Table 1.

CDC Diagnostic Criteria for Creutzfeldt-Jacob Disease

DEFINITE sCJD
Progressive neurological syndrome AND
Neuropathologically OR
Immunocytochemically OR biochemically confirmed
PROBABLE sCJD
I+2 of II and typical MRI brain scan
OR I + 2 of II and typical EEG
OR I + 2 of II and positive 14.3.3
OR Progressive neurological syndrome and positive CSF RT-quIC in CSF
or other tissues
POSSIBLE sCJD
1 + 2 of II and disease duration < 2 years
Exclusion of other cause in complete diagnostic work up
SYMPTOMS
I    Rapidly progressive cognitive impairment
II   Myoclonus
Visual or cerebellar problems
Pyramidal or extrapyramidal features
Akinetic mutism
SUPPORTIVE TESTS
MRI  High signal in caudate/putamen on MRI brain scan OR≥2
cortical regions on diffusion-weighted imaging or on FLAIR
EEG  Periodic sharp waves complexes