Anti-N-Methyl-D-Aspartate Receptor Encephalitis in a Young Woman with Ovarian Teratoma

In anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, progressive cerebellar atrophy potentially leads to severe sequelae. We encountered a patient with anti-NMDAR antibody encephalitis who showed a decrease of blood flow in the cerebellum. A 15-year-old girl presented with consciousness disturbance. Influenza encephalopathy was suspected, and she was treated with glucocorticoid pulse therapy, high-dose intravenous immunoglobulins, and plasma exchange sequentially. She subsequently underwent left oophorectomy due to the presence of anti-NMDAR antibodies and a left ovarian teratoma. In spite of the surgery, her neuropsychiatric symptoms persisted, and she recovered slowly after the introduction of oral methotrexate (MTX). Sequential cerebral blood flow monitoring with single-photon emission computed tomography showed marked cerebellar hypoperfusion. Although mild impairments including working memory and verbal fluency persisted, she eventually returned to high school 3 years after onset. Profound cerebellar hypoperfusion including lobules VI and VII may be the reason for her working memory impairment and speaking problems. Oral MTX may be a promising alternative treatment for some refractory cases of anti-NMDAR encephalitis.

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Immunopathological Significance of Ovarian Teratoma in Patients with Anti-N-Methyl-D-Aspartate Receptor Encephalitis

European Neurology ◽

10.1159/000353982 ◽

2013 ◽

Vol 71 (1-2) ◽

pp. 42-48 ◽

Cited By ~ 17

Author(s):

Emi Tabata ◽

Masanori Masuda ◽

Makoto Eriguchi ◽

Masatoshi Yokoyama ◽

Yoshiyuki Takahashi ◽

...

Keyword(s):

Ovarian Teratoma ◽

Aspartate Receptor

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Anti-n-Methyl-d-Aspartate-Receptor (NMDAR) Encephalitis in Association with Ovarian Teratoma

Cureus ◽

10.7759/cureus.1425 ◽

2017 ◽

Author(s):

Javaad Ahmad ◽

Muhammad Saad Sohail ◽

Amina Khan ◽

Ahmed H Qavi ◽

Pramod Gaudel ◽

...

Keyword(s):

Ovarian Teratoma ◽

Aspartate Receptor ◽

Nmdar Encephalitis

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Not hysteria: ovarian teratoma-associated anti-N-methyl-d-aspartate receptor encephalitis

Scottish Medical Journal ◽

10.1258/smj.2012.012026 ◽

2012 ◽

Vol 57 (3) ◽

pp. 1-3 ◽

Cited By ~ 4

Author(s):

R Roberts ◽

N J J Macdougall ◽

P O'brien ◽

K Abdelaziz ◽

J Christie ◽

...

Keyword(s):

Ovarian Teratoma ◽

Aspartate Receptor

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Anti-N-methyl-D-aspartate receptor encephalitis and ovarian teratomas: is there any association?

Voprosy ginekologii akušerstva i perinatologii ◽

10.20953/1726-1678-2020-5-124-131 ◽

2020 ◽

Vol 19 (5) ◽

pp. 124-131

Author(s):

N.M. Podzolkova ◽

◽

V.V. Korennaya ◽

O.S. Levin ◽

E.E. Vasenina ◽

...

Keyword(s):

Pathological Condition ◽

Rare Complication ◽

Autoimmune Encephalitis ◽

Neurological Deficits ◽

Ovarian Teratoma ◽

Aspartate Receptor ◽

Nmdar Encephalitis ◽

Life Threatening ◽

Causal Treatment ◽

Ovarian Teratomas

This article aims to explore the problem of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis and also describes our own experience in treating anti-NMDAR encephalitis in patients with ovarian teratomas. This pathological condition is a rare complication of cancer. Practicing gynecologists are often unfamiliar with it, although its consequences can be life-threatening. Early diagnosis and causal treatment, particularly teratoma removal, are critical for the outcome and can prevent death or the development of neurological deficits in patients. Key words: anti-NMDAR, autoimmune encephalitis, ovarian cysts, complications, ovarian teratoma, encephalitis, encephalitis in women

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HLA-A and HLA-DRB1 may play a unique role in ovarian teratoma-associated anti-N-methyl-D-aspartate receptor encephalitis

Reproductive Biology and Endocrinology ◽

10.1186/s12958-020-00661-5 ◽

2020 ◽

Vol 18 (1) ◽

Author(s):

Xiaoya Zhao ◽

Juan Li ◽

Qian Zhu ◽

Guiling Liang ◽

Wei Xia ◽

...

Keyword(s):

Western Blotting ◽

Neurological Disorder ◽

Bioinformatics Analysis ◽

Ovarian Teratoma ◽

Control Group ◽

Differentially Expressed Proteins ◽

E Proteins ◽

Unique Role ◽

Itraq Analysis ◽

Aspartate Receptor

Abstract Background Ovarian teratoma-associated anti-N-methyl-D-aspartate receptor encephalitis (NMDAR-E) is a severe autoimmune neurological disorder, and the influence of teratoma-induced autoantibodies on the pathogenesis remains unclear. Methods Ovarian teratoma tissues were collected from teratoma patients with and without NMDAR-E. Proteins were extracted and then analyzed using iTRAQ-coupled LC–MS/MS, which was followed by bioinformatics analysis. Candidate proteins were verified by Western blotting and immunohistochemistry. Results In total, 36 differentially expressed proteins (DEPs) were identified between the control group and NMDAR-E group, and the bioinformatics analysis revealed that the DEPs were mainly involved in immune-related pathways, especially HLA-A and HLA-DRB1. The western blotting results for HLA-A and HLA-DRB1 were consistent with the results of the iTRAQ analysis. Additionally, the immunohistochemical data revealed that the aggregation of HLA-A (+) and HLA-DRB1 (+) cells was more apparent in the teratoma tissues of NMDAR-E patients compared with that in the tissues of controls. Conclusion Our investigation indicated that HLA-A and HLA-DRB1 might be involved in mediating ovarian teratoma-associated NMDAR-E. These findings provide new insights into the pathophysiological mechanisms and provide information for the functional exploration of proteins in the future.

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The diffuse involvement of anti-N-methyl-D-aspartate receptor encephalitis in brain: a case report

BMC Neurology ◽

10.1186/s12883-019-1456-6 ◽

2019 ◽

Vol 19 (1) ◽

Cited By ~ 1

Author(s):

Yun Jiang ◽

Jianpeng Ma ◽

Tao Gong ◽

Hongjun Hao ◽

Haibo Chen

Keyword(s):

Basal Ganglia ◽

Brain Mri ◽

Clinical Manifestations ◽

Autoimmune Encephalitis ◽

Ovarian Teratoma ◽

Mass Effects ◽

Glucose Levels ◽

Aspartate Receptor ◽

Nmdar Encephalitis ◽

Case Presentations

Abstract Background Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe and most common autoimmune encephalitis in patients under 40 years old. Anti-NMDAR encephalitis has various clinical and neuroimaging findings. Here we report a special case of an anti-NMDAR encephalitis who had diffuse lesions in bilateral hemispheres with mild mass effects in left basal ganglia area. Case presentations A 28-year-old female anti-NMDAR encephalitis patient mainly presented with headache and fever. Brain magnetic resonance image (MRI) showed slightly contrasted diffuse lesions, involving the left temporal and frontal lobes, left basal ganglia area and splenium of corpus callosum, as well as the right frontal lobe, with mild edema surrounded in the left basal ganglia area. Cerebrospinal fluid (CSF) revealed a moderate pleocytosis with normal protein and glucose levels. Anti-NMDAR antibodies were identified in CSF. Transvaginal ovarian ultrasound did not reveal an ovarian teratoma. The patient was treated with immunoglobulin and steroid, and had a good recovery. Conclusions Anti-NMDAR encephalitis has no special clinical manifestations and brain MRI is highly variable, which could be unremarkable or abnormal involving white and grey matters. The extensive lesions in frontal and temporal lobes, and basal ganglia area, with mild mass effects, have not been described previously. Recognition of various changes in brain MRI will enable the early detection of anti-NMDAR antibody and then effective treatments.

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