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From: TSS ()
Subject: Monitoring the Potential Transmission of Chronic Wasting Disease to Humans Using a Hunter Registry Database in Wyoming
Date: August 30, 2007 at 6:46 pm PST


Monitoring the Potential Transmission of Chronic Wasting Disease to Humans Using a Hunter Registry Database in Wyoming

K. N. Weidenbach1, A. Curns2, E. Belay2, R. Maddox2, R. Holman2, L.
Schonberger2; 1Wyoming Department of Health, Cheyenne, WY, 2Division of
Viral and Rickettsial Diseases, Centers for Disease Control and Prevention,
Atlanta, GA.

Background: Chronic wasting disease (CWD) occurs among North American
deer and elk and has been identified in 10 states, including Wyoming, and
two Canadian provinces. The Wyoming Department of Health (WDH), in
collaboration with the Centers for Disease Control and Prevention and the
Wyoming Game and Fish Department, has established a “hunter database”
to monitor the potential transmission of CWD to humans.

Methods: Annually hunters register to purchase a license to hunt deer
and elk in Wyoming. Hunters registering at least once since 1996 but not
registering within the 2 most recently available years are cross-checked
the National Death Index (NDI) to assess the hunters’ mortality status and
cause of death. CJD deaths found in the Wyoming mortality database or
reported to the WDH are cross-checked with the hunter database. Family
members of hunters who die of CJD are interviewed to assess possible CJD
risk factors and hunting-related exposures.

Results: The WDH hunter database contains 829,943 records of licenses
purchased from 1996 through 2004, representing 266,535 individual hunters;
175,130 were not Wyoming residents, 246,672 were males, and the median
age was 43 years. A total of 91,725 records have been cross-checked with
NDI for the years 1996 through 2002 and 2,573 hunters were confirmed as
deceased, including one with CJD. An additional hunter who died of CJD
in 2004 was reported to WDH. Autopsy findings for one CJD case were
consistent with classic CJD and no autopsy or biopsy was performed for the
second case. Conservatively, there were an estimated 1.5 million personyears
of follow-up through 2004 among hunters who were all =12 years of
age and had sufficient identifying information. The occurrence of 2 CJD
cases among these hunters is not unexpected.

Conclusions: The hunter registry is a valuable tool for monitoring the
potential transmission of CWD to humans by determining the incidence
of CJD among hunters in the database and provides a basis for further
epidemiologic and laboratory investigation of individual CJD cases. One
of two identified CJD cases did not have an autopsy, illustrating the need
to increase efforts to arrange for autopsies among clinically diagnosed or
suspected CJD cases. ...

Slide Session 11
molecular epidemiology Around the globe:
Implications for Intervention
Imperial A
Monday, March 20, 2006, 1:15 pm - 2:45 pm

THIS is why it should be mandatory for all human TSE to be reportable in every state of all ages, and most importantly, a written CJD questionnaire asking questions pertaining to these very real and very many potential routes and sources of CWD, and all human and animal TSE. IT would have been interesting to have known if any of these hunters that died from CJD in Wyoming were VV1 CJD ???

CDC - Chronic Wasting Disease and Potential Transmission to HumansExploring
the zoonotic potential of chronic wasting disease in Wyoming: creating a
hunter registry [abstract #70648].

Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease
creates a new prion strain

Date: August 25, 2007 at 12:42 pm PST


In this study, the strain-dependent traits of sCJDMM1
prions were inherited through cross-sequence
transmission without any modification. The
humanized mice with 129V/V produced type 1 PrPres
after inoculation with sCJD-MM1 prions. Because
sCJD-VV1 cases are extremely rare (at most 1-2%
of the total number of sCJD cases) and characterized
by early onset (mean age at onset: 39.3 years) (5),


our results raise the possibility that CJD cases
classified as VV1 may include cases caused by
iatrogenic transmission of sCJD-MM1 prions or
food-borne infection by type 1 prions from animals,
e.g., chronic wasting disease prions in cervid. In fact,
two CJD-VV1 patients who hunted deer or
consumed venison have been reported (40, 41). The
results of the present study emphasize the need for
traceback studies and careful re-examination of the
biochemical properties of sCJD-VV1 prions.


In conclusion, cross-sequence transmission of
sCJD-VV2 prions generates a new prion strain with
altered conformational properties and disease
phenotypes as p-dCJD prions. Furthermore, the
newly generated prions have unique transmissibility
including the traceback phenomenon. In the future, if
atypical prion strains emerge through cross-sequence
transmission, especially from animals, traceback
studies will enable us to identify the origin of the



Re: Colorado Surveillance Program for Chronic Wasting Disease
Transmission to Humans (TWO SUSPECT CASES)

snip...full text ;

CWD experts address first meeting of advisory committee



> Tracy Kedzierski followed with a report about the CJD Foundation Family Questionnaire

> which she works tirelessly at conducting.

thanks tracy, glad it finally got done. we know how 'tirelessly' you must have worked ;-)

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...


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

The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly
Prion Diseases by Philip Yam

Philip Yam The Pathological Protein Mad Cow, Chronic Wasting, and Other
Deadly Prion Diseases 2003. Hardcover, 285 pp. Euro 29.95 (net price);
ã21.00; $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:
[log in to unmask]
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.



YOUR SPORADIC, YOUR SPONTANEOUS, in short, your expendable. ...

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

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

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

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





MARCH 26, 2003

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

disease in the United States

Email Terry S. Singeltary:

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to

comment on the CDC's attempts to monitor the occurrence of emerging

forms of CJD. Asante, Collinge et al [1] have reported that BSE

transmission to the 129-methionine genotype can lead to an alternate

phenotype that is indistinguishable from type 2 PrPSc, the commonest

sporadic CJD. However, CJD and all human TSEs are not reportable

nationally. CJD and all human TSEs must be made reportable in every

state and internationally. I hope that the CDC does not continue to

expect us to still believe that the 85%+ of all CJD cases which are

sporadic are all spontaneous, without route/source. We have many TSEs in

the USA in both animal and man. CWD in deer/elk is spreading rapidly and

CWD does transmit to mink, ferret, cattle, and squirrel monkey by

intracerebral inoculation. With the known incubation periods in other

TSEs, oral transmission studies of CWD may take much longer. Every

victim/family of CJD/TSEs should be asked about route and source of this

agent. To prolong this will only spread the agent and needlessly expose

others. In light of the findings of Asante and Collinge et al, there

should be drastic measures to safeguard the medical and surgical arena

from sporadic CJDs and all human TSEs. I only ponder how many sporadic

CJDs in the USA are type 2 PrPSc?

Copyright © 2003 Published by Elsevier Ltd.

Tracking spongiform encephalopathies in North America

Xavier Bosch

Available online 29 July 2003.

Volume 3, Issue 8, August 2003, Page 463

“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.”


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