首页|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)

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)

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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 5d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.
Clinical manifestations of nodo-paranodopathy with anti-neurofascin 186 antibody positive(report of one case)
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.

anti-neurofascin 186 antibodycranial nerves injuriesRanvier nodeplasma exchange

董思语、孙美、陈聪、王静、金庆文、程虹

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241001 皖南医学院第一附属医院弋矶山医院神经内科

南京医科大学第一附属医院神经内科

南京医科大学第一附属医院肾内科

南京医科大学附属逸夫医院神经内科

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抗神经束蛋白抗体 颅神经损害 郎飞结 血浆交换

2024

临床神经病学杂志
南京医科大学附属脑科医院

临床神经病学杂志

CSTPCD
影响因子:1.778
ISSN:1004-1648
年,卷(期):2024.37(1)
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