GM1/GalNAc-GD1a complex A target for pure motor Guillain-Barre syndrome
GM1/GalNAc-GD1a complex A target for pure motor Guillain-Barre syndrome
复制标题
DOI：
10.1212/01.wnl.0000335160.72184.7d
复制
发表时间：
2008-11-18
期刊：
影响因子：
9.9
通讯作者：
Kusunoki, S.
中科院分区：
文献类型：
--
作者：
Kaida, K.;Sonoo, M.;Kusunoki, S.
背景：GM1和GalNAc-GD1a位于运动神经轴膜上，被认为是与单纯运动性格林-巴利综合征(GBS)相关的抗原。此外，GM1和GalNAc-GD1a可能存在于轴膜附近并共存于轴膜上。与GM1或GalNAc-GD1a相关的神经节苷脂复合体(GSC)抗原可作为纯运动性GBS的靶抗原。方法：检测224例GBS患者血清中由GM1、GM2、GM3、GD1a、GD3、GD1a、GD3、GT1a、GT1a、GT1b、GQ1b和GalNAc-GD1a等9种神经节苷脂组成的GSC抗体。我们分析了GM1/GalNAc-GD1a复合体抗体阳性患者的临床和电生理特征。结果：10例GBS患者存在GM1/GalNAc-GD1a复合体抗体。10例GBS患者的临床表现以感觉系统保留和罕见的脑神经损伤为特征。根据Hadden等人建立的标准，电诊断研究显示4名患者有脱髓鞘模式，2名患者有轴索神经病模式。结论：GM1和GalNAc-GD1a可能在运动神经兰维尔结节或副神经节的轴膜上形成复合体，可能是单纯运动性格林-巴利综合征的靶抗原，尤其是急性运动传导阻滞神经病。神经病学(R)2008；71：1683-1690
Background: GM1 and GalNAc-GD1a are located on the axolemma of the motor nerves and are believed to be the antigens associated with pure motor Guillain-Barre syndrome (GBS). Furthermore, GM1 and GalNAc-GD1a may exist nearby and colocalize on the axolemma. Ganglioside complex ( GSC) antigens associated with GM1 or GalNAc-GD1a can be target antigens in pure motor GBS. We investigated GBS sera for antibodies to a GSC consisting of GM1 and GalNAc-GD1a ( GM1/GalNAc-GD1a) and analyzed the clinical and electrophysiologic findings of patients with antibodies to GM1/GalNAc-GD1a.Methods: Sera from 224 patients with GBS were surveyed for antibodies to GSCs consisting of two of nine gangliosides ( GM1, GM2, GM3, GD1a, GD3, GT1a, GT1b, GQ1b, and GalNAc-GD1a). We analyzed the clinical and electrophysiologic features of patients with IgG antibodies to the GM1/GalNAc-GD1a complex.Results: Ten patients with GBS had IgG antibodies to the GM1/GalNAc-GD1a complex. The clinical findings of the 10 patients with GBS were characterized by preserved sensory system and infrequent cranial nerve deficits. According to the criteria established by Hadden et al., electrodiagnostic studies showed a demyelinating pattern in four patients and axonal neuropathy pattern in two. Early motor conduction block at intermediate nerve segments was found in five patients.Conclusions: GM1 and GalNAc-GD1a may form a complex in the axolemma at nodes of Ranvier or paranodes of the motor nerves, and may be a target antigen in pure motor Guillain-Barre syndrome, especially in the form of acute motor conduction block neuropathy. Neurology (R) 2008;71:1683-1690