Showing papers on "Amyotrophic lateral sclerosis published in 1979"

Spirometry in amyotrophic lateral sclerosis

Abstract: • Clinical evaluation and pulmonary function tests were performed in 218 patients with motor neuron disease, mainly amyotrophic lateral sclerosis (ALS). Serial studies were obtained in 103 patients, in 31 until death from ALS. Most patients, regardless of the pattern of motor neuron involvement, had characteristic abnormalities in pulmonary function, including reduced forced vital capacity (FVC) and maximum voluntary ventilation (MVV). Reductions in the FVC and MVV to as low as 50% were commonly missed by clinical evaluators. Spirometry is therefore of value in detecting early involvement of respiratory neurons. Progressively greater reductions in the FVC and MVV in all the fatal cases indicate that serial spirometry has prognostic value in ALS.

Neurofibrillary degeneration on Guam: frequency in Chamorros and non Chamorros with no known neurological disease.

Abstract: In a neuropathological study of the brains of 69 members of the Guamanian Chamorro population without known evidence of dementia, parkinsonism or amyotrophic lateral sclerosis, the incidence and severity of neurofibrillary degeneration was determined in relation to age at death. It was found that neurofibrillary degeneration is present at an earlier age than has been reported for a comparable population in Japan, and much earlier than reported for two similarly comparable English populations. Our data suggests that neurofibrillary degeneration is the common denominator of the Guam neurological syndromes of parkinsonian dementia, amyotrophic lateral sclerosis and dementia without parkinsonism. This conclusion would imply that the aetiological factor of neurofibrillary degeneration is more widely dispersed in the population than suggested by the cases of parkinsonian dementia and amyotrophic lateral sclerosis alone. Both sexes were equally affected while clinical parkinsonian dementia is three times more frequent among males. The sample data suggests a positive association with the amyotrophic lateral sclerosis-parkinsonian dementia rate in village of birth and residence, and also with family history; the results in the small subsamples are not significant. Senile plaques were found in few control cases in this study, just as there has been a relative absence of senile plaques in cases of parkinsonian dementia. In a small group of Caucasians who had spent many of their adult years on Guam, the frequency of neurofibrillary degeneration in relationship to age was comparable to that in the English populations.

Amyotrophic Lateral Sclerosis: Impairment of Neuromuscular Transmission

Abstract: • Neuromuscular transmission was studied in the ulnar-hypothenar group in 55 patients with amyotrophic lateral sclerosis. A decremental response was found in 67.0%. The decrement was larger and present more often in muscles showing atrophy. In addition, muscles with frequent fasciculations showed a larger decrement than the ones with rare fasciculation. A temperature effect similar to that in myasthenia gravis was observed, with a reduction of the decrement following local cooling of the muscles. Administration of edrophonium chloride improved the synaptic defect. Posttetanic exhaustion was observed as well. It is thought that the defect of neuromuscular transmission is due to a decreased trophic function of the neuron followed by morphological changes at the endplate.

Hereditary canine spinal muscular atrophy.

Abstract: Hereditary canine spinal muscular atrophy is a newly recognized motor neuron disease occurring in Brittany Spaniels. The clinical manifestations, pattern of inheritance, electrodiagnostic findings, and muscle biopsies have features in common with human spinal muscular atrophy. Neuropathological examination discloses some loss of motor neurons in the spinal cord and brainstem. Many of the surviving motor neurons have neurofibrillary swellings in proximal axons, an abnormality similar to that which occurs early in the course of human amyotrophic lateral sclerosis. These axonal swellings are filled with maloriented skeins of neurofilaments. Since the proteins comprising neurofilaments are carried by slow axonal transport, their accumulation within axons suggest that the swellings may result from impaired slow transport, a hypothesis that can be tested in affected Brittany Spaniels. Hereditary canine spinal muscular atrophy is a new genetic, clinical, and pathological entity, and, at present, it appears to be the best currently available animal model of motor neuron disease.

Amyotrophic Lateral Sclerosis With Ophthalmoplegia: A Clinicopathologic Study

Abstract: • Ophthalmoplegia is rarely observed in patients with amyotrophic lateral sclerosis (ALS). We describe a patient with ALS in whom ophthalmoplegia, initially appearing midway in her course, progressed to total paralysis of extraocular movements by the time of death. In addition to the usual postmortem findings of ALS in the brain stem and spinal cord, there was extensive neuronal loss and gliosis involving the caudal portions of the dorsal and intermediate components of the oculomotor nuclei, the caudal part of the trochlear nuclei, and the abducens nuclei. This represents the first detailed report of the findings in the nuclei of cranial nerves III, IV, and VI in a patient with ALS and ophthalmoplegia.

The origin of fasciculations in motoneuron disease.

Abstract: The collision technique was used to determine the origin of distal fasciculations in 25 motor units from 9 patients with amyotrophic lateral sclerosis and 6 with other diseases involving motoneurons. Fasciculations originated in the nerve proximal to the knee or elbow in 15 and distally in 2; multiple sites of origin were found in 8 motor units. The origin was not related to the presence or absence of electromyographic evidence for collateral sprouting or lesions in descending suprasegmental pathways.

Trial of baclofen in amyotrophic lateral sclerosis.

Abstract: • A trial of the antispastic drug baclofen was made in amyotrophic lateral sclerosis. Some patients noted reduction of tonus and spasticity; but others had no benefit, nor was the course of the disease altered by long-term administration of baclofen. No major side effects occurred.

Cell-mediated immunity to polio and HLA antigens in amyotrophic lateral sclerosis.

Abstract: Cell-mediated immunity to poliovirus was demonstrated in 21 of 33 patients suffering from amyotrophic lateral sclerosis (ALS), whereas no response to poliovirus was found in patients suffering from other neurologic disorders or in healthy controls. Three of the severe bulbar cases produced a migration inhibition factor (MIF) in the presence of poliovirus, although skin tests to common antigens were negative. An increased incidence (46 percent) of HLA-A3 was found in patients with amyotrophic lateral sclerosis. Nine of the 13 patients with HLA-A3 antigen also had a positive index of MIF to poliovirus. These findings suggest a strong linkage between HLA-A3 and poliovirus in the pathogenesis of amyotrophic lateral sclerosis.

Free amino acids in motor cortex of amyotrophic lateral sclerosis.

Abstract: Free amino acids were estimated quantitatively in the motor cortex from 3 patients with amyotrophic lateral sclerosis (ALS) and 11 control subjects. Among 7 amino acids which showed statistically significant changes, taurine was the only one which was increased constantly and most markedly in the motor cortex of all the 3 ALS cases. It was suggested that the metabolism of sulfur amino acids might be affected in comparatively early stages of ALS.

Plasma exchange in motor neuron disease. A controlled study

Abstract: In vitro studies seem to indicate that a serum factor may be involved in the pathogenesis of motor neuron disease. If so, plasmaphoresis might influence the course of amyotrophic lateral sclerosis (ALS) favorably. In the present study, therefore, ALS patients were subjected to weekly 21 plasma exchanges, using a Haemonetics blood separator. Seven other ALS patients, matched as closely as possible with the treatment group regarding age, sex, duration of symptoms as well as degree of involvement, served as control group. The progression of the disease was followed by an arbitrary grading system, assessment of muscular power by Zadig's dynamometer, and by tests for motor speed, coordination and for pulmonary function. Duration of treatment was from 6 to 15 months. Monthly evaluations indicated that the rate of deterioration was approximately the same in treatment and control groups. Plasmaphoresis carried out in this way does thus not alter the downhill course of ALS.

Transfer factor is ineffective in amyotrophic lateral sclerosis

Abstract: Sixty-six patients with amyotrophic lateral sclerosis (ALS) participated in a double-blind, placebo-controlled study of transfer factor. A structured rating score of neurological function was recorded after each monthly administration of transfer factor or placebo. Statistical analysis of mean scores for all patients and for those with specific clinical abnormalities showed no significant difference between the two groups. There was no evidence of therapeutic value for transfer factor. During the observation period of nearly one year, more than two-thirds of the deterioration occurred in the first six months. Meaningful clinical trials of other potential therapeutic agents could probably be conducted within six months in double-blind crossover trials.

CSF viral antibodies. Evaluation in amyotrophic lateral sclerosis and late-onset postpoliomyelitis progressive muscular atrophy.

Abstract: • Serum and CSF from 48 patients with amyotrophic lateral sclerosis and six patients with late-onset postpoliomyelitis progressive muscular atrophy were investigated for the presence of antibody to poliovirus types 1, 2, and 3, coxsackie viruses B3 and B4, influenza A, measles, rubella, mumps, herpes simplex types 1 and 2, cytomegalovirus, varicella-zoster, and Toxoplasma gondii . These results were compared with those from 53 control patients with neuromuscular disease matched for age, sex, race, and poliovirus vaccine exposure. There was no difference either in the distribution of serum or CSF antibody titers or the geometricmean antibody titers. There was no evidence suggesting the presence of locally produced specific viral antibody within the CNS to any of the agents studied. In particular, there was no serological evidence to suggest an association between persistent infection with any poliovirus type and amyotrophic lateral sclerosis or late-onset postpoliomyelitis progressive muscular atrophy.

The effects of partial chronic denervation on forearm metabolism.

Abstract: Effects of chronic denervation upon in vivo forearm metabolism were studied in six patients and six controls. The diagnosis was amyotrophic lateral sclerosis in four patients, the neuronal form of Charcot-Marie Tooth disease in one patient, and an unclassified chronic disease of the lower motor neurons in one patient. In all cases the forearm muscles showed clinical weakness and electrical evidence of denervation, while muscle biopsy from a proximal muscle of the upper limb showed typical denervation atrophy. At rest there was increased oxygen utilization and lactate output as well as a tendency for increased uptake of glucose and long chain fatty acids from arterial blood per 100 ml of forearm tissue. During exercise the abnormally high lactate output increased further. An increased arterial lactate concentration was present during rest and exercise. Oxidation of fatty acids was not impaired. It is suggested that these abnormalities are consistent with an augmented utilization of blood borne fuels at rest by denervated muscles. A concurrent regional ischemia of muscles during rest and exercise, possibly due to defective autoregulation of skeletal muscle blood flow, may explain the abnormally high lactate generation.

Endocrinologic Regulation of Carbohydrate Metabolism: Amyotrophic Lateral Sclerosis and Parkinsonism-Dementia on Guam

Abstract: • Studies of the endocrinologic control of carbohydrate metabolism were conducted in Guamanians with parkinsonism-dementia (PD) or amyotrophic lateral sclerosis (ALS) and in Guamanian control patients who had various other neuromuscular disorders. Intravenously infused arginine tended to produce a more prolonged elevation in serum glucose levels in PD and ALS patients than in control subjects. On the other hand, the serum insulin response to arginine was significantly less in both PD and ALS patients than in controls. Arginine stimulated the release of growth hormone to a similar degree in all three patient groups. These observations support and extend previous reports of endocrinologic abnormalities in parkinsonism and ALS and might suggest that a defect in pancreatic islet cell function attends these disorders.

Amyotrophic lateral sclerosis with temporal lobe atrophy.

Abstract: Clinical and neuropathological findings are reported on a 48-year-old man who developed progressive bulbar palsy, muscle atrophy of four extremities, and hyperreflexia. Duration of the illness was about 19 months. Moderate personality changes were observed during his hospitalization. Neuropathological examination revealed the presence of an ALS: severe loss of the large motoneurons in the spinal anterior horns, and degeneration of the corticospinal tract, more prominent on the left side. The hypoglossal, the facial, and the motor trigeminal nuclei were also involved, but the oculomotor, the trochlear and the abducens nuclei were well preserved. There was bilateral, but more pronounced on the right, atrophy of the temporal poles consistent with the lesions of the temporal types of Pick's disease. This case indicates the simultaneous occurrence of degenerative diseases of the CNS, and the correspondence of laterality between the temporal lobe and the spinal cord may suggest a common etiology of these two types of disease processes.

Attempts to reproduce amyotrophic lateral sclerosis in laboratory animals by inoculation of Schu virus isolated from a patient with apparent amyotrophic lateral sclerosis.

Abstract: A virus isolated from the CSF of a patient who had amyotrophic lateral sclerosis for 7 years, and prolonged pleocytosis in the CSF, was adapted to suckling mouse brain by subsequent serial blind passages. This Schu virus belongs to the tick-borne encephalitis complex of the genus Flavivirus (Togaviridae). Suckling mouse brain homogenate of the 13th passage was used for transmission experiments in various species of laboratory animals. Golden hamsters infected subcutaneously fell ill after a number of months, lost weight, and had paresis of the legs. Histologically they had petechial hemorrhages in different parts of the CNS and inflammatory changes in the gray substance of the spinal cord. Pilot studies with repeated inoculations of small doses of different flavivirus strains suggest a course of the disease in experimental animals which resembles slow-virus infections insofar as no encephalitis is produced and degenerative changes of the anterior horn cells prevail over inflammatory signs in the spinal cord. After intracerebral application of Schu virus, cynomolgus monkeys developed the typical lesions of togavirus panencephalitis with epileptic seizures, ataxia, and paresis. After subcutaneous application, the virus seems to spread along peripheral nerves to anterior spinal roots and spinal cord, where mainly motor neurons of the anterior horn are damaged, and from there to the brain. The histological findings are such that one may assume the disease of the patient was due to the infection with the virus isolated from his CSF. Therefore, the hypothesis may be advanced that at least some of the cases diagnosed as amyotrophic lateral sclerosis are due to a togavirus infection.

Does gastrectomy predispose to amyotrophic lateral sclerosis

Abstract: • Some authors have suggested that amyotrophic lateral sclerosis (ALS) may be precipitated by a "deficiency factor" following gastrectomy. Methods to establish a statistical association of the disease with gastrectomy include a case-control study. Two such studies have been made in Japan, one involving 712 and the other 158 cases of the disease. No statistically significant association between gastrectomy and the subsequent occurrence of ALS was found, indicating it is unlikely that the operation predisposes to ALS. None of many other factors studied except mechanical injury showed a statistically significant association in either study.

Cytotoxic effect of the serum of amyotrophic lateral sclerosis patients on spinal cord motoneurons in tissue culture

Abstract: Organotypical cultures of the spinal cord of the fetal mouse were used to study the neurocytotoxic effect of sera from patients with amyotrophic lateral sclerosis (ALS). Sera from 16 patients with ALS were used in two experiments: 1. short-term treatment (up to 6 days) of the mature cultures with nutrient medium containing 30% of ALS serum; 2. long-term cultivation from the explantation up to 4 weeks of spinal cord in the medium containing 5-25% of ALS serum. No specific neurotoxic effect of ALS sera on the motor neurons of the cultured spinal cord were observed in acute or chronic experiments.

[HLA and amyotrophic lateral sclerosis (author's transl)].

Abstract: 52 patients with amyotrophic lateral sclerosis and 300 normal subjects were tested for 33 HLA-A, B, C antigens. We did not observe any statistically significant difference in antigenic frequencies between the two groups. The absence of association with HLA in amyotrophic lateral sclerosis is a distinction with multiple sclerosis, which is associated with several HLA antigens.

Increased plasma lead levels in patients with amyotrophic lateral sclerosis

Abstract: subject spread over more than a century is in itself a daunting task. Hecaen has chosen well, keeping a scrupulously fair balance between papers of differing points of view. Space does not permit a closer examination of the individual contributions. Suffice it to say that they are by leading figures in the subject such as Broca, Jackson, Babinski, Milner, Kimura, Gazzaniga, Sperry, and Hecaen. The papers are arranged in three main sections dealing with (a) the discovery and development of the concept of cerebral dominance (10 papers), (b) functional hemispheric asymmetry (16 papers), and (c) cerebral organisation in lefthanders (four papers). Each paper is preceded by a linking and summarising commentary. Both in terms of number of papers and subject matter, section (b) is by far the most important -in fact one might ask whether the attempt to provide a comprehensive historical point of view at the same time as presenting some of the compelling issues in current research has meant that some of the more recent literature has had to be omitted purely on grounds of space. In his concluding remarks, Hecaen indicates some of the directions that future research on cerebral dominance might now profitably take, given the existence of the necessary research techniques-for example, what precise role do subcortical structures play in lateralisation, is dominance exclusively a matter of genetic predetermination (an old question in itself), is dominance specific to Homo sapiens, and so on? For the French reader, this book will provide an invaluable survey of the subject. The English reader will probably wonder-and with justificationwhy a comparable English anthology on cerebral dominance has not yet appeared.