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抗N-甲基-D-天冬氨酸受体脑炎患者的临床特征、治疗及预后——中国单中心回顾性分析

Clinical features, treatments, and outcomes of patients with anti--methyl-D-aspartate encephalitis-a single-center, retrospective analysis in China.

作者信息

Xu Qianhui, Zhu Yong, Wang Qian, Han Jing, Mao Fengju, Zeng Silin, Chen Siyan, Zhao Chenyong, Gu Mei, Li Zaiwang, Luo Xiaoguang, Fu Xuejun, Huang Ying

机构信息

Department of Neurology, Shenzhen People's Hospital (The Second Clinical Medical College, Jinan University, The First Affiliated Hospital, Southern University of Science and Technology), 518020 Shenzhen, Guangdong, China.

Department of Medical Record Management, Shenzhen People's Hospital (The Second Clinical Medical College, Jinan University, The First Affiliated Hospital, Southern University of Science and Technology), 518020 Shenzhen, Guangdong, China.

出版信息

Front Biosci (Landmark Ed). 2021 Sep 30;26(9):496-503. doi: 10.52586/4962.

Abstract

: To describe the clinical features, laboratory data, treatment, and outcomes of anti--methyl-D-aspartate (NMDAR) encephalitis in Chinese patients. : This retrospective study included hospitalized patients definitively diagnosed with anti-NMDAR encephalitis and positive for anti-NMDAR antibodies in the cerebrospinal fluid (CSF) in Shenzhen People's hospital, between November 2015 and February 2020. The clinical manifestation, laboratory data, treatments and outcomes were collected retrospectively. Patients were followed up for more than 1 year. : The study included 31 patients (15 men, 48.4%) with a median age of 31 years (interquartile range 21-48). The most common clinical presentations were psychosis ( = 23, 74.2%), seizures ( = 20, 64.5%), and memory impairment ( = 20, 64.5%). Total magnetic resonance imaging abnormalities were found in 11 patients (35.5%), with the medial temporal and frontal lobes as the most commonly involved. Abnormal electroencephalogram was observed in 16 patients (51.6%). Five out of 31 patients (19.5%) were diagnosed as neoplasm, including five females with ovarian teratoma and one male with a central nervous system tumor. Multiple immune antibodies, including anti-SSA antibody in four patients (15.4%), anti-Ro52 antibody in four (15.4%), antinuclear antibody (ANT) in four (15.4%), anti-thyroglobulin antibodies (TGAb) in five (17.2%), and thyroid peroxidase antibodies (TPOAb) in three (10.3%) were present. All patients received first-line immunization therapy (intravenous immunoglobulin, glucocorticoids, or plasmapheresis alone or combined), and only two patients (7.3%) received second-line immunization therapy (rituximab). Mechanical ventilation was more necessary in women (37.5%) than in men (6.7%) ( = 0.04), and 29 (93.5%) had favorable clinical outcomes. At more than 12 months of follow-up, the median modified Rankin Scale score decreased from 4 to 0. : Patients with anti-NMDAR encephalitis in China had high rates of psychosis and seizures, with low rates of underlying neoplasms. A higher proportion of female patients required mechanical ventilation. Complications with other positive autoimmune antibodies were a common clinical symptoms of anti-NMDAR encephalitis. Majority of the patients obtained satisfactory outcomes in combination with early first-line and long-term immunization therapy.

摘要

描述中国抗N-甲基-D-天冬氨酸(NMDAR)脑炎患者的临床特征、实验室数据、治疗方法及预后。本回顾性研究纳入了2015年11月至2020年2月期间在深圳市人民医院住院、确诊为抗NMDAR脑炎且脑脊液(CSF)中抗NMDAR抗体呈阳性的患者。回顾性收集其临床表现、实验室数据、治疗方法及预后情况。对患者进行了超过1年的随访。该研究纳入了31例患者(15例男性,占48.4%),中位年龄为31岁(四分位间距为21 - 48岁)。最常见的临床表现为精神症状(23例,占74.2%)、癫痫发作(20例,占64.5%)和记忆障碍(20例,占64.5%)。11例患者(35.5%)磁共振成像检查发现异常,其中内侧颞叶和额叶最常受累。16例患者(51.6%)脑电图异常。31例患者中有5例(19.5%)被诊断为肿瘤,包括5例患有卵巢畸胎瘤的女性和1例患有中枢神经系统肿瘤的男性。存在多种自身免疫抗体,包括4例患者(15.4%)的抗SSA抗体、4例(15.4%)的抗Ro52抗体、4例(15.4%)的抗核抗体(ANA)、5例(17.2%)的抗甲状腺球蛋白抗体(TGAb)和3例(10.3%)的甲状腺过氧化物酶抗体(TPOAb)。所有患者均接受了一线免疫治疗(单独或联合使用静脉注射免疫球蛋白、糖皮质激素或血浆置换),仅有2例患者(7.3%)接受了二线免疫治疗(利妥昔单抗)。女性患者(37.5%)比男性患者(6.7%)更需要机械通气(P = 0.04),29例(93.5%)患者临床预后良好。随访超过12个月时,改良Rankin量表评分中位数从4降至0。中国抗NMDAR脑炎患者精神症状和癫痫发作发生率高,潜在肿瘤发生率低。女性患者需要机械通气的比例更高。合并其他自身免疫抗体阳性是抗NMDAR脑炎常见的临床症状。多数患者通过早期一线及长期免疫治疗获得了满意的预后。

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