[New findings on the diagnosis and therapy of prion diseases].

Spongiform encephalopathies are the fatal diseases, that affect the brain tissue of mammals. They are caused by a conformational changed prion protein. There is no adequate diagnostic test for in vivo identification of prion protein. Disease can be diagnosed only by clinical sings and EEG in new variant of Creutzfeldt-Jakob disease. Post mortem, histopathological examination of brain tissue reveals spongiform changes and immunohistochemistry detects disease-related prion protein. Appropriate diagnostic in vivo tests are not developed yet; therefore extensive researches are ongoing aimed to introduce such methods. This review describes a few promising experimental methods, which may develop into diagnostic tests in the future: detection of prions in urine samples, PMCA (protein misfolding cyclic amplification), DATAS (differential analysis of transcripts with alternative splicing), SELEX (in vitro selection), detection of prions in tonsils and detection of copper and manganese dysbalance in tissues. Current therapy strategy is based on testing of some known drugs (quinacrine, chlorpromazine), and antioxidant and antibody treatments. The detection of NSE (neuron-specific enolase) and cholesterol in meat products reveals the presence of brain and spinal cord tissue. The spreading of spongiform encephalopathies can be diminished by utilising the adequate in vivo diagnostic tests, effective therapy strategy and preventive steps.