Showing papers on "Amyotrophic lateral sclerosis published in 1974"
TL;DR: This case and others suggest that "lead neuritis" is a form of motor neuron disease, rather than a neuropathy, and that prior to making the diagnosis of ALS, lead intoxication should be considered.
Abstract: A 50-year-old battery worker had clinical signs of motor neuron disease: weakness, wasting, and active reflexes. Nerve conduction velocities, electromyogram, and motor unit territory indicated that the disorder was neuronal. Biochemical abnormalities were characteristic of lead intoxication, and the clinical disorder was reversed after treatment with penicillamine. This case and others suggest that "lead neuritis" is a form of motor neuron disease, rather than a neuropathy. Except for those persons who have clearly been exposed to lead, it is doubtful that lead intoxication is responsible for more than a small number of patients with amyotrophic lateral sclerosis (ALS). Considering the prognostic implications, however, prior to making the diagnosis of ALS, lead intoxication should be considered.
66 citations
TL;DR: Very large EMG amplitudes but normal size twitch tensions were observed among the motor neurone disease patients, indicating that, in general, motor units enlarged by sprouting are less efficient contractile units than units of normal physiological size.
Abstract: The contractile and electrical properties of motor units in the first dorsal interosseous muscle of the hand have been studied in 26 patients with ulnar neuropathies and motor neurone disease (amyotrophic lateral sclerosis). Among patients with unilateral pressure or entrapment ulnar neuropathies, there was a tendency for the twitch tensions for single motor units to be smaller, while the surface EMG amplitudes were generally larger in the affected hands. Very large EMG amplitudes but normal size twitch tensions were observed among the motor neurone disease patients, indicating that, in general, motor units enlarged by sprouting are less efficient contractile units than units of normal physiological size.
59 citations
TL;DR: Sera from patients with amyotrophic lateral sclerosis were applied to mouse anterior horn cells in tissue culture and no unique toxic effect was observed when compared with sera from normal and non-ALS neuromuscular disease subjects.
Abstract: Sera from patients with amyotrophic lateral sclerosis (ALS) were applied to mouse anterior horn cells in tissue culture. No unique toxic effect was observed when compared with sera from normal and non-ALS neuromuscular disease subjects.
45 citations
TL;DR: Methods for estimating the number of motor units in the extensor digitorum brevis and thenar muscles have provided a quantitative means for assessing the rate of loss ofMotor units with aging and disease in amyotrophic lateral sclerosis and contrary to most previous studies, digital sensory action potentials were reduced.
Abstract: Recently described methods for estimating the number of motor units in the extensor digitorum brevis and thenar muscles have provided a quantitative means for assessing the rate of loss of motor units with aging and disease. In amyotrophic. lateral sclerosis, repeated motor unit estimates showed a very rapid initial loss of motor units with slowing in the late stages. Early functional failure in giant units was suggested by abnormal decremental responses to repetitive stimulation, and late terminal collapse by the finding of small shrunken motor units. Accompanying these changes were mild slowing of the motor conduction velocity and prolongation of the motor terminal latencies. Contrary to most previous studies, digital sensory action potentials were reduced.
42 citations
TL;DR: In a case of amyotrophic lateral sclerosis, in which the occupational history and laboratory evidence indicated that exposure to lead had occured, it was found at necropsy that in tissues of nerve, spinal cord, and cardiac and skeletal muscle the lead content was 9·6 ± 0·3, 9·9 ± 1·7, 17·7 and 21·1 ± 5·1 μg/g of dry weight of tissue, respectively.
Abstract: Petkau, A., Sawatzky, A., Hillier, C. R., and Hoogstraten, J. (1974).British Journal of Industrial Medicine,31, 275-287. Lead content of neuromuscular tissue in amyotrophic lateral sclerosis. Case report and other considerations. In a case of amyotrophic lateral sclerosis, in which the occupational history and laboratory evidence indicated that exposure to lead had occured, it was found at necropsy that in tissues of nerve, spinal cord, and cardiac and skeletal muscle the lead content was 9·6 ± 0·3, 9·9 ± 1·7, 17·7, and 21·1 ± 5·1 μg/g of dry weight of tissue, respectively. Significantly elevated levels of lead were also found at necropsy in nerve, spinal cord, and muscle tissue in other cases of amyotrophic lateral sclerosis that had not been exposed to lead during life. A reassessment of the role of lead in amyotrophic lateral sclerosis is indicated.
37 citations
TL;DR: The pattern of recruiting human motor units in the first dorsal interosseous muscle of the hand has been studied in 31 patients with ulnar neuropathies and motor neurone disease, and the normal orderly pattern of recruitment was irretrievably lost.
Abstract: The pattern of recruiting human motor units in the first dorsal interosseous muscle of the hand has been studied in 31 patients with ulnar neuropathies and motor neurone disease. Two years after surgical repair of an unilateral complete severance of the ulnar nerve, the twitch tensions increased to normal size. However, the normal orderly pattern of recruiting motor units of increasing size during increasing voluntary contractions was irretrievably lost. Among patients with pressure or entrapment neuropathies, the normal orderly pattern of recruiting motor units was always retained. Similarly, in patients with motor neurone disease (amyotrophic lateral sclerosis), the orderly pattern of recruitment was not disrupted.
37 citations
TL;DR: One of the important factors contributing to increased CPK activity in amyotrophic lateral sclerosis is associated myopathic changes found in the muscles.
Abstract: Serum CPK levels were determined in a series of 94 patients with amyot-rophic lateral sclerosis. CPK levels were elevated in 75.5 percent of the patients, and were highest in those who had the disease for 3 to 15 months. Men had significantly higher CPK levels than women. Levels were significantly higher in patients showing a combination of denervation atrophy and myopathic changes in the muscle than in patients with denervation atrophy alone. However, the higher CPK values in the former group lost statistical significance with prolonged duration of the disease. There was regression of the CPK levels with longer duration of the disease that showed significant negative correlation. It is concluded that one of the important factors contributing to increased CPK activity in amyotrophic lateral sclerosis is associated myopathic changes found in the muscles.
25 citations
25 citations
TL;DR: A 41-year-old male former boxer had amyotrophic lateral sclerosis (ALS) for about eight years prior to his death from bronchopneumonia, and neuropathologic examination revealed numerous neurofibrillary changes of the neurons in the cerebrum and brain stem.
Abstract: A 41-year-old male former boxer had amyotrophic lateral sclerosis (ALS) for about eight years prior to his death from bronchopneumonia. Neuropathologic examination revealed, in addition to the classical changes of ALS, numerous neurofibrillary changes (tangles) of the neurons in the cerebrum and brain stem. The tangles were most prominent in Ammon horn, amygdaloid nuclei, and locus caeruleus. Other findings included a moderate degree of neuronal loss and gliosis of the substantia nigra. Granulovacuolar degeneration and Hirano bodies were seen in the neurons of Ammon horn. Senile plaques were not found. The distribution of the tangles resembled that reported in Guamanian ALS. Ultrastructurally, the intracytoplasmic fibrils had a twisted configuration and were similar to those described in Alzheimer disease and Guamanian ALS. The presence and distribution of the neurofibrillary changes in a patient with ALS could well be coincidental; however, the occurrence of the Guamanian type of ALS in the northeastern population of the United States is worth reporting in view of the etiologic and epidemiologic considerations of this form of ALS.
15 citations
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TL;DR: Fifty-two cases of motor neuron disease seen in two Halifax hospitals were reviewed and these cases were used to illustrate the features of this disorder and to comment on recent research observations.
Abstract: Fifty-two cases of motor neuron disease seen in two Halifax hospitals were reviewed.
Thirty-three (63.5%) had amyotrophic lateral sclerosis, 14 (26.9%) had primary muscular atrophy, four (7.7%) had bulbar palsy and one (1.9%) had primary lateral sclerosis. There were no unusual features in this series when compared with a similar series from the United States. These cases were used to illustrate the features of this disorder and to comment on recent research observations.
15 citations
TL;DR: In all patients with ALS the migration of macrophages into the inflammatory field was retarded, and in three patients the total number of cells was decreased, which could be due to a latent disorder of the autonomic nervous system.
Abstract: In ten patients with amyotrophic lateral sclerosis (ALS) and 146 control subjects, the cellular inflammatory response was studied by the "skin window" technique and 24 hours after inflicting the lesion. In all patients with ALS the migration of macrophages into the inflammatory field was retarded, and in three patients the total number of cells was decreased. This alteration could be due to a latent disorder of the autonomic nervous system.
01 Jan 1974
TL;DR: A low level of stimulation was encountered in lymphocytes from parkinsonism-dementia patients to all brain antigens, which could indicate that an immune mechanism plays a role in the pathogenesis of parkinsonic dementia, but it is more likely the response reflects a nonspecific reaction to the massive destruction of central nervous system cells in this disease.
Abstract: Lymphocytes from Guamanians with amyotrophic lateral sclerosis or parkinsonismdementia and from individuals in good health were studied to determine the possible role of cellular immunity in the pathogenesis of these diseases, which occur at extraordinarily high rates on Guam. Lymphocytes were cultered in the presence of brain material from all three groups. No stimulation was observed in lymphocytes from patients with amyotrophic lateral sclerosis. A low level of stimulation was encountered in lymphocytes from parkinsonism-dementia patients to all brain antigens. While this response could indicate that an immune mechanism plays a role in the pathogenesis of parkinsonismdementia, more likely the response reflects a nonspecific reaction to the massive destruction of central nervous system cells in this disease.
TL;DR: Mechanism of soft tissue calcification of CNS tissue in ALS cases and relationship of other divalent cations, especially manganese, to this process is discussed in regard to the etiopathogenesis of ALS.
Abstract: Content of calcium in CNS tissue was measured by neutron activation analysis in four ALS and three control cases. There was a significant difference between the average calcium content of ALS cases (4 19.108 ppm) and control cases (252f86 ppm). In ALS cases the significantly higher content of calcium was found in precentral, insula and hippocampal areas, also the thalamus, caudate nucleus, internal capsule, medulla and spinal cord. Mechanism of soft tissue calcification of CNS tissue in ALS cases and relationship of other divalent cations, especially manganese, to this process is discussed in regard to the etiopathogenesis of ALS.
TL;DR: The effect of the nervous system on the cellular invasion into the inflammatory field was studied by the "skin window" technique and invasion by macrophages was delayed in Charcot-Marie-Tooth disease, in amyotrophic lateral sclerosis, after section of the brachial plexus, in gross lesions of the brain stem and diencephalon.
Abstract: The effect of the nervous system on the cellular invasion into the inflammatory field was studied by the "skin window" technique. One-hundred twelve patients with different neurological disorders and 146 healthy persons were examined. Invasion by macrophages was delayed in Charcot-Marie-Tooth disease, in amyotrophic lateral sclerosis, after section of the brachial plexus, in gross lesions of the brain stem and diencephalon, and in lesions of the frontobasal, frontopolar, and insular regions.
01 Jan 1974
TL;DR: Lesions of the vagal nuclei of the brainstem may be caused by various diseases, and disturbance of the swallowing act is one of the most prominent symp toms.
Abstract: Lesions of the vagal nuclei of the brainstem may be caused by various diseases. Although disturbance of the swallowing act is one of the most prominent symp toms these lesions usually cause other neurological disorders also.
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TL;DR: The authors conclude that the pathogenesis of the “myogenic” scapuloperoneal syndrome differs from the pathogenic of progressive muscular dystrophy by a participation of the neurogenic disorder.
Abstract: The cellular inflammatory response was studied by the “skin window” technique in 5 patients with “myogenic” scapuloperoneal syndrome, 15 cases of progressive muscular dystrophy, 10 cases of the Charcot-Marie-Tooth disease, 10 cases of amyotrophic lateral sclerosis, and in 15 patients after acute poliomyelitis. A comparison with 146 controls showed normal conditions in patients with muscular dystrophy, whereas in 4 patients with the scapuloperoneal syndrome, in patients with Charcot-Marie-Tooth disease, in cases of ALS, and in some patients after poliomyelitis, the migration of macrophages into the inflammatory field was delayed. The authors conclude that the pathogenesis of the “myogenic” scapuloperoneal syndrome differs from the pathogenesis of progressive muscular dystrophy by a participation of the neurogenic disorder.