抗GQ1b抗体综合征中的急性眼肌麻痹：眼轮匝肌神经肌肉传递缺陷的电生理证据
Acute ophthalmoparesis in the anti-GQ1b antibody syndrome: electrophysiological evidence of neuromuscular transmission defect in the orbicularis oculi.
作者信息
Lo Y L, Chan L L, Pan A, Ratnagopal P
机构信息
Department of Neurology, National Neuroscience Institute, Singapore General Hospital, Singapore.
出版信息
J Neurol Neurosurg Psychiatry. 2004 Mar;75(3):436-40. doi: 10.1136/jnnp.2003.023630.
OBJECTIVE
To prospectively study anti-GQ1b antibody positive cases of acute ophthalmoparesis (AO) clinically and electrophysiologically.
METHODS
Nine consecutive cases presenting with predominantly acute ophthalmoplegia were assessed clinically and had stimulated single fibre electromyography (SFEMG) of the orbicularis oculi at presentation. All had magnetic resonance imaging brain scans and anti-GQ1b antibody titres determined.
RESULTS
Four cases had elevated anti-GQ1b antibody titres and abnormal SFEMG studies, which improved in tandem with clinical recovery over three months. Five other anti-GQ1b antibody negative cases were diagnosed as diabetic related cranial neuropathy, idiopathic cranial neuropathy, ocular myasthenia gravis, and Tolosa-Hunt syndrome. All five cases showed complete recovery over a three month period.
CONCLUSIONS
This study demonstrated electrophysiologically the dynamic improvement of neuromuscular transmission of anti-GQ1b antibody positive cases of AO, in tandem with clinical recovery. SFEMG is of value in differentiating weakness due to neuromuscular transmission defect from neuropathy in these clinical situations.
目的
对急性眼肌麻痹（AO）抗GQ1b抗体阳性病例进行临床和电生理前瞻性研究。
方法
对9例以急性眼肌麻痹为主的连续病例进行临床评估，并在就诊时对眼轮匝肌进行刺激单纤维肌电图（SFEMG）检查。所有病例均进行了脑部磁共振成像扫描并测定了抗GQ1b抗体滴度。
结果
4例抗GQ1b抗体滴度升高且SFEMG检查异常，在三个月内随着临床恢复而改善。另外5例抗GQ1b抗体阴性病例被诊断为糖尿病相关的颅神经病变、特发性颅神经病变、眼肌型重症肌无力和托洛萨-亨特综合征。所有5例病例在三个月内均完全恢复。
结论
本研究通过电生理方法证明了AO抗GQ1b抗体阳性病例神经肌肉传递的动态改善与临床恢复同步。在这些临床情况下，SFEMG对于区分神经肌肉传递缺陷导致的无力与神经病变具有价值。
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