Human prion diseases i.e čreutzfeldt-Jakob disease (ČJD), Gerstmann-Stráussler-Scheinkeťs syndrome, fatal insomnia and kuru are rare fatal diseases that are historically, clinically, neurobiologically and epidemiologically fascinating. The sinuous history of research into prion diseases, which started as early as 1738, continues to this today. The discovery of the nature of prions has changed the concept of infectious diseases. The disclosure of probable prion replication has altered fundamental biological dogma. The Nobel Prize was awarded for both of these breakthroughs (čarleton Gajdusek 1976, Stanley Prusiner, 1997). Prion's resistance to common methods of disinfection is the reason why there is obligatory surveillance of prion diseases in Europe. The čzech National Reference Laboratory of Human Prion Diseases (NRL) was founded in 2001 at the Department of Pathology of Thomayeťs Teaching Hospital. The NRL was included in the network of European prion surveillance laboratories in 2003. Refurbishment and expansion of the NRL in 2006 with the latest technology, has enabled the neurohistological, immunological and molecular genetic detection and differential diagnosis of human prion diseases and other neurodegenerative diseases. The čzech NRL is the first laboratory in the world to Ímmunologically examine the brains of all cornea donors. The annual incidence of sporadic ČJD in the čzech Republic is the samé as in other European countries: 1/106. However, in reality it is probably higher since not all cases of probable and possible ČJD are clinically diagnosed. As part of the NRL, the nascent centre of Neurodegenerative Diseases enables the diagnosis of prion and/or other neurodegenerative diseases for the whole of the čzech Republic.

        Key words: prion diseases, ČJD, National Reference Laboratory of Human Prion Diseases