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.  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/Greger BSE.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. [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," , when no one wanted to deal with the crisis."[88] says protein chemist Shu Chen, who directs the Center's lab.

    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 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 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 ew 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.

REFERENCES: http://www.organicconsumers.org/madcow/GregerCJDkills.cfm