抗神经束蛋白186抗体阳性的郎飞结区/结旁疾病的临床特点(附1例报告)  

Clinical manifestations of nodo-paranodopathy with anti-neurofascin 186 antibody positive(report of one case)

作　　者：董思语 孙美 陈聪 王静[3] 金庆文 程虹[2] DONG Siyu;SUN Mei;CHEN Cong(Department of Neurology,the First Affiliated Hospital of Wannan Medical College,Wuhu 241001,China)

机构地区：[1]皖南医学院第一附属医院弋矶山医院神经内科,241001 [2]南京医科大学第一附属医院神经内科 [3]南京医科大学第一附属医院肾内科 [4]南京医科大学附属逸夫医院神经内科

出　　处：《临床神经病学杂志》2024年第1期37-40,共4页Journal of Clinical Neurology

摘　　要：目的 探讨抗神经束蛋白186(NF186)抗体阳性的郎飞结区/结旁疾病(NPP)的临床特点。方法 回顾性分析1例抗NF186抗体阳性的NPP致颅神经损害患者的临床资料。结果 患者为70岁男性,1个月前突发言语、吞咽困难,糖皮质激素治疗好转后停药。患者5 d前再次出现言语、吞咽、咀嚼、鼓腮、转头和抬头动作费力,并进行性加重。肢体EMG检查正常,血清及CSF抗NF186抗体阳性。给予糖皮质激素治疗疗效不明显,改用血浆交换治疗后症状明显改善。结论 抗NF186抗体阳性的NPP发病年龄较晚、病情较重,可伴颅神经受损,常规糖皮质激素治疗效果可能不佳,血浆交换治疗有效。Objective To investigate the clinical manifestations of nodo-paranodopathy(NPP)with antineurofascin 186(NF186)antibody positive.Methods The clinical data of a NPP patient with cranial nerve damage caused by anti-NF186 antibody positive was retrospectively analyzed.Results The patient was a 70-year-old male with sudden speech disorder and dysphagia one month ago.Glucocorticoid therapy was discontinued after improvement.The patient's speech,swallowing,chewing,bristling,turning and head-up movements were laborious and progressively aggravated 5 days ago.The EMG examination of the limbs was normal,and the serum and CSF anti-NF186 antibody were positive.The curative effect of glucocorticoid treatment was not obvious,and the symptoms were significantly improved after plasma exchange treatment.Conclusions Anti-NF186 antibody-positive NPP has late onset age,severe illness and accompanied with cranial nerves damage.Conventional hormone therapy is not effective,but plasma exchange therapy is effective.

关 键 词：抗神经束蛋白抗体 颅神经损害 郎飞结 血浆交换 

分 类 号：R741[医药卫生—神经病学与精神病学]