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

Mannosidosis is a rare inborn error of metabolism characterized by deficiency of the lysosomal enzyme alpha-mannosidase and widespread storage of complex carbohydrate, which is enriched in mannose. Two affected unrelated males, aged 6 and 26 years, are reported. Both had a nonprogressive encephalopathy with moderately severe mental retardation. The older patient showed several unique features, including massive gingival hyperplasia associated with histiocytes containing large amounts of a material with the staining characteristics of glycoprotein. The best determinant of mannose storage proved to be the ratio of mannose to other carbohydrates in urinary polysaccharides. The enzyme deficiency in this disease is most convincingly demonstrated at pH values below 4.0. The ability of zinc to activate the mutant enzyme in vitro offers a possible mode of therapy for this disease. Retarded individuals with a Hurler-like appearance and gum hyperplasia of unknown cause should be screened for alpha-mannosidase deficiency.
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PMID:Mannosidosis. New clinical presentation, enzyme studied, and carbohydrate analysis. 1 32

The authors present a 60-year-old patient undergoing periodic hemodialysis who, 3 years after beginning the treatment, developed a clinical picture consisting of disturbances of language, motor dispraxia, loss of memory and concentration, irritability, great change of personality, myoclonias and asterixis. This led progressively to a total loss of motor coordination, including speech. He died 5 months later in a state of dementia, psychosis and incontinence of sphincters. The symptomatology increased after hemodialysis sessions. The normal analytical studies carried out in these cases (electrocardiogram, electromyography, complete roentgenologic study) and also Zn, Cu, and ceruloplasmin measurements were normal. The electroencephalogram showed only a slow tracing with delta waves. Various etiopathogenic possibilities are commented on, as for example alterations in the dialysis water, the use of detergents in cleaning the artificial kidney, a syndrome of imbalance, a decrease in the body potassium and poisoning caused by certain metals such as tin, zinc and aluminium or by drugs which contain benzodiazepine derivatives. The authors conclude that the picture corresponds to a metabolic encephalopathy.
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PMID:[Dementia and hemodialysis (author's transl)]. 43 Nov 64

The observation in 14 dialysis patients of an encephalopathy associating myoclonia, dysarthria, generalised seizures in some cases, worsening over a few months, led to an aetiological inquiry based upon comparative study of patients with or without encephalopathy treated in the same centre or at home, and controls. Higher levels of aluminium were found in the frontal cortex grey matter of encephalopathy patients as compared to the control group. The same applies to manganese in the white matter. Copper, zinc and iron contents were not different. Aluminium levels in blood, dialysis bath and tap water supply were higher in center dialysis than in home dialysis. Blood aluminium levels at the end of hemodialysis were correlated with bath aluminium levels. The ingestion of alumine gels was not greater in the encephalopathy patients than in other hemodialysis patients; its estimation, in each case, was not related to the blood aluminium levels at the begining of hemodialysis. These finding indicate the need of a routine measure of metal content - mainly aluminium and manganese - in tap water used for dialysis, in order to treat this water if necessary.
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PMID:[Progressive myoclonic encephalopathy in dialysis patients. The role of the water used for haemodialysis (author's transl)]. 65 14

The paper discussed a case of acute zinc intoxication in a 48-year old welder, after four days of cutting zinc-plated pipes with an oxy-acetylene torch, in poorly ventilated places. The zinc fever has been diagnosed on the basis of the symptoms and confirmed by laboratory findings: high zinc blood and erythrocyte concentration and increased urinary excretion of zinc. One year the intoxication the manifestations of the psycho-organic syndrome with predilection to pseudoneurotic reactions were still present. The non-standard factor in this case is the very short time of exposure to zinc oxide and the occurrence of chronic encephalopathy is also singular.
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PMID:[An atypical case of acute zinc poisoning]. 145 50

In Alzheimer's dementia (AD) the Primum Movens is Amyloid (AM) production on precapillaries: Dyshoric Angiopathy, and capillaries: Senile Plaques (SP) producing Blood-Brain-Barrier (BBB) disturbances, entry in the brain of toxic metals which displace the zinc. Cerebral AM alone may be asymptomatic. Clinical symptoms (Amnesia, Instrumental Disorders) appear when AM induces Neighbouring neuritic alterations: Paired Hellical Filaments (PHF) and Distant neuronal body lesions: Neurofibrillary Tangles (NFT). The AM is coded by a locus on the chromosome 21 and a duplication of this locus should be the etiology of cerebral AM in AD. In AD cerebral zinc decreases particularly in the hippocampus. The zinc-enzyme Superoxyde-Dismutase (SOD) is coded by a locus also on the chromosome 21 near AM and the plasma level of SOD is high in AD. Zinc deposits observed in capillary AM-SP, result probably from the excess of plasmatic SOD. Other metals: Iron, aluminium are also observed in the AM-SP and their excess in the brain may be related to the decrease of zinc by metal to metal displacement. The decrease of functional zinc in the brain may interfere in the pathogenesis of PHF-NFT by metalotoxicity, neighbouring and distant to AM. Without AM, NFT are produced also by metalotoxicity and therefore brain zinc displacement. a) by lead: Encephalopathia saturnica b) by many metals: Guam Encephalopathy c) by aluminium d) by BBB disturbances leading probably to an abnormal entry of metals in the brain (Dementia Pugilistica, viral encephalitides). NFT may be produced by the deficiency of the following zinc enzymes: 1. Those of DNA metabolism, indicating abnormal DNA and therefore abnormal protein synthesis: PHF-NFT. 2. Those of neuronal detoxication: SOD, Carbonic Anhydrase, Lactate Dehydrogenase leading to neuronal toxicity particularly in the hippocampus normally rich in SOD. 3. Of Glutamate (GLU) Dehydrogenase (GDH) resulting in an excitotoxic increase of GLU. 4. Those of the metabolism of neurotransmitters (NT): neuropeptides, Histamine, GABA, Acetylcholine. Therapeutic proposition: a zinc complex crossing the BBB should be useful a) to prevent that the AM produces PHF-NFT by Neighbouring and Distant metalotoxicity and DNA changes; b) to regularise zinc-enzymes of neuronal detoxification and of neurotransmitters metabolisms. Preliminary trials by zinc Aspartate give yet promising results.
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PMID:[Alzheimer's dementia and zinc]. 170 60

Neurofibrillary tangles (NFT) in human encephalopathies of various etiologies may result from a common pathogenetic mechanism: a functional zinc decrease leading to a deficiency of the DNA metabolizing zinc-enzymes, giving rise to abnormal neuronal DNA and synthesis of pathological proteins: NFT. In encephalopathia Saturnica, zinc decreases in the hippocampus displaced by lead; in Guam's encephalopathy, calcium deficiency permits the entry in the brain of toxic metals that may displace zinc; in Boxer's dementia and some viral encephalitides, blood-brain-barrier (BBB) is altered and abnormal metals may reach the brain; in Down's syndrome and Alzheimer's disease precapillary and capillary amyloidosis disturbs the BBB, metals (iron and aluminium) are encrusted in the amyloid and their brain level increases, whereas zinc decreases especially in the hippocampus. A deficiency of the zinc enzymes of neuronal detoxication, of glutamate catabolism and of some neurotransmitters metabolisms may also contribute in the neuronal dysfunction of these encephalopathies. A non-toxic zinc compound crossing the BBB may be useful for the treatment of these encephalopathies and especially for Alzheimer's disease.
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PMID:The hypothesis of zinc deficiency in the pathogenesis of neurofibrillary tangles. 194 84

After a careful characterization, a rat model of fulminant hepatic failure galactosamine-induced was utilized in order to evaluate the neurochemical changes and the histological alterations which occur during the developing of the encephalopathy. Following these studies, normal rats were treated with toxins claimed to be the primary agents of hepatic encephalopathy to recognize those which are able to mimic the behavioral, electrophysiological and neurochemical changes found in the rat model of fulminant hepatic failure. With the limit due to informations coming from an experimental model, the symptoms of HE seem to be attributable to neurotoxic agents such as ammonia. The toxicity of ammonia does not seem to be due to a mere decrease of general brain metabolism, but seems rather to be mediated by an increase, at least in some compartment, of neurotoxic amino acids such as glutamate. Both accumulation of ammonia and the neurotoxic effect of glutamate seem to be potentiated by the described zinc depletion (both in liver and in brain). Hence the final effect of these phenomena is the development of the symptoms of encephalopathy triggered by an imbalance between inhibitory and excitatory receptor systems in the brain associated with neuronal alterations which take place early and before the appearance of brain edema.
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PMID:Neurotransmission in hepatic encephalopathy. 196 5

Encephalopathy in liver disease may be unresponsive to protein restriction, lactulose, and neomycin. Zinc supplements have been reported to improve psychometric performance in liver cirrhosis, but the importance of zinc deficiency in overt hepatic encephalopathy has not yet been clearly established. A patient with severe recurrent hepatic encephalopathy was studied to determine the relation between her signs of encephalopathy and zinc deficiency. The study included a period in which zinc deficiency was artificially induced by oral histidine. An episode of overt encephalopathy occurred that was identical to earlier episodes and responded to oral zinc. The study showed an association between encephalopathy and zinc deficiency by successive zinc depletion and supplementation regimens. Long-term zinc supplementation improved severe recurrent hepatic encephalopathy and therefore the quality of life.
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PMID:Overt hepatic encephalopathy precipitated by zinc deficiency. 200 10

Abnormalities of trace elements such as aluminum, silicon, zinc, manganese and nickel were found in chronic hemodialysis (HD) patients. The relationship between serum concentration of trace elements and complications such as anemia bone disease, neurological disorder and nutritional disorder in 100 chronic HD patients was examined. Serum Al and Si levels were very high in these patients, and were negatively correlated with indicators of anemia such as RBC, hemoglobin and hematocrit values. Serum Al and Si levels in the HD patients with peripheral neurological disorder (excluding encephalopathy) and with bone disease were significantly high. The levels of serum Zn, Mn and Ni were low, and were correlated with total serum protein levels. In summary, in order to prevent some complications in chronic HD patients, it is very important to regulate levels of trace elements.
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PMID:Trace elements and complications in patients undergoing chronic hemodialysis. 192 25

A 29-year-old single woman had recurrent stroke-like episodes. She developed loss of consciousness, myoclonic seizures, and lactic acidosis. She died at the age of 30. A muscle biopsy study revealed mitochondrial myopathy, and the postmortem biochemical analysis demonstrated decreased cytochrome c oxidase activity in the skeletal muscles by 20% of normal control. The brain had multiple ischemic lesions in the cerebral cortex without major vascular occlusions. We present this case as an autopsy case of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) with a partial deficiency of cytochrome c oxidase. The analytical electron microscopic study of the calcified small vessels in the globus pallidus revealed increased calcium, phosphorus and iron. No accumulation of chromium, nickel or zinc was noted in this case, which was different from the previously reported cases of basal ganglia calcification.
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PMID:An autopsy case of mitochondrial encephalomyopathy: biochemical and electron microscopic studies of the brain. 284 99


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