Prion disease

Prion disease represents a group of conditions that affect the nervous system in humans and animals. 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). The signs and symptoms of prion disease typically begin in adulthood and worsen with time, leading to death within a few months to several years.

Other names

Inherited human transmissible spongiform encephalopathies; Prion protein diseases; Prion-associated disorders; Prion-induced disorders; Transmissible dementias; Transmissible spongiform encephalopathies; TSEs

Inheritance pattern

Autosomal dominant

Related genes

PRNP

Source: MedlinePlus Genetics, U.S. National Library of Medicine.