Table 3.
Current diagnostic criteria for vCJD as reported by Heath et al20 in 2010
| Subsection | Item | Criterion |
| 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 features* |
| B | Persistent painful sensory symptoms† | |
| 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 illness |
| B | Bilateral pulvinar high signal on MRI scan | |
| IV | A | Positive tonsil biopsy§ |
| Definite | IA and neuropathological confirmation of vCJD ¶ | |
| Probable | I and 4/5 of II and IIIA and IIIB; or I and IVA | |
| Possible | I and 4/5 of II and IIIA | |
*Depression, anxiety, apathy, withdrawal, delusions.
†This includes frank pain and/or dysaesthesia.
‡The typical appearance of the EEG in sporadic CJD consists of generalised triphasic periodic complexes at approximately 1/s. These may be occasionally seen in the late stages of vCJD.
§Tonsil biopsy is not recommended routinely, nor in cases with EEG appearances typical of sporadic CJD, but may be useful in suspect cases in which the clinical features are compatible with vCJD and MRI does not show bilateral pulvinar high signal.
¶Spongiform change and extensive prion protein deposition with florid plaques throughout the cerebrum and cerebellum.
EEG, electroencephalography; TSE, transmissible spongiform encephalopathy; vCJD, variant Creutzfeldt-Jakob disease.