Kwieciński H
J Neurol. 1978 Oct 25;219(2):107-16. doi: 10.1007/BF00314393.
Myotonia was induced in rats with clofibrate given in daily subcutaneous injections of 0.4 g/kg. The first myotonic discharges were recorded electromyographically from the extensor digitorum longus, tibialis anterior, and gastrocnemius muscles after 4 days on clofibrate, but from the soleus not until after 11 days. Clofibrate induced myotonic activity in chronically denervated muscle also. During repetitive nerve stimulation the electrical response of the muscle was declining in all myotonic rats. It did so also when repetitive stimulation was applied directly to the muscle, which would seem to suggest a myotonic defect as the cause. Several drugs were tested and diphenylhydantoin proved to inhibit myotonia most effectively. Animals on an extended clofibrate schedule (12 weeks) had ECG abnormalities resembling those seen in patients with myotonic dystrophy.
用每日皮下注射0.4 g/kg氯贝丁酯的方法在大鼠中诱发肌强直。在给予氯贝丁酯4天后,从趾长伸肌、胫骨前肌和腓肠肌通过肌电图记录到首次肌强直放电,但直到11天后才从比目鱼肌记录到。氯贝丁酯在慢性去神经支配的肌肉中也能诱发肌强直活动。在重复神经刺激期间,所有肌强直大鼠的肌肉电反应都在下降。当直接对肌肉进行重复刺激时也是如此,这似乎表明肌强直缺陷是原因。测试了几种药物,结果证明苯妥英钠对肌强直的抑制作用最有效。按照延长的氯贝丁酯给药方案(12周)处理的动物出现了类似于强直性肌营养不良患者的心电图异常。