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Query: UMLS:C0085584 (encephalopathy)
 18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Before industrial production of thiamine becam possible, among many beriberi patients some showed symptoms of encephalopathy, the cerebral form of the disease. In this animal experiment, thiamine-deficient rats showed failure or blocking of the operant behavior in the maze box, pole climbing box and shuttle box, indicating orientation disturbance and defective memory. This Wernicke's syndrome-like sign dramatically disappeared by treatment with thiamine. Potentiated narcosis with thiopental or alcohol induced in thiamine-deficient rats and mice was readily reversible by thiamine administration. These phenomena are associated with thiamine content of the brain and are found long before histopathological changes in the brain of deficient animals. It is easily surmised that thiamine deficiency in the brain may block the brain metabolism and subsequently cause changes in any chemical substances in the brain, refracting on biophysical phenomenon, such as EEG. However, in the present study, generally speaking, no meaningful results concerning these points were obtained.
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PMID:Animal experiments on thiamine avitaminosis and cerebral function. 103 13

A girl of 10-5/12 years is described, who had diabetes mellitus from the age of 5 years on and who developed bilateral ptosis, pigment degeneration of the retina and bilateral impairment of hearing at the age of nine years. A few weeks before death she suffered from an acute gastrointestinal infection which was successfully treated by a hydroxyquinoline derivative. In the days following a severe encephalopathy and signs of cardiac involvement appeared. A month later the girl died of bulbar paralysis and acute heart failure. Histology showed remnants of a granulomatous inflammation in the heart, the kidneys, the pancreas and the skeletal muscles. Furthermore there was a widespread spongiosis in the white substance of the brain, with large astrocytes, and partly also in the basal ganglia, the brain stem and the cerebellum. Foci of sudanophilic tissue necrosis resembling Wernicke's Encephalopathy were found in the medulla oblongata and the spinal cord. The peripheral nerves appeared partially demyelinated and showed axonal lesions. This case is classified as a Juvenile Type of so-called Canavan's Disease. It shows some resemblence to the "Progressive Chronic Ophthalmoplegia with Spongiform Encephalopathy described by Daroff, Kearn and Sayre. The possible neurotoxical effects of the hydroxyquinoline therapy are discussed.
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PMID:[Juvenile spongy dystrophy of CNS with necrosis of the medulla. A. complication of hydroxyquinoline therapy (author's transl)]. 124 13

Two children with osteosarcoma are presented in whom Wernicke encephalopathy with vomiting occurred during the chemotherapy. One of the children died with symptoms of toxic cardiomyopathy. Autopsy revealed Wernicke encephalopathy. The other child had similar symptoms (ocular signs, ataxia, somnolence). Parenteral thiamine had been given and after this therapy the child recovered from the encephalopathy. The authors emphasize the importance of the recognition of this neurological disorder occurring rarely in childhood: it can be cured with parenteral thiamine. Without thiamine treatment this condition is lethal.
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PMID:[Wernicke encephalopathy in childhood osteosarcoma]. 140 86

The effects of alcohol on the central nervous system can be subdivided into three main categories: the effects of acute intoxication (drunkenness, acute encephalopathy, stroke), the effects of tolerance and ethanol withdrawal (delirium tremens, seizures) and the delayed manifestations of chronic alcohol consumption (cerebellar degeneration, Wernicke's encephalopathy, dementia).
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PMID:[Main involvement of the central nervous system in alcoholism]. 141 Sep 80

In recent years there are a considerable increase in alcohol consumption in Taiwan, which may have been accompanied by increased incidence of alcohol-related physical disease. This study was designed for an understanding of neurological problems in chronic alcoholic patients. One hundred and five cases of chronic alcoholics with neurological problems were collected. All had taken more than 100 g alcohol daily for more than 8 years. They were all males, with a mean age of 47.0 +/- 1.3 years, mean daily alcohol consumption of 185.1 +/- 9.0 g (mean +/- S.E.). These chronic alcoholic patients showed various neurological problems. Patients showing typical clinical features of alcoholic neurological disease are now rather rare. Most of the patients had manifestations of more than one problems: polyneuropathy (74.3%), alcoholic tremor (37.1%), hallucinosis (30.5%), myopathy (26.7%), head injury (24.8%), withdrawal seizures (18.1%), Wernicke encephalopathy (15.2%), paranoia (13.3%), and stroke (15.2%). Furthermore, we divided all the patients into 5 categories, they were: encephalopathy, 59 cases (56.2%); stroke, 16 cases (15.2%); cerebellar degeneration, 12 cases (11.4%); neuropathy, 78 case (74.3%); and myopathy, 28 cases (16.7%). The daily alcohol consumption and duration of daily drinking were different significantly (p less than 0.05) among five different syndrome categories.
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PMID:Neurological problems in chronic alcoholics. 165 33

Alterations of excitatory amino acids in brain may be of pathophysiological significance in thiamine-deficiency encephalopathy. The present study was undertaken to evaluate the effects of thiamine deficiency induced by the central thiamine antagonist, pyrithiamine, on the glutamate content of glutamatergic nerve terminals. Electrically-stimulated, Ca(2+)-dependent release of glutamate from hippocampal slices obtained from symptomatic pyrithiamine-treated rats was significantly decreased compared to pair-fed controls. Possible explanations for the decreased "neurotransmitter pool" of glutamate in thiamine-deficient rat brain include decreased synthesis of glutamate as a result of decreased activities of the thiamine-dependent enzyme alpha-ketoglutarate dehydrogenase or increased release of glutamate per se. There is evidence to suggest that the latter mechanism with ensuing excitotoxic neuronal damage could be involved in the pathogenesis of selective neuronal death in thiamine deficiency. Similar mechanisms could be implicated in Wernicke's encephalopathy in humans.
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PMID:Pyrithiamine-induced thiamine deficiency results in decreased Ca(2+)-dependent release of glutamate from rat hippocampal slices. 168 63

Clinical and postmortem findings of a case that had combined alcoholic pellagra encephalopathy and Wernicke disease are described. This 51-year-old malnourished and chronic alcoholic man presented with progressive mental deterioration, pellagra dermatitis, hypertonus of the neck and other musculatures, myoclonic jerks with bizarre involuntary movements, in addition to total external ophthalmoplegia and gait disturbance. After administration of multivitamins, including thiamine and nicotinamide, these neurologic abnormalities were dramatically improved in a few days. However, the patient died thereafter because of sepsis associated with pneumonia. Postmortem examination revealed marked abnormalities in CNS, characterized by diffuse atrophy of gray matter and widespread neuronal degeneration and characteristic central chromatolysis in pontine nuclei, dentate nuclei, cranial nerve nuclei in the brain stem, Betz cells of the cerebral cortex, and Clarke's column and anterior horn cells of the spinal cord. There were also atrophy and gliosis of the mammillary bodies, degeneration and vascular proliferation of periaqueductal gray matter, and massive gliosis around the third ventricle. These neuropathological changes were compatible with symptoms of both alcoholic pellagra encephalopathy and Wernicke's disease, but they were also strongly suspected on clinical grounds.
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PMID:Alcoholic pellagra encephalopathy combined with Wernicke disease. 188 55

Using a monoclonal antibody to an inner mitochondrial membrane antigen and light microscopic immunohistochemistry, we investigated the distribution of increased immunostaining (mitochondrial anomalies, MA) on paraffin sections from 21 brains with infantile spongy dystrophy (Leigh's disease, 8; Canavan's disease, 4; Alpers' syndrome, 2; mixed spongy dystrophy, 7). Compared with an age-matched control group, MA were present in all cases of Leigh's disease (leptomeningeal and intracerebral endothelial and vascular smooth muscle cells, choroid plexus epithelia, ependymal cells, astrocytes or some neurons), in 2 cases of Canavan's disease and the Alpers' syndrome cases (astrocytes and occasionally some neurons). The MA were restricted to spongy areas in Canavan's disease and Alpers' syndrome, whereas they were distributed throughout the brain in Leigh's disease. In mixed spongy dystrophies the Leigh histology was associated with MA, but not the Canavan histology. Brains with Wernicke's encephalopathy (3 cases), adult infarction (3), and multicystic encephalopathy (5) showed no MA, but one with methylmalonaciduria did. Our results substantiate the classification of Leigh's disease as primary mitochondrial encephalopathy.
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PMID:Intracerebral distribution of mitochondrial abnormalities in 21 cases of infantile spongy dystrophy. 233 65

Thiamine deficiency (Wernicke-Korsakoff's disease) may not be the only mechanism whereby chronic alcohol abuse affects the brain and not all alcohol-related changes may be evident morphologically. The purpose of this study was to determine if alcohol abuse affects muscarinic cholinergic and benzodiazepine receptors in the hippocampus of histologically normal brains obtained at autopsy in a general hospital population. Because patients were excluded who had significant brain atrophy and/or dementia severe enough to require institutionalization, the reported findings are presumed to be early changes in the development of an alcohol encephalopathy. In addition, patients were excluded from this study if they had clinical brain diseases (including Wernicke's disease), died in coma, had liver disease, or received medications that could potentially alter receptor binding. The reported changes in receptor binding were therefore presumed to be related to alcohol abuse per se and not an alcohol-associated condition. We found that muscarinic cholinergic synaptic receptor density determined with 3[H] quinuclidinyl benzilate was decreased by 30% in homogenates of the hippocampus of 25 alcohol abusers compared with 25 matched nonalcoholic controls. Similarly, densities of benzodiazepine receptors determined with 3[H] flunitrazepam were also decreased by approximately 30% in alcohol abusers. The affinities of both receptor types were not affected by alcohol abuse. Age and death-autopsy time interval had no significant effects on either wet tissue protein concentrations, yields of protein after centrifugation, or receptor binding. The contributions of age and time interval were each less than 2% of the total variance of protein concentrations and receptor binding.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Loss of muscarinic and benzodiazepine neuroreceptors from hippocampus of alcohol abusers. 254 36

It is currently controversial whether all the brain damage in alcohol abusers in the result of thiamine deficiency (Wernicke-Korsakoff's disease) or whether, in addition, alcohol abuse may affect the brain by other mechanisms as well. The purpose of this study was to determine if alcohol abuse affects muscarinic cholinergic and benzodiazepine receptors in histologically normal brains obtained at autopsy in a general hospital population. Patients were excluded from this study if they had clinical brain diseases (including Wernicke's disease), died in coma, or had liver disease, significant brain atrophy, or dementia severe enough to require institutionalization. We found that muscarinic cholinergic synaptic receptor density determined with [3H] quinuclidinly benzilate was decreased by 40% in homogenates of the tempeoral cortex of 26 alcohol abusers compared with 26 matched nonalcoholic controls. The affinities of the muscarinic receptors were not significantly different between the two groups. In contrast, receptor densities and affinities of benzodiazepine receptors determined with [3H]flunitrazepam were not significantly different in the two groups. Age and death-autopsy time interval had no significant effects on either wet tissue protein concentrations, yields of protein after centrifugation, or receptor binding. The contributions of age and time interval were each less than 2% of the total variance of protein concentrations and receptor binding. When patients were excluded or included who had received cholinergic, anticholinergic, or benzodiazepine medications before death, no significant effects on the final results were observed. Pneumonia, known to be associated with acute hypoxia, and chronic obstructive pulmonary disease, known to be associated with chronic hypoxia, where approximately equally distributed between the two groups and had no significant effects on the results reported here. The loss of muscarinic and the sparing of benzodiazepine receptors occurs in the temporal cortex of histologically normal brains in the absence of significant atrophy and of gross dementia. This means that these changes are early in the development of an alcohol encephalopathy. We have previously reported a decrease in both muscarinic and benzodiazepine receptor binding in the frontal cortex and a decreasing muscarinic but not benzodiazepine receptors in the temporal cortex of alcohol abusers. Taken together, these findings suggest that alcohol neurotoxicity does not simply result in a random loss of neurons and or their associated synapses with their receptors. Instead, different types of receptors, depending upon their location in different brain regions, are specifically affected or spared. This suggests the involvement of region- and receptor-specific mechanisms in chronic alcohol toxicity.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Loss of muscarinic cholinergic receptors from the temporal cortex of alcohol abusers. 254 45


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