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发作性肌无力和发作性肌强直麻痹

Adynamia episodica and paralysis periodica paramyotonica.

作者信息

Ricker K, Rohkamm R, Böhlen R

出版信息

Neurology. 1986 May;36(5):682-6. doi: 10.1212/wnl.36.5.682.

Abstract

We studied hyperkalemic attacks in one family with adynamia episodica (AE) and one family with paralysis periodica paramyotonica (PPP). Under exercise, serum potassium increased as in healthy subjects. Thiazide did not affect this increase. Thirty minutes after exercise, a second potassium increase occurred, but could be prevented by thiazide and not by mexiletine. After cooling, muscle relaxation time was normal in AE but increased up to 100 times in PPP; this cooling effect was prevented by mexiletine. Although hyperkalemic attacks are similar in AE and in PPP, the membrane defect in PPP seems more complex.

摘要

我们研究了一个患有发作性肌无力(AE)的家族和一个患有发作性周期性麻痹(PPP)的家族中的高钾血症发作情况。在运动时,血清钾如同健康受试者一样升高。噻嗪类药物不影响这种升高。运动后30分钟,出现了第二次钾升高,但可被噻嗪类药物阻止,而美西律则不能。冷却后,AE患者的肌肉松弛时间正常,而PPP患者的肌肉松弛时间增加多达100倍;美西律可阻止这种冷却效应。尽管AE和PPP中的高钾血症发作相似,但PPP中的膜缺陷似乎更为复杂。

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