卵巢畸胎瘤相关性抗N-甲基-D-天冬氨酸受体脑炎:7例报告并文献复习

Clinical analysis of 7 cases of ovarian teratoma complicated with anti-N-methyl-D-aspartate receptor encephalitis

目的探讨卵巢畸胎瘤相关性抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的临床特征、治疗方法及预后。方法回顾性分析南方医科大学珠江医院2014年7月～2017年12月收治的7例抗NMDAR脑炎合并卵巢畸胎瘤患者的临床资料结果本报道7例患者病程中均表现有异常精神行为,其中2例患者以此为首发症状,3例患者因中枢性低通气,予气管切开机械辅助通气,1例患者仅表现为精神行为异常及前驱症状。7例患者行脑脊液特异性检测均可见抗NMDAR抗体阳性,血清学检测抗NMDAR抗体结果为6例阳性,1例阴性。7例患者病情进展不完全一致,但均在手术切除卵巢畸胎瘤后病情好转。随访至今(半年～3年),7例患者现一般情况可,精神行为基本正常,未见脑炎及卵巢畸胎瘤复发。结论抗NMDAR脑炎是一种新的边缘叶副肿瘤性脑炎,目前大部分研究提示其与卵巢畸胎瘤密切相关,合并有卵巢畸胎瘤的抗NMDAR脑炎在手术切除卵巢畸胎瘤后病情好转快,大部分患者预后良好。

Objective To evaluate the clinical characteristics,treatment and prognosis of ovarian teratoma patients complicated with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.Methods The clinical data of 7 patients with ovarian teratoma complicated with anti-NMDAR encephalitis treated in Zhujiang Hospital of Southern Medical University from July,2014 to December,2017 were collected and analyzed retrospectively.Results The average age of the 7 patients was 26 years old.All the patients had abnormal mental behavior,and 2 patients had psychosis as the first symptom.3 patients were ventilated by tracheotomy because of central hypoventilation,while 1 patient showed only abnormal mental behavior and prodromal symptoms.Anti-NMDAR antibodies were detected in cerebrospinal fluid test in all 7 patients,but Blood samples were tested only in 6 patients.All the patients Included had ovarian teratoma,and all of them improved after the resection of ovarian teratoma.No recurrence was observed during subsequent follow-up.Conclusions Anti-NMDAR encephalitis is a new type of marginal leaf paraneoplastic encephalitis,which is closely related to ovarian teratoma.The prognosis of the patients with anti NMDAR encephalitis with ovarian teratoma is better,and most of the patients can be recovered after surgical excision of ovarian teratoma.