晚发型抗N-甲基-D-天冬氨酸受体脑炎临床特征分析

目的分析晚发型抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的临床特征。方法收集2010-01-01—2019-05-01于郑州大学第一附属医院住院确诊为抗NMDAR脑炎的患者临床资料,分析晚发型(≥50岁)患者数据,并与早发型(18~49岁)患者进行对比。结果18例晚发型患者中,男11例(61%),发病年龄50~84岁。晚发型患者中,9例(50%)患者出现前驱症状,精神行为异常是最常见的首发症状(44%)和临床表现(78%);头颅磁共振提示脑实质炎性病变9例(9/17,53%);脑脊液检验结果异常17例(94%);合并肿瘤4例(22%),均非畸胎瘤。相比于早发组患者,晚发组患者有更高的自主神经功能障碍比例(72%vs 45%,P=0.032),更高的岛叶病变比例(67%vs 27%,P=0.047),更高的脑脊液蛋白升高比例(56%vs 28%,P=0.023),以及更高的脑脊液鞘内IgG合成率升高比例(73%vs 44%,P=0.041)。晚发组合并肿瘤均非畸胎瘤,早发组合并肿瘤均为畸胎瘤(P=0.001)。结论相比早发型抗NMDAR脑炎患者,晚发型患者更易出现岛叶病变,更易出现脑脊液炎症反应,其发病机制可能与畸胎瘤不相关。

Clinical characteristics analysis of late-onset anti-N-methyl-D-aspartate receptor encephalitis

Objective To analyze the clinical features of late-onset anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis.Methods The data of anti-NMDAR encephalitis which were diagnosed in the First Affiliated Hospital of Zhengzhou University from January 1,2010 to May 1,2019 was collected.The data oflate-onset(≥50 years old)anti-NMDAR encephalitis was analyzed.Comparisons were conducted between late-onset and early-onset(18-49 years old)anti-NMDAR encephalitis.Results There were 11 male patients(61%)in 18 older adults,whose onset age ranged from 50 to 84 years old.In late-onset patients with anti-NMDAR encephalitis,nine(50%)patients had prodromal symptoms,and mental and behavioral abnormalities were the most common initial symptoms(44%)and clinical manifestations(78%).Nine(53%)of 17 patients with available brain MRI had suggested inflammatory lesions;Seventeen(94%)patients had abnormal cerebrospinal fluid(CSF).Four(22%)patients had an underlying tumor,and none of them had teratoma.Compared with early-onset patients with anti-NMDAR encephalitis,late-onset patients had a higher proportion of autonomic instability(72%vs 45%,P=0.032),a higher proportion of insular lobe lesion in brain MRI(67%vs 27%,P=0.047),a higher proportion of elevated CSF protein(56%vs 28%,P=0.023),and a higher proportion of elevated 24h CSF IgG synthesis rate(73%vs 44%,P=0.041).No late-onset anti-NMDAR encephalitis was accompanied with teratoma compared with 100%of early-onset patients with tumor(P=0.001).Conclusion Compared with early-onset patients with anti-NMDAR encephalitis,late-onset patients are more vulnerable to insular lobe lesion in brain MRI and inflammatory reaction in CSF,its pathogenesis may not be related to teratoma.