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Spongiform encephalopathies

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spongiform encephalopathies

A group of diseases characterised by long incubation and fatal progressive course with characteristic spongiform degeneration of grey matter of the cortex. The two main human diseases are kuru and Creutzfeldt Jakob disease. Diseases such as scrapie, mink encephalopathy and bovine spongiform encephalopathy (bSE) are considered to be similar. Controversy still surround the causative agent, the two main theories being slow viruses or prions.

See: gerstmann Straussler Scheinker syndrome.