EMERGING AND EXOTIC DISEASES OF ANIMALS

Iowa State University, Institute for International Cooperation in Animal Biologics

Center for Food Security and Public Health
College of Veterinary Medicine
Iowa State University
Ames Iowa USA 50011
Phone: 515 294 7189
Fax: 515 294 8259
Email: cfsph@iastate.edu
For the most current version of this fact sheet, visit the CFSPH website:
http://www.cfsph.iastate.edu/DiseaseInfo (/Members/Link.plx?ID=81346)

sheep, goat, cattle, cat, mink, rat, mouse, hamster, monkey, pig, marmoset, moufflon,
nyala, gemsbok, Arabian oryx, eland, kudu, scimitar-horned oryx, ankole, bison, tiger,
puma, ocelot, cheetah, mule deer, white-tailed deer, black-tailed deer, elk
Last Updated: Aug. 25, 2009

Clinical signs
behavioral changes, isolation, hyperexcitability, high-stepping gait, hopping gait, gait
abnormality, posture abnormality, fixed stare, head raised, head lowered, ataxia,
incoordination, trembling, convulsions, nibbling movements, pruritus, apprehension,
nervousness, hyperesthesia, ataxia, pelvic swaying, hypermetria, falling, recumbency,
coma, frenzy, self-mutilation, aggression, hiding, somnolence, excessive salivation,
difficulty eating, difficulty swallowing, teeth grinding, changes in normal grooming habits,
arched tails in mink, emaciation, loss of condition, weight loss, decreased milk production,
decreased rumination, bradycardia, altered heart rhythms, death
Post mortem lesions
no gross lesions
Importance
Transmissible spongiform encephalopathies (TSEs) are progressive and fatal
neurodegenerative diseases. TSEs affecting animals include scrapie (tremblante de
mouton, rida), bovine spongiform encephalopathy (BSE, "mad cow disease"),
transmissible mink encephalopathy (TME, mink scrapie), feline spongiform
encephalopathy (FSE), chronic wasting disease (CWD), and a spongiform
encephalopathy of exotic ruminants. These diseases were once thought to be entirely
species specific, but it now appears that some agents can cross species barriers. In the
United Kingdom, a BSE epidemic may have been responsible for concurrent outbreaks of
FSE in cats and spongiform encephalopathy in exotic ruminants. The same epidemic has
also been linked to a variant of Creutzfeldt-Jakob disease (CJD) in humans.
Etiology
TSEs are caused by unconventional disease agents. These agents are resistant to the
treatments that ordinarily destroy bacteria, spores, viruses, and fungi. They are generally
thought to be prions; a minority opinion is that they may be virinos or retroviruses. Strain
variations have been seen in the scrapie agent, but not the agent of BSE.
Species affected
Scrapie
Scrapie affects sheep, goats, and moufflon. Rats, mice, hamsters, monkeys, and a variety
of other laboratory and wild animals can be infected experimentally.
BSE
BSE is seen in cattle and can be experimentally transmitted to cats, mink, mice, pigs,
sheep, goats, and marmosets.
Spongiform encephalopathy of exotic ruminants
A spongiform encephalopathy of exotic ruminants has been seen in captive nyala,
gemsbok, Arabian oryx, eland, kudu, scimitar-horned oryx, ankole, and bison.
FSE
FSE has been found in domestic cats and captive wild cats, including tigers, a puma, an
ocelot, and a cheetah.
TME
TME is seen in ranched mink and has been experimentally transmitted to cattle.
CWD
CWD affects mule deer, white-tailed deer, black-tailed deer and elk.
Geographic distribution
Scrapie
Scrapie is widespread. This disease can be found in the United Kingdom, Ireland, France,
Belgium, Iceland, Norway, Cyprus, Israel, Japan, Canada, the United States, and parts of
Asia. Outbreaks have occurred in Australia and New Zealand but the disease was
eradicated.
BSE
BSE appears to have originated in the United Kingdom in 1985. Infected cattle have since
been found in Ireland, France, Portugal, Switzerland, the Netherlands, and Japan. Cases
have also been seen in imported cattle in Belgium, Germany, Oman, Italy, Liechtenstein,
Luxembourg, the Falkland Islands, and Canada. As a result of control measures, the
current epidemics in the United Kingdom and Switzerland appear to be diminishing. BSE
has never been detected in Australia, New Zealand, the United States, or South America.
FSE and spongiform encephalopathy of exotic ruminants
FSE has been found almost exclusively in the United Kingdom, with a single isolated case
in a cat in Norway. Spongiform encephalopathy of exotic ruminants has been detected
only in captive ruminants in the United Kingdom. These two diseases have been declining
in parallel with the BSE epidemic.
TME
Outbreaks of TME have been seen in the United States, Canada, Finland, Germany, and
Russia.
CWD
CWD is endemic in the United States. It is found in wild deer and elk in northeastern
Colorado and southeastern Wyoming.
Transmission
Scrapie
Transmission of scrapie is usually oral. Most animals become infected from their dam,
either at or soon after birth. The placenta is infectious; in confined lambing areas, the
disease can spread to the offspring of uninfected sheep. The scrapie agent can also be
detected in the tonsils, lymph nodes, spleen, distal ileum, proximal colon, and nervous
system. Scrapie can spread laterally between animals by direct contact, contamination of
fomites such as knives or vaccines, or possibly by environmental contamination. Vertical
transmission may be possible, but has not been established.
BSE
The first cases of BSE appeared in the United Kingdom in 1985. The outbreak may have
resulted from the feeding of scrapie containing, sheep meat-and-bone meal to cattle or it
may have arisen as a rare spontaneous formation of a spongiform encephalopathy in a
cow which then spread to other cattle through contaminated meat and bone meal. There
is strong evidence and general agreement that the outbreak was amplified by feeding
rendered bovine meat-and-bone meal to young calves.
In naturally infected cattle, the BSE agent has been found only in the brain, spinal cord,
and retina. In experimentally infected calves, it is also seen in the distal ileum. The BSE
agent has never been detected in muscle, blood, or milk, and natural infections do not
seem to spread laterally between cattle. The offspring of BSE-infected cattle have an
increased risk of developing BSE, but it is not known whether this is due to vertical
transmission.
Spongiform encephalopathy of exotic ruminants
The outbreak of spongiform encephalopathy of exotic ruminants paralleled the BSE
epidemic, and may have been due to the same agent. Experimentally, this TSE can be
transmitted both orally and parenterally. Vertical transmission is uncertain: two offspring of
affected animals developed the disease, but vertical transmission has not been seen in
experimental infections.
FSE
The BSE agent or a related agent may also have been the source of FSE. FSE is also
transmitted orally. In domestic cats, the source of infection was thought to be pet food that
contained cattle offal. Wild felids in zoos may have been infected when they were fed
cattle carcasses.
TME
TME is thought to be transmitted orally. During an outbreak, the disease probably spreads
between animals by fighting and cannibalism. Outbreaks are thought to begin when mink
are fed infectious feed. In some cases, the proposed source was carcasses from scrapie-
infected sheep. Experimental transmission of scrapie to mink has been unsuccessful;
however, mink can be infected by BSE and develop a disease very similar to TME. Brain
tissue from mink with TME can also transmit a fatal spongiform encephalopathy to cows
and then be back-passaged into mink.
CWD
The method of transmission for CWD is completely unknown. Direct spread may occur
between animals, as the disease has a high prevalence in some areas. Vertical
transmission and oral transmission have also been suggested.
Incubation period
All TSEs have incubation periods of months or years. The incubation period of scrapie in
sheep is usually 2-5 years; cases are rare in sheep less than a year old. In goats, the
incubation period is less than three years. The incubation period of BSE is more than a
year and often several years. The peak incidence of disease occurs in 4 to 5 year old
cattle. The incubation period for mink spongiform encephalopathy is 7 to12 months.
Clinical signs
TSEs are usually insidious in onset and tend to progress slowly. In most of these
diseases, the symptoms primarily involve the nervous system. In CWD, the most
prominent symptom is wasting. Once clinical signs appear, these diseases are
relentlessly progressive and fatal.
Scrapie
The first symptoms of scrapie are usually behavioral: affected sheep tend to stand apart
from the flock and may either trail or lead when the flock is driven. As the disease
progresses, animals usually become hyperexcitable and have a high-stepping or hopping
gait, fixed stare, and head held high. Other symptoms may include ataxia, incoordination,
and trembling or convulsions when being handled. Rubbing the animal over the loins
often stimulates nibbling movements. Intense pruritus is common and may lead to
rubbing, scraping, or chewing. Most animals die 2 to 6 weeks after the onset of
symptoms, but deaths may occur up to six months later.
BSE
The clinical signs of BSE may include hyperesthesia, hindlimb ataxia, pelvic swaying,
hypermetria, tremors, falling, recumbency, and behavioral changes such as
apprehension, nervousness, and occasionally frenzy. Intense pruritus is not usually seen.
Nonspecific symptoms include loss of condition, weight loss, and decreased milk
production. Decreased rumination, bradycardia, and altered heart rhythms have also been
reported. The disease progresses to recumbency and coma, and death occurs from
weeks to months later. Rare cases may develop acutely and progress rapidly within days.
Spongiform Encephalopathy of Exotic Ruminants
The clinical signs of this disease include loss of condition, unsteadiness, incoordination,
and self-mutilation by biting. Asymptomatic cases have been described. This disease
appears to progress more rapidly than most TSEs; the mean period from the onset of
symptoms to euthanasia is 13.5 days.
FSE
The clinical signs of FSE may include behavioral changes, tremors, and ataxia. Cats may
become aggressive or tend to creep aimlessly around their home and hide. In later
stages, somnolence is common and convulsions may occur. Excessive salivation, hyper-
responsiveness to loud noises, and dilated pupils have also been seen. Death occurs
after 6 to 8 weeks
TME
The early clinical signs of TME include difficulty eating and swallowing, and changes in
normal grooming habits. Later, animals may become hyperexcitable and bite
compulsively. Affected mink often carry their tails arched over their backs like squirrels.
Other symptoms include incoordination, somnolence and sometimes convulsions. Death
occurs after 3 to 8 weeks.
Chronic Wasting Disease
The clinical signs of CWD include progressive weight loss and lassitude over several
weeks to months, with eventual severe emaciation and death. Affected animals may carry
their head low and have a fixed gaze; this may alternate with more normal alertness. In
elk, excessive salivation and teeth grinding are also common. Nervousness and
hyperexcitability may occur, but are much less common than in BSE. Pruritus has not
been seen.
Post mortem lesions
No gross lesions are found in TSEs, except emaciation or wasting of the carcass in some
cases.
The typical histopathologic lesions are confined to the central nervous system. Neuronal
vacuolation and non-inflammatory spongiform changes in the gray matter are
pathognomonic. Astrocytosis is prominent in some diseases but not others. Amyloid
plaques are seen in scrapie and CWD, but are rare in BSE and not found in TME or FSE.
Lesions are usually but not always bilaterally symmetrical.
Morbidity and Mortality
In sheep, the genetics of the host and the strain of the agent influence the onset and
severity of the disease. Theoretically, some combinations result in an incubation period
longer than the life expectancy of the sheep.
TSEs are always fatal once the symptoms appear. In scrapie, the typical mortality rate is
3-5% of the flock. In severely affected flocks, up to 20% of the animals may die annually.
In outbreaks of TME, the mortality rate may be as high as 60-80%. In 1992, the annual
incidence of BSE in United Kingdom cattle was 1%; however, the number of cases has
been decreasing in recent years. The incidence of FSE is unknown. This disease was
seen in a total of 81 domestic cats (as well as a few wild felids) in the United Kingdom, but
many cases may have been missed. The prevalence of CWD may be as high as 2.5%
among deer in Colorado and Wyoming.
Diagnosis
Clinical
TSEs should be suspected in animals that develop a slowly progressive, fatal neurologic
disease, and in elk and mule deer with chronic wasting.
Differential Diagnosis
Scrapie
The differential diagnosis of scrapie includes external parasitism (lice, mange, or itch
mites), sheep scab, Aujeszky's disease, maedi-visna, cerebral listeriosis, pregnancy
toxemia, rabies, cerebrocortical necrosis (polioencephalomalacia), abscesses or tumors
in the brain, louping ill and other tick-borne encephalitides, toxins, focal symmetrical
encephalomalacia (chronic enterotoxemia), and other degenerative central nervous
system diseases.
BSE
The differential diagnosis of BSE includes nervous ketosis, hypomagnesemia, listeriosis,
rabies, tumors, trauma to the spinal cord, and poisonings such as lead.
Laboratory Tests
TSEs have traditionally been diagnosed by histopathology. A diagnosis can also be made
by detecting PrPSc (a disease-specific isoform of the membrane protein PrP) in the
central nervous system. Accumulations of PrPSc can be found in unfixed brain extracts by
immunoblotting and in fixed brains by immunohistochemistry. The diagnosis can also be
confirmed by finding characteristic fibrils of PrPSc (scrapie-associated fibrils) with electron
microscopy in brain extracts. Some of these tests can be used on frozen or autolyzed
brains.
Scrapie can also be diagnosed by transmission tests in sheep, goats or mice and BSE
by transmission studies in mice. However, an incubation period of several months often
makes this technique impractical for diagnosis.
New commercial tests to detect BSE (PrPSc) in cattle brain samples include a modified
immunoblot, a chemiluminescent ELISA test, a sandwich immunoassay, and a two-site
noncompetitive immunometric procedure.
New tests to diagnose scrapie in live sheep are being validated. These tests include
immunohistochemical testing of eyelid associated lymphoid tissue and tonsil biopsies,
capillary electrophoresis and fluorescent labeled peptides to detect PrPSc in the blood,
and immunoblotting to detect PrPSc in blood, cerebrospinal fluid, or tissues.
Serology is not useful for diagnosis, as antibodies are not made against the TSE agents.
Samples to Collect
An animal suspected of having TSEs should be killed and the brain removed for testing.
In sheep, the cerebral spinal cord with the dorsal root ganglia should be included if
possible. Half of the longitudinally split brain is kept fresh to test for PrPSc. This portion
should be kept cold and sent to the laboratory as soon as possible on wet ice or gel
packs. The other half should be placed in 10% formo saline. In sheep, the spleen and a
variety of lymph nodes should also be collected and sent to the laboratory unpreserved.
During epidemics of BSE, it may be possible to remove only the hindbrain via the foramen
magnum for disease monitoring.
During necropsy, a standard neuropathologic approach should be followed to rule out
other causes of disease.
Recommended actions if a transmissible spongiform encephalopathy is suspected
Notification of Authorities
BSE, FSE, TME and spongiform encephalopathy of exotic ruminants are exotic diseases
and must be reported promptly to state or federal officials. Scrapie and CWD are endemic
to the United States, but are reportable diseases. An eradication program for scrapie is
ongoing in the United States.
Federal: Area Veterinarians in Charge (AVICS) (/Members/Link.plx?ID=78730)
State: State Animal Health Officials (pdf) (/Members/Link.plx?ID=78731)
Quarantine and Disinfection
Scrapie and TME can be contagious; quarantine may be necessary for their control. BSE
and some other TSEs do not appear to spread laterally.
The prototype agent, scrapie, is highly resistant to disinfectants, heat, ultraviolet
radiation, ionizing radiation, and formalin. Effective disinfection is possible with a single
porous load autoclave cycle of 134-138°C for 18 minutes. Infectious tissues should either
be autoclaved under the same conditions or incinerated. Sodium hypochlorite and sodium
hydroxide are effective chemical disinfectants; sodium hypochlorite containing 2%
available chlorine or 2 N sodium hydroxide should be applied for more than 1 hour at 20°C.
Overnight disinfection is recommended for equipment.
Public health
There is no evidence that scrapie can be transmitted to humans; however, a new variant
form of the human TSE, Creutzfeldt-Jakob disease (CJD) appears to be linked to the
agent of BSE. This human encephalopathy is progressive and fatal. There is no
treatment.
The BSE agent is classified into containment category 3.
For More Information
OIE Manual of Diagnostic Tests and Vaccines for Terrestrial Animals
(/Members/Link.plx?ID=76805)
OIE International Animal Health Code (/Members/Link.plx?ID=76790)
USDA APHIS (/Members/Link.plx?ID=78463)
USAHA Foreign Animal Diseases book (pdf) (/Members/Link.plx?ID=76798)
References
1. "Bovine spongiform encephalopathy." Animal Health Australia. The National Animal Health Information
System (NAHIS). 7 November 2001.
2. "Bovine Spongiform Encephalopathy." In Manual of Standards for Diagnostic Tests and Vaccines. Paris:
Office International des Epizooties, 2000, pp. 457-466.
3. "Bovine Spongiform Encephalopathy." In The Merck Veterinary Manual, 8th ed. Edited by S.E. Aiello and A.
Mays. Whitehouse Station, NJ: Merck and Co., 1998, pp. 897-8.
4. Irani, D.N. "Bovine Spongiform Encephalopathy." Johns Hopkins Department of Neurology. Resource on
Prion Diseases. 7 November 2001.
5. Irani, D.N. "Chronic Wasting Disease." Johns Hopkins Department of Neurology. Resource on Prion
Diseases. 7 November 2001.
6. Irani, D.N. "Feline Spongiform Encephalopathy." Johns Hopkins Department of Neurology. Resource on Prion
Diseases. 7 November 2001.
7. Irani, D.N. "Scrapie." Johns Hopkins Department of Neurology. Resource on Prion Diseases. 7 November
2001.
8. Irani, D.N. "Spongiform Encephalopathy of Exotic Ruminants." Johns Hopkins Department of Neurology.
Resource on Prion Diseases. 7 November 2001.
9. Irani, D.N. "Transmissible Mink Encephalopathy." Johns Hopkins Department of Neurology. Resource on
Prion Diseases. 7 November 2001.
10. "Scrapie." Animal Health Australia. The National Animal Health Information System (NAHIS). 7 November
2001.
11. "Scrapie." In The Merck Veterinary Manual, 8th ed. Edited by S.E. Aiello and A. Mays. Whitehouse Station,
NJ: Merck and Co., 1998, pp. 970-1.
12. "Transmissible Mink Encephalopathy." In The Merck Veterinary Manual, 8th ed. Edited by S.E. Aiello and A.
Mays. Whitehouse Station, NJ: Merck and Co., 1998, pp. 1364.
13. "Transmissible Spongiform Encephalopathies." United States Department of Agriculture Animal and Plant
Health Inspection Service. 7 November 2001.

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