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

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 we...

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Veröffentlicht in:Internal Medicine 2010, Vol.49(19), pp.2167-2173
Hauptverfasser: Tachibana, Naoko, Shirakawa, Takashi, Ishii, Keiko, Takahashi, Yukitoshi, Tanaka, Keiko, Arima, Kunimasa, Yoshida, Takuhiro, Ikeda, Shu-ichi
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container_issue 19
container_start_page 2167
container_title Internal Medicine
container_volume 49
creator Tachibana, Naoko
Shirakawa, Takashi
Ishii, Keiko
Takahashi, Yukitoshi
Tanaka, Keiko
Arima, Kunimasa
Yoshida, Takuhiro
Ikeda, Shu-ichi
description 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.
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Med.</addtitle><description>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. 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subjects alpha -Amino-3-hydroxy-5-methyl-4-isoxazole propionic acid receptors
Antibodies
Autoantibodies - blood
Autoantibodies - cerebrospinal fluid
Blood pressure
Cerebrospinal fluid
Cytoplasm
Dyskinesia
Encephalitis
Female
Glial fibrillary acidic protein
glutamate receptor
Glutamic acid receptors
Glutamic acid receptors (ionotropic)
Humans
Immunohistochemistry
Immunoreactivity
limbic encephalitis
N-methyl-D-aspartate receptor (NMDAR)
N-Methyl-D-aspartic acid receptors
Oocytes
Ovarian Neoplasms - complications
Ovarian Neoplasms - immunology
Ovarian Neoplasms - metabolism
Ovarian Neoplasms - pathology
ovarian teratoma
Ovaries
paraneoplastic syndrome
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
Seizures
Surgery
teratoma
Teratoma - complications
Teratoma - immunology
Teratoma - metabolism
Teratoma - pathology
Young Adult
title 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
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