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[1例与神经节苷脂GM1和N-乙酰半乳糖胺-神经节苷脂GD1a抗体相关的慢性炎性脱髓鞘性多发性神经病]

[A case of chronic inflammatory demyelinating neuropathy associated with antibodies to gangliosides GM1 and GalNAc-GD1a].

作者信息

Tsukaguchi M, Yamada A, Deguchi K, Takeuchi H, Yuki N

机构信息

Third Department of Internal Medicine, Kagawa Medical University.

出版信息

Rinsho Shinkeigaku. 1997 Mar;37(3):228-32.

PMID:9217422
Abstract

In January 1993, a 43-year-old man was admitted to our hospital for left wrist-drop. Neurological examinations revealed asymmetrical distal weakness in the upper limbs. Deep tendon reflexes were normal in all 4 limbs. Sensory and autonomic nervous functions were intact. CSF examinations were within normal limits. Thin-layer chromatography with immunostaining revealed serum antibodies that reacted with GM1 and GalNAc-GD1a. Motor nerve conduction studies revealed abnormal temporal dispersion, and a low amplitude of compound muscle action potential in the left radial nerve. Neurological symptoms gradually improved with prednisolone over one and a half years. He was hospitalized again in January 1995, because of right wrist-drop and slight sensory loss of the limbs. Those findings were improved by methylprednisolone (1,000 mg/day) for 3 days. The interval until maximal disability in this patient was more than one month for each admission. This case must belong to inflammatory demyelinating neuropathy.

摘要

1993年1月,一名43岁男性因左腕下垂入住我院。神经学检查发现上肢远端不对称性无力。四肢的深腱反射均正常。感觉和自主神经功能完好。脑脊液检查结果正常。免疫染色薄层色谱显示血清抗体与GM1和GalNAc-GD1a发生反应。运动神经传导研究显示左桡神经存在异常的时间离散以及复合肌肉动作电位幅度降低。在使用泼尼松龙治疗一年半的时间里,神经学症状逐渐改善。1995年1月,他因右腕下垂和四肢轻微感觉丧失再次住院。甲基泼尼松龙(1000毫克/天)治疗3天后这些症状得到改善。该患者每次入院直至出现最大残疾的间隔时间均超过一个月。此病例肯定属于炎性脱髓鞘性神经病。

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BMJ Case Rep. 2011 Mar 29;2011:bcr1120103507. doi: 10.1136/bcr.11.2010.3507.