Ryo M, Saito T, Kunii N, Hasegawa H, Kowa H
Department of Neurology, Kitasato University East Hospital.
Rinsho Shinkeigaku. 1994 Jul;34(7):702-6.
We report a 19-year-old female with chronic inflammatory demyelinating polyneuropathy (CIDP) with recurrent ophthalmoplegia. The patient had chronic, recurrent, asymmetrical, predominantly, distal limb weakness, and numbness of extremities with recurrent external ophthalmoplegia. Ophthalmoplegia developed in each attack of distal limb weakness, and also rapidly subsided with recovery of limb weakness. Motor nerve conduction studies revealed conduction block in more than one nerve and conduction velocities were generally normal in those segments of the nerve where conduction block was not detected. Serum anti-gangliosides GM1 IgM antibody investigated by ELISA was elevated. Thin-layer chromatography immunostaining also confirmed this result. Sural nerve biopsy showed normal findings. In spite of improvement of her signs and symptoms after prednisolone therapy, multifocal conduction block was persistent. Muscle power improved in association with decreased in anti-GM1 antibody activity. There were many reports of CIDP with cranial nerve involvements, but recurrent ophthalmoplegia in CIDP is rare. It is widely accepted that serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barré syndrome. However, serum anti-GQ1b IgG antibody was not detected in this case. It is unclear whether anti-GM1 antibody may play a role to pathogenesis of ophthalmoplegia or not in this case.
我们报告一名19岁患有慢性炎症性脱髓鞘性多发性神经病（CIDP）并伴有复发性眼肌麻痹的女性患者。该患者有慢性、复发性、不对称性、主要为远端肢体无力，以及伴有复发性外眼肌麻痹的肢体麻木。眼肌麻痹在每次远端肢体无力发作时出现，并且也随着肢体无力的恢复而迅速消退。运动神经传导研究显示多条神经存在传导阻滞，且在未检测到传导阻滞的神经节段，传导速度通常正常。通过酶联免疫吸附测定法检测的血清抗神经节苷脂GM1 IgM抗体升高。薄层色谱免疫染色也证实了这一结果。腓肠神经活检显示正常。尽管泼尼松龙治疗后她的体征和症状有所改善，但多灶性传导阻滞仍然存在。肌力随着抗GM1抗体活性的降低而改善。有许多关于CIDP伴脑神经受累的报道，但CIDP中的复发性眼肌麻痹很少见。人们普遍认为血清抗GQ1b IgG抗体与米勒费雪综合征和吉兰 - 巴雷综合征中的眼肌麻痹有关。然而，该病例中未检测到血清抗GQ1b IgG抗体。在该病例中，抗GM1抗体是否可能在眼肌麻痹的发病机制中起作用尚不清楚。
