Anti-NMDAR encephalitis with ovarian teratomas: Review of the literature and two case reports

Taiwan J Obstet Gynecol. 2019 May;58(3):313-317. doi: 10.1016/j.tjog.2019.03.004.

Abstract

Objective: Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis is a paraneoplastic syndrome associated with ovarian teratomas. Anti-NMDAR encephalitis patients typically present with prominent psychiatric symptoms, seizures, and involuntary movements; further, they rapidly progress to unresponsiveness with central hypoventilation and dysautonomia.

Case report: This paper presents two anti-NMDAR encephalitis cases with ovarian teratomas and reviews 13 anti-NMDAR encephalitis clinical case reports in Taiwan, of which six involved ovarian tumors, five being mature teratomas. Patients presented with acute onset of psychiatric symptoms and subsequently developed coma within a few days. Anti-NMDAR encephalitis usually occurs in young women and is often associated with ovarian tumors, specifically teratomas. Ovarian cystectomy or oophorectomy was performed, which markedly improved cognitive function.

Conclusion: Paraneoplastic neurological conditions associated with ovarian teratomas represent a fascinating disease process. Identifying the gynecological cause of a neurological condition, particularly in young women, followed by prompt treatment can remarkably improve clinical conditions and, thus, be lifesaving.

Keywords: Anti-N-Methyl-d-aspartate receptor (anti-NMDAR) encephalitis; Autoimmune encephalitis; Ovarian teratoma; Paraneoplastic syndrome; Psychosis.

MeSH terms

  • Adult
  • Anti-N-Methyl-D-Aspartate Receptor Encephalitis / drug therapy
  • Anti-N-Methyl-D-Aspartate Receptor Encephalitis / etiology*
  • Anti-N-Methyl-D-Aspartate Receptor Encephalitis / physiopathology
  • Female
  • Humans
  • Immunoglobulins / therapeutic use
  • Ovarian Neoplasms / complications*
  • Ovarian Neoplasms / diagnostic imaging
  • Ovarian Neoplasms / pathology
  • Ovarian Neoplasms / surgery
  • Taiwan
  • Teratoma / complications*
  • Teratoma / diagnostic imaging
  • Teratoma / pathology
  • Teratoma / surgery
  • Tomography, X-Ray Computed

Substances

  • Immunoglobulins

Supplementary concepts

  • Teratoma, Ovarian