Moxley R T, Ricker K, Kingston W J, Böhlen R
Department of Neurology, University of Rochester School of Medicine and Dentistry, NY.
Neurology. 1989 Jul;39(7):952-5. doi: 10.1212/wnl.39.7.952.
Previous studies have suggested that an abnormal release of potassium from muscle may accompany attacks of paramyotonic weakness. We investigated 3 patients with paramyotonia congenita before and after the induction of forearm muscle weakness by exercise in cold water. Two of these patients had paralysis periodica paramyotonica and the 3rd had paramyotonia congenita. At the time of paramyotonic weakness there was a marked increase in the arterialized-venous concentration difference of potassium across forearm muscle. This indicated a significant uptake of potassium by forearm muscle in all 3 patients. Normal controls showed a slight release of potassium both at rest and after exercise in cold water. These results suggest that (1) the sodium-potassium pump of the muscle fiber is operating efficiently during paramyotonic weakness; and (2) there is a different mechanism responsible for the generalized weakness that occurs in hyperkalemic periodic paralysis.
以往的研究表明,在僵人综合征性肌无力发作时,肌肉可能会出现钾的异常释放。我们对3例先天性僵人综合征患者在冷水锻炼诱发前臂肌肉无力前后进行了研究。其中2例患者患有发作性瘫痪性僵人综合征,第3例患有先天性僵人综合征。在僵人综合征性肌无力发作时,前臂肌肉动脉化静脉的钾浓度差显著增加。这表明所有3例患者的前臂肌肉均大量摄取钾。正常对照组在静息状态和冷水锻炼后均有轻微的钾释放。这些结果表明:(1)在僵人综合征性肌无力发作时,肌纤维的钠钾泵功能正常;(2)高钾性周期性麻痹中发生的全身性肌无力有不同的机制。