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鞘内注射利妥昔单抗治疗难治性非畸胎瘤抗 N-甲基-D-天冬氨酸受体脑炎成功:病例报告。

Successful Intrathecal Rituximab Administration in Refractory Nonteratoma Anti-N-Methyl-D-Aspartate Receptor Encephalitis: A Case Report.

机构信息

Questions or comments about this article may be directed to Maritsa Casares, BSN CNRN, at

出版信息

J Neurosci Nurs. 2019 Aug;51(4):194-197. doi: 10.1097/JNN.0000000000000450.

Abstract

N-methyl-D-aspartate receptor (NMDA-R) antibody encephalitis is an immune-mediated disorder characterized by the presence of anti-NMDA antibody in serum and cerebrospinal fluid, with a characteristic combination of psychological and neurological signs and symptoms. The scientific knowledge pertaining to the management of anti-NMDA-R encephalitis is growing. It is important that neuroscience nurses be aware of treatments as well as the newest novel treatment options available. Early aggressive intervention is imperative to recovery. The first line of treatment often includes high-dose steroids, intravenous immunoglobulin, and therapeutic plasma exchange. Second-line therapy for refractory NMDA-R encephalitis includes intravenous rituximab and cyclophosphamide. Even with these treatments, up to 25% of patients may be left with severe deficits or have a fatal outcome. It is well known that penetration of monoclonal anti-CD20 antibody therapy (rituximab) into the cerebrospinal fluid is 0.1% of that in the serum. Therefore, efficacy of rituximab in the treatment of NMDA encephalitis may be improved by intrathecal administration in selected cases with a poor response to intravenous rituximab. We present a case of anti-NMDA-R encephalitis that was refractory to first- and second-line therapies, who responded to intrathecal rituximab, to highlight a novel treatment that may be able to prevent long-term disability and improve clinical outcomes.

摘要

N-甲基-D-天冬氨酸受体(NMDA-R)抗体脑炎是一种免疫介导的疾病,其特征是血清和脑脊液中存在抗 NMDA 抗体,具有心理和神经症状和体征的特征组合。关于抗 NMDA-R 脑炎的管理的科学知识正在不断发展。神经科学护士了解治疗方法以及最新的新型治疗选择非常重要。早期积极干预对于恢复至关重要。一线治疗通常包括大剂量类固醇、静脉内免疫球蛋白和治疗性血浆置换。难治性 NMDA-R 脑炎的二线治疗包括静脉内利妥昔单抗和环磷酰胺。即使进行这些治疗,多达 25%的患者可能会留下严重的缺陷或出现致命的结果。众所周知,单克隆抗 CD20 抗体治疗(利妥昔单抗)进入脑脊液的浓度是血清中的 0.1%。因此,在对静脉内利妥昔单抗反应不佳的情况下,鞘内给予利妥昔单抗可能会改善 NMDA 脑炎的疗效。我们提出了一例抗 NMDA-R 脑炎病例,该病例对一线和二线治疗均有反应,对鞘内利妥昔单抗有反应,以强调一种可能能够预防长期残疾和改善临床结局的新型治疗方法。

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