Atypical and classic bovine spongiform encephalopathy

This chapter describes the prion diseases of cattle, or bovine transmissible spongiform encephalopathies (BoTSEs). “Classic” bovine spongiform encephalopathy (C-BSE), the major prion protein disorder of Bovidae, was first described in 1986. We also describe the spatiotemporal correlation of C-BSE to a novel form of human prion disease, variant Creutzfeldt–Jakob disease (vCJD), which led to the classification of BSE as a zoonotic disease (and the “cause” of vCJD) in 1996. From isolated cases first identified retrospectively in May 1985, a major bovine spongiform encephalopathy (BSE) epidemic peaked within the British Isles in 1991, and has so far led to over 195,000 confirmed cases in cattle, and several thousand more cases within Europe and a few elsewhere. By 2017, the disease had been essentially eradicated below the level of surveillance detection in Europe, although sporadic cases are still predicted to show up for several years to come. By 2004, other forms of BSE, H-type and L-type, had been recognized and, in the absence of information to the contrary, are also included in the classification of BSE as a zoonotic prion disease of bovine origin. This chapter aims to cover the clinical, epidemiologic, neuropathologic, biochemical, biomarker, and pathogenetic aspects of the bovine prion disease and gives a brief, but important, description of the surveillance of BSE and other animal TSEs in Europe.