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多灶性运动神经病伴持续性传导阻滞的单纤维肌电图

Single fibre electromyography in multifocal motor neuropathy with persistent conduction blocks.

作者信息

Lagueny A, Le Masson G, Burbeaud P, Deliac P

机构信息

Department of Clinical Neurology CHU Bordeaux, France.

出版信息

J Neurol Neurosurg Psychiatry. 1998 Sep;65(3):357-61. doi: 10.1136/jnnp.65.3.357.

Abstract

OBJECTIVE

To study the process of denervation-reinnervation in multifocal motor neuropathy with persistent conduction blocks in clinically affected and unaffected muscles.

METHOD

Volitional single fibre electromyography (SFEMG) was performed in the extensor digitorum communis (EDC) of seven patients. The jitter, the fibre density, and the mean interpotential interval were determined. The results before and after treatment with intravenous immunoglobulin (IVIg) between the unaffected EDC and affected EDC examined during the same SFEMG session were also compared. In addition the values of jitter, fibre density, and mean interpotential interval were analysed for correlation with the strength score on the MRC scale, the duration of the neuropathy, the number of IVIg treatment periods, and the radial nerve conduction block values.

RESULTS

Mean jitter, percentage of jitters >60 micros, and impulse blocking percentage, were higher than normal in both the affected EDCs and to a lesser degree in unaffected EDCs. Jitter decreased significantly after IVIg and correlated only with the MRC score. Fibre density and mean interpotential interval were higher than normal equally in the affected EDC and unaffected EDCs, but no correlation was found with strength, duration of the neuropathy, number of treatment periods, and conduction block values.

CONCLUSION

The major finding is the presence of SFEMG abnormalities in clinically unaffected EDCs. This shows a process of denervation-reinnervation even in the absence of clinical symptoms, probably more frequent than commonly supposed in this neuropathy. The rapid clinical improvement after IVIg infusions could be due to remyelination after demyelination and to an interference of IVIg with the blocking effect of antibodies on the Na+ channels at the motor nerve endings.

摘要

目的

研究多灶性运动神经病伴持续性传导阻滞时,临床受累及未受累肌肉的失神经-再支配过程。

方法

对7例患者的指总伸肌(EDC)进行随意单纤维肌电图(SFEMG)检查。测定抖动、纤维密度和平均电位间期。还比较了在同一次SFEMG检查中,未受累EDC和受累EDC在静脉注射免疫球蛋白(IVIg)治疗前后的结果。此外,分析抖动、纤维密度和平均电位间期的值与医学研究委员会(MRC)量表的肌力评分、神经病病程、IVIg治疗疗程数以及桡神经传导阻滞值之间的相关性。

结果

受累EDC的平均抖动、抖动>60微秒的百分比和冲动阻滞百分比均高于正常,未受累EDC的上述指标也有一定程度升高。IVIg治疗后抖动显著降低,且仅与MRC评分相关。受累EDC和未受累EDC的纤维密度和平均电位间期均高于正常,但与肌力、神经病病程、治疗疗程数及传导阻滞值均无相关性。

结论

主要发现是临床未受累的EDC存在SFEMG异常。这表明即使在没有临床症状的情况下也存在失神经-再支配过程,在这种神经病中可能比通常认为的更为常见。IVIg输注后临床症状迅速改善可能是由于脱髓鞘后的髓鞘再生以及IVIg对运动神经末梢抗体阻断钠通道作用的干扰。

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本文引用的文献

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AAEM minimonograph #25: single-fiber electromyography.美国电诊断医学学会小型专论第25号:单纤维肌电图
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Multifocal motor neuropathy.多灶性运动神经病
J Neurol Neurosurg Psychiatry. 1996 Jun;60(6):599-603. doi: 10.1136/jnnp.60.6.599.
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AAEM case report #30: multifocal motor neuropathy.美国急诊医学学会病例报告#30:多灶性运动神经病。
Muscle Nerve. 1996 Mar;19(3):269-76. doi: 10.1002/(SICI)1097-4598(199603)19:3<269::AID-MUS1>3.0.CO;2-B.
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Multifocal motor neuropathy: response to human immune globulin.
Ann Neurol. 1993 Mar;33(3):237-42. doi: 10.1002/ana.410330303.

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