Arimura K
Third Department of Internal Medicine, Kagoshima University.
Rinsho Shinkeigaku. 1997 Dec;37(12):1109-10.
Functional abnormalities, especially the excitability changes of axon in the peripheral nerve involvement, were reviewed. In GBS and CIDP, the correlation between conduction block and anti-ganglioside antibodies have been discussed. Using anti GM1 antibody positive sera, the suppression of voltage-gated sodium channels (VGSC) has been reported. Although this findings have not been confirmed, the involvement of VGSC may be an important mechanism for eliciting conduction block. In Isaacs' syndrome, voltage-gated potassium channels (VGKC) were suppressed by autoantibodies to VGKC. Furthermore, in generalized myokymia syndrome which shows only myokymia and muscle cramp without grip myotonia, VGKCs are also suppressed in some cases. These findings suggest that some patients with myokymia and neuromyotonia are induced by anti-VGKC antibodies. For evaluating the axonal excitability in vivo, the threshold electrotonus method have been developed and applied for the involvement of peripheral nerves. In ALS, impairment of potassium conductance was shown and was speculated to have the possible rrelation with fasciculation. Thus threshold electrotonus method will be an important method for evaluating axonal excitability in human. The accumulated knowledge about the involvement of axonal ion channels will expand and will be categorized as axonal channelopathies.
本文综述了功能异常，特别是周围神经受累时轴突的兴奋性变化。在吉兰-巴雷综合征（GBS）和慢性炎症性脱髓鞘性多发性神经病（CIDP）中，已经讨论了传导阻滞与抗神经节苷脂抗体之间的相关性。使用抗GM1抗体阳性血清，已有报道电压门控钠通道（VGSC）受到抑制。尽管这一发现尚未得到证实，但VGSC的参与可能是引发传导阻滞的重要机制。在艾萨克斯综合征中，电压门控钾通道（VGKC）被针对VGKC的自身抗体所抑制。此外，在仅表现为肌束震颤和肌肉痉挛而无握力性肌强直的全身性肌束震颤综合征中，某些病例中VGKC也受到抑制。这些发现表明，一些肌束震颤和神经性肌强直患者是由抗VGKC抗体引起的。为了在体内评估轴突兴奋性，已经开发了阈下电紧张方法并将其应用于周围神经受累的情况。在肌萎缩侧索硬化症（ALS）中，显示出钾电导受损，并推测其可能与肌束震颤有关。因此，阈下电紧张方法将成为评估人体轴突兴奋性的重要方法。关于轴突离子通道受累的累积知识将会扩展，并将被归类为轴突通道病。
