Showing papers on "Amyotrophic lateral sclerosis published in 1976"
TL;DR: For example, this paper found that exposure to lead and mercury, participation in athletics, and consumption of large quantities of milk are possible risk factors that may predispose to the development of amyotrophic lateral sclerosis.
Abstract: To identify antecedent events contributing to the development of amyotrophic lateral sclerosis, we studied 25 amyotrophic lateral sclerosis patients in whom we tabulated the incidence of factors previously associated with motor neuron disease and compared the incidences with those found in 25 hospitalized patients and 25 normal people. More amyotrophic lateral sclerosis patients reported exposure to lead and mercury, participation in athletics, and consumption of large quantities of milk. Exposure to lead and mercury, athletic participation, and milk ingestion are possible risk factors that may predispose to the development of amyotrophic lateral sclerosis.
144 citations
01 Sep 1976
TL;DR: It is assumption, based on data as well as on additional clinical observations, that many patients with amyotrophic lateral sclerosis live for longer than 5 years and, rarely, they may have remissions of their illness.
Abstract: Amyotrophic lateral sclerosis is usually considered a disease that will have a fatal termination in 1 to 3 years. A prospective study of 100 patients with this disorder revealed that 20 of them were living 5 years after the onset of their disorder. Review of other published series reveals that patients have been reported who lived for longer than 5 years and have then usually been reported as atypical cases, although the only way in which they are described as atypical is the duration of the disease. It is our assumption, based on these data as well as on additional clinical observations, that many patients with amyotrophic lateral sclerosis live for longer than 5 years and, rarely, they may have remissions of their illness. The possible significance of these observations is discussed.
115 citations
TL;DR: A hypothetical model of the pathogenesis of the disease is advanced and the results are discussed in relation to this model.
56 citations
53 citations
TL;DR: Histocompatibility (HL-A) phenotypes of 44 unrelated white patients from the greater Boston area with amyotrophic lateral sclerosis and 200 white controls were compared, finding that the HL-A antigens may link with disease severity in ALS.
Abstract: • Histocompatibility (HL-A) phenotypes of 44 unrelated white patients from the greater Boston area with amyotrophic lateral sclerosis (ALS) and 200 white controls were compared. In the overall ALS group, an increased frequency of HL-A3 was noted (43% vs 25%, P
41 citations
TL;DR: An epidemiologic study was conducted to seek evidence of a similar viral etiologic agent for ALS in wild mice with clinical and pathologic similarities to ALS and found the endogenous type C RNA tumor virus of these wild mice was established as the etuologic agent of this neurologic disease.
Abstract: To the Editor.— The cause of amyotrophic lateral sclerosis (ALS) is unknown. The unusual geographic distribution of this disease with focuses in Guam and the Kii Peninsula of Japan suggests an environmental trigger, 1 but no consistent hypothesis has yet been established. Recently, we described 2 a motor neuron disease in wild mice ( Mus musculus ) with clinical and pathologic similarities to ALS; the endogenous type C RNA tumor virus of these wild mice was established as the etiologic agent of this neurologic disease. 2,3 We conducted an epidemiologic study to seek evidence of a similar viral etiologic agent for ALS. An ALS registry was established in the Los Angeles area with the help of neurologists and the ALS Foundation who were contacted and asked to report prevalent and all future cases of ALS. From this source, 139 possible cases of ALS were identified. Of these, eight were subsequently excluded as
29 citations
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27 citations
TL;DR: The case seemed unique to any clinicogenetic nosology, because it was associated with a cardiac conduction defect, a mitochondrial abnormality in skeletal muscle, and a course, distribution, and degree of weakness that included features of both chronic spinal muscular atrophy and amyotrophic lateral sclerosis.
Abstract: Six members of a family had a neurologic disease with autosomal dominant transmission and adolescent onset of progressive wasting of predominantly distal axial muscles and muscles innervated by cranial nerves V, VII, X, and XII, leading to severe morbidity in adulthood. Muscle biopsy of the propositus revealed “ragged-red” fibers, while electromyography and autopsy material were consistent with a neurogenic disease. The case seemed unique to any clinicogenetic nosology, because it was associated with a cardiac conduction defect, a mitochondrial abnormality in skeletal muscle, and a course, distribution, and degree of weakness that included features of both chronic spinal muscular atrophy and amyotrophic lateral sclerosis.
26 citations
26 citations
22 citations
TL;DR: The combined incidence of known diabetes in 29 per cent of patients plus a high percentage of glucose tolerance tests interpreted as abnormal was considerably higher than the incidence of abnormal carbohydrate metabolism reported elsewhere in the general population of the United States, the tropical Pacific area, or in recent surveys on Guam.
Abstract: Amyotrophic lateral sclerosis and Parkinsonism-dementia are unusually prevalent on Guam. Carbohydrate metabolism was studied in 110 patients with evidence diagnostic of or suspecious for these diseases. The combined incidence of known diabetes in 29 per cent of them plus a high percentage of glucose tolerance tests interpreted as abnormal, even when most age-related criteria were considered, was considerably higher than the incidence of abnormal carbohydrate metabolism reported elsewhere in the general population of the United States, the tropical Pacific area, or in recent surveys on Guam itself. The diabetes was generally mild in nature and noteworthy for a lack of retinopathy and other complications. Hypertension, hypercholesterolemia, and hyperuricemia, although highly prevalent, were not consistently associated with abnormal glucose metabolism. Similarly, no consistent association was demonstrated with such factors as age, muscle atrophy, or physical activity.
01 Jan 1976
TL;DR: In certain genetically susceptible populations of wild mice a progressive motor neuron disease with a long latent period is caused by indigenous type C leukemia virus, which can be prevented by antiviral genetic means.
Abstract: In certain genetically susceptible populations of wild mice a progressive motor neuron disease with a long latent period is caused by indigenous type C leukemia virus. Neuronal damage appears to be due primarily to a direct neurotropic effect of the virus and not to an immunogenic mechanism. The disease can be prevented by antiviral genetic means. Search for a similar virus in humans with ALS has been negative.
Journal Article•
TL;DR: In none is the pathokinetic mechanism known.
Abstract: The remote effects of cancer on the neuromuscular system include type II muscle fiber atrophy, dermatomyositis/polymyositis, myasthenia gravis, the facilitating myasthenic syndrome, peripheral neuropathy (including amyloid neuropathy), and possibly amyotrophic lateral sclerosis. The clinical and pathological findings and a number of possible pathokinetic mechanisms of these disorders are discussed. In none is the pathokinetic mechanism known. Hence, much work remains in therapeutically oriented research of the mechanisms in all of the remote effects of cancer on the neuromuscular system.
TL;DR: A 32‐year‐old male with typical clinical course of progressive bulbar paresis was investigated histopathologically and the main pathological change was prominent fibrous gliosis in the brain stem and cervical cord without remarkable nerve cell loss.
Abstract: Summary
A 32-year-old male with typical clinical course of progressive bulbar paresis was investigated histopathologically. The main pathological change was prominent fibrous gliosis in the brain stem and cervical cord without remarkable nerve cell loss in the same area. Concerning the pathogenesis of this case, the significance of primary role of fibrous gliosis was discussed.
TL;DR: An aggregation of 14 cases of amyotrophic lateral sclerosis (ALS) was encountered in two families in Minnesota and although the classical clinical features of ALS predominated, some members of one family may not have had typical clinical features.
Abstract: An aggregation of 14 cases of amyotrophic lateral sclerosis (ALS) was encountered in two families in Minnesota. Although the classical clinical features of ALS predominated, some members of one family
01 Jan 1976
Journal Article•
TL;DR: In a retrospective study of the charts of 39 patients who had motor neuron disease, it was found that over 50 percent of patients had radiographic abnormalities of bone and over 20 percent had serum calcium concentrations out of the range observed in normal controls.
Abstract: In a retrospective study of the charts of 39 patients who had motor neuron disease we found that over 50 percent of patients had radiographic abnormalities of bone and over 20 percent had serum calcium concentrations out of the range observed in normal controls. Statistical analysis indicated that, in respect to serum calcium levels, patients who have motor neuron disease are a separate population. These findings, which might reflect a disturbance in bone or calcium metabolism in patients with motor neuron disease, are of interest in view of the known ability of divalent ions other than calcium, such as lead and mercury, to simulate motor neuron disease, and the discovery that patients with proven primary and secondary hyperparathyroidism may have features of amyotrophic lateral sclerosis.
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TL;DR: Twenty-five cases of amyotrophic lateral sclerosis occurring before the age of 40, collected between 1963 and 1973 in the Neurological Hospital, Lyons, have prompted the authors to make the following observations.
Journal Article•
TL;DR: The authors present two clinical observations of amyotrophic lateral sclerosis in which a loss of voluntary control was associated with retention of automatic and reflex activity of muscles innervated by cranial nerves.
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Journal Article•
01 Jan 1976
TL;DR: The administration of phthalazinol, a potent cyclic AMP phosphodiesterase inhibitor, was started under the open study and the observation has been continued every week, mainly in the out patients department, except 2 hospitalized patients.
TL;DR: Comment on the article by Smith and Norris on "Symptomatic Care of Patients with Amyotrophic Lateral Sclerosis", which suggests good results may be obtained with trihexyphenidyl hydrochloride (Artane), 2 to 4 mg three times a day, commonly used in the management of the Parkinson syndrome.
Abstract: To the Editor.— I would like to comment on the article by Smith and Norris on "Symptomatic Care of Patients with Amyotrophic Lateral Sclerosis" (234:715, 1975) and add some personal observations about this challenging problem. In most forms of amyotrophic lateral sclerosis (ALS), bulbar involvement will produce swallowing abnormalities with consequent sialorrhea. I agree with the authors that anticholinergics regularly used for gastrointestinal disorders are usually ineffective in controlling drooling in these patients, but interestingly enough, good results may be obtained with trihexyphenidyl hydrochloride (Artane), 2 to 4 mg three times a day. This anticholinergic, commonly used in the management of the Parkinson syndrome, is well tolerated by ALS patients and does not produce the undesirable sedative effects of the amitriptyline hydrochloride (Elavil) recommended by the authors. In our experience, treatment of drooling with trihexyphenidyl has eliminated the need for drastic procedures such as irradiation of the salivary glands. The