Introduction: Anti-NMDA receptor (NMDAR) encephalitis is a significant cause of novel autoimmune and paraneoplastic encephalitis, Affect one out of 1.5 million people per year. Females are four times more affected as compared to males. Patients present with a prodromal phase of constitutional symptoms followed by a spectrum of clinical manifestations eventually leading to death if left untreated. However, rapid recognition and treatment can lead to survival and a return to the baseline level of functioning in most patients. It was diagnosed by the presence of Anti-NMDAR antibodies that are directed to the extracellular domain of the GluN1 subunit in the CSF and serum.

Methods: In this review article, we have examined the treatment of Anti-NMDA encephalitis in the past ten years, based on references retrieved from PubMed, NCBI publications.

Result: Treatment of Anti-NMDAR encephalitis is based on first-line immunotherapy [corticosteroids, plasmapheresis, and intravenous immunoglobulin] and second-line immunotherapy (rituximab and/or cyclophosphamide). Early treatment leads to rapid improvement in motor skills, responsiveness, self-care, and speech, additionally in the paediatric population dramatic resolution of neurologic and psychiatric symptoms was noted. In the case of complement deficiency, plasmapheresis was found to be highly effective. When first-line treatment was ineffective, intravenous rituximab has shown noteworthy clinical improvement. In cases refractory to intravenous rituximab, the use of intrathecal rituximab has shown marked improvement mainly associated with homozygous C4B deficiency. The use of cyclophosphamide helped resolve movement disorder and brought significant improvement in the domains of cognition, language, and behaviour. When given with rituximab it demonstrates remarkable improvement. in drug-resistant status epilepticus. Methotrexate, when given intrathecally along with steroids showed tremendous improvement in some paediatric cases. Bortezomib has proven to be a reserve when secondline immunotherapy is refractory. Electroconvulsive therapy reported a vigorous response in resolving neuropsychiatric symptoms. The use of oral perampanel , an α-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid (AMPA) receptor antagonist, illustrated considerable improvement in seizure activity and abnormal behaviour. Recently the use of ACTH therapy in patients with paralysis associated with choric dyskinesia of limb recovered.

Conclusion: It was observed that early initiation of combined immunosuppressive therapy in higher dosage for a prolonged duration shortens the clinical course and potentiates the possibility for complete recovery in the case of Anti-NMDAR Encephalitis. In the last decade, only 9 drugs have shown some beneficial effects. Therefore, further studies which involve more participants and evaluate newer medications are needed.