Anti-NMDA Receptor Encephalitis in Children Is Associated with Variable Outcome (P07.140)

Abstract

Objective: To describe the presentation, clinical course and outcome of a series of pediatric anti-N-methyl-D-aspartate (NMDA) receptor encephalitis patients. Background Anti-NMDA receptor encephalitis is a recently described form of autoimmune encephalitis that typically presents with psychiatric disturbance, seizures, dyskinesias and autonomic instability. The condition is frequently associated with ovarian teratoma and is more common in adults. Prognosis is usually good with most patients experiencing either complete recovery or the persistence of only mild deficits. The clinical course and outcome is less well known in children. Design/Methods: Case series. We report on three pediatric patients who had similar clinical presentations and were ultimately found to have anti-NMDA receptor encephalitis. Results: Three previously healthy female children ages 4, 6 and 10 years presented with new onset complex partial seizures that proved intractable. Repeated electroencephalograms showed focal abnormalities. Cerebrospinal fluid studies showed mild to moderate pleocytosis with negative culture. All patients subsequently developed psychiatric disturbances varying from delirium to visual hallucinations. Oral motor dyskinesias were seen in all patients and labile blood pressure was present in two patients. CSF was found to be positive for anti-NMDA receptor antibodies in all three patients. Repeated pelvic ultrasounds have been normal. All patients received high dose steroids and IVIG and two patients received cyclophosphamide. One patient has recovered with only minimal deficits and only occasional seizures, one patient is in a vegetative state with daily seizures and one patient has severe cognitive and motor deficits with frequent seizures. Conclusions: Anti-NMDA receptor encephalitis is associated with variable outcome in children with poor outcome possibly being more prevalent than in adults. Disclosure: Dr. Aaen has nothing to disclose. Dr. Michelson has nothing to disclose. Dr. De La Pena has nothing to disclose. Dr. Przekop has nothing to disclose. Dr. Roddy has nothing to disclose. Dr. Shu has nothing to disclose. Dr. Ashwal has nothing to disclose.