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

  • Tachibana Naoko
    Department of Medicine (Neurology), Okaya City Hospital
  • Shirakawa Takashi
    Department of Gynecology, Okaya City Hospital
  • Ishii Keiko
    Department of Pathology, Okaya City Hospital
  • Takahashi Yukitoshi
    National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders
  • Tanaka Keiko
    Department of Neurology, Kanazawa Medical University
  • Arima Kunimasa
    Department of Psychiatry, National Center Hospital of Neurology and Psychiatry
  • Yoshida Takuhiro
    Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine
  • Ikeda Shu-ichi
    Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine

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Abstract

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.<br>

Journal

  • Internal Medicine

    Internal Medicine 49 (19), 2167-2173, 2010

    The Japanese Society of Internal Medicine

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