Neurológia pre prax 5/2017

Case reports of sporadic variants of Creutzfeldt-Jakob disease

Prion diseases are the group of rare neurodegenerative diseases affecting both humans and animals, characterized by a long incubation period, rapidly progressing symptoms and infaust prognosis without the possibility of influencing the course of disease by therapy. The basic pathophysiological process is accumulation of infectious protein – prion in the brain tissue with its irreversible spongiform damage. The most common human prion disease is Creutzfeldt-Jakob disease. It should be considered in differential diagnostic process especially in cases of rapidly progressive dementia in combination with other neurological symptoms. The aim of this article is to really point out the issue of prion diseases and to briefly describe it in following case-reports of sporadic Creutzfeldt-Jakob disease which was diagnosed at our department in years 2015 and 2016.

Keywords: prion diseases, prion, sporadic Creutzfeldt-Jakob disease, dementia