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抗N-甲基-D-天冬氨酸受体抗体脑炎的谱系:临床特征、管理与结局

Spectrum of Anti-NMDA Receptor Antibody Encephalitis: Clinical Profile, Management and Outcomes.

作者信息

Datta Amlan Kusum, Pandit Alak, Biswas Samar, Biswas Atanu, Roy Biman Kanti, Gangopaddhyay Goutam

机构信息

Department of Neurology, Bangur Institute of Neurosciences, Institute of Post Graduate Medical Education and Research, Annex 1, Gokhel Road, Bhowanipore, Kolkata, West Bengal, India.

出版信息

Ann Indian Acad Neurol. 2021 May-Jun;24(3):383-389. doi: 10.4103/aian.AIAN_817_20. Epub 2021 Mar 1.

Abstract

BACKGROUND

Anti-N-methyl D-aspartate receptor (anti NMDAR) antibody encephalitis is an immune-mediated entity characterised by a constellation of neuro-psychiatric symptoms.

OBJECTIVE

To describe clinical profile and treatment outcomes of patients with anti NMDAR antibody encephalitis.

SETTINGS AND DESIGN

Subjects were selected by screening for all patients satisfying Graus .'s criteria for probable anti NMDAR antibody encephalitis, admitted in neurology department of a tertiary care centre in Eastern India.

MATERIALS AND METHODS

A prospective, longitudinal study was conducted by identifying 25 patients with anti NMDAR antibodies in CSF and or serum, between September 2018 to February 2020.

STATISTICAL ANALYSIS

Chi square test was used to compare variables.

RESULTS

Out of 98 patients screened, 25 subjects (14 females: 11 male) were positive for anti NMDAR autoantibodies, with a mean age of 17 years. 13 subjects belonged to paediatric age group. Most common presenting feature was memory/learning deficit (88%) followed by behavioural abnormalities (84%) and seizures (68%). 11 patients (44%) patients needed escalation to second line therapy, rituximab. Seven (28%) and twelve (48%) patients underwent complete (mRS 0-1) and partial recovery (mRS 2-3) respectively, while 4 (16%) became disabled (mRS 4-5). Mortality was 8%. Paediatric population had a better outcome in terms of disability (p = 0.043).

CONCLUSION

Anti NMDAR-Ab encephalitis is the most common cause of antibody positive autoimmune encephalitis worldwide. There are important clinical markers and investigational profiles which carry prognostic significance.

摘要

背景

抗N-甲基-D-天冬氨酸受体(抗NMDAR)抗体脑炎是一种以一系列神经精神症状为特征的免疫介导性疾病。

目的

描述抗NMDAR抗体脑炎患者的临床特征及治疗结果。

研究背景与设计

通过筛查印度东部一家三级医疗中心神经内科收治的所有符合Graus标准的可能抗NMDAR抗体脑炎患者来选取研究对象。

材料与方法

对2018年9月至2020年2月期间脑脊液和/或血清中抗NMDAR抗体检测呈阳性的25例患者进行前瞻性纵向研究。

统计分析

采用卡方检验比较变量。

结果

在98例筛查患者中,25例(14例女性,11例男性)抗NMDAR自身抗体呈阳性,平均年龄17岁。13例属于儿童年龄组。最常见的临床表现为记忆/学习缺陷(88%),其次是行为异常(84%)和癫痫发作(68%)。11例患者(44%)需要升级至二线治疗,即使用利妥昔单抗。7例(28%)和12例(48%)患者分别实现完全恢复(改良Rankin量表评分为0 - 1)和部分恢复(改良Rankin量表评分为2 - 3),而4例(16%)患者出现残疾(改良Rankin量表评分为4 - 5)。死亡率为8%。儿童患者在残疾方面预后较好(p = 0.043)。

结论

抗NMDAR抗体脑炎是全球范围内抗体阳性自身免疫性脑炎最常见的病因。存在具有预后意义的重要临床标志物和检查特征。

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