Scrapie

• Ovine spongiform encephalopathy

• Scrapie is the oldest of prion diseases
• The earliest record of its occurrence in Britain
in1732
• Between 1878 and 1900, 17 countries
reported
• Australia and Newzealand free of this disease
• Prion disease –scrapie associated fibrils
 Spongiform encephalopathies in animals

• Scrapie of sheep and goats

• Transmissible mink encephalopathy(TME)
• Transmissible feline encephalopathy(TFE)
• Bovne spongiform encephalopathy(BSE)
• Crutzfeldt- Jacob disease(CJD)
• Kuru associated with cannibalism
• Gertmann- Straussler-Scheinker
syndrome(GSS)
 Etiology
• TSEs agent resistant to many chemical agents
including formalin, chloroform and ether also to
heat, ultraviolet and ionising radiation,
ultrasonication , proteases and nucleases
• The agent is proteinaceous infective particle –
glycosylated specific protein(a polypeptide) of
30 Kilodalton called prion protein(PrP)
• Prions contain no RNA or DNA and do not
produce any immune reaction in the host
 Transmission
• From the infected ewes to their lambs early in
life(vertical)
• Prolonged incubation period 1-5 yrs and
course of the disease 2-12 months
• The placenta is infectious
• Environmental contamination persists for long
life(2-5 yrs) restricting the disease to adult
sheep
 Pathogenesis
• Pathogenesis of TSE involves complex molecular
mechanisms
• PrP occurs normally in the CNS of all domestic
animals also humans bound to surface of neurons
• Animals susceptible to prion disease bear a gene
(PrPc gene) which convert to PrPsc (scrapie
associated fibril protein)
• It has a high beta –pleated sheet comformation
characteristic of amyloid protein
• Following oral infection at an early age the infective
agents present in low concentration in the tonsil,
suprapharyngeal lymph node and lymphoid spleen
• The infective agent continue to replicate lymphoid
tissues for even a year or more before it reaches CNS
• CNS is infected by hematogenous spread affects
medulla oblongata, diencephalon and spreads to other
parts
• Clinical disease usually occurs when sheep are 3-4 yrs
of age
 Clinical signs
• Onset of restlessness and a startled look
• The pupils are dilated
• Tremor of head and neck
• Hypersensitivity, ataxia, incoordination
• The sheep hold its head down and move it
rapidly from side by side or up and down
• Movements are aimless and stiffness of
forelegs results in trotting gait
• The animal usually grinds its teeth and voice
may be altered
• Twitching confined to lips and soon involve
the muscles of shoulder and thigh as well
• The skin irritation begins in the lumbar region
• They may involve the rest of body
• The intense pruritis causes the sheep to rub
against objects continuously until the wool
coat is almost completely lost
• Incoordination may be followed by paralysis,
inability to stand and finally dies
 Lesions
• No characteristic gross lesions are found
• Microscopic lesions are limited to CNS
• They are limited to medulla oblogata, pons, mid brain and in
spinal cord
• The presence of large vacuoles in cytoplasm of neurons
• Neuronal degeneration, astrocytosis and variable spongiosis
with neuronal and neuropil vacuolation mainly in gaey
matter
• Pronounced hypertrophy and hyperplasia of astrocytes are
characteristic of scrapie
• Abnormal protein first accumulates in astrocytes
 Diagnosis
• Clinical signs microscopic examination of the
brain and spinal cord
• Eye lid test – skin samples from sheep’s eye lid
and tested for PrP using a monoclonal
antibodies that binds tightly to protein
• IHC and ELISA
Differential diagnosis
Listeriosis, Rabies, Pregnancy toxaemia , toxins