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From: TSS ()
Subject: Re: Fifth woman dies after being diagnosed with CJD
Date: August 14, 2005 at 9:35 am PST

In Reply to: Fifth woman dies after being diagnosed with CJD posted by TSS on August 14, 2005 at 6:59 am:

>>>Hahn, along with Chris Carter of the federal public health office in Idaho and Cheryle Becker, an epidemiologist with South Central District Health, are investigating the five cases -- looking through the women's medical records, documenting family histories and looking for anything these women might have had in common. Becker said they'll compare their findings with studies that have been done in other areas.

"We have our investigation under way right now and we are looking into information on all the cases we have," Becker said earlier in the week. <<<

OH boy, cant wait. same old, same old, BSe. another coincidence, no threat, sporadic CJD. i can almost read word for word what it will say;

"The evidence does not support the existence of an outbreak of CJD "

"CJD occurs at an overall rate of approximately one case per million people per year, but at a rate of 4.1 cases per million in individuals age 55 or older"

"There has been no documented increased trend in the annual reported number of sporadic CJD"


'chance happening'

'random occurance'

"We have no evidence of mad cow disease in Texas," said Michael Kelley,
M.D., chief of TDH's bureau of communicable
disease control.

June 16, 1997

TDH: No 'Mad Cow Disease' in Texas

The Texas Department of Health (TDH) today said media reports indicating
that Creutzfeldt-Jakob disease and "mad cow
disease" are the same illness and implying that five persons in
Northeast Texas may have died of mad cow disease are wrong
on both counts.

"We have no evidence of mad cow disease in Texas," said Michael Kelley,
M.D., chief of TDH's bureau of communicable
disease control. "These people died of Creutzfeldt-Jakob disease, or
CJD, not mad cow disease." He said the formal name
for mad cow disease is bovine spongiform encephalopathy, or BSE.

"It can get confusing," Kelley acknowledged, "because CJD in humans and
BSE in cattle are similar illnesses, but they
damage the brain in distinguishably different ways."

The confusion is exacerbated, Kelley said, because some scientists
believe that several British cases of CJD in humans since
1994 are actually a new variation of CJD and may be connected to animal
cases of BSE in British cattle. "There is no
evidence of BSE in U.S. cattle and no evidence of a new variation of CJD
in humans in the U.S.," Kelley said.

Scientists do not know how CJD, a rare brain disorder, is transmitted in
all cases but say some cases are hereditary and
others the result of transplants of nerve tissue. CJD causes holes to
form in the brain and is always fatal. TDH epidemiologist
Julie Rawlings said some 149 deaths from CJD have been reported in Texas
since 1984.


(For more information, contact: Doug McBride, TDH Public Information
Officer, Austin, 512-458-7111, Ext. 2214
or 512-458-7400.)


note just came in from Nuno and Rawlings,
(what a coincidence);

There is no update. There were the 7 cases reported during that 15
month period in Public Health Region 4. No evidence of any clusters

Statewide, there were 7 confirmed/probable cases in 98, 5
in 99 and 5 in 2000.


no mad cows in TEXAS??? r i g h t ;

May 4, 2004
Media Inquiries: 301-827-6242
Consumer Inquiries: 888-INFO-FDA

Statement on Texas Cow With Central Nervous System Symptoms


USDA Chief Veterinarian John Clifford

Regarding the Epidemiological Investigation into the recently confirmed BSE case

June 29, 2005

"DNA test results have confirmed that we have identified the source herd of the animal

determined last week to be positive for BSE. Based on information we have received from the

owner, the cow was born and raised in a herd in Texas and was approximately 12 years old....

The animal was selected for testing because, as a non-ambulatory animal, it was considered to be at higher risk for BSE. An initial screening test on the animal in November 2004 was inconclusive, triggering USDA to conduct the internationally accepted confirmatory IHC tests. Those test results were negative. Earlier this month, USDA's Office of the Inspector General recommended further testing of the seven-month-old sample using another internationally recognized confirmatory test, the Western blot. Unlike the IHC, the Western blot was reactive, prompting USDA to send samples from the animal to the Weybridge laboratory for further analysis.

The laboratory in Weybridge, England, is recognized by the World Animal Health Organization, or OIE, as a world reference laboratory for BSE. Weybridge officials this week conducted a combination of rapid, IHC and Western blot testing on tissue samples from the animal in question. At the same time these diagnostic tests were being run by Weybridge, USDA conducted its own additional tests.


This confirmed case of BSE in no way impacts the safety of our nation's food supply. As the epidemiological investigation progresses, USDA will continue to communicate findings in a timely and transparent manner.

Last Modified: 06/24/2005


Statement by Chief Veterinary Medical Officer John Clifford Animal and Plant Health Inspection Service Regarding Non-Definitive BSE Test Results
July 27, 2005


The sample was submitted to us by a private veterinarian. As an extension of our enhanced surveillance program, accredited private veterinarians, who often visit farms in remote areas, collect samples when warranted. The sample in question today was taken from a cow that was at least 12 years of age and experienced complications during calving. The veterinarian treated the sample with a preservative, which readies it for testing using the immunohistochemistry (IHC) test —an internationally recognized confirmatory test for BSE. Neither the rapid screening test nor the Western blot confirmatory test can be conducted on a sample that has been preserved.

(THE FONG EFFECT OR what i like to call 'The Fong Syndrome...TSS)

WHAT we have been feeding cattle in Texas over the years ;


January 30, 2001
Print Media:
Broadcast Media:
Consumer Inquiries:


Today the Food and Drug Administration announced the results of tests
taken on feed used at a Texas feedlot
that was suspected of containing meat and bone meal from other domestic
cattle -- a violation of FDA's 1997
prohibition on using ruminant material in feed for other ruminants.
Results indicate that a very low level of
prohibited material was found in the feed fed to cattle.

FDA has determined that each animal could have consumed, at most and in
total, five-and-one-half grams -
approximately a quarter ounce -- of prohibited material. These animals
weigh approximately 600 pounds.

It is important to note that the prohibited material was domestic in
origin (therefore not likely to contain infected
material because there is no evidence of BSE in U.S. cattle), fed at a
very low level, and fed only once. The
potential risk of BSE to such cattle is therefore exceedingly low, even
if the feed were contaminated.

According to Dr. Bernard Schwetz, FDA's Acting Principal Deputy
Commissioner, "The challenge to regulators
and industry is to keep this disease out of the United States. One
important defense is to prohibit the use of any
ruminant animal materials in feed for other ruminant animals. Combined
with other steps, like U.S. Department
of Agriculture's (USDA) ban on the importation of live ruminant animals
from affected countries, these steps
represent a series of protections, to keep American cattle free of BSE."

Despite this negligible risk, Purina Mills, Inc., is nonetheless
announcing that it is voluntarily purchasing all 1,222
of the animals held in Texas and mistakenly fed the animal feed
containing the prohibited material. Therefore,
meat from those animals will not enter the human food supply. FDA
believes any cattle that did not consume
feed containing the prohibited material are unaffected by this incident,
and should be handled in the beef supply
clearance process as usual.

FDA believes that Purina Mills has behaved responsibly by first
reporting the human error that resulted in the
misformulation of the animal feed supplement and then by working closely
with State and Federal authorities.

This episode indicates that the multi-layered safeguard system put into
place is essential for protecting the food
supply and that continued vigilance needs to be taken, by all concerned,
to ensure these rules are followed

FDA will continue working with USDA as well as State and local officials
to ensure that companies and
individuals comply with all laws and regulations designed to protect the
U.S. food supply.

From: TSS (
Date: January 27, 2005 at 7:03 am PST

Risk of oral infection with bovine spongiform encephalopathy agent in primates

Corinne Ida Lasmézas, Emmanuel Comoy, Stephen Hawkins, Christian Herzog, Franck Mouthon, Timm Konold, Frédéric Auvré, Evelyne Correia, Nathalie Lescoutra-Etchegaray, Nicole Salès, Gerald Wells, Paul Brown, Jean-Philippe Deslys
Summary The uncertain extent of human exposure to bovine spongiform encephalopathy (BSE)--which can lead to variant Creutzfeldt-Jakob disease (vCJD)--is compounded by incomplete knowledge about the efficiency of oral infection and the magnitude of any bovine-to-human biological barrier to transmission. We therefore investigated oral transmission of BSE to non-human primates. We gave two macaques a 5 g oral dose of brain homogenate from a BSE-infected cow. One macaque developed vCJD-like neurological disease 60 months after exposure, whereas the other remained free of disease at 76 months. On the basis of these findings and data from other studies, we made a preliminary estimate of the food exposure risk for man, which provides additional assurance that existing public health measures can prevent transmission of BSE to man.

Published online January 27, 2005

It is clear that the designing scientists must

also have shared Mr Bradley’s surprise at the results because all the dose

levels right down to 1 gram triggered infection.


6. It also appears to me that Mr Bradley’s answer (that it would take less than say 100

grams) was probably given with the benefit of hindsight; particularly if one

considers that later in the same answer Mr Bradley expresses his surprise that it

could take as little of 1 gram of brain to cause BSE by the oral route within the

same species. This information did not become available until the "attack rate"

experiment had been completed in 1995/96. This was a titration experiment

designed to ascertain the infective dose. A range of dosages was used to ensure

that the actual result was within both a lower and an upper limit within the study

and the designing scientists would not have expected all the dose levels to trigger

infection. The dose ranges chosen by the most informed scientists at that time

ranged from 1 gram to three times one hundred grams. It is clear that the designing

scientists must have also shared Mr Bradley’s surprise at the results because all the

dose levels right down to 1 gram triggered infection.

Re: BSE .1 GRAM LETHAL NEW STUDY SAYS via W.H.O. Dr Maura Ricketts

[BBC radio 4 FARM news]

2) Infectious dose:

To cattle: 1 gram of infected brain material (by oral ingestion)


Medical Sciences
Identification of a second bovine amyloidotic spongiform encephalopathy: Molecular similarities with sporadic Creutzfeldt-Jakob disease

Cristina Casalone *, Gianluigi Zanusso , Pierluigi Acutis *, Sergio Ferrari , Lorenzo Capucci , Fabrizio Tagliavini ¶, Salvatore Monaco ||, and Maria Caramelli *

*Centro di Referenza Nazionale per le Encefalopatie Animali, Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna, 148, 10195 Turin, Italy; Department of Neurological and Visual Science, Section of Clinical Neurology, Policlinico G.B. Rossi, Piazzale L.A. Scuro, 10, 37134 Verona, Italy; Istituto Zooprofilattico Sperimentale della Lombardia ed Emilia Romagna, Via Bianchi, 9, 25124 Brescia, Italy; and ¶Istituto Nazionale Neurologico "Carlo Besta," Via Celoria 11, 20133 Milan, Italy

Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved December 23, 2003 (received for review September 9, 2003)

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are mammalian neurodegenerative disorders characterized by a posttranslational conversion and brain accumulation of an insoluble, protease-resistant isoform (PrPSc) of the host-encoded cellular prion protein (PrPC). Human and animal TSE agents exist as different phenotypes that can be biochemically differentiated on the basis of the molecular mass of the protease-resistant PrPSc fragments and the degree of glycosylation. Epidemiological, molecular, and transmission studies strongly suggest that the single strain of agent responsible for bovine spongiform encephalopathy (BSE) has infected humans, causing variant Creutzfeldt-Jakob disease. The unprecedented biological properties of the BSE agent, which circumvents the so-called "species barrier" between cattle and humans and adapts to different mammalian species, has raised considerable concern for human health. To date, it is unknown whether more than one strain might be responsible for cattle TSE or whether the BSE agent undergoes phenotypic variation after natural transmission. Here we provide evidence of a second cattle TSE. The disorder was pathologically characterized by the presence of PrP-immunopositive amyloid plaques, as opposed to the lack of amyloid deposition in typical BSE cases, and by a different pattern of regional distribution and topology of brain PrPSc accumulation. In addition, Western blot analysis showed a PrPSc type with predominance of the low molecular mass glycoform and a protease-resistant fragment of lower molecular mass than BSE-PrPSc. Strikingly, the molecular signature of this previously undescribed bovine PrPSc was similar to that encountered in a distinct subtype of sporadic Creutzfeldt-Jakob disease.


C.C. and G.Z. contributed equally to this work.

||To whom correspondence should be addressed.

E-mail: .

Global Compliance of TSE regulations? & sCJD's, scrapie and feed mils in OHIO ?
Thu, 2 Aug 2001 14:10:25 -0700
"Terry S. Singeltary Sr."
Bovine Spongiform Encephalopathy

######## Bovine Spongiform Encephalopathy #########

Greetings All,

if you read # 8, answer # 2,

if one country refused (potential BSE risks for whatever reason) but
can import to another
Country that see no risk from same product,
how can global compliance take place ?

(maybe i misunderstood this)

and still don't understand how U.S. can
have BSE Free status with the 'passive'
surveillance that has only tested 13,916
cows in 13 years???

i don't mean to sound like broken record again,
but if one refuses to test in sufficient numbers, one can stay BSE free
for a long time.
much longer than ones that are testing to find.

when U.S. ruminant-to-ruminant feed ban is
still being violated to date.
most recent posted;

Bruce A. Burgett, General Manager
The Carrollton Farmers Exchange
204 Second Street, N.W.
Carrollton, OH 44615

JULY, 12, 2001

Dear Mr. Burgett:


Our inspection found your firm failed to label
feeds that contain, or may contain, prohibited
materials with the required cautionary statement
We suggest this statement be distinguished by different type size or
color or other means of
highlighting the statement so it is easily noticed by the purchaser.

There are no written procedures for cleaning out or flushing equipment
after mixing feeds containing prohibited material. Additionally, you do
not have records documenting that the system was cleaned or flushed in
accordance with any written procedures.

You should establish adequate procedures and verify that the
flush/clean-out method you use cleans out the remainder of preceding
batches containing prohibited materials. Note: If you flush with feed
ingredients, or sequence with non-ruminant feed, you must also label
these products with the required cautionary statement "Do not feed to
Cattle or Other Ruminants".

Your customer records are not sufficient to track the distribution of
products that contain, or may contain, prohibited material



other mad cow feed violations can be located at;

simply type in 'animal protein'

I cannot digest the fact the U.S. is
BSE/TSE free in U.S. cattle with the testing
to date and violations to date of feed ban.

another thing i have noticed, look at the number
of feed ban violations in the state of OHIO.
Ohio is the number one violator (reporting).

'then' look at the amount of SCRAPIE in that

*** SIS Status Report: 27-JUL-01 ***

then consider the cross contamination risks
posed in the 'warning letters' of violators
in feed mils?

now let's look at CJD stats;

CJD stats Ohio

(you must take into consideration not
everybody knows of this site, and not
everyone has computers, so there is
probably many more)

then look at Ohio's 'official' CJD statistics,
(there is none)...

Two diseases, Creutzfeldt-Jakob Disease (CJD) and cyclosporiasis, became
reportable in July, 1998, and will be included in the 1998 Annual

seems Ohio CJD reporting system has flaws,
0 cases of CJD reported in Ohio, latest
stats below;

Ohio feed ban violations 'highest documented',

Scrapie in the state of Ohio 'highest documented',

cross contamination in Ohio 'highest documented',

sCJD seems to be rising in Ohio and other States.

could there be a link to all this and the
sporadic CJD cases that are not documented
in the state of Ohio, especially since
the most recent findings of the potential
link between some strains of Scrapie and
some strains of sporadic CJDs.

Proc. Natl. Acad. Sci. USA, Vol. 98, Issue 7, 4055-4059, March 27, 2001

Medical Sciences
Ex vivo propagation of infectious sheep scrapie
agent in heterologous epithelial cells expressing
ovine prion protein

D. Vilette*,, O. Andreoletti, F. Archer*, M. F. Madelaine*, J. L.
S. Lehmann¶, and H. Laude*

Sheep consumption: a possible source of spongiform encephalopathy in

something to ponder...

thank you,
Terry S. Singeltary Sr., Bacliff, Texas USA


OH, and the fact that the transmission studies of USA SUFFOLK SHEEP SCRAPIE TO CATTLE IN THE USA done at Mission Texas DID NOT look like UK BSE, but something totally different, so why then would all USA CJD look like UK BSE nvCJD???

Appendix 3


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:-

Expt A
6 Her x Jer calves born in 1978 were inoculated as follows with
a 2nd Suffolk scrapie passage:-

i/c 1ml; i/m, 5ml; s/c 5ml; oral 30ml.
1/6 went down after 48 months with a scrapie/BSE-like disease.

Expt B
6 Her or Jer or HxJ calves were inoculated with angora Goat
virus 2/6 went down similarly after 36 months.

Expt C
Mice inoculated from brains of calves/cattle in expts A • B
were resistant, only 1/20 going down with scrapie and this was the
reason given for not publishing.

Diagnosis in A, B, C was by histopath. No reports on SAT were given.

2. Dr Warren Foote indicated success so far in eliminating scrapie in
offspring from experimentally- (and naturally) infected sheep by ET.
He had found difficulty in obtaining embryos from naturally infected
sheep (cf SPA).

3. Prof. A Robertson gave a brief account of BSE. The US approach was to
accord it a very low profile indeed. Dr A Thiermann showed the
picture in the "Independent" with cattle being incinerated and thought
this was a fanatical incident to be avoided in the US at all costs.
BSE was not reported in USA.

4. Scrapie incidents (ie affected flocks) have shown a dramatic increase
since 1978. In 1953 when the National Control Scheme was started
there were 10-14 incidents, in 1978 - 1 and in 1988 so far 60.

5. Scrapie agent was reported to have been isolated from a solitary

6. A western blotting diagnostic technique (? on PrP) shows some promise.

7. Results of a questionnaire sent to 33 states on the subject of the
national sheep scrapie programme survey indicated

17/33 wished to drop it

6/33 wished to develop it

8/33 had few sheep and were neutral

Information obtained from Dr Wrathall's notes of a meeting of the U.S.
Animal Health Association at Little Rock, Arkansas Nov. 1988.



>> Differences in tissue distribution could require new regulations
>> regarding specific risk material (SRM) removal.


full text 33 PAGES ;

It was, however, performed in the USA in 1979, when it was shown that cattle inoculated with the scrapie agent endemic in the flock of Suffolk sheep at the United States Department of Agriculture in Mission, Texas, developed a TSE quite unlike BSE. 32 The findings of the initial transmission, though not of the clinical or neurohistological examination, were communicated in October 1988 to Dr Watson, Director of the CVL, following a visit by Dr Wrathall, one of the project leaders in the Pathology Department of the CVL, to the United States Department of Agriculture. 33 The results were not published at this point, since the attempted transmission to mice from the experimental cow brain had been inconclusive. The results of the clinical and histological differences between scrapie-affected sheep and cattle were published in 1995. Similar studies in which cattle were inoculated intracerebrally with scrapie inocula derived from a number of scrapie-affected sheep of different breeds and from different States, were carried out at the US National Animal Disease Centre. 34 The results, published in 1994, showed that this source of scrapie agent, though pathogenic for cattle, did not produce the same clinical signs of brain lesions characteristic of BSE.

1: J Infect Dis. 1994 Apr;169(4):814-20.

Intracerebral transmission of scrapie to cattle.

Cutlip RC, Miller JM, Race RE, Jenny AL, Katz JB, Lehmkuhl HD, DeBey BM, Robinson MM.

USDA, Agriculture Research Service, National Animal Disease Center, Ames, IA 50010.

To determine if sheep scrapie agent(s) in the United States would induce a disease in cattle resembling bovine spongiform encephalopathy, 18 newborn calves were inoculated intracerebrally with a pooled suspension of brain from 9 sheep with scrapie. Half of the calves were euthanatized 1 year after inoculation. All calves kept longer than 1 year became severely lethargic and demonstrated clinical signs of motor neuron dysfunction that were manifest as progressive stiffness, posterior paresis, general weakness, and permanent recumbency. The incubation period was 14-18 months, and the clinical course was 1-5 months. The brain from each calf was examined for lesions and for protease-resistant prion protein. Lesions were subtle, but a disease-specific isoform of the prion protein was present in the brain of all calves. Neither signs nor lesions were characteristic of those for bovine spongiform encephalopathy.

MeSH Terms:
Cattle Diseases/etiology*
Cattle Diseases/pathology
Encephalopathy, Bovine Spongiform/etiology*
Encephalopathy, Bovine Spongiform/pathology
Motor Neurons/physiology
Sleep Stages
Time Factors


Intracerebral transmission of scrapie to cattle FULL TEXT PDF;



WE conclude that American sources of sheep scrapie are transmissible to cattle by direct intracerebral inoculation but the disease induced is NOT identical to BSE as seen in the United Kingdom. While there were similarities in clinical signs between this experimental disease and BSE, there was no evidence of aggressiveness, hyperexcitability, hyperesthesia (tactile or auditory), or hyperemetria of limbs as has been reported for BSE (9). Neither were there extensive neurologic lesions, which are primary for BSE, such as severe vacuolation of neurons and neuropil or neuronal necrosis and gliosis. Although some vacuolation of neuropil, chromotolysis in neurons, and gliosis were seen in the brains of some affected calves, these were industinguishable from those of controls. Vacuolated neurons in the red nucleus of both challenged and normal calves were considered normal for the bovines as previously described (50).

PrP-res was found in ALL CHALLENGED CALVES REGARDLESS OF CLINCIAL SIGNS, and the amount of PrP-res positively related to the length of the incubation. ...


WE also conclude from these studies that scrapie in cattle MIGHT NOT BE RECOGNIZED BY ROUTINE HISTOPATHOLOGICAL EXAMINATION OF THE BRAIN AND SUGGEST THAT DETECTION OF PrP-res by immunohistochemistry or immunoblotting is necessary to make a definitive diagnosis. THUS, undiagnosed scrapie infection could contribute to the ''DOWNER-COW'' syndrome and could be responsible for some outbreaks of transmissible mink encephalopathy proposed by Burger and Hartsough (8) and Marsh and harsough (52). ...


Multiple sources of sheep affected with scrapie and two breeds of cattle from several sources were used inthe current study in an effort to avoid a single strain of either agent or host. Preliminary results from mouse inoculations indicate multiple strains of the agent were present in the pooled inoculum (unpublished data). ...

Transmission of the sheep scrapie to cattle was attempted in 1979 by using intracerebral, intramuscular, subcutaneous, and oral routes of inoculation of 5, 8- to 11-month old cattlw with a homologous mixture of brain from 1 affected sheep (61, 62). ONE of the 5 cattle develped neurologic signs 48 months after inoculation. Signs were disorientation, incoordination, a stiff-legged stilted gait, progressive difficulty in rising, and finally in terminal recumbency. The clinical course was 2.5 months. TWO of the 5 cattle similarly inoculated with brain tissue from a goat with scrapie exhibited similar signs 27 and 36 months after incoluation. Clinical courses were 43 an 44 days. Brain lesions of mild gliosis and vacuolation and mouse inoculation data were insufficient to confirm a diagnosis of scrapie. This work remained controversial until recent examination of the brains detected PrP-res in all 3 cattle with neurologic disease but in none of the unaffected cattle (62). Results of these studies are similar to ours and underscore the necessity of methods other than histopathology to diagnose scrapie infection in cattle. We believe that immunologic techniques for detecting PrP-res currently provide the most sensitive and reliable way to make a definitive diagnosis...

Visit to USA ... info on BSE and Scrapie

THEN, what about the potential for CWD and Scrapie to humans, what would that look like? they both are rampant in the USA, and they both transmit to primate, with no transmission studies ever done on man;


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.




CWD to CJD in humans (why not?), as easy (or difficult) as BSE/Scrapie;

The EMBO Journal, Vol. 19, No. 17 pp. 4425-4430, 2000
© European Molecular Biology Organization

Evidence of a molecular barrier limiting
susceptibility of humans, cattle and sheep to
chronic wasting disease

G.J. Raymond1, A. Bossers2, L.D. Raymond1, K.I. O?Rourke3,
L.E. McHolland4, P.K. Bryant III4, M.W. Miller5, E.S. Williams6, M.
and B. Caughey1,7

1NIAID/NIH Rocky Mountain Laboratories, Hamilton, MT 59840,
3USDA/ARS/ADRU, Pullman, WA 99164-7030, 4USDA/ARS/ABADRL,
Laramie, WY 82071, 5Colorado Division of Wildlife, Wildlife Research
Center, Fort Collins, CO 80526-2097, 6Department of Veterinary Sciences,
University of Wyoming, Laramie, WY 82070, USA and 2ID-Lelystad,
Institute for Animal Science and Health, Lelystad, The Netherlands
7Corresponding author e-mail: Received June 7, 2000;
revised July 3, 2000; accepted July 5, 2000.


Chronic wasting disease (CWD) is a transmissible
spongiform encephalopathy (TSE) of deer and elk,
and little is known about its transmissibility to other
species. An important factor controlling
interspecies TSE susceptibility is prion protein (PrP)
homology between the source and recipient
species/genotypes. Furthermore, the efficiency with which
the protease-resistant PrP (PrP-res) of one
species induces the in vitro conversion of the normal PrP
(PrP-sen) of another species to the
protease-resistant state correlates with the cross-species
transmissibility of TSE agents. Here we
show that the CWD-associated PrP-res (PrPCWD) of cervids
readily induces the conversion of recombinant cervid PrP-sen
molecules to the protease-resistant state in accordance
with the known transmissibility of CWD between cervids. In contrast,
PrPCWD-induced conversions of human and bovine PrP-sen were
much less efficient, and conversion of ovine PrP-sen was
intermediate. These results demonstrate a barrier at the
molecular level that should limit the susceptibility of these non-cervid
species to CWD.


Clearly, it is premature to draw firm conclusions about CWD
passing naturally into humans, cattle and sheep, but the present
results suggest that CWD transmissions to humans would be as
limited by PrP incompatibility as transmissions of BSE or sheep
scrapie to humans. Although there is no evidence that sheep
scrapie has affected humans, it is likely that BSE has caused variant
CJD in 74 people (definite and probable variant CJD cases to
date according to the UK CJD Surveillance Unit). Given the
presumably large number of people exposed to BSE infectivity,
the susceptibility of humans may still be very low compared with
cattle, which would be consistent with the relatively inefficient
conversion of human PrP-sen by PrPBSE. Nonetheless, since
humans have apparently been infected by BSE, it would seem prudent
to take reasonable measures to limit exposure of humans
(as well as sheep and cattle) to CWD infectivity as has been
recommended for other animal TSEs.


Scrapie to Humans USA?

1: Cent Eur J Public Health 2003 Mar;11(1):19-22

Analysis of unusual accumulation of Creutzfeldt-Jakob disease cases
in Orava and Liptov regions (northern Slovak focus) 1983-2000.

Mad'ar R, Maslenova D, Ranostajova K, Straka S, Baska T.

Institute of Epidemiology, Jessenius Faculty of Medicine, Comenius
University, Sklabinska 26, Martin, 037 53 Slovakia.

While familial cases of Creutzfeldt-Jakob disease are extremely rare
all over the world, 3 familial clusters were observed between
1983-2000 in a relatively small area situated in the North of
Slovakia. Prevalence of CJD in this area exceeded the overall
prevalence in Slovakia more than 8 times. The majority of CJD
patients admitted consuming sheep brain. Most patients lived in
small secluded villages with rather common familial intermarriage.
CJD affected both sexes equally. All patients were prior to the
disease mentally normal individuals. Shortly after the onset of CJD
their mental status deteriorated remarkably with an average survival
rate of 3.6 months.

PMID: 12690798


1: Eur J Epidemiol 1991 Sep;7(5):520-3

"Clusters" of CJD in Slovakia: the first laboratory evidence of scrapie.

Mitrova E, Huncaga S, Hocman G, Nyitrayova O, Tatara M.

Institute of Preventive and Clinical Medicine, Bratislava.

Epidemic-like occurrence of Creutzfeldt-Jakob disease was observed in
1987 in Slovakia (Orava). Search for the cause of CJD focus indicated a
coincidence of genetic and environmental risks in clustering patients.
Since Spongiform Encephalopathies might be transmitted orally, (Bovine
Spongiform Encephalopathy), the possibility of zoonotic source of CJD
cases in Orava was also considered. A deficient knowledge about the
occurrence of scrapie in Slovakia stimulated an examination of sheep
with signs of CNS disorders in two flocks of Valasky breed in Orava. In
one flock, neurohistopathological examination revealed in sheep brains
lesions characteristic for scrapie. Frozen brain tissue of these animals
were used for the detection of scrapie associated fibrils. They were
found in 2 animals from the same flock. This is the first laboratory
confirmation of scrapie in Czecho-Slovakia. The possible epidemiological
and economical implications are emphasized.


AS of March 31, 2005, there were 70 scrapie infected source flocks (Figure 3). There were 11 new infected and source flocks reported in March (Figure 4) with a total of 51 flocks reported for FY 2005 (Figure 5). The total infected and source flocks that have been released in FY 2005 are 39 (Figure 6), with 1 flock released in March. The ratio of infected and source flocks released to newly infected and source flocks for FY 2005 = 0.76 : 1. IN addition, as of March 31, 2005, 225 scrapie cases have been confirmed and reported by the National Veterinary Services Laboratories (NVSL), of which 53 were RSSS cases (Figure 7). This includes 57 newly confirmed cases in March 2005 (Figure 8). Fourteen cases of scrapie in goats have been reported since 1990 (Figure 9). The last goat cases was reported in January 2005. New infected flocks, source flocks, and flocks released or put on clean-up plans for FY 2005 are depicted in Figure 10. ...



May 7, 2004 / 53(Early Release);1-4

Creutzfeldt-Jakob Disease Not Related to a Common Venue --- New Jersey, 1995--2004

An Evaluation of a Suspected Cluster

of Creutzfeldt-Jakob Disease (CJD) in

New Jersey

May 2004

Division of Epidemiology, Environmental and

Occupational Health

Annals of Clinical Laboratory Science, vol 31, no. 2, 2001

Letter to the Editor:
A Cluster of Creutzfeldt-Jacob Disease Patients from Nassau County; New
York, USA

Debasis Adikari and Peter Farmer
Department of Pathology; North Shore University, Hospital, Manhasset,
New York
(received 13 January, 2001, accepted 18 January 2001)

Keywords: Creutzfeldt-Jacob disease, spongiform encephalopathy, prion,
disease cluster

Dear Editor

Creutzfeldt-Jacob disease (CJD) is a rare and transmissible neurological
disorder, which has been increasing in frequency in the United States
over the past two decades [1]. CJD is a spongiform encephalopathy
characterized by a conformational change of prion protein [2]. The death
rate for CJD in the USA is 0.9 per million [1].

We report 7 cases of CJD from North Shore University Hospital, a
community-based teaching institution that serves Nassau County, on Long
Island, NY. These cases were diagnosed and died during the 12-mo period
from mid-June 1999 to mid June 2000. The estimated population af Nassau
County in 1997 was 1,281,500, according to the New York State Department
of Health [3]. These data suggest a CJD death rate in Nassau County 0f
5.5 per million, which is approximately 6 times the national rate.
During the previous l-yr period, no case of CJD was diagnosed in our

The diagnosis of CJD in the 7 cases was based on pathological
examinations performed by at least 2 pathologists. Three cases were
confirmed by brain biopsy and 4 were diagnosed at autopsy. In 1 case,
CJD was not suspected during life. Pertinent data are summarized in
Table 1. The patients' clinical histories were significant for rapidly
deteriorating higher cortical functions, with confusion, speech
disorders, nystagmus, ataxia, and/or myoclonus. Only 1 patient had a
family history of CJD.

The autopsied brains were grossly unremarkable. At microscopic
examination, all specimens showed the pathological features of CJD,
including spongy degeneration, loss of neurons, and gliosis in the
cerebral cortex. The basal ganglia were also involved. No Kuru plaques
or inflammatory changes were noted. The patients' relatively advanced
ages (median = 75 yr, range = 57 to 82 yr), the rapid course of their
disease (median = 8 wk; range = 2 to 10 wk), and the absence of Kuru
plaques suggest that these cases represent classical CJD, as opposed to
new variant CJD [2].

The authors are concerned that the number of CJD cases in our catchment
area appears to have increased dramatically during the 12-mo period.
There is no clustering of the patients in any particular neighborhood.
While the causes for the apparent increase of CJD cases are unknown,
enhanced alertness for the disease and early recognition of its symptoms
may be possible explanations. The cluster of CJD patients ought to be
investigated thoroughly, which is beyond the scope of this letter.

Address correspondence to Peter Farmer, M.D., Department of Pathology,
North Shore University Hospital, 300 Community Drive, Manhasset, NY
11030, USA: rel 516-562-3676 fax 516-562-4591

212 Annals of Clinical & Laboratory Science

Table 1. Available information regarding the patients with
Creutzfeldt-Jacob disease from
Nassau County, New York.

Patient #
Age (yr)
Duration of symptoms (wk)
Family history of CJD
Signs & symptoms
Method of diagnosis
Other things

1. (BP)
no response to commands, speech
EEG, CT, LP negative

2. (DE)
ataxia, dysarthria, nystagmus

3. (ER)
memory loss, jerking motion of extremities

4. (EL)
post-operative progressive confusion, dementia
CT diffuse atrophy: no focal lesion

5. (LH)
progressive loss of cognition, myoclonus, ataxia
autopsy MRI non-contributory. EEG (arrow pointed
up) amplitude

6. (MW)
abnormal speech and movement

7. (AS)
rr 3rd nerve palsy, progressive encephalopathy
CT, MRI - meningioma

na = not available


1. Holman RC, Kahn AS, Belay ED, Schonberger LB. Creutzfeldt Jacob
disease in the United States. 1979-1994, using national mortality data
to assess the possible occurrence of variant cases. MMWR 1996:2:333-337.

2. Weihl CC, Roos RP: Creutzfeldt-Jacob disease, new variant
Creutzfeldt-Jacob disease, and bovine spongiform encephalopathy. Neurol
Clin 1994;4:835-859.

3. New York State Department of Health. Internet Home Page, (downloaded on 8
December 2000).


Julie Rawlings of the Texas Department of Health's main office in Austin said CJD was given "reportable" disease status by her agency in 1998, following "a cluster of CJD in northeast Texas" in 1997.

In '97, Rawlings said there were seven confirmed cases of CJD in her agency's Region IV, which extends to Oklahoma, Arkansas and Louisiana, while being connected by Paris, Palestine and Carthage.

"We never found anything," Rawlings said about the concentration of cases in northeast Texas. "We asked enough questions to fill a 70-page questionnaire ... You name it, we probably asked it. Again, we didn't come up with anything significant."

Since becoming reportable in 1998, there have been a total of 23 confirmed CJD cases in Texas, Rawlings said, adding last year's data is not totally complete.

Farmer PM, Kane WC, Hollenberg-Sher J.

Incidence of Creutzfeldt-Jakob disease in Brooklyn and Staten Island.

N Engl J Med. 1978 Feb 2;298(5):283-4. No abstract available.
PMID: 339092

2: Nosal R, Kapoor A, Shanin R.

Cluster of cases of Creutzfeldt-Jakob disease--Ontario.
Can Dis Wkly Rep. 1991 Jan 19;17(3):12. No abstract available.
PMID: 2001596

There is one report of a possible cluster of CJD cases in Canada;
between April 1989 and October 1990, six cases were reported in the
province of Ontario, from a population of 9.5 million (1986 census
figure). Two of the patients had come from areas of Czechoslovakia with
a high incidence of familial-type disease, but no other risk factors
were associated with these cases (7).

The Queniborough CJD cluster

The Times - front page
Alert on village's cluster of CJD deaths
AN URGENT government investigation was underway last night into a
cluster of cases of the human form of "mad cow" disease in
Leicestershire, where four people have died.
The investigation follows advice that the four deaths and another
probable case - out of only 75 nationwide - were unlikely to have
occurred by chance. And experts are pointing to the village of
Queniborough as a possible link between them.
Glenn Day, 35, lived there, Stacey Robinson, 18, was a former resident,
and Pamela Beyless, 24, often visited. All three died in 1998
A 19-year-old man who died in May and a 24-year-old who is thought to
have CJD also come from the same part of Leicestershire. It was the
fifth diagnosis in the area that triggered the investigation.


and how far does the Queniborough CJD cluster really reach ?

I was just looking at this email you sent me. Thurcaston
is about 6 miles from Queniborough - near enough. Is there
any way of contacting this family?


I've just been talking to professor bob will who runs the CJD
surveillance unit. He is very keen to learn more about the US case you
mentioned to me - the woman who lived in Thurmaston. So am I.

Could you contact them asap and ask if they will speak to us?


Sadly, everything points to Mrs Soukup being a victim of sporadic CJD.
In particular, she died aged 74 - whereas the oldest victim to date has
been 54. Also she and her husband were in britain in the 1970s - before
any cattle had come down with BSE. IN fact before any of the cattle
destined to get BSE had even been born. Despite this Professor Bob Will
does want to speak to the Soukups to find out more about the case.


personal email tss

since then, we have a documented case of nv/v CJD in 74 year old.


Join Date: Sep 2002
Location: Bacliff, TEXAS USA
Posts: 242


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:

From: "Belay, Ermias" To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias" Sent: Monday, September 30, 2002 9:22 AM Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS 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 investigated. 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 > > Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG > > HUNTERS continued...TSS

70 PAGE CJD QUESTIONNAIRE, that one was pretty thourough, but seldom used. the only way we are going to find answers is to make cjd reportable in every state and to have a cjd quesionnaire, one asking thourough questions pertaining to all potential routes and sources of agent. THEN, we have to have an health agency that will do something with the data, and keep us abreast as to what the data says in a timely manner. ...TSS

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