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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.

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。

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