Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The high incidence rates of amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia (PD) occurring among the Chamorros of Guam have declined to rates only slightly higher than those observed in the continental United States. This decline has occurred principally among males, especially those born after 1920 and living in areas where calcium and magnesium levels are low in soil and water. The male-to-female ratio among affected patients now approaches unity, compared with ratios of 2 to 1 for ALS and 3 to 1 for PD three decades ago. These changes are consistent with the hypothesis that the previously high incidence resulted from defects in mineral metabolism and secondary hyperparathyroidism, provoked by nutritional deficiencies of calcium and magnesium, with resultant deposition of calcium and aluminum in neurons.
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PMID:Disappearance of high-incidence amyotrophic lateral sclerosis and parkinsonism-dementia on Guam. 396 6

Two case-control studies of motor neuron disease that involved 712 cases and 158 cases, respectively, showed that (1) mechanical injuries were two to three times more frequent in both sexes, heralding amyotrophic lateral sclerosis, progressive bulbar paralysis, and progressive muscular atrophy; (2) the head, neck, spine, and the extremities were more often traumatized; (3) traumatized parts were not correlated with the initial manifestation of the disease; and (4) more males were traumatized, but males still predominated among uninjured cases. These results suggested that mechanical injuries were not the cause, but probably one of the risk factors of the disease. No association was observed with smoking, drinking, residence, home space, drinking water, animals, experience as a war prisoner, stay on Guam, parental consanguinity, measles, polio, mumps, tuberculosis, rheumatism, prothesis of the total teeth, shell splinters retained in the body, occupational exposures to radiations, chemicals, or gases, atomic bombings, electric injuries, surgical operations, and occupations.
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PMID:Case-control studies of motor neuron disease: association with mechanical injuries. 701 Dec 80

Amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia (PD) occur in the highest recorded incidence among primitive Auyu and Jakai people on the southern coastal plain of West New Guinea, in association with a heretofore unrecognized subacute, often recurrent, paralytic "poliomyeloradiculitis" (PMR). Ninety-seven cases of ALS, 19 cases of PD and 18 cases of PMR were recorded, with mean ages of onset of 33, 43, and 26, respectively, in a small affected population of only about 7000. The ecology, culture, and diet of the remote, primitive ALS- and PD-affected people are indistinguishable from that of their unaffected neighbors, except for a remarkable deficiency of calcium and magnesium in their soil and drinking water. The distribution of affected and nonaffected villages indicates that communicable infectious or genetic etiology is unlikely. As a result of the isolation and primitive technology, domestic animals (except dogs and pigs) were not found among the Auyu and Jakai, and no manufactured products (including metals, ceramics, textiles, petrochemicals, medicines, food additives, condiments, paints, dyes, or solvents) were available to them.
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PMID:Amyotrophic lateral sclerosis and parkinsonian syndromes in high incidence among the Auyu and Jakai people of West New Guinea. 719 38

In three patients with lesions of peripheral regions of the central nervous system and paralyses of the respiratory muscles (malignant myasthenia, amyotrophic lateral sclerosis and residual manifestations after poliomyelitis), artificial ventilation has been performed continuously for periods from 7 years 4 months to 18 years 9 months using some degree of hyperventilation (PCO2 19.5--26.8 mmHg). The mechanics of breathing were studied by pneumotachography with synchronous recording of transpulmonary and oesophageal pressures using cuffed tubes. The findings were a decrease of total compliance of the respiratory apparatus (Ctot 23--28 ml x cm x H2O-1), a severe decrease in compliance of the lung tissue (CL 24--30 ml x cm x H2O-1), a sharp increase of bronchial resistance during inspiration (RresLI 15.0--23 cm x H2O l-1 s-1), and during expiration (RresLE 16.5--30.7 cm x H2O l-1 s-1), an elevation of compliance of the chest (Cw 358--555 ml x cm x H2O-1), a reduction of non-elastic resistance of the chest (Rres w 0.2--0.6 cm x H2O l-1 s-1). These parameters indicated progressive changes of the structures of the lung tissue and of the tracheobronchial tree which were most pronounced in the case of amyotrophic lateral sclerosis.
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PMID:Mechanics of breathing during prolonged artificial ventilation. 744 8

The non-protein amino acid, beta-N-methyl-amino-L-alanine (L-BMAA) from Cycas circinalis seeds, has been implicated as a causative agent of amyotrophic lateral sclerosis-Parkinsonism dementia complex (ALS-PDC), a disease known from Guam and other areas in the western Pacific. We analyzed C. circinalis seeds for additional free non-protein amino acids by a recently developed GC-MS method. The samples were prepared by water extraction of seed flour. The amino acids present in the extract were derivatized by N(O,S)-isobutyloxycarbonylation of the amine functional groups and then tert-butyldimethylsilylation of the carboxyl functional groups. Peaks for a total of seventeen derivatives of non-protein amino acids were detected by GC-MS. In addition to L-BMAA, four other non-protein amino acids were identified as beta-alanine, gamma-amino-butyric acid, pyroglutamic acid, and alpha-aminoadipic acid.
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PMID:Screening for non-protein amino acids in seeds of the Guam cycad, Cycas circinalis, by an improved GC-MS method. 770 Sep 95

Treatment of dysphagia resulting from bulbar amyotrophic lateral sclerosis has included cricopharyngeal myotomy for many years but is successful in only a minority of cases. The purpose of this study was to explore the rationale for this procedure with modern manometric techniques. The results of pharyngoesophageal manometry in 13 patients with amyotrophic lateral sclerosis were compared with 13 age- and sex-matched healthy volunteers by Mann-Whitney analysis. There was no significant difference between patients and control subjects in distal esophageal or lower esophageal sphincter motility nor any pressure parameter of pharyngoesophageal motility. Separate analysis of the seven significantly dysphagic subjects showed a significantly reduced upper esophageal sphincter after-contraction amplitude during water and bread swallows in patients than in control subjects. These data suggest that the dysphagia of amyotrophic lateral sclerosis is not due to upper esophageal sphincter spasm and that treatment by cricopharyngeal myotomy may be inappropriate.
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PMID:Analysis of the pharyngoesophageal pressure profile in amyotrophic lateral sclerosis. 783 49

Skin sympathetic nerve activity (SSNA) was microneurographically analyzed in five patients with amyotrophic lateral sclerosis (ALS) (four men, one woman; age ranges between 36 and 68 years) and 16 age- and sex-matched controls. The duration of illness after the onset was ranged between 1 and 3 years. Three cases were the common form of ALS showing a moderate to severe involvement of the upper extremities and mild degree of bulbar signs. Two were the pseudopolyneuritic form with lower motor neuron sign predominant in the legs. All these patients could walk by alone and did not show any respiratory distress. SSNA was recorded directly by inserting a tungsten microelectrode into the tibial nerve at the popliteal fossa as described before. The sweat rate and skin blood flow on the area innervated by the tibial nerve were also monitored. Resting activity of the SSNA (bursts/minute) was significantly higher (p < 0.05) in patients with ALS as compared with the controls. The rate of increase in SSNA under the load of mental arithmetic, tone, electric stimulation of the median nerve and immersion of the hand into cold water were only slightly higher in ALS. The rate of increase in SSNA response by these stimuli against the basal SSNA, however, was not significant. These results indicate that skin sympathetic nerve functions are hyperactive in ALS particularly in the basal resting level, though the underlying mechanism is unclear.
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PMID:[Skin sympathetic nerve activity in amyotrophic lateral sclerosis]. 802 34

MR imaging using the conventional spin each technique along with diffusion weighted imaging and water-fat imaging was performed in 16 patients with amyotrophic lateral sclerosis (ALS), 20 normal subjects, and 113 controls with other neurological disorders. Diffusion weighted images in the patients with ALS and the controls disclosed a high signal band from the subcortical area to the medullary pyramids. The high signal band on the diffusion weighted images corresponded to the pyramidal tract in the anatomical atlas described by Talairach. The T1- and T2-relaxation times, proton density, diffusion coefficient and diffusion anisotropy were measured at the points where high signal bands appeared on the diffusion weighted images. The T2-weighted images revealed high signal areas on the posterior limbs of the internal capsules in all the patients with ALS, 60% of the normal subjects, and 73% of the disease controls. The T1-weighted images disclosed high signal areas on the posterior limbs in 62% of the patients with ALS, but not in any of the normal subjects and the disease controls. The proton weighted images disclosed high signal areas on the posterior limbs in all the patients with ALS and 5% of the disease controls, but not in any of the normal subjects. Analysis of diffusion weighted images revealed no significant difference between the patients with ALS and the normal subjects in diffusion coefficient and diffusion anisotropy on the posterior limbs. Measurement of MR parameters (T1- and T2-relaxation times and proton density) showed that the proton density at the posterior limbs increased in ALS. Water-fat images using the method of Dixon revealed abnormal signals in the water images. These signal abnormalities were more prominent in the internal capsule than in the medullary pyramids. Our findings confirm that there is an increase in water molecules that have normal diffusion coefficient and diffusion anisotropy values in patients with ALS.
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PMID:[MR findings of the pyramidal tract in amyotrophic lateral sclerosis]. 826 94

The purpose of this study was to determine the degree of respiratory muscle impairment in patients with newly diagnosed amyotrophic lateral sclerosis (ALS) and the subsequent rate of decline of respiratory function. Thirty-one of 36 patients had respiratory muscle weakness at presentation, although only 7 complained of any respiratory symptoms. Vital capacity (percent predicted) was significantly lower in the symptomatic group (55.9 +/- 20.3) compared with the asymptomatic group (76.4 +/- 21.0). Respiratory muscle impairment as measured by vital capacity (percent predicted) was related to stage of disease at presentation. Rate of decline of respiratory muscle strength as measured by VC (-3.5 percent/month), negative inspiratory pressure (NIF) (+2.9 cm H2O/month), and positive expiratory pressure (PEP) (-3.4 cm H2O/month) tended to be linear with a great deal of interpatient variability. It is concluded that early measurement of respiratory muscle strength in ALS with subsequent follow-up studies may be useful in determining overall prognosis and in decision making.
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PMID:Pulmonary function at diagnosis of amyotrophic lateral sclerosis. Rate of deterioration. 843 45

The paradox of aerobic life, or the 'Oxygen Paradox', is that higher eukaryotic aerobic organisms cannot exist without oxygen, yet oxygen is inherently dangerous to their existence. This 'dark side' of oxygen relates directly to the fact that each oxygen atom has one unpaired electron in its outer valence shell, and molecular oxygen has two unpaired electrons. Thus atomic oxygen is a free radical and molecular oxygen is a (free) bi-radical. Concerted tetravalent reduction of oxygen by the mitochondrial electron-transport chain, to produce water, is considered to be a relatively safe process; however, the univalent reduction of oxygen generates reactive intermediates. The reductive environment of the cellular milieu provides ample opportunities for oxygen to undergo unscheduled univalent reduction. Thus the superoxide anion radical, hydrogen peroxide and the extremely reactive hydroxyl radical are common products of life in an aerobic environment, and these agents appear to be responsible for oxygen toxicity. To survive in such an unfriendly oxygen environment, living organisms generate--or garner from their surroundings--a variety of water- and lipid-soluble antioxidant compounds. Additionally, a series of antioxidant enzymes, whose role is to intercept and inactivate reactive oxygen intermediates, is synthesized by all known aerobic organisms. Although extremely important, the antioxidant enzymes and compounds are not completely effective in preventing oxidative damage. To deal with the damage that does still occur, a series of damage removal/repair enzymes, for proteins, lipids and DNA, is synthesized. Finally, since oxidative stress levels may vary from time to time, organisms are able to adapt to such fluctuating stresses by inducing the synthesis of antioxidant enzymes and damage removal/repair enzymes. In a perfect world the story would end here; unfortunately, biology is seldom so precise. The reality appears to be that, despite the valiant antioxidant and repair mechanisms described above, oxidative damage remains an inescapable outcome of aerobic existence. In recent years oxidative stress has been implicated in a wide variety of degenerative processes, diseases and syndromes, including the following: mutagenesis, cell transformation and cancer; atherosclerosis, arteriosclerosis, heart attacks, strokes and ischaemia/reperfusion injury; chronic inflammatory diseases, such as rheumatoid arthritis, lupus erythematosus and psoriatic arthritis; acute inflammatory problems, such as wound healing; photo-oxidative stresses to the eye, such as cataract; central-nervous-system disorders, such as certain forms of familial amyotrophic lateral sclerosis, certain glutathione peroxidase-linked adolescent seizures, Parkinson's disease and Alzheimer's dementia; and a wide variety of age-related disorders, perhaps even including factors underlying the aging process itself. Some of these oxidation-linked diseases or disorders can be exacerbated, perhaps even initiated, by numerous environmental pro-oxidants and/or pro-oxidant drugs and foods. Alternatively, compounds found in certain foods may be able to significantly bolster biological resistance against oxidants. Currently, great interest centres on the possible protective value of a wide variety of plant-derived antioxidant compounds, particularly those from fruits and vegetables.
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PMID:Oxidative stress: the paradox of aerobic life. 866 Mar 87


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