Abstract
Prion diseases are caused by proteinous infectious particles, which are very resistant to usual sterilization procedures. This text should be very short review of variants of Creutzfeldt Jacob disease, when the most frequentley form is the new variant, which we diagnosed on our clinic, like illustrate our 3 casuistries.
The diagnosis is based on clinical findings (rapidly progressive dementia, pyramidal or extrapyramidal involvement, myoclonus and akinetic mutism) and positive 14-3-3 protein in CSF. Later were added the hyperintensities on MR (DWI and FLAIR) in subcortical (putamen, caudate) and some cortical areas.
Final diagnosis is possible only post mortem. CJD is lethal, rapidly progressive disease without casual therapy.