抗N-甲基-D-天冬氨酸受体脑炎八例分析

Analysis of 8 Patients of Anti-N-Methyl-D-Aspartate Receptor Encephalitis

目的:探讨抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎患者的临床表现、辅助检查特点、治疗和预后。方法:回顾性分析我院8例确诊为抗NMDAR脑炎患者的临床资料。结果:5例患者出现前驱症状;所有患者临床症状均出现快速进展的精神行为异常、认知障碍,4例为首发症状,此外表现有言语障碍、癫痫发作、运动障碍、意识水平下降及自主神经功能障碍等;8例患者脑脊液抗NMDAR抗体阳性,3例头MRI检查显示异常病灶,位于大脑皮质、丘脑、海马、脑干等部位。6例患者脑电图异常,为弥漫性慢波或局灶性痫样放电。所有患者均接受一线免疫治疗,延误诊治的1例患者对治疗反应差并出现复发。结论:抗NMDAR脑炎临床表现复杂多样,但具有其特点,对于出现不明原因的精神行为异常或认知障碍的青年患者,及时行抗NMDAR抗体筛查十分必要,早期治疗预后良好。

Objective:To investigate the clinical presentation,auxiliary examination,treatment,and prognosis of anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis.Methods:The clinical data of 8 patients with anti-NMDAR encephalitis were retrospectively analyzed.Results:Five patients experienced a prodrome.All patients developed rapidly progressive psychiatric symptoms or cognitive impairment,and these were the initial symptom for 4 patients.Additional clinical manifestations included speech disorders,epileptic seizures,movement disorders,decreased level of consciousness,and autonomic nerve dysfunction.All patients were diagnosed by the presence of NMDAR antibodies in the cerebrospinal fluid;3 patients presented with abnormal lesions in brain magnetic resonance imaging(MRI)located in areas including the cerebral cortex,thalamus,hippocampus,and brain stem.Abnormal electroencephalograms showing diffuse slow waves or focal epileptic discharge were found in 6 patients.All patients received first-line immunotherapy,and a delayed diagnosis in 1 patient resulted in poor response to treatment and recurrence.Conclusion:The clinical manifestations of anti-NMDAR encephalitis are complex and varied but have distinctive characteristics.For young patients with unexplained mental/behavioral abnormalities or cognitive impairment,timely screening for anti-NMDAR antibodies is necessary,as early treatment leads to a good outcome.