Rituximab for Treatment of Refractory Anti-NMDA Receptor Encephalitis in a Pediatric Patient.J Pediatr Pharmacol Ther. 2017 Mar-Apr; 22(2):118-123.JP
Anti-N-methyl d-aspartate receptor (anti-NMDAR) encephalitis is a devastating disease that is increasingly being identified in both children and adults with psychosis, language disturbances, behavioral changes, and motor deficits. Currently no consensus guidelines exist for the optimal management of patients with this disease, although intravenous immune globulin (IVIG) therapy is often considered first-line pharmacotherapy. We present a case of an otherwise healthy 4 year-old-child who presented with seizures, loss of age-appropriate language skills, and behavioral changes, in whom anti-NMDAR was subsequently diagnosed. After marked intolerance to corticosteroid therapy and inadequate clinical response to IVIG, immunotherapy with rituximab was initiated. The patient had rapid return of language skills and complete resolution of dyskinesia after a single rituximab infusion, with no residual deficits at her 6-month follow-up visit. Early intervention in patients with anti-NMDAR encephalitis is of paramount importance for successful outcomes and baseline recovery. Only approximately half of patients respond to first-line immunotherapy, necessitating further evaluation of alternative therapies and the development of a treatment algorithm for practitioners. This case report builds upon previous findings illustrating rapid symptom resolution after rituximab infusion and adds to the available body of evidence for management of pediatric patients with anti-NMDAR.