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From: TSS (
Subject: Tracking Spongiform Encephalopathies in North America (Lancet Infectious Disease Volume 3, Number 8 01 August 2003)
Date: August 14, 2003 at 6:56 pm PST

In Reply to: Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. [FULL TEXT] posted by TSS on March 28, 2003 at 9:32 am:


Volume 3, Number 8 01 August 2003


Tracking spongiform encephalopathies in North America

Xavier Bosch

My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost
my mom to hvCJD (Heidenhain variant CJD) and have been searching for
answers ever since. What I have found is that we have not been told the
truth. CWD in deer and elk is a small portion of a much bigger problem.

49-year-old Singeltary is one of a number of people who have remained
largely unsatisfied after being told that a close relative died from a
rapidly progressive dementia compatible with spontaneous
Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of
documents on transmissible spongiform encephalopathies (TSE) and
realised that if Britons could get variant CJD from bovine spongiform
encephalopathy (BSE), Americans might get a similar disorder from
chronic wasting disease (CWD)the relative of mad cow disease seen among
deer and elk in the USA. Although his feverish search did not lead him
to the smoking gun linking CWD to a similar disease in North American
people, it did uncover a largely disappointing situation.

Singeltary was greatly demoralised at the few attempts to monitor the
occurrence of CJD and CWD in the USA. Only a few states have made CJD
reportable. Human and animal TSEs should be reportable nationwide and
internationally, he complained in a letter to the Journal of the
American Medical Association (JAMA 2003; 285: 733). I hope that the CDC
does not continue to expect us to still believe that the 85% plus of all
CJD cases which are sporadic are all spontaneous, without route or source.

Until recently, CWD was thought to be confined to the wild in a small
region in Colorado. But since early 2002, it has been reported in other
areas, including Wisconsin, South Dakota, and the Canadian province of
Saskatchewan. Indeed, the occurrence of CWD in states that were not
endemic previously increased concern about a widespread outbreak and
possible transmission to people and cattle.

To date, experimental studies have proven that the CWD agent can be
transmitted to cattle by intracerebral inoculation and that it can cross
the mucous membranes of the digestive tract to initiate infection in
lymphoid tissue before invasion of the central nervous system. Yet the
plausibility of CWD spreading to people has remained elusive.

Part of the problem seems to stem from the US surveillance system. CJD
is only reported in those areas known to be endemic foci of CWD.
Moreover, US authorities have been criticised for not having performed
enough prionic tests in farm deer and elk.

Although in November last year the US Food and Drug Administration
issued a directive to state public-health and agriculture officials
prohibiting material from CWD-positive animals from being used as an
ingredient in feed for any animal species, epidemiological control and
research in the USA has been quite different from the situation in the
UK and Europe regarding BSE.

Getting data on TSEs in the USA from the government is like pulling
teeth, Singeltary argues. You get it when they want you to have it,
and only what they want you to have.

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


>>>he complained in a letter to the Journal of the American Medical
Association (JAMA 2003; 285: 733). I hope that the CDC does not
continue to expect us to still believe that the 85% plus of all CJD
cases which are sporadic are all spontaneous, without route or source.<<<

actually, that quote was from a more recent article in the Journal of
Neurology (see below), not the JAMA article...TSS

Full Text
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734.

Re: 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?

PLEASE note;

''Answering critics like Terry Singeltary, who feels that the US
undercounts CJD, Schonberger _conceded_ that the current surveillance
system has errors but stated that most of the errors will be confined to
the older population''...

The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly
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Philip Yam The Pathological Protein Mad Cow, Chronic Wasting, and Other
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Asante/Collinge et al, 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;

File Format: PDF/Adobe Acrobat -
Page 1. J Freas, William From: Sent: To: Subject: Terry S. Singeltary
Sr. [] Monday, January 08,200l 3:03 PM freas ...





AND what will CJD from CWD infected deer and elk, OR
from cattle infected from a TSE that may be from CWD infected
deer and elk or any of the many strains of scrapie infected sheep in
the USA, what will this phenotype of TSE look like?

cwd apparently not having any distinctive signature in the experimental
cross-species transmissions. that is, unlike bse with its distinctive
florid plaques and strain-typing signature, cwd in humans at this point
is not readily distinguishable from sporadic cjd.

further, there is currently no experimental basis for the assumption that
met/met humans at codon 129 of the prion protein will be more susceptible
to cwd oral (or inadvertent surgical) transmission than met/val or val/val.
elk may have a leucine at this position, a total wild card.

the species barrier is very near unpredictable. surprisingly, it can be
greater in phylogenetically closer species such as mouse/hamster than it is

thus we are all perplexed by CDC and what scientific basis they used to
rule out cwd in the hunter cluster. but that cluster does not really speak
in any event to the status of the 100k or more people likely to have been
exposed to cwd venison, they may be infected, infectious, and pre-clinical
by many years. something to ponder?

AS implied in the Inset 25 we must not _ASSUME_ that
transmission of BSE to other species will invariably
present pathology typical of a scrapie-like disease.


CJD screening may miss thousands of cases

By Steve Mitchell
UPI Medical Correspondent
Published 7/22/2003 10:35 A

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

TO BE SO RELIABLE (per CDC et al);



Localised areas with high incidence of CJD have been described in England,
Hungary, Czechoslovakia, ''U.S.A.'', and Italy, but in the absence of a
comparison with the national incidence, these ''CLUSTERS'' may well have
been discovered by chance...[hense, the reason USA still refuses to have _NO_
CJD Questionnaire...TSS]


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...



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