Yamada S, Tomita Y
Division of Child Neurology, Tottori University, School of Medicine.
No To Hattatsu. 1991 Jan;23(1):15-9.
We studied juvenile ocular myasthenia gravis (MG) with special reference to evoked EMG in orbicularis oculi muscle. The subjects consisted of 5 cases aged 1-4 years and 3 cases over 10 years. Young children were examined during drug-induced sleep. Stimulation was delivered to preauricular facial nerve, and M waves were recorded from ipsilateral orbicularis oculi muscle. Examinations were also carried out in thenar or hypothenar muscles by stimulating the median or ulnar nerves respectively. On Harvey-Masland test, 6 out of 8 cases showed waning of more than 10%. On M-wave recovery cycle, 3 out of 6 cases examined showed decrement during stimulation interval of 100 msec to 500 msec. In 2 of these, the peak of recovery cycle curve rose over 100% during stimulation interval of 30 to 60 msec on a usual dose treatment of anti-cholinesterase, while on prednisolone treatment this peak did not increase over 100% and the pattern of recovery cycle changed to almost normal. On post-tetanic cycle study, only 1 out of 4 cases examined showed post-tetanic facilitation and exhaustion. The response of the belly-tendon in the hand showed no abnormality. Although there still remains technical difficulty in the examination of evoked EMG in orbicularis oculi muscle, this examination is very useful in diagnosis, evaluation and understanding of pathophysiology of MG.
我们专门针对眼轮匝肌的诱发肌电图研究了青少年型重症肌无力(MG)。研究对象包括5例1 - 4岁的儿童和3例10岁以上的患者。年幼患儿在药物诱导睡眠状态下接受检查。刺激耳前面神经,记录同侧眼轮匝肌的M波。还分别通过刺激正中神经和尺神经在大鱼际或小鱼际肌进行检查。在Harvey - Masland试验中,8例中有6例显示衰减超过10%。在M波恢复周期方面,6例受检者中有3例在100毫秒至500毫秒的刺激间隔期间出现递减。其中2例在抗胆碱酯酶常规剂量治疗时,恢复周期曲线的峰值在30至60毫秒的刺激间隔期间上升超过100%,而在泼尼松龙治疗时,该峰值未超过100%,且恢复周期模式变为几乎正常。在强直后周期研究中,4例受检者中只有1例显示强直后易化和耗竭。手部肌腹 - 肌腱反应未显示异常。尽管眼轮匝肌诱发肌电图检查仍存在技术困难,但该检查对MG的诊断、评估及病理生理学理解非常有用。