Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0085584 (encephalopathy)
 18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Research into bovine spongiform encephalopathy (BSE) commenced immediately following its discovery in November 1986. Formal epidemiological studies commenced in June 1987 and were part of a large research programme set up mainly at the Central Veterinary Laboratory Weybridge and the Institute for Animal Health, AFRC/MRC Neuropathogenesis Unit in Edinburgh. This programme also covered the clinicopathology of BSE, transmission studies and molecular chemistry. Research results have shown that BSE is a member of the group of diseases known as the sub-acute spongiform encephalopathies caused by unconventional transmissible agents and which includes scrapie of sheep, from which BSE was probably derived, and Creutzfeldt Jakob disease (CJD) of man. The agent causing BSE closely resembles strains of the scrapie agent but is not identical. Spongiform encephalopathy has developed in sheep, goats, pigs, cattle, marmosets and mice but not hamsters following experimental inoculation of brain material from confirmed clinical cases of BSE. BSE agent has been detected by mouse bio-assay in brain from cows from several sources confirmed to have BSE. Infectivity has not been detected in spleen, buffy coat, semen, muscles, placenta and bone marrow (all following parenteral inoculation) nor in milk/mammary gland, spleen, placenta and a variety of lymph nodes following substantial oral exposure. This latter route successfully transmitted disease to mice with brain/cerebrospinal fluid. The introduction of modified rendering systems, which did not employ hydrocarbon solvent extraction, were probably responsible for an increase in exposure of cattle from 1981-1988 sufficient to cause the disease. Experiments are in progress to investigate the effectiveness of different rendering systems used in the European Community and of chemical and physical de-activating procedures for destroying BSE and scrapie infectivity. A clear genetic influence on disease susceptibility has been suggested but is not yet proven. The Agricultural and Food Research Council have set up a large Biology of Spongiform Encephalopathies Research Programme to address some of the more fundamental unknowns of this group of diseases but it is too early to report results. As with scrapie, no epidemiological relationship has been demonstrated between BSE and the human diseases through the monitoring programme that has been established by the Department of Health to detect such an occurrence. BSE research results so far show that controls to protect animal and human health and based initially on scrapie research findings, are sound.(ABSTRACT TRUNCATED AT 400 WORDS)
 ...
PMID:The research programme on transmissible spongiform encephalopathies in Britain with special reference to bovine spongiform encephalopathy. 827 Jan 5

We report a short-term double-blind, crossover study of CoQ10 in 8 patients with mitochondrial encephalomyopathies. Four patients had myoclonus epilepsy with ragged-red fibers syndrome, 3 had mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes syndrome, and 1 had chronic progressive external ophthalmoplegia with myopathy. A trend of effectiveness of CoQ10 in several parameters was noted. Fatigability of daily activities was alleviated. The endurance to muscle exercise was augmented. Global muscle strength scored by Medical Research Council scale was increased. The extent of elevation in serum lactate and pyruvate levels after exercise was decreased. However, only the global MRC index score had a statistical significance (p < 0.05). There were no side effects during therapy. The serum CoQ10 levels were significantly lower in patients than in normal controls before CoQ10 treatment and increased significantly after treatment.
 ...
PMID:Coenzyme Q10 treatment in mitochondrial encephalomyopathies. Short-term double-blind, crossover study. 920 60

Mixed dementia (MD) refers to a combination of definite Alzheimer disease (AD) and vascular encephalopathy, but the distinction between both disorders is controversial. For the diagnosis of MD the clinical/neuroimaging criteria of possible AD plus cerebrovascular disease (CVD) as separate entities are used, but causal relations between vascular brain lesions and dementia are unclear. We proposed the combination of autopsy-proven AD with multiple vascular or ischemic lesions with about 30-50 ml of infarcted/damaged brain tissue. The population-based prevalence of MD is unknown. In retrospective and prospective autopsy studies, it ranges from 2 to 58% with reasonable means of 6-12%. In a consecutive autopsy series of 1500 demented elderly subjects, 830 of which with clinically probable AD, in Vienna, Austria, 41.5 to 52.0% showed "pure" AD, 7% atypical AD, 16-20% AD plus cerebrovascular lesions, and 9% AD plus Lewy body pathology; MD was diagnosed in 4.6 and 2.4%, and "pure" vascular dementia (VaD) in 11 and 2.0%, respectively, while 16.3/6.1% were other dementing disorders, and 1% showed no specific pathology. Like the MRC-CFAS and other studies, this indicates frequent coexistence of AD with multiple cerebrovascular lesions in cognitively impaired patients. In both AD and VaD, vascular lesions frequently involved subcortical regions (basal ganglia, thalamus, hippocampus, and white matter) or were multiple microinfarcts, whereas in MD, large/hemispheral infarcts and multiple microinfarcts were more frequent, suggesting different pathogenic mechanisms. In early/mild AD, critically located small vascular lesions may induce/promote cognitive decline, but in full-blown AD they appear of minor importance. Discussion of the major pathogenic factors inducing AD, VaD and MD suggests synergistic relations between these disorders. However, currently available morphological criteria for AD and VaD are of limited value for the diagnosis of MD and generally accepted and validated histopathological criteria for the diagnosis of VaD and MD are currently not available. Therefore, more distinct and critically evaluated clinico-pathological criteria are warranted.
 ...
PMID:Neuropathological evaluation of mixed dementia. 1732 42