- Any disorder of the brain. SYN: cerebropathia, cerebropathy, encephalopathia, encephalosis. [encephalo- + G. pathos, suffering]- bovine spongiform e. (BSE) a disease of cattle first reported in 1986 in Great Britain; characterized clinically by apprehensive behavior, hyperesthesia, and ataxia, and histologically by spongiform changes in the gray matter of the brain stem; caused by a prion, like spongiform encephalopathies of other animals ( e.g., scrapie) and human beings (Creutzfeldt-Jakob disease). See Creutzfeldt-Jakob disease. SYN: mad cow disease.In the middle 1990s, an unusual number of cases of Creutzfeldt-Jakob disease (CJD) were reported in persons under 30 years of age in Great Britain. These patients displayed typical clinical features but not the EEG changes characteristic of CJD, and autopsy specimens showed unusual amyloid plaques resembling those of kuru but not previously observed in CJD. These cases of variant Creutzfeldt-Jakob disease (V-CJD) were speculatively associated with an epizootic of bovine spongiform e. (mad cow disease) that killed more than 150,000 cattle in Britain between 1986 and 1996. Although the link between BSE and V-CJD cannot be confirmed on the basis of existing data, the mere possibility of such a link has already led to the development of recommendations to help reduce or prevent the occurrence of BSE in cattle worldwide. There is no evidence from U.S. surveillance activities or from scientific studies to indicate that BSE exists in the U.S. Since 1990, laboratory testing of brain specimens from cattle with CNS signs has shown no evidence of BSE. Since July 1989 the importation of cattle and cattle products from the U.K. has been banned by the U.S. Department of Agriculture. According to mortality statistics, the annual incidence of CJD in the U.S. remained stable at approximately 1 case per million persons between 1979 and 1994. WHO consultants have condemned the practice of feeding ruminant-derived meat-and-bone meal to cattle and urged the adoption of measures to ensure that no part of any animal that shows signs of a spongiform e. enters any human or animal food chain. Milk, dairy products, gelatin, and lard are considered safe.- demyelinating e. extensive idiopathic loss of myelin sheaths in the brain, as occurs in leukodystrophy.- hypertensive e. a metabolic e. caused by diffuse cerebral edema; follows an abrupt elevation of blood pressure in a long-term hypertensive patient.- hypoxic-hypercarbic e. SYN: hypoventilation coma.- hypoxic ischemic e. permanent brain injury due to a lack of oxygen or adequate blood flow to the brain.- lead e. a metabolic e., caused by the ingestion of lead compounds and seen particularly in early childhood; it is characterized pathologically by extensive cerebral edema, status spongiosus, neurocytolysis, and some reactive inflammation; clinical manifestations include convulsions, delirium, and hallucinations. SEE ALSO: lead poisoning. SYN: lead encephalitis, saturnine e..- metabolic e. coma or its precursors resulting from a diffuse abnormality of cerebral neuronal or glial cell metabolism. Primary metabolic e. is due to any of the degenerative cerebral disorders that culminate in coma; secondary metabolic e. results when brain metabolism is disturbed by extracerebral disorders causing intoxication, electrolyte imbalances, or nutritional deficiencies, e.g., hepatic or renal disease or exogenous poisons.- necrotizing e. SYN: Leigh disease.- portal-systemic e. an e. associated with cirrhosis of the liver, attributed to the passage of toxic nitrogenous substances from the portal to the systemic circulation; cerebral manifestations may include coma. SYN: hepatic e..- recurrent e. [MIM*130950] a progressive form of e. occurring in young members of the same family; characterized by headache, vertigo, truncal ataxia, drowsiness and stupor, speech impairments, choreic-athetoid movements, and sometimes convulsions; probably autosomal dominant inheritance.- severe postanoxic e. SYN: delayed coma after hypoxia.- subacute spongiform e. a form of spongiform e. that is associated with a “slow virus,” which to date has not been adequately described, is transmissible, and has a rapidly progressive, fatal course; e.g., Creutzfeldt-Jakob disease, kuru, Gerstmann-Sträussler syndrome, scrapie. See prion.- traumatic progressive e. chronic progressive brain damage resulting from multiple brain injuries, e.g., dementia pugilistica.
* * *en·ceph·a·lop·a·thy in-.sef-ə-'läp-ə-thē n, pl -thies a disease of the brain esp one involving alterations of brain structureen·ceph·a·lo·path·ic -lə-'path-ik adj
* * *n.any of various diseases that affect the functioning of the brain. See hepatic encephalopathy, spongiform encephalopathy, Wernicke's encephalopathy.
* * *en·ceph·a·lop·a·thy (en-sef″ə-lopґə-the) [encephalo- + -pathy] any degenerative disease of the brain.
Medical dictionary. 2011.