USA Today Plays Down Human
Mad Cow Disease Cases in Michigan

Michigan brain-disease deaths 'unusual, disturbing'
By Anita Manning
April 29, 2002
Page 7D

Two young men, ages 26 and 28, died last fall in the same Michigan hospital
of a rare brain disease that occurs mainly in elderly people.

The incident, which raised fears that the human form of mad cow disease, or
something similar, had emerged in the USA, prompted a swift investigation by
federal health officials, but doctors familiar with the cases say there is
no evidence to support that fear. They say autopsies and other tests
indicate the victims died from so-called ''classic'' forms of
Creutzfeldt-Jakob disease (CJD).

While the cases are ''highly unusual and disturbing,'' says University of
Michigan neurologist Norman Foster, the data show that the forms of CJD
suffered by the young men are ones seen previously in older individuals.

CJD occurs at the rate of about 1 person per million per year, almost always
in people over age 60. What doctors feared is that a new form of CJD,
possibly similar to a variant that emerged in the mid-1990s in the United
Kingdom and linked to consumption of mad-cow-infected beef, had struck.

Unlike classic CJD, the new variant, vCJD, strikes mainly young adults. It
has killed more than 100 people. The only known case of vCJD in the USA was
diagnosed recently in a 22-year-old British woman living in Florida, who is
thought to have contracted the disease in England.

Mad cow disease has not been detected in cattle in the USA, but a similar
disease in deer and elk is spreading in the Midwest. Chronic wasting disease
(CWD) is fatal to deer and elk but is not known to cause illness in humans.

Lawrence Schoenberger of the Centers for Disease Control and Prevention in
Atlanta says the agency sent investigators to Michigan in late August, when
the victims were still alive.

''The key thing here is the two were right together. We were worried that
there was maybe a common exposure, but our investigation revealed that was
not the case.'' The men lived in adjacent counties but did not know each
other, he says.

In the rare cases when CJD strikes before age 30, it is often caused by a
hereditary form of the disease, says Foster, and ''tests are continuing to
see if that may be a factor in these cases.''

But extensive family interviews determined that neither man had a family
history of dementia, nor had they eaten venison or elk meat or visited
countries where mad cow disease has been detected.

''We feel as comfortable as anyone can that this is not related to either
CWD or (mad cow disease),'' says Foster, who treated the patients at the
University of Michigan Medical Center in Ann Arbor.

Not everyone is comfortable. ''I discount the statement that these two young
people, dying at the same time in the same hospital in southeast Michigan,
did not eat venison, after living their entire lives in that state,'' says
John Stauber of the Center for Media & Democracy and co-author of Mad Cow

He suspects a new American variant of CJD, perhaps related to chronic
wasting disease, may be emerging. ''Any attempt to portray these CJD
deaths as some sort of 'normal' occurrence that has simply, to date, gone
unobserved is absurd,'' Stauber says.

Current estimates of only five cases per billion of CJD in people 30 and
younger may be incorrect, says Foster, who co-wrote a report on the cases
presented this month at a meeting of the American Academy of Neurology.

''The fact that they both occurred at the same time in a relatively small
population suggests that (CJD in younger people) may be more common than
previously suspected,'' Foster says. Doctors don't expect to see it in young
people, so misdiagnosis may occur. ''Any young individual with progressive
neurologic disease should be considered for CJD.''

He says the cases also underscore the need for a national system to seek out
and report all cases of CJD. ''There certainly is the possibility that other
cases have been seen and not diagnosed, or even if diagnosed, not

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