Elsevier

Journal of Neuroimmunology

Volume 349, 15 December 2020, 577365
Journal of Neuroimmunology

Short Communication
Cerebrospinal fluid CD20 positive B-cell expansion in a case of anti-NMDAR encephalitis

https://doi.org/10.1016/j.jneuroim.2020.577365Get rights and content

Highlights

  • CSF CD20+ cells increased in an anti-NMDAR encephalitis.

  • Low dose of rituximab was used in this patient.

  • These findings support the use of rituximab.

Abstract

Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is a potentially fatal autoimmune encephalitis with a strong B-cell response. We measured the proportion of CD20 positive and CD19 positive B-cells in the CSF from a case of severe anti-NMDAR encephalitis. The proportion of CD20 positive B-cells in the CSF was 15.0%, higher than CD19 positive B cells (10.1%), and higher than that seen in non-inflammatory neurological disorders (<1%). After the treatments of steroids, intravenous immunoglobulin and rituximab to eliminate B-cells, she recovered. These findings further support the use of rituximab that targets CD20 positive B-cells in anti-NMDAR encephalitis.

Introduction

Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is a potentially fatal autoimmune encephalitis, characterized by abnormal behavior or cognitive dysfunction, seizures, abnormal movements, or coma, and defined by the presence of CSF antibodies that bind to the GluN1 subunit of NMDAR (Dalmau et al., 2019). Increasing recognition of the role of B cells in the adaptive immune response makes B-cells an important therapeutic target in autoimmunity. A strong B-cell response is detected in anti-NMDAR encephalitis (Dale et al., 2013; Zhang et al., 2019; Martinez-Hernandez et al., 2011). Rituximab, targeting CD20 positive B-cells, is one of the second-line treatments against anti-NMDAR encephalitis (Dalmau et al., 2019). The potential differences between drugs targeting CD19 and CD20 in eliminating B-cells in anti-NMDAR encephalitis are unclear (Forsthuber et al., 2018). Here we present our findings of CSF CD20 positive and CD19 positive B-cells, in a patient with acute severe NMDAR encephalitis and treated with rituximab.

Section snippets

Case report

A previously well 20-year-old Chinese female presented with a 9-day history of acute behavioral change, delusions, rigidity, and fever. The abnormal behavior included episodic agitation and inappropriate behavior. The episodic rigidity of her limbs last for half an hour, and attacked two or three times a day at first, and became persistent after admission. Her temperature was as high as 37.8 °C. She also experienced focal seizures with impaired awareness. Initial clinical examination revealed

Discussion

Here we report a case of severe anti-NMDAR encephalitis, with a typical clinical manifestation and confirmed by a positive CSF anti-NMDAR antibody test. We measured the proportion of CD20 positive and CD19 positive B-cells in the CSF of this patient by flow cytometry. The proportion of CD20 positive B-cells in the CSF was as high as 15%, higher than seen in non-inflammatory neurological disorders (<1%) (Dale et al., 2011). Previous literature reported that CD19 positive B-cells were

Ethics approval and consent to participate

Informed consent was obtained from the patient to publish this case, and approval for this study was provided by the Research Ethics Committee of The First Hospital of Peking University.

Authors'contributions

YZ contributed to the drafting, and reporting of the case. JJL and HQJ contributed to revision of the manuscript. FG and HJH contributed to the concept and revision of the manuscript. All authors have read and approved the final manuscript.

Funding

The study was supported by Scientific Research Seed Fund of Peking University First Hospital (2018SF033). Funding bodies did not play a role in the collection, analysis, and interpretation of data. Funding bodies did not contribute to the writing of this manuscript.

Declaration of Competing Interest

The authors declare that they have no competing interests.

Acknowledgements

The authors extend their appreciation to the patient and her parents for their support. Special acknowledgements are to Ms. Jing Guo and Ms. Yan Yao for their help with flow cytometry operations, and Dr. Yun Yuan, Dr. Wei Sun and Dr. Zhaoxia Wang in the help of treatment decision of this patient. Special  appreciation to Dr. Yining Huang for the support of flow cytometry test.

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