抗神经束蛋白155-IgG4抗体阳性的慢性炎性脱髓鞘性多发性神经根神经病临床特点分析  

Characteristics in Chronic Inflammatory Demyelinating Polyradiculoneuropathy Associated with Anti-neurofascin 155 IgG4 Antibodies

作　　者：孙翀 林洁 乔凯[2] 焦宇琼 刘炳佑 刘小妮[3] 陈向军 赵重波[1] 卢家红 SUN Chong;LIN Jie;QIAO Kai;JIAO Yu-qiong;LIU Bing-you;LIU Xiao-ni;CHEN Xiang-jun;ZHAO Chong-bo;LU Jia-hong(Deparment of Neurology,Huashan Hospital,Fudan University,Shanghai 200040,China;Department of Electromyography,Huashan Hospital,Fudan University,Shanghai 200040,China;Institute of Neurology,Huashan Hospital,Fudan University,Shanghai 200040,China)

机构地区：[1]复旦大学附属华山医院神经内科,上海200040 [2]复旦大学附属华山医院神经肌电图室,上海200040 [3]复旦大学神经病学研究所,上海200040

出　　处：《中国临床神经科学》2019年第4期411-418,共8页Chinese Journal of Clinical Neurosciences

摘　　要：目的分析抗神经束蛋白(NF) 155-IgG4抗体阳性的慢性炎性脱髓鞘性多发性神经根神经病(CIDP)患者的临床特点。方法回顾性分析2015至2018年在复旦大学附属华山医院就诊的10例经细胞免疫荧光法(CBA)明确诊断为抗NF155-IgG4抗体阳性CIDP患者的临床表现、实验室和影像学检查结果,并随访治疗效果。结果 10例抗NF155-Ig G4抗体阳性的CIDP患者平均起病年龄(27.80±11.84)岁,均为男性。Romberg征阳性10例(100%)患者中表现为远端无力、麻木为主的周围神经病8/10例(80%),伴肢体远端震颤7/10例(70%)。脑脊液蛋白平均(3 190.89±1 007.31) mg·L^-1。MRI示臂丛神经明显水肿增粗。免疫抑制剂治疗明显改善率:激素2/9例(22.2%),静脉注射丙种球蛋白1/5例(20%),美罗华2/2例(100%)。结论抗NF155-Ig G4抗体阳性的CIDP起病年龄早,男性多见。以远端麻木无力、深感觉障碍为主要表现,常伴有上肢远端姿位性震颤。脑脊液蛋白显著升高和臂丛神经水肿增粗是重要的辅助检查特点。Aim To analyze the characteristics of ten patients with chronic inflammatory demyelinating polyradiculoneuropathy( CIDP) associated with anti-neurofascin 155( NF155) IgG4 antibodies.Methods Ten patients with anti-NF155 IgG4 antibodies CIDP were accumulated in this cohort.Clinical features,laboratory exams,electrophysiological study and MR imaging were retrospectively analyzed.Results Ten patients were all male.Average onset age was( 27.80 ± 11.84) years old.Nine patients developed progressively and one patient was acute onset.100%( 10/10) patients had positive Romberg’s sign.80%( 8/10) patients presented distal weakness and numbness.The numbness in lower limbs was severer than upper limbs.70%( 7/10) patients presented distal tremor.The protein in cerebrospinal fluid was elevated, average( 3 190.89 ± 1 007.31) mg·L^-1.Electrophysiological study showed motor and sensory demyelinating polyneuropathy.Demyelination was found in all patients.Marked symmetric hypertrophy and edema of brachial and lumbosacral plexuses were observed by MR imaging.Patients had good response to rituximab and poor response to intravenous immunoglobulin and steroid.Conclusion CIDP patients with anti-NF155 IgG4 antibodies presented male dominant,young age onset,distal weakness and numbness,abnormality in deep sensory and distal postural tremor.Elevated protein in cerebrospinal fluid and hypertrophy and edema of the branchial plexuses were supportive evidences.

关 键 词：抗神经束蛋白155-IgG4抗体 慢性炎性脱髓鞘性多发性神经根神经病 临床特点 治疗 

分 类 号：R742[医药卫生—神经病学与精神病学]