Variant CJD May Be More Infectious: Expert

September 18, 2001 Reuters by Richard Woodman
NEW YORK (Reuters Health) - Britain's Department of Health is greatly concerned that variant CJD may be more infectious than the agent responsible for sporadic CJD, a leading expert said on Tuesday.

Sporadic CJD, or Creutzfeldt-Jakob disease, is a very rare degenerative brain disorder mainly found in older people. Variant CJD (vCJD) is a form of the disease that is believed to be caused by eating meat from cows with bovine spongiform encephalopathy, or ``mad cow'' disease. All of these diseases are caused by malformed protein particles called prions.

Professor Robert Will, director of the National CJD Surveillance Unit, said tests to find out if the tonsils, spleen, lymph nodes and appendix of vCJD victims were infected with prions had all proved positive, whereas tests on patients with sporadic disease were all negative.

He told a conference at the Royal Society of Medicine in London this implied that the new infectious agent was present at greater levels in peripheral parts of the body. This suggests, he added, that vCJD is more infectious and more likely to be spread through blood or contaminated surgical instruments.

``What does this mean in terms of secondary transmission? Well, it is causing a great deal of concern and a lot activity in the Department of Health,'' Will said.

He presented a slide showing a list of common surgical procedures carried out in 41 cases of vCJD before the onset of clinical signs. In none of these procedures had any precautions been taken apart from normal sterilisation procedures.

He pointed out that prion proteins are remarkably resistant to sterilisation. The Department of Health had a surgical instrument panel of experts considering these issues.

In the case of blood donations, Will said, ``We have found 22 individuals who have received blood that was donated by someone who developed vCJD. None of these individuals have yet developed vCJD.''

The Health Department announced in January it would spend 200 million ($300 million US) modernising National Health Service sterilisation equipment and buying disposable surgical tools.

Will, who is based at the Western General Infirmary in Scotland, also questioned the findings of an official investigation last year into the causes of a cluster of vCJD cases in Leicestershire, central England.

The inquiry report linked the cases to cross contamination caused when a butcher removed the cows' heads with the same instruments used to cut meat from the carcass.

``The problem with that argument is that the butcher in question retired in December 1982,'' Will said. ``At that stage the incidence of BSE (news - web sites) would have been incredibly low. If you have a cluster of four or five cases related to that particular practice it is a matter of grave concern for the future.''

Will said that the mean age of onset of vCJD in some 106 cases so far identified in the UK was 27 years and the mean age of death 29 years. The number of cases was doubling every 3 years.

He said diagnosis is difficult as most patients usually presented with depression, anxiety or withdrawal symptoms, which cannot be distinguished from common psychiatric disorders.

As a result, cases had been misdiagnosed, greatly upsetting the families of victims. However, one helpful clue was that half the patients complained of painful tingling, often in their fingers or feet, in addition to psychiatric symptoms, before they developed neurological symptoms.

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