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From: TSS ()
Date: November 17, 2006 at 6:58 pm PST


November 16, 2006

Senator Carol A. Roessler and

Representative Suzanne Jeskewitz, Co-chairpersons

Joint Legislative Audit Committee

State Capitol

Madison, Wisconsin 53702

Dear Senator Roessler and Representative Jeskewitz:

As requested by the Joint Legislative Audit Committee, we have completed an evaluation of state

efforts to manage chronic wasting disease (CWD), a fatal neurological disease of deer. The

Department of Natural Resources (DNR) is responsible for coordinating CWD management in the

wild deer population. Farm-raised deer are the responsibility of the Department of Agriculture,

Trade and Consumer Protection (DATCP). The Wisconsin Veterinary Diagnostic Laboratory

provides CWD testing and carcass disposal services, while the Department of Health and Family

Services investigates possible effects on human health. Through fiscal year (FY) 2005-06, these

agencies spent $32.3 million managing the disease.

DNR has developed several strategies to manage CWD, within the geographic areas in which

infected deer are known to live, which are known as CWD zones. They include altering the length

and rules of hunting seasons, establishing a ban on baiting and feeding deer, using sharpshooters,

and creating monetary incentives for hunters to shoot more deer.

To date, DNR’s efforts to eradicate CWD in the free-ranging deer population have not been

effective. Neither the estimated number of deer in CWD zones nor the percentage infected with

CWD has decreased. In addition, fewer deer have been killed in the CWD zones: the number

declined from 23.1 deer per square mile in the 2003 hunting season to 17.4 deer per square mile in

the 2005 hunting season.

In an October 2006 report to the Natural Resources Board, DNR conceded the need to modify its

management efforts to more effectively address CWD. We include options for DNR and the

Legislature to more effectively address the disease and control costs in the future.

We appreciate the courtesy and cooperation extended to us by staff of DNR, other state agencies,

and interest groups. DNR’s response follows the report.

Respectfully submitted,

Janice Mueller

State Auditor



Monitoring Related Human Diseases

The primary CWD-related activity undertaken by DHFS has been to

monitor cases of Creutzfeldt-Jakob disease, although to date there is

no evidence to suggest that eating CWD-infected venison can cause

it or any other disease in humans. However, Bovine Spongiform

Encephalopathy, which is better known as “mad cow disease” and is

caused by prions, has been linked to a new variant form of

Creutzfeldt-Jakob disease that was transmitted to humans in Great

Britain and other European countries.

In 2002, as a precautionary measure, DHFS convened a workgroup

of 13 experts, primarily neurologists and neuropathologists, to

determine how to monitor any possible connection between CWD

and Creutzfeldt-Jakob disease. Like CWD in deer, Creutzfeldt-Jakob

disease in humans is confirmed through post-mortem testing.

However, in July 2004 the workgroup informed all Wisconsin

neurologists that it had developed clinical criteria for identifying

suspected cases of Creutzfeldt-Jakob disease for reporting to DHFS.

To investigate reported cases, DHFS collects information from

medical records, autopsy results, and details from family members

about the person’s background, including hunting history and

whether the person ate venison. DHFS has also reviewed Wisconsin

death certificates going back to 1997. By identifying cases that differ

from expected frequencies or demographic characteristics, DHFS

staff hope to determine whether occurrences of Creutzfeldt-Jakob

disease were likely to have been caused by exposure to CWD.

The federal Centers for Disease Control and Prevention indicate the

normal incidence of Creutzfeldt-Jakob disease in the United States is

There is no evidence, to

date, that CWD causes

human illness.


within the range of one to two cases per million per year, and the

disease typically occurs sporadically. As shown in Table 40, cases in

Wisconsin have not exceeded the normal incidence during the

period reviewed by DHFS.

Table 40

Annual Incidence of Creutzfeldt-Jakob Disease in Wisconsin

Calendar Year

Incidence per Million


1997 1.2

1998 1.3

1999 0.8

2000 0.4

2001 0.9

2002 1.5

2003 1.1

2004 1.1

2005 2.0

It was widely reported in 2002 that three hunters who

participated in wild game feasts that included venison had

contracted fatal illnesses in the 1990s that were possibly linked

to CWD-positive deer. A joint investigation by DHFS and the

Centers for Disease Control and Prevention concluded that only

one of these individuals died of Creutzfeldt-Jakob disease, and

its onset was not linked to consumption of venison but was the

typical sporadic form. The other two individuals died of

unrelated causes.

The joint investigation also inquired into the sources of venison

served at the wild game feasts, as well as the health of the other feast

participants. The investigators determined that the venison was

primarily from Wisconsin. Although the three deceased hunters had

also brought game from the western United States back to

Wisconsin, CWD was not known to be endemic where or when their

hunting activities took place. Investigators also determined that 4 of

31 other individuals who reported attending the wild game feasts

were deceased, but none had a cause of death associated with

Creutzfeldt-Jakob disease, and none of the living participants had

any signs or symptoms consistent with the disease.

snip...famous last words...tss

From: TSS (
Date: September 30, 2002 at 7:06 am PST

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

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

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

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

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

Sunday, November 10, 2002 6:26 PM ......snip........end..............TSS


A. Aguzzi - Chronic Wasting Disease (CWD) also needs to be addressed. Most
serious because of rapid horizontal spread and higher prevalence than BSE in
UK, up to 15% in some populations. Also may be a risk to humans - evidence
that it is not dangerous to humans is thin.

1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype
of 'UNKNOWN' strain growing. ...

There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.

He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.

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.


76 pages on hound study;

The spongiform changes were not pathognomonic (ie.
conclusive proof) for prion disease, as they were atypical,
being largely present in white matter rather than grey matter in
the brain and spinal cord. However, Tony Scott, then head of
electron microscopy work on TSEs, had no doubt that these
SAFs were genuine and that these hounds therefore must have
had a scrapie-like disease. I reviewed all the sections
myself (original notes appended) and although the pathology
was not typical, I could not exclude the possibility that this was
a scrapie-like disorder, as white matter vacuolation is seen
in TSEs and Wallerian degeneration was also present in the
white matter of the hounds, another feature of scrapie.

38.I reviewed the literature on hound neuropathology, and
discovered that micrographs and descriptive neuropathology from
papers on 'hound ataxia' mirrored those in material from
Robert Higgins' hound survey. Dr Tony Palmer (Cambridge) had
done much of this work, and I obtained original sections
from hound ataxia cases from him. This enabled me provisionally to
conclude that Robert Higgins had in all probability detected
hound ataxia, but also that hound ataxia itself was possibly a
TSE. Gerald Wells confirmed in 'blind' examination of single
restricted microscopic fields that there was no distinction
between the white matter vacuolation present in BSE and
scrapie cases, and that occurring in hound ataxia and the hound
survey cases.

39.Hound ataxia had reportedly been occurring since the 1930's,
and a known risk factor for its development was the feeding
to hounds of downer cows, and particularly bovine offal.
Circumstantial evidence suggests that bovine offal may also be
causal in FSE, and TME in mink. Despite the inconclusive
nature of the neuropathology, it was clearly evident that this
putative canine spongiform encephalopathy merited further

40.The inconclusive results in hounds were never confirmed,
nor was the link with hound ataxia pursued. I telephoned Robert
Higgins six years after he first sent the slides to CVL.
I was informed that despite his submitting a yearly report to the
CVO including the suggestion that the hound work be continued,
no further work had been done since 1991. This was
surprising, to say the very least.

41.The hound work could have provided valuable evidence
that a scrapie-like agent may have been present in cattle offal long
before the BSE epidemic was recognised. The MAFF hound
survey remains unpublished.

Histopathological support to various other published
MAFF experiments

42.These included neuropathological examination of material
from experiments studying the attempted transmission of BSE to
chickens and pigs (CVL 1991) and to mice (RVC 1994).

It was thought likely that at least some, and probably all, of the cases
in zoo animals were caused by the BSE agent. Strong support for this
hypothesis came from the findings of Bruce and others (1994)
( Bruce, M.E., Chree, A., McConnell, I., Foster, J., Pearson, G. &
Fraser, H. (1994) Transmission of bovine spongiform encephalopathy and
scrapie to mice: strain variation and species barrier. Philosophical
Transactions of the Royal Society B 343, 405-411: J/PTRSL/343/405
), who demonstrated that the pattern of variation in incubation period
and lesion profile in six strains of mice inoculated with brain
homogenates from an affected kudu and the nyala, was similar to that
seen when this panel of mouse strains was inoculated with brain from
cattle with BSE. The affected zoo bovids were all from herds that were
exposed to feeds that were likely to have contained contaminated
ruminant-derived protein and the zoo felids had been exposed, if only
occasionally in some cases, to tissues from cattle unfit for human



Department for Environment,
Food & Rural Affairs

Area 307, London, SW1P 4PQ
Telephone: 0207 904 6000
Direct line: 0207 904 6287


Mr T S Singeltary
P.O. Box 42
USA 77518

21 November 2001

Dear Mr Singeltary TSE IN HOUNDS

Thank you for e-mail regarding the hounds survey. I am sorry for the long delay in responding.

As you note, the hound survey remains unpublished. However the Spongiform Encephalopathy Advisory Committee (SEAC), the UK Government's independent Advisory Committee on all aspects related to BSE-like disease, gave the hound study detailed consideration at their meeting in January 1994. As a summary of this meeting published in the BSE inquiry noted, the Committee were clearly concerned about the work that had been carried out, concluding that there had clearly been problems with it, particularly the control on the histology, and that it was more or less inconclusive. However was agreed that there should be a re-evaluation of the pathological material in the study.

Later, at their meeting in June 95, The Committee re-evaluated the hound study to see if any useful results could be gained from it. The Chairman concluded that there were varying opinions within the Committee on further work. It did not suggest any further transmission studies and thought that the lack of clinical data was a major weakness.

Overall, it is clear that SEAC had major concerns about the survey as conducted. As a result it is likely that the authors felt that it would not stand up to peer review and hence it was never published. As noted above, and in the detailed minutes of the SEAC meeting in June 95, SEAC considered whether additional work should be performed to examine dogs for evidence of TSE infection. Although the Committee had mixed views about the merits of conducting further work, the Chairman noted that when the Southwood Committee made their recommendation to complete an assessment of possible spongiform disease in dogs, no TSEs had been identified in other species and hence dogs were perceived as a high risk population and worthy of study. However subsequent to the original recommendation, made in 1990, a number of other species had been identified with TSE ( e.g. cats) so a study in hounds was less critical. For more details see-

As this study remains unpublished, my understanding is that the ownership of the data essentially remains with the original researchers. Thus unfortunately, I am unable to help with your request to supply information on the hound survey directly. My only suggestion is that you contact one of the researchers originally involved in the project, such as Gerald Wells. He can be contacted at the following address.

Dr Gerald Wells, Veterinary Laboratories Agency, New Haw, Addlestone, Surrey, KT 15 3NB, UK

You may also wish to be aware that since November 1994 all suspected cases of spongiform encephalopathy in animals and poultry were made notifiable. Hence since that date there has been a requirement for vets to report any suspect SE in dogs for further investigation. To date there has never been positive identification of a TSE in a dog.

I hope this is helpful

Yours sincerely 4



3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse Models
Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western Reserve University
Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain discovered recently in Italy, and similar or different atypical BSE cases were also reported in other countries. The infectivity and phenotypes of these atypical BSE strains in humans are unknown. In collaboration with Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have inoculated transgenic mice expressing human prion protein with brain homogenates from BASE or BSE infected cattle. Our data shows that about half of the BASE-inoculated mice became infected with an average incubation time of about 19 months; in contrast, none of the BSE-inoculated mice appear to be infected after more than 2 years. These results indicate that BASE is transmissible to humans and suggest that BASE is more virulent than classical BSE in humans.

[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine
Spongiform Encephalopathy (BSE) Singeltary submission


Research Project: Study of Atypical Bse

Location: Virus and Prion Diseases of Livestock

Project Number: 3625-32000-073-07

Project Type: Specific C/A

Start Date: Sep 15, 2004

End Date: Sep 14, 2007


The objective of this cooperative research project with Dr. Maria Caramelli from the Italian BSE Reference Laboratory in Turin, Italy, is to

conduct comparative studies with the U.S. bovine spongiform encephalopathy (BSE) isolate and the atypical BSE isolates identified in Italy.

The studies will cover the following areas: 1. Evaluation of present diagnostics tools used in the U.S. for the detection of atypical BSE cases. 2.

Molecular comparison of the U.S. BSE isolate and other typical BSE isolates with atypical BSE cases. 3. Studies on transmissibility and tissue

distribution of atypical BSE isolates in cattle and other species.


This project will be done as a Specific Cooperative Agreement with the Italian BSE Reference Laboratory, Istituto

Zooprofilattico Sperimentale del Piemonte, in Turin, Italy. It is essential for the U.S. BSE surveillance program to

analyze the effectiveness of the U.S diagnostic tools for detection of atypical cases of BSE. Molecular comparisons of

the U.S. BSE isolate with atypical BSE isolates will provide further characterization of the U.S. BSE isolate.

Transmission studies are already underway using brain homogenates from atypical BSE cases into mice, cattle and

sheep. It will be critical to see whether the atypical BSE isolates behave similarly to typical BSE isolates in terms of

transmissibility and disease pathogenesis. If transmission occurs, tissue distribution comparisons will be made between

cattle infected with the atypical BSE isolate and the U.S. BSE isolate. Differences in tissue distribution could require

new regulations regarding specific risk material (SRM) removal.

Page 5 of 98



[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk
Materials for Human Food and Requirement for the Disposition of
Non-Ambulatory Disabled Cattle



Full Text

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734.




Terry S. Singeltary Sr.

P.O. Box 42

Bacliff, Texas USA 77518

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