

"However there is no test available to detect those who may be infected with CJD in the pre-clinical (before people fall ill) stage.

"Developing such a test remains one of the Department of Health's research priorities."

Six months to diagnosis

Most CJD victims live with the disease for 14 months, but some survive for more than three years.

The new criteria mean that patients should be able to be identified as "probable" CJD sufferers within six months of the first signs.

So far 52 people have died from new variant CJD since it was identified in 1995.

Scientists believe CJD is caused by particle known as a prion.

The prion is a misfit because it ignores the rules that are supposed to govern biological reproduction.

Unlike other agents that cause disease, like a virus, it contains none of the genetic information necessary to spread itself through its victim.

Instead, the prion corrupts a perfectly normal protein, PrP, which usually sits on the external surface of brain cells.

This leads to insoluble deposits building up within brain cells, which then no longer function properly and die.

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See also:

13 Nov 99 | Health
Blood test for CJD created
14 Jan 00 | Health
CJD treatment 'draws closer'
21 Dec 99 | Health
CJD: What is the risk?
05 Mar 00 | Health
CJD threat to mothers
19 Jan 00 | Health
CJD 'will not be an epidemic'
18 Aug 99 | Medical notes
Prion diseases: A brief history
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