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重症肌无力与肌肉萎缩

[Myasthenia gravis and muscle atrophy].

作者信息

Sanadze A G, Gilvanova O V

机构信息

Moscow Myasthenic Center of the City Clinical Hospital No. 51, Moscow, Russia.

出版信息

Zh Nevrol Psikhiatr Im S S Korsakova. 2021;121(2):79-87. doi: 10.17116/jnevro202112102179.

Abstract

Muscle atrophy is uncommon in myasthenia gravis. There are some reports in the literature of muscle atrophy in MUSK-positive myasthenia gravis. The authors present eight cases of AChR-positive myasthenia gravis associated with muscle atrophy. Symmetrical atrophy of the forearms with preserved hand muscles was identified in six cases and scapular winging in two cases. Atrophy appeared 3-18 years after the onset of myasthenia gravis and after a period of progression, the condition was stabilized. In all cases, there were no pain syndrome, no sensory loss, normal creatine phosphokinase (CPK) levels, no significant abnormality on MRI of the cervical spine. Concomitant neuropathies, amyotrophic lateral sclerosis and other neuromuscular diseases were excluded. All patients underwent quantitative electromyography and repetitive nerve stimulation. The authors suggest that the atrophy is not the sign of myasthenia gravis in this case and is caused by other neuromuscular diseases.

摘要

肌肉萎缩在重症肌无力中并不常见。文献中有一些关于MUSK阳性重症肌无力合并肌肉萎缩的报道。作者报告了8例与肌肉萎缩相关的乙酰胆碱受体(AChR)阳性重症肌无力病例。6例患者出现前臂对称性萎缩,手部肌肉保留,2例出现肩胛翼状肩胛。萎缩出现在重症肌无力发病后3 - 18年,经过一段时间的进展后病情稳定。所有病例均无疼痛综合征、无感觉丧失、肌酸磷酸激酶(CPK)水平正常、颈椎MRI无明显异常。排除了合并的神经病变、肌萎缩侧索硬化症和其他神经肌肉疾病。所有患者均接受了定量肌电图和重复神经刺激检查。作者认为,在这种情况下,萎缩并非重症肌无力的体征,而是由其他神经肌肉疾病引起的。

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