Prion diseases, also called transmissible spongiform encephalopathies (TSEs), are a family of neurodegenerative disorders affecting both humans and animals. They are caused by the accumulation of an abnormal form of a protein known as prion that results in neuronal death and a characteristic spongiform appearance of the brain tissue. Human prion diseases can be sporadic, acquired or hereditary. Acquired prion diseases have been linked to entering contaminated food into the human food chain, failure to completely disinfect or sterilize contaminated surgical instruments, patients receiving tissues and organs from infected donors, recipients of blood and other biological contaminated products, and potentially to cross infection in dental procedures. At present, there is unfortunately no efficient therapy that can be administered to clinically infected patients with prion diseases. Moreover, there are no simple diagnostic tests that can be used to show the agent of transmissible spongiform encephalopathy during the preclinical phase of the disease. Therefore, to prevent the spread of this emerging infectious agent it is necessary to implement several health control strategies and maintain surveillance for subclinical infections.