OBJECTIVE Chronic inflammatory demyelinating polyradiculoneuropathies (CIDP) with antibodies against neurofascin 155 (Nfasc155) or contactin-1 (CNTN1) have distinctive clinical features. Knowledge on their electrophysiological characteristics is still scarce. In this study, we are investigating whether these patients have specific electrophysiological characteristics. METHODS The electrophysiological data from 13 patients with anti-Nfasc155 IgG4 antibodies, 9 with anti-CNTN1 IgG4 antibodies were compared with those of 40 consecutive CIDP patients without antibodies. RESULTS All the patients with antibodies against Nfasc155 or CNTN1 fulfilled the EFNS/PNS electrodiagnostic criteria for definite CIDP. There was no electrophysiological difference between patients with anti-CNTN1 and anti-Nfasc155 antibodies. Nerve conduction abnormalities were heterogeneously distributed along nerves trunks and roots. They were more pronounced than in CIDP without antibodies. Motor conduction velocity on median nerve <24 m/s or motor velocity on ulnar nerve <26 m/s or motor distal latency on ulnar nerve >7.4 ms were predictive of positive antibodies against the node of Ranvier with a sensitivity of 59% and a specificity of 93%. CONCLUSIONS Marked conduction abnormalities may suggest the presence of positive antibodies against the node of Ranvier. SIGNIFICANCE Anti-Nfasc155 and anti-CNTN1 antibodies target the the paranodal axo-glial domain but are associated with nerve conduction abnormalities mimicking a "demyelinating" neuropathy.

中文翻译：

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与靶向 Neurofascin 155 或 Contactin1 糖蛋白的 IgG4 抗体相关的慢性炎性脱髓鞘性多发性神经根神经病的电生理特征

目的 慢性炎症性脱髓鞘性多发性神经根神经病 (CIDP) 具有抗神经成束蛋白 155 (Nfasc155) 或 contactin-1 (CNTN1) 的抗体，具有独特的临床特征。关于它们的电生理特性的知识仍然很少。在这项研究中，我们正在调查这些患者是否具有特定的电生理特征。方法将13例抗Nfasc155 IgG4抗体患者、9例抗CNTN1 IgG4抗体患者的电生理数据与40例连续无抗体CIDP患者的电生理数据进行比较。结果所有具有Nfasc155或CNTN1抗体的患者均符合明确CIDP的EFNS/PNS电诊断标准。具有抗 CNTN1 和抗 Nfasc155 抗体的患者之间没有电生理差异。神经传导异常沿神经干和神经根分布不均。它们比没有抗体的 CIDP 更明显。正中神经运动传导速度 <24 m/s 或尺神经运动速度 <26 m/s 或尺神经运动远侧潜伏期 >7.4 ms 预测 Ranvier 结抗体阳性，敏感性为 59% 和特异性为 93%。结论 明显的传导异常可能表明存在针对 Ranvier 结的阳性抗体。意义 抗 Nfasc155 和抗 CNTN1 抗体靶向副结轴突神经胶质结构域，但与模拟“脱髓鞘”神经病变的神经传导异常相关。正中神经运动传导速度 <24 m/s 或尺神经运动速度 <26 m/s 或尺神经运动远侧潜伏期 >7.4 ms 预测 Ranvier 结抗体阳性，敏感性为 59% 和特异性为 93%。结论 明显的传导异常可能表明存在针对 Ranvier 结的阳性抗体。意义 抗 Nfasc155 和抗 CNTN1 抗体靶向副结轴突神经胶质结构域，但与模拟“脱髓鞘”神经病变的神经传导异常相关。正中神经运动传导速度 <24 m/s 或尺神经运动速度 <26 m/s 或尺神经运动远侧潜伏期 >7.4 ms 预测 Ranvier 结抗体阳性，敏感性为 59% 和特异性为 93%。结论 明显的传导异常可能表明存在针对 Ranvier 结的阳性抗体。意义 抗 Nfasc155 和抗 CNTN1 抗体靶向副结轴突神经胶质结构域，但与模拟“脱髓鞘”神经病变的神经传导异常相关。结论 明显的传导异常可能表明存在针对 Ranvier 结的阳性抗体。意义 抗 Nfasc155 和抗 CNTN1 抗体靶向副结轴突神经胶质结构域，但与模拟“脱髓鞘”神经病变的神经传导异常相关。结论 明显的传导异常可能表明存在针对 Ranvier 结的阳性抗体。意义 抗 Nfasc155 和抗 CNTN1 抗体靶向副结轴突神经胶质结构域，但与模拟“脱髓鞘”神经病变的神经传导异常相关。