Classical and Atypical Bovine Spongiform Encephalopathy: Epidemiology, Pathogenesis and Diagnosis

Abstract

Classical bovine spongiform encephalopathy (C-BSE) is a fatal neurodegenerative disease of cattle, detected in the United Kingdom and many other countries since the 1980s. The origin of C-BSE is uncertain, but epidemiological studies suggest that the source of this disease was cattle feed prepared from prion-infected animal tissues. To date, cattle populations have been monitored through passive and active surveillance programs. From 2004, two different forms of BSE termed as L-BSE, also known as bovine amyloidotic spongiform encephalopathy (BASE), and H-BSE have been discovered in Italy and France. All these atypical cases have been detected in animals over 8 years of age. To date, there is no comprehensive information about the origin of the atypical BSEs (sporadic vs. acquired). Moreover, there are only very limited data available, concerning the pathogenesis of both atypical forms, as compared to C-BSE. This chapter provides a well-organized overview of what is known about classical and atypical BSE. It will review information on the main epidemiological features, pathogenesis, and the criteria for the routine diagnosis based on rapid tests, histological, immunohistochemical, and Western blot examinations.