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 The
Queniborough Cluster: Proof of a Human Connection to Mad
Cow?
AUBURN, Jan 16, 2004 ---
The suffering associated with a quaint English village
of 2,300 is embodied in the tragedy of Sarah Roberts.
Roberts was a happy, fit, fun-loving young woman in her
late twenties, her father recalled, when she was struck
by a debilitating brain disease in the late 1990’s.
From
the standpoint of medical investigators desperately
trying to account for the spike in outbreaks of
Creutzfeldt-Jakob disease that have occurred in Britain
within the last decade, her circumstances are as
significant as they are tragic. Roberts lived in
Queniborough, which has the dubious distinction of
having an unusually large number of deaths associated
with what appears to be a new form of Cruetzfeldt-Jakob
disease. This condition is often associated with bovine
spongiform encephalopathy, so-called mad cow disease.
Equally
significant, from the standpoint of medical
investigators, is that her death not only occurred in
Queniborough but it occurred in close proximity to
another young victim of the rare disease. Matthew
Parker, a 19-year-old man, not only lived on the same
street, just a couple of hundred yards away, but
attended the same school as Roberts and played with her
as a child. These circumstances, along with the fact
that the other three victims of the region who died of
the disease were also young and lived in or near the
village, have led doctors to search for some common tie.
“Medical investigators consider this a very significant
factor, lending support to the prevailing view that what
is perceived as a new form of CJD associated with these
young people stemmed from some kind of environmental
factor, most likely from eating tainted beef from BSE-infected
cattle,” said Dr. Jean Weese, an Alabama Cooperative
Extension System food scientist and Auburn University
associate professor of nutrition and food science.
The
young ages of these victims, coupled with the fact that
their deaths occurred at the same time BSE was being
detected in large numbers of British cattle, lent
further support to the investigators’ notion that they
were dealing with something entirely new.
While
the evidence continues to intrigue investigators, it
still does not answer the question researchers have been
asking for the last decade: Can eating tainted beef from
mad cow-infected cattle put humans at risk of developing
a similar brain disease?
“All
sorts of theories have been formulated about the root
cause of this perceived new form of CJD,” Weese said.
“For a time, they suspected school lunches of minced
meat and sausage that were produced from the
old-fashioned slaughtering methods rather than the more
advanced meat recovery methods currently prescribed as a
safeguard against infection. They also suspected a
polio vaccine.”
Finally, in March 2001, investigators with the
Leicestershire Health Authority concluded that the most
likely sources of infection were local butcher shops
using older methods of meat extraction that may have
increased the likelihood that meat cuts were tainted
with spine and brain tissue.
Still,
the evidence remains inconclusive. The health authority
report states that only four out of five CJD victims
within the Queniborough area may have been exposed to
the possibly tainted meat, leaving one unaccounted for.
Further
complicating matters is the fact that the inquiry
uncovered no evidence of butchers using meat extraction
methods considered illegal under British law. Also, the
data used by the researchers to reach their conclusion
was partly based on anecdotal accounts of family
members, drawn from memories stretching back as far as
20 years.
The
investigators even conceded that “on a national basis”
the unusual cluster around Queniborough “is unlikely to
explain how all of the people who developed this disease
were exposed to the BSE agent.”
One
other factor continues to baffle investigators. With
almost 200,000 cows testing positive for mad cow, why
hasn’t a similar spike in variant CJD occurred in
humans?
“What
we have here is evidence that is compelling but still
inconclusive,” Weese said. “We have plenty of reasons
to suspect a link between mad cow disease and CJD in
humans but no smoking gun --- not yet, at least.”
(Source: Dr. Jean Weese,
Alabama
Cooperative Extension System Food Scientist and
Auburn
University
Associate Professor of Nutrition and Food Science,
334-844-3269; Writer:
Jim Langcuster, Extension News and Public Affairs
Specialist, 334-844-5686.)
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