Expression of various glutamate receptors including N-methyl-D-aspartate receptor (NMDAR) in an ovarian teratoma removed from a young woman with anti-NMDAR encephalitis

Intern Med. 2010;49(19):2167-73. doi: 10.2169/internalmedicine.49.4069. Epub 2010 Oct 1.


A 21-year-old woman developed psychiatric symptoms, progressive unresponsiveness, generalized seizures, severe dyskinesia, marked fluctuation of blood pressure, and hypersalivation after a flu-like episode. Anti-glutamate receptor (GluR)ε2 and anti-N-methyl-D-aspartate receptor (NMDAR) antibodies were positive in both her serum and CSF. After she recovered five months later she underwent surgery to remove a right ovarian teratoma. Immunohistochemical examinations of her teratoma disclosed abundant expression of various GluRs including NR2B subunit of NMDAR, GluR1, and GluR2/3. These immunoreactivities of GluRs were seen not only in small areas of neural tissue identified as anti-glial fibrillary acidic protein (GFAP)-immunoreactive areas but also in other large areas of undifferentiated neuroepithelial tissue without GFAP immunoreactivity. Our findings strongly support the recent idea that neural elements in ovarian teratoma play an important role in the production of antibodies to NMDARs in anti-NMDAR encephalitis. Additionally, the study of control ovaries clearly showed NR2B-related immunoreactivity in the cytoplasm of oocytes, indicating that the normal ovary itself has expression of NMDARs. This finding might provide a clue to understand the pathogenesis of this disease in female patients without ovarian teratoma.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Autoantibodies / blood
  • Autoantibodies / cerebrospinal fluid
  • Female
  • Humans
  • Immunohistochemistry
  • Ovarian Neoplasms / complications
  • Ovarian Neoplasms / immunology*
  • Ovarian Neoplasms / metabolism*
  • Ovarian Neoplasms / pathology
  • Paraneoplastic Syndromes, Nervous System / etiology
  • Paraneoplastic Syndromes, Nervous System / immunology*
  • Paraneoplastic Syndromes, Nervous System / metabolism*
  • Receptors, AMPA / immunology
  • Receptors, AMPA / metabolism
  • Receptors, Glutamate / immunology
  • Receptors, Glutamate / metabolism*
  • Receptors, N-Methyl-D-Aspartate / antagonists & inhibitors
  • Receptors, N-Methyl-D-Aspartate / immunology*
  • Receptors, N-Methyl-D-Aspartate / metabolism*
  • Teratoma / complications
  • Teratoma / immunology*
  • Teratoma / metabolism*
  • Teratoma / pathology
  • Young Adult


  • Autoantibodies
  • NR2B NMDA receptor
  • Receptors, AMPA
  • Receptors, Glutamate
  • Receptors, N-Methyl-D-Aspartate
  • glutamate receptor ionotropic, AMPA 3
  • glutamate receptor ionotropic, AMPA 2
  • glutamate receptor ionotropic, AMPA 1