Creutzfeldt Jakob disease

Case classification

A. Possible case
All of the following:
– Progressive dementia; and
– EEG atypical or not known and
– Duration <two years
and
– at least 2 out of the following four clinical features: myoclonus, visual or cerebellar disturbance, pyramidal/extrapyramidal dysfunction, akinetic mutism
B. Probable case
(in the absence of an alternative diagnosis from routine investigation)
– Progressive dementia
and
– At least 2 of the following 4 clinical features: myoclonus, visual or cerebellar disturbance, pyramidal/extrapyramidal dysfunction, akinetic mutism
and
– A typical EEG, whatever the clinical duration of the disease
and/or
– A positive 14-3-3 assay for CSF and a clinical duration to death <2 years
C. Confirmed case
– Neuropathological confirmation and/or
– Confirmation of protease-resistant prion protein (PrP) (immunocytochemistry or Western blot)
and/or
– Presence of scrapie-associated fibrils

Current as of: 1 January 2019