抗N-甲基-D-天冬氨酸受体抗体脑炎复发的临床特征

目的总结复发性抗N-甲基-D-天冬氨酸受体(NMDAR)抗体脑炎的临床特点、诊断及治疗。方法回顾性分析2013年5月至2017年2月郑州大学人民医院诊治的57例抗NMDAR抗体脑炎中10例复发者的临床症状、实验室检查、影像学特点和免疫治疗。结果 10例(19%)复发中男5例,女5例,平均年龄27.5岁,10例共复发14次,其中2例复发2~3次,平均复发间隔时间7.1个月(1~19月)。复发时最常见症状为癫痫(8/14)和精神行为异常(8/14),其中1例次复发仅表现为不典型症状,如眩晕、行走不稳和复视。复发时13例次行抗NMDAR抗体检测,脑脊液抗体阳性率100%(13/13),血清抗体阳性率69%(9/13)。13例次复发再次给予免疫治疗仍有效,1例次拒绝治疗,出院后平均随访18.9月,3例完全恢复正常,4例遗留轻微后遗症,3例遗留较明显后遗症,影响日常生活。结论抗NMDAR脑炎复发时可表现为典型或不典型症状,脑脊液抗NMDAR抗体检测有助诊断,积极免疫治疗仍有效。

Clinical relapse of anti-N-methyl-D-aspartate receptor encephalitis

Objective To investigate the clinical features,diagnosis,and therapeutic strategies of relapsing anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.Methods A retrospective analysis was performed on 10 relapsing patients from 57 patients with antiNMDAR encephalitis who were diagnosed and treated in the People's Hospital of Zhengzhou University from May 2013 to February 2017 to investigate their clinical symptoms,laboratory examinations,radiological features,and immunotherapy.Results In the 10 relapsing patients,there were 5 males and 5 females,with a mean age of 27.5 years.Totally,14 relapses occurred in the 10 cases,with 2-3 relapses in 2 cases,and the mean interval between two relapses was 7.1 months (1-19 months).The clinical symptoms of the relapses were epilepsy (8/14),mental and behavioral disorders (8/14),and atypical relapsing symptoms such as dizziness,ataxia,and diplopia (1/14).Anti-NMDAR detection was performed in the relapsing patients (13 relapses).The positive detection rate was 100％ (13/13) for cerebrospinal fluid samples and 69％ (9/13) for serum samples.Immunotherapy was effective for the 13 relapses,and was refused by 1 relapsing case.These 10 patients underwent a follow up of mean 18.9 months,with complete recovery in 3 cases,mild sequelae in 4 cases,and severe sequelae in 3 cases.Conclusions Both typical and atypical symptoms are found when relapse occurs in patients with anti-NMDAR encephalitis.Test for anti-NMDAR antibody may help to make an early diagnosis.An active immunotherapy strategy may lead to a better prognosis.