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
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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DOI：
10.2169/internalmedicine.49.4069
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发表时间：
2010-01-01
影响因子：
1.2
通讯作者：
Ikeda, Shu-ichi
中科院分区：
文献类型：
--
作者：
Tachibana, Naoko;Shirakawa, Takashi;Ikeda, Shu-ichi
1例21岁女性出现精神症状、进行性无反应、全身性癫痫、严重运动障碍、血压显著波动和流感样发作后多尿。抗谷氨酸受体(GluR)epsilon 2和抗N-甲基-D-天冬氨酸受体(NMDAR)抗体均阳性。五个月后康复后，她接受了手术切除右侧卵巢畸胎瘤。免疫组织化学检测显示多种GluR在Her畸胎瘤中大量表达，包括NMDAR的NR2B亚单位、GluR1和GluR2/3。这些GluRs的免疫反应不仅见于神经组织中的抗胶质纤维酸性蛋白(GFAP)免疫反应区，也见于其他未分化的神经上皮组织中，而不是GFAP免疫反应区。我们的发现有力地支持了最近的观点，即卵巢畸胎瘤中的神经元件在抗NMDAR脑炎的NMDAR抗体的产生中起着重要作用。此外，对照卵巢的研究清楚地显示NR2B相关的免疫反应位于卵母细胞的胞浆中，这表明正常卵巢本身也有NMDAR的表达。这一发现可能为了解无卵巢畸胎瘤的女性患者的发病机制提供线索。
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)epsilon 2 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.