Table 4.

Modified diagnostic criteria for sporadic Creutzfeldt-Jakob disease (CJD)

Definite CJD
• Neuropathologically confirmed and/or
• Abnormal prion protein isoform immunochemically confirmed by immunocytochemistry or western blot
• Scrapie-associated fibrils
Probable CJD
• Progressive dementia with at least two of four clinical features:
1. Myoclonus
2. Visual or cerebellar signs
3. Pyramidal or extrapyramidal signs
4. Akinetic mutism
• Periodic sharp and slow wave complexes (PSWC) in EEG
• 14-3-3 proteins in CSF and duration of <2 years
Possible CJD
• Clinical features as above
• No PSWC in EEG
• No 14-3-3 detection in CSF
• Duration of <2 years