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1988 - 2008年兰伯特 - 伊顿肌无力综合征:97例患者的临床情况

The Lambert-Eaton myasthenic syndrome 1988-2008: a clinical picture in 97 patients.

作者信息

Titulaer M J, Wirtz P W, Kuks J B M, Schelhaas H J, van der Kooi A J, Faber C G, van der Pol W L, de Visser M, Sillevis Smitt P A E, Verschuuren J J G M

机构信息

Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands.

出版信息

J Neuroimmunol. 2008 Sep 15;201-202:153-8. doi: 10.1016/j.jneuroim.2008.05.025. Epub 2008 Jul 21.

Abstract

BACKGROUND

Neuromuscular symptoms in patients with Lambert-Eaton myasthenic syndrome (LEMS) and a small cell lung cancer (SCLC) develop more rapidly than in LEMS patients without a SCLC. We studied how this clinical information, which is readily available at the first consultation, can be used to predict the presence of SCLC.

PATIENTS AND METHODS

In our study we included 52 LEMS patients with SCLC and 45 non-tumor patients (NT-LEMS). We interviewed patients using a structured checklist and reviewed their clinical records. We compared frequency and onset of symptoms during the course of LEMS.

RESULTS

In the first six months, over half the SCLC-LEMS patients had developed seven separate symptoms, while NT-LEMS patients developed only two symptoms. Proximal leg weakness and dry mouth were early symptoms in both groups. Rapid involvement of proximal arm muscles (p=0.0001), distal arm muscles (p=0.0037), distal leg muscles (p=0.0002), dysartria (p=0.0091) and the presence of erectile dysfunction (p=0.007) were found significantly more often in SCLC-LEMS patients in both cohorts. Cerebellar symptoms, although present in 9% of LEMS patients, were almost exclusively related to SCLC-LEMS.

CONCLUSION

A rapidly progressive course of disease from onset in LEMS patients should raise a high suspicion of SCLC. Special attention should be paid to involvement of upper extremities, involvement of distal arm and distal leg muscles, to erectile dysfunction and probably ataxia in order to discriminate between SCLC-LEMS and NT-LEMS.

摘要

背景

兰伯特-伊顿肌无力综合征(LEMS)合并小细胞肺癌(SCLC)的患者,其神经肌肉症状的发展速度比不合并SCLC的LEMS患者更快。我们研究了在首次会诊时即可获得的这些临床信息如何用于预测SCLC的存在。

患者与方法

我们的研究纳入了52例合并SCLC的LEMS患者和45例非肿瘤患者(非肿瘤性LEMS,NT-LEMS)。我们使用结构化检查表对患者进行访谈,并查阅他们的临床记录。我们比较了LEMS病程中症状的频率和发作情况。

结果

在前六个月中,超过一半的合并SCLC的LEMS患者出现了七种不同症状,而NT-LEMS患者仅出现了两种症状。两组的早期症状均为近端腿部无力和口干。在两个队列中,合并SCLC的LEMS患者更常出现近端手臂肌肉快速受累(p = 0.0001)、远端手臂肌肉受累(p = 0.0037)、远端腿部肌肉受累(p = 0.0002)、构音障碍(p = 0.0091)以及勃起功能障碍(p = 0.007)。小脑症状虽然在9%的LEMS患者中出现,但几乎仅与合并SCLC的LEMS相关。

结论

LEMS患者起病时疾病进展迅速应高度怀疑SCLC。应特别关注上肢受累、远端手臂和远端腿部肌肉受累、勃起功能障碍以及可能的共济失调,以便区分合并SCLC的LEMS和NT-LEMS。

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