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From: TSS (216-119-144-45.ipset24.wt.net)
Subject: Other animal prion diseases FULL TEXT
Date: January 25, 2005 at 1:45 pm PST

-------- Original Message --------
Subject: Other animal prion diseases FULL TEXT
Date: Tue, 25 Jan 2005 15:35:03 -0600
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@LISTSERV.KALIV.UNI-KARLSRUHE.DE


##################### Bovine Spongiform Encephalopathy #####################

British Medical Bulletin 66:199-212 (2003)
© 2003 The British Council


Other animal prion diseases

Christina J Sigurdson* and Michael W Miller{dagger}

*Department of Microbiology, Immunology, and Pathology, Colorado State
University, Fort Collins, Colorado, USA
{dagger} Colorado Division of Wildlife, Wildlife Research Center, Fort
Collins, Colorado, USA

In addition to bovine spongiform encephalopathy (BSE) of cattle and
scrapie of sheep and goats, a few other animal prion diseases have been
reported. These include feline spongiform encephalopathy of zoological
and domestic cats (FSE) and transmissible spongiform encephalopathy
(TSE) of zoological ruminants and non-human primates, as well as chronic
wasting disease of deer and elk (CWD) and transmissible mink
encephalopathy of farmed mink (TME). The origins of TSE in cats, zoo
bovids, and non-human primates are clearly linked to the BSE epidemic;
however, the origins of CWD and TME are less clear, but are not
epidemiologically linked to the BSE epidemic. Here we review the
epidemiology, transmission, clinical features and pathology of these
other animal prion diseases.

http://bmb.oupjournals.org/cgi/content/abstract/66/1/199

Other animal prion diseases FULL TEXT

Christina J Sigurdson* and Michael W Miller

*Department of Microbiology, Immunology, and Pathology, Colorado State
University, Fort Collins,
Colorado, USA and Colorado Division of Wildlife, Wildlife Research
Center, Fort Collins,
Colorado, USA

In addition to bovine spongiform encephalopathy (BSE) of cattle and
scrapie of
sheep and goats, a few other animal prion diseases have been reported. These
include feline spongiform encephalopathy of zoological and domestic cats
(FSE)
and transmissible spongiform encephalopathy (TSE) of zoological
ruminants and
non-human primates, as well as chronic wasting disease of deer and elk
(CWD) and
transmissible mink encephalopathy of farmed mink (TME). The origins of
TSE in
cats, zoo bovids, and non-human primates are clearly linked to the BSE
epidemic;
however, the origins of CWD and TME are less clear, but are not
epidemiologically
linked to the BSE epidemic. Here we review the epidemiology,
transmission, clinical
features and pathology of these other animal prion diseases.

Spongiform encephalopathy in zoo ruminants, cats, and nonhuman
primates

BSE transmits to non-domestic bovids, felids, and non-human primates
Parallel to the BSE epidemic in cattle beginning in the 1980s, 15
additional species have contracted a spongiform encephalopathy,
virtually tripling the number of animal species known world-wide to
develop a TSE naturally. Seven bovid, 4 felid, and 4 primate species were
afflicted with a TSE, primarily in zoological collections in Great Britain
but also in France (Table 1)15. At the time of their diagnoses, the
geographic and temporal association with BSE suggested possible links
to the epidemic, and further epidemiological and experimental evidence
has bolstered this premise. Affected animals had either consumed
cattlederived
protein supplements or were in contact with prion-infected
individuals1,6. Additionally, mice inoculated with brain homogenates
from TSE-infected kudu, nyala, or domestic cats developed a spongiform
encephalopathy with profiles of histological lesions and incubation
periods virtually identical to those seen with BSE in mice7,8. Moreover,
the similar biochemical profiles of the protease-resistant prion protein,
PrPres, in experimental murine BSE, FSE, and experimental BSE in a
macaque supported the hypothesis that these apparently novel TSEs had
the same origin  BSE9. Thus, the assemblage of epidemiological and
biochemical clues has provided compelling evidence that these newly
described TSEs arose from BSE that had crossed species barriers.

Although a multitude of zoo species were exposed to BSE-contaminated
meat and bone meal, only a small group of animals developed
disease. The exotic zoo ruminants that died of TSE include greater kudu,
eland, nyala, gemsbok, Arabian oryx, a scimitar-horned oryx, and a
bison1,5; all are members of the family Bovidae. Most affected animals
had consumed diets that included ruminant-derived meat and bone
meal. The possible exception was greater kudu. Epidemiological studies
initially suggested that kudu developed TSE from exposure to foodborne
BSE, but then maintained the infection by horizontal spread
among animals in a manner similar to scrapie and CWD6; however, the
apparently prolonged epidemic may have been the product of sustained
exposure to BSE-contaminated feed5.

Feline spongiform encephalopathy
The prion diseases of non-domestic cats were likely due to ingestion of
BSE-infected cattle carcasses. Feline spongiform encephalopathy has
been described in a captive cheetah, puma, an ocelot, and a tiger from
zoological collections in Great Britain1,5.
In addition to the non-domestic felids, 87 domestic cats in Great Britain
and sporadic cases in Norway, Northern Ireland and Liechtenstein have
been diagnosed with FSE10. All cats were > 2 years old. Clinically, affected
cats initially demonstrated behaviour changes (more timid or
aggressive), with subsequent ataxia, hypermetria, and hyperesthesia to
sound and touch11,12. Histopathology revealed spongiform degeneration
in the neuropil of the brain and spinal cord with the most severe lesions
localized to the medial geniculate nucleus of the thalamus and the basal
nuclei10. A ban on bovine spleen and CNS tissue in pet foods was
initiated in 1990, and all but one of the FSE cases to date occurred in
cats born prior to the ban13.

Prions for physicians
British Medical Bulletin 2003;66
Table 1 Zoo animals diagnosed with transmissible spongiform
encephalopathy between
1985 and 19981,35
Species Number affected
Greater kudu (Tragelaphus strepsiceros) 6
Eland (Taurotragus oryx) 5
Gemsbok (Oryx gazella) 1
Nyala (Tragelaphus angasi) 1
Arabian oryx (Oryx leucoryx) 1
Scimitar-horned oryx (Oryx dammah) 1
Bison (Bison bison) 1
Cheetah (Acinonyx jubatus) 7
Puma (Felis concolor) 3
Ocelot (Felis pardalis) 2
Tiger (Panthera tigris) 1
Mayotte brown lemur (Eulemur fulvus mayottensis) 2
White fronted brown lemur (Eulemur fulvus albifrons) 1
Mongoose lemur (Eulemur mongoz) 1
Rhesus macaque (Macaca mulatta) 1
201

Spongiform encephalopathy in non-human primates

Lemurs and a rhesus macaque from a zoo and three primate facilities in
France naturally developed TSE in the 1990s. Primate diets had included
meat-meal supplements that were likely contaminated by British beef4.
Indeed, lemurs experimentally infected with BSE developed brain lesions
that were similar to those seen in naturally infected lemurs. Additionally,
the immunohistochemical staining patterns in natural and experimental
cases were similar and revealed PrPres in tonsil, Peyers patches, lymph
nodes and spleen4.

Chronic wasting disease: a prion disease in North American
deer and elk

Transmission and epidemiology
Chronic wasting disease (CWD) is the only prion disease known to
affect free-ranging wild-life. First recognized as a clinical syndrome of
captive mule deer (Odocoileus hemionus) in Colorado in the 1960s,
CWD was not diagnosed as a TSE until 1978, and was diagnosed in
captive research deer and captive Rocky Mountain elk (Cervus elaphus
nelsoni) in southeastern Wyoming soon thereafter14,15. Beginning in
1981, cases of CWD were diagnosed in free-ranging mule deer, whitetailed
deer (O. virginanus) and Rocky Mountain elk (cervids) on the
eastern slope of the Rocky Mountains and extending out on the plains
following river valleys within Colorado and Wyoming16,17. The origin of
CWD in captive or free-ranging deer remains enigmatic17,18.
CWD was first diagnosed in Canadas farmed elk industry in 1996,
and in the US elk industry in 1997. More recently, CWD-infected
ranched elk have been discovered in several other states and in Canada
and South Korea; these discoveries have heightened international
awareness and concern regarding CWD and other animal TSEs. Prior to
2000, CWD in free-ranging deer was believed to be limited to a focal
geographic region of the US. Unfortunately, in the last two years, CWD
has been detected in free-ranging deer in Wisconsin, Nebraska, South
Dakota, New Mexico, and western Colorado, and in Saskatchewan,
Canada (Fig. 1); the origins of these recent outbreaks remain under
investigation, but in most cases spill-over from infected game farms
seems the most plausible explanation. The appearance of CWD in wild
deer presents significant challenges to disease control or eradication due
to the extensive geographic range of North American deer and elk, lack
of ante-mortem diagnostic tests, as well as an inability to rid the
environment of potential prion-contaminated excreta18.

CWD is naturally transmitted with remarkable efficiency. Estimates of
CWD-infected deer revealed a prevalence of 115% within a defined
endemic region in northeastern Colorado and southeastern Wyoming17.
The efficiency of CWD transmission was also evident in a captive mule
deer herd, wherein ~90% of animals (n = 60) resident for 2 years or
longer developed CWD between 1970 and 198114. The original source
of infection was not determined, but these animals had not been fed
meat and bone meal19. The mechanism of CWD agent shedding and
natural transmission among free-ranging herbivores is unknown.
Epidemiological studies of natural disease suggest horizontal spread
potentially via the ingestion of forage or water contaminated by
infectious secretions, excretions, or other tissue source (e.g. placenta or
decomposed carcasses), although vertical transmission has not been
excluded1720. The abundant presence of the pathogenic prion protein,
PrPCWD, in alimentary mucosal-associated lymphoid tissues may favour
prion shedding into the environment via faeces or saliva5,18,21.
CWD surveillance within and around the endemic areas of northeastern
Colorado and southeastern Wyoming from 1997 to present has
been extensive. Brain samples have been acquired from over 12,000 deer
and elk sampled via geographically-focused random surveys and were
tested by immunohistochemistry using anti-PrP monoclonal
antibodies17,22. Brain and lymphoid tissue sections have been examined
for PrPCWD. Results of the surveys within the suspected endemic area
have revealed that ~5% of mule deer, 2% of white-tailed deer, and < 1%
of elk are infected, with wide variation within a species sampled from
different subpopulations17. Over a 3-year period of sampling, CWD
prevalence appeared stable17; however, more recent trend analyses
suggest prevalence is slowly increasing (MW Miller, unpublished
findings). Population models predict that if epidemics continue
unmanaged, then mule deer populations would be expected to decline
dramatically over a 3050-year period17,23. It is not known whether
multiple strains exist.

National surveillance efforts

National programmes for CWD surveillance and management are
currently under development in both the US and Canada. The United
States Department of Agriculture (USDA) currently encourages CWD
screening of all captive ranched elk and deer mortalities. In the absence of
national programmes, many states and provinces have developed their
own surveillance and certification programmes and have restricted
movement of deer or elk across state boundaries18. Due to lack of an
antemortem
diagnostic assay in elk and deer, captive cervid herds with a
documented CWD-positive animal are typically eliminated. Thus far, there
have been 25 CWD-positive deer and elk herds detected in the US, 40 in
Canada, and at least one in South Korea. The USDA has depopulated 11
known-infected herds, plus additional herds in the endemic areas of
Colorado and Nebraska (L Creekmore, USDA, personal communication).
Surveillance for CWD in free-ranging cervids, conducted largely by
state and provincial wild-life management agencies, employs a
combination of symptomatically-targeted surveillance and random
surveys of harvested animals17,18. Surveillance data from free-ranging
cervids outside endemic portions of Colorado and Wyoming have been
assembled annually by the Southeastern Cooperative Wildlife Disease
Study (SCWDS). From 1998 to mid-2002, SCWDS received reports
indicating that 14,181 deer and elk had been tested for CWD24. In 2002,
Wisconsin wild-life officials reported CWD-positive deer had been
detected in hunter-harvested animals; shortly thereafter, officials in New
Mexico reported a confirmed case in a clinical suspect. Discovery of
these wild CWD-infected deer in states far from the original endemic
area raised several questions. How had CWD spread to these deer
populations? Had scrapie repeatedly jumped the species barrier to cause
CWD in geographically distant locations? Is CWD a spontaneously
arising disease, caused by a potentially enhanced susceptibility of cervids
for prion protein conversion? Is CWD spread by infected ranched deer
or elk? Or had CWD-infected deer and elk been illegally translocated
from Colorado into other states? Hopefully, on-going experimental and
epidemiological investigations will answer some, if not all, of these
questions.

Clinical signs

Early signs of CWD in clinically affected deer and elk are extremely
subtle and include weight loss, behavioural alterations (such as loss of
fear of humans), a lowered head and drooping ears. As clinical disease
progresses, more noticeable signs like flaccid hypotonic facial muscles,
excessive salivation, regurgitation of ruminal fluid, ruminal atony, and
polyuria and polydipsia arise. Individuals may develop aspiration
pneumonia in late stage disease14. Affected animals are typically > 2
years old (average, 35 years), with an equal prevalence seen among
males and females (Fig 2). Deer may survive up to 78 months after
onset of clinical signs14; elk may survive even longer20.

Gross and histological pathology

On gross necropsy examination, end-stage clinical CWD cervids are
consistently emaciated with serious atrophy of fat; frothy rumen
contents, abomasal ulcers, and aspiration pneumonia are observed with
less consistency14,16. Characteristic histological lesions are confined to
the central nervous system and are similar to the other TSEs, namely:
intraneuronal vacuolation, neuronal degeneration and loss, extensive
neuropil spongiosis, astrocytic hypertrophy and hyperplasia, and
occasional amyloid plaques14,16,19,25. Spongiform lesions predominate
within the thalamus, hypothalamus, midbrain, pons and medulla
oblongata as well as in the olfactory tubercle and cortex14,16. Severe
lesions in the supra-optic and paraventricular nuclei, where anti-diuretic
hormone is produced, may be responsible for the clinical signs of
polyuria and polydipsia and the low urine specific gravity in clinically
dehydrated animals14.

Interestingly, the distribution of lesions in deer and elk is similar to
lesions of BSE in cattle or scrapie in sheep25 and differs from the lesion
distribution of TME, which predominates in the cerebral cortex and
basal nuclei26. The most consistent histological lesion and PrPCWD
immunohistochemical stain of brain is within the dorsal motor nucleus
of the vagus nerve21, which is notably the first site of PrPCWD
accumulation (ES Williams and MW Miller, unpublished findings).

Transmission experiments

In the 1980s, Williams and Young demonstrated that CWD was transmissible
by intracerebral (IC) inoculation of CWD brain homogenate
into deer with an incubation period of 1721 months19. Recently,
experiments demonstrated that oral exposure of mule deer fawns to
CWD using brain homogenate results in detection of PrPCWD in
lymphoid tissues (retropharyngeal lymph node, tonsil, Peyers patches,
ileocaecal lymph node) within 6 weeks postexposure27 and clinical
disease with an incubation period of 1525 months (ES Williams and
MW Miller, unpublished findings). PrPCWD accumulates within the
lymphoid germinal centres in a manner similar to vCJD and scrapie.
Phenotyping studies have revealed that within germinal centres, PrPCWD
accumulates on cell membranes of follicular dendritic cells and/or B cells
and within the cytoplasm of tangible body macrophages28. In advanced
cases of CWD in naturally infected deer, PrPCWD accumulates in tonsil,
spleen, Peyers patches, and lymph nodes throughout the body, as well
as nerves and ganglia, pancreatic islets, and adrenal medulla21,29.

Fig. 2 Deer with clinical CWD; signs are emaciation, depression,
weakness, drooping ears, and vacant stare (a). Brain
histopathology (b) is characterized by neuronal vacuoles and spongiform
degeneration of the neuropil.
206

Abundance of PrPCWD in lymphoid tissues: implications for diagnosis and
transmission

Large accumulations of PrPCWD are detectable by immunohistochemistry
in tonsil and other lymphoid tissues of animals affected with CWD21.
Similar accumulations are also seen in mule deer prior to onset of
clinical CWD22. Therefore, immunohistochemistry on tonsil biopsies has
recently been investigated as a means for ante-mortem diagnosis of
CWD in deer30,31. Interestingly, Kimberlin and others have associated
infection of the lymphoreticular system with the transmissibility of
scrapie among sheep32. It is plausible that the abundant PrPres in
alimentary mucosa-associated lymphoid tissues may promote the
shedding and efficient transmission of CWD (and scrapie) prions.

Experimental intra- and inter-species transmission of CWD

The natural host range of CWD appears limited to deer (Odocoileus spp.)
and elk. As with other prion diseases, however, the range of species
susceptible to experimental inoculation is somewhat broader. Studies by
Marsh and Williams in the mid 1980s demonstrated that the CWD agent
could be transmitted to ferrets, mink, squirrel monkeys, and a goat19. Bruce
and colleagues found mice relatively resistant to CWD infection such that
strain typing was problematic33. More recently, Bartz and colleagues
demonstrated the susceptibility of ferrets to intracerebral inoculation of
CWD with an incubation period of 1721 months34. Racoons were
reported to be susceptible to scrapie and TME, but not CWD, after
intracerebral
challenge35. As with other TSEs, the susceptibility of other wild-life
or domestic species to CWD prions has yet to be studied comprehensively.

Transmission to humans or domestic livestock

Investigations of unusual CJD cases in the US over the last decade have
identified no causal relationship with CWD exposure36, but these
investigations, as well as other retrospective and prospective studies of
CJD risk factors, are on-going. No higher incidence or unusual clusters
of CJD cases have been observed in northeastern Colorado or
southeastern Wyoming (J Pape, Colorado Department of Public Health
and Environment, personal communication). Hunting continues as a
population management tool within the CWD endemic regions, but
public health officials recommend that CWD-infected carcasses not be
consumed. The actual risk of CWD jumping the species barrier and
causing human disease is unknown. A recent study by Raymond et al37
examined the ability of PrPCWD to convert human PrPC in vitro and
determined that the conversion was inefficient, but similar to the
efficiency of PrPBSE or ovine PrPSc to convert human PrPC. Because BSE
is apparently infectious to humans, at least at a low level, it would seem
prudent to limit exposure of humans to CWD:

Could CWD also cross species barriers and infect cattle and sheep
sharing grazing areas with CWD-infected deer? CWD transmission to
cattle causing a new BSE strain would be economically devastating for
the US beef industry. There are several studies, both completed and ongoing,
to determine whether cattle might be susceptible to CWD:

1 Conversion of bovine PrPC by PrPCWD was relatively inefficient37 compared
to conversions by PrPBSE or ovine PrPSc.
2 Cattle have been exposed to CWD-infected brain homogenates by the
most extreme and unnatural route, intracerebral inoculation. Thus far, at
27 months post-inoculation (p.i.), 3 of 13 cattle have developed
detectable PrPres in brain by Western blot and immunohistochemistry38.
3 Cattle have been orally inoculated with CWD brain homogenate and
show no clinical signs at 62 months p.i. (ES Williams and MW Miller,
unpublished findings).
4 Cattle are living in research facilities among a deer population with a
historically high prevalence of CWD and are in close association with
deer showing clinical signs of CWD. None of these animals have
developed signs of TSE after 63 months exposure (MW Miller and ES
Williams, unpublished findings).
5 A histological and immunohistochemical surveillance of brainstem from
262 cattle over 4 years old grazing in CWD endemic areas has not
revealed any suspect lesions of TSE39.

Thus far, data from these studies indicate that CWD will not readily
transmit to cattle.

Genetics

Within the PrP gene of mule deer, white-tailed deer and Rocky
Mountain elk, there are three known polymorphisms. Mule deer and
white-tailed deer have residues G/S at position 96 and S/N at position
13837,
but no apparent relationship between genotype and CWD susceptibility has
been demonstrated to date in either species. In contrast, the PrP
polymorphism in elk occurs at position 132 (M/L), and to date only elk
with 132 M/M or M/L have developed disease. In a study by ORourke et
al, elk expressing M/M at residue 132 were significantly over represented
among CWD-infected individuals from both free-ranging and captive
populations40, suggesting that the 132 PrP polymorphism (M/L) may
influence susceptibility in this species. Evidence of genetic
susceptibility to
CWD in elk resembles observations in two other host species: Human 129
PrP polymorphism (M/V) has been linked to susceptibility to vCJD and
some forms of CJD41. Similarly, sheep 136 (V/A) PrP polymorphism has
been linked to scrapie susceptibility, as has a second at 171 (Q/R).
In contrast to intensely bred domestic ruminant populations, the PrP
genetics of free-ranging cervid populations of different species,
subspecies, or breeding populations will require large-scale surveys.
Further, since the prevalence of CWD is so low in most populations,
estimating relative genetic susceptibility based on field exposure is not
likely to be useful, and experimental oral inoculations of animals of
various genotypes using an inocula of different genotypes will be
necessary (K ORourke, personal communication).

Transmissible mink encephalopathy

Epidemiology and transmission  a controversial arena
Transmissible mink encephalopathy (TME), initially recognized in
Wisconsin and Minnesota in 1947, has sporadically appeared in farmed
mink in several countries where farmed mink are raised, including the
US, Canada, Finland, Russia, and East Germany42. Nonetheless, TME
outbreaks are rare; the most recent occurrence in the US was in 1985.
Epidemiological studies of outbreaks indicate that the disease is causally
linked to the ingestion of prion-contaminated meat, potentially scrapie
sheep43. However, in the 1985 TME outbreak in Stetsonville, Wisconsin,
the mink rancher stated with certainty that sheep were not fed to mink.
Instead, downer (ill) cattle were the primary source of mink food  a
discovery which has led to much speculation on a potentially
unrecognized BSE-like disease of American cattle43. Despite such
speculation, the ultimate origins of TME epidemics remain uncertain.
To further investigate potential food-borne sources of TME, mink
were intracerebrally (IC) exposed to UK- and North American-derived
sheep scrapie brain homogenates. Mink were highly susceptible to the
Suffolk sheep scrapie from the US, but only after IC inoculation44. Mink
did not develop disease from ingesting scrapie brain45. These studies
suggested, at minimum, that mink are susceptible to scrapie. However,
further experiments demonstrated that TME could pass into cattle and,
moreover, that brain from these cattle could transmit the TME agent
efficiently to mink by either the IC or the oral route, with an incubation
period of only 4 and 7 months, respectively. This indicates that TSEs can
be transmitted efficiently between cattle and mink46, although the
epidemiological significance of these findings are less clear.
Extensive studies of TME performed at the University of Wisconsin,
Madison have demonstrated experimental transmission to sheep, goats,
striped skunk, squirrel monkey, stump-tailed and rhesus monkey, and
hamster as reviewed by Rhein et al47. TME in hamsters presents as two
different clinical pictures with unique incubation periods, histological
lesions, and biochemical profiles. The two strains are referred to as
hyper and drowsy, and reflect the manifestations of clinical disease48.

Clinical signs

The incubation period of natural TME has been estimated at 712
months, based on observations following epizootics. Initially, infected
mink display behavioural changes including increased aggressiveness
and hyperesthesia which progresses to ataxia, occasionally tremors or
circling, and compulsive biting of self or objects (Fig. 3)47. Clinical
signs
usually progress over weeks but can range from 1 week to several
months prior to death42.

Histopathology

The most salient histological feature in the TME brain is the extensive
neuropil vacuolation. Additionally, there is neuronal degeneration and
astrocytosis characteristic of TSEs. Lesions are well developed in the
cerebral cortex, particularly in the frontal cortex, as well as the corpus
striatum, thalamus and hypothalamus, and are less severe in the midbrain,
pons and medulla. Spongiform change is not usually evident in the
cerebellum and spinal cord47.

In contrast to CWD, little evidence of prion infection can be detected
in extraneural tissues of TME-infected mink. However, low concentrations
of infectivity have been demonstrated in spleen, intestine, and
mesenteric lymph node by bioassay49.

Fig. 3 TME in a mink. Clinical signs of TME include a rough hair coat,
extended tail, and ataxia (a). Brain from a
mink with TME depicting severe spongiform degeneration in the
hippocampus (b). Images were generously
provided by Dr Jason Bartz, Creighton University, Nebraska, USA.
210

Prevention of TME

Similar to FSE, TME apparently has arisen from exposure to a foodborne
prion agent, likely scrapie or a BSE-like agent in downer cattle.
The heightened awareness of TME by mink ranchers has likely led to the
exclusion of sheep or cattle as a food source. Therefore, future TME
infections are expected to be exceedingly rare.

Acknowledgements

We thank our colleagues for their contributions and helpful discussions: D
Gould, C Mathiason, J Bartz, J Fischer, R Allison, M Perrott, E Hoover, T
Spraker, E Williams, L Creekmore, and Katherine ORourke. This work
was supported, in part, by a grant from the National Institutes of Health.

References

1 Kirkwood JK, Cunningham AA. Epidemiological observations on spongiform
encephalopathies
in captive wild animals in the British Isles. Vet Rec 1994; 135: 296303
2 Pearson GR et al. Feline spongiform encephalopathy: fibril and PrP
studies. Vet Rec 1992; 131:
30710
3 Taylor DM, Woodgate SL. Bovine spongiform encephalopathy: the causal
role of ruminantderived
protein in cattle diets. Rev Sci Tech 1997; 16: 18798
4 Bons N et al. Natural and experimental oral infection of nonhuman
primates by bovine
spongiform encephalopathy agents. Proc Natl Acad Sci USA 1999; 96: 404651
5 Williams ES, Kirkwood JK, Miller MW. In: Williams ES, Barker IK. (eds)
Infectious Diseases
of Wild Mammals. Ames, Iowa: Iowa State University Press, 2001; 292312
6 Kirkwood JK, Cunningham AA, Wells GA, Wilesmith JW, Barnett JE. Spongiform
encephalopathy in a herd of greater kudu (Tragelaphus strepsiceros):
epidemiological
observations. Vet Rec 1993; 133: 3604
7 Bruce M et al. Transmission of bovine spongiform encephalopathy and
scrapie to mice: strain
variation and the species barrier. Philos Trans R Soc Lond B Biol Sci
1994; 343: 40511
8 Fraser H et al. Transmission of feline spongiform encephalopathy to
mice. Vet Rec 1994; 134: 449
9 Collinge J, Sidle KC, Meads J, Ironside J, Hill AF. Molecular analysis
of prion strain variation
and the aetiology of new variant CJD [see comments]. Nature 1996; 383:
68590
10 Ryder SJ, Wells GA, Bradshaw JM, Pearson GR. Inconsistent detection
of PrP in extraneural
tissues of cats with feline spongiform encephalopathy. Vet Rec 2001;
148: 43741
Prions for physicians
British Medical Bulletin 2003;66
211
11 Wyatt JM et al. Naturally occurring scrapie-like spongiform
encephalopathy in five domestic
cats. Vet Rec 1991; 129: 2336
12 Bratberg B, Ueland K, Wells GA. Feline spongiform encephalopathy in a
cat in Norway. Vet Rec
1995; 136: 444
13 Nathanson N, Wilesmith J, Wells GA, Griot C. In: Prusiner SB. (ed)
Prion Biology and
Diseases. New York: Cold Spring Harbor Laboratory Press, 1999; 43163
14 Williams ES, Young S. Chronic wasting disease of captive mule deer: a
spongiform
encephalopathy. J Wildl Dis 1980; 16: 8998
15 Williams ES, Young S. Spongiform encephalopathy of Rocky Mountain
elk. J Wildl Dis 1982;
18: 46571
16 Spraker TR et al. Spongiform encephalopathy in free-ranging mule deer
(Odocoileus
hemionus), white-tailed deer (Odocoileus virginianus) and Rocky Mountain
elk (Cervus
elaphus nelsoni) in northcentral Colorado. J Wildl Dis 1997; 33: 16
17 Miller MW et al. Epizootiology of chronic wasting disease in
free-ranging cervids in Colorado
and Wyoming. J Wildl Dis 2000; 36: 67690
18 Williams ES, Miller MW. Chronic wasting disease in deer and elk in
North America. Rev Sci
Tech 2002; 21: 30516
19 Williams ES, Young S. Spongiform encephalopathies in Cervidae. Rev
Sci Tech 1992; 11:
55167
20 Miller MW, Wild MA, Williams ES. Epidemiology of chronic wasting
disease in captive Rocky
Mountain elk. J Wildl Dis 1998; 34: 5328
21 Spraker TR et al. Comparison of histological lesions and
immunohistochemical staining of
proteinase-resistant prion protein in a naturally occurring spongiform
encephalopathy of freeranging
mule deer (Odocoileus hemionus) with those of chronic wasting disease of
captive deer.
Vet Pathol 2002; 39: 1109
22 Miller MW, Williams ES. Detecting PrPCWD in mule deer by
immunohistochemistry of lymphoid
tissues. Vet Rec 2002; 151: In press
23 Gross JE, Miller MW. Chronic wasting disease in mule deer: disease
dynamics and control. J
Wildl Manag 2001; 65: 20515
24 Fischer J, Nettles V, Creekmore L. In: St Louis, MO: Proceedings of
the 106th Meeting of the
United States Animal Health Association, 2002
25 Williams ES, Young S. Neuropathology of chronic wasting disease of
mule deer (Odocoileus
hemionus) and elk (Cervus elaphus nelsoni). Vet Pathol 1993; 30: 3645
26 Eckroade RJ, ZuRhein GM, Hanson RP. In: Prusiner SB, Hadlow WJ. (eds)
Slow Transmissible
Diseases of the Nervous System. New York: Academic Press, 1979; 40941
27 Sigurdson CJ et al. Oral transmission and early lymphoid tropism of
chronic wasting disease
PrPres in mule deer fawns (Odocoileus hemionus). J Gen Virol 1999; 80:
275764
28 Sigurdson CJ et al. PrPCWD lymphoid cell targets in early and
advanced chronic wasting disease
of mule deer. J Gen Virol 2002; 83: 261728
29 Sigurdson CJ, Spraker TR, Miller MW, Oesch B, Hoover EA. PrPCWD in
the mysenteric plexus,
vagosympathetic trunk and endocrine glands of deer with chronic wasting
disease. J Gen Virol
2001; 82: 232734
30 Wild MA, Spraker TR, Sigurdson CJ, ORourke KI, Miller MW.
Preclinical diagnosis of chronic
wasting disease in captive mule deer (Odocoileus hemionus) and
white-tailed deer (Odocoileus
virginianus) using tonsillar biopsy. J Gen Virol 2002; 83: 262934
31 Wolfe LL et al. Evaluation of antemortem sampling to estimate chronic
wasting disease
prevalence in free-ranging mule deer. J Wildl Manage 2002; 66: 56473
32 Kimberlin RH, Walker CA. Pathogenesis of scrapie in mice after
intragastric infection. Virus
Res 1989; 12: 21320
33 Bruce M, Chree A, Williams ES, Fraser H. In: Birmingham, UK: XIVth
International Congress
of Neuropathology, 2000
34 Bartz JC, Marsh RF, McKenzie DI, Aiken JM. The host range of chronic
wasting disease is
altered on passage in ferrets. Virology 1998; 251: 297301
35 Hamir AN, Miller JM. In: Denver, CO: Chronic Wasting Disease
Symposium 14, 2002
36 Belay ED et al. Creutzfeldt-Jakob disease in unusually young patients
who consumed venison.
Arch Neurol 2001; 58: 16738
Other animal prion diseases
British Medical Bulletin 2003;66
212
37 Raymond GJ et al. Evidence of a molecular barrier limiting
susceptibility of humans, cattle and
sheep to chronic wasting disease. EMBO J 2000; 19: 442530
38 Hamir AN et al. Preliminary findings on the experimental transmission
of chronic wasting
disease agent of mule deer to cattle. J Vet Diagn Invest 2001; 13: 916
39 Gould DH et al. Survey of cattle in northeast Colorado for evidence
of chronic wasting disease:
geographical and high risk targeted sample. J Vet Diagn Invest 2002;
Submitted
40 ORourke KI et al. PrP genotypes of captive and free-ranging Rocky
Mountain elk (Cervus
elaphus nelsoni) with chronic wasting disease. J Gen Virol 1999; 80: 27659
41 Will RG et al. Diagnosis of new variant Creutzfeldt-Jakob disease.
Ann Neurol 2000; 47:
57582
42 Marsh RF, Hadlow WJ. Transmissible mink encephalopathy. Rev Sci Tech
1992; 11: 53950
43 Marsh RF, Bessen RA. Epidemiologic and experimental studies on
transmissible mink
encephalopathy. Dev Biol Stand 1993; 80: 1118
44 Hanson RP et al. Susceptibility of mink to sheep scrapie. Science
1971; 172: 85961
45 Marsh RF, Hanson RP. In: Prusiner SB, Hadlow WJ. (eds) Slow
Transmissible Diseases of the
Nervous System. New York: Academic Press, 1979; 45160
46 Marsh RF, Bessen RA, Lehmann S, Hartsough GR. Epidemiological and
experimental studies
on a new incident of transmissible mink encephalopathy. J Gen Virol
1991; 72: 58994
47 Rhein GMZ, Eckroade RJ, Grabow JD. In: Zeman W, Lennett E, Brunson J.
(eds) Slow Virus
Diseases. Baltimore, MD: Williams and Wilkins, 1974; 1638
48 Bessen RA, Marsh RF. Identification of two biologically distinct
strains of transmissible mink
encephalopathy in hamsters. J Gen Virol 1992; 73: 32934
49 Hadlow WJ, Race RE, Kennedy RC. Temporal distribution of
transmissible mink
encephalopathy virus in mink inoculated subcutaneously. J Virol 1987;
61: 323540
Prions for physicians

British Medical Bulletin 2003; 66: 199212
DOI 10.1093/bmb/dg66.199
The British Council 2003
Correspondence to:
Prof. Christina Sigurdson,
Research Fellow,
Institute of Neuropathology,
University
Hospital of Zürich,
Schmelzbergstrasse 12,
Zürich CH 8091,
Switzerland
Email:
Christina.Sigurdson@usz.ch


British Medical Bulletin 2003;66TSS

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