Table 4

Current (2002) diagnostic criteria for variant Creutzfeld-Jakob disease (vCJD)

Definite: 1A (progressive neuropsychiatric disorder) and neuropathological confirmation of vCJD.
Probable: All group 1 items and four or five from group 2 and 3a and 3b (or all group 1 items and group 4a).
Group 1 features
(a) Progressive neuropsychiatric disorder (depression, anxiety, withdrawal, delusions)
(b) Duration of illness > 6 months
(c) Routine investigations do not suggest an alternative diagnosis
(d) No history of potential iatrogenic exposure
Group 2 features
(a) Early psychiatric symptoms (depression, anxiety, apathy, withdrawal, delusions)
(b) Persistent painful sensory symptoms (includes frank pain and/or unpleasant dysaesthesia)
(c) Ataxia
(d) Myoclonus or chorea or dystonia
(e) Dementia
Group 3
(a) EEG does not show the typical appearance of sporadic CJD (or no EEG performed)
(b) Bilateral pulvinar high signal on MRI scan
Group 4
(a) Positive tonsil biopsy