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32例非典型重症肌无力患者的临床表现及电生理变化分析

[Analysis of the clinical manifestations and electrophysiological changes in 32 patients with atypical myasthenia gravis].

作者信息

Wei Hui-xing, Wu Gang, Yang Jin-shan

机构信息

Department of Neurology, First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, China.

出版信息

Nan Fang Yi Ke Da Xue Xue Bao. 2009 Dec;29(12):2437-41.

Abstract

OBJECTIVE

To study the clinical characteristics and electrophysiological changes in patients with atypical myasthenia gravis (MG).

METHODS

The characteristics of the clinical symptoms and electrophysiological changes were investigated in 32 patients with atypical MG diagnosed in our hospital from January 2004 to December 2008.

RESULTS

The ages of the patients ranged from 7 to 70 years. Five patients were diagnosed to have ocular MG (OMG), among whom 2 patient only complained of eye discomfort and blurred vision. Twenty-seven patients had generalized MG, and 6 of them showed muscle weakness of the limbs with or without mild difficulty in swallowing or respiratory muscles, but free of muscle dysfunctions in muscles of eyes, head, neck or face. Another 2 patients manifested muscular atrophy. Twenty-three patients (71.9%) displayed both fluctuating symptoms and positive results of fatigue test. Twenty-nine patients (90.6%) have positive results in the neostigmine test. Two patients in the OMG group (40.0%) showed positive results in the low frequency repetitive nerve stimulation (LFRNS), as compared with the 21 patients in the generalized MG group (71.9%) showing positive results. The total positivity rate of LFRNS was 71.9% in the total patients, consistent with the published data.

CONCLUSIONS

In MG patients with atypical clinical symptoms, negative results of neostigmine test and fatigue test, LFRNS test can be an indispensable method to increase detection rate of MG and reduce erroneous or missed diagnosis.

摘要

目的

研究非典型重症肌无力(MG)患者的临床特征及电生理变化。

方法

对2004年1月至2008年12月在我院确诊的32例非典型MG患者的临床症状特点及电生理变化进行研究。

结果

患者年龄7至70岁。5例诊断为眼肌型MG(OMG),其中2例仅诉眼部不适及视物模糊。27例为全身型MG,其中6例表现为肢体肌无力,伴或不伴有轻度吞咽困难或呼吸肌无力,但眼、头、颈或面部肌肉无功能障碍。另外2例表现为肌肉萎缩。23例(71.9%)症状有波动且疲劳试验阳性。29例(90.6%)新斯的明试验阳性。OMG组2例(40.0%)低频重复神经电刺激(LFRNS)阳性,全身型MG组21例(71.9%)阳性。全部患者LFRNS总阳性率为71.9%,与已发表数据一致。

结论

对于临床症状不典型、新斯的明试验及疲劳试验阴性的MG患者,LFRNS检查可作为提高MG检出率、减少误诊漏诊的必要手段。

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