摘要目的:分析抗神经束蛋白（NF）155 IgG4抗体阳性（抗NF155阳性）的慢性炎性脱髓鞘性多发性神经根神经病（CIDP）患者的电生理特点。方法:回顾性分析2018年9月至2021年5月在首都医科大学附属北京天坛医院确诊的6例抗NF155阳性CIDP患者，对每例均行周围神经运动传导、感觉传导、针极肌电图检测，并探讨其临床指标与电生理参数的相关性。结果:胫神经运动传导各项检测异常率总体上差异有统计学意义（χ2=11.08， P=0.011），各项检测异常中以运动神经传导速度（MCV）减慢和末端运动潜伏期（DML）延长最多见。下肢神经传导总异常率（30/32，93.8%）与上肢神经传导总异常率（22/22，100.0%）、感觉神经传导总异常率（52/54，96.3%）与运动神经传导总异常率（42/42，100.0%）差异无统计学意义（χ2=1.43， P=0.508；χ2=1.59， P=0.503）。正中神经、尺神经和腓肠神经感觉神经各项检测异常率总体上差异有统计学意义（χ2=14.96， P=0.001；χ2=10.00， P=0.007；χ2=9.95， P=0.008），感觉神经传导异常以感觉神经动作电位未引出最多见。针极肌电图检查异常率为9/14，上、下肢肌肉异常率分别为4/8和5/6，主要表现为自发电位合并运动单位动作电位时限增宽波幅升高。病程与尺神经复合肌肉动作电位（CMAP）波幅和负波面积均呈负相关（ r=-0.84， P=0.036； r=-0.76， P=0.011）；年龄与正中神经MCV和尺神经MCV均呈正相关（ r=0.89， P=0.019； r= 0.95， P=0.003）。正中神经DML与CMAP波幅呈负相关（ r=-0.63， P=0.049），胫神经F波潜伏期与胫神经DML、CMAP波幅和MCV显著相关（ r= 0.90， P=0.039； r=-0.96， P=0.012； r=-0.96， P=0.010）。 结论:抗NF155阳性CIDP患者运动和感觉神经、髓鞘和轴索均受累。尺神经CMAP波幅和负波面积对病程判断具有一定临床意义。年龄越小，上肢神经脱髓鞘程度越重。胫神经F波潜伏期不仅反映近端病变程度，还可反映远端神经脱髓鞘与轴索病变。

abstractsObjective:To investigate the electrophysiological features of patients with anti-neurofascin 155 (NF155) IgG4 antibodies positive chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).Methods:The electrophysiological data of 6 anti-NF155 IgG4 antibodies positive CIDP patients in Beijing Tiantan Hospital, Capital Medical University from September 2018 to May 2021 were retrospectively analyzed. Parameters studies included standard motor and sensory nerve conduction studies, electromyography (EMG) and F waves studies. Correlation analysis was made to explore the relationships between clinical indicators and electrophysiological data.Results:There was statistically significant difference in the motor nerve conduction study on abnormal rate of tibial nerve (χ 2=11.08, P=0.011). Motor nerve conduction abnormalities were presented in a majority of patients with decreased motor conduction velocity (MCV) and prolonged distal motor latency (DML). There was no statistically significant difference in the overall abnormal rate between lower limbs and upper limbs (30/32, 93.8% vs 22/22, 100.0%; χ 2=1.43, P=0.508), sensory nerve conduction and motor nerve conduction (52/54, 96.3% vs 42/42, 100.0%; χ 2=1.59, P=0.503). There was statistically significant difference in the overall abnormal rate in median nerve, ulnar nerve and sural nerve (χ 2=14.96, P=0.001;χ 2=10.00, P=0.007; χ 2=9.95, P=0.008),and absent sensory nerve action potential was the most common abnormality in sensory nerve conduction abnormalities. The abnormal rate of EMG was 9/14, which was constituted by upper limbs (4/8) and lower limbs (5/6), and the spontaneous activity accompanied with prolonged duration and increased amplitude of motor unit action potential was common. Significant negative correlations were established between disease duration and compound muscle action potential (CMAP) amplitude and negative area of ulnar nerve ( r=-0.84, P=0.036; r =-0.76, P=0.011), and the correlations between age and MCV of median nerve and ulnar nerve were also found ( r=0.89, P=0.019; r=0.95, P=0.003). The DML of median nerve was negatively correlated with CMAP amplitude ( r=-0.63, P=0.049). Moreover, the correlation analysis revealed associations of the F wave latency with the DML, CMAP amplitude and MCV of tibial nerve ( r= 0.90, P=0.039; r=-0.96, P=0.012; r=-0.96, P=0.010). Conclusions:The motor nerve and sensory nerve, the myelin sheath and axon of peripheral nerves in anti-NF155 IgG4 CIDP patients were largely affected. The CMAP amplitude and negative area of ulnar nerve might be of certain clinical value in reflecting the disease duration.The younger the patients, the more severe the demyelination degree of the upper limbs. The F wave latency of tibial nerve not only reflected the abnormal proximal conduction, but also the distal damage degree of myelin sheath and axon.