# Creutzfeldt-Jakob Disease in Deer and Elk Hunters



## terry (Sep 13, 2002)

Subject: Creutzfeldt-Jakob Disease in Deer and Elk Hunters
Date: Thu, 10 Apr 2003 09:25:04 -0500
From: "Terry S. Singeltary Sr." <[email protected]>
Reply-To: Bovine Spongiform Encephalopathy <[email protected]>
To: [email protected]

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

Creutzfeldt-Jakob Disease in Deer and Elk Hunters

Natalia Murinova, Ali Samii, Melanie Walker, Gregg D.
Meekins, Michael Shadlen, Seattle, WA
OBJECTIVE: To present the cases of two deer and elk
hunters who developed CJD.
BACKGROUND: BSE, a prion disease in cattle, has been
shown to cause a form of CJD in humans. Recent
research has examined the possibility of human
infection from deer and elk with Chronic Wasting
Disease.
DESIGN/METHODS: Case Reports
RESULTS: Two recent patients at the Seattle VA
hospital developed rapidly progressive dementia. Both
patients hunted elk and deer for many years until they
became ill.

Full text (subscription required):

[P03.028] Creutzfeldt-Jakob Disease in Deer and Elk Hunters
Natalia Murinova, Ali Samii, Melanie Walker, Gregg D. Meekins, Michael
Shadlen, Seattle, WA

OBJECTIVE: To present the cases of two deer and elk hunters who
developed CJD.
BACKGROUND: BSE, a prion disease in cattle, has been shown to cause a
form of CJD in humans. Recent research has examined the possibility of
human infection from deer and elk with Chronic Wasting Disease.
DESIGN/METHODS: Case Reports
RESULTS: Two recent patients at the Seattle VA hospital developed
rapidly progressive dementia. Both patients hunted elk and deer for many
years until they became ill. Neither had a history of travel abroad or
iatrogenic exposure to CJD, or a family history of dementia. The first
patient, 64, presented to the hospital with mental status changes,
including paranoia, fear of poisoning, and inappropriate reactions. He
worsened quickly and three months after first presentation, was oriented
only to self, followed simple commands, and had an MMSE of 14/30. His
neurologic exam was nonfocal. He developed increasing agitation and
paranoia, became disoriented and noncommunicative, and developed ataxia
and myoclonus. His EEG showed bilateral periodic lateralized
epileptiform discharges. A brain MRI with diffusion showed T2 signal
abnormalities in patchy distribution in the cerebral cortex. He died
almost 4 months after the onset of illness. At autopsy, his brain showed
widespread spongiform changes and varying degrees of gliosis sparing no
brain region. The patients family stated that he was an avid deer and
elk hunter in western Washington. The second patient, 54, presented with
balance problems and vertigo dating back several years. Over a two-month
period, he developed severe short-term memory loss and quit his job. He
had an MMSE of 27/30 at presentation, but on admission two weeks later
had a score of 20/30 and was confused and ataxic. His EEG demonstrated
diffuse slowing. His brain MRI showed T2 prolongation within the caudate
and the putamen nuclei bilaterally. CSF testing for 14-3-3 protein was
positive. He died four months after admission. At autopsy, his brain
demonstrated diffuse spongiform encephalopathy. Prion protein genotype
was homozygous Val/Val at codon 129, and the prion protein was Scrapie
protein type 2 by electrophoresis. Per his family, he resided in rural
Alaska and hunted deer and elk for food.
CONCLUSIONS: Although these cases differ clinically, the
neuropathological similarities are striking. Of concern is that they may
represent a new entity in the spectrum of prion disease. Neither patient
had a known history of consuming deer or elk meat from CWD-endemic
areas; however recent reports have expanded the area in which CWD is
found in the wild. As it is not currently possible to predict the
characteristics of a hypothetical case of CWD-related CJD, the
collection and comparison of further CJD cases in consumers of venison
will help determine causality and learn more about a potentially
devastating emerging disease.
Category - Infection/AIDS/Prion Disease
SubCategory - Other

http://www.abstracts-on-line.com/abstracts/aan/aolstatement.asp


TSS

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


----------

