Creutzfeldt-Jacob Disease (CJD) (prion Disease)

Your Path

  • Creutzfeldt-Jacob Disease (CJD) (prion disease)


  • Transmissible Spongiform Encephalopathy (TSE)
  • Infectious variant (uncommon) can be spread by contact w/infected brain matter (via prions)
  • Most common is sporadic form, which is non-infectious
  • Also a hereditary form

Risk Factors

  • Avg age onset in 60s
  • Though can affect old & young (<50)


  • Rapidly progressive decline in memory, cognition, thinking
  • Other manifestations include aphasia, disorientation, apraxia, visual-spatial impairment
  • Onset to death w/in 1y
  • Coordination problems and sudden jerking movements (myoclonus) may come first

Physical Exam Findings

  • Coordination problems and myoclonus
  • Sometimes ataxia, nystagmus
  • Sometimes hyperreflexia