Getting to heart of CJD

November 4, 2002 Nottingham Evening Post

The families of CJD victims can only watch powerlessly as the distressing brain disease takes over and destroys their loved ones. Initial symptoms are vague - from depression to aching limbs. But the signs become more obvious as the disease attacks the brain, causing personality changes, loss of sight and loss of balance. Death usually occurs within a year.

Unfortunately by the time obvious symptoms are clear, it may be too late to offer any effective treatment. But that problem could be about to change thanks to a Nottingham company which has pioneered technology to identify BSE in cattle up to eight months before physical signs become apparent.

It believes that within two years, the same technology and principles could be used to identify CJD in humans - helping doctors to find cures for the devastating illness.

TSEnse Diagnostics Ltd, has developed a high resolution electrocardiogram (ECG) machine - a super-charged monitor which builds up a picture of heart rhythms that is five times more accurate than a standard ECG.

By wiring cattle up to the machine, animals infected with BSE were discovered to have a unique heart rate pattern.

Now scientists from the University of Manchester have identified a similar patter in CJD sufferers.

A healthy person's heart rate quickens as you breathe in and slows as you breathe out.

Research suggests that one of the first thing variant CJD attacks is the respiratory system. So abnormalities picked up by the machine could be an early indication that a patient is suffering from the disease.

Research associate in anaesthesia at the university, Laura Woolfson said: "At present the main way of diagnosing the variant CJD is by carrying out a biopsy of the brain or tonsils, which obviously involves invasive surgery.

"These are often the last areas of the body to be affected by variant CJD, so by the time diagnosis has been confirmed there's little chance of successfully treating the patient.

"By using this we can carry out simple tests without the need for surgery and make earlier diagnosis. This will give us the chance to offer treatment much earlier." Adrian Presbury, project manger at TSEnse, said: "It's really new science and is quite ground breaking. In the not-too-distant future I foresee a machine which can tests somebody for CJD and flashes up 'Yes', 'No' or 'Caution' on the screen.

He added: "A test that is reliable in humans long before they display clinical signs is an urgent priority." Last week CJD hit the national headlines, after 24 patients in a Middlesbrough hospital were told they had been at risk of contracting the disease. They had all been operated on with instruments which had been used in surgery on a CJD victim.

"If this test had been employed, the person who was operated on may have been identified as having CJD and those 24 people would not have been put at risk," said Mr Presbury.

"You could also use it to screen blood donors - to ensure anyone suffering CJD would not be donating to the blood bank." The company, which is based in Daleside Road, has been awarded a GBP 211,000 government grant for the work - primarily into BSE.

"That will cover about 25% of the costs so it needs more government investment,"said Mr Presbury. "Testing patients who are suspected of having CJD could be done almost immediately, and the scientists in Manchester are in the process of getting permission to do that. This will open up chance for research and to try new treatments.

"Screening before surgery and before blood donation needs more work - but realistically that could be done in two years.

He added: "There is a big ethical issue surrounding it, though, because at the moment there is no known cure for CJD. So if you screen someone and there's suggestion they have CJD, do you tell them they have a disease that can't be cured? "What ever the dilemmas, what is clear is that the less backing the Government gives it, the longer it will take. The longer it takes, the more people will be at risk." What is CJD? CJD, Creutzfeldt-Jakob disease, is a progressively worsening form of senile dementia which attacks the brain.

It causes personality change, loss of sight, and the deterioration of physical co-ordination, such as loss of balance.

Death usually occurs in sufferers in six months, although some have lived up to five years.

Beef infected with BSE - commonly known as mad cow disease - is believed to have entered the human food chain in the late 1980s and led directly to the development of a new variant of CJD.

Since 1986, around 115 people have died from confirmed or probable CJD.

The first BSE case was detected in 1986, and two years later scientists admitted there were concerns for the safety of the food chain.

In 1990, however, Government ministers insisted British beef was safe to eat until admitting a link between beef and CJD in 1996.

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