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From: TSS ()
Subject: Public Comment Sought On Options For Managing An Outbreak Of Chronic Wasting Disease MONTANA
Date: August 2, 2005 at 2:52 pm PST

Monday, August 01, 2005
Public Comment Sought On Options For Managing An Outbreak Of Chronic Wasting Disease
Bull Elk Herd

Bull elk herd in winter.
PDF Version
Montana Fish, Wildlife & Parks officials are seeking public comment on management decisions that might be necessary should chronic wasting disease be discovered in the state’s wild deer and elk herds. CWD, a fatal brain disease in deer and elk, has not yet been found in the wild herds in Montana.

"We’ve prepared an environmental assessment to help identify the choices the agency would have to make should CWD be discovered in Montana’s wild deer or elk herds," said Tim Feldner, FWP’s CWD plan coordinator. "Now it is time for the public to take a look at those potential actions and let us know which they think would be the most acceptable and effective."

CWD has been detected in Wyoming, South Dakota, Utah, Colorado and Saskatchewan, among other states and provinces. No one is sure where CWD came from. It first showed up in the wild in 1981. Since then it has been found in wild herds or alternative livestock ranches, or game farms, in 12 states and two provinces.

Since 1998, FWP has tested more than 7,000 wild elk or deer in Montana for CWD and has not yet found any evidence of the disease. CWD was diagnosed in 1999 in nine captive elk on an alternative livestock facility near Philipsburg. All the animals there were destroyed and the facility was quarantined. Montana voters passed an initiative the following year that prohibits transfer of existing game farm licenses, ends new licensing, and forbids shooting captive elk.

"It appears from the way the disease has spread in the past several years in adjacent states, that it is highly likely CWD will appear here in wild deer and elk herds at some point," Feldner said. "We’re preparing now to manage that situation as effectively as possible."

Feldner said the CWD environmental assessment describes actions the agency might take if CWD is identified, the impact on wildlife populations and the related economic, agricultural, environmental and social issues. The plan makes recommendations on prevention, surveillance, management, public information and research. Management alternatives include reducing the number of deer or elk in the area where the disease is identified to reduce or stop its spread.

A series of public meetings to discuss the environmental assessment will held from 7-9 p.m. at the following FWP regional offices:

Aug. 16 Missoula 3201 Spurgin Rd.

Aug. 23 Bozeman 1400 South 19th

Aug. 25 Great Falls 4600 Giant Springs Rd.

Sept. 1 Kalispell 490 N. Meridian Rd.

Sept. 13 Billings 2300 Lake Elmo Dr.

Sept. 14 Miles City Industrial Site W

Sep. 15 Glasgow Rural Route 1-4210

Copies of the CWD environmental assessment are available at FWP regional and the headquarters office, on the FWP web site, under Public Notices, Chronic Wasting Disease Mgmt Plan-EA, or by calling 406-444-2452.

The public may comment on the EA during the public meetings, or until Sept. 23 by email to . Those reviewing the document on the FWP web site may email comments from there, or comment by mail to: Montana Fish, Wildlife & Parks, Attn: Chronic Wasting Disease EA, 1420 East Sixth Ave., P.O. Box 200701, Helena, MT 59620-0701.

Chronic Wasting Disease Mgmt Plan-EA
Environmental Assessment

This environmental assessment (EA) evaluates potential effects on the human environment from actions proposed by the Montana Department of Fish, Wildlife & Parks intended to prevent and/or control Chronic Wasting Disease (CWD) in Montana’s free-ranging deer and elk populations. Any actions taken, or any actions not taken per the “no action alternative,” have the potential to affect wildlife populations as well as the economic, agricultural, environmental and social issues directly tied to those wildlife populations. This document represents a coordinated effort using the “best science” currently available to state and federal agencies and their scientific staffs in the development of a CWD management plan for the state of Montana.

Contact Information
Tim Feldner, Program Manager
Commercial Wildlife Permit Program
Law Enforcement Division
Montana Fish, Wildlife & Parks
PO Box 200701
Helena MT 59620

Notice Attachments
Chronic Wasting Disease Mgmt Plan-EA [1,431,531 bytes]





AUGUST, 2005

Wildlife Division, Helena, MT 59620


Table of Contents………………………………………………… i

List of Tables……………………………………………………… iii

List of Figures…………………………………………………….. iii

Glossary…………………………………………………………… iv

Introduction……………………………………………………… 1

Background……………………………………………………… 1

Chapter 1: Purpose of and Need for Action…………………… 7

1.1: Proposed action……………………………………… 7

1.2: Location………………………………………………. 7

1.3: Need for the Action……………………………………7

1.4: Objectives of the Action……………………………… 8

1.4.1 Objective #1…………………………………8

1.4.2. Objective #2…………………………………8

1.4.3. Objective #3…………………………………8

1.4.4. Objective #4…………………………………8

1.5: Decisions to be Made………………………………… 8

1.6: History of the Planning and Scoping Process………… 8

1.7: Relationship to Other Plans and Goals……………….. 9

1.8: Permits, Licenses, or other Authorizations Required… 10

1.9: Issues………………………………………………….. 10



Chapter 2: Alternatives………………………………………….. 15

2.1: Introduction…………………………………………… 15

2.2: Description of the Alternatives……………………….. 15

2.2.1: Alternative I – No Action……………………15

2.2.2: Alternative II – Enhanced Surveillance……. 16

2.2.3: Alternative III – Enhanced Prevention…….. 18

2.2.4: Alternative IV – Control at 1%…………….. 20

2.2.5: Alternative V – Control at 0%……………… 22

2.2.6: Alternative VI – Aggressive Elimination……24

2.3: Process Used to Develop the Alternatives……………. 26

2.4: Summary Comparison of the Activities of Each………27

Alternative, Predicted Achievement of Objectives, and

Predicted Environmental Effects on Issue Resources

Chapter 3: Affected Environment……………………………… 34

3.1: Introduction………………………………………….. 34

3.2: Description of Relevant Affected Resources………… 34

3.2.1: Montana’s Native Deer and Elk Populations.. 34


3.2.2: Wildlife Management Tools……………….. 39

3.2.3: Wildlife and Montana’s Economy…………. 40

3.2.4: Human Health……………………………… 41

3.2.5: Alternative Livestock………………………. 42

3.2.6: Disposal/Environmental Contamination…… 44

3.3: Other Regulatory Agencies…………………………… 50

Chapter 4: Environmental Consequences ………………………54

4.1: Introduction…………………………………………… 54

4.2: Cumulative Impacts……………………………………54

4.3: Irreversible and Irretrievable Commitments………….. 55

4.4: Environmental Consequences of CWD on…………… 55

Identified Issues

4.4.1: Effect on Montana’s Wild Deer and Elk…… 55

4.4.2: Effect on Wildlife Management Tools……... 57

4.4.3: Effect on Montana’s Economy…………….. 58

4.4.4: Effect on the Alternative Livestock Industry.. 60

4.4.5: Effect on Human Health……………………. 62

4.4.6: Effect on Environmental Contamination and..62

Carcass Disposal

4.5: Predicted Environmental Impacts of Alternative II….. 63

4.5.1: Biological Environment……………………. 64

4.5.2: Physical Environment………………………. 64

4.5.3: Socioeconomic Environment……………….. 65

4.6: Predicted Environmental Impacts of Alternative III….. 65

4.6.1: Biological Environment……………………. 65

4.6.2: Physical Environment……………………… 66

4.6.3: Socioeconomic Environment………………. 67

4.7: Predicted Environmental Impacts of Alternative IV…. 67

4.7.1: Biological Environment……………………. 68

4.7.2: Physical Environment………………………. 69

4.7.3: Socioeconomic Environment………………. 69

4.8: Predicted Environmental Impacts of Alternative V….. 70

4.8.1: Biological Impacts………………………….. 70

4.8.2: Physical Impacts……………………………. 71

4.8.3: Socioeconomic Impacts…………………….. 71

4.9: Predicted Environmental Impacts of Alternative VI…. 72

4.9.1: Biological Impacts………………………….. 72

4.9.2: Physical Impacts…………………………… 72

4.9.3: Socioeconomic Impacts…………………….. 73

Chapter 5: List of Individuals Associated with the Project ……74

Chapter 6: List of Persons and Agencies Consulted ……………75

Bibliography………………………………………………………. 76

Appendix 1: "Public Information Plan – Chronic Wasting.…….. 79

Disease in Montana"

Appendix 2: "Mule Deer Population Ecology and Chronic ………87

Wasting Disease Study"



Table 1. 2.4.1. Summary Comparison of the Activities of Each

Alternative……………………………………………… 28

Table 2. 2.4.2 Summary Comparison of the Achievement of

Project Objectives………………………………………. 30

Table 3. 2.4.3 Summary Comparison of Predicted Environmental

Effects…………………………………………………… 31

Table 4. CWD Survey Result Totals for Montana from 1998

through 2004…………………………………………… 39


Figure 1. Distribution of CWD in both captive and

wild deer and elk populations as of 2004.

(from CWD Alliance webpage, …… 4

Figure 2. 2004 Montana Mule Deer Distribution and

Population Estimate…………………………………. 34

Figure 3. 2004 Montana White-tailed Deer Distribution

and Population Estimate……………………………… 35

Figure 4. 2004 Montana Elk Distribution and Population

Estimate……………………………………………….. 37

Figure 5. Model of Deer Herds with Chronic Wasting

Disease (an average of 250 simulations)……………….. 56



Biopsy: Removal of tissue from a living body for microscopic examination to establish


Cervid: Members of the cervidae family, which include deer, elk, moose, and caribou.

Ecological niche: The area within a particular habitat occupied by an animal in which it

interacts with other different species of animals and plants.

Emigration: To leave one area to settle elsewhere.

Endemic area: A geographical area where a disease has been present in the population for

a period of time or has become established.

Epizootic: A term used to describe a disease that attacks a large number of animals


High-risk area: A geographical area identified as having a higher probability of finding

CWD within its boundaries.

Incubation period: The period of time between becoming infected with a disease causing

organism or a prion and actually showing outward signs of the disease.

Immigration: To enter and settle in a new region.

Infective dose: The amount of infectious material required to establish an infection or

disease in a susceptible animal.

Morbidity: An unhealthy condition indicating that a disease is ongoing in an animal.

Mortality: The frequency of number of deaths in proportion to a population.

Offal: Waste parts of a butchered animal.

Pathogen: An agent that causes a disease (bacterium, virus, prion, etc.)

Pathogenesis: The development of a diseased or morbid condition. Generally refers to the

mechanism by which a pathogen is able to affect the cells, organs, and organ systems of an

animal eventually causing disease.

Prevalence: The percentage of animals affected in a given population.


Spongiform lesions: Microscopic "holes" appearing in the brain tissue that are

characteristic of transmissible spongiform encephalopathies.

Telemetry: The science and technology of automatic data measurement and transmission

by radio from remote sources to a receiving station for analysis. Used in wildlife biology to

locate and track animals that have been fitted with a device that sends out a radio signal.

Ungulate: Hoofed animals such as horses, cattle, swine, deer, and sheep.

Zoonotic: A term used to describe a disease that can be transmitted from animals to man.





This environmental assessment (EA) evaluates potential effects on the human environment

from actions proposed by the Montana Department of Fish, Wildlife & Parks intended to

prevent and/or control Chronic Wasting Disease (CWD) in Montana’s free-ranging deer

and elk populations. Any actions taken, or any actions not taken per the "no action

alternative," have the potential to affect wildlife populations as well as the economic,

agricultural, environmental and social issues directly tied to those wildlife populations.

This document represents a coordinated effort using the "best science" currently available

to state and federal agencies and their scientific staffs in the development of a CWD

management plan for the state of Montana. Although we can benefit from the experience

gained in other states where actions have been taken to manage ongoing CWD problems,

conclusions regarding the success of those actions are premature at this time. Almost all

scientists and wildlife managers in states faced with ongoing CWD problems have agreed,

however, that action must be taken to prevent and/or control CWD. To be passive in the

fight against this disease can only result in decreased wildlife populations and decreased

opportunities for the enjoyment of this valuable resource in the state of Montana.


Disease has been defined as a "deviation from the normal" for a functioning organism or

their systems. Bacteria, viruses, rickettsia, parasites, and fungi, generally referred to as

"pathogens", have historically caused transmissible diseases. A new concept in

transmissible disease has emerged in the last 20 years proposing that a set of diseases called

transmissible spongiform encephalopathies (TSE’s) are not caused by "historical"

pathogens, but by a protein referred to as a "prion." A prion lacks the genetic information

(DNA or RNA) that all of the previously mentioned pathogens rely on for their infective

nature. Although the "prion theory" has not enjoyed full acceptance in the scientific

community, prions have been implicated in a number of diseases in a wide variety of

mammalian species. These diseases include Kuru and Creutzfeldt-Jakob Disease (CJD) in

humans, scrapie in sheep, bovine spongiform encephalopathy (BSE or mad cow disease) in

cattle, transmissible mink encephalopathy in mink, feline spongiform encephalopathy in

cats, and CWD in deer and elk. There is no cure for any of the TSE’s - all of these diseases

cause the eventual death of the affected animal.

CWD was first identified as a fatal condition of unknown cause in captive deer at a

Colorado wildlife research facility in the late 1960’s. In 1978, that condition was linked to

abnormalities found in the brains of the infected deer. Those abnormalities had a

remarkable resemblance to what was observed in the brains of sheep infected with scrapie,

a TSE disease that has afflicted sheep for centuries. This finding led scientists to conclude

that the disease affecting the captive deer was a TSE. Field observations, proven later by

experimental studies, indicate that CWD is transmissible from animal to animal, although


the exact method or natural route(s) of transmission remains unclear. Most researchers

believe that transmission occurs when a susceptible animal is directly exposed to the urine,

feces, or saliva of an infected animal. Transmission of CWD has also been shown to occur

through exposure of non-infected animals either to pastures contaminated as long as two

years earlier by infected animals or by exposure to decomposed carcasses of animals that

have died of CWD. The role played by environmental contamination in the natural

transmission of CWD is presently being evaluated. What is known is that the prion protein

is very difficult to destroy and that normal inactivating agents such as alcohol, UV light,

heat, and oxidizing agents normally effective in destroying bacteria, virus, etc. are much

less effective in destroying the prions. Because of this, prions may remain in the

environment for much longer than the classical pathogens.

CWD was first recognized as a disease in a free ranging elk in Colorado in 1981 and in

Wyoming in 1985. By 1990, CWD had been found in free ranging mule and white-tailed

deer in both states. Although the disease seemed to remain relatively localized for two to

three decades within northeastern Colorado and southeastern Wyoming, appearance of

CWD in captive game farm elk in Saskatchewan in 1996 preceded findings in game farms

in South Dakota, Colorado, Oklahoma, Montana, Nebraska, Kansas, Wisconsin,

Minnesota, New York, and in the province of Alberta. The movement of captive deer and

elk by the game farm industry has been implicated in the spread of CWD to both captive

cervids and free ranging cervids in other states. Increased surveillance efforts for CWD by

many states may also have added to the known geographical distribution of CWD in the

United States outside the original endemic area. In addition to Colorado and Wyoming,

CWD has now been detected in free ranging deer or elk populations in Nebraska, South

Dakota, Wisconsin, Illinois, Utah, New Mexico, New York, and in the province of


The minimum incubation period prior to the observation of clinical signs in animals

experimentally infected with CWD is 15 months; incubation periods are variable and may

exceed 25 months in deer and 34 months in elk (Williams et al. 2002a). The time course

to observation of clinical signs, however, appears to be inversely related to the infective

dose, so experimental data probably underestimates the incubation periods seen in natural

infections (Williams et al. 2002b). Clinical signs in infected animals include weight loss,

increased drinking, increased salivation and drooling, lowered head, drooping ears, and

behavioral changes. Because these clinical signs mimic those seen in animals affected by

other diseases, the only way to accurately diagnose CWD in deer and elk is by direct

examination of either a section of the brain (obex) or of specific lymph nodes (lymph nodes

of choice for diagnosis are retropharyngeal lymph nodes) from the affected animal.

Previously, positive CWD diagnosis was accomplished by either microscopic examination

of the collected tissues for evidence of characteristic "spongiform lesions" or by a

technique employing specialized staining of the tissues called immunohistochemistry

(IHC). Recent advances in diagnostic techniques, including development of an enzyme-

linked immunosorbent assay (ELISA) for diagnosis of CWD, have decreased both the cost

of performing the test and the time required to provide results. These advances in testing

methodology and efficiency have come at a time when more states across the U.S have


either experienced CWD in their wildlife populations or have become concerned about it’s

possible presence resulting in dramatically expanded surveillance programs for CWD.

There is a good deal of work going on in the United States and around the world focused

on the development of a diagnostic test for CWD and other TSE’s. The goal of that work is

to develop a diagnostic test that can be performed on a sample that can be collected from a

live animal. Recently, a Canadian company announced progress in the development of a

test that detects a specific protein in the blood of mammals that may indicate, among other

things, infection with a TSE.

CWD remained limited in distribution to Colorado and Wyoming for 20-30 years.

Although it was a new disease and of interest to researchers, effects on the deer and elk

populations were thought to be minimal and there was little concern regarding public health

or environmental issues. Experience with another TSE in the United Kingdom, however,

brought international attention to the prion diseases when BSE (mad cow disease) was

associated with a condition in humans called variant Creutzfeldt-Jakob disease (vCJD).

Although sporadic CJD has occurred in the human population at a rate of approximately 1

per one million people for at least the last century, vCJD was suddenly implicated as a

disease that was transmitted to humans from BSE infected cattle. Whereas sporadic CJD

did not seem to be transmissible person-to-person and generally affected individuals 50

years or older, vCJD affected a younger population, seemed to be transmitted through

eating contaminated meat from BSE affected cattle, and caused some distinguishing

diagnostic characteristics in its victims allowing differentiation from sporadic CJD. As of

December 1, 2003, a total of 153 cases of vCJD had been reported in the world: 143 from

the United Kingdom, six from France, and one each from Canada, Ireland, Italy, and the

United States (note: the Canadian, Irish, and U.S. cases were reported in persons who

resided in the United Kingdom during a key exposure period of the U.K. population to the

BSE agent) (Center for Disease Control, 2004). Concern over direct transmission of TSE

diseases from other animal species to humans suddenly became a public issue. Public

concern over the potential connection between mad cow disease and vCJD surfaced just as

the distribution of CWD in deer and elk was increasing in the United States.

To date, there have been no cases of human prion disease that have been associated with

CWD. Because of differing experiences seen with two more common animal TSEs, BSE

and scrapie, there is a lingering uncertainty about assessing any potential risk that CWD

may pose to humans (Williams et al. 2002a). Scrapie, a TSE disease-affecting sheep, has

been found in sheep populations in many countries for at least 200 years. Although there is

a long history of human exposure to the scrapie infective agent (prion) through handling

sheep and consuming sheep tissues, including brain, there is no evidence that this exposure

presents a risk to human health. In contrast, BSE, as discussed, has been implicated in the

deaths of 153 humans due to vCJD. In the absence of complete information on risk, and in

light of similarities between animal and human TSEs, public health officials and wildlife

management professionals recommend that hunters, meat processors and taxidermists

handling deer and elk carcasses from areas where CWD has been diagnosed should take

measures to avoid exposure to the CWD agent and to other known zoonotic pathogens.

Boning game meat is recommended as an effective way to reduce the potential for


exposure. It is also recommended that hunters not consume the brain, spinal cord, eyes,

spleen, tonsils, or lymph nodes from harvested deer and elk, particularly those harvested

from areas where CWD has been identified in the free ranging populations. These

precautionary measures should serve as a "firewall" to further reduce any human risk from

CWD. ...

snip...full text 96 pages

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

Suggested citation for this article: Belay ED, Maddox RA, Williams
ES, Miller MW, Gambetti P, Schonberger LB. Chronic wasting disease
and potential transmission to humans. Emerg Infect Dis [serial on
the Internet]. 2004 Jun [date cited]. Available from:


Chronic wasting disease (CWD) of deer and elk is endemic in a
tri-corner area of Colorado, Wyoming, and Nebraska, and new foci of
CWD have been detected in other parts of the United States. Although
detection in some areas may be related to increased surveillance,
introduction of CWD due to translocation or natural migration of
animals may account for some new foci of infection. Increasing
spread of CWD has raised concerns about the potential for increasing
human exposure to the CWD agent. The foodborne transmission of
bovine spongiform encephalopathy to humans indicates that the
species barrier may not completely protect humans from animal prion
diseases. Conversion of human prion protein by CWD-associated prions
has been demonstrated in an in vitro cell-free experiment, but
limited investigations have not identified strong evidence for CWD
transmission to humans. More epidemiologic and laboratory studies
are needed to monitor the possibility of such transmissions. ...


in the lab...


######### ##########

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

PMID: 6997404

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

Xavier Bosch

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

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

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

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

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

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

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

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

Norman Foster, director of the Cognitive Disorders Clinic at the
University of Michigan (Ann Arbor, MI, USA), says that current
surveillance of prion disease in people in the USA is inadequate to
detect whether CWD is occurring in human beings; adding that, the
cases that we know about are reassuring, because they do not suggest the
appearance of a new variant of CJD in the USA or atypical features in
patients that might be exposed to CWD. However, until we establish a
system that identifies and analyses a high proportion of suspected prion
disease cases we will not know for sure. The USA should develop a
system modelled on that established in the UK, he points out.

Ali Samii, a neurologist at Seattle VA Medical Center who recently
reported the cases of three 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

SEAC Statement
18th January 2005
Position statement - Chronic wasting disease in UK deer


1. The Food Standards Agency asked SEAC to consider the possible public and animal health implications of chronic wasting disease (CWD), in particular the level of risk posed to consumers of meat from infected animals. The committee also considered the possibility that BSE may be present in UK deer.


2. CWD has emerged as an endemic transmissible spongiform encephalopathy (TSE) in certain captive and free-ranging species of cervid (deer) in some areas of North America. The disease is characterised by weight loss and behavioural changes in infected animals, usually over a period of weeks or months leading to death. CWD has not been found in the UK or elsewhere in Europe. No definitive or suspected cases of transmission of CWD to humans have been reported.

3. SEAC considered a review of the published, and some unpublished, research on CWD, together with surveillance data on TSEs in European cervids and information on UK cervid populations (1).


4. The origins of CWD are unknown. On the basis of epidemiological data, it is highly improbable that CWD originated from the recycling of mammalian protein in processed feed. It has been suggested that CWD may have arisen from transmission and adaptation of scrapie from sheep to cervids, as a result of a spontaneous change of endogenous prion protein (PrP) to an abnormal disease-associated form, or from an unknown source.

5. Data supporting any of these possible origins of CWD are either absent or equivocal. Although CWD could have originated from scrapie, the differing properties of the two prion diseases in strain typing bioassays, whilst limited, do not support this hypothesis. Evidence for multiple strains of CWD is equivocal. It seems most likely that CWD arose from a spontaneous change of endogenous PrP resulting in a disease-associated and laterally-transmissible form of PrP, although direct data to support this hypothesis are lacking.

Host range

6. The known natural hosts for CWD are mule deer (Odocoileus hemionus hemionus), black-tailed deer (Odocoileus hemionus columbianus), white-tailed deer (Odocoileus virginianus) and Rocky Mountain elk (Cervus elaphus nelsoni). The prevalence and geographical distribution of CWD in these species appears to be increasing in North America in a manner which is unlikely to be due simply to increased surveillance.

7. There are no direct data relating to the transmissibility of CWD to UK cervid species. However, comparison of a limited number of PrP codons indicates some homology in the endogenous PrP gene of European and North American cervid species. Thus, the possibility that UK cervids may be susceptible to CWD cannot be excluded, in particular red deer (Cervus elaphus elaphus) which are closely related to elk.

8. There is no evidence to suggest that CWD is present in UK cervids. However, because surveillance in the UK is very limited, a low level prevalence of CWD cannot be ruled out. The committee endorsed the opinion of the European Food Safety Authority on CWD surveillance in the European Union (2004) .

9. Transmission studies using parenteral routes of administration to cattle, sheep and a single goat, together with data from in vitro PrP conversion experiments, suggest that a significant barrier to CWD transmission to these species may exist. No transmission has been evident so far in an on-going oral transmission study in cattle after six years. Furthermore, no signs of infection have been observed from monitoring of cattle co-habiting areas with infected cervids, or in cattle, sheep or goats in close contact with infected cervids in research facilities. Thus, although the data are limited, there is currently no evidence to suggest that CWD can be transmitted naturally to cows, sheep or goats, and it is likely that there is a strong species barrier to such transmission.

Routes of transmission

10. Epidemiological data indicate that lateral transmission between infected and susceptible cervids occurring naturally is sufficiently effective to maintain epidemics in both captive and free-living populations. There is good evidence from studies of cervids inhabiting paddocks previously inhabited by infected animals or contaminated with infected carcases, that CWD can be transmitted laterally between animals via the environment. The precise mechanism of transmission is unclear. It is possible that the infectious agent is shed in the saliva, faeces or urine or as a result of decomposition of infected carcases and transferred to other cervids grazing the contaminated areas. It is also possible that some maternal transmission occurs.

11. There have also been suggestions that the lateral transmission of CWD may be influenced by environmental factors.


12. Information on the pathogenesis of CWD is limited. The data show that, following oral challenge, PrPCWD is first detected in the oral and gut-associated lymphoid tissues before spreading more widely within the lymphoid system and then to the brain. Involvement of the retropharyngeal lymph nodes or tonsils in the pathogenesis may not occur in some elk. At the microscopic level, the nature and distribution of the tissue lesions are similar to those found for scrapie. The available data suggest the pathogenesis of CWD is similar to scrapie.

BSE in UK deer

13. Both captive and free-ranging cervids in the UK may have been exposed to contaminated feed prior to the reinforced mammalian meat and bone meal ban instituted in 1996. A study to look at the potential susceptibility of red deer to BSE has shown no signs of transmission of the disease by the oral route, but it is at a very preliminary stage. Although a theoretical possibility exists, there is no evidence from the very limited surveillance data to suggest that BSE is present in the UK cervid population.

Human health implications

14. Epidemiological data on possible CWD infection of humans are very limited. The possibility that clinical symptoms of CWD in humans differ from those of Creutzfeldt-Jakob Disease (CJD) cannot be excluded. There is no significant difference between the prevalence of CJD in CWD endemic areas and other areas of the world. However, because CJD surveillance in the USA is relatively recent, not all CJD cases may have been identified. Additionally, detection of a small increase in prevalence of such a rare disease is very difficult. Investigation of six cases of prion disease in young people (< 30 years of age) in the USA found no definite causal link with consumption of venison from known CWD endemic areas. The disease characteristics in these cases were indistinguishable from sporadic CJD or Gerstmann-Strδussler-Scheinker syndrome. Likewise, in a study of three hunters (> 54 years of age) diagnosed with sporadic CJD, no link with consumption of venison from CWD endemic areas was found. No causal link was found in an investigation of three men with neurological illnesses who were known to partake in “wild game feasts”. Only one of these subjects was found to have a prion disease and this was also indistinguishable from sporadic CJD.

15. Preliminary results from transmission experiments in transgenic mice expressing human PrP suggest the presence of a significant species barrier to transmission of CWD to humans. However, these findings must be interpreted with caution as they may not accurately predict the human situation. Data from in vitro experiments on conversion of human PrP by disease-associated forms of PrP, including PrPCWD, are equivocal.

16. The committee concluded there is no evidence of transmission of CWD to humans from consumption of venison, and that there may be significant barriers to transmission. Nevertheless, as the data are extremely limited a risk cannot be ruled out should CWD enter UK herds.


17. There is no evidence that CWD (or BSE) is present in the UK cervid population. However, because only limited surveillance is conducted in the cervid population, a low level prevalence of CWD cannot be ruled out. It is recommended that further surveillance of TSEs in UK cervids is conducted.

18. There is no evidence of transmission of CWD to humans from consumption of meat from infected cervids. Although epidemiological and experimental data on potential transmission of CWD are extremely limited, they suggest that there may be a significant species barrier. It would be helpful if further studies were available assessing the potential species barrier for transmission to humans.

19. Although limited, there is no evidence CWD can be transmitted to cattle, sheep or goats by natural means.

20. In summary, it appears that CWD currently poses relatively little risk to human health, or to the health of cattle, sheep or goats in the UK. Nevertheless, as a risk cannot be excluded a watching brief should be maintained.

January 2005


Chronic Wasting Disease in UK deer

Alan Harvey (FSA).Wildlife Information Network.


Annex 1 (pdf)

Annex 2 (pdf):

Annex 3 (incl. appendices) (pdf)

Annex 4 (pdf)

Annex 5 (pdf):

ALL of these false reassurances we have heard time and time again, and ALL have been proven wrong.

AS with the BSE TO HUMANS AND BSE to GOAT. IT was always it never happend under natural conditions, just in the lab, so not to worry. NOW WE HAVE TO WORRY;


Evidence For CWD/Mad Cow/TSEs
In The Environment

In Confidence - Perceptions of unconventional slow virus diseases
of animals in the USA - APRIL-MAY 1989 - G A H Wells

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

-------- Original Message -------- Subject: DOCKET-- 03D-0186 -- FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal Feed; Availability
Date: Fri, 16 May 2003 11:47:37 -0500
From: "Terry S. Singeltary Sr."

Greetings FDA,i would kindly like to comment on;Docket 03D-0186FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal Feed; AvailabilitySeveral factors on this apparent voluntary proposal disturbs me greatly,please allow me to point them out;1. MY first point is the failure of the partial ruminant-to-ruminant feedban of 8/4/97. this partial and voluntary feed ban of some ruminantmaterials being fed back to cattle is terribly flawed. without the_total_ and _mandatory_ ban of all ruminant materials being fedback to ruminants including cattle, sheep, goat, deer, elk and mink,chickens, fish (all farmed animals for human/animal consumption),this half ass measure will fail terribly, as in the past decades... 2. WHAT about sub-clinical TSE in deer and elk? with the recentfindings of deer fawns being infected with CWD, how many couldpossibly be sub-clinically infected. until we have a rapid TSE test toassure us that all deer/elk are free of disease (clinical and sub-clinical),we must ban not only documented CWD infected deer/elk, but healthyones as well. it this is not done, they system will fail...3. WE must ban not only CNS (SRMs specified risk materials),but ALL tissues. recent new and old findings support infectivityin the rump or ass muscle. wether it be low or high, accumulationwill play a crucial role in TSEs.4. THERE are and have been for some time many TSEs in theUSA. TME in mink, Scrapie in Sheep and Goats, and unidentifiedTSE in USA cattle. all this has been proven, but the TSE in USAcattle has been totally ignored for decades. i will document thisdata below in my references.5. UNTIL we ban all ruminant by-products from being fed backto ALL ruminants, until we rapid TSE test (not only deer/elk) butcattle in sufficient numbers to find (1 million rapid TSE test inUSA cattle annually for 5 years), any partial measures such as theones proposed while ignoring sub-clinical TSEs and not rapid TSEtesting cattle, not closing down feed mills that continue to violate theFDA's BSE feed regulation (21 CFR 589.2000) and not makingfreely available those violations, will only continue to spread theseTSE mad cow agents in the USA. I am curious what we willcall a phenotype in a species that is mixed with who knowshow many strains of scrapie, who knows what strain or how manystrains of TSE in USA cattle, and the CWD in deer and elk (notelling how many strains there), but all of this has been renderedfor animal feeds in the USA for decades. it will get interesting oncesomeone starts looking in all species, including humans here in theUSA, but this has yet to happen... 6. IT is paramount that CJD be made reportable in every state(especially ''sporadic'' cjd), and that a CJD Questionnaire mustbe issued to every family of a victim of TSE. only checking deathcertificates will not be sufficient. this has been proven as well(see below HISTORY OF CJD -- CJD QUESTIONNAIRE)7. WE must learn from our past mistakes, not continue to makethe same mistakes... REFERENCES


Oral transmission and early lymphoid tropism of chronic wasting disease
PrPres in mule deer fawns (Odocoileus hemionus )
Christina J. Sigurdson1, Elizabeth S. Williams2, Michael W. Miller3,
Terry R. Spraker1,4, Katherine I. O'Rourke5 and Edward A. Hoover1

Department of Pathology, College of Veterinary Medicine and Biomedical
Sciences, Colorado State University, Fort Collins, CO 80523- 1671, USA1
Department of Veterinary Sciences, University of Wyoming, 1174 Snowy
Range Road, University of Wyoming, Laramie, WY 82070, USA 2
Colorado Division of Wildlife, Wildlife Research Center, 317 West
Prospect Road, Fort Collins, CO 80526-2097, USA3
Colorado State University Veterinary Diagnostic Laboratory, 300 West
Drake Road, Fort Collins, CO 80523-1671, USA4
Animal Disease Research Unit, Agricultural Research Service, US
Department of Agriculture, 337 Bustad Hall, Washington State University,
Pullman, WA 99164-7030, USA5

Author for correspondence: Edward Hoover.Fax +1 970 491 0523. e-mail

Mule deer fawns (Odocoileus hemionus) were inoculated orally with a
brain homogenate prepared from mule deer with naturally occurring
chronic wasting disease (CWD), a prion-induced transmissible spongiform
encephalopathy. Fawns were necropsied and examined for PrP res, the
abnormal prion protein isoform, at 10, 42, 53, 77, 78 and 80 days
post-inoculation (p.i.) using an immunohistochemistry assay modified to
enhance sensitivity. PrPres was detected in alimentary-tract-associated
lymphoid tissues (one or more of the following: retropharyngeal lymph
node, tonsil, Peyer's patch and ileocaecal lymph node) as early as 42
days p.i. and in all fawns examined thereafter (53 to 80 days p.i.). No
PrPres staining was detected in lymphoid tissue of three control fawns
receiving a control brain inoculum, nor was PrPres detectable in neural
tissue of any fawn. PrPres-specific staining was markedly enhanced by
sequential tissue treatment with formic acid, proteinase K and hydrated
autoclaving prior to immunohistochemical staining with monoclonal
antibody F89/160.1.5. These results indicate that CWD PrP res can be
detected in lymphoid tissues draining the alimentary tract within a few
weeks after oral exposure to infectious prions and may reflect the
initial pathway of CWD infection in deer. The rapid infection of deer
fawns following exposure by the most plausible natural route is
consistent with the efficient horizontal transmission of CWD in nature
and enables accelerated studies of transmission and pathogenesis in the
native species.


These results indicate that mule deer fawns develop detectable PrP res
after oral exposure to an inoculum containing CWD prions. In the
earliest post-exposure period, CWD PrPres was traced to the lymphoid
tissues draining the oral and intestinal mucosa (i.e. the
retropharyngeal lymph nodes, tonsil, ileal Peyer's patches and
ileocaecal lymph nodes), which probably received the highest initial
exposure to the inoculum. Hadlow et al. (1982) demonstrated scrapie
agent in the tonsil, retropharyngeal and mesenteric lymph nodes, ileum
and spleen in a 10-month-old naturally infected lamb by mouse bioassay.
Eight of nine sheep had infectivity in the retropharyngeal lymph node.
He concluded that the tissue distribution suggested primary infection
via the gastrointestinal tract. The tissue distribution of PrPres in the
early stages of infection in the fawns is strikingly similar to that
seen in naturally infected sheep with scrapie. These findings support
oral exposure as a natural route of CWD infection in deer and support
oral inoculation as a reasonable exposure route for experimental studies
of CWD.




April 9, 2001 WARNING LETTER


Brian J. Raymond, Owner
Sandy Lake Mills
26 Mill Street
P.O. Box 117
Sandy Lake, PA 16145

Tel: 215-597-4390

Dear Mr. Raymond:

Food and Drug Administration Investigator Gregory E. Beichner conducted
an inspection of your animal feed manufacturing operation, located in
Sandy Lake, Pennsylvania, on March 23,
2001, and determined that your firm manufactures animal feeds including
feeds containing prohibited materials. The inspection found significant
deviations from the requirements set forth in
Title 21, code of Federal Regulations, part 589.2000 - Animal Proteins
Prohibited in Ruminant Feed. The regulation is intended to prevent the
establishment and amplification of Bovine Spongiform Encephalopathy
(BSE) . Such deviations cause products being manufactured at this
facility to be misbranded within the meaning of Section 403(f), of the
Federal Food, Drug, and Cosmetic
Act (the Act).

Our investigation found failure to label your
swine feed with the required cautionary statement "Do Not Feed to cattle
or other Ruminants" The FDA suggests that the statement be
by different type-size or color or other means of highlighting the
statement so that it is easily noticed by a purchaser.

In addition, we note that you are using approximately 140 pounds of
cracked corn to flush your mixer used in the manufacture of animal
feeds containing prohibited material. This
flushed material is fed to wild game including deer, a ruminant animal.
Feed material which may potentially contain prohibited material should
not be fed to ruminant animals which may become part of the food chain.

The above is not intended to be an all-inclusive list of deviations from
the regulations. As a manufacturer of materials intended for animal
feed use, you are responsible for assuring that your overall operation
and the products you manufacture and distribute are in compliance with
the law. We have enclosed a copy of FDA's Small Entity Compliance Guide
to assist you with complying with the regulation... blah, blah, blah...


full text;

Guidance for Industry: Use of Material From Deer and Elk In Animal Feed

Terry S. Singeltary Sr.
Vol #:

From: TSS ()
Subject: Low frequency of PrP genotype 225SF among free-ranging mule deer (Odocoileus hemionus) with chronic wasting disease
Date: July 20, 2005 at 1:17 pm PST

Low frequency of PrP genotype 225SF among free-ranging mule deer (Odocoileus hemionus) with chronic wasting disease

Jean E. Jewell1, Mary M. Conner2, Lisa L. Wolfe3, Michael W. Miller3 and Elizabeth S. Williams1,

1 Department of Veterinary Sciences, University of Wyoming, Wyoming State Veterinary Laboratory, 1174 Snowy Range Road, Laramie, WY 82070, USA
2 Department of Forest, Range, and Wildlife Sciences, Utah State University, Logan, UT 84322-5230, USA
3 Colorado Division of Wildlife, Wildlife Research Center, Fort Collins, CO 80526-2097, USA

Jean E. Jewell

The prion protein (PrP) gene was characterized in 1482 free-ranging mule deer (Odocoileus hemionus) from Wyoming and Colorado. Using DNA sequences from 363 deer, dimorphisms at codons 20 (aspartate/glycine) and 225 [serine (S)/phenylalanine (F)] were found; silent changes occurred at codons 131 (tyrosine) and 247 (isoleucine). The remaining samples were surveyed for codon 225 genotype and all were characterized for chronic wasting disease (CWD) infection status. A total of 112 deer with the genotype 225SF or FF were found, of which one was CWD-positive; 1370 were 225SS, with 289 positive for CWD. Among CWD-negative deer, the frequency of 225SF/FF genotypes was 9·3 % but among CWD-positive deer it was only 0·3 %. For all samples combined, CWD status was not independent of codon 225 genotype (P<0·0001). The odds that a deer of the 225SS genotype was CWD-infected were 30 times greater (95 % confidence intervals=4–213) than for a 225SF deer. The proportion of 225SF animals in sampled subpopulations varied from 0 to 18 %; the CWD prevalence varied from 0 to 25 %. However, no relationship was observed between genotype frequency and CWD prevalence in different areas. The PrP sequences of experimentally infected mule deer were analysed from pre-existing projects and 10 animals were found with 225SF genotypes, all of which were positive for CWD. Data available from some of these animals suggest that the 225SF genotype could be associated with longer incubation periods in CWD infection compared with the 225SS genotype.

Published online ahead of print on 27 May 2005 as DOI 10.1099/vir.0.81077-0.

Deceased 29 December 2004.

>>>225SF genotype could be associated with longer incubation periods in CWD infection compared with the 225SS genotype.<<<

makes those oral transmissions studies of cwd to cattle that much more interesting. or did the kill that study?

Title: Experimental Transmission of Chronic Wasting Disease (Cwd Mule Deer) Agent to Cattle by Intracerebral Route


Hamir, Amirali
Kunkle, Robert - bob
Cutlip, Randall - ARS RETIRED
Miller, Janice - ARS RETIRED
O'Rourke, Katherine
Williams, Elizabeth - UNIVERSITY OF WYOMING
Chaplin, Melanie - VET SERVICES AGENCY, UK
Richt, Juergen

Submitted to: Journal Of Veterinary Diagnostic Investigation
Publication Acceptance Date: January 3, 2005
Publication Date: N/A

Interpretive Summary: This communication reports final observations on experimental transmission of chronic wasting disease (CWD) from mule deer to cattle. Thirteen calves were inoculated into the brain with brain suspension from mule deer naturally affected with CWD. Three other calves were kept as uninoculated controls. The experiment was terminated 6 years post inoculation (PI). During that time, abnormal prion protein was demonstrated in the brain and spinal cord of 5 cattle by laboratory tests. However, consistent clinical signs and microscopic changes were not seen in any of these cattle. Age related changes were seen in both inoculated and control cattle. Findings of this study show that only 38% of the inoculated cattle were positive for CWD agent. Although inoculation directly into the brain is an unnatural route of exposure, and is the most severe challenge possible, this experiment shows that CWD transmission in cattle could have long incubation periods (up to 5 years). This finding suggests that oral exposure of cattle to CWD agent, a more natural potential route of exposure, would require not only a much larger dose of inoculum, but also, may not result in amplification of CWD agent within brain and spinal cord tissues during the normal lifespan of cattle. It is possible that a second bovine passage of material (cattle brain infected with CWD) from this study may result in a larger incidence of affected cattle with a shortened incubation time, and may produce different clinical and pathological findings. Such a study is now in progress. Also, experimental inoculations of cattle with CWD isolates from white-tailed deer and elk are needed to compare findings with the present study and these studies will be initiated in the near future. Impact: Results of this study show that although cattle inoculated directly into the brain with CWD succumb to the disease, the attack rate was rather small (38%) with this unnatural route of transmission. It is speculated that the oral route of infection may not result in replication of the agent during normal lifespan of cattle.
Technical Abstract: This communication reports final observations on experimental transmission of chronic wasting disease (CWD) from mule deer to cattle by the intracerebral route. Thirteen calves were inoculated intracerebrally with brain suspension from mule deer naturally affected with CWD. Three other calves were kept as uninoculated controls. The experiment was terminated 6 years post inoculation (PI). During that time, abnormal prion protein (PrPres) was demonstrated in the central nervous system (CNS) of 5 cattle by both immunohistochemistry (IHC) and Western blot (WB). However, microscopic lesions suggestive of spongiform encephalopathy in the brains of these PrPres positive animals were subtle in 3 cases and absent in 2 cases. The 3 uninoculated control cattle and 8 other inoculated animals euthanized during this time did not have PrPres in their CNS. Degenerative changes indicative of neuroaxonal dystrophy (NAD) were seen in dorsal medulla oblongata and appeared to be related to advancing age in both inoculated and control cattle. Analysis of the gene encoding bovine PRNP revealed homozygosity for alleles encoding 6 octapeptide repeats, serine (S) at codon 46 and S at codon 146 in all samples. Findings of this study show that although PrPres amplification occurred following direct inoculation into the brain, none of the affected animals had classic histopathologic lesions of spongiform encephalopathy. Furthermore, only 38% of the inoculated cattle demonstrated amplification of PrPres. Although intracerebral inoculation is an unnatural route of exposure, and is the most severe challenge possible, this experiment shows that CWD transmission in cattle could have long incubation periods (up to 5 years). This finding suggests that oral exposure of cattle to CWD agent, a more natural potential route of exposure, would require not only a much larger dose of inoculum, but also, may not result in amplification of PrPres within CNS tissues during the normal lifespan of cattle.

HELLO Tim Feldner,

please don't be to complacent on the real potential of CWD transmission to humans. they said this about BSE of cattle to humans, as now documented nvCJD. but this is only one strain. we now have BASE in cattle that seems to be very similar to some of the sporadic CJDs;

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 Ά, 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 ΆIstituto 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.


WITH all the many documented TSEs in many different species in the USA.

WITH all rendered and fed back to animals for human and animal consumption for decades.

WITH the fact already proven that USA SCRAPIE TO USA CATTLE does NOT produce UK BSE, but something different;

SCRAPIE to humans, why not;

Adaptation of the bovine spongiform encephalopathy agent to primates
and comparison with Creutzfeldt- Jakob disease: Implications for
human health

THE findings from Corinne Ida Lasmιzas*, [dagger] , Jean-Guy Fournier*,
Virginie Nouvel*,

Hermann Boe*, Domνnνque Marcι*, Franηois Lamoury*, Nicolas Kopp [Dagger

] , Jean-Jacques Hauw§, James IronsideΆ, Moira Bruce [||] , Dominique

Dormont*, and Jean-Philippe Deslys* et al, that The agent responsible
for French iatrogenic growth hormone-linked CJD taken as a control is
very different from vCJD but is similar to that found in one case of
sporadic CJD and one sheep scrapie isolate;

HOW MANY of the sporadic CJDs in the USA are of animal origin, either directly or indirectly via 2nd, or 3rd passage via the medical or surgical arena and OR blood transfusion ???

HOW many of the sporadic CJDs i.e. 85%+ of all _documented_ cases, how many of these are truely sporadic/spontaneous without any route or source, if one believes in spontaneous TSE at all ???

Terry S. Singeltary Sr.

P.O. Box 42

Bacliff, Texas USA 77518

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