prion diseases


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Related to prion diseases: Gerstmann-Straussler-Scheinker syndrome

prion diseases

[′Prī‚än di‚zēz·əz]
(medicine)
A group of invariably fatal disorders affecting humans and animals that are clinically characterized by neurological and behavioral degeneration caused by the cerebral accumulation of an abnormal prion protein which is resistant to proteolytic enzymes and, in contrast to other infectious agents, does not require nucleic acid for replication. The diseases are transmissible either genetically (for example, Creutzfeldt-Jakob disease) or via infection (new variant Creutzfeldt-Jakob disease and mad cow disease) or can occur spontaneously (classical or sporadic Creutzfeldt-Jakob disease). Also known as spongiform encephalopathies.
References in periodicals archive ?
Prion diseases originate when normally harmless prion protein molecules become abnormal and gather in clusters and filaments in the human body and brain.
Prion diseases cause brain damage that worsens rapidly over time and are invariably fatal.
Polymorphism at codon 129 of the human PrP gene (PRNP), where methionine (Met) or valine (Val) can be encoded, strongly affects susceptibility to human prion diseases (16-20).
People who eat people with a type of prion disease, a category of illness that also includes Creutzfeldt-Jakob disease in humans and Mad Cow Disease in animals, tend to have brains that look like sponges and neurological damage that takes away control over the body.
A scientist friend came by with a flash drive loaded with research papers about prion diseases.
The Vesiculovirus Piry infection generates human disease characterized by rapid onset, high fever, headache, chills, photophobia, myalgia, dizziness, and weakness [11] and, in adult mice, a nonlethal CNS infection and injury to the limbic system including the hippocampus [12], a target region of the degenerative process induced by prion disease in mice [13].
Molecular biology and pathology of prion strains in sporadic human prion diseases.
Even so, as a physician I don't fret much about epidemics of prion diseases turning the population into brain-eating zombies.
An easy-to-use diagnostic test would let doctors clearly differentiate prion diseases from other brain maladies.
There is a chapter on bovine spongiform encephalopathy (BSE) and Scrapie in sheep as well as a chapter on chronic wasting disease and various other animal prion diseases.
Unlike prion diseases, which can be infectious, Alzheimer's, Parkinson's, and other neurodegenerative diseases can not be passed from person to person under normal circumstances.