摘要目的:探讨抗郎飞结区和结旁区相关抗体在慢性炎症性脱髓鞘性多发性神经根神经病（CIDP）的检测及其临床特征。方法:收集2018年1月至2021年7月在复旦大学附属华山医院住院和门诊就诊及外院送检的212例临床诊断CIDP患者血清样本，采用细胞底物法检测抗郎飞氏结区和结旁区相关自身抗体（抗NF155、抗NF186、抗CNTN1抗体）和IgG亚型。根据检测结果分为抗NF155抗体阳性、抗NF186抗体阳性和抗CNTN1抗体阳性组，回顾性分析各组患者肢体无力、深浅感觉异常、震颤、脑脊液蛋白水平、臂丛核磁共振（MRI）等临床特点，并进行对比。结果:共发现抗NF155抗体阳性患者23例（10.8%，23/212），抗NF186抗体阳性12例（5.7%，12/212），抗CNTN1抗体阳性4例（1.9%，4/212）。抗NF155抗体阳性组和抗CNTN1抗体阳性组抗体均为IgG4亚型。抗NF186抗体阳性组（12例）患者IgG均阳性，4例可检测到抗体亚型，但优势亚型不明。抗NF155抗体阳性组（23例）患者均有肢体无力和深感觉障碍，19例有（19/23，82.6%）浅感觉障碍，22例（95.7%，22/23）为对称性受累，18例（78.3%，18/23）有震颤；19例行臂丛MRI，均为异常。抗NF186抗体阳性组（12例）患者均有肢体无力，浅感觉和深感觉障碍分别有9和6例，8例为不对称性受累，仅1例出现震颤；7例行臂丛MRI，1例异常。抗CNTN1抗体阳性组（4例）患者均表现为对称性肢体无力和深浅感觉障碍，3例有震颤，4例均有臂丛MRI异常。抗NF155抗体阳性组、抗NF186抗体阳性组和抗CNTN1抗体阳性组在发病年龄［分别为（25.8±13.8）、（43.4±20.7）和（61.2±5.1）岁］、深感觉异常、震颤和臂丛MRI异常上的差异均有统计学意义（ P均<0.01）。 结论:抗NF155、抗NF186、抗CNTN1抗体阳性CIDP患者在发病年龄、深感觉异常、震颤和臂丛MRI异常等临床特征上有差异。

abstractsObjective:To investigate the detecting method and clinical characteristics of anti-nodal/paranodal antibodies in chronic inflammatory demyelinating polyradiculopathy.Methods:Serum samples were collected from 212 patients with chronic inflammatory demyelinating polyradiculopathy who were admitted to Huashan Hospital of Fudan University or from other clinical centers from January 2018 to July 2021. Autoantibodies (anti-NF155, anti-NF186, anti-CNTN1) and IgG subtypes were detected with cell-based assay. According to the test results, patients were divided into anti-NF155 positive group, anti-NF186 positive group and anti-CNTN1 positive group, clinical characteristics of patients in each group, including limb weakness, superficial sensation and proprioception, tremor, cerebrospinal fluid protein level, brachial plexus magnetic resonance (MRI) were retrospectively analyzed and compared.Results:A total of 23 patients (10.8%,23/212) were positive for anti-NF155 antibody, 12 (5.7%,12/212) for anti-NF186 antibody, and 4 (1.9%,4/212) for anti-CNTN1 antibody. IgG 4 was the predominant subtype in anti-NF155 and anti-CNTN1 groups. In the anti-NF186 group, all cases were IgG positive and antibody subtypes could be detected in 4 cases (4/12). In anti-NF155 group, 23 patients (100%,23/23) had limb weakness and deep sensory disturbance, 19 patients (82.6%,19/23) had superficial sensory disturbance, 22 patients (95.7%,22/23) were symmetrically involved, 18 patients (78.3%,18/23) showed tremor, 19 patients (19/19) showed abnormal in brachial plexus MRI. In anti-NF186 group, 12 patients had limb weakness (12/12), 9 patients (9/12) and 6 patients (6/12) had superficial sensory disturbance and deep sensory disturbance respectively, 8 patients (8/12) were asymmetrically involved, and only 1 patient (1/12) showed tremor, 1 (1/7) showed abnormal brachial plexus MRI. In anti-CNTN1 group, 4 cases showed symmetrical limb weakness and sensory disturbance, 3 patients had tremor, and four patients showed brachial plexus MRI abnormality. There were statistically significant differences in onset age, proprioception, tremor and MRI abnormalities of brachial plexus among the 3 groups ( P<0.01). Conclusions:The clinical characteristics of CIDP patients with anti-NF155, anti-NF186 and anti-CNTN1 antibodies are different. Screening anti-nodal/paranodal antibodies is of great significance for accurate diagnosis and treatment of patients with peripheral neuropathy.