sporadic cJD

- affects older age (peak incidence 7th decade), as opposed to variant cJD (younger age)
- presents with rapidly progressive dementia with associated neurological signs (cerebellar ataxia and visual dysfunction)
- myoclonic jerks are characteristic
- typical EEG changes (1Hz periodic discharge)
- positive CSF result for 14-3-3 protein
- progression to akinetic mute state followed by death (2/3 die within 6 months)