摘要
目的研究不典型重症肌无力患者的临床表现及其电生理的变化。方法回顾性分析自2004年1月～2008年12月以来到我院就诊的32例不典型重症肌无力患者的临床特点及电生理异常的特征。结果全组年龄7～70岁。单纯眼肌型5例,其中2例仅有眼睛不适或视物模糊感。全身型27例,其中6例表现为四肢乏力或者伴有轻度吞咽、呼吸肌的受限无眼肌、头颈、面肌的障碍。2例患者有肌肉萎缩。全组中症状有晨轻暮重及波动性表现且疲劳试验阳性的有23例,占71.9%;新斯的明试验阳性者29例,占90.6%。重复频率神经刺激:眼肌型患者中,低频刺激阳性的2例,阳性率40.0%;全身型患者中,低频刺激阳性的21例,阳性率77.8%。全组低频刺激总阳性率71.9%,与文献报道一致。结论一些临床表现不典型,疲劳试验阴性和新斯的明试验呈阴性的重症肌无力患者,肌电图低频刺激仍可作为一项不可或缺的重要辅助检查手段,提高重症肌无力的检出率,减少漏诊误诊。
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. Conclusion 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.
出处
《南方医科大学学报》
CAS
CSCD
北大核心
2009年第12期2437-2441,共5页
Journal of Southern Medical University
基金
YZD-1福建医科大学附属第一医院重点学科发展基金
关键词
不典型重症肌无力
临床表现
低频重复神经电刺激
atypical
myasthenia gravis
clinical symptoms
low frequency repetitive nerve stimulation
