Abstract:

Objective  To study clinical features, diagnosis, therapy response and prognosis of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis.  Methods  Three cases with anti-NMDAR encephalitis were reported. The clinical features, laboratory examinations, imaging, EEG and therapy response of 3 cases were retrospectively analyzed, and also related literatures were reviewed.  Results  Two patients were young male and one patient was old female. Main symptoms included psychiatric symptoms in 3 cases (mania in 2 male patients and stupor in the female patient), epilepsy in 2 cases and respiratory failure in one case. The results of MRI examination revealed normal, while EEG examination showed abnormal in all cases. No tumor was detected in any of these patients. Lumbar puncture revealed normal cerebrospinal fluid (CSF) pressure (3 cases), elevated white blood cell (WBC, 3 cases) and protein quantification (one case). All cases were confirmed to have the disease by detection of anti-NMDAR antibodies in serum and CSF. One male patient got better after receiving immunotherapy with methylprednisolone and intravenous immunoglobulin (IVIg), but psychiatric symptoms were left over. Another male patient had no response to the above treatment. But the female patient was improved without immunotherapy. All 3 cases were followed up for one year after being discharged. One male patient died by accident because of mental disorders. Another male patient showed no sign of relief. The female patient got mild personality and memory change.  Conclusions  Anti-NMDAR encephalitis is a new type of autoimmune encephalitis. It is characterized by fever, memory deficits, seizures, disturbance of consciousness, and autonomic dysfunction in males and females of all ages. This type of encephalitis is often associated with teratoma, and has a good response to immunotherapy. There is a certain correlation between progression and prognosis.

Key words: Receptors, N-methyl-D-aspartate, Encephalitis

摘要：

目的 探讨抗N-甲基-D-天冬氨酸受体（NMDAR）脑炎的临床表现、诊断、治疗及预后，以期引起临床医师的重视。方法与结果 回顾分析3 例抗NMDAR 脑炎患者的诊断与治疗经过，结合临床表现、实验室检查、影像学、脑电图及对治疗的反应，并复习相关文献。3 例患者中2 例为青年男性、1 例为老年女性；均有精神行为异常，2 例青年男性患者表现为精神亢奋、1 例老年女性患者表现为木僵状态；2 例伴癫痫发作；1 例呼吸机辅助通气。腰椎穿刺脑脊液压力正常，白细胞计数升高，1 例蛋白定量升高。血清和脑脊液抗NMDAR 阳性。头部MRI 未见明显异常。脑电图呈现异常。2 例青年男性患者予抗病毒、激素和静脉注射免疫球蛋白治疗，1 例病情好转但遗留精神行为异常，1 年后随访，因家属照料疏忽而意外死亡，1 例病情无变化；1 例老年女性患者仅予抗病毒治疗，遗留轻度记忆和人格变化。结论 抗NMDAR 脑炎是一种新型自身免疫性脑炎，临床表现多样，无特异性，主要为发热、记忆障碍、癫痫发作、意识障碍、自主神经系统功能紊乱，伴或不伴畸胎瘤，对免疫治疗有效。病程与预后存在一定的相关性。

关键词: 受体, N-甲基-D-天冬氨酸, 脑炎