Showing papers on "Amyotrophic lateral sclerosis published in 1978"
TL;DR: The clinical diagnosis of amyotrophic lateral sclerosis was identified in 668 patients who were followed up for periods up to three years and the spinal form was associated with a threefold better five-year survival than the bulbar form.
Abstract: The clinical diagnosis of amyotrophic lateral sclerosis was identified in 668 patients who were followed up for periods up to three years. This disease was found to be more common in women than previously believed. The five-year survival for all patients was found to be 39.4%, but younger patients had a substantially better prognosis than those in whom the disease was diagnosed when over the age of 50 years. When the mode of onset was considered, the spinal form was associated with a threefold better five-year survival than the bulbar form.
136 citations
TL;DR: The results indicate that reinnervation in motor neur one disease is sufficient to compensate completely for the loss of up to 50% of the motor neurone pool supplying the muscle.
Abstract: Thirty-two patients with motor neurone disease were investigated using quantitative electrophysiological techniques. Estimates of the number of surviving motor units in the extensor digitorum brevis muscle and measurements of the electrophysiological parameters of these units are present along with the values for motor nerve conduction velocities. The results indicate that reinnervation in motor neurone disease is sufficient to compensate completely for the loss of up to 50% of the motor neurone pool supplying the muscle. The capacity for reinnervation is greater than we have found in a number of neuropathies but the efficiency of reinnervation decreases as the number of surviving motor units falls. Reinnervation appears to cease when 5% or less of the motor units remain viable. There is no electrophysiological evidence of a preferential loss of fast conducting axons, of pathological slowing of conduction nor of a dying-back process affecting the motor axon. Comparison of the electrophysiological parameters in progressive muscular atrophy and amyotrophic lateral sclerosis shows no significant differences. The underlying pathophysiological mechanisms are discussed in terms of the results.
123 citations
TL;DR: Patients withAmyotrophic lateral sclerosis have complaints relating to the head and neck (bulbar palsy); thus, the otolaryngologist may be the first physician to see them.
Abstract: Amyotrophic lateral sclerosis is a progressive dengenerative neuromuscular disease of insidious onset. It involves upper and lower motor neurons and causes both spastic and atrophic muscular symptoms. More than one fourth of patients have complaints relating to the head and neck (bulbar palsy); thus, the otolaryngologist may be the first physician to see them. Predominant symptoms are slurred speech, hoarseness, dysphagia, and dyspnea. Muscular weakness, atrophy, and fasciculation are noted on examination. The course is relentless, and only 20% of patients survive five years after diagnosis.
74 citations
01 Mar 1978
TL;DR: A functional staging of patients is proposed which is helpful in categorizing them in reference to the type of rehabilitative techniques required and the kinds of assistive devices needed.
Abstract: Rehabilitation techniques for the management of patients with amyotrophic lateral sclerosis are aimed at maintaining the patients at their optimum functional levels for as long as possible and at preventing complications secondary to disuse of muscles and immobilization. A functional staging of patients is proposed which is helpful in categorizing them in reference to the type of rehabilitative techniques required and the kinds of assistive devices needed.
66 citations
TL;DR: Electromyography (EMG) of anal sphincter muscles was different in patients with amyotrophic lateral sclerosis (ALS) and Shy-Drager syndrome, where there seems to be specific damage of lower motor neurons that innervate the external spHincter muscle of the anus.
Abstract: Electromyography (EMG) of anal sphincter muscles was different in patients with amyotrophic lateral sclerosis (ALS) and Shy-Drager syndrome. In 30 patients with ALS, EMG of the external sphincter muscle was essentially normal, with no signs of denervation. In eight cases of Shy-Drager syndrome, however, motor unit potentials of the anal sphincter had highly polyphasic forms of long duration and high amplitude. In the Shy-Drager syndrome, there seems to be specific damage of lower motor neurons that innervate the external sphincter muscle of the anus.
63 citations
TL;DR: The association appeared disease-related, with onset concomitant with the neurologic expression of Guamanian amyotrophic lateral sclerosis and Parkinsonism-dementia, and there was no association of diminished cellular immunity with HLA-Bw35.
Abstract: To test the hypothesis that host resistance factors may be abnormal in Guamanians in whom amyotrophic lateral sclerosis and Parkinsonism-dementia develop, cellular immunity was evaluated in both diseases and compared to that of Guamanians with other nervous-system diseases, normal adult Guamanians and non-Guamanians with amyotrophic lateral sclerosis and Parkinsonism. Diminished responses to skin-test antigens, lymphopenia, diminished per cent and total T cells and, less frequently, decreased mitogen responses were seen in Guamanian patients with amytorophic lateral sclerosis and Parkinsonism-dementia but not in the other patient or normal groups. Guamanian patients with amyotrophic lateral sclerosis and diminished cellular immunity had an increased frequency of HLA-Bw35 (P less than 0.005) and shorter mean duration of disease (P less than 0.05) than those with normal cellular immunity. In Parkinsonism dementia diminished cellular immunity was less strongly associated with HLA-BW35 (P less than 0.05) and was not associated with differences in duration of disease. Normal Guamanians and those with other nervous-system diseases showed no association of diminished cellular immunity with HLA-Bw35. The association appeared disease-related, with onset concomitant with the neurologic expression of Guamanian amyotrophic lateral sclerosis and Parkinsonism-dementia.
49 citations
45 citations
TL;DR: A significant increase in the level of serine protease was found in muscle of patients with Duchenne-type muscular dystrophy and with Becker-type Muscular Dystrophy, but the activity was not increased in Muscle of a patient with amyotrophic lateral sclerosis.
Abstract: The activities of serine protease in muscles from normal persons and from patients with progressive muscular and neuromuscular diseases have been determined. A significant increase in the level of serine protease was found in muscle of patients with Duchenne-type muscular dystrophy and with Becker-type muscular dystrophy, but the activity was not increased in muscle of a patient with amyotrophic lateral sclerosis.
35 citations
TL;DR: A quantitive study of the terminal innervation ratio (TIR) was conducted using 18 amyotrophic lateral sclerosis and 12 Charcot‐Marie‐Tooth disease muscle biopsies and revealed that CMT has a better capacity for collateral reinnervation than ALS.
Abstract: A quantitive study of the terminal innervation ratio (TIR) was conducted using 18 amyotrophic lateral sclerosis (ALS) and 12 Charcot-Marie-Tooth disease (CMT) muscle biopsies. Morphometric and histochemical analyses of muscle fibers were performed in 9 ALS and 6 CMT biopsies. The results revealed that TIR and type grouping were significantly greater in CMT than in ALS. The proportion of type 3 fibers was higher in ALS, though the proportion of intermediate and type 0 fibers was significantly higher in CMT. The atrophy factor was significantly greater in type 3 than in types 1 and 2 fibers, but it was not significantly different in type 0 and intermediate fibers as compared to types 1 and 2, It appears, therefore, that CMT has a better capacity for collateral reinnervation than ALS. Type 0 and intermediate fibers may represent altered endproducts of successful collateral reinnervation.
35 citations
TL;DR: The concentration of lead in the vastus lateralis muscle was determined in patients with amyotrophic lateral sclerosis and control subjects by flameless atomic absorption spectrophotometry as mentioned in this paper.
Abstract: The concentration of lead in the vastus lateralis muscle was determined in patients with amyotrophic lateral sclerosis and control subjects by flameless atomic absorption spectrophotometry. No statistically significant differences were found between these groups, and in both the figures were of the same magnitude as those earlier reported for normal individuals. Increased plasma lead concentrations do not seem to cause a significant deposition of lead in skeletal muscle. Therefore, plasma rather than skeletal muscle is the more likely source if pathological amounts of lead enter the motoneurones via the retrograde axoplasmic flow in amyotrophic lateral sclerosis.
27 citations
TL;DR: The rate of amyotrophic lateral sclerosis among stateside construction workers on Guam was similar to that of US resident white men, and residence on Guam as an adult did not predispose to a high rate of ALS.
Abstract: The rate of amyotrophic lateral sclerosis (ALS) among stateside construction workers on Guam was similar to that of US resident white men. Of 12,601 men who worked on Guam for one year or more from 1945 to 1954, we were able to follow up 10,049 until 1970 through the Social Security Administration. Of these workers, 1,958 had died, and the cause of death was recorded for all but 335. There were three deaths from ALS. Residence on Guam as an adult did not predispose to a high rate of ALS. ( JAMA 240:551-552, 1978)
TL;DR: The hypothesis is put forward that in patients with ALS there is not only a lesion of the anterior horn, but also an involvement of sympathic structures responsible for the innervation of adipose tissue vessels that may lead to an inhibition of the lipolysis, and consequently to an enlargemnt of the fat cell.
TL;DR: Serological results did not indicate any relationship between these viruses and either amyotrophic lateral sclerosis or multiple sclerosis.
Abstract: • Complement fixation and hemagglutination inhibition tests were conducted on the serums of patients with amyotrophic lateral sclerosis and multiple sclerosis using a variety of arboviral antigens. Seventy-eight complement fixation and 15 hemagglutination-inhibition viral antigens were used representing togaviruses, orbiviruses, rhadoviruses, bunyaviruses, arenaviruses, and several ungrouped agents. The serological results did not indicate any relationship between these viruses and either amyotrophic lateral sclerosis or multiple sclerosis.
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TL;DR: On the average, patients with this form of ALS die within a year, although some patients expire from cardiac failure and cerebrovascular accident, and a significant number succumb to pneumonia due to insufficient ventilation or aspiration.
Abstract: Amyotrophic Lateral Sclerosis Mrs. R. suffers from the progressive bulbar form of ALS [Figure 1). The pathology is centered in the motor nuclei of the medulla, pons, and midbrain. Initial manifestations may be atrophy and fasicular twitchings of the tongue. Fasiculations and atrophy in the lower part of the face and around the lips a re also present. Weakness of the lower mandible makes the jaws difficult to close. Dysarthria, due in part to the involvement of the nucleus of the facial nerve, becomes apparent to others often before the patient notices it. Involvement of the trigeminal, vagus, and hypoglossal nerves produces difficulty in eating and swallowing. The spinal accessory nerve becomes involved in the later course of the disease with atrophy of the u p per parts of the trapezius and sternocleidomastoid muscles. This has been termed the ultimum rnoriens of the disease. On the average, patients with this form of the disease die within a year. Cause of death is usually bulbar paralysis and respiratory failure, although some patients expire from cardiac failure and cerebrovascular accident. A significant number succumb to pneumonia due to insufficient ventilation or aspiration.
TL;DR: Statistical data is presented regarding the identification of viral antigen in the jejunum of patients with multiple sclerosis (MS) and other neurological disease, in particular amyotrophic lateral sclerosis (motor neuron disease).
Abstract: Statistical data is presented regarding the identification of viral antigen in the jejunum of patients with multiple sclerosis (MS) and other neurological disease, in particular amyotrophic lateral sclerosis (motor neuron disease). The viral antigens were identified using fluorescent antibody techniques previously described (1–3). Jejunal specimens were obtained by conventional passage of a small peroral tube with an attached capsule. The position of the capsule was identified radiographically to be in the proximal jejunum. In some instances, particularly in patients with amyotrophic lateral sclerosis (ALS), impairment of swallowing necessitated the use of a fiber-optic jejunoscope.
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TL;DR: A study of the rosetteforming possibilities of T-lymphocytes in the peripheral blood of patients with disseminated sclerosis and lateral amyotrophical sclerosis detected a significant deficit of a subpopulation of T -cells, which form the most active rosettes of the morula type.
Abstract: A study of the rosetteforming possibilities of T-lymphocytes in the peripheral blood of patients with disseminated sclerosis and lateral amyotrophical sclerosis detected a significant deficit of a subpopulation of T-cells, which form the most active rosettes of the morula type. Besides, in patients with disseminated sclerosis the relative amount of rosetteforming lymphocytes in the peripheral blood is significantly lower.
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TL;DR: It was demonstrated that the serum of patients with lateral amyotrophical sclerosis introduced into nourishing medium in a concentration of 25% does not lead to an expressed demyelination of the nervous fibres.
Abstract: In order to study the myelinotoxicity of the blood serum in patients with lateral amyotrophical sclerosis the authors used myelinated explants of the spinal cord of mice embryo. A total amount of 17 blood sera of patients with lateral amyotrophical sclerosis was studied. It was demonstrated that the serum of such patients introduced into nourishing medium in a concentration of 25% does not lead to an expressed demyelination of the nervous fibres. Cultivation of the spinal cord in a medium, containing 5--25% of the patient sera from the moment of explanation does not influence the process of myeline sheath formation. These data indicate that the serum of patients with lateral amyotrophical sclerosis do not possess myelinotoxic and myelininhibiting properties.