Rudolf Magnus Institute of Neuroscience, Department of Neurology, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX Utrecht, The Netherlands.
Neurology. 2010 Nov 30;75(22):1961-7. doi: 10.1212/WNL.0b013e3181ff94c2. Epub 2010 Oct 20.
To determine the prevalence and specificity of antibodies against single gangliosides and ganglioside complexes in serum from 88 patients with multifocal motor neuropathy (MMN) and to study the association with clinical features.
ELISA was used to detect immunoglobulin (Ig)M, IgG, and IgA antibodies against GM1, GM2, GD1a, GD1b, GM1b, GT1a, GT1b, GQ1b, GalNAc-GD1a, and the glycolipid SGPG; absorption studies were performed to study cross-reactivity. Presence of antibodies against ganglioside complexes consisting of any of combinations of GM1, GM2, GD1a, GD1b, GT1b, and GQ1b was also tested.
Anti-GM1 IgM, IgG, and IgA antibodies were detected in serum from 43%, 1%, and 5% of patients with MMN. Anti-GM2 IgM antibodies were detected in 6% and anti-GD1b IgM antibodies in 9% of patients. Patients with MMN with anti-GM1 IgM antibodies had more severe weakness (p < 0.01), more disability (p < 0.01), and more axon loss (p = 0.05) than patients without anti-GM1 IgM antibodies. Anti-GM1 IgM antibody titers correlated with Medical Research Council scores (correlation coefficient = 0.43; p < 0.0001). Anti-GD1b IgM antibody activity was associated with reduced vibration sense (p < 0.01). Absorption studies showed that anti-GD1b and anti-GM2 IgM antibodies cross-reacted with GM1. Antibodies against ganglioside complexes were not detected. Complexes containing GD1a, GD1b, GT1b, or GQ1b with GM1 lowered antibody activity against GM1.
Anti-ganglioside IgM antibodies in MMN display limited specificity and are associated with severity and clinical characteristics. Results of this study suggest that anti-GM1 IgM antibodies may play a role in MMN pathogenesis.
确定 88 例多灶性运动神经病（MMN）患者血清中针对单个神经节苷脂和神经节苷脂复合物的抗体的流行率和特异性，并研究其与临床特征的关系。
采用 ELISA 法检测 GM1、GM2、GD1a、GD1b、GM1b、GT1a、GT1b、GQ1b、GalNAc-GD1a 和神经节苷脂 SGPG 的免疫球蛋白（Ig）M、IgG 和 IgA 抗体；进行吸收研究以研究交叉反应。还检测了由 GM1、GM2、GD1a、GD1b、GT1b 和 GQ1b 的任意组合组成的神经节苷脂复合物的抗体存在情况。
在 43%、1%和 5%的 MMN 患者血清中检测到抗 GM1 IgM、IgG 和 IgA 抗体。在 6%的 MMN 患者中检测到抗 GM2 IgM 抗体，在 9%的 MMN 患者中检测到抗 GD1b IgM 抗体。与无抗 GM1 IgM 抗体的 MMN 患者相比，有抗 GM1 IgM 抗体的 MMN 患者的肌无力更为严重（p < 0.01）、残疾更为严重（p < 0.01）和轴索丢失更多（p = 0.05）。抗 GM1 IgM 抗体滴度与医学研究委员会评分相关（相关系数 = 0.43；p < 0.0001）。抗 GD1b IgM 抗体活性与振动觉降低有关（p < 0.01）。吸收研究表明，抗 GD1b 和抗 GM2 IgM 抗体与 GM1 发生交叉反应。未检测到针对神经节苷脂复合物的抗体。含有 GD1a、GD1b、GT1b 或 GQ1b 与 GM1 的复合物降低了针对 GM1 的抗体活性。
MMN 中的抗神经节苷脂 IgM 抗体显示出有限的特异性，并与严重程度和临床特征相关。本研究结果表明，抗 GM1 IgM 抗体可能在 MMN 的发病机制中起作用。
