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Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To further elucidate the involvement of metals as a factor in the pathogenesis of amyotrophic lateral sclerosis (ALS) on the Kii peninsula of Japan, a well-known high incidence area of ALS with low calcium and magnesium contents in soil and drinking water, we determined concentrations of these metals in samples of central nervous system tissue taken from postmortem ALS cases. Calcium content was determined by neutron activation analysis and magnesium by inductively coupled argon plasma emission spectrometry. From 5 ALS cases and 5 neurologically normal controls, we examined tissues from the precentral gyrus, including the motor cortex, internal capsule, crus cerebri and spinal cord, and from 22 other areas. The average calcium content in precentral gyrus, internal capsule, crus cerebri and spinal cord in ALS cases was higher than that in the controls, and the mean value of all 26 areas in the ALS cases was also higher than that of the controls. The average magnesium content of each region as well as the mean value of the 26 regions in the ALS cases was significantly lower than that in the controls. The Ca/Mg ratio of the 26 ALS regions was significantly higher than that of controls. This study strengthens our hypothesis that an abnormal metal metabolism plays a responsible role in the Ca-hydroxyapatite formation observed in central nervous system tissue of ALS cases, leading to motor neuron death and degeneration of the pyramidal tracts.
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PMID:Magnesium and calcium contents in CNS tissues of amyotrophic lateral sclerosis patients from the Kii peninsula, Japan. 131 86

A 71-year-old female with amyotrophic lateral sclerosis (ALS) developed the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) during respiratory failure due to atrophy of the respiratory muscles. Serum sodium concentration fell to 116 mEq/l and then returned to the normal range after water restriction and respiratory care. This is considered to be the first case report of ALS associated with SIADH.
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PMID:Amyotrophic lateral sclerosis associated with the syndrome of inappropriate secretion of antidiuretic hormone. 147 61

The Eighth International Neurotoxicology Conference, Role of Toxicants in Neurological Disorders (1990), evaluated the evidence that chemical exposures may play a role in the development of neurodegenerative disorders. This article describes the major neurodegenerative disorders (Amyotrophic Lateral Sclerosis, Huntington disease, Parkinson disease, and Alzheimer disease) addressed at the conference, followed by a description of test systems or models developed to study behavioral aspects of these disorders in animals. However, due to the complexity of the disorders and the species in which they are found, fully-developed models in animals of neurodegenerative disorders are lacking. This suggests the need for a clear strategy for selecting behavioral tests in animals to study aspects of any neurodegenerative disorders. Such a strategy is here exemplified for Alzheimer disease (AD) as a prototypical neurodegenerative disorder. Since an animal model cannot provide the full range of effects of human neurodegenerative diseases, particularly AD which produces incompletely characterized cognitive deficits, a rodent model must at this time be drawn from multiple sources, including: (1) Tests currently used to identify in rodents deficits associated with AD; (2) tests to identify Alzheimer-related signs in patients; and, (3) tests that relate to theoretical constructs of human and animal cognition. A battery that draws from those sources could include tests of: (a) Spatial learning and memory (Morris Water Maze and Radial Arm Maze), (b) delayed recall match-to-sample; (c) serial response learning; and, (d) visual discrimination (e.g., vertical vs. horizontal stimuli). This battery will identify behavioral changes characteristic of early-, middle- and late-stage AD, afford the potential to relate the findings to theoretical constructs of cognition, and evaluate learning capabilities not previously studied in rodent models of neurodegenerative disorders.
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PMID:Animal test systems to study behavioral dysfunctions of neurodegenerative disorders. 174 32

The epidemiology of amyotrophic lateral sclerosis (ALS) in the Western Pacific indicates that low concentrations of calcium (Ca) and magnesium (Mg) and high levels of aluminum (Al) in soil and water in these foci are etiologically important. To determine the biochemical derangements and metal deposition induced by chronic dietary deficiencies of Ca, we maintained experimental animals on several regimens. Male Wistar rats, weighing 100g, were fed either a standard diet, low Ca diet, low Ca-Mg diet, or low Ca-Mg diet with high Al for 90 days. Ca, Mg and Al content was determined in central nervous system (CNS) tissues and bone using inductively coupled plasma emission spectrometry (ICP). In separate studies, five male Japanese macaques (Macaca fuscata), weighing 3.5 to 5 kg, were fed alternately with diets, normal in Ca, low in Ca, low in Mg, low in Ca-Mg, or low in Ca-Mg with added Al for four-week periods. Serum Ca, Mg, Al, parathyroid hormone (PTH), bone Gla-protein (BGP) and alkaline phosphatase (ALP) were measured after feeding each dietary regimen. Ca and Mg levels in lumbar vertebrae and femur were significantly reduced and bone Al levels were significantly increased in rats fed diets deficient in Ca alone or diets low in Ca-Mg with or without added Al. Al content in bones was also higher in rats fed the Ca deficient diets. In monkeys fed the low Ca-Mg diet with added Al, reduced levels of serum Ca and Mg, serum PTH, BGP, and ALP were apparent. Our data support the conjecture that deranged bone mineralization induced by chronic dietary deficiency of Ca accelerates mobilization of Ca and Mg from bone and deposition in brain.
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PMID:Evaluation of magnesium, calcium and aluminum metabolism in rats and monkeys maintained on calcium-deficient diets. 174 43

The etiology of the nigrostriatal pathway degeneration in Parkinson's disease (PD) is unknown but there is a growing pool of evidence that environmental factors may be involved in the genesis of this disorder. The discovery of the N-Methyl 4-Phenyl 1,2,3,6-Tetrahydro-Pyridine (MPTP)-induced injury in late 1970s provided the first experimental model of PD and stimulated dramatically the epidemiological research. An excitotoxic amino acid contained in Cycadales, which is thought to be responsible for the amyotrophic lateral sclerosis-parkinsonism-dementia complex of Guam, provides another example of toxin-induced parkinsonism. This amino acid is present in most seeds common in the Western diet. In developed countries, prevalence of PD is 2 to 5 times as high than in developing countries. PD patients in developed countries are more likely than controls to have lived in rural environment. Case control studies have suggested that this positive association is possibly related to pesticides and herbicides exposures or well water drinking. Dietary surveys are now going on and several hypothesis are tested including high MPTP-structural analogs or seeds consumption in PD patients and low antioxidants consumption. The negative association between smoking habits and PD has been recognized for more than 20 years. There is evidence that this association is not an artefact due to the disease affecting smoking habits. Its origin is unknown but it could provide important aetiological clues for PD. The most recent hypothesis concerning the relationships between these environmental factors and PD are reviewed and pertinent suitable surveys for the future are discussed.
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PMID:[Parkinson's disease and environmental factors]. 175 3

Current changing epidemiological pattern in the Western Pacific area suggests a contribution of the environmental factors to the pathogenetic process of amyotrophic lateral sclerosis (ALS). The condition of unbalanced mineral levels found in the soil and drinking water samples from the ALS foci showing low content of calcium (Ca) and magnesium (Mg) plus high content of aluminum (Al) was experimentally mimicked in this study using rats. In the groups fed low Ca, low Ca-Mg, and low Ca-Mg plus high Al diets, serum Ca levels were lower than that in the group fed the standard diet. Ca content of CNS tissues showed higher values in the unbalanced diet groups, especially in the spinal cord of low Ca-Mg plus high Al diet group, than those in the standard diet group, determined by inductively-coupled plasma emission spectrometry (ICP). Ca content in heart, liver, kidney, and abdominal aorta in groups fed low Ca-Mg, and low Ca-Mg plus high Al diets was higher than that in low Ca, and standard group. Ca content in muscle in the three unbalanced diet groups was significantly higher than in the standard diet group. Ca and Mg contents in lumbar spine and cortical bone showed lower values in the unbalanced diet groups than those values in the standard diet group. These findings suggest that under the condition of derangement of bone mineralization induced by unbalanced mineral diets fed to the experimental rats, Ca and Mg may be mobilized from bone, keeping their content in soft tissues, including CNS tissue, for utilization of vital activities, thereby resulting in a deposition of Ca while maintaining an almost normal value of magnesium in the CNS tissues.
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PMID:Distribution of calcium in central nervous system tissues and bones of rats maintained on calcium-deficient diets. 175 97

Recent decline in incidence rates of ALS in Guam and the Kii Peninsula of Japan strongly implicates environmental factors rather than inheritance in its causation. Environmental studies in Western Pacific foci showed identical mineral compositions in the soils and drinking water, i.e., extremely low calcium (Ca) and magnesium (Mg) and high aluminum (Al) and manganese (Mn). Series of trace-elemental analyses of the CNS tissue of ALS patients have revealed a high contents of Al and Ca with significant positive correlations between Al and Ca and/or between Ca and Mn, suggesting the prolonged exposure to these trace environment to cause abnormal mineral metabolism detrimental to neurons. Using electron energy loss spectrometry (EELS), Al was found to accumulate within DNA-containing chromatins and rRNA-containing cellular components, i.e., nucleolus, heterochromatin, rough endoplasmic reticulum, in lumbar motor neurons of ALS. Thus, Al may preferentially bind to nucleic acids and cause a progressive inhibition of the protein synthesis of rRNA and the transcription or gene modulation of DNA, leading to neuronal degeneration.
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PMID:[Environmental factors in western Pacific foci of ALS and a possible pathogenetic role of aluminum (Al) in motor neuron degeneration]. 181 96

Elevated levels of aluminum in brain tissue have been found in demented patients with Alzheimer's disease, with ALS-PD complex of Guam and with dialysis encephalopathy. A possible etiological relationship between enhanced aluminum exposure and impaired mental function was suggested both for ALS-PD of Guam (a region where high contents of aluminum in water are found) and for dialysis encephalopathy which appears in dialyzed patients exposed to high doses of aluminum in medications and in dialysate fluid. The role of aluminum in Alzheimer's disease is not known as is the question of life-long aluminum accumulation in healthy human beings. In this review we have limited ourselves to the issue of oral aluminum ingestion and the possible neurotoxic consequences of such exposure. The following topics are summarized: 1. Physiological mechanisms involved in ingestion and intestinal absorption of aluminum and the influences of pH and available organic complexing agents on these processes. 2. Effects of an aluminum-enriched diet on behavior and on brain metabolism. 3. Dietary sources of aluminum and elevated loads of this substance due to prolonged intake of aluminum-containing medications. The main conclusion of this summary is that aluminum is absorbed and may accumulate in different organs in both adults and infants. Two groups seem to be at particular risk for aluminum related toxicity: people with chronic renal failure treated with aluminum-containing medications and pre-term infants fed on aluminum containing formulate. It seems probable that at least upon short term exposure the healthy human body can defend itself adequately from aluminum's toxic effects. However, not enough information is available on possible effects of life-long exposure to aluminum in the environment, diet and medications, which over decades may lead to accumulation of this substance with expressions of toxicity. Therefore, the question of aluminum's relevance to dementive diseases cannot yet be adequately answered.
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PMID:Aluminum ingestion--is it related to dementia? 184 54

Current changing epidemiological pattern in the Western Pacific strongly suggests a contribution of the environmental factors to the pathogenetic process of amyotrophic lateral sclerosis (ALS). As a reflection of excess metals and a deficiency of minerals in soil and water samples in these foci, this study was designed experimentally to evaluate the concentration of calcium (Ca), magnesium (Mg) and aluminum (Al) in the bones of rats fed unbalanced mineral diets. Twenty-eight male Wistar rats, weighing 200 g, were fed either a standard diet, a low Ca diet, a low Ca-Mg diet, or a low Ca-Mg diet with high Al for 90 days. The composition of the diet/100 g consists of Ca 1250 mg, Mg 300 mg, Al 10 mg, Zn 4 mg in the standard diet; Ca 3 mg and Mg 2 mg in the low Ca-Mg diet; and Al 194 mg in the high Al diet, Al supplied as Al lactate. Ca, Mg and Al concentrations were determined by using inductively coupled plasma emission spectrometry (ICP) for bone and atomic absorption spectrometry for serum. Serum Ca levels in the groups fed unbalanced mineral diets were lower than those in the group fed standard diet. Serum Mg levels were markedly decreased in the groups fed low Ca-Mg diet and low Ca-Mg plus high Al diet, compared with those in the groups fed standard diet and low Ca diet.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Evaluation of magnesium, calcium and aluminum deposition in bone in situ]. 191 Sep 40

ALS and ALR mice were developed as mouse models of alloxan-induced diabetes. These strains do not show spontaneous onset of diabetes. When an obesity gene (Ay) was introduced to these two strains, severe diabetic conditions occurred spontaneously in the produced ALS-Ay and ALR-Ay strains. These strains were examined body weight gain, food consumption, water consumption, urinary sugar content, ketone body level and blood sugar level, and subjected to glucose tolerance test. As a result, in comparison with ALS mice, male ALS-Ay mice showed no obesity and very low tolerance to the glucose tolerance test performed 24 weeks after birth. The level of insulin secretion was 5.0 microU/ml or less, showing hardly any secretory reaction. On the other hand, female ALS-Ay mice were obese and showed no marked decrease in glucose tolerance. The level of insulin secretion was high, and the secretory reaction was strong. In ALR-Ay strain, both male and female mice were obese and showed diabetic conditions similar to those of ALS-Ay mice, though the severity tended to be lower. The characteristic features of diabetic conditions in these mice suggest that these strains, particularly ALS-Ay, may serve as useful new-type models of diabetes.
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PMID:[Diabetic peculiarity of the ALS-Ay and ALR-Ay strains]. 191 99


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