HuntingNet.com Forums - Field Dressing Deer/elk CWD/TSEs aka MAD COW DISEASE

wrong, i am not anti-anything, but pro-truth. my grandfather was a great
hunter, i used to hunt the saddle back in Llano Texas. I use to love to hunt,
but am disabled now with a damn neck injury. i really don't care if you
believe me or not, it will not change anything. love guns, and love to fish.
still eat meat occassionally. i only seek the truth, and i only wish to pass
that to everyone. i do not want anyone to go through what my mother went
through. 10 weeks from 1st of symptoms to death. she did everything
Linda Blair did in the exorcists movie except spin here head 360 degrees.
it took 3 of us to hold her down at times from the jerking. it will be 6 years
Dec 14. there are always people like you that want to stick your head in the
sand and ignore problems, and this is fine, i only hope to teach you what
you don't know about. if i succeed in only convincing a few, then i have
succeeded. but again, don't shoot the messenger.....

1: J Infect Dis 1980 Aug;142(2):205-8

Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.

Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.

Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.

PMID: 6997404
http://www.ncbi.nlm.nih.gov/entrez/q...&dopt=Abstract

1: J Neurol Neurosurg Psychiatry 1994 Jun;57(6):757-8

Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery.

Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC.

Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892.

Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.

PMID: 8006664 [PubMed - indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/entrez/q...&dopt=Abstract

Lancet Infectious Disease Journal August 2003 issue

Issue 8, 01 August 2003

http://infection.thelancet.com/journ.../iss8/contents

Tracking spongiform encephalopathies in North America [Full Text] [PDF]
Xavier Bosch
Newsdesk

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 hunters—two of whom were friends—who 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 limited”.

http://infection.thelancet.com/journal/journal.isa

Greetings,

>>>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. regardless, i said it and meant it...TSS

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

http://jama.ama-assn.org/cgi/content...urnalcode=jama

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

Email Terry S. Singeltary:
[email protected]

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?

http://www.neurology.org/cgi/eletters/60/2/176#535

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

snip...

The general opinion of those present was that BSE, as an
overt disease phenomenon, _could exist in the USA, but if it did,
it was very rare. The need for improved and specific surveillance
methods to detect it as recognised...

snip...

It is clear that USDA have little information and _no_ regulatory
responsibility for rendering plants in the US...

snip...

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_...

snip...

http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf

To be published in the Proceedings of the
Fourth International Scientific Congress in
Fur Animal Production. Toronto, Canada,
August 21-28, 1988

Evidence That Transmissible Mink Encephalopathy
Results from Feeding Infected Cattle

R.F. Marsh* and G.R. Hartsough

â¬¢Department of Veterinary Science, University of Wisconsin-Madison, Madison,
Wisconsin 53706; and ^Emba/Creat Lakes Ranch Service, Thiensville, Wisconsin 53092

ABSTRACT
Epidemiologic investigation of a new incidence of
transmissible mink encephalopathy (TME) in Stetsonville, Wisconsin
suggests that the disease may have resulted from feeding infected
cattle to mink. This observation is supported by the transmission of
a TME-like disease to experimentally inoculated cattle, and by the
recent report of a new bovine spongiform encephalopathy in
England.

INTRODUCTION

Transmissible mink encephalopathy (TME) was first reported in 1965 by Hartsough
and Burger who demonstrated that the disease was transmissible with a long incubation
period, and that affected mink had a spongiform encephalopathy similar to that found in
scrapie-affecied sheep (Hartsough and Burger, 1965; Burger and Hartsough, 1965).
Because of the similarity between TME and scrapie, and the subsequent finding that the
two transmissible agents were indistinguishable (Marsh and Hanson, 1969), it was
concluded that TME most likely resulted from feeding mink scrapie-infecied sheep.
The experimental transmission of sheep scrapie to mink (Hanson et al., 1971)
confirmed the close association of TME and scrapie, but at the same time provided
evidence that they may be different. Epidemiologic studies on previous incidences of
TME indicated that the incubation periods in field cases were between six months and
one year in length (Harxsough and Burger, 1965). Experimentally, scrapie could not be
transmitted to mink in less than one year.
To investigate the possibility that TME may be caused by a (particular strain of
scrapie which might be highly pathogenic for mink, 21 different strains of the scrapie
agent, including their sheep or goat sources, were inoculated into a total of 61 mink.
Only one mink developed a progressive neurologic disease after an incubation period of
22 mon..s (Marsh and Hanson, 1979). These results indicated that TME was either caused
by a strain of sheep scrapie not yet tested, or was due to exposure to a scrapie-like agent
from an unidentified source.

OBSERVATIONS AND RESULTS

A New Incidence of TME. In April of 1985, a mink rancher in Stetsonville, Wisconsin
reported that many of his mink were "acting funny", and some had died. At this time, we
visited the farm and found that approximately 10% of all adult mink were showing
typical signs of TME: insidious onset characterized by subtle behavioral changes, loss of
normal habits of cleanliness, deposition of droppings throughout the pen rather than in a
single area, hyperexcitability, difficulty in chewing and swallowing, and tails arched over
their _backs like squirrels. These signs were followed by progressive deterioration of
neurologic function beginning with locomoior incoordination, long periods of somnolence
in which the affected mink would stand motionless with its head in the corner of the
cage, complete debilitation, and death. Over the next 8-10 weeks, approximately 40% of
all the adult mink on the farm died from TME.
Since previous incidences of TME were associated with common or shared feeding
practices, we obtained a careful history of feed ingredients used over the past 12-18
months. The rancher was a "dead stock" feeder using mostly (>95%) downer or dead dairy
cattle and a few horses. Sheep had never been fed.

Experimental Transmission. The clinical diagnosis of TME was confirmed by
histopaihologic examination and by experimental transmission to mink after incubation
periods of four months. To investigate the possible involvement of cattle in this disease
cycle, two six-week old castrated Holstein bull calves were inoculated intracerebrally
with a brain suspension from affected mink. Each developed a fatal spongiform
encephalopathy after incubation periods of 18 and 19 months.

DISCUSSION
These findings suggest that TME may result from feeding mink infected cattle and
we have alerted bovine practitioners that there may exist an as yet unrecognized
scrapie-like disease of cattle in the United States (Marsh and Hartsough, 1986). A new
bovine spongiform encephalopathy has recently been reported in England (Wells et al.,
1987), and investigators are presently studying its transmissibility and possible
relationship to scrapie. Because this new bovine disease in England is characterized by
behavioral changes, hyperexcitability, and agressiveness, it is very likely it would be
confused with rabies in the United Stales and not be diagnosed. Presently, brains from
cattle in the United States which are suspected of rabies infection are only tested with
anti-rabies virus antibody and are not examined histopathologically for lesions of
spongiform encephalopathy.
We are presently pursuing additional studies to further examine the possible
involvement of cattle in the epidemiology of TME. One of these is the backpassage of
our experimental bovine encephalopathy to mink. Because (here are as yet no agent-
specific proteins or nucleic acids identified for these transmissible neuropathogens, one
means of distinguishing them is by animal passage and selection of the biotype which
grows best in a particular host. This procedure has been used to separate hamster-
adapted and mink-udapted TME agents (Marsh and Hanson, 1979). The intracerebral
backpassage of the experimental bovine agent resulted in incubations of only four months
indicating no de-adaptation of the Stetsonville agent for mink after bovine passage.
Mink fed infected bovine brain remain normal after six months. It will be essential to
demonstrate oral transmission fiom bovine to mink it this proposed epidemiologic
association is to be confirmed.

ACKNOWLEDGEMENTS
These studies were supported by the College of Agricultural and Life Sciences,
University of Wisconsin-Madison and by a grant (85-CRCR-1-1812) from the United
States Department of Agriculture. The authors also wish to acknowledge the help and
encouragement of Robert Hanson who died during the course of these investigations.

REFERENCES
Burger, D. and Hartsough, G.R. 1965. Encephalopathy of mink. II. Experimental and
natural transmission. J. Infec. Dis. 115:393-399.
Hanson, R.P., Eckroade, R.3., Marsh, R.F., ZuRhein, C.M., Kanitz, C.L. and Gustatson,
D.P. 1971. Susceptibility of mink to sheep scrapie. Science 172:859-861.
Hansough, G.R. and Burger, D. 1965. Encephalopathy of mink. I. Epizoociologic and
clinical observations. 3. Infec. Dis. 115:387-392.
Marsh, R.F. and Hanson, R.P. 1969. Physical and chemical properties of the
transmissible mink encephalopathy agent. 3. ViroL 3:176-180.
Marsh, R.F. and Hanson, R.P. 1979. On the origin of transmissible mink
encephalopathy. In Hadlow, W.J. and Prusiner, S.P. (eds.) Slow transmissible
diseases of the nervous system. Vol. 1, Academic Press, New York, pp 451-460.
Marsh, R.F. and Hartsough, G.R. 1986. Is there a scrapie-like disease in cattle?
Proceedings of the Seventh Annual Western Conference for Food Animal Veterinary
Medicine. University of Arizona, pp 20.
Wells, G.A.H., Scott, A.C., Johnson, C.T., Cunning, R.F., Hancock, R.D., Jeffrey, M.,
Dawson, M. and Bradley, R. 1987. A novel progressive spongiform encephalopathy
in cattle. Vet. Rec. 121:419-420.

MARSH

http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf

Docket Management Docket: 02N-0273 - Substances Prohibited From Use in
Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed
Comment Number: EC -10
Accepted - Volume 2

http://www.fda.gov/ohrms/dockets/dai.../8004be07.html

PART 2

http://www.fda.gov/ohrms/dockets/dai.../8004be09.html

with kindest regards,

Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, TEXAS USA 77518

A final greetings and Happy Holidays to all, for now,

> i really dont have the time to read through all this.

you have _no_ idea, and this is all i do, neither do i,
so this is my last post. the data is here, read it, delete it and stick
your head back in the sand. have a great hunt.

> we take the risk of carrying a loaded gun through the woods, climbing trees

ah yes, the same mentality with AIDS, the old 'only a gay thing' or
'it will not transmit by blood myth', and look at the mess we are in
now. only difference is the incubation period, and it's catchen up,
we have floundered much too long. mark my word here and now,
once the USA, Canada and Mexico convince the OIE to change
there BSE guidelines (and i believe they will because too many
other countries are in the same boat, but if you look at the known
BSE countries to date, the same ones that followed those same
_very weak_ guidelines, once they get the game down pat, with the
case study they are doing with CWD population, once they start rapid
TSE testing the USA bovine population in sufficient numbers to
find (not only 57,000 to date in 13 years), then the price of poker will
go up, and the truth will be known. the deer and elk industry were
pawns in a sea of politics, something they ignored for decades,
but they knew, that's the difference. the mad cow scare pre-2001
in the USA, a year later was changed to CWD. concindence you
ask, make your own mind up, you will have to do that eventually
with all this, maybe not now, but sooner or later.

> do you think maybe you could sum it up in a few sentences?
> yes and no, the short verse first;

you've been lied too. and or told only half truths.

my personal opinion of research over the last 6
years and through confidential data (most of which
is not so confidential anymore) I recieved through the
FOIA (pre this administration, FACT, both parties to blame),
from both the UK and the USA. CWD is only a small part of
a much larger problem. But for now, we will speak of the
topic at hand, but if you don't think some strain of BSE/TSE
whether atypical or not is in the USA cattle herd, then again,
find that same hole in the sand, or just go hunting or fishing,
some youngster caught a 10 pound flatty a few weeks ago,
i could see that sucker stuffed and baken in the oven.

I am NO Doctor, I have NO PhDs, and am President of NOTHING,

I only seek the truth...

NOW, since I am through here for now, the _long version_,
or the 'other side of the story'...later...TSS

########### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ############

Subject: CWD testing in Texas
Date: Sun, 25 Aug 2002 19:45:14 -0500
From
To: [email protected]
CC:

Dear Dr. Singletary,
In Fiscal Year 2001, seven deer from Texas were tested by the National
Veterinary Services Laboratory (NVSL) for CWD (5 fallow deer and 2
white-tailed deer). In Fiscal Year 2002, seven elk from Texas were
tested at NVSL (no deer). During these two years, an additional six elk
and one white-tailed deer were tested at the Texas Veterinary Medical
Diagnostic Laboratory (TVMDL). In Fiscal Year 2002, four white-tailed
deer (free-ranging clinical suspects) and at least eight other
white-tailed deer have been tested at TVMDL. One elk has been tested at
NVSL. All of these animals have been found negative for CWD. Dr. Jerry
Cooke of the Texas Parks and Wildlife Department also has records of 601
clinically ill white-tailed deer which were necropsied at Texas A&M
during the late 1960's and early 1970's, and no spongiform
encepalopathies were noted.
Thank you for your consideration.

xxxxxxxx, DVM, PhD
Texas Animal Health Commission
=================================

Dr. Jerry
Cooke of the Texas Parks and Wildlife Department also has records of 601
clinically ill white-tailed deer which were necropsied at Texas A&M
during the late 1960's and early 1970's, and no spongiform
encepalopathies were noted.

were they even looking for a SE and would they have even known what it was
if they had seen it back in 1960-70 ?...TSS
================================================== ===================

APPENDIX A: Results of CWD Sampling

Sampling and testing results for CWD from June, 2002 to April 1, 2003 are presented below:
Sampling and testing results for CWD from June, 2002 to April 1, 2003 TPWD TAHC Private Sector
1349 CWD Negative Deer 335 CWD Negative Deer 336 CWD Negative Deer
23 CWD Negative Exotics No Exotics No Exotics
1372 Total 335 Total 336 Total

The Grand Total of all samples collected and known 4/1/03 is 2043 of which 2020 deer and 23 exotics were found CWD negative. Samples were collected from 143 of 254 counties in Texas, and seven counties had 50 or more samples collected. Five ecoregions had 160 or more samples collected (150 samples from each ecoregion was the goal). The geographic distribution of sampling is currently not considered adequate for determining whether or not CWD exists in Texas (see map pg. 15). The goal is to improve upon distribution of samples collected within ecoregions and within counties. The goal of 2003-2004 and the next three to five years, is to collect 5000 samples (500 from each ecoregion) each sample year. The increased sampling is to have a 99 per-cent confidence level in detecting CWD if only one per-cent of the population is infected. Long-term surveillance sampling for CWD is required, as little is known about the incubation and infectious periods of the disease.

http://www.tpwd.state.tx.us/hunt/chr...an/appendix_a/

another coincidence, why no sampling in the obvious place, WHITE SANDS NM BORDER?
compare sampling maps to NW and then look at TEXAS, they simply ignore the most
obvious plase to sample?

Date: Mon, 21 Apr 2003 11:53:45 -0500
Reply-To: Bovine Spongiform Encephalopathy
Sender: Bovine Spongiform Encephalopathy
From: "Terry S. Singeltary Sr."

Subject: Chronic Wasting Disease (CWD) Gaining status: TEXAS

Chronic Wasting Disease (CWD) Gaining status: An annual inventory of the
cervids in the herd must be verified by the TAHC, USDA or an accredited
veterinarian. Animals over one year must have an approved identification
device, and animals less than a year are identified at change of
ownership. Appropriate samples must be collected and submitted from
cervids over 16 months of age that die or are hunter-harvested.
Slaughtered animals are exempt, if they are examined by state or federal
meat inspectors. Herd status depends on the total years of participating
in the plan.

http://www.tahc.state.tx.us/news/bro...ain_status.pdf

Greetings,

i am curious as to how a slaughtered animal can gain exempt status, and
claimed to be CWD free, simply by being examined by state or federal
meat inspectors. how do they claim CWD free of a dead animal already
slaughtered without CWD/TSE testing of that animal? what about
sub-clinical TSE?

NEGATIVE CWD SAMPLING IN TEXAS ECO REGION (MAP) JUNE 1, 2002 TO PRESENT

http://www.tahc.state.tx.us/animal_h...mar21-2003.gif

With approximately 4 million animals, Texas has the nation's largest
white-tailed deer population. In addition, about 19,000 white-tailed
deer and 17,000 elk are held in private facilities....

April 16, 2003

USDA Provides Funding for Chronic Wasting Disease Monitoring in Texas

AUSTIN, Texas -- Texas is likely to get a share of $4 million in new
federal funding to help detect the presence of chronic wasting disease,
a fatal deer and elk brain illness.

The U.S. Department of Agriculture, Animal and Plant Health Inspection
Service this week announced it is providing $4 million nationwide for
CWD surveillance. $1 million of that is available to be split among 14
states, including Texas. Last year, the International Association of
Fish and Wildlife Agencies provided about $6,700 to support Texas CWD
monitoring.

As of April 1 in Texas, samples from 2,043 wild white-tailed deer and
mule deer had been tested, all with negative results. Even though the
disease has not been detected in Texas, authorities say sampling is
incomplete. They plan to use USDA funding to continue testing this fall
when hunting season begins.

The illness has infected deer in 12 U.S. states and two Canadian
provinces. Last year, it was detected for the first time in southern New
Mexico, where seven deer have tested positive.

CWD is in the family of diseases called transmissible spongiform
encephalopathies (TSE). The disease is detected in infected animals'
neural tissue such as brains and spinal cords, as well as eyes and lymph
nodes. The TSE in domestic sheep is called scrapie, and in cattle it's
bovine spongiform encephalopathy (BSE). Similar diseases in humans
include Creuzfeldt-Jacobs disease (CJD) and its new variant, kuru, and
fatal familial insomnia. CWD should not be confused with BSE, scrapie or
CJD.

The World Health Organization has said there is no scientific evidence
CWD can infect humans. (After more than 16 years of monitoring in the
affected area in Colorado, no disease has been detected in people or
cattle living there.) However, the organization also says no people or
animals should consume any part of potentially CWD-infected deer or elk.
Hunters are advised to wear latex gloves when field dressing game, to
de-bone all meat and avoid consuming any neural tissue, such as brain or
spinal cords of animals.

With approximately 4 million animals, Texas has the nation's largest
white-tailed deer population. In addition, about 19,000 white-tailed
deer and 17,000 elk are held in private facilities. To determine if CWD
is present in captive herds, Texas Parks and Wildlife Department and
Texas Animal Health Commission are working with breeders to monitor
their herds.

http://www.tpwd.state.tx.us/news/news/030416a.htm

Media Contacts: Steve Lightfoot: (512) 389-4701,
[email protected]
Business Hours (512) 389-8046
E-mail [email protected] Public
Information: (800) 792-1112

April 18, 2003

Public Invited To Comment on Mule Deer Permit Proposal

AUSTIN, Texas -- The Texas Parks and Wildlife Department will hold
public hearings to take public comments on a proposed wildlife
regulation change that would expand the Managed Lands Deer (MLD) permit
program for white-tailed deer to include mule deer.

The MLD permit program is incentive-based and habitat-driven and is
designed to facilitate deer herd management goals of landowners and land
managers by having a more flexible season.

The public is invited to attend any of the following hearings and
provide comment. All meetings are set for 7 p.m.

* Morton, April 28, County Room Downtown * Turkey, April 29, Senior
Citizens Room at City Hall * Dumas, April 30, County Annex Room *
Alpine, May 6, Brewster County Courthouse Annex * Fort Stockton, May 7,
Small Community Building * Van Horn, May 8, Van Horn Convention Center *
Iraan, May 12, Community Bldg. (6th Street)

Questions about the meetings or the proposed wildlife regulation change
can be directed to TPWD staff at the Wildlife District office in Alpine
(915) 837-2051, Kerrville (830) 896-2500, or Canyon (806) 655-3782.

Individuals who cannot attend local public hearings can send their
comments on the proposed regulation change to Robert Macdonald, TPWD,
4200 Smith School Road, Austin, TX, 78744; phone (512) 389-4775; email
[email protected]
or by voicing comments at the
TPW Commission meeting May 29 in Austin at TPWD Headquarters.

If approved the TPW Commission, the proposed program will take effect
September 1, 2003.

http://www.tpwd.state.tx.us/news/news/030418a.htm

Texas Chronic Wasting Disease Management Plan

Draft (February 10, 2003)

Texas Chronic Wasting Disease Management Plan Links:

* Executive Summary

* Purpose

* Problem

* Introduction

* Texas Chronic Wasting Disease Status

* Plan for Management of the Disease in Texas

* Components of the Plan

* Education and Information

* Ongoing Targeted Surveillance

* Implementation of Geographically-Focused Surveillance

* Actions Should a CWD Positive Be Detected

* Appendix A. CWD Status of Current Knowledge

* Appendix B. Importation of Cervids

* Appendix C. Response Plan for CWD If Discovered

* Appendix C2. USDA-APHIS, Regulatory Statistics

* Appendix E. Media Response Plan

http://www.tpwd.state.tx.us/hunt/chr...nagement_plan/

MRC-43-00 [ ] [Text only version of this site] [Print this page] Issued:
Monday, 28 August 2000 NEW EVIDENCE OF SUB-CLINICAL PRION INFECTION:
IMPORTANT RESEARCH FINDINGS RELEVANT TO CJD AND BSE

A team of researchers led by Professor John Collinge at the Medical
Research Council Prion Unit1 report today in the Proceedings of the
National Academy of Sciences, on new evidence for the existence of a
'sub-clinical' form of BSE in mice which was unknown until now.

The scientists took a closer look at what is known as the 'species
barrier' - the main protective factor which limits the ability of
prions2 to jump from one species to infect another. They found the mice
had a 'sub-clinical' form of disease where they carried high levels of
infectivity but did not develop the clinical disease during their normal
lifespan. The idea that individuals can carry a disease and show no
clinical symptoms is not new. It is commonly seen in conventional
infectious diseases.

Researchers tried to infect laboratory mice with hamster prions3 called
Sc237 and found that the mice showed no apparent signs of disease.
However, on closer inspection they found that the mice had high levels
of mouse prions in their brains. This was surprising because it has
always been assumed that hamster prions could not cause the disease in
mice, even when injected directly into the brain.

In addition the researchers showed that this new sub-clinical infection
could be easily passed on when injected into healthy mice and hamsters.

The height of the species barrier varies widely between different
combinations of animals and also varies with the type or strain of
prions. While some barriers are quite small (for instance BSE easily
infects mice), other combinations of strain and species show a seemingly
impenetrable barrier. Traditionally, the particular barrier studied here
was assumed to be robust.

Professor John Collinge said: "These results have a number of important
implications. They suggest that we should re-think how we measure
species barriers in the laboratory, and that we should not assume that
just because one species appears resistant to a strain of prions they
have been exposed to, that they do not silently carry the infection.
This research raises the possibility, which has been mentioned before,
that apparently healthy cattle could harbour, but never show signs of, BSE.

"This is a timely and unexpected result, increasing what we know about
prion disease. These new findings have important implications for those
researching prion disease, those responsible for preventing infected
material getting into the food chain and for those considering how best
to safeguard health and reduce the risk that theoretically, prion
disease could be contracted through medical and surgical procedures."

ISSUED FRIDAY 25 AUGUST UNDER EMBARGO. PLEASE NOTE THAT THE EMBARGO IS
SET BY THE JOURNAL.

FOR FURTHER INFORMATION CONTACT THE MRC PRESS OFFICE ON 020 7637 6011
(OFFICE HOURS) OR 07818 428297 OR 0385 774357 (OUT-OF-OFFICE-HOURS) OR
PROFESSOR JOHN COLLINGE ON 020 7594 3760. PLEASE NOTE THAT OWING TO
TRAVEL COMMITMENTS PROFESSOR COLLINGE WILL ONLY BE AVAILABLE UNTIL 16.30
ON FRIDAY 25 AUGUST AND CONTACTABLE AGAIN ON MONDAY 28 AUGUST VIA THE
MRC PRESS OFFICE. DR ANDREW HILL (A CO-AUTHOR ON THE PAPER) FROM THE
DEPARTMENT OF PATHOLOGY AT THE UNIVERSITY OF MELBOURNE WILL BE AVAILABLE
ON 00 61 3 8344 3995 (DURING OFFICE HOURS) OR 00 61 3 9443 0009
(OUT-OF-OFFICE HOURS). PLEASE NOTE THAT AUSTRALIA IS TEN HOURS AHEAD OF
UK TIME.

NOTES FOR EDITORS

Professor Collinge is a consultant neurologist and Director of the newly
formed MRC Prion Unit based at The Imperial College School of Medicine
at St Mary's Hospital. He is also a member of the UK Government's
Spongiform Encephalopathy Advisory Committee (SEAC). The MRC prion unit
is was set up in 1999, and its work includes molecular genetic studies
of human prion disease and transgenic modelling of human prion diseases.

Prions are unique infectious agents that cause fatal brain diseases such
as Creutzfeldt-Jakob disease (CJD) in humans and scrapie and BSE (mad
cow disease) in animals. In some circumstances prions from one species
of animals can infect another and it is clear that BSE has done this to
cause the disease variant CJD in the UK and France. It remains unclear
how large an epidemic of variant CJD will occur over the years ahead.

The strain of prion used here to infect the mice is the Sc237 strain
(also known as 263K) which infects hamsters, and until now was assumed
not to infect mice.

This research was funded by the Medical Research Council and Wellcome
Trust.

The Medical Research Council (MRC) is a national organisation funded by
the UK tax-payer. Its business is medical research aimed at improving
human health; everyone stands to benefit from the outputs. The research
it supports and the scientists it trains meet the needs of the health
services, the pharmaceutical and other health-related industries and the
academic world. MRC has funded work which has led to some of the most
significant discoveries and achievements in medicine in the UK. About
half of the MRC's expenditure of Â£345 million is invested in over 50 of
its Institutes and Units, where it employs its own research staff. The
remaining half goes in the form of grant support and training awards to
individuals and teams in universities and medical schools.

The Wellcome Trust is the world's largest medical research charity with
a spend of some Â£600 million in the current financial year 1999/2000.
The Wellcome Trust supports more than 5,000 researchers, at 400
locations, in 42 different countries to promote and foster research with
the aim of improving human and animal health. As well as funding major
initiatives in the public understanding of science, the Wellcome Trust
is the country's leading supporter of research into the history of
medicine.

Â©2002 Medical Research Council Data Protection policy | Contact the MRC

=========================================

TSS

Getting a gauge on CWD in Texas

http://www.vegsource.com/talk/madcow/messages/1138.html

Chronic Wasting Disease (CWD) Gaining status: TEXAS

http://pub70.ezboard.com/fwolftracks...icID=189.topic

Wildlife policies

http://pub70.ezboard.com/fwolftracks...opicID=6.topic

SEWING THE SEEDS OF MAD COW (CWD) THROUGH ANIMAL PROTEIN $

http://www.americansportsman.com/mes...nting&post=642

http://www.deerfarms.com/messages/189.shtml

5TH COW AND 1 SHEEP GO DOWN WITH CWD IN TESTING

http://www.stopcwd.org/discussion/showPost.cfm?post=49

Subject: CWD SAMPLING IN TEXAS NOT CONSIDERED ADEQUATE FOR DETERMINING
EXISTANCE OF CWD
Date: October 28, 2003 at 1:55 pm PST

Samples were collected from 143 of the states 254 counties, and seven
counties had 50 or more samples collected. However, the geographic
distribution of sampling was not considered adequate for determining
whether or not CWD exists in Texas, Edwards said, so a minimum of 5,000
samples are scheduled to be collection statewide during the next three
to five years.

http://www.empiretribune.com/EMPIRET...25&PubID=14513

Subject: BSE/TSE RAPID TESTING EU/USA (why does USA not rapid Test$)
Date: December 9, 2003 at 7:16 pm PST

http://www.vegsource.com/talk/madcow...ges/91483.html

Re: BSE/TSE RAPID TESTING EU/USA (why does USA not rapid Test$)
TSS 12/09/03 (0)

http://www.vegsource.com/talk/madcow...ges/91484.html

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

snip...

The general opinion of those present was that BSE, as an
overt disease phenomenon, _could exist in the USA, but if it did,
it was very rare. The need for improved and specific surveillance
methods to detect it as recognised...

snip...

It is clear that USDA have little information and _no_ regulatory
responsibility for rendering plants in the US...

snip...

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_...

snip...

http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf

To be published in the Proceedings of the
Fourth International Scientific Congress in
Fur Animal Production. Toronto, Canada,
August 21-28, 1988

Evidence That Transmissible Mink Encephalopathy
Results from Feeding Infected Cattle

R.F. Marsh* and G.R. Hartsough

"Department of Veterinary Science, University of Wisconsin-Madison, Madison,
Wisconsin 53706; and ^Emba/Creat Lakes Ranch Service, Thiensville,
Wisconsin 53092

ABSTRACT
Epidemiologic investigation of a new incidence of
transmissible mink encephalopathy (TME) in Stetsonville, Wisconsin
suggests that the disease may have resulted from feeding infected
cattle to mink. This observation is supported by the transmission of
a TME-like disease to experimentally inoculated cattle, and by the
recent report of a new bovine spongiform encephalopathy in
England.

INTRODUCTION

snip...

Since previous incidences of TME were associated with common or
shared feeding practices, we obtained a careful history of feed
ingredients used over the past 12-18 months. The rancher was a
"dead stock" feeder using mostly (>95%) downer or dead dairy
cattle and a few horses. Sheep had never been fed.

MARSH

http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf

just what is sporadic CJD ???

It apparently arises from a one-in-a-million spontaneous conversion of a normal cellular protein (encoded by a gene on chromosome 20) into an abnormal 3-dimensional shape, or configuration.

WRONG, THIS HAS NEVER BEEN PROVEN !!!

85% OF ALL CJDs, ''SPORADIC'' DO NOT JUST HAPPEN WITHOUT
ROUTE AND SOURCE, THIS IS ONLY A MYTH. please read the data
below, if you are seeking the truth.

there are new findings that dispute this. why does the USA ignore these findings?
i thought i might share this with you. please see below;

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;

http://www.fda.gov/ohrms/dockets/ac/...3923s1_OPH.htm

Re: RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Terry S. Singeltary Neurology Online, 27 Jan 2003 [Full text]

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States 26 March 2003

Next Post-Publication Peer Review Top Terry S. Singeltary,

retired (medically)

CJD WATCH

Send Post-Publication Peer Review to journal:

Re: RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Email Terry S. Singeltary:

[email protected]

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?

http://www.neurology.org/cgi/eletters/60/2/176#535

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

IN the reply from Dr. Maddox to Terry S. Singeltary Sr., he states;

snip...

If BSE causes a sporadic CJD-like illness in humans, an increase in sporadic CJD cases would be expected to first occur in the United Kingdom, where the vast majority of vCJD cases have been reported. In the United Kingdom during 1997 through 2002, however, the overall average annual mortality rate for sporadic CJD was not elevated; it was about 1 case per million population per year. In addition, during this most recent 6-year period following the first published description of vCJD in 1996, there was no increasing trend in the reported annual number of UK sporadic CJD deaths.[3, 5] Furthermore, surveillance in the UK has shown no increase in the proportion of sporadic CJD cases that are homozygous for methionine (Will RG, National CJD Surveillance Unit, United Kingdom, 2003; personal communication)...

snip...

http://www.neurology.org/cgi/eletters/60/2/176#535

THERE seems to be some difference of opinion;

Mouse model sheds new light on human prion disease

snip...

Professor John Collinge said We are not saying that all or even most cases of sporadic CJD are as a result of BSE exposure, but some more recent cases may be the incidence of sporadic CJD has shown an upward trend in the UK over the last decade. While most of this apparent increase may be because doctors are now more aware of CJD and better at diagnosing it, serious consideration should be given to a proportion of this rise being BSE-related. Switzerland, which has had a substantial BSE epidemic, has noted a sharp recent increase in sporadic CJD.

snip...

http://www.mrc.ac.uk/txt/index/publi...oradic_cjd.htm

ALSO, Dr. Maddox states;

make routine mortality surveillance a useful surrogate for ongoing CJD surveillance...

THIS has proven not very useful in the U.K.;

THE EPIDEMIOLOGY OF CJD RG WILL 1984 (182 PAGES)

snip...

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...

snip...

http://www.bseinquiry.gov.uk/files/mb/m26/tab01.pdf

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.

snip...

http://www.bseinquiry.gov.uk/files/y...1/04004001.pdf

DR. Maddox states here;

In collaboration with appropriate local and state health departments and the National Prion Disease Pathology Surveillance Center, CDC is facilitating or conducting such surveillance and case- investigations, including related laboratory studies to characterize CJD and CWD prions.

HOWEVER in a recent article in the UPI out of Washington;

CJD screening may miss thousands of cases

By Steve Mitchell UPI Medical Correspondent Published 7/21/2003 3:00 PM

snip...

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.

snip...

http://www.upi.com/view.cfm?StoryID=...1-102924-4786r

ALSO in Philip Yams book 'The Pathological Protein';

''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''....

http://www.thepathologicalprotein.com/

THERE has been a _documented_ case of nv/v CJD in a 74 year old, so the errors Schonberger speaks of (above) would be of significant importance, if one believes in the nv/v CJD 'only' theory.

NOW we have _documented_ cases of very young CJD victims in the USA continuing to appear in several different states.

HOW does Dr. Maddox explain this, and does he still believe that a National CJD surveillance program with a CJD questionnaire to every victims family is still not warranted?

WITH CWD and Scrapie running rampant in the USA, with BSE now _documented_ in North America, with the feeding of ruminant-to-ruminant animal protein still happening in the USA in 2003 even though there has been a partial voluntary ban on ruminant feeding since 8/4/97, with only 48,000 BSE/TSE tests done on USA cattle in some 14 years of surveillance, when in any given year there are 100 million cattle in the USA, with all this, i think refusing to make CJD/TSEs reportable Nationally in the USA is not ONLY a grave mistake, but in my opinion, should be looked at with great suspicion...

PDF]Freas, William TSS SUBMISSION

File Format: PDF/Adobe Acrobat -

Page 1. J Freas, William From: Sent: To: Subject: Terry S. Singeltary

Sr. [[email protected]] Monday, January 08,200l 3:03 PM freas ...

http://www.fda.gov/ohrms/dockets/ac/.../3681s2_09.pdf

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

http://www.fda.gov/ohrms/dockets/ac/.../3681s2_09.pdf

BRITISH MEDICAL JOURNAL

SOMETHING TO CHEW ON

BMJ

http://www.bmj.com/cgi/eletters/319/7220/1312/b#EL2

BMJ

http://www.bmj.com/cgi/eletters/320/7226/8/b#EL1

Subject: The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases by Philip Yam
Date: Sun, 1 Jun 2003 00:17:59 -0500
From: "Terry S. Singeltary Sr." Reply-To: Bovine Spongiform Encephalopathy To: [email protected]

May 2003 A bizarre misshapen protein The story of the mysterious, devastating prion in a new book from Springer In the space of 12 months, Stephen Churchill lost his focus, his memory, most of his speech, then even the ability to dress, feed, and clean himself. He developed an excessive fear of water and sharp objects and refused to bathe or shave. And before long, with his unsteady gait and his tendency to fall, he spent his days slumped in a wheelchair or confined to a bed. To the staff of the nursing home where Stephen lived, the relentless decline was depressingly familiar-it had the earmarks of Alzheimer's disease. But something in the picture did not fit. The patient, when he died, was only 19 years old. Doctors later discovered that Stephen had succumbed to a new kind of killer, the prion, now known to be the cause of mad cow disease in cattle, chronic wasting disease in American deer and elk, Creutzfeldt-Jakob disease and fatal insomnia in humans, among other exotic ailments. Doctors and researchers have been aware of some of these diseases for a century and more, but only in the last two decades have scientists even begun to understand just how the pathological protein spreads to new species and kills its victims. In The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases, Philip Yam describes the history of the scientific effort to track down and understand the prion, and the medical effort, still underway, to devise treatments for those who suffer from its ravages. Philip Yam has been writing and editing for Scientific American since 1989 and is currently the magazine's news editor. This is his first book. Detailed information and order possibility: Philip Yam The Pathological Protein Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases 2003. Hardcover, 285 pp. Euro 29.95 (net price); ÃÂ£21.00; $27.50; sFr 51.50 ISBN 0-387-95508-9 Contact and review copies: Joan Robinson Springer-Verlag Press and Public Relations Tel.: +49- (0) 6221-487-8130, Fax: +49- (0) 6221-487-8141, E-mail: [email protected] http://www.springer.de/press/newbooks/protein.html The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases Philip Yam List Price: $27.50 Our Price: $19.25 You Save: $8.25 (30%) Availability: Usually ships within 24 hours. http://www.target.com/gp/detail.html...sin=0387955089

Greetings, got this book in the mail and just about fell out of the chair. Mr. Yam mentions my endevours and sporadic CJD. and even a bit where schonberger states; ''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''.... got a phone call a year or so ago and Mr. Yam interviewed me for about 2 hours, then i passed mountains of data to him. never thought i would hear from him again, much less a mention of me seeking the truth about what killed my mother and many more. most media take the data and run, never to be heard from again. then i got a signed book in the mail with a nice note. gonna sit back and read it. here is a bit about sporadic CJD and surveillance (or the lack of) from his book;

CHAPTER 14 Laying Odds Are prion diseases more prevalent than we thought? Researchers and government officials badly underestimated the threat that mad cow disease posed when it first appeared in Britain. They didn't think bovine spongifbrm encephalopathy was a zoonosisan animal disease that can sicken people. The 1996 news that BSE could infect humans with a new form of Creutzfeldt-Jakob disease stunned the world. It also got some biomedical researchers wondering whether sporadic CJD may really be a manifestation of a zoonotic sickness. Might it be caused by the ingestion of prions, as variant CJD is? Revisiting Sporadic CJD It's not hard to get Terry Singeltary going. "I have my conspiracy theo- ries," admitted the 49-year-old Texan.1 Singeltary is probably the nation's most relentless consumer advocate when it comes to issues in prion diseases. He has helped families learn about the sickness and coordinated efforts with support groups such as CJD Voice and the CJD Foundation. He has also connected with others who are critical of the American way of handling the threat of prion diseases. Such critics include Consumers Union's Michael Hansen, journalist John Stauber, and Thomas Pringle, who used to run the voluminous www.mad- cow.orgWeb site. These three lend their expertise to newspaper and magazine stories about prion diseases, and they usually argue that 223 224 CHAPTER 14 prions represent more of a threat than people realize, and that the gov- ernment has responded poorly to the dangers because it is more con- cerned about protecting the beef industry than people's health. Singeltary has similar inclinations, but unlike these men, he doesn't have the professional credentials behind him. He is an 11-grade dropout, a machinist who retired because of a neck injury sustained at work. But you might not know that from the vast stores of information in his mind and on his hard drive. Over the years, he has provided unac- knowledged help to reporters around the globe, passing on files to such big-time players as The New Tork Times, Newsweek, and USA Today. His networking with journalists, activists, and concerned citizens has helped medical authorities make contact with suspected CJD victims. He has kept scientists informed with his almost daily posting of news items and research abstracts on electronic newsgroups, including the bulletin board on www.vegsource.com and the BSE-listserv run out of the University of Karlsruhe, Germany. His combative, blunt, opinion- ated style sometimes borders on obsessive ranting that earns praise from some officials and researchers but infuriates othersespecially when he repeats his conviction that "the government has lied to us, the feed industry has lied to usall over a buck." As evidence, Singeltary cites the USDA's testing approach, which targets downer cows and examined 19,900 of them in 2002. To him, the USDA should test 1 mil- lion cattle, because the incidence of BSE may be as low as one in a mil- lion, as it was in some European countries. That the U.S. does not, he thinks, is a sign that the government is really not interested in finding mad cows because of fears of an economic disaster. Singeltary got into the field of transmissible spongiform encepha- lopathy in 1997, just after his mother died of sporadic CJD. She had an especially aggressive versionthe Heidenhain variantthat first causes the patient to go blind and then to deteriorate rapidly She died just ten weeks after her symptoms began. Singeltary, who said he had watched his grandparents die of cancer, considered her death by CJD to be much, much worse: "It's something you never forget." Her uncon- trollable muscle twitching became so bad "that it took three of us to hold her one time," Singeltary recalled. "She did everything but levitate in bed and spin her head." Doctors originally diagnosed Alzheimer's disease, but a postmortem neuropathological exam demanded by Singeltary revealed the true nature other death. Laying Odds 225 Classifying a disease as "sporadic" is another way for doctors to say they don't know the cause. Normal prion proteins just turn rogue in the brain for no apparent reason. The term "sporadic" is often particularly hard for the victims' families to accept, especially when the patient was previously in robust health. Maybe it was something in the water, they wonder, or in the air, or something they atethe same questions CJD researchers tried to answer decades ago. The names "sporadic CJD" and "variant CJD" also confuse the public and raise suspicions that U.S. authorities are hiding something when they say there have been no native variant CJD cases in the country. Singeltary suspected an environmental cause in his mother's demise a feeling reinforced a year later when a neighbor died of spo- radic CJD. For years, the neighbor had been taking nutritional supple- ments that contained cow brain extracts. Researchers from the National Institutes of Health collected samples of the supplement, Singeltary recounted, and inoculated suspensions into mice. The mice remained healthywhich only means that those supplement samples tested were prion-free. Scientists have made several attempts during the past few decades to find a connection between sporadic CJD and the environment. Often, these studies take the form of asking family members about CJD vic- timstheir diet, occupation, medical history, hobbies, pets, and so forthand comparing them with non-CJD subjects. Such case-control CJD studies have produced some intriguingand sometimes contra- dictoryresults. In 1985, Carleton Gajdusek and his NIH colleagues reported a correlation between CJD and eating a lot of roast pork, ham, hot dogs, and lamb, as well as rare meats and raw oysters.2 Yet they also recognized that the findings were preliminary and that more studies were needed. Following up, Robert Will of the U.K. National CJD Surveillance Unit and others pooled this data with those from two other case-con- trol studies on CJD (one from Japan and one from the U.K.). In particu- lar, they figured the so-called odds ratiocalculated by dividing the fre- quency of a possible factor in the patient group by the frequency of the factor in the control group. An odds ratio greater than I means that the factor may be significant. In their study, Will and his collaborators found an increase of CJD in people who have worked as health profes- sionals (odds ratio of 1.5) and people who have had contact with cows 226 CHAPTER 14 (1.7) and sheep (1.6). Unfortunately, those connections were not statisti- cally significant: The numbers of pooled patients (117) and control sub- jects (333) were so small that the researchers felt the odds ratios needed to reach 2.5 to 8 (depending on the assumptions) before they could be deemed statistically significant. The only statistically significant corre- lations they found were between CJD and a family history of either CJD (19.1) or other psychotic disease (9.9), although the latter might simply be correlated because psychotic disease may be an early symp- tom of undiagnosed CJD.3 In contrast with earlier findings, the team concluded that there was no association between sporadic CJD and the consumption of organ meats, including brains (0.6). Although these case-control studies shed a certain amount of light on potential risk factors for CJD, it's impossible to draw firm conclu- sions. Obtaining data that produces statistically meaningful results can be difficult because of the rarity of CJD and hence the shortage of sub- jects. Human memory is quite fragile, too, so patients' families may not accurately recall the lifestyle and dietary habits of their loved ones over the course of a decade or more. Consequently, researchers must cope with data that probably contain significant biases. In a review paper on CJD, Joe Gibbs of the NIH and Richard T.Johnson of Johns Hopkins University concluded that "the absence of geographic differences in incidence is more convincing evidence against major dietary factors, since large populations eschew pork and some consume no meat or meat products."A CJD study of lifelong vegetarians, they proposed, could produce some interesting data.4 The inconclusive results of case-control studies do not completely rule out the environment as a possible cause of CJD. "Dr. Prusiner's theory does fit much of the data of spontaneous generation of [mal- formed] PrP somewhere in the brain," Will remarkedthat is, the idea that sporadic CJD just happens by itself falls within the realm of the prion theory. Still, "it's very odd, if you look at all the forms of human prion diseases there are, all of them are transmissible in the laboratory and could be due to some sort of infectious agent."5 One of the great difficulties, he explained, is that "given that this is a disease of an extraordinarily long incubation period, are we really confident that we can exclude childhood exposure that is transmitted from person to person, as people move around? It's difficult to be sure about that." There might a "carrier state" that leaves people healthy yet still able to Laying Odds 227 infect others. If so, "you would never be able to identify what's causing the spread of the disease," concluded Will, who hasn't stopped looking for a possible environmental link. He has some preliminary data based on studies that trace CJD victims' lives well before the time symptoms beganup to 70 years; they suggest some degree of geographic cluster- ing, but no obvious candidates for a source of infection. A Case for Undercounting The difficulty in establishing causal links in sporadic prion diseasesif there are any in the first placeunderlines the importance of thorough surveillance. The U.K. has an active program, and when a victim of CJD is reported, one of Robert Will's colleagues visits and questions the victim's family. "No one has looked for CJD systematically in the U.S.," the NIH's Paul Brown noted. "Ever."6 The U.S., through the Centers for Disease Control and Prevention, has generally maintained a more passive system, collecting information from death certificates from the National Center for Health Statistics. Because CJD is invariably fatal, mortality data is considered to be an effective means of tabulating cases. The CDC assessed the accuracy of such data by comparing the numbers with figures garnered through an active search in 1996: Teams covering five regions of the U.S. contacted the specialists involved and reviewed medical records for CJD cases between 1991 and 1995. Comparing the actively garnered data with the death certificate infor- mation showed that "we miss about 14 percent," said CDC epidemiolo- gist Lawrence Schonberger. "That's improving. Doctors are becoming more knowledgeable," thanks to increased scientific and media atten- tion given to prion diseases.7 The active surveillance study of 1996, however, only looked at cases in which physicians attributed the deaths to CJD. Misdiagnosed patients or patients who never saw a neurologist were not tabulated thus CJD may be grossly underreported. Many neurological ailments share symptoms, especially early on. According to various studies, autopsies have found that CJD is misdiagnosed as other ills, such as dementia or Alzheimer's disease, 5 to 13 percent of the time. The CDC finds that around 50,000 Americans die from Alzheimer's each year 228 CHAPTER 14 (about 4 million have the disease, according to the Alzheimer's Association). Therefore, one could argue that thousands of CJD cases are being missed. (On the flip side, CJD could be mistakenly diagnosed as Alzheimer's disease or dementia, but the number of CJD patients is so small that they wouldn't dramatically skew the statistics for other neurological ills.) In part to address the issue of misdiagnosis, CJD families have asked the CDC to place the disease on the national list of officially notifiable illnesses, which tends to include more contagious conditions such as AIDS, tuberculosis, hepatitis, and viral forms of encephalitis. Currently, only some states impose this requirement. CDC officials have discounted the utility of such an approach, arguing that it would duplicate the mortality data, which is more accurate than early diag- noses of CJD, anyway. Moreover, mandatory reporting of CJD cases does not necessarily guarantee the end to missed cases.8 One clue suggests that the passive system is undercounting CJD in the U.S.: racial difference. The number of black CJD victims is about 38 percent that of white victims. Rather than sporadic CJD being a one- in-a-million lottery, it's more like one-in-2.5-million for African- Americans. Access to medical care might be one reason. Schonberger recounted that the CDC had asked other countries with substantial black populations to submit CJD figures for comparison but found that the surveillance in those countries was inadequate. "We haven't been able to find any comparable literature on this issue, so it's still up in the air," Schonberger said. On the other hand, Alzheimer's disease is more common among black people than whites, with an estimated higher prevalence ranging from 14 percent to almost 100 percent, according to a February 2002 report by the Alzheimer's Association. Are some black CJD cases being misdiagnosed as Alzheimer's? 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 population. As Schonberger pointed out, no doctor would misdiagnose a 30-year-old CJD patient as having Alzheimer's. The average age of the first 100 variant CJD victims was 29; should the epidemiology of vCJD changeif older people start coming down with itthen there would be problems. "The adequacy of our overall CJD surveillance would be Laying Odds 229 greatly reduced should the proportion of older individuals affected by variant CJD substantially increase," Schonberger explained.9 To date, only brain autopsies can confirm CJD. To encourage the necessary neuropathological studies, in 1997 the CDC helped establish the National Prion Disease Pathology Surveillance Center at Case Western Reserve University, under the directorship of Pierluigi Gambetti. But the number of brains examined has fallen far short of the number of CJD cases in the U.S.: Gambetti's lab, which receives brains based on referrals from local physicians and families, looked at only 99 sporadic CJD cases in 2000 and 138 in 2001, when about 300 each year are expected. "I'm very unhappy with the numbers," Gambetti lamented. "European countries see 100 or 90 percent of all the cases suspected. We see 30 to 40 percent."10 Most families don't think about having an autopsy done (which can cost upward of $1,500 if the hospitals don't pick up the tab), and mem- bers of the support group CJD Voice have said they were too distraught to think of shipping a loved one's brain by Federal Express to Gambetti's lab. (For accurate analyses of brain tissue, the autopsy must be performed within 72 hours of death, assuming the body has been kept refrigerated.) Moreover, physicians often do not suggest an autopsy, perhaps because of liability fears should the postmortem reveal that the original diagnosis was wrong. Gambetti has been work- ing on establishing a network that would enable postmortems to be done near where the deceased person lived and without cost to the family. He is also working on advertising the existence of his surveil- lance center, via meetings and letters to neurologists, pathologists, and other specialists. Gambetti is also attempting to combat what he termed "hysteria" over the potential for infection that has pathologists irrationally shunning CJD cases while they willingly conduct arguably riskier AIDS autopsies. "In order to make people aware, you have to keep informing them over and over and over," he said. Money is the main reason why the U.S. lags behind Europe in terms of surveillance. To adequately survey the 290 U.S. million residents, "you need a lot of money," Robert Will explained. "There was a CJD meeting of families in America in which poor old Larry {Schonberger] got attacked fairly vigorously because there wasn't proper surveillance. You could only do proper surveillance if you have adequate resources. 230 CHAPTER 14 That's the bottom line. We're very fortunate in the U.K.; we have very generous resources for CJD surveillance." Moreover, the U.K. makes feline spongifbrm encephalopathy an offi- cially notifiable disease. Domestic cats proved to be good sentinel ani- mals because they dine on the meat not fit for human consumption the parts more likely to harbor prion infectivity. In the U.S., FSE isn't federally notifiable. And while the USDA says it has sent educational material to private veterinarians and works with vet schools,21 it's not clear just how many vets can spot FSE, which has never been reported in the U.S. Certainly, not many cat postmortems are done. The only active portion of the U.S. CJD surveillance system are the follow-up investigations conducted for victims of CJD under 55 years of age. It began in 1996, when young people in the U.K. started succumb- ing to variant CJD. Victims under 30 years of age especially arouse interest, because such cases could indicate an infection from the envi- ronment. Except for the variant CJD case in Florida, the CDC has clas- sified all of these more youthful cases of CJD as having either sporadic or familial origins. One such age cluster involved the three venison eaters that the CDC tried unsuccessfully to link to the deer-and-elk borne chronic wasting disease. A second grouping occurred in 2002 in a pair of Michigan men. The twoone 26 years old, the other 28 did not know each other but lived in neighboring counties in Michigan and went to the same hospi- tal for diagnosis.12 The CDC's investigation turned up nothing that suggested a new form of CJD had emerged. But the increased frequency of young CJD cases is disturbing. In the 18-year period between 1979 and 1996, the U.S. had 12 cases in patients under 30, and only one of them had the sporadic form of CJD. (The other cases resulted from heredity or from transmission via contami- nated growth hormone or dura mater grafts.13) Between 1997 and 2001, five people under 30 died of sporadic CJD: the three venison eaters and the two Michigan patients. That represents a substantial blip of five young cases in five years, as opposed to only one case in 18 years. Physicians at the University of Michigan Health System who examined the two Michigan men concluded: As a result of our findings, we feel that sporadic CJD may be more common than previously thought, that it may occur in younger indi- Laying Odds 231 viduals than currently perceived, and that some cases may go undiag- nosed due to insufficient testing. . . . We recommend that physicians everywhere begin to consider CJD in rapidly progressive neurological decline of unknown causes in people under 30 years of age, and that brain biopsy and autopsy with genetic and prion analysis be performed in all such cases.14 Pathologically, the recent bout of young casualties in the U.S. appears to be no different from CJD already seen in America. Yet theoretically it may have come from a new source of infection, based on an unex- pected result announced in late November 2002. John Collinge of the British Medical Research Council's Prion Unit found that not all trans- genic mice infected with BSE prions developed the neuropathological and molecular characteristics of variant CJD; some of the mice instead generated the molecular features of sporadic CJD. Therefore, some CJD cases classified as sporadic may have actually been caused by BSE prions, Collinge hypothesized.15 So far, the epidemiology of CJD in the U.K. does not bear out that suppositionthere has been no substantial uptick in sporadic CJD as would be expected if BSE could paint more than one pathological picture. But the preliminary study, taken at face value, could be seen as evidence that something infectious is happening in the cases of young, sporadic CJD victims in the U.S. Another mouse study, reported in March 2002, fueled concern that prion infections may be more common than previously thought.16 Stanley Prusiner's lab found that mice infected with mouse prions accu- mulated PrPSc in their skeletal muscles, mostly in those in the hind limbs. In some mice, each gram of muscle contained some 10 million infectious doseson par with that in the brain in other experiments involving intracerebral inoculation. To some CJD researchers, this find- ing suggested that muscle meat from cows might not be safe, after all, and that the measures taken in Europe to protect the food supply banning high-risk cow partsmay not be enough. Although this study may seem alarming, its implications are not as sweeping as they may appear. Only a minority of results in mouse stud- ies end up having a direct analog in humans. The skeletal muscle discov- ery warrants further examination, but it would be premature to alter food policies. Prions are different for each species, and accumulation of prions varies from species to species and from disease to disease. Furthermore, BSE cattle muscle has failed to sicken mice in bioassay 232 CHAPTER 14 work, suggesting that little or no infectious prions lurk there. What such findings truly reveal is that prion diseases are complicated and still mysterious, and trying to quantify the risks for human health is fraught with uncertainties...snip...284 pages...thanks Philip...TSS The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases, Philip Yam Philip Yam News Editor Scientific American www.sciam.com http://www.thepathologicalprotein.com/

CJD screening may miss thousands of cases

By Steve Mitchell
UPI Medical Correspondent
Published 7/21/2003 3:00 PM
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WASHINGTON, July 21 (UPI) -- The federal government's monitoring system
for cases of Creutzfeldt-Jakob disease, a fatal human brain illness,
could be missing tens of thousands of victims, scientists and consumer
advocates have told United Press International.

Creutzfeldt-Jakob disease or CJD can be caused by eating beef
contaminated with mad cow disease, but the critics assert without a
better tracking system it might be impossible to determine whether any
CJD cases are due to mad cow or obtain an accurate picture of the
prevalence of the disorder in the United States.

Beginning in the late 1990s, more than 100 people contracted CJD in the
United Kingdom and several European countries after eating beef infected
with bovine spongiform encephalopathy -- the clinical name for mad cow
disease.

No case of mad cow has ever been detected in U.S. cattle and the Centers
for Disease Control and Prevention's monitoring system has never
detected a case of CJD due to eating contaminated American beef.
Nevertheless, critics say, the CDC's system misses many cases of the
disease, which currently is untreatable and is always fatal.

The first symptoms of CJD typically include memory loss and difficulty
keeping balance and walking. As the disease destroys the brain, patients
rapidly progress in a matter of months to difficulty with movement, an
inability to talk and swallow and, finally, death.

Spontaneously-occurring or sporadic CJD is a rare disorder. Only about
300 cases appear nationwide each year, but several studies have
suggested the disorder might be more common than thought and as many as
tens of thousands of cases might be going unrecognized.

Clusters of CJD have been reported in various areas of the United States
-- Pennsylvania in 1993, Florida in 1994, Oregon in 1996, New York in
1999-2000 and Texas in 1996. In addition, several people in New Jersey
developed CJD in recent years, including a 56 year old woman who died on
May 31, 2003. Although in some instances, a mad cow link was suspected,
all of the cases ultimately were classified as sporadic.

People who develop CJD from eating mad-cow-contaminated beef have been
thought to develop a specific form of the disorder called variant CJD.
But new research, released last December, indicates the mad cow pathogen
can cause both sporadic CJD and the variant form.

"Now people are beginning to realize that because something looks like
sporadic CJD they can't necessarily conclude that it's not linked to
(mad cow disease)," said Laura Manuelidis, section chief of surgery in
the neuropathology department at Yale University, who conducted a 1989
study that found 13 percent of Alzheimer's patients actually had CJD.

Several studies, including Manuelidis', have found that autopsies reveal
3 percent to 13 percent of patients diagnosed with Alzheimer's or
dementia actually suffered from CJD. Those numbers might sound low, but
there are 4 million Alzheimer's cases and hundreds of thousands of
dementia cases in the United States. A small percentage of those cases
could add up to 120,000 or more CJD victims going undetected and not
included in official statistics.

Experiences in England and Switzerland -- two countries that discovered
mad cow disease in their cattle -- have heightened concerns about the
possibility some cases of sporadic CJD are due to consuming
mad-cow-tainted beef. Both countries have reported increases in sporadic
CJD since mad cow was first detected in British herds in 1986.

Switzerland discovered last year its CJD rate was twice that of any
other country in the world. Switzerland had been seeing about eight to
11 cases per year from 1997 to 2000. Then the incidence more than
doubled, to 19 cases in 2001 and 18 cases in 2002.

The CDC says the annual rate of CJD in the United States is one case per
million people, but the above studies suggest the true prevalence of CJD
is not known, Manuelidis told UPI.

Diagnosing CJD or Alzheimer's is difficult because no test exists that
can identify either disease in a living patient with certainty. So
physicians must rely on the patient's symptoms to determine which
illness might be present. Sometimes, however, the symptoms of one
disease can appear similar to the other disorder. The only way to
determine the disease conclusively is to perform an autopsy on the brain
after death.

Unfortunately, although autopsies once were performed on approximately
half of all corpses, the frequency has dropped to 15 percent or less in
the United States. The National Center for Health Statistics -- a branch
of the CDC -- stopped collecting autopsy data in 1995.

"If we don't do autopsies and we don't look at people's brains ... we
have no idea about what is the general prevalence of these kinds of
infections and (whether) it is changing," Manuelidis said.

At the same time autopsies have been declining, the number of deaths
attributed to Alzheimer's has increased more than 50-fold since 1979,
going from 857 deaths then to nearly 50,000 in 2000. Though it is
unlikely the dramatic increase in Alzheimer's is due entirely to
misdiagnosed CJD cases, it "could explain some of the increase we've
seen," Manuelidis said.

"Neurodegenerative disease and Alzheimer's disease have become a
wastebasket" for mental illness in the elderly that is difficult to
diagnose conclusively, she said. "In other words, what people call
Alzheimer's now is more broad than what people used to call it, and that
has the possibility of encompassing more diseases -- including CJD."

The autopsy studies that found undiagnosed CJD cases raise the question
of whether the United States "already has an undetected epidemic here,"
Jeff Nelson, director of vegsource.com, a vegetarian advocacy Web site,
told UPI.

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