抗N-甲基-D-天冬氨酸受体脑炎9例临床分析

Clinical analysis of 9 cases of anti-NMDAR encephalitis

目的探讨抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的发病机制及临床特点。方法回顾性分析2013年10月-2015年10月陆军总医院神经内科诊治的9例序贯抗NMDAR脑炎患者的临床特点,并通过复习文献,总结本病的病理机制、临床表现、诊断、治疗及预后情况。结果 9例患者中,男3例,女6例,平均年龄39岁。其中5例有前驱非特异性感染;神经系统首发症状6例为精神行为异常(均为女性)、3例为癫痫(均为男性)。病程中9例患者均存在精神行为异常、7例癫痫、8例意识障碍、5例不自主运动、3例通气不足。9例患者的血与脑脊液抗NMDAR抗体均为阳性,均接受人血丙种球蛋白和激素冲击治疗,其中8例治愈或好转,1例无效;随访6个月,8例生活自理、预后良好,1例致残。结论前驱非特异性感染基础上突发精神行为异常合并癫痫,血与脑脊液抗NMDAR抗体阳性是NMDAR脑炎的临床特点,丙种球蛋白和激素冲击治疗有助于临床恢复。

Objective To explore the pathogenesis and clinical features of anti- N-methyl-D-aspartate receptor （NMDAR） encephalitis. Methods The clinical characteristics of 9 patients with anti-NMDAR encephalitis in Department of Neurology, Army General Hospital of Chinese PLA from October 2013 to October 2015, were retrospectively analyzed. And by literatures review, the pathogenesis, clinical feature, diagnosis, treatments and prognosis of this disease were summarized. Results The average age of the 9 patients （3 men and 6 women） was 39 years, 5 of them had precursor nonspecific infection. The initial symptom of neurologic system was mental-behavior disorder in all the 6 females, or epilepsy in all the 3 males. Mental-behavior disorder existed in all the 9 cases during the disease course; including 7 cases of epilepsy, 8 cases of consciousness disturbance, 5 cases of involuntary movement, and 3 cases of inadequate ventilation. The anti-NMDAR antibodies were positively detected from the cerebrospinal fluid and blood of all the 9 cases. After gamma globulin and hormone therapy, 8 cases were cured or better, and only 1 case invalid. A six months follow-up found that 8 cases were independent with eusemia and 1 case got disability. Conclusions The clinical characteristics of anti-NMDAR encephalitis are mental-behavior disorder, or epilepsy upon the basis of a precursor infection, with positive anti- NMDAR antibodies detected from the cerebrospinal fluid and blood. Gamma globulin and hormone therapy may help the clinical recovery.