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Showing papers on "Amyotrophic lateral sclerosis published in 1969"


Journal ArticleDOI
TL;DR: Light and electron microscopic observations from an unusual sporadic case of motor neuron disease characterized by neurofibrillary tangles have been observed in the anterior horn cells.
Abstract: IN THE ADULT, motor neuron disease or amyotrophic lateral sclerosis can be subdivided into several groups on the basis of clinical, pathologic, and epidemiologic features. 1 The histologic changes observed in the classic sporadic cases include nonspecific degeneration and loss of motor neurons in the spinal cord, brain stem, and cerebral cortex. Demyelination of the pyramidal tracts often accompanies the neuronal losses. 2 A familial variant further characterized by demyelination of the posterior columns and spinocerebellar tracts has been reported. 3 In a few residual motor neurons from these cases, Hirano et al 4 described accumulations of hyalinized material, occasionally with acidophilic cores that resemble Lewy bodies. In still another variant, the amyotrophic lateral sclerosis and Parkinsonism-dementia complex on Guam, neurofibrillary tangles have been observed in the anterior horn cells. 5 This report presents light and electron microscopic observations from an unusual sporadic case of motor neuron disease characterized by

112 citations


Journal ArticleDOI
TL;DR: The clinical and histopathological findings in a patient affected by a hereditary form of amyotrophic lateral sclerosis (ALS) are reported and a clinical description of 11 other members of the same family are included.

46 citations



Journal ArticleDOI
TL;DR: Cineradiography is of strategic value in distinguishing the particular mechanisms of motor disability, whether paralysis, contracture, or atrophy, and in the identification of motor compensations, and should be employed consistently in the evaluation and care of the ALS patient.
Abstract: During the clinical progression of amyotrophic lateral sclerosis (ALS) various disorders of the motor effector mechanisms of the pharynx may develop, impairing its functions of airway maintenance, swallow, or speech. Among ALS patients the disorders vary in distribution and sequence, but potentially affect each of these functions. Cineradiography is of strategic value in distinguishing the particular mechanisms of motor disability, whether paralysis, contracture, or atrophy, and also in the identification of motor compensations (6, 9). It is useful in demonstrating severity of disabilities and their potentially critical elements, as failure of laryngeal occlusion in swallowing. This information is essential for therapy, for facilitation of care and comfort, and at times for survival. Cineradiography, now generally available in medical centers, should be employed consistently in the evaluation and care of the ALS patient. Procedures Observations on 6 ALS subjects were selected from data of continuing studi...

33 citations


Journal ArticleDOI
TL;DR: The present study was undertaken primarily to determine the frequency of both ALS and PD in this enlarging population of Guamanians, and comparison is made with the nonmigrant population on Guam.

27 citations



Journal ArticleDOI
14 Jul 1969-JAMA
TL;DR: The experience with such a treatment program given to patients with ALS who have participated in an ongoing series of drug trials over the past three years demonstrates the merit of serial quantitative muscle testing (QMT) to evaluate the course of the patients.
Abstract: It has been reported that patients with amyotrophic lateral sclerosis (ALS) are deficient in exocrine secretion of the pancreas, and that treatment of ALS with large dosages of pancreatic extract and DL-alpha tocopherol (vitamin E) given with a diet high in protein and low in fat and carbohydrate beneficially modifies the course of the disease.1-3This brief report summarizes our experience with such a treatment program given to patients with ALS who have participated in an ongoing series of drug trials over the past three years and demonstrates the merit of serial quantitative muscle testing (QMT) to evaluate the course of the patients. Methods The diagnosis of ALS was based upon the following clinical observations, which are applicable to these patients but not intended to be general criteria for diagnosing every case of ALS: (1) onset during middle-age or later of a sub-acutely progressive disorder characterized by (2) "anterior

10 citations



Journal ArticleDOI
TL;DR: The present study resolved a discrepancy in the occurrence of amyotrophic lateral sclerosis in patients who had undergone previous gastrectomy and claimed that clinical improvement resulted from treatment with pancreatic extract and vitamin E.
Abstract: THE ETIOLOGY of amyotrophic lateral sclerosis is unknown.' Ask Upmark2 noted its occurrence in five patients after gastric resection. However, Stiel did not find any patient who had undergone previous gastrectomy in his series of 125 patients with motor neuron disease.3 Quick and Greer have reported abnormal exocrine and endocrine pancreatic function in amyotrophic lateral sclerosis.\".\" Furthermore, they claimed that clinical improvement resulted from treatment with pancreatic extract and vitamin E. Verbal discussion has indicated that other workers have failed to reproduce these results.6.7 The present study was undertaken to resolve this discrepancy.

8 citations




Journal ArticleDOI
07 Jul 1969-JAMA
TL;DR: In the Jan 13, 1969 issue ofThe Journala MEDICAL NEWS report (p 249) appeared entitled, "Drug May Arrest or Slow ALS," in which it is claimed that amitriptyline (Elavil) hydrochloride beneficially modifies the course of amyotrophic lateral sclerosis (ALS) as mentioned in this paper.
Abstract: To the Editor:— In the Jan 13, 1969, issue ofThe Journala MEDICAL NEWS report (p 249) appeared entitled, "Drug May Arrest or Slow ALS," in which it is claimed that amitriptyline (Elavil) hydrochloride beneficially modifies the course of amyotrophic lateral sclerosis (ALS) Although this report claims amelioration in the clinical course of ALS, no acceptable evidence is presented to support this assertion Although weight gain might indicate a real increase in muscle bulk and arrest of the disease, it might also represent increased fatty tissue or fluid A true decrease in fasciculations could indicate change in the course of the disease, but one requires a quantitative technique to demonstrate that the fasciculation rate has indeed changed In the presence of weight gain a thicker mantle of fatty tissue may obscure the observation of fasciculations It was not mentioned in the report whether objective measurements of strength, functional performance