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重症肌无力-兰伯特-伊顿重叠综合征

Myasthenia gravis Lambert-Eaton overlap syndrome.

作者信息

Oh Shin J

机构信息

Department of Neurology, University of Alabama at Birmingham, Birmingham, Alabama.

出版信息

Muscle Nerve. 2016 Jan;53(1):20-6. doi: 10.1002/mus.24921. Epub 2015 Nov 26.

Abstract

INTRODUCTION

To assess whether a myasthenia gravis (MG) Lambert-Eaton overlap syndrome (MLOS) exists.

METHODS

Case reports that met the universally accepted diagnostic criteria for MG and Lambert-Eaton myasthenic syndrome (LEMS) were sought through a PubMed search. Fifty-five possible cases of MLOS were identified.

RESULTS

Thirty-nine cases met the diagnostic criteria for MG and LEMS. Analysis of clinical features showed that these patients have common MG and LEMS symptoms: oculo-bulbar paresis and good response to anti-cholinesterase for MG and limb weakness and decreased or absent reflexes for LEMS. All had the classical LEMS pattern in the repetitive nerve stimulation test: low compound muscle action potential amplitude and incremental response > 60% with brief exercise or at high rate of stimulation. Eight patients had combined positive acetylcholine receptor antibody (AChR-ab) or muscle-specific kinase-ab and voltage-gated calcium channel- ab tests.

CONCLUSIONS

A myasthenia gravis Lambert-Eaton overlap syndrome (MLOS) does exist.

摘要

引言

评估重症肌无力(MG)-兰伯特-伊顿综合征重叠综合征(MLOS)是否存在。

方法

通过PubMed检索符合普遍接受的MG和兰伯特-伊顿肌无力综合征(LEMS)诊断标准的病例报告。共识别出55例可能的MLOS病例。

结果

39例符合MG和LEMS的诊断标准。临床特征分析表明,这些患者具有MG和LEMS的共同症状:MG的眼球-延髓肌无力及对抗胆碱酯酶反应良好,LEMS的肢体无力及反射减弱或消失。所有患者在重复神经电刺激试验中均呈现典型的LEMS模式:复合肌肉动作电位波幅低,短暂运动或高频刺激时递增反应>60%。8例患者乙酰胆碱受体抗体(AChR-ab)或肌肉特异性激酶抗体及电压门控钙通道抗体检测呈联合阳性。

结论

重症肌无力-兰伯特-伊顿综合征重叠综合征(MLOS)确实存在。

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