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Transmissible Spongiform Encephalopathies (TSE’s) Deer Brain Tissue Normal CWD Spongiform Vacuoles Neurons Transmissible Spongiform Encephalopathies (TSE’s) Animal TSE’s Human TSE’s CJD vCJD BSE CWD Scrapie Others Others BSE Prevention

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Deer Brain Tissue

Normal CWD

Spongiform

Vacuoles

Neurons


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Transmissible Spongiform Encephalopathies

(TSE’s)

Animal TSE’s

Human TSE’s

CJD

vCJD

BSE

CWD

Scrapie

Others

Others

BSE Prevention


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Forms of Transmissible Spongiform Encephalopathies (TSE’s)

  • Sporadic

  • Inherited

  • Acquired

  • Iatrogenic


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Characteristics of all TSE’s

  • Central Nervous System Failure

  • Long Incubation Period

  • No Cure

  • Death


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Human Transmissible Spongiform Encephalopathies

  • Creutzfeldt-Jakob Disease (CJD)

  • Fatal Familial Insomnia (FFI)

  • Gerstmann-Straussler Syndrome (GSS)

  • Kuru

  • Variant Creutzfeldt-Jakob Disease (vCJD)


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Animal Transmissible Spongiform Encephalopathies

  • Scrapie

  • Transmissible Mink Encephalopathy (TME)

  • Chronic Wasting Disease (CWD)

  • Bovine Spongiform Encephalopathy (BSE)


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Animal Transmissible Spongiform Encephalopathies

  • Scrapie in Sheep and Goats

  • Transmissible Mink Encephalopathy (TME)in Mink

  • Chronic Wasting Disease (CWD)in Deer and Elk

  • Bovine Spongiform Encephalopathy (BSE)

  • in Cattle, Cats, Kudu, and Nyala


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Cause?

  • Agent not fully characterized

  • Smaller than smallest known virus

  • No detectable immune or inflammatory response

  • Disinfectants

  • 600oC (1112oF)

  • 3 year burial


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Agent Theories

  • VIRUS

  • VIRION

  • PRION


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Agent Theories

  • VIRUS

  • VIRION

  • PRION


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vCJD from BSE?

  • vCJD -Human

  • BSE – Cattle

  • Prion Protein is Indistinguishable

  • Feline Spongiform Encephalopathy (FSE)

  • TSE in Kudu and Nyala


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Confirmed Cases BSE in UK

C

O

W

S

Data from Office International Des Epizooties



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UK

CowsBSE

UK

PeoplevCJD


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BSE Diagnostics

  • No live animal test

  • Need brain tissue

  • “Negatives” do not guarantee absence of infectivity


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Distribution of Infectivity

  • NeverFound in

  • Milk

  • Skeletal muscle of food animals

spinal cord

dorsal root ganglia

brain

trigeminal ganglia

eye

bone marrow

distal ileum


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Distribution of Infectivity

BSE in Sheep

pituitary

brain

spinal cord

vagus nerve

Blood

lymph nodes

spleen

intestine


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Prevention in U.S.

  • Import Regulations

  • Feed Rule

  • Surveillance

  • Formal Risk Assessment

  • Response Plan


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Import Regulations

  • Import Restrictions - Europe

    • December 1997

    • All live ruminants and most ruminant products


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Import Regulations

  • Restrictions extended to Japan September 2001

  • Evaluations of other countries


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  • Blood Products

  • Gelatin

  • Pure Swine (Pork)

  • Pure Equine (Horse)

  • Plate Waste

U.S. Feed Rule

Prohibits Feeding Ruminants Mammalian Proteinexcept:


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  • Pure Equine (Horse)

U.S. Feed Rule

Prohibits Feeding Ruminants Mammalian Proteinexcept:

  • Milk Products

  • Blood Products

  • Gelatin

  • Plate Waste


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Confirmed Cases BSE in UK

C

O

W

S

Feed Ban


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BSE Samples May 1990 through September 30, 2002

C

O

W

S

Total 36,594

40

Year

Data from USDA APHIS NVSL


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Formal Risk Assessment

  • Harvard / Tuskegee Universities

  • Began in 1998

  • Released November 30, 2001


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Harvard / Tuskegee Risk Assessment

  • Describe potential pathways of BSE infectivity into national herd or food supply.

  • Characterize and quantify steps in pathways.

  • Identify key steps for research or risk management.


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Harvard/Tuskegee Study Conclusion

  • “Our analysis finds that the U.S. is highly resistant to any introduction of BSE or a similar disease.”

  • “BSE is extremely unlikely to become established in the U.S.”


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BSE Response Plan

  • First version early 1990's

  • Updated often to reflect new scientific knowledge and personell changes.


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Plan Expectations

  • Herd Quarantined

  • Presumptive Positive Sample Hand Carried to UK for Confirmation.

  • Herd Purchased and Destroyed by Government

  • Trace Animals and Feed


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Producer Preventive Actions

  • Look for and obey cautionary statement “Do Not Feed Cattle or Other Ruminants” on feed labels.

  • Be aware that Pet Food often contains prohibited materials.

  • Avoid cross contamination of ruminant feed with feeds that contain prohibited material.

  • Keep feed records.


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Record Keeping Requirement for Ruminant Producers

  • Any feeds fed containing any animal protein, regardless of species fed, excluding pet food fed to pets.

  • Invoices – date, quantity, supplier

  • Feed Labels

  • Minimum of 1 year


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BSE NEVERFound in U.S.


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http://www.wa.gov/agr

Neil Lanning

[email protected]

360-902-2052


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Scrapie ID Program

http://www.aphis.usda.gov/vs/scrapie.htm

http://animalagriculture.org/scrapie/scrapie.htm

[email protected]

Olympia Office USDA APHIS VS

360-753-9430




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Deer Brain Tissue Normal

Sectioning Artifact

Neurons

BloodVessel

Nuclei of Glial Cells


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Deer Brain Tissue Chronic Wasting Disease

Nuclei of Glial Cells

Neurons

Neuron

Spongiform Vacuoles

Blood Vessel



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Bone Meal





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