Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a severe but potentially reversible neurologic disorder that is clinically recognizable in children and adolescents. Prompt diagnosis and treatment are essential to facilitate recovery. Treatment consists of corticosteroids, intravenous immunoglobulin, or plasma exchange as first-line therapy followed by cyclophosphamide or rituximab, if necessary, as second-line immunotherapy. Patients with tumor-associated encephalitis benefit from tumor resection. More than 75% of patients make a substantial recovery, which occurs in the reverse order of symptom presentation associated with a decline in antibody titers.
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