Transmissible Spongiform Encephalopathy

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Transmissible Spongiform Encephalopathy

• Kuru, a Prion Disease

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Origin and Discovery

• First reported in 1957 by Gajdusek and Zigas,
  Kuru was the first human neurodegenerative
  disease discovered
• Believed to have first developed at the turn on
  the 20th century
• Restricted to natives of the Fore linguistic
  group in Papua New Guinea
• Was first classified as a slow virus disease
• Originally called cassowary disease

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Transmission

• Possibly contracted through cannibalism
• 10-15 of cases are inherited
• Many cases acquired by unknown transmission

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Pathology

• Creates Amyloid plaques and PrP (prion protein)
  deposits in the cerebral cortex and cerebellum
• Methionine at a polymorphic position 129 of the
  prion protein were preferentially affected

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Pathology

• Creates Amyloid plaques and PrP (prion protein)
  deposits in the cerebral cortex and cerebellum
• Methionine at a polymorphic position 129 of the
  prion protein were preferentially affected

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PrPsc catalyzes the transformation of PrPc into
PrPsc leading to progressive accumulation of
PrPsc in cells and tissue plaques
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Signs and Symptoms

• Limb ataxia
• Dysarthria
• Shivering tremors
• Leads to complete motor and mental incapacity and
  death

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Treatment
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Other Forms ofTransmissible Spongiform
Encephalopathy

• Creutzfeldt-Jakob disease (vCJD) -human
• Gatal familial insomnia (FFI) -human
• Scrapie - sheep
• Bovine spongiform encephalopathy (BSE) - cows

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References

• Palpers M Kuru Surveillance Team.The
  epidemiology of kuru in the period 1987 to
  1995.Commun Dis Intell. 2005
• Liberski PP, Brown P. Kuru a half-opened window
  onto the landscape of neurodegenerative
  diseases.Folia Neuropathol. 2004
• Johnson RT.Prion diseases.Lancet Neurol. 2005
  Oct
• Collinge J. Molecular neurology of prion
  disease.J Neurol Neurosurg Psychiatry. 2005 Jul
• Goldfarb LG, Cervenakova L, Gajdusek DC. Genetic
  studies in relation to kuru an overview.Curr
  Mol Med. 2004 Jun
• Aguzzi A, Polymenidou M. Comments on present-day
  spread and epidemiology of BSE and prion
  diseasesGesundheitswesen. 2004 Feb
• Goldfarb LG. Kuru the old epidemic in a new
  mirror.Microbes Infect. 2002 Jul