23例抗N-甲基-D-天冬氨酸受体脑炎的临床特点及预后分析

The clinical characteristic and prognostic analysis of 23 cases of anti-N-methyl-D-aspartate receptor encephalitis

目的探讨抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的临床特点及预后。方法回顾性分析23例抗NMDAR脑炎患者(其中重症9例)的临床资料。结果重症患者9例(男2例,女7例)入住ICU治疗,其余14例(男3例,女11例)入住神经内科治疗;发病年龄(32.6±14.6)岁;病程(26.7±36.9)d;住院时间(34.2±27.5)d,其中重症患者ICU平均住院时间(22.2±13.7)d。主要临床表现有精神行为异常(21例),发热(13例)、癫痫发作(19例)、意识障碍(15例)、运动功能异常(2例)、自主神经功能障碍(13例)及低通气(5例)。1例合并畸胎瘤。所有患者CSF抗NMDAR抗体均阳性,18例血清抗NMDAR抗体阳性。5例患者头颅MRI示颞叶、海马或额顶叶异常信号。19例患者EEG示异常慢波。23例患者接受糖皮质激素、丙种球蛋白或血浆置换等免疫调节治疗。6例患者完全恢复,17例患者症状改善伴残留症状。结论抗NMDAR脑炎常表现为进展迅速的精神行为改变、癫痫等症状,还有意识障碍、运动障碍、自主神经功能障碍,合并肿瘤并不多见。抗NMDAR脑炎患者积极免疫治疗预后较好。

Objective To investigate the clinical characteristics and prognosis of anti-N-methyl-D-aspartate receptor（ NMDAR） encephalitis. Methods The clinical data of 23 anti-NMDAR encephalitis patients,including 9 cases of severe patients were retrospectively analyzed. Results There were 9 cases of severe patients（ including 2 males and 7 females） admitted to ICU for the treatment,and 14 patients（ including 3 males and 11 females）admitted to the neurology department for the treatment. The age of the patients was（ 32. 6 ± 14. 6） years,the course of disease was（ 26. 7 ± 36. 9） d,and the length of stay was（ 34. 2 ± 27. 5） days. In critically ill patients,the average length of stay was（ 22. 2 ± 13. 7） d. The main clinical manifestations included 21 patients with mental and behavioral abnormalities, 13 patients with fever, 19 patients with epilepsy, 15 patients with conscious disturbance,2 patients with motor dysfunction,13 patients with dysautonomia,and 5 patients with hypoventilation.There was 1 case of patients with teratoma. The anti-NMDAR antibodies of CFS were positive in all patients,of which18 cases of serum anti-NMDAR antibody were positive. Five patients of head MRI showed the abnormal signal of the temporal lobe,hippocampus or frontal lobe. The EEG of 19 patients showed abnormal slow wave. 23 patients received the glucocorticoid,the gamma globulin,the plasma exchange and other immunomodulatory treatment. Six patients were completely cured,and 17 patients were improved but with residual symptoms. Conclusions The patients with Anti-NMDAR encephalitis usually manifest such as the rapid changes in mental behavior,the epilepsy and other symptoms, as well as the disturbance of consciousness, the movement disorders, the autonomic dysfunction,but of which combined tumors are rare. The prognosis of patients with anti NMDAR encephalitis is better,who received positive immunotherapy.