Tsai C P, Lin K P, Liao K K, Wang S J, Wang V, Kao K P, Wu Z A
Neurological Institute, Veterans General Hospital, Taipei, Taiwan.
Eur Neurol. 1993;33(6):446-9. doi: 10.1159/000116991.
We report the results of immunosuppressive treatment with intravenous cyclophosphamide in 12 patients with lower motor neuron syndrome and elevated titers of serum autoantibodies to GM1 ganglioside. All patients had lower motor neuron dysfunction including proximal or distal weakness, fasciculation and muscle atrophy, but no upper motor neuron dysfunction such as hyperreflexia, spasticity or Babinski's sign. Electrophysiological studies revealed no evidence of conduction block, but EMG findings of acute or chronic denervation in the limbs were present. Serum biochemistry and immunological studies were negative for M protein. After a 6-month follow-up, despite a fall in antibody titer, there was no significant clinical improvement in any of the patients.
我们报告了12例下运动神经元综合征且血清抗GM1神经节苷脂自身抗体滴度升高的患者接受静脉注射环磷酰胺免疫抑制治疗的结果。所有患者均有下运动神经元功能障碍，包括近端或远端肌无力、肌束震颤和肌肉萎缩，但无诸如反射亢进、痉挛或巴宾斯基征等上运动神经元功能障碍。电生理研究未发现传导阻滞的证据，但四肢存在急性或慢性去神经支配的肌电图表现。血清生化和免疫学研究M蛋白均为阴性。经过6个月的随访，尽管抗体滴度有所下降，但所有患者均无明显临床改善。
