Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0040822 (tremor)
 18,428 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

As a possible preventive measure for brain dysfunction in Menkes disease, prenatal treatment by maternal administration of zinc, vitamin E and copper was examined in brindled mutant mice. During pregnancy and lactation, female heterozygous mice received 20 ppm zinc or 0.004% alpha-tocopherol acetate (vitamin E) throughout and 6 ppm copper from gestational day 13 in the drinking fluid, ad libitum. The maternal administration of zinc and vitamin E, as antioxidants, or copper resulted in decreased fetal and neonatal death of offspring, especially those of hemizygous males, as compared with the administration of water only. When offspring did not grow, maternal abnormal movements, which comprised rotatory movements of high speed with tremor and ataxia, were frequently observed. In the heterozygotes with abnormal movements, the level of lipid peroxidation in cerebrum and the concentration of copper in kidney were much higher than those in the heterozygotes with normal movement. Morphologically, in cerebellum of the heterozygotes with abnormal movements, the loss of Purkinje cells, abundance of lipofuscin granules and abnormal mitochondria or degenerative bodies of high electron density were frequently observed, as compared with heterozygotes with normal movement. These findings suggest that the development of hemizygous male mice may be influenced by both copper and oxygen radical metabolism.
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PMID:Abnormal movements in brindled mutant mouse heterozygotes: as related to the development of their offspring--biochemical and morphological studies. 216 11

Sibling cases of familial vitamin E deficiency accompanied by ataxia, polyneuropathy and mental retardation were reported. Case 1 was a 37-year-old male who developed progressive gait disturbance, deformity of the feet and head tremor from childhood, after normal delivery and development of early childhood. On physical examination, he had cataract, high arched palate and pes cavus. Neurological examination revealed mental retardation (WAIS 68), scanning speech, muscular atrophy of the face and extremities with predominance in the lower limbs, absent Achilles tendon reflex, disturbance of superficial and deep sensation predominant in distal limbs, and marked gait ataxia. Ataxia was both cerebellar and sensory in nature. Laboratory data of the blood showed no significant abnormalities including blood glucose and vitamin B12 except a markedly low level of serum vitamin E. The brain CT scan revealed severe cerebellar atrophy and marked dilatation of the cisterna magna and the subarachnoid space around the cerebellum. Motor nerve conduction velocity in the leg was decreased. Biopsy specimen from the quadriceps muscle showed neurogenic atrophy. Sural nerve biopsy revealed decrease in large myelinated fibers with axonal degeneration and regeneration. Oral administration of alpha-tocopherol acetate, 600 mg per day, diminished ataxia significantly. Based on lysosomal enzyme activity in leukocytes, clinical and laboratory examination, lipidosis or spinocerebellar degeneration was excluded. Chronic lipid malabsorption or beta lipoprotein deficiency which can cause decrease in vitamin E absorption, was not recognized. On oral loading with 2 g of alpha-tocopherol acetate, the decrease rate of serum vitamin E was normal. Consequently the low vitamin E was considered to have resulted from selective impairment of vitamin E absorption.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Familial idiopathic vitamin E deficiency associated with cerebellar atrophy]. 226 7

Patients with cystic fibrosis (CF) and pancreatic malabsorption frequently have vitamin E deficiency. Affected patients may develop spinocerebellar degeneration with dysarthria, ataxia, proximal weakness, proprioceptive loss and areflexia. Of a highly selected group of 10 patients with vitamin E levels below 5 micrograms/ml (normal 5-20 micrograms/ml), 7 had abnormal neurological examinations, predominantly affecting vibration and joint position perception with some severely affected patients manifesting diminished visual acuity, tremor, ataxia and diffuse weakness. Evoked potential studies showed marked abnormalities in 3 patients, demonstrating deficits in the optic pathways and in the cervical cord dorsal column pathways. Evoked potential studies may supplement careful neurological examination in patients with CF before and after supplementation with vitamin E to evaluate their progression and response to treatment.
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PMID:Visual and somatosensory evoked potentials in vitamin E deficiency with cystic fibrosis. 245 91

A simple fluorometric method suitable for serial determinations is reported for simultaneous determination of vitamins A and E in serum. The solvents are pretreated chemically and by distillation. After shaking with ascorbic acid in ethanol, the vitamins are extracted from the serum samples with petroleum ether. The extract is used for the determination. The sensitivity of the method is 20 micrograms/100 ml for vitamin A and 0.04 micrograms/ml for vitamin E. Recovery rates are between 87 and 106%. The method was tested on a total of 160 serum samples of healthy animals belonging to 8 different species. The results showed good agreement with the so-called reference values published in the literature.
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PMID:Simultaneous fluorometric determination of vitamins A and E in serum. 262 97

Four-to-five-week-old turkey poults fed a diet markedly deficient in vitamin E (alpha-tocopherol) abruptly developed neurologic signs such as tremor, incoordination, and recumbency shortly after being moved to new quarters. Serum concentrations of alpha-tocopherol in birds on this diet were significantly lower than control values. Associated lesions included recent ischemic necrosis of the cerebellum and spinal cord. The condition closely resembled nutritional encephalomalacia of chicks. This report represents the initial published description of that entity in turkeys.
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PMID:Encephalomalacia associated with hypovitaminosis E in turkey poults. 402 40

Large intramuscular doses of a water-miscible preparation of vitamin A (500,000 I.U. retinyl acetate/ml), vitamin E (50 I.U./ml) and vitamin D2 (50,000 I.U./ml) were administered to young monkeys (Macacus fascicularis) weighing 1-1.8 kg. At vitamin A doses equivalent to 200 mg retinol/kg or higher, early signs of acute toxicity included yawning, apparent drowsiness, nausea and vomiting, head shaking, neck hyperextension, motor hyperactivity and coordination. These immediate signs were first noted 3-35 minutes after injection. Following apparent recovery at 1-2 hrs, longer term signs of toxicity, such as decreased activity, malaise, drowsiness, loss of appetite, loss of weight, and itchiness of the skin, appeared within 1-6 days, depending on the dose. Monkeys receiving the highest lethal doses became progressively weaker, showed labored breathing, lapsed into a coma, lost simple reflexes and then died. Respiratory failure usually preceded the cessation of heart beat. In some monkeys on a lower but lethal dose, death was preceded by generalized convulsive seizures. The time of onset of the first sign and survival time were inversely proportional to the dosage, but in individual monkeys no correlation existed between onset time and survival time. Female monkeys seemed to succumb faster to a lethal dose than male monkeys. All animals receiving the equivalent of 300 mg retinol/kg died. Under the conditions used, the LD50 was estimated to be 168 mg retinol (560 000 I>U.) per body weight.
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PMID:A lethal hypervitaminosis A syndrome in young monkeys (Macacus fascicularis) following a single intramuscular dose of a water-miscible preparation containing vitamins A, D2 and E. 697 50

One-month-old male Sprague-Dawley rats were fed a basal vitamin E deficient diet with or without 50 ppm vitamin E supplementation for 7 months. The washed red cells were suspended in a saline-phosphate buffer, pH 7.4, that contained either 0, 0.011 or 0.055 M glucose and were incubated at 37 C with constant shaking. Catalase activity in the red cells of vitamin E deficient rats was decreased 74% (P less than 0.001) at the end of the 22-hour incubation, and only 9% of the initial value was retained at the end of 46 hours. In the red cells of the vitamin E supplemented group, 82% and 48% of catalase activity was retained at the end of 22 and 46 hours, respectively. Glucose in the medium significantly increased catalase activity during the early hours of incubation and retarded the enzyme inactivation at the end of 22 and 46 hours in both groups of animals. The activities of superoxide dismutase and glutathione peroxidase were not significantly altered by the presence of glucose or by the status of dietary vitamin E during the incubation. The results suggest that both glucose and dietary vitamin E provide protection against inactivation of catalase under the experimental conditions.
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PMID:Glucose and dietary vitamin E protection against catalase inactivation in the red cells of rats. 720 46

Chronic adriamycin (ADR) intoxication with cardiomyopathy developed in young Beagle dogs given weekly IV injections (1 mg/kg of body weight) for 20 weeks (cumulative dose 400 mg/m2). Eighteen dogs were allotted equally to three groups: group A received ADR only, group B was given ADR and simultaneous weekly doses of vitamin E (17 mg/kg of body weight as alpha-tocopherol acetate), and group C received ADR, weekly doses of vitamin E as in group B, and selenium (0.06 mg/kg of body weight as selenite). The dogs reacted with cutaneous hyperemia, head shaking, and vomiting immediately after ADR injection. After 4 to 6 weekly injections, all the dogs developed alopecia that was present initially over the head and subsequently extended to the ventral portions of the neck, thorax, and abdomen and the proximal inner areas of the limbs. Other skin lesions present in alopecic areas were secondary ulcerative dermatitis and melanosis. Testicular atrophy and cachexia developed in the dogs, but damage was not present in bone marrow, alimentary tract, kidney, and bone with the dosage schedule utilized. Hematologic studies showed no significant alterations. Supplementation with vitamin E alone or with selenium failed to alter the incidence and severity of extracardiac ADR-induced lesions. This study shows that the dog is a good model for studies of chronic ADR-induced cardiotoxicity, as cardiac damage was consistently produced and ADR-associated extracardiac lesions were of minimal severity.
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PMID:Clinical observations, cutaneous lesions, and hematologic alterations in chronic adriamycin intoxication in dogs with and without vitamin E and selenium supplementation. 740 87

It was noticed that monotonous diet which imitated the composition of diet for the susceptible population of Keshan disease with a habit of food preference was prominently low in calcium and selenium. The plasma calcium ion content of rats kept on a monotonous diet was significantly lowered to merely half the content of the stock diet group with significant lowering of GSH-px activity. After peritoneal injection of furosemide, the plasma calcium ion contents of the monotonous diet group were further lowered significantly and signs of calcium deficiency, such as muscle tremor, spasm and convulsion might occur. If anoxic factor NaNO2 acted simultaneously, acute and severe myocardial necrosis developed. Morphologically, the myocardial necrosis was similar to that in Keshan disease. When monotonous diet was supplemented with only calcium, the growth state of the rats was significantly improved, and the degree of myocardial necrosis was significantly decreased. Supplement of calcium, selenium as well as vitamin E, seemed more effective. It is suggested that low selenium is the basic factor of endemic pathogenesis of Keshan disease, and low calcium intake by food preference among the susceptible population of Keshan disease plays an important role in the pathogenesis of myocardial necrosis in Keshan disease.
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PMID:[Effects of low calcium on myocardial necrosis of Keshan disease by food preference]. 822 83

Parkinson's disease, known also as striatal dopamine deficiency syndrome, is a degenerative disorder of the central nervous system characterized by akinesia, muscular rigidity, tremor at rest, and postural abnormalities. In early stages of parkinsonism, there appears to be a compensatory increase in the number of dopamine receptors to accommodate the initial loss of dopamine neurons. As the disease progresses, the number of dopamine receptors decreases, apparently due to the concomitant degeneration of dopamine target sites on striatal neurons. The loss of dopaminergic neurons in Parkinson's disease results in enhanced metabolism of dopamine, augmenting the formation of H2O2, thus leading to generation of highly neurotoxic hydroxyl radicals (OH.). The generation of free radicals can also be produced by 6-hydroxydopamine or MPTP which destroys striatal dopaminergic neurons causing parkinsonism in experimental animals as well as human beings. Studies of the substantia nigra after death in Parkinson's disease have suggested the presence of oxidative stress and depletion of reduced glutathione; a high level of total iron with reduced level of ferritin; and deficiency of mitochondrial complex I. New approaches designed to attenuate the effects of oxidative stress and to provide neuroprotection of striatal dopaminergic neurons in Parkinson's disease include blocking dopamine transporter by mazindol, blocking NMDA receptors by dizocilpine maleate, enhancing the survival of neurons by giving brain-derived neurotrophic factors, providing antioxidants such as vitamin E, or inhibiting monoamine oxidase B (MAO-B) by selegiline. Among all of these experimental therapeutic refinements, the use of selegiline has been most successful in that it has been shown that selegiline may have a neurotrophic factor-like action rescuing striatal neurons and prolonging the survival of patients with Parkinson's disease.
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PMID:Oxidative stress and antioxidant therapy in Parkinson's disease. 883 Mar 46


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