: Hundreds of thousands may be dying of undiagnosed `Mad Cow' disease in US

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> United Press International: CJD screening may miss thousands of cases

> CJD screening may miss thousands of cases

>

> By Steve Mitchell

> UPI Medical Correspondent

> Published 7/22/2003 10:35 AM

> http://www.upi.com/print.cfm?StoryID=20030721-102924-4786r

>

> WASHINGTON, July 21 (UPI) -- The federal government's monitoring system

for

> cases of Creutzfeldt-Jakob disease, a fatal human brain illness, could be

> missing tens of thousands of victims, scientists and consumer advocates

have

> told United Press International.

> Creutzfeldt-Jakob disease or CJD can be caused by eating beef contaminated

> with mad cow disease, but the critics assert without a better tracking

> system it might be impossible to determine whether any CJD cases are due

to

> mad cow or obtain an accurate picture of the prevalence of the disorder in

> the United States.

> Beginning in the late 1990s, more than 100 people contracted CJD in the

> United Kingdom and several European countries after eating beef infected

> with bovine spongiform encephalopathy -- the clinical name for mad cow

> disease.

> No case of mad cow has ever been detected in U.S. cattle and the Centers

for

> Disease Control and Prevention's monitoring system has never detected a

case

> of CJD due to eating contaminated American beef. Nevertheless, critics

say,

> the CDC's system misses many cases of the disease, which currently is

> untreatable and is always fatal.

> The first symptoms of CJD typically include memory loss and difficulty

> keeping balance and walking. As the disease destroys the brain, patients

> rapidly progress in a matter of months to difficulty with movement, an

> inability to talk and swallow and, finally, death.

> Spontaneously-occurring or sporadic CJD is a rare disorder. Only about 300

> cases appear nationwide each year, but several studies have suggested the

> disorder might be more common than thought and as many as tens of

thousands

> of cases might be going unrecognized.

> Clusters of CJD have been reported in various areas of the United

States --

> Pennsylvania in 1993, Florida in 1994, Oregon in 1996, New York in

1999-2000

> and Texas in 1996. In addition, several people in New Jersey developed CJD

> in recent years, including a 56 year old woman who died on May 31, 2003.

> Although in some instances, a mad cow link was suspected, all of the cases

> ultimately were classified as sporadic.

> People who develop CJD from eating mad-cow-contaminated beef have been

> thought to develop a specific form of the disorder called variant CJD. But

> new research, released last December, indicates the mad cow pathogen can

> cause both sporadic CJD and the variant form.

> " 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), " said Laura Manuelidis, section chief of surgery in the

> neuropathology department at Yale University, who conducted a 1989 study

> that found 13 percent of Alzheimer's patients actually had CJD.

> Several studies, including Manuelidis', have found that autopsies reveal 3

> percent to 13 percent of patients diagnosed with Alzheimer's or dementia

> actually suffered from CJD. Those numbers might sound low, but there are 4

> million Alzheimer's cases and hundreds of thousands of dementia cases in

the

> United States. A small percentage of those cases could add up to 120,000

or

> more CJD victims going undetected and not included in official statistics.

> Experiences in England and Switzerland -- two countries that discovered

mad

> cow disease in their cattle -- have heightened concerns about the

> possibility some cases of sporadic CJD are due to consuming

mad-cow-tainted

> beef. Both countries have reported increases in sporadic CJD since mad cow

> was first detected in British herds in 1986.

> Switzerland discovered last year its CJD rate was twice that of any other

> country in the world. Switzerland had been seeing about eight to 11 cases

> per year from 1997 to 2000. Then the incidence more than doubled, to 19

> cases in 2001 and 18 cases in 2002.

> The CDC says the annual rate of CJD in the United States is one case per

> million people, but the above studies suggest the true prevalence of CJD

is

> not known, Manuelidis told UPI.

> Diagnosing CJD or Alzheimer's is difficult because no test exists that can

> identify either disease in a living patient with certainty. So physicians

> must rely on the patient's symptoms to determine which illness might be

> present. Sometimes, however, the symptoms of one disease can appear

similar

> to the other disorder. The only way to determine the disease conclusively

is

> to perform an autopsy on the brain after death.

> Unfortunately, although autopsies once were performed on approximately

half

> of all corpses, the frequency has dropped to 15 percent or less in the

> United States. The National Center for Health Statistics -- a branch of

the

> CDC -- stopped collecting autopsy data in 1995.

> " If we don't do autopsies and we don't look at people's brains ... we have

> no idea about what is the general prevalence of these kinds of infections

> and (whether) it is changing, " Manuelidis said.

> At the same time autopsies have been declining, the number of deaths

> attributed to Alzheimer's has increased more than 50-fold since 1979,

going

> from 857 deaths then to nearly 50,000 in 2000. Though it is unlikely the

> dramatic increase in Alzheimer's is due entirely to misdiagnosed CJD

cases,

> it " could explain some of the increase we've seen, " Manuelidis said.

> " Neurodegenerative disease and Alzheimer's disease have become a

> wastebasket " for mental illness in the elderly that is difficult to

diagnose

> conclusively, she said. " In other words, what people call Alzheimer's now

is

> more broad than what people used to call it, and that has the possibility

of

> encompassing more diseases -- including CJD. "

> The autopsy studies that found undiagnosed CJD cases raise the question of

> whether the United States " already has an undetected epidemic here, " Jeff

> Nelson, director of vegsource.com, a vegetarian advocacy Web site, told

UPI.

> " What's the source of that? " Nelson asked. " Could it be the same source of

> encephalopathy we saw in minks? "

> Nelson referred to an outbreak of a mad-cow-type disorder in minks in

> Wisconsin in the 1980s. The origin was traced back to the animals' diet,

> which included parts of so-called downer cattle -- sick cows that are

unable

> to stand, which often indicates a neurological disease, including mad cow.

> The mink disease raised concerns about whether U.S. cattle were carrying a

> mad-cow-like pathogen even prior to the U.K. epidemic that began in 1986.

> Andrew Monjan, chief of the neuropsychology of aging program at the

National

> Institute of Aging -- part of the National Institutes of Health in

Bethesda,

> Md. -- acknowledged there has been an increase in U.S. Alzheimer's cases.

> However, he told UPI, this probably is due to the aging of the

population --

> as people grow older, they develop a higher risk of developing

Alzheimer's.

> " There's been no change in the number of CJD cases in the country and

there

> has been clearly a tracking of the unusual cases of CJD " that could be due

> to mad cow disease, Monjan said. However, Terry Singletary, coordinator of

> CJD Watch -- an organization founded to track CJD cases -- says efforts to

> track the disease have been close to nonexistent. For example, only 12

> states require such reports. Therefore, many cases might be going

> undetected, unreported or misdiagnosed.

> If more states made CJD a reportable illness, there would be more clusters

> detected across the United States, said Singletary, who became involved

with

> CJD advocacy after his mother died from a form of CJD known as Heidenhain

> variant. In the 18-year period between 1979 and 1996, he noted, the

country

> saw a jump from one case of sporadic CJD in people under the age of 30 --

a

> warning sign for a link to mad cow because nearly all of the U.K. victims

> were 30 years of age or younger -- to five cases in five years between

1997

> and 2001. " That represents a substantial blip, " he told UPI.

> Singletary also said there have been increases in sporadic CJD in France,

> Germany and Italy, all of which have detected mad cow disease in their

> cattle.

> So far, the CDC has refused to impose a national requirement that

physicians

> and hospitals report cases of the disease. The agency has not chosen to

make

> CJD a reportable disease because " making it reportable is not necessarily

> directly helpful in surveillance because in some states where it's

> reportable you may not get the physician to report it, " said Dr. Ermias

> Belay, CDC's medical epidemiologist working on CJD.

> Instead, the agency relies on other methods, including death certificates

> and urging physicians to send suspicious cases to the National Prion

Disease

> Pathology Surveillance Center at Case Western Reserve University in

> Cleveland, which is funded by the CDC. However, because autopsies

generally

> are not done, if a CJD case is misdiagnosed as Alzheimer's or dementia, a

> correct diagnosis might never be determined and therefore the cause of

death

> listed on a death certificate might be inaccurate.

> Belay told UPI he discounted this possibility. It is unlikely to happen,

he

> said, because it is easy to distinguish CJD from Alzheimer's -- the two

> conditions display different symptoms.

> Manuelidis disagreed. It can be quite difficult to determine accurately if

a

> patient has CJD, as evidenced by her study, in which respected and

competent

> neurologists and psychiatrists at Yale originally diagnosed patients with

> Alzheimer's, yet were wrong at least 13 percent of the time. Another study

> conducted at the University of Pennsylvania, which found 6 percent of

> dementia patients actually were suffering from CJD, supports the

difficulty

> in distinguishing the illnesses correctly.

> The U. Penn. researchers concluded: " These results show that in patients

> with a clinical diagnosis of dementia, the etiology (cause) cannot be

> accurately predicted during life. "

> In addition, the NPDPSC sees less than half of all the CJD cases each

year,

> so the CDC's investigational system not only is missing many of the

> misdiagnosed CJD cases, it also is not conducting autopsies on most of the

> detected cases.

> Belay said the CDC follows up on all cases of CJD that occur in people

under

> age 55, as these could be linked to variant -- mad-cow-related -- CJD. But

> so far, all have turned out to be sporadic forms of the disease. About 30

> cases of the disorder occur each year in the United States in this age

> group, while the remaining 270 or so are older.

> The case of Carrie Mahan -- a Philadelphia woman who developed a brain

> disorder that appeared to be CJD and died from it in 2000 at the age of

> 29 -- illustrates just how difficult it can be to diagnose the disease.

> Mahan's physician, Dr. Peter Crinos of the University of Pennsylvania

> Medical Center, ruled out other disorders and felt certain the young woman

> had died of CJD, a concern that raised the possibility of a link to mad

cow

> disease because of her young age. When neuropathologist Nicholas Gonatas,

> who had seen CJD before, examined Mahan's brain after her death, he,

> likewise, was confident he detected the microscopic, sponge-like holes

> caused by the disease. But when he sent brain samples to the NPDPSC, the

> results came back negative. Gonatas, convinced the surveillance center's

> finding was erroneous, sent off two more samples, only to have them both

> come back negative.

> Subsequent research, however, has shown the test used by the surveillance

> center cannot rule out CJD, said Crinos, an assistant professor of

> neurology.

> " There's no question that Carrie had a spongiform encephalopathy, " Crinos

> said, but added although it appeared to be CJD, it is difficult if not

> impossible to say if it was due to mad cow disease.

> Crinos told UPI until the CDC implements a better tracking system, a lot

of

> questions will remain about CJD and cases like Carrie Mahan's. One central

> question: Why are cases of what is presumed to be a rare disease popping

up

> in clusters in certain areas of the country? Crinos said the clustering

> suggests an environmental or food-borne cause, but so far, " No one knows

the

> answer to that. "

>

> 2001-2003 United Press International

>

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