P2.246: Terror-toma: When the Ovary Strikes the Brain

Introduction: Anti-NMDA receptor encephalitis is a rare autoimmune disorder characterized by psychiatric symptoms, cognitive decline, dyskinesia, and autonomic instability. In females, it is often associated with ovarian teratomas, making timely diagnosis and treatment critical to improving outcomes.

Case Description: We present the case of a previously healthy 14-year-old female who presented due to concern of altered mental status, which developed over one week. Initial symptoms included rapid speech, episodic agitation, unintelligible speech, and lethargy. Episodes featured non-directed aggression, crying, and grasping at her chest and shoulders. Despite fluctuating periods of lucidity, her agitation increased, and sleep became severely disrupted. Neurological evaluation showed disorganized behavior without focal deficits. Routine lab tests were unremarkable except for dehydration. Brain imaging was normal, but cerebrospinal fluid analysis revealed lymphocytic pleocytosis. Continuous EEG demonstrated intermittent delta slowing in the right posterior temporal region. Anti-NMDA receptor antibodies were detected. A pelvic MRI identified a 3.8 cm left ovarian teratoma with a 1.4 cm nodule containing macroscopic fat, consistent with a mature cystic teratoma. Tumor marker levels, including CA-125 and alpha-fetoprotein, were within normal limits. The patient underwent a successful laparoscopic left salpingo-oophorectomy, with histopathology confirming the teratoma. Immunotherapy included high-dose methylprednisolone, intravenous immunoglobulin, plasmapheresis, and rituximab. During plasmapheresis, the patient required sedation in the intensive care unit due to agitation and risk of self-injury. Post-treatment, she exhibited waxing and waning catatonia but has shown gradual improvement with psychiatric management and tapering of sedative medications.

Discussion: This case highlights the importance of considering anti-NMDA receptor encephalitis in adolescents with complex neuropsychiatric symptoms and altered consciousness, even without seizures. The diagnostic process was complicated by the episodic nature of symptoms and the absence of focal neurological signs. Early detection and excision of the teratoma, coupled with immunotherapy, were crucial for disease management. This report underscores the necessity for a multidisciplinary approach and illustrates the variable presentation of anti-NMDA receptor encephalitis, advocating for further research into optimizing treatment protocols.

Conclusion: This case emphasizes the critical need to recognize anti-NMDA receptor encephalitis in adolescents presenting with acute psychiatric and neurological symptoms. Prompt identification of the underlying teratoma and initiation of immunotherapy significantly influenced the patient’s recovery. Multidisciplinary coordination was essential in managing both medical and behavioral complications. As awareness grows, early diagnosis can lead to better outcomes and reduced long-term morbidity in affected patients.