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