2017 Volume 69 Issue 1 Pages 37-44
Anti-NMDA receptor encephalitis is a paraneoplastic encephalitis associated with ovarian teratoma. It is a non-herpetic limbic form that occurs most frequently in young women. Here we present a difficult-to-diagnose case of ovarian mature cystic teratoma associated with anti-NMDA receptor encephalitis. A 25-year-old nulligravid woman presenting with fever, headache, vomiting, and neck stiffness was admitted to our neurology department. Although she was initially treated for aseptic meningitis, she experienced consciousness disturbance and convulsive seizures. We suspected encephalitis based on her symptoms and steroid pulse therapy was initiated. However, her condition suddenly deteriorated and she was transferred to the ICU with intratracheal intubation. An abdominal MRI revealed a 33 × 25 mm multi-cystic ovary. She underwent a left adnexectomy on the 36th day in the hospital. Histopathological examination of the excised specimen revealed a 2 cm mature cystic teratoma. Anti-NMDA receptor antibodies were detected in spinal fluid collected at admission, which led to a definitive diagnosis. She gradually regained consciousness after the operation and was discharged 114 days after admission. Anti-NMDA receptor encephalitis is usually severe and can be fatal, but is potentially reversible. Management of anti-NMDA receptor encephalitis should be initially focused on immunotherapy, and the detection and removal of a teratoma. [Adv Obstet Gynecol, 69 (1) : 37-44, 2017 (H29.2)]