Murase Nagako, Goto Masahiro, Kohara Nobuo, Kimura Jun
Department of Neurology, National Hospital Organization, Kyoto Medical Center, 1-1 Mukoubata-cho, Fukakusa, Fushimi-ku, Kyoto City, Kyoto 612-8555, Japan.
Department of Neurology, Kobe City Medical Center General Hospital, 1-1, 2 cho-me, Minamimachi, Minatojima, Chuou-ku, Kobe City, Kobe 650-0047, Japan.
Clin Neurophysiol Pract. 2020 Dec 17;6:36-40. doi: 10.1016/j.cnp.2020.11.003. eCollection 2021.
We report a case of sustained atypical myokymia associated with short bursts of neuromyotonic discharges involving the abductor pollicis brevis (APB) muscle and describe a useful way of detecting a focal slowing involving a small number of median nerve motor fibers with a concentric needle using the filter setting for single fiber electromyography (EMG).
A 62-year-old woman developed right thumb twitches at regular interval of 1.7-3.3 s (0.6-0.3 Hz), which continued for more than four months. Muscle twitches remained the same during altered hand position, psychological stress, or sleep. A concentric needle inserted in the active zone of the APB muscle revealed myokymic bursts with a characteristic of neuromyotonic discharges. Inching study, stimulating at 5 mm increment along the median nerve and recording with a concentric needle using a filter setting for single fiber EMG, revealed a focal slowing of the motor fibers at a point 5-10 mm distal from the distal crease of the wrist, an entrapment site occasionally seen in the carpal tunnel syndrome. One injection of botulinum toxin type A eliminated the myokymia, which then recurred two and a half years later, showing less prominent muscle twitches.
Sustained atypical myokymia seen in our case represented bursts of neuromyotonic discharges originated from a focal demyelinating lesion involving a few median nerve motor fibers.
我们报告一例与拇短展肌(APB)的短阵神经肌强直放电相关的持续性非典型肌束震颤病例,并描述一种使用单纤维肌电图(EMG)的滤波设置,通过同心针检测少量正中神经运动纤维局灶性减慢的有效方法。
一名62岁女性出现右拇指抽搐,间隔规律,为1.7 - 3.3秒(0.6 - 0.3赫兹),持续四个多月。手部位置改变、心理压力或睡眠期间肌肉抽搐情况不变。插入APB肌肉活动区的同心针显示出具有神经肌强直放电特征的肌束震颤爆发。沿正中神经以5毫米增量进行刺激,并使用单纤维EMG的滤波设置通过同心针记录的微移研究显示,在腕部远侧横纹远侧5 - 10毫米处运动纤维存在局灶性减慢,这是腕管综合征中偶尔可见的卡压部位。一次注射A型肉毒毒素消除了肌束震颤,两年半后复发,肌肉抽搐不那么明显。
我们病例中所见的持续性非典型肌束震颤代表源自涉及少数正中神经运动纤维的局灶性脱髓鞘病变的神经肌强直放电爆发。