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伴有抗N-乙酰半乳糖胺-神经节苷脂1a抗体的快速进展型、以运动症状为主的吉兰-巴雷综合征

Rapidly progressive, predominantly motor Guillain-Barré syndrome with anti-GalNAc-GD1a antibodies.

作者信息

Ang C W, Yuki N, Jacobs B C, Koga M, Van Doorn P A, Schmitz P I, Van Der Meché F G

机构信息

Department of Neurology, Erasmus University Medical Centre Rotterdam, The Netherlands.

出版信息

Neurology. 1999 Dec 10;53(9):2122-7. doi: 10.1212/wnl.53.9.2122.

DOI:10.1212/wnl.53.9.2122
PMID:10599792
Abstract

OBJECTIVE

To investigate the presence of anti-GalNAc-GD1a antibodies in patients with Guillain-Barré syndrome (GBS) and to determine the relation of anti-ganglioside antibodies with clinical features.

BACKGROUND

The GBS is heterogeneous with regard to clinical manifestations, antecedent infections, and the presence and specificity of anti-ganglioside antibodies. Recently, antibodies to minor gangliosides have been identified in serum from GBS patients.

METHODS

The authors used ELISA to detect anti-ganglioside antibodies in 132 GBS patients and then correlated results with a database containing information on antecedent infections and clinical parameters.

RESULTS

Anti-GalNAc-GD1a antibodies could be detected in 19 (14%) GBS patients. The presence of anti-GalNAc-GD1a antibodies was related to antecedent Campylobacter jejuni infection (p<0.001). GBS patients with anti-GalNAc-GD1a antibodies had a rapidly progressive, more severe, and predominantly distal weakness. Furthermore, they had less sensory loss, paresthesia, and cranial nerve involvement. In most patients, this reactivity was independent of reactivity to GM1. Dividing patients into separate groups based on their reactivity to GalNAc-GD1a and GM1 enabled the authors to delineate more homogeneous subgroups with regard to clinical features.

CONCLUSIONS

This study provides further evidence for the hypothesis that antecedent infections and the specificity of subsequent anti-neural antibody responses determine the clinical manifestations in GBS patients.

摘要

目的

研究吉兰-巴雷综合征(GBS)患者中抗GalNAc-GD1a抗体的存在情况,并确定抗神经节苷脂抗体与临床特征的关系。

背景

GBS在临床表现、前驱感染以及抗神经节苷脂抗体的存在和特异性方面具有异质性。最近,在GBS患者血清中发现了针对次要神经节苷脂的抗体。

方法

作者使用酶联免疫吸附测定(ELISA)检测了132例GBS患者的抗神经节苷脂抗体,然后将结果与一个包含前驱感染和临床参数信息的数据库进行关联。

结果

19例(14%)GBS患者可检测到抗GalNAc-GD1a抗体。抗GalNAc-GD1a抗体的存在与空肠弯曲菌前驱感染有关(p<0.001)。具有抗GalNAc-GD1a抗体的GBS患者病情进展迅速、更严重,且主要为远端肌无力。此外,他们的感觉丧失、感觉异常和颅神经受累较少。在大多数患者中,这种反应性与对GM1的反应性无关。根据患者对GalNAc-GD1a和GM1的反应性将患者分为不同组,使作者能够在临床特征方面描绘出更同质的亚组。

结论

本研究为以下假设提供了进一步证据,即前驱感染和随后抗神经抗体反应的特异性决定了GBS患者的临床表现。

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