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.
作者
董思语
孙美
陈聪
王静
金庆文
程虹
DONG Siyu;SUN Mei;CHEN Cong(Department of Neurology,the First Affiliated Hospital of Wannan Medical College,Wuhu 241001,China)
出处
《临床神经病学杂志》
CAS
2024年第1期37-40,共4页
Journal of Clinical Neurology
关键词
抗神经束蛋白抗体
颅神经损害
郎飞结
血浆交换
anti-neurofascin 186 antibody
cranial nerves injuries
Ranvier node
plasma exchange
