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prion disease
any of a group of fatal degenerative diseases of the nervous system caused by abnormalities in the metabolism of prion protein. These diseases are unique in that they may be transmitted genetically as an autosomal dominant trait, or by infection with
abnormal forms of the protein (prions). Inherited forms result from mutations in the gene that codes for prion protein; such mutations may also occur sporadically.
Hereditary forms include some forms of Creutzfeldt-Jakob disease, Gerstmann-Sträussler syndrome, and fatal familial insomnia. Infectious forms of the disease result from ingestion of infected tissue or the introduction of infected tissue into the
body (kuru and some forms of Creutzfeldt-Jakob disease). The latter has occasionally occurred during surgical procedures; it has also occurred as the result of injection of human
growth hormone prepared from infected pituitary glands. Prion diseases also occur in animals. Called also transmissible neurodegenerative disease and subacute spongiform or transmissible spongiform encephalopathy.
Copyright 2007. An Elsevier publication. All rights reserved.
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