A 33-year-old nulligravid woman started having musical hallucinations and generalized malaise, and 24 hours later she became disoriented and nonsensical. Her boyfriend brought her to the emergency department, where she grew paranoid and aggressive, requiring restraints. She was admitted to the hospital with a diagnosis of acute schizophrenia and given valproic acid, risperidone, halolperidol, and lorazepam before she was transferred to a psychiatric hospital.
The patient’s medical, surgical, and obstetrical histories were unremarkable. Her menses were regular but with dysmenorrhea. According to her family, she did not have any psychiatric history but had a sister with bipolar disorder. She worked as a construction worker and lived with her significant other. She denied use of illicit substances, tobacco, or alcohol. She did not take any prescription medications; however, a bottle of OxyElite (USPlabs LLC) diet pills was found at her house, and her family reported that she had taken weight loss medications in the past. Her family was unsure whether she had taken a diet pill before the onset of symptoms.
By the third day in the psychiatric facility, the patient’s neurologic status worsened and generalized seizures developed. She was transferred to the community teaching hospital for further evaluation. Her examination results were unchanged, and a computed tomographic (CT) scan of the head did not reveal any abnormality. A neurologist evaluated the patient for these new-onset seizures and added levitiracetam to her regimen. Further encephalopathy workup with serum toxicity screening, blood alcohol level, thyroid stimulating hormone, and syphilis antibodies was performed along with an autoimmune workup, testing for levels of vitamins and herpes antibodies, and lumbar puncture. The aforementioned test results were all normal. The lumbar puncture specimen was clear, with 100% lymphocytosis, a white blood cell count of 13/mm, 0 monocytes, a protein level of 19 mg/dL, and a glucose level of 65 mg/dL.
Because of the lack of response to the standard treatments for patients with encephalitis and seizures, by hospital day (HD) 9, a diagnosis of anti–NMDA receptor encephalitis was suspected. A CT scan of the abdomen and pelvis was ordered because of the association of anti–NMDA receptor encephalitis with teratomas, and a serum antibody titer was concurrently sent to an outside laboratory to confirm the diagnosis. The CT scan showed a complex left ovarian mass measuring 9.2 × 8.7 × 10 cm with differing densities (
Figure 1). No ascites, omental caking, or other findings consistent with malignancy were noted. Gynecologic consult was requested, and the mass was confirmed on pelvic examination. Tumor marker levels were: α-fetoprotein, 25 ng/mL (reference range, <9 ng/mL); lactate dehydrogenase, 167 U/L (reference range, 100-200 U/L); CA-125, 35.6 U/mL (reference range, <35 U/mL); and β human chorionic gonadotropin, 0.6 mIU/mL (reference range, <2.9 mIU/mL).
The patient was given high-dose steroids on HD 13, with 1 g of solumedrol administered intravenously. Owing to deteriorating mental status on this day, a midline laparotomy with left salpingo-oophorectomy was performed. The mass was smooth, intact, and without excrescences. It contained cystic components as well as skin, hair, brain, bone, and cartilage (
Figure 2). Staging was not performed because of the emergent nature of the case. The abdominal and pelvic regions showed no other pathologic findings. Endometriosis implants were noted but not excised. The final pathologic results confirmed the frozen section diagnosis of immature teratoma (
Figure 3), and the antibody titers confirmed the suspicion of anti-NMDA receptor encephalitis.
After the solumedrol dose was completed, 0.4 mg/kg of intravenous immunoglobulin was started, but the course was discontinued because of mental status deterioration. She was given plasmapheresis through a Quinton catheter in the groin on HD 22, which improved her psychosis and agitation. Owing to swallowing difficulties, on HD 30 a percutaneous endoscopic gastrostomy tube was placed, and enteral feeding was initiated. By HD 41, she was transferred to inpatient rehabilitation and discharged 7 days later. She was scheduled to receive 3 courses of bleomycin-etoposide-platinum chemotherapy, and she continued to take anticonvulsant medication. If neuropsychiatric deterioration were to recur, she would have received second-line chemotherapy with cyclophosphamide and rituximab at 750 mg/m2 monthly for 4 to 6 months and 375 mg/m2 weekly for 4 weeks, respectively.