254.3 📌 章末速蚘

  • Prion: protein-only infectious agent (PrPSc); causes TSE (transmissible spongiform encephalopathy)
  • Sporadic CJD (85%): rapid dementia + myoclonus + ataxia; median survival 4-6 mo
  • vCJD: BSE-related; younger onset 28 yr; tonsil + appendix biopsy+
  • MRI: cortical ribbon + basal ganglia hyperintensity (DWI/FLAIR); “hockey stick sign” in vCJD
  • EEG: periodic sharp wave complexes (PSWC) in sCJD
  • CSF: RT-QuIC (most sensitive + specific) + 14-3-3 + tau
  • No treatment; universally fatal
  • Special prion decontamination for surgical instruments
  • Differential: rule out treatable causes (autoimmune encephalitis, HSV, etc.)
  • CWD in deer/elk → no documented human transmission but vigilance
254.3.0.0.1 盧醫垫 hint
  • Rapidly progressive dementia + myoclonus + ataxia → CJD considered; RT-QuIC + MRI + EEG; rule out treatable (autoimmune encephalitis especially)
  • Family history of dementia → genetic CJD / familial prion testing
  • UK resident 1980-1996 + neurodegeneration → vCJD consideration
  • DON’T MISS treatable causes of rapidly progressive dementia