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无症状性进行性多灶性白质脑病:一例报告并文献复习

Asymptomatic progressive multifocal leukoencephalopathy: a case report and review of the literature.

作者信息

Zhang Yinan, Wright Crystal, Flores Angela

机构信息

Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX, 75390, USA.

出版信息

J Med Case Rep. 2018 Jul 1;12(1):187. doi: 10.1186/s13256-018-1727-7.

Abstract

BACKGROUND

We report the development of asymptomatic progressive multifocal leukoencephalopathy in a patient with multiple sclerosis on natalizumab therapy. Progressive multifocal leukoencephalopathy often presents with debilitating neurologic symptoms. Very few cases have documented a completely asymptomatic course of the disease.

CASE PRESENTATION

A 26-year-old white woman with multiple sclerosis was treated with natalizumab. She was diagnosed as having progressive multifocal leukoencephalopathy based on characteristic magnetic resonance imaging lesions after 27 infusions of natalizumab. She had no neurologic deficits at the time of diagnosis and John Cunningham virus in cerebrospinal fluid was detected at 15 copies/ml. She was initially treated with mefloquine and mirtazapine and remained asymptomatic for 3 months. She later developed worsening magnetic resonance imaging lesions related to immune reconstitution inflammatory syndrome. At that time, she received intravenously administered immunoglobulin and high-dose intravenously administered methylprednisolone with radiologic improvement of the lesions.

CONCLUSIONS

Our case report illustrates that early detection of asymptomatic progressive multifocal leukoencephalopathy and its subsequent treatment resulted in a benign clinical course. In consideration of the additional small number of cases of asymptomatic progressive multifocal leukoencephalopathy that have been reported, we conclude that routine magnetic resonance imaging surveillance is important for patients with multiple sclerosis who are at high risk for developing natalizumab-associated progressive multifocal leukoencephalopathy.

摘要

背景

我们报告了1例接受那他珠单抗治疗的多发性硬化症患者发生无症状性进行性多灶性白质脑病的情况。进行性多灶性白质脑病通常会出现使人衰弱的神经系统症状。记录到疾病完全无症状病程的病例非常少。

病例报告

1例26岁的白人女性多发性硬化症患者接受了那他珠单抗治疗。在输注那他珠单抗27次后,基于特征性磁共振成像病变,她被诊断为进行性多灶性白质脑病。诊断时她没有神经功能缺损,脑脊液中检测到约翰·坎宁安病毒,拷贝数为每毫升15个。她最初接受了甲氟喹和米氮平治疗,并且3个月内一直无症状。她后来出现了与免疫重建炎症综合征相关的磁共振成像病变恶化。当时,她接受了静脉注射免疫球蛋白和大剂量静脉注射甲泼尼龙治疗,病变在影像学上有所改善。

结论

我们的病例报告表明,无症状性进行性多灶性白质脑病的早期检测及其后续治疗导致了良性的临床病程。考虑到已报告的无症状性进行性多灶性白质脑病的其他少数病例,我们得出结论,对于有发生那他珠单抗相关进行性多灶性白质脑病高风险的多发性硬化症患者,常规磁共振成像监测很重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2b9c/6026501/a0036b84b249/13256_2018_1727_Fig1_HTML.jpg

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