抗N-甲基-D-天冬氨酸受体脑炎患者的临床、磁共振成像和脑脊液特征分析

The characteristics of clinical manifestations, brain MRI and cerebrospinal fluid findings in patients with anti-N-methyl-D-aspartate receptor encephalitis

目的 分析我国成年人抗N-甲基-D-天冬氨酸（NMDA）受体脑炎患者的临床、头MRI和脑脊液特点.方法 收集29例抗NMDA受体脑炎患者的临床、MRI和脑脊液资料.结果 29例抗NMDA受体脑炎中,主要临床表现为精神异常占86％（25例）,癫痫83％（24例）,意识障碍55％（16例）,不自主运动55％（16例）,中枢性通气障碍34％（10例）,大量唾液分泌17％（5例）,尚有自主神经紊乱、偏瘫、失语等.14％（4例）伴有畸胎瘤.头MRI异常者占62％（18例）,病灶分布于颞叶、海马、丘脑、脑干、扣带回、额顶叶、胼胝体、内囊/基底节/脑室旁.83％腰椎穿刺结果异常,其中脑脊液寡克隆区带阳性率为95％ （19/20）.3年内复发率为31％ （9/29）.结论 抗NMDA受体脑炎患者主要临床表现为精神异常、癫痫,特征性临床表现有不自主运动、中枢性通气障碍、大量唾液分泌;MRI病灶分布不仅局限于边缘叶;是复发率较高但可治疗的疾病.

Objective To analyze the clinical manifestations and the features of brain MRI and cerebrospinal fluid （CSF） findings in adult Chinese patients with anti-N-methyl-D-aspartate receptor （NMDAR） encephalitis.Methods We reviewed the clinical manifestations,brain MRI and CSF examinations of 29 patients who were diagnosed as anti-NMDAR encephalitis.Results The major clinical features of anti-NMDAR encephalitis patients included psychiatric symptoms （86%,25/29）,seizures （83%,24/29）,decreased consciousness （55%,16/29）,involuntary movements （55%,16/29）,central hypoventilation （34%,10/29）,and hypersalivation （17%,5/29）.Some patients also experienced autonomic instability,hemiplegia and aphasia.Underlying ovarian teratoma was identified in 14% of affected patients（4/29）.Brain MRI was found abnormal in up to 62% patients （18/29）,located in the temporal lobes,hippocampus,thalamus,brain stem,cingulate gyrus,frontal and parietal cortex,corpus callosum,internal capsule,basal ganglia and periventricular area.CSF findings were abnormal in 83% of patients with anti-NMDAR encephalitis.Oligoclonal banding in CSF was positive in 95% patients （19/20）.The recurrence rate during 3 years was 31% （9/29）.Conclusions Anti-NMDAR encephalitis is a treatable disease,yet with high recurrence rate.Its predominant clinical features are psychiatric symptoms and seizures,while involuntary movements,central hypoventilation and hypersalivation are its characteristic manifestations.Lesions in MRI are widespread,not only restricted to limbic lobe.