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CNS Infection

Transmissible spongiform encephalopathies

(Prion disease) Q
  1. Creutzfeldt - Jakob disease
  2. Gestmann - straussler - scheinker syndrome (GSS)
  1. Fatal familial insomnia
  2. Kuru
Scrapie Q → in sheep & goats
Mink transmissible eucephalopathy
Bovine spongiform encephalopathy (mad cow disease)
Clinically →Dementia
'Prusiner' Won noble prize in 1997 for discovery of prions
PrpSc (n α - helix isoform) β pleated sheet isoform (PrPSC / Prpres)  Q
Higher rate if mutations present in Prpsc (like occurs in familial forms)
pApsc, facilitates, in cooperative manner comparable transfer of other molecules PrPSC infectious nature of Prpsc molecule comes from their ability to corrupt the integrity of normal cellular constituents
Gene on chromosome 20, termed PRNP, codes for Prpsc
In familial → point mutation or Met/val polymorphism at codon 12q
(Met → FFI
Val →     CID

Light Microscopy Spongiform transformation of cerebral cortex, & deep cortex, & deep gray matter structure

Kenn plaques: Congo red +ve, PAS +ve
Clinically: 85% sporadic; Rapidly programe dementia. Q
Iatrogenic → corneal implants, deep implantation electrodes, human GH
AT 7th decade
Fatal, duration - 7 months
Variant CJD: Q
  1. Younger adults
  2. Slower progression
  3. Behaviour disorders in early stages

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