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重症肌无力患者健康亲属中的抗独特型抗体、乙酰胆碱受体抗体与神经肌肉功能紊乱

Anti-idiotypic antibodies, acetylcholine receptor antibodies and disturbed neuromuscular function in healthy relatives to patients with myasthenia gravis.

作者信息

Lefvert A K, Pirskanen R, Svanborg E

出版信息

J Neuroimmunol. 1985 Jul;9(1-2):41-53. doi: 10.1016/s0165-5728(85)80005-9.

Abstract

Fifty-eight first-degree relatives to 40 patients with myasthenia gravis were investigated regarding presence of acetylcholine receptor antibodies, anti-idiotypic antibodies against the receptor antibodies and clinical and electrophysiological signs of disturbed neuromuscular function. No relative had clinical signs of muscle weakness. The prevalence of low concentrations of receptor antibodies was 54%, of anti-idiotypic antibodies 37% and of pathological and borderline single fibre EMG 45%. No sibling, only 2/11 children and 3/14 parents were normal in all three tests. A combination of receptor antibodies and anti-idiotypic antibodies was the most common finding and was especially frequent in children. In female siblings and children there was a positive correlation between the presence of HLA-antigen A1 and/or B8 and that of receptor antibodies and anti-idiotypic antibodies. Male siblings and children showed no such correlation but had a higher frequency of pathological single fibre EMG than females.

摘要

对40例重症肌无力患者的58名一级亲属进行了调查,检测他们是否存在乙酰胆碱受体抗体、针对受体抗体的抗独特型抗体以及神经肌肉功能紊乱的临床和电生理体征。没有亲属有肌肉无力的临床体征。受体抗体低浓度的患病率为54%,抗独特型抗体为37%,病理和临界单纤维肌电图为45%。在所有三项检测中,没有一个兄弟姐妹、只有2/11的子女和3/14的父母结果正常。受体抗体和抗独特型抗体同时出现是最常见的情况,在儿童中尤为频繁。在女性兄弟姐妹和子女中,HLA抗原A1和/或B8的存在与受体抗体和抗独特型抗体的存在之间呈正相关。男性兄弟姐妹和子女未显示出这种相关性,但病理单纤维肌电图的发生率高于女性。

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