Objective: Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is an autoimmune disorder often presenting with psychiatric symptoms, seizures, and characteristic movement disorders such as orofacial dyskinesia. Its occurrence during pregnancy is rare and poses significant diagnostic and therapeutic challenges, particularly when managing severe, refractory abnormal movements.

Background: A 32-year-old female at 9 weeks of gestation was initially admitted to the psychiatric ward for acute psychosis. She subsequently developed a sudden fever and altered mental status, prompting a transfer to the neurology department. An initial brain MRI revealed hyperintense signals in the left temporal lobe and hippocampus on FLAIR imaging. Under the suspicion of Herpes Simplex Virus (HSV) encephalitis due to accompanying clinical seizures, intravenous acyclovir was initiated in the intensive care unit.

Method: Despite the treatment, the patient developed continuous generalized myoclonus and severe perioral dyskinesia. These abnormal movements were refractory to anti-seizure medications, including levetiracetam and clonazepam. Continuous EEG monitoring showed no evidence of status epilepticus, suggesting the movements were not epileptic in origin. To control the intractable movement disorders, the patient was intubated and managed with midazolam-induced coma therapy.

Results: Cerebrospinal fluid (CSF) analysis ultimately returned positive for anti-NMDA receptor antibodies. Following extensive multidisciplinary counseling and family consent, a therapeutic abortion was performed. Subsequent abdominal and pelvic CT scans revealed no evidence of ovarian teratoma. The patient was aggressively treated with high-dose intravenous methylprednisolone pulse therapy, intravenous immunoglobulin (IVIG), and rituximab. Following this intensive immunomodulatory regimen, her systemic myoclonus and perioral dyskinesia were successfully controlled, and her level of consciousness has been gradually recovering.

Conclusion: This case highlights the importance of considering anti-NMDA receptor encephalitis in pregnant women presenting with acute psychosis and new-onset refractory movement disorders. Aggressive immunotherapy following the prompt diagnosis can lead to favorable neurological outcomes and complete resolution of movement disorders even in severe, medically refractory cases requiring coma therapy.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/a-case-of-anti-nmda-receptor-encephalitis-presenting-with-refractory-myoclonus-and-perioral-dyskinesia-during-the-first-trimester-of-pregnancy/