Anti-N-methyl-D-aspartate receptor encephalitis with accompanying ovarian teratoma in female patients from East China: Clinical features, treatment, and prognostic outcomes

Purpose: The association between anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDAR encephalitis) and teratoma is formally recognized. This study compared the clinical features, treatments and outcomes between female patients with or without accompanying teratomas and determined the potential influences of coexisting teratomas.Method: Fifty-six female patients diagnosed with anti-NMDAR encephalitis were enrolled in two major tertiary hospitals in East China from January 2013 through March 2018 and were grouped as patients with or without teratoma. The clinical features were reviewed, and follow-up studies were performed. Comparisons were made between the two groups.Results: Patients with teratoma reported fewer viral prodromes (p = 0.0085) and stronger positive intensity of anti-NMDAR antibodies in cerebrospinal fluid (CSF) (p = 0.0368), while nontumor patients tended to demonstrate lymphocytic pleocytosis in CSF (p = 0.0306). Seizure types varied between individuals, with complex partial seizures more common in teratoma patients (p = 0.0105). Nontumor patients frequently required combinations of first-line and second-line immunotherapy (p = 0.0014), which may be attributed to higher mRS scores at admission (p = 0.0300). Also, they had higher mean mRS scores since the 12-month follow-up and greater probability of relapse than did patients with teratomas (p = 0.0286).Conclusions: Symptomatology and auxiliary findings indicate that ovarian teratoma may be the immunologic trigger for anti-NMDAR encephalitis patients, while viral infection is likely to play a major part in pathogenesis for those without any detectable tumor. Overall, anti-NMDAR encephalitis patients with teratomas present with milder neurological symptoms and have better long-term outcomes after tumor removal.

目的：抗N -甲基- D -天冬氨酸受体脑炎（抗NMDAR脑炎）与畸胎瘤之间的关联已得到正式确认。本研究比较了伴有和不伴有畸胎瘤的女性患者的临床特征、治疗方法和预后，并确定了共存畸胎瘤的潜在影响。 方法：2013年1月至2018年3月，中国东部两家大型三级医院共纳入56例确诊为抗NMDAR脑炎的女性患者，根据是否伴有畸胎瘤分为两组。回顾临床特征并进行随访研究，对两组进行比较。 结果：畸胎瘤患者报告的病毒前驱症状较少（p = 0.0085），脑脊液（CSF）中抗NMDAR抗体阳性强度更强（p = 0.0368），而非肿瘤患者往往在脑脊液中表现出淋巴细胞增多（p = 0.0306）。癫痫发作类型因人而异，复杂部分性发作在畸胎瘤患者中更为常见（p = 0.0105）。非肿瘤患者经常需要一线和二线免疫治疗联合使用（p = 0.0014），这可能是由于入院时改良Rankin量表（mRS）评分较高（p = 0.0300）。此外，在12个月的随访中，他们的平均mRS评分较高，复发的可能性也比畸胎瘤患者大（p = 0.0286）。 结论：症状学和辅助检查结果表明，卵巢畸胎瘤可能是抗NMDAR脑炎患者的免疫触发因素，而病毒感染可能在无任何可检测肿瘤的患者的发病机制中起主要作用。总体而言，伴有畸胎瘤的抗NMDAR脑炎患者神经症状较轻，肿瘤切除后长期预后较好。