中国现代神经疾病杂志 ›› 2013, Vol. 13 ›› Issue (4): 330-333. doi: 10.3969/j.issn.1672-6731.2013.04.015

• 临床研究 • 上一篇    下一篇

2 单唾液酸四己糖神经节苷脂相关性吉兰-巴雷综合征:三例报告并文献复习

蒋科, 王学峰, 曾可斌   

  1. 400016 重庆医科大学附属第一医院神经内科[蒋科(现在四川省遂宁市第一人民医院神经内科,邮政编码:629000)]
  • 出版日期:2013-04-25 发布日期:2013-05-01
  • 通讯作者: 曾可斌 (Email:zkebin2008@yahoo.com.cn)

Guillain-Barré syndrome associated with monosialotetrahexosylganglioside: three cases study and literature review

JIANG Ke, WANG Xue-feng, ZENG Ke-bin   

  1. Department of Neurology, the First Affiliated Hospital of Chongqing Medical University, Chongqing 400016, China
  • Online:2013-04-25 Published:2013-05-01
  • Contact: ZENG Ke-bin (Email: zkebin2008@yahoo.com.cn)

摘要: 研究背景 单唾液酸四己糖神经节苷脂钠(神经节苷脂)注射液系从猪脑中提取获得的神经细胞功能物质,参与多种细胞病理生理过程,在神经组织发生、生长、分化过程中发挥重要作用。神经节苷脂对血管源性脑损伤、创伤性脑脊髓损伤神经元的恢复具有促进和保护作用,可改善帕金森病行为障碍。但随着临床的广泛应用,其不良反应逐渐被发现,其中注射神经节苷脂后并发的类似吉兰-巴雷综合征表现的病例较为少见,其临床特点和机制尚需进一步总结和探讨。方法 分析3 例神经节苷脂治疗后发生吉兰-巴雷综合征患者的临床、神经电生理及脑脊液特征。结果 患者均为39~65岁男性,分别于静脉注射神经节苷脂后9 ~ 14d 出现四肢瘫痪、肌张力减退及腱反射消失等肌肉病症状与体征,可伴呼吸困难(1 例)或脑脊液蛋白-细胞分离现象(1 例)或周围神经轴索变性(1 例)等表现,符合神经节苷脂相关性吉兰-巴雷综合征诊断。结论 单唾液酸四己糖神经节苷脂静脉注射后可诱发吉兰-巴雷综合征,多发生于静脉注射后9 ~ 14d ,患者预后不良。其可能机制与外源性神经节苷脂通过诱导免疫机制导致神经轴索变性有关。

关键词:  格林-巴利综合征, 神经节苷脂类, 药物毒性, 药物过敏

Abstract: Background  Monosialotetrahexosylganglioside (GM1) is a kind of ganglioside extracted from the neural cells of pig brain, which involves in the pathophysiological processes of neurogenesis, and plays an important role in neural formation, growth and differentiation. GM1 is widely used in the treatment for vascular brain injury and traumatic brain and spinal cord injury, promoting and protecting the recovery of nerve cells. Besides, it can improve the behavior disorders of patients with Parkinson's disease. However, as it is widely used in clinical practice, its adverse reaction has been gradually discovered. There is some evidence to suggest Guillain-Barré syndrome (GBS) may occur after GM1 injection intravenously. Both clinical manifestations and possible mechanism of GBS associated with GM1 are unclear, and need further study. Methods  Three cases of GBS associated with GM1 were clinically observed including cerebrospinal fluid (CSF) testing and nerve conduction studies. These cases were analyzed and subjected to assessment with literature review. Results  Three male patients (with the age 39-65) of GBS were observed after injection of GM1 intravenously. At 9-14 days, they developed weakness of all limbs and were unable to stand upright with decreased muscle tone in limbs and absent deep tendon reflexes, accompanied by dyspnea (1 case), albuminocytological dissociation (1 case) and axonal degeneration of peripheral nerve (1 case). Conclusion  GBS may occur occasionally in patients treated with GM1 injection intravenously for 9-14 days, and the prognosis is not favorable. The possible mechanism is that exogenous gangliosides could be immunogenic and may occasionally result in neural axonal degeneration.

Key words: Guillain-Barré, syndrome, Gangliosides, Drug toxicity, Drug hypersensitivity