Barchi R L
Electroencephalogr Clin Neurophysiol Suppl. 1978(34):559-70.
The myotonic syndrome of delayed relaxation in striated muscle associated with repetitive membrane electrical activity is characteristic of a number of hereditary disorders in animals and man. This state of pathologic membrane hyperexcitability is also seen as a consequence of intoxication with several classes of chemicals. In most of these states an increase in muscle membrane resistance due to a specific reduction in sarcolemmal GCl can be demonstrated. Computer simulations have indicated that such a reduction in GCl alone could account for the observed repetitive electrical activity. Controlled reduction of GCl in normal muscle, using aromatic monocarboxylic acids, produces a myotonic syndrome. This myotonia resembles that seen in hereditary disease in its contractile characteristics, its intracellular electrical behavior and its response to diphenylhydantoin and procainamide. The effects of variations in temperature and divalent cation concentration seen in this system parallel those predicted by computer models of myotonia based on the chloride hypothesis. Comparison of the behavior of this and similar model systems with the hereditary myotonias of man and goat allow conclusions to be drawn concerning the underlying pathophysiologic mechanisms at work in the hereditary disorders. The concept of an abnormally reduced GCl as the final common factor in many of these disorders emerges as the most tenable hypothesis, although the mechanism by which this conductance is altered may be unique in each case.
与重复性膜电活动相关的横纹肌延迟松弛的强直性综合征是动物和人类多种遗传性疾病的特征。这种病理性膜过度兴奋性状态也可因几类化学物质中毒而出现。在大多数这些状态下，可证明由于肌膜GCl特异性降低导致肌肉膜电阻增加。计算机模拟表明，仅GCl的这种降低就可以解释观察到的重复性电活动。使用芳香族单羧酸对正常肌肉中的GCl进行可控降低会产生强直性综合征。这种肌强直在收缩特性、细胞内电行为以及对苯妥英和普鲁卡因胺的反应方面类似于遗传性疾病中所见的情况。该系统中观察到的温度和二价阳离子浓度变化的影响与基于氯化物假说的肌强直计算机模型预测的情况相似。将该模型系统及类似模型系统的行为与人类和山羊的遗传性肌强直进行比较，可以就遗传性疾病中潜在的病理生理机制得出结论。尽管每种情况下改变这种电导的机制可能是独特的，但GCl异常降低作为许多这些疾病的最终共同因素这一概念成为最合理的假设。
