Variant Creuzfeld-Jacob Disease

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  • linked to consumption of beef contaminated with Bovine Spongiform Encephalopathy
  • prion disease

Epidemiology

  • first recognised in 1996
  • affects young people in mid-20s
  • only get if homozygous for methionine at 129 of protein

Clinical Features

  • psychiatric symptoms
    • depression
    • hallucinations
    • delusions
  • neurological
    • sensory
      • paraesthesia
    • motor
      • gait
      • chorea
  • cognitive
    • forgetfulness
  • death
    • due to bulbar paralysis

Investigations

  • biopsy of tonsils / brain
  • MRI
    • pulvinar (in the thalamus) hyper-intensity
 

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