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Transmissible Spongiform Encephalopathies (TSE’s)

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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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Presentation Transcript
slide3
Deer Brain Tissue

Normal CWD

Spongiform

Vacuoles

Neurons

slide4
Transmissible Spongiform Encephalopathies

(TSE’s)

Animal TSE’s

Human TSE’s

CJD

vCJD

BSE

CWD

Scrapie

Others

Others

BSE Prevention

characteristics of all tse s
Characteristics of all TSE’s
  • Central Nervous System Failure
  • Long Incubation Period
  • No Cure
  • Death
human transmissible spongiform encephalopathies
HumanTransmissible Spongiform Encephalopathies
  • Creutzfeldt-Jakob Disease (CJD)
  • Fatal Familial Insomnia (FFI)
  • Gerstmann-Straussler Syndrome (GSS)
  • Kuru
  • Variant Creutzfeldt-Jakob Disease (vCJD)
animal transmissible spongiform encephalopathies
AnimalTransmissible Spongiform Encephalopathies
  • Scrapie
  • Transmissible Mink Encephalopathy (TME)
  • Chronic Wasting Disease (CWD)
  • Bovine Spongiform Encephalopathy (BSE)
animal transmissible spongiform encephalopathies9
AnimalTransmissible 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
cause
Cause?
  • Agent not fully characterized
  • Smaller than smallest known virus
  • No detectable immune or inflammatory response
  • Disinfectants
  • 600oC (1112oF)
  • 3 year burial
agent theories
Agent Theories
  • VIRUS
  • VIRION
  • PRION
agent theories12
Agent Theories
  • VIRUS
  • VIRION
  • PRION
vcjd from bse
vCJD from BSE?
  • vCJD -Human
  • BSE – Cattle
  • Prion Protein is Indistinguishable
  • Feline Spongiform Encephalopathy (FSE)
  • TSE in Kudu and Nyala
confirmed cases bse in uk
Confirmed Cases BSE in UK

C

O

W

S

Data from Office International Des Epizooties

slide16
UK

CowsBSE

UK

PeoplevCJD

bse diagnostics
BSE Diagnostics
  • No live animal test
  • Need brain tissue
  • “Negatives” do not guarantee absence of infectivity
distribution of infectivity
Distribution of Infectivity
  • NeverFound in
  • Milk
  • Skeletal muscle of food animals

spinal cord

dorsal root ganglia

brain

trigeminal ganglia

eye

bone marrow

distal ileum

slide19
Distribution of Infectivity

BSE in Sheep

pituitary

brain

spinal cord

vagus nerve

Blood

lymph nodes

spleen

intestine

prevention in u s
Prevention in U.S.
  • Import Regulations
  • Feed Rule
  • Surveillance
  • Formal Risk Assessment
  • Response Plan
slide21
Import Restrictions - countries with BSE
    • July 1989 - live ruminants
    • November 1989 - ruminant products

Import Regulations

  • Import Restrictions - Europe
    • December 1997
    • All live ruminants and most ruminant products
slide22
Import Restrictions - Europe
    • December 2000 – all rendered animal protein regardless of species

Import Regulations

  • Restrictions extended to Japan September 2001
  • Evaluations of other countries
u s feed rule
Milk Products
  • Blood Products
  • Gelatin
  • Pure Swine (Pork)
  • Pure Equine (Horse)
  • Plate Waste
U.S. Feed Rule

Prohibits Feeding Ruminants Mammalian Proteinexcept:

u s feed rule24
Pure Swine (Pork)
  • Pure Equine (Horse)
U.S. Feed Rule

Prohibits Feeding Ruminants Mammalian Proteinexcept:

  • Milk Products
  • Blood Products
  • Gelatin
  • Plate Waste
bse samples may 1990 through september 30 2002
BSE Samples May 1990 through September 30, 2002

C

O

W

S

Total 36,594

40

Year

Data from USDA APHIS NVSL

formal risk assessment
Formal Risk Assessment
  • Harvard / Tuskegee Universities
  • Began in 1998
  • Released November 30, 2001
harvard tuskegee risk assessment
Harvard / TuskegeeRisk 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.
harvard tuskegee study conclusion
Harvard/Tuskegee StudyConclusion
  • “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.”
bse response plan
BSE Response Plan
  • First version early 1990's
  • Updated often to reflect new scientific knowledge and personell changes.
plan expectations
Plan Expectations
  • Herd Quarantined
  • Presumptive Positive Sample Hand Carried to UK for Confirmation.
  • Herd Purchased and Destroyed by Government
  • Trace Animals and Feed
producer preventive actions
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.
record keeping requirement for ruminant producers
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
slide35
http://www.wa.gov/agr

Neil Lanning

nlannning@agr.wa.gov

360-902-2052

scrapie id program
Scrapie ID Program

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

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

Linda.V.Carpenter@aphis.usda.gov

Olympia Office USDA APHIS VS

360-753-9430

slide39
DeerBrain Tissue Normal

Sectioning Artifact

Neurons

BloodVessel

Nuclei of Glial Cells

slide40
Deer Brain Tissue Chronic Wasting Disease

Nuclei of Glial Cells

Neurons

Neuron

Spongiform Vacuoles

Blood Vessel

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