Department of Neurology, the Affiliated Brain Hospital of Nanjing Medical University, Nanjing, Jiangsu, 210029, People's Republic of China.
BMC Neurol. 2019 Aug 28;19(1):210. doi: 10.1186/s12883-019-1436-x.
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis, which is the most common type of autoimmune encephalitis, is caused by the production of autoantibodies against NMDA receptor. Anti-NMDAR encephalitis patients present with various non-specific symptoms, such as abnormal psychiatric or behaviour, speech dysfunction, cognitive dysfunction, seizures, movement disorders, decreased level of consciousness, and central hypoventilation or autonomic dysfunction.
A 67-year-old man presented with new-onset focal seizures. The brain magnetic resonance imaging (MRI) plain scan and enhanced scan showed abnormal signal on the proximal midline frontoparietal junction region. Anti-NMDAR antibody was detected in cerebrospinal fluid (CSF) and serum using a commercial kit (Euroimmune, Germany) by indirect immunofluorescence testing (IIFT) according to the manufacturer's instructions for twice. Both of the test results were positive in CSF and serum. The patient was diagnosed as anti-NMDAR encephalitis and then was treated repeatedly with large dose of intravenous corticosteroids and gamma globulin. Accordingly, the refractory nature of seizures in this case may be attributed to NMDAR autoantibodies. When the patient presented at the hospital for the third time, the brain MRI revealed an increase in the size of the frontal parietal lesion and one new lesion in the left basal ganglia. The patient underwent a surgical biopsy and astrocytoma was confirmed by histopathology.
Although the sensitivity and specificity of anti-NMDAR-IgG antibodies in CSF to diagnose anti-NMDAR encephalitis are close to 100%, it is not absolute. Anti-NMDAR antibodies were positive, which might make the diagnosis more complex. The diagnosis of atypical presentation of anti-NMDAR encephalitis requires reasonable exclusion of other disorders.
抗 N-甲基-D-天冬氨酸受体(anti-NMDAR)脑炎是最常见的自身免疫性脑炎类型,由针对 NMDA 受体的自身抗体产生引起。抗 NMDAR 脑炎患者表现出各种非特异性症状,如异常精神或行为、言语功能障碍、认知功能障碍、癫痫发作、运动障碍、意识水平下降、中枢性低通气或自主神经功能障碍。
一名 67 岁男性出现新发局灶性癫痫发作。脑部磁共振成像(MRI)平扫和增强扫描显示中线额顶交界区近端异常信号。根据制造商的说明,使用商业试剂盒(德国 Euroimmune)通过间接免疫荧光检测(IIFT)在脑脊液(CSF)和血清中检测到抗 NMDAR 抗体,结果为两次阳性。CSF 和血清中的测试结果均为阳性。患者被诊断为抗 NMDAR 脑炎,然后反复接受大剂量静脉注射皮质类固醇和丙种球蛋白治疗。因此,本例癫痫发作的难治性可能归因于 NMDAR 自身抗体。当患者第三次到医院就诊时,脑部 MRI 显示额顶病变的大小增加,左侧基底节区出现一个新病变。患者接受了手术活检,组织病理学证实为星形细胞瘤。
尽管 CSF 中抗 NMDAR-IgG 抗体诊断抗 NMDAR 脑炎的灵敏度和特异性接近 100%,但并非绝对。抗 NMDAR 抗体阳性可能使诊断更加复杂。对不典型抗 NMDAR 脑炎表现的诊断需要合理排除其他疾病。