Yeungnam Univ J Med.  2021 Oct;38(4):350-355. 10.12701/yujm.2020.00794.

Anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma in Korea: three case reports

• Lee J
• Kang S
• Chang HJ
• Lee YH
• Son JH
• Kong TW
• Chang SJ
• Hwang KJ
• Kim M

• KMID: 2521715
• DOI: http://doi.org/10.12701/yujm.2020.00794

Abstract

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe autoimmune paraneoplastic syndrome associated with ovarian teratomas. Most patients develop neurologic symptoms, including psychosis, memory deficits, seizures, or abnormal movements, and experience abdominal pain related to ovarian neoplasm. We present a case report of three patients diagnosed with anti-NMDAR encephalitis accompanied by ovarian teratomas at Ajou University Hospital in Korea. The patients demonstrated a different clinical course of the disease. However, upon diagnosis, all patients underwent surgical removal of the ovarian teratoma followed by intensive immunotherapy. The symptoms progressively improved following treatment. This is a case report of a rare autoimmune anti-NMDAR encephalitis associated with ovarian neoplasms, including immature teratoma.

Keyword

Anti--methyl-D-aspartate receptor encephalitis; Immature ovarian teratoma; Mature ovarian teratoma

Figure

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  Fig. 1. Imaging and pathology findings for case 1. (A) Abdominal computed tomography findings. Unilocular 6.7-cm cyst (arrow) with solid and calcified portion. (B) Intraoperative image of a left ovarian cyst. (C) The immature teratoma reveals immature neuroepithelial tubules (arrows) in the background of mature teratoma containing skin adnexa, bone, and cartilage with choroid plexus (hematoxylin and eosin stain, ×100). (D) Glial fibrillary acidic protein immunostaining reveals an abundance of mature and immature neural and brain tissues (immunohistochemical stain, x100).

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  Fig. 2. Imaging and pathology findings for case 2. (A) Abdominal computed tomography findings. A 4-cm cystic mass with fat component (arrow) suggesting a teratodermoid tumor of the left ovary. (B) Intraoperative image of a left ovarian cyst. (C) Mature cystic teratoma shows a small amount of mature neuroglial tissue (arrows) with mature skin adnexal tissue, fat, and choroid plexus (hematoxylin and eosin stain, ×100). (D) Glial fibrillary acidic protein immunostaining highlights mature neuroglial tissue (arrows) in the mature cystic teratoma (immunohistochemical stain, x100).

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  Fig. 3. Imaging and pathology findings for case 3. (A) Abdominal computed tomography findings. A 3-cm cystic mass with fat component (arrow) suggesting a teratodermoid tumor of the left ovary. (B) Intraoperative image of a left ovarian cyst. (C) The mature cystic teratoma containing skin adnexa, squamous epithelium, fat tissue, cartilage with respiratory epithelium, and mature neural tissue (arrows) (hematoxylin and eosin stain, ×100). (D) Glial fibrillary acidic protein immunostaining demonstrates mature neuroglial tissue (arrows) (immunohistochemical stain, x100).

Reference

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