Probable vCJD

Criteria for Probable Variant Jakob-Creutzfeldt Disease
(UK DoH 2003)

There are two sets of criteria for patients with probable vCJD.

Criteria A

1. Progressive neuropsychiatric disorder for more than 6 months
2. Routine investigations do not suggest an alternative diagnosis
3. At least 4 of the following 5 symptoms:
   1. Early psychiatric symptoms (depression, anxiety, apathy withdrawal, delusions)
   2. Persistent painful sensory symptoms (including both frank pain and/or unpleasant dysaesthesia)
   3. Ataxia
   4. Myoclonus, chorea or dystonia
   5. Dementia
4. EEG does not show the typical appearances of sporadic CJD or no EEG has been performed
5. Symmetrical high signal in the posterior thalamus on a MRI brain scan (Zeidler et al 2000)
6. No history of potential iatrogenic exposure

Criteria B

1. Progressive neuropsychiatric disorder for a period of longer than 6 months
2. Routine investigations do not support an alternative diagnosis
3. No history of potential iatrogenic exposure
4. Positive tonsil biopsy, which is positive for PrP-res