Extended Clinical Spectrum of Anti-NMDA Receptor Encephalitis in Children (I11.007)

Objective: To emphasize wide spectrum of the clinical manifestations of Anti NMDAR encephalitis in children. Background: We study 11 children and adolescent from the tertiary referral centers in USA and India who presented with neurological or psychiatric symptoms and were finally diagnosed with Anti NMDAR encephalitis. Methods: We report experience with patients presenting to tertiary referral centers in the USA and India. Results: We are describing 11 patients (median age: 7 years, range: 10 months - 19 years). The initial manifestations were neurological (seizures or movement disorders) in 7 patients and psychiatric (e.g. emotional labiality, hallucination) in 4 patients. Symptoms included seizures in 10 patients (status epileptics in 3), behavioral changes (aggressiveness, insomnia, irritability) in 9 patients, and movement disorders (dyskinesia and choreiform movements) in 10 patients. Autonomic features were seen in 5 patients (e.g. thermal instability). The EEG in all patients showed diffuse background slowing (epileptiform activity in 2 patients). MRI was abnormal in 1 patient and concomitant infection (HSV1 and influenza A) was found in 2 patients. CSF analyses were abnormal in a case with post HSV. Treatment included IVIG/Methylprednisolone (10 patients), Rituximab (7 patients), plasmapheresis (2 patients), and cyclophosphamide (1 patient). One patient underwent brain biopsy and oophorectomy. No malignancy was found in any patient. Five patients recovered completely, six has mild to moderate neurological deficits and one died with autonomic instability. Two patients relapsed. Conclusion: Anti NMDAR encephalitis can present with non-specific symptoms such as seizures and behavioral changes, usually followed by a movement disorder. Psychiatric symptoms are difficult to recognize in children. Strong clinical suspicion is needed to make early diagnosis and treatment. Disclosure: Dr. Goenka has nothing to disclose. Dr. Nariai has nothing to disclose. Dr. Steinschneider has nothing to disclose. Dr. Spiro has nothing to disclose. Dr. Jain has nothing to disclose.