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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Many New Zealand soils are naturally deficient in cobalt and/or selenium and/or copper, or are naturally high in molybdenum. Livestock grazing pasture grown on such soils may be deficient in one or more of these trace elements. In the 1940s and 1950s, New Zealand researchers were at the forefront of research to define the cause of trace-element related ill-thrift and clinical diseases like white muscle disease, peat scours and enzootic ataxia. New Zealanders have devised production-related reference ranges for blood and liver copper, vitamin B12 and selenium that are used for the diagnosis and prevention of deficiencies. A range of supplementation procedures has been devised, from topdressing or spraying pasture to direct animal supplementation, to suit the range of livestock management systems found in New Zealand. Trace-element monitoring programmes are now a routine procedure for farmers grazing cattle, sheep, and deer on trace-element deficient land. Copper deficiency is the main trace-element deficiency diagnosed in deer.
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PMID:Major trace elements limiting livestock performance in New Zealand. 1603 34

The first deer farms were established in New Zealand about 30 years ago. Extensive studies on trace elements in sheep and cattle have resulted in clarification of the requirements of those species and the development of protocols to diagnose and prevent deficiencies. In contrast, there have been very few studies conducted with deer. This review summarises information available on trace element nutrition of deer and concludes that, in New Zealand, cobalt (Co), vitamin B12, selenium (Se) and iodine (I) deficiencies are of lesser importance than copper (Cu), which can have a significant impact on deer health and performance. However, on individual farms, Se and I deficiency may cause significant production losses if not managed appropriately. There are no reports of production limitations caused by Co deficiency. Copper deficiency manifests itself as clinical disease, namely enzootic ataxia and osteochondrosis. Growth responses to Cu supplementation have only been reported in 2/11 trials and were not predicted from low serum and/or liver Cu concentrations. On the basis of clinical signs of Cu deficiency, the proposed reference ranges used to predict Cu status from serum Cu concentrations (micromol/l) are: 5, deficient; 5-8, marginal and; 8, adequate; and for liver Cu concentrations (micromol/kg fresh tissue) are: 60, deficient; 60-100, marginal and; 100, adequate. Copper supplementation strategies based on Cu-EDTA injections, Cu-oxide needles or the application of Cu to pasture are effective at increasing Cu status for varying periods. More recent research suggests that alternative forage species that have a high Cu content (10 mg/kg dry matter (DM), may play a role in the prevention of Cu deficiency.
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PMID:Trace element metabolism, dietary requirements, diagnosis and prevention of deficiencies in deer. 1603 81

Acquired copper deficiency presents with a spastic gait and sensory ataxia. Spinal cord magnetic resonance imaging (MRI) in patients with copper deficiency myelopathy may show increased T2 signal, most commonly in the dorsal midline cervical and thoracic cord. These imaging findings may be reversible with normalization of serum copper. The clinical and imaging picture is very similar to the subacute combined degeneration seen in patients with vitamin B12 deficiency. Neuroradiologists should consider this possibility when a long segment of symmetric dorsal spinal cord T2-hyperintensity is identified.
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PMID:Imaging features of copper deficiency myelopathy: a study of 25 cases. 1626 34

Wilson disease (WD) is an autosomal recessive inherited disorder of copper metabolism, resulting in pathological accumulation of copper in many organs and tissues. The hallmarks of the disease are the presence of liver disease, neurologic symptoms, and Kayser-Fleischer corneal rings. The leading neurologic symptoms in WD are dysathria, dyspraxia, ataxia, and Parkinsonian-like extrapyramidal signs. Changes in the basal ganglia in brain magnetic resonance imaging (MRI) are characteristic features of the disease. In presence of liver cirrhosis, some features may resemble hepatic encephalopathy. Symptoms and MRI abnormalities may be fully reversible on treatment with zinc or copper chelators. Improvement can be monitored by serial recording of brain-stem-evoked responses. The basic defect is an impaired trafficking of copper in hepatocytes. ATP7B is the gene product of the WD gene located on chromosome 13 and resides in hepatocytes in the trans-Golgi network, transporting copper into the secretory pathway for incorporation into apoceruloplasmin and excretion into the bile. While about 40% of patients preset with neurologic symptoms, little is known about the role of copper and ATP7B in the central nervous system. In some brain areas, like in the pineal gland, ATP7B is expressed and functionally active. Increasing evidence supports an important role for metals in neurobiology. Two proteins related to neurodegeneration are copper-binding proteins (1) the amyloid precursor protein (APP), a protein related to Alzheimer's disease, and (2) the Prion protein, related to Creutzfeldt-Jakob disease. A major source of free-radical production in the brain derives from copper. To prevent metal-mediated oxidative stress, cells have evolved complex metal transport systems. APP is a major regulator of neuronal copper homeostasis and has a copper-binding domain (CuBD). The surface location of this site, structural homology of CuBD to copper chaperones, and the role of APP in neuronal copper homeostasis are consistent with the CuBD acting as a neuronal metallotransporter. There are several copper-containing enzymes in the brain, like dopamine beta hydroxylase or Cu/Zn superoxide dismutase (SOD1). Their function may be altered because of copper overload. WD appears to be associated with a dopaminergic deficit. Mutations in the SOD1gene cause familial amyotrophic lateral sclerosis. Survival of transgenic mice with a mutant SOD1 which fails to incorporate Cu((2+)) in its active site was improved by copper depletion. Wilson disease (WD) is an autosomal recessive inherited disorder in which copper pathologically accumulates primarily within the liver and subsequently in the neurologic system and many other organs and tissues. Presence of liver disease, neurologic symptoms, and Kayser-Fleischer corneal rings are the hallmarks of the disease.
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PMID:Wilson disease. 1638 40

Copper deficiency in humans is a rare cause of myeloneuropathy that usually presents with a spastic ataxic gait, hyperreflexia, and distal sensory loss similar to that seen in patients with subacute combined degeneration. We describe three copper-deficient patients, two of whom were referred with a presumptive diagnosis of amyotrophic lateral sclerosis, who had progressive asymmetric weakness or electrodiagnostic findings of proximal and distal denervation suggestive of lower motor neuron disease. Copper replacement resulted in stabilization or mild improvement in weakness. The clinical spectrum of human copper deficiency should include lower motor neuron disease in addition to a syndrome of spastic ataxia.
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PMID:Motor neuron disease associated with copper deficiency. 1692 46

Ataxia-telangiectasia (A-T) is characterized by ataxia, genomic instability, and increased cancer incidence. Previously, iron chelator concentrations which suppressed normal cell colony formation increased A-T cell colony formation. Similarly, iron chelators preferentially increased A-T cell colony formation following peroxide exposure compared to normal cells. Last, A-T cells exhibited increased short-term sensitivity to labile iron exposure compared to normal cells, an event corrected by recombinant ATM (rATM) expression. Since chromosomal damage is important in A-T pathology and iron chelators exert beneficial effects on A-T cells, we hypothesized that iron chelators would reduce A-T cell chromosomal breaks. We treated A-T, normal, and A-T cells expressing rATM with labile iron, iron chelators, antioxidants, and t-butyl hydroperoxide, and examined chromosomal breaks and ATM activation. Additionally, the effect of ATM-deficiency on transferrin receptor (TfR) expression and TfR activity blockage in A-T and syngeneic A-T cells expressing rATM was examined. We report that (1) iron chelators and iron-free media reduce spontaneous and t-butyl hydroperoxide-induced chromosomal breaks in A-T, but not normal, or A-T cells expressing rATM; (2) labile iron exposure induces A-T cell chromosomal breaks, an event lessened with rATM expression; (3) desferal, labile iron, and copper activate ATM; (4) A-T cell TfR expression is lowered with rATM expression and (5) blocking TfR activity with anti-TfR antibodies increases A-T cell colony formation, while lowering chromosomal breaks. ATM therefore functions in iron responses and the maintenance of genomic stability following labile iron exposure.
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PMID:Iron chelators reduce chromosomal breaks in ataxia-telangiectasia cells. 1695 48

Within a 1-year period, three calves from the same herd developed ataxia of the hind limbs and urinary incontinence at about 6 months of age. Signs progressed and the calves were slaughtered 1-8 months after the onset of signs. The calves belonged to a suckling beef herd of 35 cattle. Blood samples from 11 cattle of different ages were collected and glutathione peroxidase and copper levels were measured. Glutathione peroxidase levels were below the normal range in all cattle and copper levels were below the normal range in 7 of ll cattle. Pathological examination of an affected calf revealed a Wallerian type of degeneration of myelinated nerve fibres in the lateral and ventral spinal cord tracts. In addition, the hepatic copper content was very low. Copper deficiency is a well-known cause of swayback in young sheep and goats. To our knowledge, this is the first report of a possible association between a swayback-like syndrome and copper deficiency in calves.
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PMID:[Suckling calves with symptoms of swayback]. 1701 93

The hematologic manifestations of copper deficiency are well known and include anemia and neutropenia. In the past few years, the neurological manifestations of acquired copper deficiency in humans has been recognized, the most common being a myelopathy presenting with a spastic gait and prominent sensory ataxia. The known causes of acquired copper deficiency include prior gastric surgery, excessive zinc ingestion, and malabsorption; however, often the cause is unclear. Hyperzincemia may be present even in the absence of exogenous zinc ingestion. The clinical features and neuroimaging findings are similar to the subacute combined degeneration seen in patients with vitamin B12 deficiency. Copper and vitamin B12 deficiency may coexist. The neurological syndrome may be present without the hematologic manifestations. Copper supplementation resolves the anemia and neutropenia promptly and completely and may prevent the neurological deterioration. Improvement, when it occurs, is often subjective and preferentially involves sensory symptoms. This article describes patients with copper deficiency myelopathy seen at the Mayo Clinic in Rochester, Minn, and reviews the literature on neurological manifestations of acquired copper deficiency in humans.
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PMID:Copper deficiency myelopathy (human swayback). 1703 63

Oxidative stress is an abnormal phenomenon occurring inside our cells or tissues when production of oxygen radicals exceeds their antioxidant capacity. Excess of free radicals damage essential macromolecules of the cell, leading to abnormal gene expression, disturbance in receptor activity, proliferation or cell dye, immunity perturbation, mutagenesis, protein or lipofushin deposition. Numerous human diseases involve during the pathological process such a stress, localized or general (in the same way as inflammation). In many serious diseases such as cancer, ocular degeneration (age related macular degeneration or cataract), neurodegenerative diseases (ataxia, amyotrophic lateral sclerosis, Alzheimer's disease) stress is the factor original. In familial amyotrophic lateral sclerosis the genetic abnormality occurred an abnormal coding for an antioxidant enzyme, copper-zinc super oxide dismutase. In various other diseases oxidative stress occur secondary to the initial disease but plays an important in role immune or vascular complications. This is the case in infectious disease such as AIDS or septic shock, Parkinson's disease or renal failure. So antioxidant treatment seems logical to be tested in these pathologies. But they have to be applied early in the process, before irreversible mechanisms. They need also to be prescribed at low doses as baseline free radical production have to be preserved to maintain useful activity that cannot be suppressed.
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PMID:[Oxidative stress in human diseases]. 1711 68

Polioencephalomalacia (PEM), hereafter used to refer to the specific lesion of cerebrocortical necrosis, developed in 11 of 110 mature cattle on pasture in central Saskatchewan. The primary water source contained a markedly elevated level of sodium sulfate (7200 ppm). The significant clinical findings of the herd investigation included depression, ataxia, cortical blindness, dysphagia, and death. Diagnosis of PEM was confirmed by histopathological evidence of cerebrocortical and subcortical necrosis with microvascular fibrinoid necrosis predominantly in the thalamic region of three affected cattle. The histopathology of sulfate-associated PEM observed in this herd appears to be unique and its features are presented and discussed. Mean levels for serum transketolase, copper, red blood cell transketolase activity, and thiamine (vitamin B(1)) in all exposed young (n = 100) and mature (n = 99) animals did not reveal evidence of deficiencies. Although the blood thiamine status of the seven surviving, affected animals was not evaluated before treatment with exogenous thiamine, 199 members of the herd had blood thiamine levels within the reference range at the time of the outbreak. The outbreak resolved after cattle were moved to a water source containing acceptable levels of sodium sulfate.
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PMID:Polioencephalomalacia in cattle consuming water with elevated sodium sulfate levels: A herd investigation. 1742 82


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