transmissible spongiform encephalopathies

Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a group of progressive conditions that affect the brain (encephalopathies) and nervous system of many animals, including humans. According to the most widespread hypothesis, they are transmitted by prions, though some other data suggest an involvement of a Spiroplasma infection. Mental and physical abilities deteriorate and myriad tiny holes appear in the cortex causing it to appear like a sponge (hence spongiform) when brain tissue obtained at autopsy is examined under a microscope. The disorders cause impairment of brain function, including memory changes, personality changes and problems with movement that worsen over time. Prion diseases of humans include classic Creutzfeldt–Jakob disease, new variant Creutzfeldt–Jakob disease (nvCJD, a human disorder related to Bovine spongiform encephalopathy), Gerstmann–Sträussler–Scheinker syndrome, fatal familial insomnia,  kuru, and the recently discovered Variably protease-sensitive prionopathy. These conditions form a spectrum of diseases with overlapping signs and symptoms.