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

VernacularTitle: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)
Author: Siyu DONG ¹ ; Mei SUN ; Cong CHEN
Author Information

1. 241001 皖南医学院第一附属医院弋矶山医院神经内科
Keywords: anti-neurofascin 186 antibody; cranial nerves injuries; Ranvier node; plasma exchange
From: Journal of Clinical Neurology 2024;37(1):37-40
CountryChina
Language:Chinese
Abstract: Objective To investigate the clinical manifestations of nodo-paranodopathy(NPP)with anti-neurofascin 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.