Paraneoplastic Limbic Encephalitis Associated With Anti-N-methyl-D Aspartate Receptor Antibodies and an Ovarian Teratoma: A Case for Concern

Abstract

Background: An association of paraneoplastic limbic encephalitis (PLE) with ovarian teratomas containing neural tissue has been reported in the neurologic literature. In a small number of patients, surgical excision of the ovarian teratoma has resulted in clinical improvement in some of the affected patients. Case: Our patient is a previously healthy 14 year old girl who presented to an outside hospital with vomiting, confusion, restlessness and agitation. She began to hallucinate, talk ‘‘gibberish’’, and have choreiform movements. She was treated with intravenous immunoglobulin for suspected acute disseminated encephalomyelitis and empirically treated with antibiotics. She failed to respond, became comatose, was intubated and then underwent a tracheotomy for airway protection. She was then transferred to a tertiary care center for plasmapheresis. Paraneoplastic syndrome was considered and imaging studies revealed a 7.3cm X 6.8 cm complex mass in the right adnexa. Based on literature reports of an association of PLE with pelvic tumors and resolution of the neurologic symptoms in some of the patients who underwent resection of the tumors, the family consented to an exploratory laparotomy. A right salpingoophorectomy was done. The pathologic diagnosis was a mature teratoma. Central nervous system tissue, skin, respiratory tract, fat, cartilage, salivary gland, bone, and colon were identified within the tumor. The patient’s CSF was positive for antibodies to NR1/NR2 heteromers of N-methyl-D-aspartate (NMDA) receptors. Unfortunately removal of the ovarian teratoma did not result in improvement of this young woman’s condition. Five months after her initial hospitalization she was placed in a long-term care facility. Comments: Mature cystic teratomas, also known as dermoid cysts, are one of the most common benign ovarian tumors in women of reproductive age. Surgical excision has long been accepted as the standard treatment for ovarian dermoid tumors, not only for definitive diagnosis, but to also prevent future complications-most commonly torsion, rupture, or the small risk of malignant transformation. While an association of ovarian teratomas with neural elements and PLE is published in the neurologic literature, there is only emerging mention of this association in the gynecologic literature. Even though this young woman had anti-NMDA receptor antibodies and neural elements in her tumor, she did not improve once the teratoma was resected. This case report serves to heighten awareness in the gynecologic community of this association.