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Prion research Göttingen

 

Diagnostic criteria for sporadic CJD

Rapidly progressive dementia (obligatory)

I. clinical signs:
1. dementia
2. cerebellar or visual
3. pyramidal or extrapyramidal
4. akinetic mutism

II. tests:
1. PSWCs in EEG
2. 14-3-3 detection in CSF (in patients with a disease duration of less than 2 year)
3. high signal abnormalities in caudate nucleus and putamen or at least two cortical regions (temporal-parietal-occipital) either in DWI or FLAIR

Probable CJD
Rapidly progressive dementia and two out of I and at least one out of II

Possible CJD
Rapidly progressive dementia and two out of I and duration less than 2 years