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小儿麻痹后遗症神经肌肉症状患者的长期随访研究

A long-term follow-up study of patients with post-poliomyelitis neuromuscular symptoms.

作者信息

Dalakas M C, Elder G, Hallett M, Ravits J, Baker M, Papadopoulos N, Albrecht P, Sever J

出版信息

N Engl J Med. 1986 Apr 10;314(15):959-63. doi: 10.1056/NEJM198604103141505.

DOI:10.1056/NEJM198604103141505
PMID:3007983
Abstract

A "post-polio" syndrome characterized by new neuromuscular symptoms, including muscle weakness, may develop years after recovery from acute paralytic poliomyelitis. We studied 27 patients (mean age, 50.6 years) in whom new muscle weakness developed a mean of 28.8 years after recovery from acute polio. We reevaluated these patients during a mean follow-up period of 8.2 years (range, 4.5 to 20) after they were originally studied at the National Institutes of Health. The total mean follow-up period after the onset of new weakness was 12.2 years (range, 6 to 29). The patients were assessed with quantitative muscle testing, muscle biopsy, electromyography, and virologic and immunologic examination of the cerebrospinal fluid. Muscle strength had declined in all patients. The rate of decline averaged 1 percent per year. The decrease was irregular, with subjective plateau periods that ranged from 1 to 10 years. None of the patients had amyotrophic lateral sclerosis. Oligoclonal bands (IgG) were found in the cerebrospinal fluid of 7 of 13 patients studied, but no specific elevation of antibodies to poliovirus was observed in the cerebrospinal fluid. The newly affected muscles that were evaluated longitudinally with follow-up muscle biopsies and electromyography showed signs of chronic and new denervation. Groups of atrophic muscle fibers (group atrophy) and "neurogenic jitter" were not present. New post-polio muscle weakness is not a life-threatening form of motor-neuron deterioration. It appears that this weakness is not due to a loss of whole motor neurons, as in amyotrophic lateral sclerosis, but that it is due to a dysfunction of the surviving motor neurons that causes a slow disintegration of the terminals of individual nerve axons.

摘要

一种以新出现的神经肌肉症状(包括肌肉无力)为特征的“小儿麻痹后遗症”,可能在急性麻痹性脊髓灰质炎恢复数年之后出现。我们研究了27例患者(平均年龄50.6岁),这些患者在急性脊髓灰质炎恢复后平均28.8年出现了新的肌肉无力症状。在国立卫生研究院对这些患者进行首次研究后,我们在平均8.2年(范围4.5至20年)的随访期内对他们进行了重新评估。新出现无力症状后的总平均随访期为12.2年(范围6至29年)。对患者进行了定量肌肉测试、肌肉活检、肌电图检查以及脑脊液的病毒学和免疫学检查。所有患者的肌肉力量均有所下降。下降速率平均为每年1%。下降并不规律,有1至10年不等的主观平稳期。所有患者均未患肌萎缩侧索硬化症。在接受研究的13例患者中,有7例脑脊液中发现了寡克隆带(IgG),但脑脊液中未观察到针对脊髓灰质炎病毒的抗体有特异性升高。通过随访肌肉活检和肌电图对新受影响的肌肉进行纵向评估,结果显示有慢性和新出现的失神经支配迹象。未出现萎缩肌纤维群(群组萎缩)和“神经源性颤抖”。小儿麻痹后遗症新出现的肌肉无力并非一种危及生命的运动神经元退化形式。看来这种无力并非像肌萎缩侧索硬化症那样是由于整个运动神经元丧失所致,而是由于存活的运动神经元功能障碍,导致单个神经轴突终末缓慢解体。

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A long-term follow-up study of patients with post-poliomyelitis neuromuscular symptoms.小儿麻痹后遗症神经肌肉症状患者的长期随访研究
N Engl J Med. 1986 Apr 10;314(15):959-63. doi: 10.1056/NEJM198604103141505.
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New neuromuscular symptoms after old polio ("the post-polio syndrome"): clinical studies and pathogenetic mechanisms.陈旧性脊髓灰质炎后的新型神经肌肉症状(“脊髓灰质炎后综合征”):临床研究与发病机制
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Neurological manifestations of the post-polio syndrome.小儿麻痹后遗症的神经学表现。
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