Discrimination of Classical and Atypical BSE by a Distinct Immunohistochemical PrPSc Profile

Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease in cattle belonging to the group of transmissible spongiform encephalopathies. Hallmark of the disease is the accumulation of the pathological prion protein (PrPSc) in the brain. Classical BSE (C-type) and two atypical BSE forms (L- and H-type) are known, and can be discriminated by biochemical characteristics. The data presented here underline that immunohistochemistry can also be used to identify type-specific PrPSc profiles which can be used for discriminatory purposes. For this brain samples from 21 cattle, intracerebrally inoculated with C-, H-, and L-type BSE, were used as well as three orally C-type BSE infected animals. Using six brain regions distinct lesion (H&E staining) and PrPSc profiles were determined. While the neuroanatomical distribution of lesions and the PrPSc accumulation were highly consistent between the groups, the topographic and cellular PrPSc profile revealed characteristic pattern for the different BSE types.