Showing papers on "Amyotrophic lateral sclerosis published in 1982"

Intraneuronal aluminum accumulation in amyotrophic lateral sclerosis and Parkinsonism-dementia of Guam.

Abstract: Scanning electron microscopy with energy-dispersive x-ray spectrometry was used to analyze the elemental content of neurofibrillary tangle (NFT)-bearing and NFT-free neurons within the Sommer's sector (H1 region) of the hippocampus in Guamanian Chamorros with amyotrophic lateral sclerosis and parkinsonism-dementia and in neurologically normal controls. Preliminary data indicate prominent accumulation of aluminum within the nuclear region and perikaryal cytoplasm of NFT-bearing hippocampal neurons, regardless of the underlying neurological diagnosis. These findings further extend the association between intraneuronal aluminum and NFT formation and support the hypothesis that environmental factors are related to the neurodegenerative changes seen in the Chamorro population.

Amyotrophic lateral sclerosis and parkinsonian syndromes in high incidence among the Auyu and Jakai people of West New Guinea

Abstract: 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.

The Onuf's nucleus and the external anal sphincter muscles in amyotrophic lateral sclerosis and Shy-Drager syndrome.

Abstract: The anterior sacral horns and external anal sphincter muscle were examined histologically with special reference to Onuf's nucleus in amyotrophic lateral sclerosis (ALS) and Shy-Drager syndrome. Onuf's nucleus and the external anal sphincter muscles were well preserved in ALS, but in Shy-Drager syndrome both structures showed marked degenerative changes. These findings suggest that Onuf's nucleus does not belong to the autonomic neurons, but to the motoneurons in anterior sacral horn, and it innervates the external sphincter muscles of the anus and urethra.

Eye movements in amyotrophic lateral sclerosis.

Abstract: • Amyotrophic lateral sclerosis (ALS) is characterized by degeneration of the upper motor neurons in the frontal cortex and the motor neurons of the brain stem and spinal cord. Cranial nerve nuclei III, IV, and VI are usually normal histopathologically, as are the eye movements clinically. Using electro-oculography, we found decreased saccadic or smooth pursuit velocities in four of ten patients, unidirectional saccadic pursuit in one of these four, and progressively decreasing saccadic velocities in another. We believe the defect is supranuclear and that the ocular motor system is more often abnormal in patients with ALS than previously recognized.

A New Hypothesis of the Etiology of Amyotrophic Lateral Sclerosis: The DNA Hypothesis

Abstract: • Evidence is accumulating that a number of previously unexplained human diseases may arise from a deficiency of DNA repair enzymes. Studies on the motoneurons of patients with amyotrophic lateral sclerosis (ALS), and those of an animal model of motoneuronal degeneration, the wobbler mouse, indicate the presence of major abnormalities of RNA metabolism. We advance the hypothesis that the primary abnormality in ALS is the accumulation of abnormal DNA, which is unable to undertake normal transcription, in motoneurons. This abnormal DNA may arise from a deficiency of an isozyme of one of the DNA repair enzymes.

Classic Amyotrophic Lateral Sclerosis With Dementia

Abstract: • Documented cases of classic amyotrophic lateral sclerosis (ALS) combined with severe dementia are few and come from the isolated populations of the Western Pacific. In this report, three women had a clinically and neuropathologically documented combination of ALS and dementia. In all cases the symptoms and signs of ALS were mainly bulbar. In two of them dementia appeared first, followed by ALS. At autopsy, there was a marked discrepancy between the severe degree of dementia of Alzheimer's type and the slight nonspecific neuropathologic findings without Alzheimer's changes. Our cases bear a close resemblance to recently published Japanese cases. It may be concluded that the combination of sporadic ALS and progressive dementia seems to be a clinical entity without consistent neuropathologic changes and that it also occurs in the western countries.

Regular involvement of Clarke's nucleus in sporadic amyotrophic lateral sclerosis.

Abstract: • Morphometric study of Clarke's nucleus in 12 cases of sporadic amyotrophic lateral sclerosis and 12 normal control subjects showed an average 36% reduction in the neuronal population in the disease group, with involvement in each case. Histopathologic findings of cell degeneration were definite but less noticeable than in the ventral horns. The process seems likely to be similar to that affecting motor neurons but is less advanced at the time of death.

Neuron specific in vitro cytotoxicity of sera from patients with amyotrophic lateral sclerosis.

Abstract: Sera from patients with amyotrophic lateral sclerosis (ALS) were assayed for a neuron-specific cytotoxic effect on long-term organized cultures of neonatal mouse anterior horn segments. Blind studies show that ALS sera when incorporated into the culture media have a greater degree of antineuronal toxicity than sera from patients with other neurological diseases or from family members of ALS patients.

Immunological profile of amyotrophic lateral sclerosis patients and their cell-mediated immune responses to viral and CNS antigens

Abstract: The `immunological profile' of amyotrophic lateral sclerosis (ALS) patients was established from standard tests for B- and T-cell function. This showed no significant difference from age and sex-matched other neurological (CNS) disease controls and normal subjects. Immune complex (IC) levels in ALS serum differed significantly from normal controls but not from CNS controls. There was no relation between the various indices of immune activity of IC levels and the clinical disability of the ALS patient or progression of the disease. Distribution of complement-fixing antibodies to poliovirus was similar in sera of ALS and control groups. The in vitro cell-mediated immune responses to poliovirus, however, were significantly greater in ALS patients than in CNS controls and were inversely related to the ALS disability score. Poliovirus has not been demonstrated in the CNS or extra-CNS tissues of ALS patients by conventional means but, if latent or defective poliovirus or related virus were present, this could account for sensitization and a possible autoimmune mechanism. ALS patients exhibited in vitro cellular immunity to ALS and normal CNS subfractions. These responses were not related to the ALS disability score or progression of the disease and probably represent epiphenomena.

Amyotrophic lateral sclerosis and paraproteinemia.

Abstract: A patient with ALS had an IgG kappa monoclonal gammopathy. This raises the possibility that monoclonal paraproteins may sometimes play a role in the etiology of motor neuron disease.

Virological studies in amyotrophic lateral sclerosis

Abstract: Complement-fixing antibody response to eleven different viruses were measured in amyotrophic lateral sclerosis (ALS) patients, contacts of ALS patients, neurological controls, and normal controls. The normal controls showed a decreased response to adeno-associated virus and an increased response to adenovirus when compared to the other groups. Levels of interferon-like substances also were investigated in sera and cerebrospinal fluids of ALS patients and neurological controls. Responses were of a low titer and were not increased in the ALS group. Explant cultures were established from tissues of 24 ALS autopsy cases. Cultures were investigated directly or following fusion to various indicator cell lines for viral-like agents. Techniques such as interference assays, 5-bromodeoxyuridine (BudR) induction, hemadsorption, and fluorescent antibody staining failed to detect virus. By addition of helper adenovirus to primary explant cultures, adeno-associated virus was isolated from 2 of 11 ALS cases.

Immunology of amyotrophic lateral sclerosis.

Abstract: ConclusionThe antibody detected in the ALS sera with suppressive activity for terminal sprouting is likely directed against an antigen that is normally sequestered. Tolerance to the 56 kd antigen described here is not exhibited in rats, as syngenic immunizations induce an immune response (M. E. Gurney, unpublished observation). Freshly denervated muscle secretes very low amounts of the 56 kd antigen, and the level of secretion rises 2–3 days after denervation in vivo. Of the few antecedent events associated with ALS by case studies, mechanical injuries, participation in physical activities, or bone fractures might all produce nerve damage or periods of limb immobilization [48]. Similar physical insults are stimuli for sprouting in animal models [13, 39], and might result in elevated secretion of sprouting factor, resulting in induction of an immune response in susceptible individuals.The antibodies detected in this study could arise as a secondary response to neuromuscular destruction, as occurs following tissue injury in many organs. The absence of inhibitory activity in sera of patients with neuropathy suggests that denervation per se need not give rise to development of such antibodies, although the extent of denervation and muscle atrophy in the ALS patients was, in general, markedly greater than in patients with neuropathy. Whether sprouting inhibitory antibodies prove to be of primary pathogenic significance, or are only a secondary phenomenon, they provide potentially significant reagents with which to elucidate the existence and nature of human motor neuron growth factors.

Response of Patients With Amyotrophic Lateral Sclerosis to Testosterone Therapy: Endocrine Evaluation

Abstract: Four men with amyotrophic lateral sclerosis (ALS) were treated with 200 mg of intramuscular testosterone weekly. Endocrine evaluation, using a gonadotropin-releasing hormone infusion test, indicated the expected degree of suppression of pituitary luteinizing hormone and follicle-stimulating hormone production. These data suggest that testosterone's (androgen) interaction with its receptors in the hypothalamic-pituitary axis is normal in patients with ALS.

Lecithin is not effective in amyotrophic lateral sclerosis

Abstract: Eleven patients with motor neuron disease (9 with amyotrophic lateral sclerosis, one with spinal muscular atrophy, and one with postpolio motor neuron disease) were treated with lecithin (55% phosphatidylcholine, 20 gm per day) for 3 to 12½ months. Response was measured by quantitated muscle testing and functional evaluations of patients and untreated, matched controls. Lecithin therapy did not provide therapeutic benefit.

Motor neuron(e) disease.

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Skeletal muscle catabolism in amyotrophic lateral sclerosis and chronic spinal muscular atrophy

Abstract: ALS and chronic spinal muscular atrophy are characterized by wasting of skeletal muscle, suggesting accelerated catabolism or reduced synthesis of muscle protein. We studied seven patients with ALS and three with chronic spinal muscular atrophy using 24-hour urinary 3-methylhistidine excretion as a measure of the rate of muscle catabolism and 24-hour urinary creatinine excretion as an index of significantly and similarly higher in both groups of patients than in controls (p less than 0.0005), implying a state of accelerated skeletal muscle protein catabolism in these diseases.

Thyroid function in 44 patients with amyotrophic lateral sclerosis.

Abstract: In an endemic goiter area of West Germany (incidence of goiter, 19% to 32%), thyroid function was evaluated in 44 patients with amyotrophic lateral sclerosis (ALS) with radioimmunoassay of triiodothyronine, thyroxine, thyroxine-binding globulin, and thyroid-stimulating hormone, including the protirelin stimulation test. The presence of circulating anti-thyroid antibodies was sought by hemagglutination techniques. All patients were found to be euthyroid and thyroid antibody-negative. Clinical examination disclosed concomitant goiter in 36% (16/44). The data suggest that thyroid status is not relevant to the pathogenesis of ALS.

HLA frequencies in amyotrophic lateral sclerosis.

Abstract: • Our study of the distribution of HLA-A, -B, and -C in patients with amyotrophic lateral sclerosis (ALS) found no statistically significant deviation. We found a trend, however, toward a decrease in HLA-A9, as we had reported previously, and toward an increase in HLA-Bw35 and -Cw4. The worldwide incidence of ALS is uniform except in Guam and on Japan's Kii peninsula, and published reports from many countries, including Guam, show no consistent deviation in HLA frequencies related to ALS.