Kuwabara Satoshi, Misawa Sonoko, Takahashi Hirokatsu, Sawai Setsu, Kanai Kazuaki, Nakata Miho, Mori Masahiro, Hattori Takamichi, Yuki Nobuhiro
Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.
J Neuroimmunol. 2007 Sep;189(1-2):158-62. doi: 10.1016/j.jneuroim.2007.07.001. Epub 2007 Jul 27.
Anti-ganglioside GQ1b antibody induces neuromuscular blocking on mouse phrenic nerve-diaphragm preparations. Several reports suggest that patients with this antibody show abnormal neuromuscular transmission in the facial or limb muscles, but limb muscle weakness is unusual in Miller Fisher syndrome that is often associated with anti-GQ1b antibody. To determine whether anti-GQ1b sera affect neuromuscular transmission in human limb muscles, axonal-stimulating single fiber electromyography was performed in the forearm muscle of seven patients with anti-GQ1b antibody. All showed normal jitter and no blocking. Anti-GQ1b antibody does not affect neuromuscular transmission in human limb muscles. The different findings in mouse and human may be explained by the extent of expression of GQ1b on the motor nerve terminals in the muscle examined.
抗神经节苷脂GQ1b抗体可在小鼠膈神经-膈肌标本上诱导神经肌肉阻滞。有几份报告表明，患有这种抗体的患者在面部或肢体肌肉中表现出异常的神经肌肉传递，但肢体肌肉无力在常与抗GQ1b抗体相关的米勒-费希尔综合征中并不常见。为了确定抗GQ1b血清是否影响人类肢体肌肉的神经肌肉传递，对7例抗GQ1b抗体患者的前臂肌肉进行了轴突刺激单纤维肌电图检查。所有患者均表现出正常的颤抖且无阻滞现象。抗GQ1b抗体不影响人类肢体肌肉的神经肌肉传递。小鼠和人类的不同研究结果可能是由于所检查肌肉中运动神经末梢上GQ1b的表达程度不同所致。
