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目的研究抗N-甲基-D-天门冬氨酸(NMDA)受体脑炎患者的临床特点及治疗体会,提高对本病的认识。方法对22例经血清或脑脊液抗NMDA受体抗体检测确诊为抗NMDA受体脑炎的患者进行回顾性分析,观察其人口学资料、病史、临床表现、实验室检查、影像学检查及治疗效果等资料。结果 22例患者中女15例(68%),男7例(32%),主要临床表现为精神行为异常(91%)、癫痫发作(86%)、意识障碍(50%)。实验室检查:22例均为血清和(或)脑脊液抗NMDA受体抗体阳性。脑电图表现为额颞顶慢波活动8例(47%),颞额顶区尖棘波或尖棘慢波发放3例(18%),额颞顶慢波活动伴有尖波、部分棘波4例(24%),未见异常2例(22%)。头颅MRI+增强显示(软)脑膜强化10例(45%),弥漫性脑萎缩3例(14%)。腹部彩超或CT检查显示合并肿瘤6例(27%)。给予静脉用丙种球蛋白、甲泼尼龙冲击、控制癫痫发作、改善精神症状等综合治疗后,取得理想疗效16例(73%),疗效较好5例(23%),疗效差1例(4%)。其中发现肿瘤者均为女性,附件肿瘤5例,纵隔肿瘤1例,均在药物治疗病情相对稳定的情况下行手术摘除术4例。无意识障碍患者11例,治疗显效率91%,有效率9%;2周内出现意识障碍患者6例,治疗显效率33%,有效率50%;2周以上昏迷患者5例,治疗显效率80%,有效率20%。结论抗NMDA受体脑炎可在儿童和成人出现,以女性多见,临床常表现为精神行为异常、癫痫发作及意识障碍三主症,可伴发肿瘤,伴发肿瘤者宜尽早手术治疗。此病的预后与患者起病至昏迷时间有关,越短预后越差,无意识障碍患者治愈率高,而预后与抗NMDA滴度不呈线性关系。
Objective To study the clinical features and treatment of patients with anti-N-methyl-D-aspartate (NMDA) receptor encephalitis and to improve their understanding of the disease. Methods Twenty-two patients with anti-NMDA receptor antibodies detected by serum or cerebrospinal fluid anti-NMDA receptor encephalitis were retrospectively analyzed. Demographic data, medical history, clinical manifestations, laboratory tests, imaging findings and therapeutic effects Other information. Results Among the 22 patients, 15 were female (68%) and 7 were male (32%). The main clinical manifestations were mental disorders (91%), seizures (86%) and disturbance of consciousness (50%). Laboratory tests: All 22 patients were positive for anti-NMDA receptor antibodies in serum and / or cerebrospinal fluid. Electroencephalography showed frontotemporal and slow wave activities in 8 cases (47%), temporal frontal area spike or spine spike slow waves were issued in 3 cases (18%), frontotemporal and temporal wave activity associated with sharp waves, part of Spikes in 4 cases (24%), no abnormalities in 2 cases (22%). Cranial MRI + enhancement showed (soft) meningeal enhancement in 10 (45%) and diffuse brain atrophy in 3 (14%). Abdominal ultrasonography or CT examination showed 6 cases (27%) with tumor. After intravenous administration of gamma globulin, methylprednisolone, seizure control and psychiatric symptoms, 16 patients (73%) achieved satisfactory results, 5 patients (23%) had good response, 1 patient had poor response (4 patients) %). Among them, the tumors were found in women, 5 cases of accessory tumor and 1 case of mediastinal tumor, all of which underwent surgical resection in the relatively stable condition of drug treatment. In 11 patients with unconsciousness, the effective rate was 91% and the effective rate was 9%. Six patients with disturbance of consciousness in two weeks showed a markedly effective rate of 33% and an effective rate of 50%. Five patients with unconsciousness more than 2 weeks showed a markedly effective rate of 80% %, Efficient 20%. Conclusion Anti-NMDA receptor encephalitis can occur in children and adults, more common in women, the clinical manifestations of mental disorders, seizures and disturbance of consciousness three main symptoms may be associated with cancer, associated with tumor surgery should be as soon as possible. The prognosis of the disease and the onset of the patient to a coma time, the shorter the worse the prognosis, unconscious patients with high cure rate, and prognosis and anti-NMDA titer was not linear.