Durelli L, Mutani R, Fassio F, Satta A, Bartoli E
Ann Neurol. 1982 Mar;11(3):258-65. doi: 10.1002/ana.410110305.
Progressively increasing concentrations of potassium chloride in Evans blue saline were administered to patients affected with myotonic dystrophy and to healthy volunteers before and after parenteral treatment with taurine. Excitability changes of thenar eminence muscles were related to the venous potassium and chloride concentrations. The actual electrolyte concentrations were compared to those to be expected if no infused electrolytes had been transported into cells. The expected concentrations were calculated by means of Evans blue dilution. This method permitted quantification of changes of muscle-excitability in terms of the potassium chloride concentration capable of disturbing the electrical activity of the studied muscles. The method also provided an indirect evaluation of electrolyte movements across muscle membrane in vivo in humans. Dystrophic myotonic muscles appeared highly sensitive to extracellular potassium and, unlike normal muscles, were unable to accumulate potassium-induced muscle hyperexcitability and favored electrolyte accumulation in dystrophic myotonic muscles. The stabilizing effect of taurine is discussed in relation to its ability to increase intracellular potassium concentration, membrane conductance, or both.
在给予患有强直性肌营养不良症的患者以及健康志愿者静脉注射牛磺酸前后,分别向他们输注了浓度逐渐增加的氯化钾伊文思蓝盐水。鱼际肌的兴奋性变化与静脉血中的钾和氯浓度相关。将实际电解质浓度与未输注电解质时若未转运至细胞内所预期的浓度进行比较。预期浓度通过伊文思蓝稀释法计算得出。该方法能够依据可干扰所研究肌肉电活动的氯化钾浓度对肌肉兴奋性变化进行量化。此方法还能对人体体内电解质跨肌肉膜的运动进行间接评估。营养不良性强直性肌对细胞外钾高度敏感,与正常肌肉不同,它无法积累钾诱导的肌肉过度兴奋,且有利于电解质在营养不良性强直性肌中积累。文中讨论了牛磺酸的稳定作用与其增加细胞内钾浓度、膜电导或两者的能力之间的关系。