Diagnostic criteria for the new variant of CJD (vCJD)

(according to the WHO) [Will et al. 2000]

A Progressive neuropsychiatric disorder
B Disease duration of more than six months
C Routine examinations do not suggest any other diagnosis
D No indication of a possible iatrogenic cause
E No indication of a familial prion disease

A Psychiatric symptoms early in the disease
B Persistently impaired sensation
C Ataxia
D Myoclonus or other movement disorders
E Dementia

A No CJD-typical EEG changes
B Bilateral pulvinar sign on MRI

A Positive tonsillar biopsy *

Definite: I A and neuropathological confirmation of vCJD
Probable: I and 4/5 of II and III A and III B or I and IV A
Possible: I and 4/5 of II and III A

* Tonsillar biopsy is not recommended as a routine procedure, not even in cases of “CJD-typical EEGs”. However, it may be helpful in patients with clinical symptoms similar to those of vCJD but with no bilateral pulvinar sign on MRI.