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From: TSS (
Subject: TSE REPORT USA September 14, 2004
Date: September 14, 2004 at 12:47 pm PST

-------- Original Message --------
Subject: TSE REPORT USA September 14, 2004
Date: Tue, 14 Sep 2004 14:54:52 -0500
From: "Terry S. Singeltary Sr."
To: Bovine Spongiform Encephalopathy

Prepared by
National Center for Animal Health Programs
Eradication and Surveillance Team
July 31, 2004



AS of July 31, 2004, there were 71 scrapie infected and source flocks
(figure 3). There were 4 new infected and source flocks reported in July
(figure 4) with a total of 90 flocks reported for FY 2004 (figure 5).
The total infected and source flocks that have been released in FY 2004
are 62 (figure 6), with 15 flocks released in July. The ratio of
infected and source flocks released to newly infected and source flocks
for FY 2004 = 0.69 : 1.
BEEN CONFIRMED and reported by the National Veterinary Services
Laboratories (NVSL) in FY 2004, OF WHICH 42 WERE RSSS CASES (figure 7).
This includes 60 NEWLY CONFIRMED CASES IN JULY 2004 (figure 8). THIRTEEN
cases of scrapie in GOATS have been reported since 1990 (figure 9). ONE
NEW GOAT CASE WAS REPORTED IN FY 2004. New infected flocks, source
flocks, and flocks released or put on clean-up plans for FY 2004 are
depicted in figure 10...


Emergency Programs


Imported Belgium/Netherlands Sheep Test Results

APRIL 2002


The Western-blot test however cannot differentiate
between scrapie and BSE. The only known validated
method to differentiate between these two diseases
requires a series of mouse bioassay systems, which
take at least 23 years for completion.


-------- Original Message --------
Subject: Re: AW: [BSE-L] USDA did not test possible mad cows - Dr.
Detwiler, what about those sheep?
Date: Sun, 13 Jun 2004 11:27:24 -0500
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy

Greetings list members,

Thought I should let the list know that Dr. Detwiler kindly replied to
my question about the delayed 'atypical' TSE testing in the Vermont
sheep and tried to explain what caused the delay. If I interpreted it
correctly, seems it was the fault of the U.K. ;

-------- Original Message --------

Subject: Sheep Date: Sat, 12 Jun 2004 14:26:04 EDT



Mr. Singeltary. I hope this finds you well. As you are aware I left the
USDA last year. I can only update you on the sheep before that time.
Contact was established with the UK on doing the bioassay studies. They
agreed. However, we were prioritized after their own needs, hence the
delay. I am aware that there are now additional labs in Europe running
the mouse bioassay strain typing. You will have to contact USDA for
further word.

Linda Detwiler
My reply to Dr. Detwiler;

-------- Original Message --------

Subject: Re: Sheep
Date: Sat, 12 Jun 2004 13:53:57 -0500
From: "Terry S. Singeltary Sr."
References: <>

hello Dr. Detwiler, thanks for your kind reply.

> However, we were prioritized after their own needs, hence the delay.
not sure i understand that?
> You will have to contact USDA for further word. already done that,
and there answer was;
> >Dear Mr. Singeltary,
> >The Western blot tests on these animals were completed in April of
this >year. That means that we can begin the mouse inoculations. To get
the >results of the Western blot tests, you will need to submit a
Freedom of >Information Act request through our FOIA office. The FAX
number there is
> >Have a nice day,
> >Jim Rogers
> and with my previous attempts for information via the FOIA through
this administration (as you are probably very well aware of) they have
all been ignored/refused. so any further attempts would be fruitless i
am sure. thanks anyway... kindest regards, Terry wrote:
> Mr. Singeltary. snip... TSS



Office Note


A The Present Position with respect to Scrapie
A] The Problem

Scrapie is a natural disease of sheep and goats. It is a slow
and inexorably progressive degenerative disorder of the nervous system
and it ia fatal. It is enzootic in the United Kingdom but not in all

The field problem has been reviewed by a MAFF working group
(ARC 35/77). It is difficult to assess the incidence in Britain for
a variety of reasons but the disease causes serious financial loss;
it is estimated that it cost Swaledale breeders alone $l.7 M during
the five years 1971-1975. A further inestimable loss arises from the
closure of certain export markets, in particular those of the United
States, to British sheep.

It is clear that scrapie in sheep is important commercially and
for that reason alone effective measures to control it should be
devised as quickly as possible.

Recently the question has again been brought up as to whether
scrapie is transmissible to man. This has followed reports that the
disease has been transmitted to primates. One particularly lurid
speculation (Gajdusek 1977) conjectures that the agents of scrapie,
kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of
mink are varieties of a single "virus". The U.S. Department of
Agriculture concluded that it could "no longer justify or permit
scrapie-blood line and scrapie-exposed sheep and goats to be processed
for human or animal food at slaughter or rendering plants" (ARC 84/77)"
The problem is emphasised by the finding that some strains of scrapie
produce lesions identical to the once which characterise the human

Whether true or not. the hypothesis that these agents might be
transmissible to man raises two considerations. First, the safety
of laboratory personnel requires prompt attention. Second, action
such as the "scorched meat" policy of USDA makes the solution of the
acrapie problem urgent if the sheep industry is not to suffer




April 2004

Volume 3, Number 8 01 August 2003
Tracking spongiform encephalopathies in North America
Xavier Bosch


Norman Foster, director of the Cognitive Disorders Clinic at the

University of Michigan (Ann Arbor, MI, USA), says that current

surveillance of prion disease in people in the USA is inadequate to

detect whether CWD is occurring in human beings; adding that, the

cases that we know about are reassuring, because they do not suggest the

appearance of a new variant of CJD in the USA or atypical features in

patients that might be exposed to CWD. However, until we establish a

system that identifies and analyses a high proportion of suspected prion

disease cases we will not know for sure. The USA should develop a

system modelled on that established in the UK, he points out.

Ali Samii, a neurologist at Seattle VA Medical Center who recently

reported the cases of three hunterstwo of whom were friendswho died

from pathologically confirmed CJD, says that at present there are

insufficient data to claim transmission of CWD into humans; adding that

[only] by asking [the questions of venison consumption and deer/elk

hunting] in every case can we collect suspect cases and look into the

plausibility of transmission further. Samii argues that by making both

doctors and hunters more aware of the possibility of prions spreading

through eating venison, doctors treating hunters with dementia can

consider a possible prion disease, and doctors treating CJD patients

will know to ask whether they ate venison.

CDC spokesman Ermias Belay says that the CDC will not be investigating

the [Samii] cases because there is no evidence that the men ate

CWD-infected meat. He notes that although the likelihood of CWD

jumping the species barrier to infect humans cannot be ruled out 100%

and that [we] cannot be 100% sure that CWD does not exist in humans&

the data seeking evidence of CWD transmission to humans have been very



From: "Belay, Ermias"
Cc: "Race, Richard (NIH)" ; ; "Belay,
Sent: Monday, September 30, 2002 9:22 AM

Dear Sir/Madam,
In the Archives of Neurology you quoted (the abstract of which was
attached to your email), we did not say CWD in humans will present like
variant CJD.

That assumption would be wrong. I encourage you to read the whole
article and call me if you have questions or need more clarification
(phone: 404-639-3091). Also, we do not claim that "no-one has ever been
infected with prion disease from eating venison." Our conclusion stating
that we found no strong evidence of CWD transmission to humans in the
article you quoted or in any other forum is limited to the patients we

Ermias Belay, M.D.
Centers for Disease Control and Prevention

> > -----Original Message-----
> > From:
> > Sent: Sunday, September 29, 2002 10:15 AM
> > To:;; ebb8@CDC.GOV


Chronic Wasting Disease and Potential Transmission to Humans

Ermias D. Belay,*Comments
Ryan A.
Maddox,* Elizabeth S. Williams, Michael W. Miller,! Pierluigi Gambetti,§
and Lawrence B. Schonberger*
*Centers for Disease Control and Prevention, Atlanta, Georgia, USA;
University of Wyoming, Laramie, Wyoming, USA; !Colorado Division of
Wildlife, Fort Collins, Colorado, USA; and §Case Western Reserve
University, Cleveland, Ohio, USA

Environmental Sources of Prion Transmission in Mule Deer

Michael W. Miller,*Comments
Elizabeth S.
Williams, N. Thompson Hobbs,! and Lisa L. Wolfe*
*Colorado Division of Wildlife, Fort Collins, Colorado, USA; University
of Wyoming, Laramie, Wyoming, USA; and !Colorado State University, Fort
Collins, Colorado, USA

Suggested citation for this article: Miller MW, Williams ES, Hobbs
NT, Wolfe LL. Environmental sources of prion transmission in mule
deer. Emerg Infect Dis [serial on the Internet]. 2004 Jun [date
cited]. Available from:


Transmission Studies

Mule deer transmissions of CWD were by intracerebral inoculation and
compared with natural cases resulted in a more rapidly
progressive clinical disease with repeated episodes of synocopy ending
in coma. One control animal became affected, it is believed through
contamination of inoculam (?saline). Further CWD transmissions were
carried out by Dick Marsh into ferret, mink and squirrel monkey.
Transmission occurred in all of these species with the shortest
incubation period in the ferret.


The occurrence of CWD must be viewed against the context of the
locations in which it occurred. It was an incidental and unwelcome
complication of the respective wildlife research programmes. Despite its
subsequent recognition as a new disease of cervids, therefore justifying
direct investigation, no specific research funding was forthcoming.
The USDA viewed it as a wildlife problem and consequently not their




1. Dr Clark lately of the Scrapie Research Unit, Mission Texas has
successfully transmitted ovine and caprine scrapie to cattle. The
experimental results have not been published but there are plans to do
this. This work was initiated in 1978. A summary of it is:-


Gerald Wells: Report of the Visit to USA, April-May 1989

Aguzzi warns of CWD danger

The TSE family of diseases also includes chronic wasting disease (CWD)
in deer, a condition that has spread in the US in recent years (Nature
416, 569; 2002). Speaking at the Days of Molecular Medicine conference
in La Jolla in March, prion expert Adriano Aguzzi issued a strong
warning against underestimating this form of TSE.

"For more than a decade, the US has by-and-large considered mad cows
to be an exquisitely European problem. The perceived need to protect
US citizens from this alien threat has even prompted the deferral of
blood donors from Europe," he said. "Yet the threat-from-within
posed by CWD needs careful consideration, since the evidence that CWD
is less dangerous to humans than BSE is less-than-complete. Aguzzi
went on to point out that CWD is arguably the most mysterious of all
prion diseases.

"Its horizontal spread among the wild population is exceedingly
efficient, and appears to have reached a prevalence unprecedented even
by BSE in the UK at its peak. The pathogenesis of CWD, therefore,
deserves a vigorous research effort. Europeans also need to think
about this problem, and it would be timely and appropriate to increase
CWD surveillance in Europe too." Aguzzi has secured funding from the
National Institutes of Health to investigate CWD, and the effort will
be lead by Christina Sigurdson in his department at the University of

This quote from Dr. Gambetti is especially significant since he is the
rather cautious TSE researcher under contract with the Centers for Disease
Control to examine the brains of individuals who have died of CJD.

Pierluigi Gambetti, director of the National Prion Disease Pathology
Surveillance Center at Case Western Reserve University in Cleveland,
said all deer should be tested for chronic wasting disease before any
processing is done.

"There is no way around it," he said. "Nobody should touch that meat
unless it has been tested."


Transmissible Mink Encephalopathy

Transmissible mink encephalopathy (TME) is a rare
illness that affects the central nervous system of
ranch-raised mink. It was first detected in the United
States in 1947. Since then, TME outbreaks have
been reported in numerous locations worldwide,
including the United States, Canada, Finland,
Germany, and the republics of the former Soviet


The first documented TME outbreak in the United
States occurred in 1947 on one ranch in Wisconsin
and then on a ranch in Minnesota that had received
mink from the Wisconsin ranch. In 1961, TME outbreaks
occurred on five ranches in Wisconsin. In
1963, outbreaks occurred in Idaho, Minnesota, and
Wisconsin. Epidemiologic data from the Minnesota
and Wisconsin outbreaks trace the cases in those
States to one common purchased food source.
The 1985 Stetsonville Outbreak
The most recent TME outbreak occurred on one
mink ranch in Stetsonville, WI, in 1985. In the herd of
7,300 adult mink, 60 percent of the animals died.
Clinical signs included tail arching, incoordination, and
hyperexcitability. At the most advanced stages of the
disease, the animals were in trancelike states and
eventually died.
The outbreak lasted 5 months. Microscopic
examination of sections of the brain confirmed the
spongelike changes characteristic of TME. Diagnostic
tests identified the prion protein. The following year,
mink born during the outbreak showed no signs of
The late Richard Marsh, a veterinary virologist at
the University of Wisconsin who studied the transmission
of TME and other TSEs, investigated this outbreak.
Marsh learned that the mink were fed a diet
composed of fresh meat products from downer cattle
and commercial sources of fish, poultry, and cereal.
Downer cattle are nonambulatory and cannot rise
because they are affected with a condition such as a
metabolic disease, broken limbs, or a central nervous
system disorder. Marsh theorized that the meat from
these downer cattle introduced a TSE agent to the
mink in which TME resulted.
Although Marshs hypothesis is based on speculation
and anecdotal evidence, in 1993 APHIS adjusted
its national BSE surveillance program to include testing
downer cattle for evidence of a TSE. The brains of
more than 20,141 cattle have been examined at
APHIS National Veterinary Services Laboratories and
other State diagnostic laboratories. Not a single tissue
sample has revealed evidence of BSE or another
TSE in cattle...



No mad cow results for nearly 500 cows

By Steve Mitchell
United Press International
Published 8/11/2004 11:23 AM

WASHINGTON, Aug. 11 (UPI) -- The U.S. Department of Agriculture failed
to test for mad cow disease or collect the correct portion of the brain
on nearly 500 suspect cows over the past two years -- including some in
categories considered most likely to be infected -- according to agency
records obtained by United Press International.

The testing problems mean it may never be known with certainty whether
these animals were infected with the deadly disease. Department
officials said these animals were not included in the agency's final
tally of mad cow tests, but the records, obtained by UPI under the
Freedom of Information Act, indicate at least some of them were counted...



Steve Mitchell is UPI's Medical Correspondent. E-mail
Copyright © 2001-2004 United Press International

July 13, 2004

IG Audit Finds Multiple Flaws in Mad Cow Surveillance Plan
Rep. Waxman raises questions about the effectiveness and credibility of
USDA's response to mad cow disease, citing an audit by the USDA
Inspector General that finds systemic deficiencies in the Department's
surveillance plan and new evidence that USDA misled the public in the
wake of the detection of an infected cow in Washington State.

- Letter to USDA

IG Draft Audit

May 13, 2004

Failure To Test Staggering Cow May Reflect Wider Problems
Rep. Waxman raises concerns that the recent failure of USDA to test an
impaired cow for BSE may not be an isolated incident, citing the failure
of USDA to monitor whether cows condemned for central nervous system
symptoms are actually tested for mad cow disease.

- Letter to USDA

EFSA Scientific Report on the Assessment of the Geographical BSE-Risk
(GBR) of the United States of America (USA)

Adopted July 2004 (Question N° EFSA-Q-2003-083)

[20 August 2004]

From: Terry S. Singeltary Sr. []
Sent: Tuesday, July 29, 2003 1:03 PM
Cc:;; BSE-L
Subject: Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION
TO DOCKET 2003N-0312]

Greetings FDA,


PLUS, if the USA continues to flagrantly ignore the _documented_ science
to date about the known TSEs in the USA (let alone the undocumented TSEs
in cattle), it is my opinion, every other Country that is dealing with
BSE/TSE should boycott the USA and demand that the SSC reclassify the
USA BSE GBR II risk assessment to BSE/TSE GBR III 'IMMEDIATELY'. for the
SSC to _flounder_ any longer on this issue, should also be regarded with
great suspicion as well. NOT to leave out the OIE and it's terribly
flawed system of disease surveillance. the OIE should make a move on CWD
in the USA, and make a risk assessment on this as a threat to human
health. the OIE should also change the mathematical formula for testing
of disease. this (in my opinion and others) is terribly flawed as well.
to think that a sample survey of 400 or so cattle in a population of 100
million, to think this will find anything, especially after seeing how
many TSE tests it took Italy and other Countries to find 1 case of BSE
(1 million rapid TSE test in less than 2 years, to find 102 BSE cases),
should be proof enough to make drastic changes of this system. the OIE
criteria for BSE Country classification and it's interpretation is very
problematic. a text that is suppose to give guidelines, but is not
understandable, cannot be considered satisfactory. the OIE told me 2
years ago that they were concerned with CWD, but said any changes might
take years. well, two years have come and gone, and no change in
relations with CWD as a human health risk. if we wait for politics and
science to finally make this connection, we very well may die before any
decisions or changes are made. this is not acceptable. we must take the
and the industry out of any final decisions of the Scientific community.
this has been the problem from day one with this environmental man made
death sentence. some of you may think i am exaggerating, but you only
have to see it once, you only have to watch a loved one die from this
one time, and you will never forget, OR forgive...yes, i am still very
angry... but the transmission studies DO NOT lie, only the politicians
and the industry do... and they are still lying to this day...TSS

EFSA Scientific Report on the Assessment of the Geographical BSE-Risk
(GBR) III of the United States of America (USA)

Adopted July 2004 (Question N° EFSA-Q-2003-083)

[20 August 2004]


Dave Louthan - Killed the Mad Cow

Stanley Prusiner

''nobody has ever ask us to comment''

''they don't want us to comment''

''they never ask''

i tried to see Venemon, after Candian cow was discovered with BSE.
went to see lyle. after talking with him... absolute ignorance... then
thought i should see Venemon... it was clear his entire policy was to
get cattle boneless beef prods across the border... nothing else
mattered... his aids confirmed this... 5 times i tried to see Venemon,
never worked... eventually met with carl rove the political... he is the
one that arranged meeting with Venemon... just trying to give you a sense
of the distance... healh public safety... was never contacted... yes i
believe that prions are bad to eat and you can die from them...END

Dr. Stan bashing Ann Veneman - 3 minutes

On September 13, 2004, no positive or inconclusive test results were

Weekly Summary

Cumulative Total from June 1, 2004: 57,064

Research Project: Bovine Spongiform Encephalopathy and Other
Transmissible Spongiform Encephalopathies


Virus and Prion Diseases of Livestock

Title: Experimental Inoculation of Tme, Scrapie, and Cwd to Raccoons
(Procyon Lotor) and the Utilization of Raccoons for Strain-Typing of
Unknown Tses in the United States

item Hamir, Amirali

item Miller, Janice
item Cutlip, Randall

item Stack, M - VSA, WEYBRIDGE, UK
item Chaplin, M - VSA, WEYBRIDGE, UK
item Jenny, A - USDA, APHIS
item Williams, E - UNIVERSITY OF

Submitted to: American Association Of Veterinary Laboratory Diagnosticians
Publication Acceptance Date: April 29, 2002
Publication Date: October 17, 2002
Abstract only
Technical Abstract: Raccoons (Procyon lotor) are omnivorous and their
diet may include carrion. It is, therefore, possible that in the wild
they may get exposed to carcasses of animals with transmissible
spongiform encephalopathies (TSEs). To determine the susceptibility of
raccoons to transmissible mink encephalopathy (TME), scrapie, and
chronic wasting disease (CWD), each of these agents was inoculated
intracerebrally into a group of 4 kits. Three uninoculated kits served
as controls. All raccoons in the TME-inoculated group developed
neurologic signs and were euthanized within 6 months post inoculation
(PI). In the scrapie-inoculated group, 3 animals became sick and were
euthanized between 18 and 22 PI. Although the fourth raccoon in this
group did not show any clinical signs, it was euthanized at 24 months
PI. At present, 3 years PI, all CWD-infected raccoons are alive and
apparently healthy. At necropsy all clinically affected raccoons had
extensive microscopic lesions of spongiform encephalopathy and
protease-resistant prion protein (PrPres) was detected in the CNS by
immunohistochemistry and Western blot. These preliminary findings
demonstrate that TME and scrapie can be transmitted to raccoons within 6
months and 2 years, respectively, whereas CWD cannot. Based on these
incubation periods, it may be possible to differentiate these 3 TSEs.
Such a laboratory model would be relatively simple, fast and inexpensive
for strain-typing of unknown TSEs in the United States.




Answering critics like Terry Singeltary, who feels that the U.S. under-
counts CJD, Schonberger conceded that the current surveillance system
has errors but stated that most of the errors will be confined to the older


CJD screening may miss thousands of cases

By Steve Mitchell
UPI Medical Correspondent
Published 7/21/2003 3:00 PM
View printer-friendly version

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

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, a vegetarian advocacy Web site,
told UPI.

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

Copyright © 2001-2003 United Press International



One reason for this was the _inaccuracy_ in coding of cases correctly
certified as CJD Coding is carried out by staff who are not medically
qualified and it is not surprising that coding errors occur in the
processing of large numbers of certificates. In 1982, 12,000
certificates per week were processed at the office of population
censuses and surveys bu 15 coders and 6 checkers (Alderson et al.,
1983). The occurrence of both inter- and intra-observer coding errors
has been described (Curb et al., 1983) and the _inaccuracies_ of BOTH
certification and coding discovered in this study _support_ the
introduction of a more accurate system of death certificates and a more
detailed and specific coding system...


RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States

Email Terry S. Singeltary:

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to

comment on the CDC's attempts to monitor the occurrence of emerging

forms of CJD. Asante, Collinge et al [1] have reported that BSE

transmission to the 129-methionine genotype can lead to an alternate

phenotype that is indistinguishable from type 2 PrPSc, the commonest

sporadic CJD. However, CJD and all human TSEs are not reportable

nationally. CJD and all human TSEs must be made reportable in every

state and internationally. I hope that the CDC does not continue to

expect us to still believe that the 85%+ of all CJD cases which are

sporadic are all spontaneous, without route/source. We have many TSEs in

the USA in both animal and man. CWD in deer/elk is spreading rapidly and

CWD does transmit to mink, ferret, cattle, and squirrel monkey by

intracerebral inoculation. With the known incubation periods in other

TSEs, oral transmission studies of CWD may take much longer. Every

victim/family of CJD/TSEs should be asked about route and source of this

agent. To prolong this will only spread the agent and needlessly expose

others. In light of the findings of Asante and Collinge et al, there

should be drastic measures to safeguard the medical and surgical arena

from sporadic CJDs and all human TSEs. I only ponder how many sporadic

CJDs in the USA are type 2 PrPSc?


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