Le Encefalopatie Spongiformi Transmissibili (TSE)

Abstract

Transmissible Spongiform Encephalopathies (TSE) are a group of neurodegenerative diseases affecting the central nervous system of men and animals. All Transmissible Spongiform Encephalopathies are characterized by a long incubation period, by a slow-progressing but fatal clinical course and by central nervous system injured tissues. This disease is called after the encephalic lesions, which appear under the microscope as optically empty areas and which resemble a "sponge". No inflammatory lesions nor immune reactions within the body are detected, while a specific protein, referred to as a prion, is constantly present. Such protein is called PrPres or PrPsc. So far, the following forms have been described in the human beings: Creutzfeldt-Jakob disease (CJD); Gerstmann-Straussler-Scheinker Syndrome (GSS); Kuru; Fatal familial insomnia (IFF); variant Creutzfeldt-Jakob disease (vCJD). In the animals, they are represented by goat/sheep/moufflon Scraple; Bovine Spongiform Encephalopathy (BSE); Transmissible Mink Encephalopathy (TME); Deer/Elk Chronic Wasting Disease (CWD); Feline Spongiform Encephalopathy (FSE); Exotic Ruminant Spongiform Encephalopathy; Ostrich Spongiform Encephalopathy.