Successful treatment with efgartigimod as an add-on therapy for acute attack of anti-NMDA receptor encephalitis: a case report

Abstract Background Anti-NMDA receptor encephalitis is an autoimmune, antibody-mediated inflammatory disease of the brain characterized by the presence of IgG antibodies targeting the excitatory N-methyl-D-aspartate receptor (NMDAR). Previous research has established that the neonatal Fc receptor (F...

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Main Authors: Huasheng Huang, Yizhi Wei, Huihui Qin, Guangshun Han, Jie Li
Format: Article
Language:English
Published: BMC 2025-01-01
Series:BMC Neurology
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Online Access:https://doi.org/10.1186/s12883-025-04034-6
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author Huasheng Huang
Yizhi Wei
Huihui Qin
Guangshun Han
Jie Li
author_facet Huasheng Huang
Yizhi Wei
Huihui Qin
Guangshun Han
Jie Li
author_sort Huasheng Huang
collection DOAJ
description Abstract Background Anti-NMDA receptor encephalitis is an autoimmune, antibody-mediated inflammatory disease of the brain characterized by the presence of IgG antibodies targeting the excitatory N-methyl-D-aspartate receptor (NMDAR). Previous research has established that the neonatal Fc receptor (FcRn) regulates the transport and circulation of immunoglobulins (IgG). Efgartigimod, an FcRn antagonist, has been shown to enhance patient outcomes by promoting IgG clearance, and it has exhibited substantial clinical efficacy and tolerability in the treatment of myasthenia gravis. Efgartigimod has demonstrated potential efficacy in the treatment of various IgG-mediated autoimmune diseases. Nonetheless, to date, no studies have investigated the use of efgartigimod in the treatment of anti-NMDAR encephalitis. Case presentation We present a case of a 42-year-old male patient diagnosed with anti-NMDAR encephalitis, initially treated with intravenous methylprednisolone(IVMP) and human immunoglobulin (IVIG) without clinical improvement. Subsequent administration of efgartigimod resulted in rapid clinical improvement; however, the patient experienced a relapse upon discontinuation of efgartigimod. Reintroduction of efgartigimod led to rapid and significant clinical improvement, accompanied by a marked decrease in anti-NMDAR antibodies and serum IgG levels in both serum and cerebrospinal fluid. The patient remained relapse-free during a 2-month follow-up period. Conclusion This case demonstrates that efgartigimod is a potentially rapid and effective therapy for the treatment of the acute phase of anti-NMDAR encephalitis.
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spelling doaj-art-4050fe0f14184476a56d8a3cd9e8403c2025-01-26T12:39:25ZengBMCBMC Neurology1471-23772025-01-012511810.1186/s12883-025-04034-6Successful treatment with efgartigimod as an add-on therapy for acute attack of anti-NMDA receptor encephalitis: a case reportHuasheng Huang0Yizhi Wei1Huihui Qin2Guangshun Han3Jie Li4Department of Neurology, Liuzhou People’s Hospital affiliated to Guangxi Medical UniversityDepartment of Neurology, Liuzhou People’s Hospital affiliated to Guangxi Medical UniversityDepartment of Neurology, Liuzhou People’s Hospital affiliated to Guangxi Medical UniversityDepartment of Neurology, Liuzhou People’s Hospital affiliated to Guangxi Medical UniversityDepartment of Neurology, Liuzhou People’s Hospital affiliated to Guangxi Medical UniversityAbstract Background Anti-NMDA receptor encephalitis is an autoimmune, antibody-mediated inflammatory disease of the brain characterized by the presence of IgG antibodies targeting the excitatory N-methyl-D-aspartate receptor (NMDAR). Previous research has established that the neonatal Fc receptor (FcRn) regulates the transport and circulation of immunoglobulins (IgG). Efgartigimod, an FcRn antagonist, has been shown to enhance patient outcomes by promoting IgG clearance, and it has exhibited substantial clinical efficacy and tolerability in the treatment of myasthenia gravis. Efgartigimod has demonstrated potential efficacy in the treatment of various IgG-mediated autoimmune diseases. Nonetheless, to date, no studies have investigated the use of efgartigimod in the treatment of anti-NMDAR encephalitis. Case presentation We present a case of a 42-year-old male patient diagnosed with anti-NMDAR encephalitis, initially treated with intravenous methylprednisolone(IVMP) and human immunoglobulin (IVIG) without clinical improvement. Subsequent administration of efgartigimod resulted in rapid clinical improvement; however, the patient experienced a relapse upon discontinuation of efgartigimod. Reintroduction of efgartigimod led to rapid and significant clinical improvement, accompanied by a marked decrease in anti-NMDAR antibodies and serum IgG levels in both serum and cerebrospinal fluid. The patient remained relapse-free during a 2-month follow-up period. Conclusion This case demonstrates that efgartigimod is a potentially rapid and effective therapy for the treatment of the acute phase of anti-NMDAR encephalitis.https://doi.org/10.1186/s12883-025-04034-6Anti-NMDA-receptor antibody encephalitisEfgartigimodFcRn inhibitorIgG
spellingShingle Huasheng Huang
Yizhi Wei
Huihui Qin
Guangshun Han
Jie Li
Successful treatment with efgartigimod as an add-on therapy for acute attack of anti-NMDA receptor encephalitis: a case report
BMC Neurology
Anti-NMDA-receptor antibody encephalitis
Efgartigimod
FcRn inhibitor
IgG
title Successful treatment with efgartigimod as an add-on therapy for acute attack of anti-NMDA receptor encephalitis: a case report
title_full Successful treatment with efgartigimod as an add-on therapy for acute attack of anti-NMDA receptor encephalitis: a case report
title_fullStr Successful treatment with efgartigimod as an add-on therapy for acute attack of anti-NMDA receptor encephalitis: a case report
title_full_unstemmed Successful treatment with efgartigimod as an add-on therapy for acute attack of anti-NMDA receptor encephalitis: a case report
title_short Successful treatment with efgartigimod as an add-on therapy for acute attack of anti-NMDA receptor encephalitis: a case report
title_sort successful treatment with efgartigimod as an add on therapy for acute attack of anti nmda receptor encephalitis a case report
topic Anti-NMDA-receptor antibody encephalitis
Efgartigimod
FcRn inhibitor
IgG
url https://doi.org/10.1186/s12883-025-04034-6
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