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The group of transmissible fatal neurodegenerative human and animal diseases was called as TSE (transmissible spongiform encephalopathy). TSE was called sometimes as prion diseases, prion dementia, transmissible degenerative encephalopathy and also as infection cerebral amyloidosis. Isoform cellular protein PrP was considered for diseases genesis. The gene, which encode of prion have in nucleus every cell of human organism occurred. This gene has exclusively in nervous, lymphatic or internal organs tissues transcript. Common characteristic of these diseases are long incubation period (a symptomatic preclinical phase were take several years), some spongiform neurodegenerative changes in brain without original inflammatory process, permanently progressive course without remission with lethal prognosis. Typical pathological finding in brain are vacuolar degeneration of neurons (particularly in grey matter), spongiform degeneration of neurofils, apoptosis and neurons dieback. Further find is accumulation of amyloid scales, there are formed from changed prion protein which is encoded in genetic information of host. This paper gives information about some new knowledge about prion diseases occurrences at people and animals, clinical characteristic, diagnosing and precaution against spread of BSE in the Slovak Republic.