ABSTRACT

INTRODUCTION Background Prion diseases, also known as the transmissible spongiform encephalopathies (TSEs), are rare fatal neurodegenerative diseases affecting humans and a range of other mammalian species.304 In humans, prion diseases are unique among neurodegenerative disorders, because they occur in idiopathic, genetically-determined and acquired forms (Table 18.1).170,309 As a group, human prion diseases are rare, with an annual mortality rate of 1-1.5 cases per 1 million population.220