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Anti-N-Methyl-D-Aspartate Receptor Encephalitis (NMDARE) is a complex clinical entity that is both diagnostically and therapeutically challenging. A 17-year-old girl presented to the emergency room with worsening anxiety, confusion, and acute psychosis. While in an inpatient psychiatric unit, she developed progressive catatonia with decreased responsiveness. After transfer to the pediatric inpatient unit, she became hyperreflexic with seizure-like activity consisting of back arching, left arm extension, and right arm flexion refractory to lorazepam. Electroencephalogram (EEG) monitoring was started on the first hospital day and within twenty-four hours showed extreme delta brush pattern and abnormal background activity. Cerebrospinal fluid analysis demonstrated pleocytosis with a lymphocytic predominance. Imaging studies were notable for a left ovarian mass and non-specific sulcal hyperintensity on magnetic resonance imaging of the brain. The ovarian mass was subsequently resected, and tumor pathology was consistent with a grade II immature teratoma. Serum and CSF NMDAR IgG antibody titers wereboth elevated to 1:320, which combined with extreme delta brush pattern on EEG confirmed the diagnosis of paraneoplastic NMDARE. Further treatment for the patient’s NMDARE after tumor removal included intravenous immune globulin, high dose pulse steroids, plasmapheresis, and rituximab. The patient’s hospital course was complicated by episodes of autonomic instability. Prevention of dysautonomic crises, agitation, and rigidity were managed with benzodiazepines, benztropine, and diphenhydramine. Antipyretics were given for unexplained fevers while multiple antihypertensive agents were given for episodic hypertension. Over several weeks, the patient demonstrated gradual neurocognitive improvement to near-baseline levels and was discharged to a rehabilitation facility for further recovery. NMDARE is a major cause of disease with both psychiatric and neurologic features. Symptomatic treatment can be challenging requiring a balance of pharmacologic therapies. In the pediatric population, NMDARE is an important diagnosis to consider in applicable cases since it is a highly treatable disease with an overall good prognosis after adequate treatment.

Progression from Acute Psychosis to Seizures and Autonomic Instability in an Adolescent with Ovarian Mass