Pestronk A, Cornblath D R, Ilyas A A, Baba H, Quarles R H, Griffin J W, Alderson K, Adams R N
Department of Neurology, Johns Hopkins School of Medicine, Baltimore, MD 21205.
Ann Neurol. 1988 Jul;24(1):73-8. doi: 10.1002/ana.410240113.
We report 2 patients with a treatable, immune-mediated motor polyneuropathy associated with antibodies to defined neural antigens. In these patients asymmetrical weakness developed in one arm and progressed over 2 to 3 years to involve the other arm, legs, and trunk. Both patients were initially diagnosed as having lower motor neuron forms of amyotrophic lateral sclerosis. However, repeated electrophysiological testing eventually showed multifocal conduction blocks in motor but not sensory fibers compatible with patchy selective demyelination. Serum testing by thin-layer chromatography and enzyme-linked immunosorbent assay revealed that both patients had high titers of antibody directed against GM1 and other gangliosides. Initial therapeutic trials of prednisone (100 mg daily for 4 to 6 months) and plasmapheresis were unsuccessful. Treatment with cyclophosphamide, however, was followed by marked improvement in strength in both patients.
我们报告了2例患有可治疗的免疫介导性运动性多神经病的患者，该疾病与针对特定神经抗原的抗体相关。在这些患者中，一侧手臂出现不对称性无力，并在2至3年内进展至累及另一侧手臂、腿部和躯干。两名患者最初均被诊断为患有下运动神经元型肌萎缩侧索硬化症。然而，反复的电生理测试最终显示运动纤维而非感觉纤维存在多灶性传导阻滞，这与斑片状选择性脱髓鞘相符。通过薄层色谱法和酶联免疫吸附测定进行的血清检测显示，两名患者均具有高滴度的针对GM1和其他神经节苷脂的抗体。泼尼松（每日100 mg，持续4至6个月）和血浆置换的初始治疗试验均未成功。然而，环磷酰胺治疗后，两名患者的肌力均有显著改善。
