# Fears Grow Over ''mad Elk Disease''



## terry (Sep 13, 2002)

Subject: Fears grow over 'mad elk disease'
Date: Thu, 3 Apr 2003 08:15:12 -0600
From: "Terry S. Singeltary Sr." <[email protected]>
Reply-To: Bovine Spongiform Encephalopathy <[email protected]>
To: [email protected]

######## Bovine Spongiform Encephalopathy <[email protected]> #########

Fears grow over 'mad elk disease'

19:00 02 April 03 Exclusive from New Scientist Print Edition

The death of three hunters from Creutzfeldt-Jakob disease is likely to
heighten fears that people in North America are contracting a new form
of the fatal brain disorder from deer. But the surveillance system in
the US is so woefully inadequate that even if these fears are unfounded,
it is impossible for researchers to rule out the possibility.

Chronic wasting disease (CWD) is a prion disease spreading among elk and
mule deer in North America. It is similar to mad cow disease, raising
fears people eating contaminated venison could develop a form of
Creutzfeldt-Jakob disease (CJD) like that blamed on contaminated beef,
which has killed about 200 people in Britain.

This is why the latest cases of CJD in hunters, to be reported at the
American Academy of Neurology's meeting in Honolulu this week, are
causing concern. Two of the men, one aged 64 from Washington state and
the other aged 54 from Alaska, were treated by neurologists Natalia
Murinova and Ali Samii at the Seattle VA hospital.

"These two cases may well have no relationship to CWD in elk and deer,"
says Samii. "But the fact that it happened in these two patients brings
up that question." The third man died in a different hospital but was
from the same town as the hunter from Washington. The two were friends
and hunted together.


Disease-free state

However, the Centers for Disease Control and Prevention (CDC) will not
be investigating the cases because there is no evidence that the men ate
CWD-infected meat, says spokesman Ermias Belay.

So far the CDC has only investigated CJD cases from states where deer
are known to have the disease. But Washington and Alaska are not
necessarily disease-free: states do not have to test for CWD until the
disease is known to be present.

The CDC has investigated a few suspect CJD cases in the past, and found
no compelling evidence of CWD transmission to humans. However, "the data
seeking such evidence are very limited", Belay admits.

And that is because not many doctors are looking. For example, Pierluigi
Gambetti of the National Prion Disease Pathology Surveillance Center in
Cleveland, Ohio, has found that between 1997 and 2002 about 60 per cent
of the 921 suspect cases were confirmed as being a prion disease.

But these cases amount to fewer than a third of the number that would be
expected for the same period based on the rates of sporadic and familial
CJD elsewhere, he will tell the Honolulu meeting this week.


No guidelines

More on this story


Related Stories

Predicted deaths from vCJD slashed
26 February 2003

BSE may cause more CJD cases than thought
28 November 2002

Prion diseases' deadly action revealed
17 October 2002


For more related stories
search the print edition Archive



Weblinks

Chronic Wasting Disease Alliance

Centers for Disease Control and Prevention

National Prion Disease Pathology Surveillance Center

Neurology, University of Michigan

American Academy of Neurology



That shows how ineffective the surveillance is, says Norman Foster, a
neurologist at the University of Michigan in Ann Arbor. So if
CWD-contaminated venison does trigger a new form of CJD in people, there
could be dozens of extra cases of CJD without them being detected.

Part of the problem is that CJD resembles common diseases such as
dementia in the elderly. Doctors have no set guidelines on what to look
for and only autopsies can confirm a diagnosis. But few are carried out
and CJD is not a reportable disease in the US.

Autopsies can also distinguish between sporadic CJD and the variant form
triggered by mad cow disease. But since no one knows what the brains of
people with "CWD-CJD" would look like - if the disease does indeed exist
- it is hard for pathologists to be sure that a particular CJD case is
not related to CWD.

To compound matters further, different federal agencies are responsible
for surveillance in humans and animals. Foster and others are pushing
for a surveillance programme similar to Britain's mad cow disease and
CJD surveillance programmes.

Anil Ananthaswamy

http://www.newscientist.com/news/news.jsp?id=ns99993577

# Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States [FULL TEXT] - TSS 2/22/03 (0)

http://www.vegsource.com/talk/madcow/messages/9912538.html

TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES (Williams et al) {rebuttal,
TSS et me;-}

PART 1

http://www.vegsource.com/talk/madcow/messages/9912592.html

part II

http://www.vegsource.com/talk/madcow/messages/9912593.html

TSS

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


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## terry (Sep 13, 2002)

Subject: Fatal Degenerative Neurologic Illnesses in Men Who Participated in Wild Game Feasts - Wisconsin, 2002
Date: Thu, 03 Apr 2003 15:58:57 -0600
From: "Terry S. Singeltary Sr." <[email protected]>
To: Bovine Spongiform Encephalopathy <[email protected]>
CC: [email protected], bloodcjd <[email protected]>

Fatal Degenerative Neurologic Illnesses in Men Who Participated in Wild 
Game Feasts - Wisconsin, 2002

from Morbidity & Mortality Weekly Report
Posted 04/02/2003

Content

Creutzfeldt-Jakob disease (CJD) is a fatal neurologic disorder in 
humans. CJD is one of a group of conditions known as transmissible 
spongiform encephalopathies (TSEs), or prion diseases, that are believed 
to be caused by abnormally configured, host-encoded prion proteins that 
accumulate in the central nervous tissue.[1] CJD has an annual incidence 
of approximately 1 case per million population in the United States[1] 
and occurs in three forms: sporadic, genetically determined, and 
acquired by infection. In the latter form, the incubation period is 
measured typically in years. Recent evidence that prion infection can 
cross the species barrier between humans and cattle has raised 
increasing public health concerns about the possible transmission to 
humans of a TSE among deer and elk known as chronic wasting disease 
(CWD).[2] During 1993-1999, three men who participated in wild game 
feasts in northern Wisconsin died of degenerative neurologic illnesses. 
This report documents the investigation of these deaths, which was 
initiated in August 2002 and which confirmed the death of only one 
person from CJD. Although no association between CWD and CJD was found, 
continued surveillance of both diseases remains important to assess the 
possible risk for CWD transmission to humans.
Case Reports

Case 1. In December 1992, a Wisconsin man aged 66 years with a history 
of seizures since 1969 sought treatment for recurring seizures, 
increasing forgetfulness, and worsening hand tremors. 
Electroencephalographic (EEG) examination demonstrated focal 
epileptiform activity and nonspecific diffuse abnormalities, but no 
specific diagnosis was made. In February 1993, he was hospitalized for 
increasing confusion, ataxia, and movement tremors of his extremities. A 
magnetic resonance image (MRI) demonstrated mild, nonspecific 
enhancement along the inferior parasagittal occipital lobe. A repeat EEG 
showed bifrontal intermittent, short-interval, periodic sharp waves, 
suggesting a progressive encephalopathy; a diagnosis of CJD was 
suspected. The man died later that month; neuropathologic examination of 
brain tissue during autopsy indicated subacute spongiform 
encephalopathy, compatible with CJD.

The man was a lifelong hunter who ate venison frequently. He hunted 
primarily in northern Wisconsin but also at least once in Montana. He 
hosted wild game feasts at his cabin in northern Wisconsin from 1976 
until shortly before his death. Fixed brain tissue obtained during the 
autopsy was sent for analysis to the National Prion Disease Pathology 
Surveillance Center (NPDPSC) and reexamined at the institution where the 
autopsy was conducted. Histopathologic examination did not substantiate 
the diagnosis of prion disease. In addition, 27 brain tissue sections 
were negative for prions by immunostaining despite positive antibody 
reactions against other proteins (controls), which indicated that other 
epitopes in the tissue samples were preserved.

Case 2. In May 1999, a Minnesota man aged 55 years with no previous 
history of a neurologic disease sought evaluation and treatment 
following a 3-month history of progressive difficulty in writing and 
unsteadiness of gait. A computerized tomography (CT) scan and MRI 
examination of his head did not indicate any abnormality. In June 1999, 
he was hospitalized following onset of dementia, speech abnormalities, 
and myoclonic jerking. An EEG indicated left-hemispheric periodic sharp 
waves and moderate generalized background slowing; CJD was diagnosed 
clinically. In July 1999, following worsening symptoms and development 
of right upper extremity dystonia, the patient died. Neuropathologic 
evaluation of brain tissue during autopsy demonstrated widespread 
subcortical spongiform lesions, consistent with CJD.

The man was not a hunter but had a history of eating venison. He made an 
estimated 12 visits to the cabin where the wild game feasts were held, 
but he participated in only one feast during the mid-1980s. Sections of 
fixed and frozen brain tissue obtained during autopsy were analyzed at 
NPDPSC, and prion disease was confirmed by immunohistochemical and 
Western blot testing. The Western blot characteristics and prion disease 
phenotype in this patient were consistent with the most common form of 
sporadic CJD, classified as M/M (M/V) 1.[3] Subsequent genetic typing 
confirmed the presence of methionine homozygosity (M/M) at codon 129 of 
the patient's prion protein gene.

Case 3. In June 1992, a Wisconsin man aged 65 years sought treatment for 
progressive slowing of speech, worsening memory, and personality 
changes. By January 1993, his speech was reduced to one-word utterances. 
Neurologic examination showed a flat affect, decreased reflexes, and 
apraxia. A CT head scan showed mild atrophy, and an EEG was normal. 
Pick's disease was diagnosed. By May, he was unable to perform any daily 
living activities; he died in August 1993. Neuropathologic evaluation of 
brain tissue during autopsy showed symmetrical frontal lobe cerebral 
cortical atrophy and mild temporal lobe atrophy. No Pick's bodies or 
spongiform lesions were observed.

The man had a history of eating venison and participated regularly in 
wild game feasts held at the cabin owned by patient 1. He was a lifelong 
hunter and hunted mostly in Wisconsin but also in Wyoming and British 
Columbia. No game was brought to the wild game feasts from his hunting 
trips outside of Wisconsin. Examination of fixed brain tissue sent to 
NPDPSC demonstrated no lesions indicative of CJD, and 
immunohistochemical testing with antibody to the prion protein did not 
demonstrate the granular deposits seen in prion diseases.
Epidemiologic Investigation

Wild game feasts consisting of elk, deer, antelope, and other game that 
occurred at a cabin in northern Wisconsin owned by patient 1 began in 
1976 and continued through 2002. These feasts typically involved 10-15 
participants and usually occurred on weekends before or during hunting 
seasons in the fall and occasionally in the spring. Wild game brought to 
these feasts usually were harvested in Wisconsin, but three men who 
attended these feasts reported hunting in the western United States and 
bringing game back to Wisconsin. These activities took place in Colorado 
(near the towns of Cortez, Trinidad, Collbran, Durango, and Meeker), 
Wyoming (near the towns of Gilette and Cody), and Montana (near the town 
of Malta). CWD was not known to be endemic in these areas at the time 
that these hunting activities took place.

Information was obtained for 45 (85%) of 53 persons who were identified 
as possibly participating in the wild game feasts; all were male. 
Information was obtained by direct interview or from family members of 
decedents. Of the 45 persons, for whom information was obtained, 34 were 
reported to have attended wild game feasts. Seven of the 34 feast 
attendees were deceased, including the three patients. None of the four 
other decedents had a cause of death attributed to or associated with a 
degenerative neurologic disorder. None of the living participants had 
any signs or symptoms consistent with a degenerative neurologic disorder.

Reported by:JP Davis, MD, J Kazmierczak, DVM, M Wegner, MD, R Wierzba, 
Div of Public Health, State of Wisconsin Dept of Health and Family Svcs. 
P Gambetti, National Prion Disease Pathology Surveillance Center, Case 
Western Reserve University, Cleveland, Ohio. L Schonberger, MD, R 
Maddox, MPH, E Belay, MD, Div of Viral and Rickettsial Diseases, 
National Center for Infectious Diseases; V Hsu, MD, EIS Officer, CDC.

Section 1 of 2 Continue

http://www.medscape.com/viewarticle/450914

Bizarre link in Male Fatal Degenerative Neurologic Illnesses
from Morbidity & Mortality Weekly Report

Editorial Note

CWD was first described in the United States in the 1960s and classified 
as a TSE in 1978. Previously localized to a contiguous endemic area in 
northeastern Colorado and southeast Wyoming, since 2000, CWD has been 
found in free-ranging deer or elk in Illinois, Nebraska, New Mexico, 
South Dakota, Wisconsin, and outside the previously known endemic areas 
of Colorado and Wyoming. CWD has been identified also in captive deer or 
elk in Colorado, Kansas, Minnesota, Montana, Nebraska, Oklahoma, South 
Dakota, and Wisconsin.[4] Because a variant form of CJD, with specific 
neuropathologic and molecular characteristics that distinguish it from 
sporadic CJD, has been associated with eating cattle products infected 
with a prion that causes bovine spongiform encephalopathy,[5] concern 
has been raised about the possibility that the prion associated with CWD 
might be transmitted to humans in a similar way.

In this investigation, because only one of the three cases in Wisconsin 
had neuropathologic confirmation of a prion disease, no association 
could be made between case participation in the wild game feasts and the 
development of CJD. Although patient 2 had confirmed CJD, he was 
unlikely to have eaten CWD-infected venison at these feasts because 
venison and other game from outside Wisconsin that was served at these 
feasts did not originate from known CWD-endemic areas, and the man 
participated in the feasts only once. In addition, the prion disease in 
this case was consistent with the most common form of sporadic CJD, 
without apparent unusual neuropathologic or molecular characteristics 
that might occur if the prion related to CWD had been responsible for 
the disease.

The findings in this report are subject to at least two limitations. 
First, not all members participating in wild game feasts could be 
identified, and not all persons listed as participating could be 
contacted for interviews. Second, interviews that were conducted 
required recall of events that occurred up to 25 years ago, limiting the 
detail or accuracy of events. However, the similar responses obtained 
from different sources support the accuracy of the investigation findings.

A previous investigation of unusually young CJD patients in whom the 
transmission of CWD was suspected also did not provide convincing 
evidence for a causal relationship between CWD and CJD.[2] However, 
limited epidemiologic investigations cannot rule out the possibility 
that CWD might play a role in causing human illness. Ongoing 
surveillance of CJD, particularly in states with CWD, is important to 
assess the risk, if any, for CWD transmission to humans. Because the 
confirmation of CJD and the detection of a new prion disease require 
neuropathologic study of brain tissue, physicians are encouraged to 
contact NPDPSC (http://www.cjdsurveillance.com; telephone, 216-368-0587) 
to confirm diagnoses of CJD and to distinguish its various subtypes. 
Because of the known severity of TSEs in humans and the possibility that 
the CWD prion can affect humans, animals with evidence of CWD should be 
excluded from the human food or animal feed chains. Hunters and wild 
venison consumers should follow precautionary guidelines available from 
the Wisconsin Department of Agriculture, Trade, and Consumer Protection 
(http://datcp.state.wi.us/core/consumerinfo) to prevent potential 
exposures to the CWD agent.

Section 2 of 2 Previous

MMWR 52(7):125-127, 2003. © 2003 Centers for Disease Control and 
Prevention (CDC)

http://www.medscape.com/viewarticle/450914_2

TSS

Views & Reviews
Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States
Ermias D. Belay, MD, Ryan A. Maddox, MPH, Pierluigi Gambetti, MD and Lawrence B. Schonberger, MD

From the Division of Viral and Rickettsial Diseases (Drs. Belay and Schonberger and R.A. Maddox), National Center for Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, GA; and National Prion Disease Pathology Surveillance Center (Dr. Gambetti), Division of Neuropathology, Institute of Pathology, Case Western Reserve University, Cleveland, OH.

Address correspondence and reprint requests to Dr. Ermias D. Belay, 1600 Clifton Road, Mailstop A-39, Atlanta, GA 30333.

Transmissible spongiform encephalopathies (TSEs) attracted increased attention in the mid-1980s because of the emergence among UK cattle of bovine spongiform encephalopathy (BSE), which has been shown to be transmitted to humans, causing a variant form of Creutzfeldt-Jakob disease (vCJD). The BSE outbreak has been reported in 19 European countries, Israel, and Japan, and human cases have so far been identified in four European countries, and more recently in a Canadian resident and a US resident who each lived in Britain during the BSE outbreak. To monitor the occurrence of emerging forms of CJD, such as vCJD, in the United States, the Centers for Disease Control and Prevention has been conducting surveillance for human TSEs through several mechanisms, including the establishment of the National Prion Disease Pathology Surveillance Center. Physicians are encouraged to maintain a high index of suspicion for vCJD and use the free services of the pathology center to assess the neuropathology of clinically diagnosed and suspected cases of CJD or other TSEs.

Post-Publication Peer Reviews:

Read all Post-Publication Peer Reviews

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States
Terry S. Singeltary Neurology Online, 27 Jan 2003 [Full text]

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

Reply to Singletary
Ryan A. Maddox, MPH, et al. Neurology Online, 26 Mar 2003 [Full text]

http://www.neurology.org/cgi/eletters/60/2/176#582

my 2nd submission questioning Maddox et al reply not published yet,
may not be, but i did post here, skroll down;

http://www.vegsource.com/talk/madcow/messages/245.html

and even PRIONICS thought the same as i;

http://www.vegsource.com/talk/madcow/messages/246.html

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

Full Text

Tue, 13 Feb 2001 JAMA Vol. 285 No. 6, February 14, 2001 Letters

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

To the Editor:

In their Research Letter in JAMA. 2000;284:2322-2323, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.

Terry S. Singeltary, Sr Bacliff, Tex

http://jama.ama-assn.org/cgi/conten...&stored_search=&FIRSTINDEX=0&journalcode=jama

# Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States [FULL TEXT] - TSS 2/22/03 (0)

http://www.vegsource.com/talk/madcow/messages/9912538.html

TSS


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