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The page below is a sample from the LabCE course Overview of Prion Diseases. Access the complete course and earn ASCLS P.A.C.E.-approved continuing education credits by subscribing online.

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How do prions kill?

It has been noted rather appropriately that “Prion diseases . . . have stumped scientists for decades with a complex ‘whodunnit’ complete with many suspects and a missing murder weapon.” Basically, the brain is killed by clogging with clumps of prion proteins, which have been changed into a rare, abnormally folded state called prions. The classical histopathological features of prion diseases include the formation of “holes” or vacuoles in brain tissue that result in a spongy architecture (hence the name spongiform, resembling a sponge in appearance, encephalopathy, involving alterations of brain structure). Also occurring are a loss of neurons and an increase in astrocytes. For these reasons, when the characteristic signs and symptoms of prion diseases appear, the condition is always fatal.