BACKGROUND: Creutzfeldt-Jakob disease (CJD) is a prion disease. It is a rare, rapidly progressing fatal disorder of the central nervous system, which occurs in four forms: sporadic (sCJD), genetic/familial (gCJD), iatrogenic (iCJD), and variant (vCJD). METHODS: CJD research in the Czech Republic (CR) is conducted by the National Reference Laboratory for Human Transmissible Spongiform Encephalopathies and Creutzfeldt-Jakob Disease, Department of Pathology and Molecular Medicine, established in 2001 at the Department of Pathology, Thomayer Hospital, Prague. In 2003, this NRL was included in the European network of laboratories monitoring prion diseases. The purpose of the article is to analyse data reported to the EPIDAT system. RESULTS: From June 2000 to June 2017, 207 deaths in persons diagnosed with CJN and four suspected deaths due to gCJD were reported to the EPIDAT system (national program of reporting, recording, and analysis of data on transmissible diseases in the CR). CONCLUSION: Reporting CJD cases to the EPIDAT is helpful in meeting the important goals, i.e. monitoring the incidence and trends of the disease. The incidence of gCJD in particular requires improved diagnosis based on a detailed personal and family history, and thorough epidemiological investigation is crucial to detect possible iatrogenic diseases.

Detection of Prions in Brain Homogenates and CSF Samples Using a Second-Generation RT-QuIC Assay: A Useful Tool for Retrospective Analysis of Archived Samples