transmissible spongiform encephalopathy

(redirected from Prion diseases)
Also found in: Dictionary, Encyclopedia.
Related to Prion diseases: Gerstmann-Straussler-Scheinker syndrome

encephalopathy

 [en-sef″ah-lop´ah-the]
any degenerative disease of the brain.
AIDS encephalopathy HIV encephalopathy.
anoxic encephalopathy hypoxic encephalopathy.
biliary encephalopathy (bilirubin encephalopathy) kernicterus.
bovine spongiform encephalopathy a prion disease of adult cattle in the British Isles with neurologic symptoms. It is transmitted by feed containing protein in the form of meat and bone meal derived from infected animals. The etiologic agent is also the cause of new variant Creutzfeldt-Jakob disease. Called also mad cow disease.
boxer's encephalopathy (boxer's traumatic encephalopathy) a syndrome due to cumulative head blows absorbed in the boxing ring, characterized by slowing of mental function, occasional bouts of confusion, and scattered memory loss. It may progress to the more serious boxer's dementia. See also postconcussional syndrome.
dialysis encephalopathy a degenerative disease of the brain associated with longterm use of hemodialysis, marked by speech disorders and constant myoclonic jerks, progressing to global dementia.
hepatic encephalopathy a condition, usually occurring secondary to advanced liver disease, marked by disturbances of consciousness that may progress to deep coma (hepatic coma), psychiatric changes of varying degree, flapping tremor, and fetor hepaticus.
HIV encephalopathy (HIV-related encephalopathy) a progressive primary encephalopathy caused by infection with human immunodeficiency virus type I, manifested by a variety of cognitive, motor, and behavioral abnormalities. Called also AIDS encephalopathy.
hypernatremic encephalopathy a severe hemorrhagic encephalopathy induced by the hyperosmolarity accompanying hypernatremia and dehydration.
hypertensive encephalopathy a complex of cerebral phenomena such as headache, convulsions, and coma that occur in the course of malignant hypertension.
hypoxic encephalopathy encephalopathy caused by hypoxia from either decreased rate of blood flow or decreased oxygen content of arterial blood; mild cases cause temporary intellectual, visual, and motor disturbances, and severe cases can cause permanent brain damage within five minutes. Called also anoxic encephalopathy.
lead encephalopathy brain disease caused by lead poisoning.
mitochondrial encephalopathy encephalopathy associated with mitochondrial abnormalities, such as melas syndrome and merrf syndrome.
portal-systemic encephalopathy (portasystemic encephalopathy) hepatic encephalopathy.
progressive subcortical encephalopathy Schilder's disease.
subacute spongiform encephalopathy (transmissible spongiform encephalopathy) prion disease.
traumatic encephalopathy
Wernicke's encephalopathy a neurological disorder characterized by confusion, apathy, drowsiness, ataxia of gait, nystagmus, and ophthalmoplegia; it is due to thiamine deficiency, usually from chronic alcohol abuse. It is almost invariably accompanied by or followed by korsakoff's syndrome and frequently accompanied by other nutritional polyneuropathies. See also wernicke-korsakoff syndrome.

transmissible spongiform encephalopathy

n. Abbr. TSE
Any of a group of neurodegenerative diseases of humans and other animals, including scrapie, bovine spongiform encephalopathy, and Creutzfeldt-Jakob disease, that are thought to be caused by prions and can be transmitted by contact from one individual to another.

transmissible spongiform encephalopathy

a group of fatal neurodegenerative diseases that are unique in having either infectious or genetic causes. Examples include Creutzfeldt-Jakob disease and Gerstmann-Sträussler syndrome. A homozygous prion protein genotype predisposes individuals to susceptibility to the diseases.

transmissible spongiform encephalopathy

Encephalopathy marked by rapidly developing dementia or the sudden onset of psychiatric illnesses, often with myoclonus, ataxia, and aphasia. Death may occur within months of onset. These illnesses are believed to be caused by prions. Examples include kuru, mad cow disease (bovine spongiform encephalopathy), and Creutzfeldt-Jakob disease.
See also: encephalopathy
References in periodicals archive ?
They found that their monoclonal antibodies reacted to an intermediate, or "oligomer" state of the amyloid and tau proteins seen in Alzheimer's disease, as well as to prion disease proteins.
Other prion diseases include bovine spongiform encephalopathy (mad cow disease) in cattle and Creutzfeldt-Jakob disease in humans.
Human-to-human transmission of prion diseases is of great concern for public health reasons (11-13).
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.
Humans exposed to contaminated beef products can acquire a human form of BSE, known as vCJD due to its similarities with other human prion diseases.
The ability of [this assay] to detect prions in plasma samples raises the possibility that this assay could be used to improve prion disease diagnosis in humans and animals and to screen the blood supply for prion contamination," wrote Dr.
Covance's neuroscience portfolio offers a wide range of high performing antibodies to key targets in neurodegenerative conditions including Alzheimer's, Parkinson's, and prion diseases.
Krasner (biology, Providence College) gives the background and the real stories behind the development of the Salk polio vaccine and nine other projects to cure, treat or resist such plagues as Chagas Disease, typhus, influenza, smallpox, prion diseases, AIDS, bacterially based ulcers, and Guinea worm disease, as well as the development of the first antibiotics.
Juergen Richt, USDA-ARS Virus and Prion Diseases of Livestock Research Unit, Ames, Iowa; phone (515) 663-7366, e-mail juergen.
It belongs to a group of prion diseases, named for proteins that change into abnormal, infectious clumps that destroy brain cells.
One of the many goals of the Rural Family Health project is to develop a national cohort of farmers and ranchers (men and women) who are willing to participate in a longitudinal study of the impact of prion diseases, such as BSE, on the determinants of health.