Anti-NMDA receptor encephalitis is an often missed immune-mediate disease process which results in a myriad of symptoms ranging from psychosis, altered levels of consciousness, memory impairment as well as autonomic instability.METHODS: Patient is a 28 year old female with no past medical history who presented for altered mental status and suicidal ideation. She had lucid intervals when she reported that she could not recall anything which had happened in the last few days but she felt she was going to go to Hell and wanted to kill herself. Her short term memory was impaired and she was found to have increasingly bizarre and psychotic behavior with unintelligible speech. RESULTS:No electrolyte abnormalities were noted and physical exam was unremarkable. She had an episode of seizure-like activity with foaming at the mouth, posturing and unresponsiveness for which she was intubated. EEG was negative. There was concern for meningitis and patient was started on empiric antibiotics after a lumbar puncture was performed. Lumbar puncture, CT head, and MRI head were performed which were all negative for any acute process. CT abdomen and pelvis was benign other than an adnexal mass which triggered a concern for encephalitis. NMDA R antibodies from the serum were ordered and were negative.CONCLUSIONS: Due to high clinical suspicion for limbic encephalitis despite negative testing, patient was started on and completed a course of pulse steroids as well as IVIG without any improvement in her mentation. She continued to have seizurelike activity and rhythmic myoclonic jerking of her extremities as well as ocullomasticatory myorhythmic contractions. Repeat EEG was again negative. A second lumbar puncture was performed and was significant for elevated opening pressure and oligoclonal bands. Plasma exchange was started with no improvement after 1 week. NMDA from the CSF was eventually confirmed as positive and after extensive infectious workup, rituximab was started. Patient was taken to the OR for removal of the adnexal mass and tracheostomy placement.CLINICAL IMPLICATIONS: NMDA-R encephalitis is a relatively new and rare diagnosis presenting with a range of seemingly independent and unrelated symptoms. With an incidence of 1.5 per million, it is an often missed clinical diagnosis in otherwise young and healthy patients. This case helps emphasizes the importance of identifying patients with the disease process as there is 80% cure rate with first and second line therapies. Furthermore, it demonstrates the importance of CSF assessment of NMDA receptors as the sensitivity of serum versus CSF antibodies is 85% vs 100%. Although NMDA-R encephalitis is a less frequently encountered cause of altered mental status, physicians should be aware of the presentation, age group and association with ovarian teratomas and herpes simplex virus.
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