Irani P F, Purohit A V, Wadia N H
Acta Neurol Scand. 1977 Apr;55(4):273-88. doi: 10.1111/j.1600-0404.1977.tb05647.x.
Four patients with the syndrome of continuous muscle fiber activity were seen in a period of 6 years. Young females predominated. Remarkable improvement followed phenytoin sodium and carbamazepine administration in three patients, one of whom was "cured" within 4 years. In the remaining patient the response was inconstant. Electromyography showed abnormal spontaneous activity with diphasic and triphasic potentials appearing as doublets and multiplets. Waxing and waning was observed. D-tubocurarine and succinylcholine abolished the spontaneous activity excluding the muscle and the myoneural junction as its source. Spinal anesthesia, thiopental sodium, sleep and baclofen had no effect on it, ruling out a central source. In three patients, nerve blocks at the knee and elbow or wrist abolished this activity pointing to a proximal site of origin in the nerve somewhere between the spinal cord and the nerve block. In the remaining patient such a block significantly reduced but did not abolished the activity suggesting a dual source above and below the block. Finally successive examinations in one of our patients led us to believe that this activity may arise from different sites at varying times. It appears that regardless of the site of origin of the activity in the motor axon the counter part clinical syndrome remains the same.
在6年的时间里,共诊治了4例持续性肌肉纤维活动综合征患者。以年轻女性为主。3例患者在服用苯妥英钠和卡马西平后有显著改善,其中1例在4年内“治愈”。其余1例患者的反应不稳定。肌电图显示异常的自发活动,出现双相和三相电位,呈成对和多发放电。观察到活动增强和减弱。筒箭毒碱和琥珀酰胆碱可消除自发活动,排除了肌肉和肌神经接头作为其来源。脊髓麻醉、硫喷妥钠、睡眠和巴氯芬对其无影响,排除了中枢来源。3例患者在膝部、肘部或腕部进行神经阻滞可消除这种活动,提示起源于脊髓与神经阻滞之间神经的近端部位。其余1例患者进行这种阻滞可显著减少但不能消除活动,提示在阻滞部位上下有双重来源。最后,对我们的1例患者进行连续检查后,我们认为这种活动可能在不同时间起源于不同部位。看来,无论运动轴突中活动的起源部位如何,相应的临床综合征都是相同的。