by Michael Greger, M.D.
January 7, 2004

October 2001, 34-year-old Washington State native Peter Putnam
started losing his mind. One month he was delivering a keynote
business address, the next he couldn't form a complete sentence. Once
athletic, soon he couldn't walk. Then he couldn't eat. After a brain
biopsy showed it was Creutzfeldt-Jakob disease, his doctor could no
longer offer any hope. "Just take him home and love him," the doctor
counseled his family.[1,2,3] Peter's tragic death, October 2002, may
have been caused by Mad Cow disease.

Seven years earlier and 5000 miles away, Stephen Churchill was the
first in England to die. His first symptoms of depression and
dizziness gave way to a living nightmare of terrifying
hallucinations; he was dead in 12 months at age 19.[4] Next was Peter
Hall, 20, who showed the first signs of depression around Christmas,
1994. By the next Christmas, he couldn't walk, talk, or do anything
for himself.[5] Then it was Anna's turn, then Michelle's. Michelle
Bowen, age 29, died in a coma three weeks after giving birth to her
son via emergency cesarean section. Then it was Alison's turn. These
were the first five named victims of Britain's Mad Cow epidemic. They
died from what the British Secretary of Health called the worst form
of death imaginable, Creutzfeldt-Jakob disease, a relentlessly
progressive and invariably fatal human dementia.[6] The announcement
of their deaths, released on March 20, 1996 (ironically, Meatout
Day[7]), reversed the British government's decade-old stance that
British beef was safe to eat.[8]

It is now considered an "incontestable fact" that these human deaths
in Britain were caused by Bovine Spongiform Encephalopathy (BSE), or
Mad Cow disease.[9] Bovine means "cow or cattle," spongiform means
"sponge-like," and encephalopathy means "brain disease." Mad Cow
disease is caused by unconventional pathogens called
prions--literally infectious proteins--which, because of their unique
structure, are practically invulnerable, surviving even
incineration[10] at temperatures hot enough to melt lead.[11] The
leading theory as to how cows got Mad Cow disease in the first place
is by eating diseased sheep infected with a sheep spongiform
encephalopathy called scrapie.[12]

In humans, prions can cause Creutzfeldt-Jakob disease (CJD), a human
spongiform encephalopathy whose clinical picture can involve weekly
deterioration into blindness and epilepsy as one's brain becomes
riddled with tiny holes.

We've known about Creutzfeldt-Jakob disease for decades, since well
before the first mad cow was discovered in 1985. Some cases of CJD
seemed to run in families; other cases seemed to just arise
spontaneously in about one in a million people every year, and were
hence dubbed "sporadic." The new form of CJD caused by eating beef
from cows infected with Mad Cow disease, though, seemed to differ
from the classic sporadic CJD.

The CJD caused by infected meat has tended to strike younger people,
has produced more psychotic symptoms, and has often dragged on for a
year or more. The most defining characteristic, though, was found
when their brains were sampled. The brain pathology was vividly
reminiscent of Kuru, a disease once found in a New Guinea tribe of
cannibals who ate the brains of their dead.[13] Scientists called
this new form of the disease "variant" CJD.

Other than Charlene, a 24 year old woman now so tragically dying in
Florida, who was probably infected in Britain, there have been no
reported cases of variant CJD in the U.S.[14] Hundreds of confirmed
cases of the sporadic form of Creutzfeldt-Jakob disease, however,
arise in the United States every year,[15] but the beef industry is
quick to point out these are cases of sporadic CJD, not the new
variant known to be caused by Mad Cow disease.[16] Of course, no one
knows what causes sporadic CJD. New research, discussed below,
suggests that not hundreds but thousands of Americans die of sporadic
CJD every year, and that some of these CJD deaths may be caused by
eating infected meat after all.

Although the fact that Mad Cow disease causes variant CJD had already
been strongly established, researchers at the University College of
London nevertheless created transgenic mice complete with "humanized"
brains genetically engineered with human genes to try to prove the
link once and for all. When the researchers injected one strain of
the "humanized" mice with infected cow brains, they came down with
the same brain damage seen in human variant CJD, as expected. But
when they tried this in a different strain of transgenic "humanized"
mice, those mice got sick too, but most got sick from what looked
exactly like sporadic CJD! The Mad Cow prions caused a disease that
had a molecular signature indistinguishable from sporadic CJD. To the
extent that animal experiments can simulate human results, their
shocking conclusion was that eating infected meat might be
responsible for some cases of sporadic CJD in addition to the
expected variant CJD. The researchers concluded that "it is therefore
possible that some patients with [what looks like]... sporadic CJD
may have a disease arising from BSE exposure."[17] Laura Manuelidis,
section chief of surgery in the neuropathology department at Yale
University comments, "Now people are beginning to realize that
because something looks like sporadic CJD they can't necessarily
conclude that it's not linked to [Mad Cow disease]..."[18]

This is not the first time meat was linked to sporadic CJD. In 2001,
a team of French researchers found, to their complete surprise, a
strain of scrapie--"mad sheep" disease--that caused the same brain
damage in mice as sporadic CJD.[19] "This means we cannot rule out
that at least some sporadic CJD may be caused by some strains of
scrapie," says team member Jean-Philippe Deslys of the French Atomic
Energy Commission's medical research laboratory.[20]

Population studies had failed to show a link between CJD and lamb
chops, but this French research provided an explanation why. There
seem to be six types of sporadic CJD and there are more than 20
strains of scrapie. If only some sheep strains affect only some
people, studies of entire populations may not clearly show the
relationship. Monkeys fed infected sheep brains certainly come down
with the disease.[21] Hundreds of "mad sheep" were found in the U.S.
in 2003.[22] Scrapie remains such a problem in the United States that
the USDA has issued a scrapie "declaration of emergency."[23] Maybe
some cases of sporadic CJD in the U.S. are caused by sheep meat as
well.[24]

Pork is also a potential source of infection. Cattle remains are
still boiled down and legally fed to pigs (as well as chickens) in
this country. The FDA allows this exemption because no "naturally
occurring" porcine (pig) spongiform encephalopathy has ever been
found. But American farmers typically kill pigs at just five months
of age, long before the disease is expected to show symptoms. And,
because pigs are packed so tightly together, it would be difficult to
spot neurological conditions like spongiform encephalopathies, whose
most obvious symptoms are movement and gait disturbances. We do know,
however, that pigs are susceptible to the disease--laboratory
experiments show that pigs can indeed be infected by Mad Cow
brains[25]--and hundreds of thousands of downer pigs, too sick or
crippled by injury to even walk, arrive at U.S. slaughterhouses every
year.[26]

A number of epidemiological studies have suggested a link between
pork consumption and sporadic CJD. Analyzing peoples' diet histories,
the development of CJD was associated with eating roast pork, ham,
hot dogs, pork chops, smoked pork, and scrapple (a kind of pork
pudding made from various hog carcass scraps). The researchers
concluded, "The present study indicated that consumption of pork as
well as its processed products (e.g., ham, scrapple) may be
considered as risk factors in the development of Creutzfeldt-Jakob
disease." Compared to people that didn't eat ham, for example, those
who included ham in their diet seemed ten times more likely to
develop CJD.[27] In fact, the USDA may have actually recorded an
outbreak of "mad pig" disease in New York 25 years ago, but still
refuses to reopen the investigation despite petitions from the
Consumer's Union (the publishers of Consumer Reports magazine).[28]

Sporadic CJD has also been associated with weekly beef
consumption,[29] as well as the consumption of roast lamb,[30] veal,
venison, brains in general,[31] and, in North America,
seafood.[32,33] The development of CJD has also, surprisingly, been
significantly linked to exposure to animal products in
fertilizer,[34] sport fishing and deer hunting in the U.S.,[35] and
frequent exposure to leather products.[36]

We do not know at this time whether chicken meat poses a risk. There
was a preliminary report of ostriches allegedly fed risky feed in
German zoos who seemed to come down with a spongiform
encephalopathy.[37] Even if chickens and turkeys themselves are not
susceptible, though, they may become so-called "silent carriers" of
Mad Cow prions and pass them on to human consumers.[38] Dateline NBC
quoted D. Carleton Gajdusek, the first to be awarded a Nobel Prize in
Medicine for his work on prion diseases,[39] as saying, "it's got to
be in the pigs as well as the cattle. It's got to be passing through
the chickens."[40] Dr. Paul Brown, medical director for the US Public
Health Service, believes that pigs and poultry could indeed be
harboring Mad Cow disease and passing it on to humans, adding that
pigs are especially sensitive to the disease. "It's speculation," he
says, "but I am perfectly serious."[41]

The recent exclusion of most cow brains, eyes, spinal cords, and
intestines from the human food supply may make beef safer, but where
are those tissues going? These potentially infectious tissues
continue to go into animal feed for chickens, other poultry, pigs,
and pets (as well as being rendered into products like tallow for use
in cosmetics, the safety of which is currently under review[42]).
Until the federal government stops the feeding of slaughterhouse
waste, manure, and blood to all farm animals, the safety of meat in
America cannot be guaranteed.

The hundreds of American families stricken by sporadic CJD every year
have been told that it just occurs by random chance. Professor
Collinge, the head of the University College of London lab, noted
"When you counsel those who have the classical sporadic disease, you
tell them that it arises spontaneously out of the blue. I guess we
can no longer say that."

"We are not saying that all or even most cases of sporadic CJD are as
a result of BSE exposure," Professor Collinge continued, "but some
more recent cases may be--the incidence of sporadic CJD has shown an
upward trend in the UK over the last decade... serious consideration
should be given to a proportion of this rise being BSE-related.
Switzerland, which has had a substantial BSE epidemic, has noted a
sharp recent increase in sporadic CJD."[43] In the Nineties,
Switzerland had the highest rate of Mad Cow disease in continental
Europe, and their rate of sporadic CJD doubled.[44]

We don't know exactly what's happening to the rate of CJD in this
country, in part because CJD is not an officially notifiable
illness.[45] Currently only a few states have such a requirement.
Because the Centers for Disease Control (CDC) does not actively
monitor the disease on a national level,[46] a rise similar to the
one in Europe could be missed.[47] In spite of this, a number of U.S.
CJD clusters have already been found. In the largest known U.S.
outbreak of sporadic cases to date,[48] five times the expected rate
was found to be associated with cheese consumption in Pennsylvania's
Lehigh Valley.[49] A striking increase in CJD over expected levels
was also reported in Florida[50] and New York (Nassau County)[51]
with anecdotal reports of clusters of deaths in Oregon[52] and New
Jersey.[53]

Perhaps particularly worrisome is the seeming increase in CJD deaths
among young people in this country. In the 18 years between 1979 and
1996, only a single case of sporadic CJD was found in someone under
30. Whereas between 1997 and 2001, five people under 30 died of
sporadic CJD. So five young Americans dying in five years, as opposed
to one young case in the previous 18 years. The true prevalence of
CJD among any age group in this country remains a mystery, though, in
part because it is so commonly misdiagnosed.[54]

The most frequent misdiagnosis of CJD among the elderly is
Alzheimer's disease.[55] Neither CJD nor Alzheimer's can be
conclusively diagnosed without a brain biopsy,[56] and the symptoms
and pathology of both diseases overlap. There can be spongy changes
in Alzheimer's, for example, and senile Alzheimer's plaques in
CJD.[57] Stanley Prusiner, the scientist who won the Nobel Prize for
his discovery of prions, speculates that Alzheimer's may even turn
out to be a prion disease as well.[58] In younger victims, CJD is
more often misdiagnosed as multiple sclerosis or as a severe viral
infection.[59]

Over the last 20 years the rates of Alzheimer's disease in the United
States have skyrocketed.[60] According to the CDC, Alzheimer's
Disease is now the eighth leading cause of death in the United
States,[61] afflicting an estimated 4 million Americans.[62] Twenty
percent or more of people clinically diagnosed with Alzheimer's
disease, though, are found at autopsy not to have had Alzheimer's at
all.[63] A number of autopsy studies have shown that a few percent of
Alzheimer's deaths may in fact be CJD. Given the new research showing
that infected beef may be responsible for some sporadic CJD,
thousands of Americans may already be dying because of Mad Cow
disease every year.[64]

Nobel Laureate Gajdusek, for example, estimates that 1% of people
showing up in Alzheimer clinics actually have CJD.[65] At Yale, out
of a series of 46 patients clinically diagnosed with Alzheimer's, six
were proven to have CJD at autopsy.[66] In another study of brain
biopsies, out of a dozen patients diagnosed with Alzheimer's
according to established criteria, three of them were actually dying
from CJD.[67] An informal survey of neuropathologists registered a
suspicion that CJD accounts for 2-12% of all dementias in
general.[68] Two autopsy studies showed a CJD rate among dementia
deaths of about 3%.[69,70] A third study, at the University of
Pennsylvania, showed that 5% of patients diagnosed with dementia had
CJD.[71] Although only a few hundred cases of sporadic CJD are
officially reported in the U.S. annually,[72] hundreds of thousands
of Americans die with dementia every year.[73] Thousands of these
deaths may actually be from CJD caused by eating infected meat.

The incubation period for human spongiform encephalopathies such as
CJD can be decades.[74] This means it can be years between eating
infected meat and getting diagnosed with the death sentence of CJD.
Although only about 150 people have so far been diagnosed with
variant CJD worldwide, it will be many years before the final death
toll is known. In the United States, an unknown number of animals are
infected with Mad Cow disease, causing an unknown number of human
deaths from CJD. The U.S. should immediately begin testing all cows
destined for human consumption, as is done in Japan, should stop
feeding slaughterhouse waste to all farm animals (see
http://organicconsumers.org/madcow/GregerBSE.cfm), and should
immediately enact an active national surveillance program for CJD.[75]

Five years ago this week, the Center for Food Safety, the Humane
Farming Association, the Center for Media & Democracy, and ten
families of CJD victims petitioned the FDA and the CDC to immediately
enact a national CJD monitoring system, including the mandatory
reporting of CJD in all 50 states.[76] The petition was denied.[77]
The CDC argued that their passive surveillance system tracking death
certificate diagnoses was adequate. Their analysis of death
certificates in three states and two cities, for example, showed an
overall stable and typical one in a million CJD incidence rate from
1979 to 1993.[78] But CJD is so often misdiagnosed, and autopsies are
so infrequently done, that this system may not provide an accurate
assessment.[79]

In 1997, the CDC set up the National Prion Disease Pathology
Surveillance Center at Case Western Reserve University to analyze
brain tissue from CJD victims in the U.S. in hopes of tracking any
new developments. In Europe, surveillance centers have been seeing
most, if not all, cases of CJD. The U.S. center sees less than half.
"I'm very unhappy with the numbers," laments Pierluigi Gambetti , the
director of the Center. "The British and Germans politely smile when
they see we examine 30% or 40% of the cases," he says. "They know
unless you examine 80% or more, you are not in touch."[80] "The
chance of losing an important case is high."[81]

One problem is that many doctors don't even know the Center exists.
And neither the CDC nor the Center are evidently authorized to reach
out to them directly to bolster surveillance efforts, because it's
currently up to each state individually to determine how--or even
whether--they will track the disease. In Europe, in contrast, the
national centers work directly with each affected family and their
physicians.[82] In the U.S., most CJD cases--even the confirmed
ones--seem to just fall through the cracks. In fact, based on the
autopsy studies at Yale and elsewhere, it seems most CJD cases in the
U.S. aren't even picked up in the first place.

Autopsy rates have dropped in the U.S. from 50% in the Sixties to
less than 10% at present.[83] Although one reason autopsies are
rarely performed on atypical dementia cases is that medical
professionals are afraid of catching the disease,[84] the primary
reason for the decline in autopsy rates in general appears to be
financial. There is currently no direct reimbursement to doctors or
hospitals for doing autopsies, which often forces the family to
absorb the cost of transporting the body to an autopsy center and
having the brain samples taken, a tab that can run upwards of
$1500.[85]

Another problem is that the National Prion Disease Pathology
Surveillance Center itself remains underfunded. Paul Brown, medical
director for the National Institutes of Health, has described the
Center's budget as "pitiful," complaining that "there isn't any
budget for CJD surveillance."[86] To adequately survey America's 290
million residents, "you need a lot of money." UK CJD expert Robert
Will explains, "There was a CJD meeting of families in America in
which... [the CDC] got attacked fairly vigorously because there
wasn't proper surveillance. You could only do proper surveillance if
you have adequate resources."[87] "I compare this to the early days
of AIDS," says protein chemist Shu Chen, who directs the Center's
lab, "when no one wanted to deal with the crisis."[88]

Andrew Kimbrell, the director of the Center for Food Safety, a
D.C.-based public interest group, writes, "Given what we know now, it
is unconscionable that the CDC is not strictly monitoring these
diseases."[89] Given the presence of Mad Cow disease in the U.S., we
need to immediately enact uniform active CJD surveillance on a
national level, provide adequate funding not only for autopsies but
also for the shipment of bodies, and require mandatory reporting of
the disease in all 50 states. In Britain, even feline spongiform
encephalopathy, the cat version of Mad Cow disease, is an officially
notifiable illness. "No one has looked for CJD systematically in the
U.S.," notes NIH medical director Paul Brown. "Ever."[90]

The animal agriculture industries continue to risk public safety, and
the government seems to protect the industries' narrow business
interests more than it protects its own citizens. Internal USDA
documents retrieved through the Freedom of Information Act show that
our government did indeed consider a number of precautionary measures
as far back as 1991 to protect the American public from Mad Cow
disease. According to one such document, however, the USDA explained
that the "disadvantage" of these measures was that "the cost to the
livestock and rendering industries would be substantial."[91]

Plant sources of protein for farm animals can cost up to 30% more
than cattle remains.[92] The Cattlemen's Association admitted a
decade ago that animal agribusiness could indeed find economically
feasible alternatives to feeding slaughterhouse waste to other
animals, but that the they did not want to set a precedent of being
ruled by "activists."[93]

Is it a coincidence that USDA Secretary Veneman chose Dale Moore,
former chief lobbyist for the National Cattlemen's Beef Association,
as her chief of staff?[94] Or Alison Harrison, former director of
public relations for the Cattlemen's Association, as her official
spokeswoman?[95] Or that one of the new Mad Cow committee appointees
is William Hueston, who was paid by the beef industry to testify
against Oprah Winfrey in hopes of convicting her of beef
"disparagement"?[96] After a similar conflict of interest unfolded in
Britain, their entire Ministry of Agriculture was dissolved and an
independent Food Safety Agency was created, whose sole responsibility
is to protect the public's health. Until we learn from Britain's
lesson, and until the USDA stops treating this as a PR problem to be
managed instead of a serious global threat,[97] millions of Americans
will remain at risk.

PLEASE FEEL FREE TO FORWARD OR REPRINT ANY PART OF THIS REPORT.

For updates on this evolving crisis, visit
http://www.organicconsumers.org/madcow.htm or send a blank email to
mailto:DrGregerMadCowUpdates-subscribe@lists.riseup.net

For background on this important issue, read the excellent book Mad
Cow U.S.A., the full text of which is available free online at
http://www.prwatch.org, or my article "U.S. Violates WHO Guidelines
for Mad Cow Disease" at
http://organicconsumers.org/madcow/GregerBSE.cfm

Michael Greger, M.D., has been the Chief BSE Investigator for Farm
Sanctuary since 1993 and the Mad Cow Coordinator for the Organic
Consumers Association since 2001. Dr. Greger has debated the National
Cattlemen's Beef Association before the FDA and was invited as an
expert witness at the infamous Oprah Winfrey "meat defamation" trial.
He has contributed to many books and articles on the subject,
continues to lecture extensively, and currently runs the Mad Cow
disease website http://www.organicconsumers.org/madcow.htm. Dr.
Greger is a graduate of the Cornell University School of Agriculture
and the Tufts University School of Medicine. He can be reached for
media inquiries at (206) 312-8640 or mhg1@cornell.edu.

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--
Michael Greger, M.D.
Chief BSE Investigator for Farm Sanctuary
http://www.nodowners.org
Mad Cow Coordinator for the Organic Consumers Association
http://www.organicconsumers.org/madcow.htm

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