Research progress in teratoma-associated anti-N-methyl-D-aspartate receptor encephalitis: The gynecological perspective

Abstract

Aim: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is one of the autoimmune encephalitis, which often occurs in children and young adults, especially in young women. In addition to viral infection, teratoma is an important causative factor. Anti-NMDAR encephalitis is well known to neurologist. However, this is less well known to gynecologists, who may have a decisive role in etiological management. Therefore, from the perspective of gynecologists, we provide an overview of the relationship between the encephalitis and ovarian teratoma, the clinical features and treatment of this disease, and prognosis of pregnant women with this encephalitis.

Methods: We searched the PubMed database using the search terms in various combinations "teratoma," "dermoid cyst," "ovary," "ovarian," "anti-NMDAR encephalitis," and "N-methyl-D-aspartate receptor."

Results: According to the current literature, the mechanism of anti-NMDAR encephalitis is probably due to the ectopic expression of the NMDAR in the teratomas tissue. The disease has a high mortality rate if not treated promptly and effectively. An important way to improve patients' outcome is to remove the ovarian tumor in time. Pregnant women are also at risk of developing anti-NMDAR encephalitis during pregnancy. The fetal outcome is usually better in patients with mid- to late-pregnancy onset than in early pregnancy, and patients are often able to maintain their pregnancies into late pregnancy.

Conclusion: Young women with sudden onset of abnormal psychiatric behavior should be actively screened for ovarian tumors and, if confirmed to be teratoma-associated anti-NMDAR encephalitis, should undergo gynecologic surgery as early as possible, which can help improve their prognosis.

Keywords: anti-NMDAR encephalitis; mechanism; oophorocystectomy; ovarian teratoma; pregnancy.