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静脉注射免疫球蛋白治疗对免疫球蛋白反应性多灶性运动神经病时间离散度的影响

The effect of treatment upon temporal dispersion in IvIg responsive multifocal motor neuropathy.

作者信息

Ghosh A, Virgincar A, Kennett R, Busby M, Donaghy M

机构信息

Department of Clinical Neurology, Radcliffe Infirmary, Oxford OX2 6HE, UK.

出版信息

J Neurol Neurosurg Psychiatry. 2005 Sep;76(9):1269-72. doi: 10.1136/jnnp.2004.050252.

Abstract

BACKGROUND

Multifocal motor neuropathy with conduction block (MMN) is a treatable disorder that can be mistaken for other lower motor neurone syndromes. Existing electrophysiological diagnostic criteria for MMN are restrictive. In particular, many are cautious about diagnosing conduction block (CB) in the presence of abnormal temporal dispersion (TD).

OBJECTIVE

To study the significance of TD in MMN, its relationship to CB in intravenous immunoglobulin (IvIg) responsive patients, and its utility in detecting a treatment response.

METHODS

We compared pre- and post-treatment changes in CB and TD in nine patients who satisfied clinical and electrophysiological criteria for MMN and responded to IvIg.

RESULTS

TD improved in one or more nerve segments in eight of nine patients tested. There was marked improvement in 65% of all nerve segments, and 60% of those segments with CB. By comparison, significant improvement in CB occurred in only 33% of segments. Of segments with significantly better CB after treatment, all but one showed similar improvements in TD. Such changes were not related to the degree of TD before treatment, being seen in segments with abnormal as well as normal TD. There was no correlation between improvements seen in TD and CB.

CONCLUSION

We believe that TD should be considered an inherent feature of MMN. Improvement in TD is an independent marker of electrophysiological improvement in this disorder and is likely to be more useful than CB. When MMN is clinically suspected, the use of stringent criteria for CB in the presence of TD should be avoided.

摘要

背景

多灶性运动神经病伴传导阻滞(MMN)是一种可治疗的疾病,可能被误诊为其他下运动神经元综合征。现有的MMN电生理诊断标准具有局限性。特别是,许多人在存在异常时间离散(TD)的情况下对诊断传导阻滞(CB)持谨慎态度。

目的

研究TD在MMN中的意义、其与静脉注射免疫球蛋白(IvIg)反应性患者中CB的关系以及其在检测治疗反应中的作用。

方法

我们比较了9例符合MMN临床和电生理标准且对IvIg有反应的患者治疗前后CB和TD的变化。

结果

在接受测试的9例患者中,有8例患者的一个或多个神经节段的TD有所改善。所有神经节段中有65%有显著改善,有CB的节段中有60%有显著改善。相比之下,只有33%的节段CB有显著改善。治疗后CB有显著改善的节段中,除一个节段外,其他所有节段的TD都有类似改善。这些变化与治疗前TD的程度无关,在TD异常和正常的节段中均可见。TD和CB的改善之间没有相关性。

结论

我们认为TD应被视为MMN的一个固有特征。TD的改善是该疾病电生理改善的一个独立标志,可能比CB更有用。当临床怀疑为MMN时,应避免在存在TD的情况下使用严格的CB标准。

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