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From: TSS (
Subject: GAO September 2004 EMERGING INFECTIOUS DISEASE Review of State and Federal Disease Surveillance Efforts GAO-04-877
Date: November 1, 2004 at 12:16 pm PST

-------- Original Message --------
Subject: GAO September 2004 EMERGING INFECTIOUS DISEASE Review of State and Federal Disease Surveillance Efforts GAO-04-877
Date: Mon, 01 Nov 2004 14:24:33 -0600
From: "Terry S. Singeltary Sr."
To: Bovine Spongiform Encephalopathy ,,

Report to the Chairman, Permanent
Subcommittee on Investigations,
Committee on Governmental Affairs,
U.S. Senate
United States Government Accountability Office
September 2004
Review of State and
Federal Disease
Surveillance Efforts


Description of U.S.
List of Nationally
Notifiable Infectious
Diseases, 2004
(no human TSE was listed...TSS)


Selected Worldwide
Emerging Infectious

Variant Creutzfeldt-Jakob disease (vCJD) is a rare, degenerative,
fatal brain disorder in humans. It is believed that vCJD is contracted
through the consumption of cattle products contaminated with the agent
of bovine spongiform encephalopathy (BSE) or mad cow diseasea
slowly progressive, degenerative, fatal disease affecting the central
nervous system of adult cattle. There is no known treatment of vCJD.


1. Emerging Infectious Diseases: Review of State and Federal Disease
Surveillance Efforts. GAO-04-877, September 30.
To view the full product, including the scope
and methodology, click on the link above.
For more information, contact Majorie Kanof
at (202) 512-7119.
Highlights of GAO-04-877, a report to the
Chairman, Permanent Subcommittee on
Investigations, Committee on
Governmental Affairs, U.S. Senate
September 2004
Review of State and Federal Disease
Surveillance Efforts
The threat posed by infectious
diseases has grown. New diseases,
unknown in the United States just a
decade ago, such as West Nile virus
and severe acute respiratory
syndrome (SARS), have emerged.
To detect cases of infectious
diseases, especially before they
develop into widespread outbreaks,
local, state, and federal public
health officials as well as
international organizations conduct
disease surveillance. Disease
surveillance is the process of
reporting, collecting, analyzing, and
exchanging information related to
cases of infectious diseases.
In this report GAO was asked to
examine disease surveillance
efforts in the United States.
Specifically, GAO described
(1) how state and federal public
health officials conduct
surveillance for infectious diseases
and (2) initiatives intended to
enhance disease surveillance.
GAO reviewed documents, such as
policy manuals and reports related
to disease surveillance, and
interviewed officials from selected
federal departments and agencies,
including the Departments of
Defense (DOD), Agriculture
(USDA), and Homeland Security
(DHS) as well as the Food and
Drug Administration (FDA), and
the Centers for Disease Control
and Prevention (CDC). GAO
conducted structured interviews of
state public health officials from
11 states.
Surveillance for infectious diseases in the United States comprises a variety
of efforts at the state and federal levels. At the state level, state health
departments collect and analyze data on cases of infectious diseases. These
data are required to be reported by health care providers and others to the
state. State public health departments verify reported cases of diseases,
monitor disease incidence, identify possible outbreaks within their state, and
report this information to CDC. At the federal level, agencies and
departments collect and analyze disease surveillance data and maintain
disease surveillance systems. For example, CDC uses the reports of diseases
from the states to monitor national health trends, formulate and implement
prevention strategies, and evaluate state and federal disease prevention
efforts. FDA analyzes information on outbreaks of infectious diseases that
originate from foods that the agency regulates. Some federal agencies and
departments also fund and operate their own disease surveillance systems
and laboratory networks and have several means of sharing surveillance
information with local, state, and international public health partners.
State and federal public health officials have implemented a number of
initiatives intended to enhance disease surveillance, but challenges remain.
For example, officials have implemented and expanded syndromic
surveillance systems, which monitor the frequency and distribution of
health-related symptoms among people within a specific geographic area.
Although syndromic surveillance systems are used by federal agencies and
departments and in all of the states whose officials GAO interviewed,
concerns have been raised about this approach to surveillance. Specifically,
syndromic surveillance systems are relatively costly to maintain compared
to other types of surveillance and are still largely untested. Public health
officials are also implementing initiatives designed to enhance public health
communications and disease reporting. For example, CDC is working to
increase the number of participants using its public health communication
systems. In addition, state public health departments and CDC are
implementing an initiative designed to make electronic disease reporting
more timely, accurate, and complete. However, the implementation of this
initiative is incomplete. Finally, federal public health officials have
enhanced federal coordination on disease surveillance and expanded
training programs for epidemiologists and other public health experts.
In commenting on a draft of this report, the Department of Health and
Human Services (HHS) said the report captures many important issues in
surveillance. HHS also provided suggestions to clarify the discussion.

Highlights -

Greetings GAO,

I am deeply disturbed by the fact that not only the USDA/FDA/CDC/APHIS,
BUT most dirturbing the GAO refuse to acknowledge ALL Human/Animal
TSEs. With not only BSE in cattle, you now have BASE in cattle,
showing up in more and more countries and we now have another
atypical TSE, not sheep now, but a GOAT, that is indistinguishable
from BSE. THERE are atypical TSEs showing up in humans, being
documented as sporadic CJD, while we have a new TSE in CATTLE
'BASE' and the title of the study speaks for itself;

Medical Sciences

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

TO pour more gas on the fire we have other atyical TSEs that are
going ignored, and this is what got us in this mess. Please do not
continue to make the same mistakes. sporadic CJD does have
routes and sources. THIS is why all Human/Animal TSEs must
be made reportable across the board. THERE is no other answer...

How can you have this ;

> A notifiable disease is an infectious disease for which regular,
> frequent, and timely information on individual cases is considered
> necessary for the prevention and control of the disease...

THEN have this;

> The diseases that must be reported vary by state...

AND at the same time stop the spread of human/animal TSEs across the board?

answer: you can't, and the agent continues to spread.

you must not continue to ignore the obvious;

BSE prions propagate as either variant CJD-like or sporadic CJD-like
prion strains in transgenic mice expressing human prion protein

THE new findings of BASE in cattle in Italy of Identification of a
second bovine amyloidotic spongiform encephalopathy: Molecular
similarities with sporadic Creutzfeldt-Jakob disease

Characterization of two distinct prion strains
derived from bovine spongiform encephalopathy
transmissions to inbred mice

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

In an experimental study of the transmissibility of BSE to the pig,
seven of 10 pigs, infected at 1-2 weeks of age by multiple-route
parenteral inoculation with a homogenate of bovine brain from
natural BSE cases developed lesions typical of spongiform

THE recent discoveries of previously unidentified strains of
Scrapie such as 221C44 and the Nor9845;


UK Strategy for Research and
Development on Human and Animal
Health Aspects of Transmissible
Spongiform Encephalopathies


Commission submits French Research Findings on TSE in a goat to Expert Panel
Reference: IP/04/1324 Date: 28/10/2004


Brussels, 28 October 2004

Commission submits French Research Findings on TSE in a goat to Expert

Following the findings by a research group in France that they suspect
the presence of a TSE infection in a goats brain which tests cannot
distinguish from BSE, the European Commission has submitted data
received from the French authorities to the Community Reference
Laboratory (CRL) for TSEs based in Weybridge, England, for an evaluation
by an expert panel. TSEs are transmissible spongiform encephalopathies,
namely BSE affecting cattle, and scrapie affecting goats and sheep. The
expert panel will evaluate, over the next two weeks or so, the
scientific evidence to see if it indicates the presence of BSE in the
goat. This isolated incident does not present a risk to public health as
the goat and its herd did not enter the food and feed chain.


WHEN in fact, the findings from Marsh and the findings at
MISSION, TEXAS support even further evidence that there
are further strains of TSE in the USA besides that one
accidentally documented BSE case in Washington on
Dec. 23, 2003;

In Confidence - Perceptions of unconventional slow virus diseases of
animals in the USA - Report of a visit to the USA - April-May 1989 - G A H
Wells [head of England's main veterinary lab]

Evidence That Transmissible Mink Encephalopathy
Results from Feeding Infected Cattle

Prepared by
National Center for Animal Health Programs
Eradication and Surveillance Team
July 31, 2004



AS of July 31, 2004, there were 71 scrapie infected and source flocks
(figure 3). There were 4 new infected and source flocks reported in July
(figure 4) with a total of 90 flocks reported for FY 2004 (figure 5).
The total infected and source flocks that have been released in FY 2004
are 62 (figure 6), with 15 flocks released in July. The ratio of
infected and source flocks released to newly infected and source flocks
for FY 2004 = 0.69 : 1.
BEEN CONFIRMED and reported by the National Veterinary Services
Laboratories (NVSL) in FY 2004, OF WHICH 42 WERE RSSS CASES (figure 7).
This includes 60 NEWLY CONFIRMED CASES IN JULY 2004 (figure 8). THIRTEEN
cases of scrapie in GOATS have been reported since 1990 (figure 9). ONE
NEW GOAT CASE WAS REPORTED IN FY 2004. New infected flocks, source
flocks, and flocks released or put on clean-up plans for FY 2004 are
depicted in figure 10...


Emergency Programs

Imported Belgium/Netherlands Sheep Test Results

APRIL 2002


The Western-blot test however cannot differentiate
between scrapie and BSE. The only known validated
method to differentiate between these two diseases
requires a series of mouse bioassay systems, which
take at least 23 years for completion.



April 2004

Prions in skeletal muscle

Patrick J. Bosque*,dagger ,Dagger , Chongsuk Ryou*, Glenn Telling*,§,
David Peretz*,dagger , Giuseppe Legname*,dagger , Stephen J.
DeArmond*,dagger ,¶, and Stanley B. Prusiner*,dagger ,||,**

* Institute for Neurodegenerative Diseases and Departments of dagger
Neurology, ¶ Pathology, and || Biochemistry and Biophysics, University
of California, San Francisco, CA 94143

Contributed by Stanley B. Prusiner, December 28, 2001

Considerable evidence argues that consumption of beef products from
cattle infected with bovine spongiform encephalopathy (BSE) prions
causes new variant Creutzfeldt-Jakob disease. In an effort to prevent
new variant Creutzfeldt-Jakob disease, certain "specified offals,"
including neural and lymphatic tissues, thought to contain high titers
of prions have been excluded from foods destined for human consumption
[Phillips, N. A., Bridgeman, J. & Ferguson-Smith, M. (2000) in The BSE
Inquiry (Stationery Office, London), Vol. 6, pp. 413-451]. Here we
report that mouse skeletal muscle can propagate prions and accumulate
substantial titers of these pathogens. We found both high prion titers
and the disease-causing isoform of the prion protein (PrPSc) in the
skeletal muscle of wild-type mice inoculated with either the Me7 or
Rocky Mountain Laboratory strain of murine prions. Particular muscles
accumulated distinct levels of PrPSc, with the highest levels observed
in muscle from the hind limb. To determine whether prions are produced
or merely accumulate intramuscularly, we established transgenic mice
expressing either mouse or Syrian hamster PrP exclusively in muscle.
Inoculating these mice intramuscularly with prions resulted in the
formation of high titers of nascent prions in muscle. In contrast,
inoculating mice in which PrP expression was targeted to hepatocytes
resulted in low prion titers. Our data demonstrate that factors in
addition to the amount of PrP expressed determine the tropism of prions
for certain tissues. That some muscles are intrinsically capable of
accumulating substantial titers of prions is of particular concern.
Because significant dietary exposure to prions might occur through the
consumption of meat, even if it is largely free of neural and lymphatic
tissue, a comprehensive effort to map the distribution of prions in the
muscle of infected livestock is needed. Furthermore, muscle may provide
a readily biopsied tissue from which to diagnose prion disease in
asymptomatic animals and even humans. Dagger Present address: Department
of Medicine, Denver Health Medical Center, Denver, CO 80204.

§ Present address: Department of Microbiology and Immunology, University
of Kentucky, Lexington, KY 40536-0230.

** To whom reprint requests should be addressed. E-mail:

Extraneural Pathologic Prion Protein in Sporadic Creutzfeldtâ¬Jakob

Markus Glatzel, M.D., Eugenio Abela, Manuela Maissen, M.S., and Adriano
Aguzzi, M.D., Ph.D.


Conclusions Using sensitive techniques, we identified extraneural
deposition of PrPSc in
spleen and muscle samples from approximately one third of patients who
died with
sporadic Creutzfeldtâ¬Jakob disease. Extraneural PrPSc appears to
correlate with
a long duration of disease.

EMBO reports AOP Published online: 11 April 2003

Widespread PrPSc accumulation in muscles of hamsters orally infected
with scrapie

Achim Thomzig, Christine Kratzel, Gudrun Lenz, Dominique KrÒ¼ger &
Beekes Robert Koch-Institut, P26, Nordufer 20, D-13353 Berlin, Germany

Received 13 February 2003; Accepted 13 March 2003; Published online 11
April 2003.

Abstract :

Scrapie, bovine spongiform encephalopathy and chronic wasting disease
are orally communicable, transmissible spongiform encephalopathies
(TSEs). As zoonotic transmissions of TSE agents may pose a risk to human
health, the identification of reservoirs for infectivity in animal
tissues and their exclusion from human consumption has become a matter
of great importance for consumer protection. In this study, a variety of
muscles from hamsters that were orally challenged with scrapie was
screened for the presence of a molecular marker for TSE infection, PrPSc
(the pathological isoform of the prion protein PrP). Sensitive western
blotting revealed consistent PrPSc accumulation in skeletal muscles from
forelimb and hindlimb, head, back and shoulder, and in tongue.
Previously, our animal model has provided substantial baseline
information about the peripheral routing of infection in naturally
occurring and orally acquired ruminant TSEs. Therefore, the findings
described here highlight further the necessity to investigate thoroughly
whether muscles of TSE-infected sheep, cattle, elk and deer contain
infectious agents.

Chronic Wasting Disease and Potential Transmission to Humans

Ermias D. Belay,*Comments
Ryan A.
Maddox,* Elizabeth S. Williams, Michael W. Miller,! Pierluigi
Gambetti,§ and Lawrence B. Schonberger*
*Centers for Disease Control and Prevention, Atlanta, Georgia, USA;
University of Wyoming, Laramie, Wyoming, USA; !Colorado Division of
Wildlife, Fort Collins, Colorado, USA; and §Case Western Reserve
University, Cleveland, Ohio, USA

Environmental Sources of Prion Transmission in Mule Deer

Michael W. Miller,*Comments
Elizabeth S.
Williams, N. Thompson Hobbs,! and Lisa L. Wolfe*
*Colorado Division of Wildlife, Fort Collins, Colorado, USA; University
of Wyoming, Laramie, Wyoming, USA; and !Colorado State University, Fort
Collins, Colorado, USA

Suggested citation for this article: Miller MW, Williams ES, Hobbs
NT, Wolfe LL. Environmental sources of prion transmission in mule
deer. Emerg Infect Dis [serial on the Internet]. 2004 Jun [date
cited]. Available from:

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

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

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.

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

CDC/NIH et al are wrong about sporadic CJD not being on the
rise. IN the USA you would not know, due to the lack of any creditable
human TSE surveillance, it's about as bad as the surveillance for animal

IN the UK and EU, sporadic CJD has been rising for years to 2002;

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


P.S. I will not even go into those VERMONT SHEEP that had the
'atypical' TSE, that the USDA/APHIS et al were suppose to have
been testing. THE testing has been put on hold for over 2 years.
NOW I hear it is the fault of the UK Gov (personal communication
Dr. Detwiler)... more 'floundering'...TSS

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

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