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

Low plasma levels of branched-chain amino acids, leucine, isoleucine, and valine are postulated to play an etiologic role in hepatic encephalopathy. Supplementation is advocated to reverse encephalopathy and improve nutritional status and survival. We measured in vivo leucine metabolism in normal individuals (n = 5) and in two groups of patients with cirrhosis (n = 8) with a primed continuous infusion of L-[15N, 1-13C] leucine to quantitate the following parameters of leucine metabolism: nitrogen and carbon fluxes, oxidation, contribution to protein synthesis, breakdown of endogenous protein to leucine, deamination and reamination to/from ketoisocaproate. Studies were performed in the fasting and fed states with a conventional enteral diet (Propac) and a branched chain-enriched diet (one third Propac plus two thirds Hepatic-Aid). In vivo leucine metabolism was similar in the fasting and fed states in normal individuals in patients with cirrhosis and with both diets when studied at a protein intake of 0.6 gm/kg ideal body weight/day. When fed these diets, oxidation increased (p less than 0.05) and breakdown decreased (p less than 0.05). The Hepatic-Aid diet increased (p less than 0.05) nitrogen and carbon fluxes significantly more than did the standard diet. Four additional patients with cirrhosis on a diet with more protein were studied (0.75 gm/kg ideal body weight/day). Carbon and nitrogen fluxes, oxidation, synthesis, and deamination were increased (p less than 0.05) when patients with cirrhosis were fed the Propac diet compared with those who fasted. The Hepatic-Aid diet further increased (p less than 0.05) all parameters except synthesis and did not decrease protein breakdown. These data show that patients with cirrhosis metabolize leucine in vivo in a manner identical to that of normal subjects and that leucine-enriched formulas increase oxidation to CO2 without improving protein synthesis.
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PMID:In vivo measurement of leucine metabolism with stable isotopes in normal subjects and in those with cirrhosis fed conventional and branched-chain amino acid-enriched diets. 403 63

Carbon disulfide toxicity is well characterized. The principal target organ is the nervous system, although cardiovascular, reproductive, ophthalmologic, and other effects are also recognized. The neurotoxicity manifests in three ways: encephalopathy, peripheral and cranial nerve dysfunction, and movement abnormalities. This report describes a case of olivopontocerebellar atrophy, a form of multiple system atrophy, developing in an adult after over 30 years of occupational exposure to carbon disulfide. The patient presented with the insidious onset of balance problems, impotence, and irritability, without tremor, cogwheel rigidity, bradykinesia, or changes in facial expression. Over the next few years severe ataxia developed, and the clinical diagnosis was confirmed with computed tomography and magnetic resonance imaging scans. The patient experienced multiple medical complications and died approximately 9 years after diagnosis. This case is consistent with a large body of clinical and experimental literature, much of it 50 years old, showing that carbon disulfide can cause movement disorders. It also serves as a reminder that movement disorders, ranging from parkinsonism to dystonia, are associated with a variety of toxic exposures such as manganese, carbon monoxide, 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine, and medications.
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PMID:Multiple system atrophy following chronic carbon disulfide exposure. 1070 37

Carbon disulfide is a poison of particularly neotropic properties. In order to diagnose chronic occupational intoxication with carbon disulfide, a very careful examination of the central and peripheral nervous systems in required. The presence of subjective disorders only does not as yet provide grounds for diagnosing chronic intoxication. Organic changes like chronic encephalopathy or polyneuropathy after excluding the so called 'idiopathic' neurological diseases, may serve as a basis for certifying occupational disease.
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PMID:[Diagnostic neurologic procedures and certification in persons chronically exposed to carbon disulfide in light of personal cases]. 1105 7

Carbon disulfide (CS2) intoxication may induce peripheral neuropathy, encephalopathy, and cardiovascular diseases. In our studies, abnormalities of the peripheral nerves including clinical symptoms and electrophysiological findings were still present 3 years after cessation of CS2 exposure. The data indicate that CS2 neuropathy may persist for a period of time. The involvement of central nervous system may continue even longer. Brain magnetic resonance images usually show multiple high signal intensities in the basal ganglia and subcortical white matter suggesting a vascular event particularly in the small vessels. In addition, a patient with diffuse demyelination in the cerebral hemispheres also showed a diffuse decrease of regional cerebral blood flow indicating a microangiopathy. Therefore, CS2 exposure should be considered as a risk factor for strokes and one of the causes for diffuse leucoencephalopathy. Because CS2 may induce parkinsonian features, a differential diagnosis between CS2 parkinsonism and idiopathic parkinsonism is important. In our study, dopamine transporter with 99mTc-TRODAT-1 brain single photon emission computed tomography showed a normal uptake in the corpus striatum. The data suggest a normal presynaptic dopaminergic pathway function and provide useful information in differentiation. The involvement of cardiovascular systems may be due to thrombotic effects rather than atherogenic effects. In addition, absorption of CS2 through skin is also significant particularly in workers with skin lesions.
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PMID:Carbon disulfide neurotoxicity: Taiwan experience. 1531 94