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WHO diagnostic criteria for Creutzfeldt-Jakob disease (CJD)
Diagnosis:
1) probable CJD (all 4)
a) progressive dementia
b) at least 2 of the following
- myoclonus
- visual or cerebellar disorder
- pyramidal &/or extrapyramidal disorder
- akinetic mutism
c) characteristic electroenephalogram pattern with periodic sharp wave complexes &/or 14-3-3 identified in cerebrospinal fluid & clinical duration to death < 2 years
d) routine investigation does not support alternative diagnosis
2) 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
Related
Creutzfeldt-Jakob [CJ] disease
World Health Organization (WHO)
General
criteria
References
- Medical Knowledge Self Assessment Program (MKSAP) 15, 18.
American College of Physicians, Philadelphia 2009, 2018.