Both humans and animals can get them. In people, these conditions impair brain function, causing changes in memory, personality, and behavior; a decline in intellectual function (dementia); and abnormal movements, particularly difficulty with coordinating movements (ataxia).

Prion diseases are also called transmissible spongiform encephalopathies, or TSEs. Prion diseases comprise several conditions.

Infection with this disease leads to death usually within 1 year of onset of illness. This leads to a progressive decline in brain function. This disease is rapidly progressive and always fatal. Creutzfeldt-Jakob disease (CJD) is a rapidly progressive, invariably fatal neurodegenerative disorder believed to be caused by an abnormal isoform of a cellular glycoprotein known as the prion protein. About 300 cases are reported each year in the U.S. Types of p…

Prion diseases are rare. Prion diseases are a rare group of neurodegenerative disorders caused by abnormally folded protein in your brain. A prion is a type of protein that can trigger normal proteins in the brain to fold abnormally. The most common form of prion disease that affects humans is Creutzfeldt-Jakob disease (CJD). The hy… Prion diseases or transmissible spongiform encephalopathies (TSEs) are a family of rare progressive neurodegenerative disorders that affect both humans and animals. Prions are misfolded proteins with the ability to transmit their misfolded shape onto normal variants of the same protein. The word prion derives from "proteinaceous infectious particle". They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response. Most prion diseases are fatal within a few months, though some can last a few years [Pocchiari 2004]. They characterize several fatal and transmissible neurodegenerative diseases in humans and many other animals. Prion diseases can affect both humans and animals and are sometimes spread to humans by infected meat products. Prion diseases affecting animals include scrapie, bovine spongiform encephalopathy (commonly called mad cow disease), and chronic wasting disease of mule deer and elk. Prion disease represents a group of conditions that affect the nervous system in humans and animals. It is not known what causes the normal protein to misfold, but the abnormal three-dimensional structureis suspected of conferring infectious properties, collapsing nearby protein molecules into the same shape. Prion diseases in humans are fairly rare – about 1 to 2 people out of every 1 million people dies of a prion disease each year . Prion diseases can come about in one of three ways: acquired, genetic or sporadic. Prion diseases that affect people include: Creutzfeldt-Jakob disease ;

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