This case describes a 19-year-old African American female with a strong family history of epilepsy who presented to the ED following two weeks of headaches, multiple generalized, tonic-clonic seizures, and significant behavioral changes. Her family witnessed her having a generalized seizure lasting about eight minutes, which prompted them to bring her to the hospital. This episode included tongue biting, urinary incontinence, and a post-ictal state lasting 20 minutes. The patient had no previous history of seizures, head trauma, or substance use. After being brought to the ED, she had another tonic-clonic seizure involving all four extremities, which lasted 90 seconds and was terminated with Ativan. Her symptoms progressed from post-ictal psychosis with hallucinations and somnolence to a full loss of consciousness. She also had dysautonomia, ileus, intermittent urinary retention, and papilledema.

Initial blood work was unremarkable, and a MRI of the brain was interpreted as normal. EEG recording was abnormal and suggested a right frontal potential for seizures and a nonspecific, generalized disturbance of cerebral function. A lumbar puncture revealed an elevated opening pressure of 55 cmH20. A meningitis panel was negative, and a CSF culture revealed no organisms. A subsequent CT of the abdomen and pelvis revealed a 5 x 4.2 cm ovarian cyst. Further examination of the cyst with ultrasound revealed areas of calcification. The initial differential diagnosis included a paraneoplastic syndrome and HSV encephalitis, and she was subsequently started on acyclovir and IVIG.

After a discussion with neuroradiology about the differential diagnosis, a repeat MRI brain and a MRI of the abdomen, to establish better detail of the cyst, were done. The MRI brain revealed minimal hyperintensities within the temporal lobes bilaterally (Figure 1), and the MRI abdomen and pelvis showed evidence of previously unidentified bilateral dermoid cysts (Figure 2). Testing for anti-NMDA receptor in the CSF came back positive, and the patient underwent left salpingoophorectomy and right ovarian cystectomy. Microscopy of the excised lesions revealed both to be mature cystic teratomas. In addition to IVIG, she was also treated with Solu-Medrol, PLEX, and rituximab. She also had to have a ventriculoperitoneal shunt placed for grade II papilledema and recurrent elevated lumbar pressures. Testing for HSV eventually came back negative and acyclovir was discontinued.

Despite removal of the bilateral teratomas, she showed minimal improvement over the next two months, with continued poverty of speech, impaired cognition, and a fluctuating level of consciousness. She continued to have hallucinations along with violent and psychotic behavior. After a four-month hospital course, her condition gradually improved enough so that she could be discharged. Currently, the patient’s mental status has returned to and is maintained at her baseline. However, she continues to have intermittent, spontaneous movements once every 30 minutes. An EEG will be done in the near future to further characterize these movements.