Table 1.
Diagnostic criteria CJD based on the Rotterdam criteria and the UK criteria
| SPORADIC CDJ | ||
|---|---|---|
| Criteria | ||
| I | Rapidly progressive dementia | |
| II | A | Myoclonus |
| B | Visual or cerebellar problems | |
| C | Pyramidal or extrapyramidal features | |
| D | Akinetic mutism | |
| III | CJD-typical periodic sharpwave complexes in electroencephalography | |
| IVa | High signal in caudate/putamen on MRI brain scan | |
| Classification | ||
| Confirmed sporadic CJD | Neuropathological/immunocytochemical confirmation | |
| Probable sporadic CJD | I and 2 of II and III OR I and 2 of II and IVa OR possible sporadic CJD and positive 14-3-3 | |
| Possible sporadic CJD | I and 2 of II and duration < 2 years | |
| aAs of 2010 | ||
| IATROGENIC CJD | ||
| Progressive cerebellar syndrome in a pituitary hormone recipient OR sporadic CJD with a recognised exposure risk, e.g., dura mater transplant | ||
| FAMILIAL CJD | ||
| Confirmed or probable CJD plus confirmed or probable CJD in a first-degree relative OR neuropsychiatric disorder plus disease-specific PRNP mutation | ||
| VARIANT CDJ | ||
| Criteria | ||
| I | A | Progressive neuropsychiatric disorder |
| B | Duration of illness > 6 months | |
| C | Routine investigations do not suggest an alternative diagnosis | |
| D | No history of potential iatrogenic exposure | |
| E | No evidence of a familial form of TSE | |
| II | A | Early psychiatric featuresa |
| B | Persistent painful sensory symptomsb | |
| C | Ataxia | |
| D | Myoclonus or chorea or dystonia | |
| E | Dementia | |
| III | A | EEG does not show the typical appearance of sporadic CJD in the early stages of illnessc |
| B | Bilateral pulvinar high signal on MRI scan | |
| IV | A | Positive tonsil biopsy |
| Classification | ||
| Confirmed variant CJD | IA and neuropathological confirmation of vCJDd | |
| Probable variant CJD | I and 4/5 of II and IIIA and IIIB OR 1 and IVA | |
| Possible variant CJD | I and 4/5 of II and IIIA | |
aDepression, anxiety, apathy, withdrawal, delusions
bIncluding both frank pain and/or dysaesthesia
cGeneralised triphasic periodic complexes at approximately one per second
dSpongiform change and extensive PrP deposition with florid plaques, throughout the cerebrum and cerebellum