Abstract
Objective To study the clinical characteristics and surgical outcomes of anti-NMDAR encephalitis and the immunopathology of associated teratomas. Methods Twenty-one patients were enrolled in this retrospective study, who were diagnosed with anti-NMDAR encephalitis with ovarian teratoma and admitted to two tertiary hospitals in South China from July 2014 to December 2019. The clinical data of patients were reviewed. Comparisons were made between the patients with different outcomes after surgery. Immunohistochemical analyses of associated ovarian teratomas were performed. Results The mean age of the patients was 24.33 ± 5.12 years. The peak seasons of disease onset were autumn and winter (30.61% and 32.65%). The symptoms could be divided into 8 categories, including psychiatric abnormalities, seizures, movement dysfunction, consciousness disorders, autonomic dysregulation, speech disturbance, central hypoventilation, and memory deficits. All patients developed four or more categories of symptoms within the first four weeks. Twelve patients (57.1%) had a maximum mRS of 5, and 11 patients (52.4%) were admitted to ICU. Twenty patients received surgery, and only 3 patients were diagnosed pathologically with immature ovarian teratomas, while the other 17 patients had mature ovarian teratomas. After surgery, 17 patients (85.0%) got clinical improvement. The central hypoventilation symptom and mature ovarian teratomas were associated with surgical outcome. Immunohistochemical analysis revealed that there were NMDAR-positive neural tissues in all 8 teratomas and in which 3 cases also contained large numbers of NMDAR-positive sebaceous glands and squamous epithelial tissues. Conclusion The disease is of high prevalence in autumn and winter. The central hypoventilation symptom and mature ovarian teratomas were associated with surgical outcome. NMDAR-positive neural tissue is not the only etiological factor of encephalitis. We speculate that encephalitis development in some patients may result from NMDAR expression in sebaceous glands and squamous epithelial tissues.
Highlights
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis belongs to immune-mediated disorder with prominent neuropsychiatric symptoms, which is serious and potentially lethal
Patients diagnosed with anti-NDAMR encephalitis with ovarian teratoma, who were admitted into the Third Affiliated Hospital, Sun Yat-sen University, and Zhujiang Hospital, Southern Medical University, Guangzhou, China, from July 1, 2014 to December 30, 2019, were enrolled in this study, which was approved by the ethics committee of the Third Affiliated Hospital, Sun Yat-sen University (No [2017]2-198)
3 patients’ ovarian teratomas showed positive for NDMAR1, NMDAR2A, and NMDAR2B in the sebaceous glands and squamous tissues (Figure 4). This retrospective study demonstrates that patients with anti-NMDAR encephalitis with ovarian teratoma tend to present more neuropsychiatric symptoms than gynecological symptoms
Summary
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis belongs to immune-mediated disorder with prominent neuropsychiatric symptoms, which is serious and potentially lethal. It was firstly identified by Dalmau et al [1]. Along with the progress of anti-NMDAR antibody detection and the growing attention paid to the disease, related researches began to rise rapidly. Several autoimmune diseases are known to have well-established seasonal variation. There is still little knowledge about the anti-NDMAR encephalitis’s onset season. Teratoma is a confirmed trigger of anti-NMDAR encephalitis [2]. About 50% of the female patients with antiNMDAR encephalitis were identified to have teratoma [3]
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