We review here the basic neuropathology of transmissible spongiform encephalopathies (TSE) or prion diseases. The classic hallmark of TSE neuropathology is a combination (in different proportions in different diseases) of spongiform change, astrocytosis, neuronal loss and amyloid plaques. Immunohistochemically, accumulation of the abnormal isoform of prion protein (PrP(sc) or PrP(d)) is regarded as a diagnostic for TSE. We also review the peculiarities of kuru, variant Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker disease.