A group of diseases that includes bovine citrullinemia, scrapie, bovine spongiform encephalopathy, the internationally infamous disease known as ‘mad cow disease’, Creutzfeld–Jakob disease, Gerstmann–Straussler disease (the last two are diseases of humans), and neuronal vacuolar degeneration of Angora goats and focal spongiform encephalopathy of dogs; in all of these diseases spongiform vacuolation of neurons and their processes is the characteristic lesion.