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肌无力性神经肌病。一种罕见的神经肌肉疾病。

Myasthenic neuromyopathy. An unusual neuromuscular disorder.

作者信息

Kinoshita M, Nakazato H, Wakata N, Satoyoshi E

出版信息

Eur Neurol. 1982 Jan-Feb;21(1):52-8. doi: 10.1159/000115454.

DOI:10.1159/000115454
PMID:7094950
Abstract

4 cases with an identical neuromuscular disorder are presented. The disorder was characterized by slowly progressive weakness and wasting of the proximal muscles together with either ocular or bulbar symptoms, fatigability, positive tensilon test, myasthenic response on repetitive nerve stimulations, elevated serum creatine phosphokinase, and neuropathic and myopathic muscle pathology. All the patients showed a fairly good response to anti-ChE medications and steroid administration. Serum creatine phosphokinase levels returned to normal values after steroid therapy. Myasthenia, polymyositis or other neuromuscular disorders were discussed in the differential diagnosis.

摘要

本文报告了4例患有相同神经肌肉疾病的病例。该疾病的特征为近端肌肉进行性缓慢无力和萎缩,并伴有眼部或延髓症状、易疲劳、腾喜龙试验阳性、重复神经刺激时出现肌无力反应、血清肌酸磷酸激酶升高以及神经源性和肌源性肌肉病理改变。所有患者对抗胆碱酯酶药物和类固醇治疗反应良好。类固醇治疗后血清肌酸磷酸激酶水平恢复正常。鉴别诊断中讨论了重症肌无力、多发性肌炎或其他神经肌肉疾病。

相似文献

1
Myasthenic neuromyopathy. An unusual neuromuscular disorder.肌无力性神经肌病。一种罕见的神经肌肉疾病。
Eur Neurol. 1982 Jan-Feb;21(1):52-8. doi: 10.1159/000115454.
2
[Adrenal cortex steroid-responsive myasthenic neuromyopathy].[肾上腺皮质类固醇反应性肌无力性神经肌病]
Rinsho Shinkeigaku. 1977 Mar;17(3):142-8.
3
Ocular myasthenia: evaluation of Tensilon tonography and electronystagmography as diagnostic tests.眼肌型重症肌无力:腾喜龙眼压描记法和眼震电图作为诊断测试的评估
J Neurol Neurosurg Psychiatry. 1970 Oct;33(5):639-46. doi: 10.1136/jnnp.33.5.639.
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Chronic neurogenic quadriceps amyotrophy.慢性神经源性股四头肌肌萎缩
Ann Neurol. 1977 Dec;2(6):528-30. doi: 10.1002/ana.410020614.
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[The repetitive stimulation test in the diagnosis of neuromuscular fatigue syndromes. Critical review of the results in 62 cases].[重复刺激试验在神经肌肉疲劳综合征诊断中的应用。62例病例结果的批判性综述]
Riv Neurol. 1979 Jul-Aug;49(4):297-309.
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Unusual myasthenic state in a teen-age boy.
Neurology. 1974 Sep;24(9):897-901. doi: 10.1212/wnl.24.9.897.
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Remediable neuromuscular disorders.可治愈的神经肌肉疾病。
Prim Care. 1975 Jun;2(2):235-57.
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Myasthenia gravis presenting with unusual neurogenic muscle atrophy.重症肌无力伴不寻常的神经源性肌肉萎缩。
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[Usefulness of electromyography in diagnostics of the neuro-muscular diseases].[肌电图在神经肌肉疾病诊断中的应用价值]
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Electrodiagnostic evaluation of patients with myasthenia gravis and related disorders.
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