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抗神经束蛋白 155 抗体相关格林-巴利综合征的临床特征。

Clinical features of Guillain-Barré syndrome with anti-neurofascin 155 antibody.

机构信息

Department of Neurology, Tianjin Neurological Institute, Tianjin Medical University General Hospital, Tianjin, China.

出版信息

Acta Neurol Scand. 2022 Nov;146(5):553-561. doi: 10.1111/ane.13678. Epub 2022 Aug 4.

Abstract

OBJECTIVE

Anti-neurofascin 155 (NF155) antibody has been discovered in chronic demyelinating conditions. However, the positive rate and clinical description were insufficient in acute demyelinating conditions, such as Guillain-Barré syndrome (GBS). This study aimed to explore the positive rate of anti-NF155 antibody in GBS patients and determine whether there were unique clinical characteristics in these patients.

MATERIALS & METHODS: Serum anti-NF155 antibody was detected from 94 GBS patients and 50 sex- and age-matched healthy controls using cell-based assay and tissue-based assay with immunostaining of mouse teased sciatic nerve fibers. Clinical characteristics, laboratory data, and electrophysiology examinations were retrospectively collected.

RESULTS

Seven of 94 (7.45%) GBS patients were positive for anti-NF155 antibody, and the main IgG subclass was IgG1. Compared with anti-NF155 antibody-negative GBS patients, anti-NF155 antibody-positive GBS patients had a higher GBS disability score at nadirs (p = .010), higher modified Erasmus GBS outcome score (p = .022), higher rate of abnormal compound motor action potential (CMAP) amplitude (p = .002), higher frequency of prolonged F-wave latency (p < .001), lower frequency of abnormal sensory conduction velocity (p < .001) and sensory nerve action potential amplitude (p < .001), more axonal type (p = .040), and poorer therapeutic effect (p = .017).

CONCLUSIONS

Anti-NF155 antibody exists in a small portion of GBS patients. Anti-NF155 antibody-positive GBS patients possibly have a more severe clinical course, less sensory nerves involved, higher proportion of axonal type, poorer therapeutic effect, and worse prognosis, but the pathogenicity of the anti-NF155 antibody in GBS needs further study.

摘要

目的

抗神经束蛋白 155(NF155)抗体已在慢性脱髓鞘疾病中被发现。然而,在急性脱髓鞘疾病(如吉兰-巴雷综合征(GBS))中,其阳性率和临床描述尚不足。本研究旨在探讨 GBS 患者中抗 NF155 抗体的阳性率,并确定这些患者是否存在独特的临床特征。

材料与方法

采用基于细胞和基于组织的免疫染色法,检测了 94 例 GBS 患者和 50 名性别和年龄匹配的健康对照者血清中的抗 NF155 抗体。回顾性收集了临床特征、实验室数据和电生理学检查结果。

结果

94 例 GBS 患者中有 7 例(7.45%)抗 NF155 抗体阳性,主要 IgG 亚类为 IgG1。与抗 NF155 抗体阴性的 GBS 患者相比,抗 NF155 抗体阳性的 GBS 患者在疾病高峰时的 GBS 残疾评分更高(p=0.010),改良 Erasmus GBS 结局评分更高(p=0.022),异常复合运动动作电位(CMAP)振幅的发生率更高(p=0.002),F 波潜伏期延长的发生率更高(p < 0.001),感觉传导速度异常和感觉神经动作电位振幅异常的发生率更低(p < 0.001),轴索性病变的发生率更高(p=0.040),治疗效果更差(p=0.017)。

结论

抗 NF155 抗体存在于一小部分 GBS 患者中。抗 NF155 抗体阳性的 GBS 患者可能具有更严重的临床病程,感觉神经受累较少,轴索性病变比例较高,治疗效果较差,预后较差,但抗 NF155 抗体在 GBS 中的致病性仍需进一步研究。

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