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