Tom Morang
04-29-2002, 09:06 PM
04/28/2002 - Updated 07:42 PM ET
Brain-disease deaths 'unusual, disturbing'
By Anita Manning, USA TODAY
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 USA .
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 reported."
Brain-disease deaths 'unusual, disturbing'
By Anita Manning, USA TODAY
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 USA .
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 reported."