transmissible spongiform encephalopathy (redirected from Transmissible spongiform encephalopathies)

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-Stäussler syndrome. A homozygous prion protein genotype predisposes individuals to susceptibility to the diseases.

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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

1. boxer's encephalopathy.

2. postconcussional syndrome.

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.