Anti-N-methyl-D-aspartate receptor encephalitis: A review of pathogenic mechanisms, treatment, prognosis.

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a treatable autoimmune disorder characterized by prominent neuropsychiatric symptoms that predominantly affects children and young adults. In this review, we discuss the pathogenic mechanisms and immunologic triggers of anti-NMDAR encephalitis, and provide an overview of treatment and prognosis of this disorder, with specific focus on the management of common symptoms, complications, and patients during pregnancy. Most patients respond well to first-line treatment and surgical resection of tumors. When first-line immunotherapy fails, second-line immunotherapy can often improve outcomes. In addition, treatment with immunomodulators and tumor resection are effective treatment strategies for pregnant patients. Benzodiazepines are the preferred treatment for patients with catatonia, and electroconvulsive therapy (ECT) may be considered when pharmacological treatment is ineffective. Age, antibody titer, cerebellar atrophy, levels of biomarkers such as C-X-C motif chemokine 13 (CXCL13), cell-free mitochondrial (mt)DNA in cerebral serum fluid (CSF), and timing from symptom onset to treatment are the main prognostic factors. Patients without tumors or those who receive insufficient immunotherapy during the first episode are more likely to relapse.