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 (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.