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

Toxic metals encountered industrially or environmentally may produce the following syndromes: 1) Peripheral neuropathy: which is mainly sensory in arsenic and entirely motor with inorganic lead, organophosphorus compounds and tallium produce a mixed form of peripheral neuropathy. 2) Encephalopathy: usually with lead poisoning where ataxia and hemiplegia or optic atrophy may occur. 3) Optic neuritis: transient or permanent impairment of vision in arsenic poisoning and blurring of vision followed by field fedects with thallium poisoning. 4) Cerebellar disturbances: in the form of ataxia in organic mercury. 5) Parkinsonism: extrapyramidal signs occurs in manganese poisoning shown as mask face and rigidity of muscles. 6) Mental changes: as acute psychosis in organic lead and erethism in organic mercury.
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PMID:Neurological syndromes produced by some toxic metals encountered industrially or environmentally. 35 38

Nineteen calves with a clinical history of posterior weakness or ataxia were examined at necropsy. Dorsoventral narrowing of the vertebral canal and myelopathy were found in the thoracic and lumbar areas of the spinal cord; vertebral metaphyseal growth plates had focal areas of premature closure. Malformation of the cranial base and hydrocephalus were also observed. Shortening of long bones and malformation of long bone epiphyses were prominent findings and were associated with focal premature closure of metaphyseal growth plates. The cause of these lesions is unknown; possible causes, including a maternal deficiency of manganese, are discussed.
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PMID:Congenital spinal stenosis in beef calves in western Canada. 230 77

Hyperendemic foci of motor neurone disease, and other neurodegenerative disorders, are located in the western Pacific area, in the Japanese of the Kii Peninsula of Honshu Island, the Chamorros of the Mariana Islands, the Auyu and Jakai of West New Guinea. It is suspected that there is a common aetiologic pathway from toxic metal and essential minerals in these three foci. A fourth focus of motor neurone disease occurs in an isolated tribal group living on the same Pacific longitude, at Angurugu on Groote Eylandt in the Gulf of Carpentaria, Northern Australia. This environment is also characterized by local ecological extremes, including low calcium and iron, and high manganese. The "Angurugu syndrome", described in this paper, shows dysfunction of motor neurones, including upper and lower motor, cerebellar, extrapyramidal and cranial nerves, especially oculomotor. About half the cases emerge in adult life. The others are evident in early childhood. The syndrome is viewed not as simple manganism but as manganism synergistic with accompanying mineral changes. No autopsy studies have been carried out. This paper suggests that this syndrome incurs a loss of the neurotransmitter dopamine. A case study is presented that indicates the unusual range of symptoms, including ataxia, and partial relief by L Dopa (Sinemet).
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PMID:Motor neurone disease of the western Pacific: do the foci extend to Australia? 269 31

A specific congenital ataxia may be caused by presence of mutant genes and by manganese deficiency during prenatal development in normal mice. Supplementation of the diet of mutant mice with manganese during prenatal development rectifies the aberrant development, resulting in normal behavior. The congential ataxa results from defective development of the the otoliths.
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PMID:Neurological defect: manganese in phenocopy and prevention of a genetic abnormality of inner ear. 594 42

Manganese, zinc and copper are essential for normal prenatal and neonatal development. Manganese deficiency causes skeletal abnormalities, congenital ataxia due to abnormal inner ear development, and abnormal brain function. Depression of mucopolysaccharide synthesis and manganese superoxide dismutase activity may be fundamental to ultrastructural and other defects. In copper deficiency, neurological and skeletal abnormalities are due to impairment of phospholipid synthesis and collagen crosslinking, and possibly to low activity of copper metalloenzymes. The fundamental defect leading to the extremely teratogenic effects of zinc deficiency is related to depressed synthesis of DNA. In the neonatal period, poor survival and growth and depressed function of the immune system are salient features. Developmental patterns of trace element concentrations in various tissues suggest that important changes in metabolic regulation of trace elements may occur during the neonatal period. This hypothesis is being investigated by studies of molecular localization of trace elements in certain neonatal tissues, in conjunction with similar observations in milk.
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PMID:The roles of trace elements in foetal and neonatal development. 611 92

With reference to previous reports on hypocalcemia in horses special attention is given to the principal symptoms such as anxiety, increased muscular tension, esp. of the musculature of the extremities, in severe cases leading to ataxia, and to a pulse-synchronized respiration (Synchronous Diaphragmatic Flutter). The time of appearance of the symptoms are much more variable than it is the case for hypocalcemic agalactia in cows. Furthermore the condition has been reported in foals as well as in geldings. Thus the triggering factor seems to be somewhat different from that of agalactia. Hypocalcemic horses also develop decreased manganese blood-level. Treatment with Ca-borogluconat in the horse can be as efficient as is the case in agalactia in the cows, but quite often it is necessary to repeat the treatment. This is illustrated by the fact that the relatively high dose (17 mg Ca/kg) administered to the present patient only increased the Calcium blood level to somewhat below normal. The triggering condition, estrus, weaning of the foal, stress, hard (sweat-provoking) work etc., in many cases perhaps a kind of mental stress, must be adequately treated. An all-round and sufficient feed ration in accordance with the working load and eventually with milk production may prove to be much more essential than realized so far for the prevention of hypocalcemia in horses.
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PMID:[Hypocalcemia in the horse. A case report]. 738 45

Toxic chemicals in the environment can cause a wide range of neurological disease. High-dose exposures to environmental neurotoxicants have produced encephalopathy in children ingesting chips of lead-based paint, blindness in persons who ingested methanol, blindness and ataxia in persons who consumed organic mercury, spinal cord degeneration and peripheral neuropathy in persons exposed to tri-ortho-cresyl phosphate (TOCP), and Parkinsonism in persons exposed to MPTP or to manganese. Environmental neurotoxicants have also been shown to produce a wide range of subclinical neurotoxic effects, including reduction in intelligence, impairment in reasoning ability, shortening of attention span, and alternation of behavior. The first step in the prevention of environmental neurotoxicity is to test chemicals for their toxic potential. More than 70,000 chemicals are currently in commerce. However, except for pharmaceuticals, fewer than 10% of these chemicals have been tested for neurotoxicity. A logical approach to neurotoxicologic assessment of chemical substances will build on and extend currently available test systems. It will have a tiered structure. The first or screening tier will consist of tests to measure obvious structural and functional changes, often a functional observational battery. Subsequent levels of testing will be guided by the results of initial screening. Toxicologic testing must be supplemented by epidemiologic surveillance of populations exposed to known and suspect neurotoxicants. Screening programs in these populations designed to detect excessive absorption of a neurotoxic agent or subclinical neurological dysfunction can be useful in identifying affected individuals before severe disability occurs.
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PMID:Strategies for the prevention of environmental neurotoxic illness. 847 70

Chronic acquired hepatocerebral degeneration (CAHD) is a heterogeneous disorder that can occur with a primary neurologic, hepatic, or combined presentation. Little has been added to the understanding of this disorder since the detailed, early clinical and pathological descriptions. The spectrum of clinical presentations can be neuropsychiatric (apathy, lethargy, excessive somnolence), a movement disorder (ataxia, tremor, chorea, parkinsonism, myoclonus, dystonia), or both. Cortical laminar necrosis and polymicrocavitation in the cortex and basal ganglia are combined with cerebral and cerebellar atrophy. Microscopically, Alzheimer type II astrocytes and cytoplasmic glycogen granules are characteristic. Recent neuroradiological observations in patients with liver failure have shown a specific magnetic resonance (MR) imaging appearance with a hyperintense T1 signal in the pallidum, putamen, and, rarely, mesencephalon. Using clues from a similar MR appearance in patients receiving total parenteral nutrition as well as animals given parenteral manganese, and the knowledge that manganese is cleared by the hepatobiliary system, deposition of manganese in the brain is postulated in patients with CAHD. In this review we describe three cases of CAHD with detailed clinical and radiological documentation and discuss the aforementioned pathogenetic mechanisms.
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PMID:Chronic acquired hepatocerebral degeneration: case reports and new insights. 886 9

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

In experimental animals, dietary manganese deficiency can result in numerous biochemical and structural abnormalities. Deficient animals can be characterized by impaired insulin production, alterations in lipoprotein metabolism, an impaired oxidant defense system, and perturbations in growth factor metabolism. If the deficiency occurs during early development, there can be pronounced skeletal abnormalities and an irreversible ataxia. Several lines of evidence suggest that manganese deficiency may be a problem in some human populations. Manganese toxicity can also pose a significant health risk. In experimental animals, acute manganese toxicity can result in numerous biochemical pathologies. However, the above occurs typically when the manganese is given via injection; most animals show considerable resistance to dietary manganese toxicosis. Similarly, confirmed cases of manganese toxicity in humans are currently restricted to cases of exposure to high levels of airborne manganese, and to cases when manganese excretory pathways are compromised.
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PMID:Nutritional aspects of manganese from experimental studies. 1038 85


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