WHO diagnostic criteria for Creutzfeldt-Jakob disease (CJD)

Diagnostic criteria

• probable CJD (all 4)
  • progressive dementia
  • at least 2 of the following
    • myoclonus
    • visual or cerebellar disorder
    • pyramidal &/or extrapyramidal disorder
    • akinetic mutism
  • characteristic electroenephalogram pattern with periodic sharp wave complexes &/or 14-3-3 identified in cerebrospinal fluid & clinical duration to death < 2 years
  • routine investigation does not support alternative diagnosis
• definitive CJD
  • probable CJD plus at least one of the following
    • loss of neurons, gliosis, spongiform degeneration, or plaques positive for PrPSc on histopathology from brain biopsy
    • positive PrPsc staining following pretreatment of brain tissue with proteinase K to destroy PrPC activity
    • positive histoblotting of brain tissuw extracts for treatment with proteinase K to destroy PrPC activity
    • transmission of characteristic neurodegenerative disease to experimental animals
    • identification of PRNP gene mutations

More general terms

• criteria

Additional terms

• Creutzfeldt-Jakob [CJ] disease
• World Health Organization (WHO)