Abstract: Objective To summarize the clinical, laboratory, imaging, electrophysiological and histopathological features and therapeutic responses of patients with paranodopathy with positive anti-neurofascin 155 (NF155) antibody. Methods and Results Five patients who visited Xuanwu Hospital, Capital Medical University in January 2017 to August 2020 with positive anti-NF155 antibody, were men with onset ages 10 to 61 years old. The clinical manifestations were subacute onset, gradual progress, distal limb weakness, numbness, tremor and ataxia. The distal limbs were more severe than the proximal and lower limbs were more severe than the upper. Five patients were positive NF155-IgG4. Cerebrospinal fluid protein was significant increased. There were cervical and lumbosacral nerve roots enlargement. The distal latency of compound muscle action potential (CMAP) prolonged, the conduction velocity decreased, and the F-wave latency extended. Sural nerve biopsy showed axonal degeneration, the Ranvier node widened, the transverse band disappeared, and the space between the myelin loop and axon increased without inflammatory cell infiltration and "onion bulb" structure. Glucocorticoid therapy and intravenous immunoglobulin (IVIg) were not effective or initially effective, then failed, while rituximab was effective. Conclusions Patients with positive anti-NF155 antibody paranodopathy showed heterogeneity in clinical characteristics and treatment. Early stage should do antibody examination and diagnosis. Early treatment could prevent myelin and axonal injury and preserve nerve function.

Key words: Polyradiculoneuropathy, chronic inflammatory demyelinating, Ranvier's nodes, Antibodies

摘要： 目的 总结抗NF155抗体阳性的结旁病患者的临床、实验室、影像学、神经电生理学和组织病理学特点以及治疗反应。方法与结果 纳入2017年1月至2020年8月首都医科大学宣武医院收治的5例抗NF155抗体阳性的结旁病患者,均为男性,发病年龄10~61岁,亚急性发病,逐渐进展,临床表现为四肢远端无力、麻木、震颤、共济失调,远端重于近端、下肢重于上肢;血清NF155-IgG4阳性,脑脊液蛋白定量升高;颈神经根和腰骶丛神经根增粗;远端复合肌肉动作电位潜伏期延长、传导速度下降,F波潜伏期延长;腓肠神经纤维轴索变性,郎飞结增宽、横带消失、髓袢与轴突间隙增大,未见炎性细胞浸润和"洋葱球"样结构;激素和静脉注射免疫球蛋白无效或起初有效、后失效,利妥昔单抗有效。结论 抗NF155抗体阳性的结旁病患者临床特点和治疗反应均异质性较大,早期诊断、及时治疗十分重要,可以阻止髓鞘和轴索损伤,保留神经功能。

关键词: 多发性神经根性神经病, 慢性感染性脱髓鞘性, 郎飞结, 抗体