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