Case Presentation: A 23 year old female with a history of ovarian torsion, s/p ovarian cystectomy, presented to the emergency department in status epilepticus. Over the past week, the patient experienced a rapid deterioration in behavior associated with visual/auditory hallucinations, and panic attacks. The patient was afebrile, blood pressure of 135/70, heart rate of 120, and in respiratory distress. She was given Ativan, intubated, and admitted to the medicine ICU for further work up. Her physical exam was significant for facial dyskinesia, clonus in all extremities, and weak brainstem reflexes. Her initial brain MRI showed some leptomeningeal enhancement with negative repeat studies.. CSF studies for EBV, HIV, HSV1&2, West Nile, Dengue, Lyme disease, syphilis, Cryptococcus and Coccidioides were all negative. A pan-CT was significant for bilateral dermoid ovarian cysts with a left ovarian dermoid tumor. The patient was treated supportively for presumed viral meningoencephalitis with no improvement over the next several months, and eventually developed multiple nosocomial infections requiring a tracheostomy. During the patient’s stay, a nurse was reading a science fiction novel about a rare disorder known as anti-NMDAR encephalitis, which had a similar presentation to the patient. He discussed his findings with the patient’s primary team which raised suspicion for the disease. CSF studies returned positive for high titers of NMDAR antibodies. The patient was immediately started on IVIG, rituximab, and underwent a left oophorectomy for removal of the ovarian malignancy, which was suspected to be the source of the NMDAR antibodies. After six months of treatment, the patient regained autonomic stability, was decannulated, and achieved control over her seizures. After an 11 month hospital course, the patient is pending discharge.
Discussion: Anti-NMDAR encephalitis is a newly emerging autoimmune disease that is fatal with delayed diagnosis. Since its recent discovery, the disease has required recognition and management by hospitalists, intensivists, neurologists, psychiatrists, and gynecologists. While the pathogenesis is unknown, the disease often manifests as a paraneoplastic syndrome secondary to ovarian malignancy in young women. Patients initially present with acute psychosis and generalized seizures that progress to catatonia, autonomic instability, and respiratory failure. Early recognition and diagnosis via detection of NMDAR antibodies in CSF is essential for a faster recovery of autonomic stability and reduced complications. Treatment modalities include IVIG with rituximab and surgery to remove any ovarian malignancy.
Conclusions: Anti-NMDAR encephalitis requires management from a multidisciplinary team for early diagnosis and swift recovery. A young female who presents to a physician of any specialty with acute psychosis, pelvic pain, seizures, and autonomic instability should prompt suspicion for anti-NMDAR encephalitis.