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Spectrum of anti-NMDA receptor antibody encephalitis: Clinical profile, management and outcomes
Annals of Indian Academy of Neurology ( IF 1.7 ) Pub Date : 2021-05-01 , DOI: 10.4103/aian.aian_817_20 Amlan Kusum Datta 1 , Alak Pandit 1 , Samar Biswas 1 , Atanu Biswas 1 , Biman Kanti Roy 1 , Goutam Gangopaddhyay 1
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 et al.'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.
中文翻译：
抗 NMDA 受体抗体脑炎谱：临床概况、管理和结果
背景：抗 N-甲基 D-天冬氨酸受体（抗 NMDAR）抗体脑炎是一种免疫介导的实体，其特征是一系列神经精神症状。目的：描述抗 NMDAR 抗体脑炎患者的临床特征和治疗结果。设置和设计：通过筛选所有满足 Graus等人的患者来选择受试者。的可能抗 NMDAR 抗体脑炎的标准，在印度东部的一家三级护理中心的神经内科住院。材料和方法：在 2018 年 9 月至 2020 年 2 月期间，通过确定 25 名在 CSF 和/或血清中具有抗 NMDAR 抗体的患者进行了一项前瞻性纵向研究。统计分析：卡方检验用于比较变量。结果：在筛选的 98 名患者中，25 名受试者（14 名女性：11 名男性）抗 NMDAR 自身抗体呈阳性，平均年龄为 17 岁。13名受试者属于儿科年龄组。最常见的表现特征是记忆/学习缺陷 (88%)，其次是行为异常 (84%) 和癫痫发作 (68%)。11 名 (44%) 患者需要升级至二线治疗利妥昔单抗。7 名 (28%) 和 12 名 (48%) 患者分别接受了完全 (mRS 0-1) 和部分恢复 (mRS 2-3)，而 4 (16%) 名患者被禁用 (mRS 4-5)。死亡率为8%。儿科人群在残疾方面有更好的结果（p = 0.043）。结论：抗 NMDAR-Ab 脑炎是全球抗体阳性自身免疫性脑炎的最常见原因。有重要的临床标志物和研究概况具有预后意义。
更新日期：2021-05-01
Annals of Indian Academy of Neurology ( IF 1.7 ) Pub Date : 2021-05-01 , DOI: 10.4103/aian.aian_817_20 Amlan Kusum Datta 1 , Alak Pandit 1 , Samar Biswas 1 , Atanu Biswas 1 , Biman Kanti Roy 1 , Goutam Gangopaddhyay 1
Affiliation
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 et al.'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.
中文翻译：
抗 NMDA 受体抗体脑炎谱：临床概况、管理和结果
背景：抗 N-甲基 D-天冬氨酸受体（抗 NMDAR）抗体脑炎是一种免疫介导的实体，其特征是一系列神经精神症状。目的：描述抗 NMDAR 抗体脑炎患者的临床特征和治疗结果。设置和设计：通过筛选所有满足 Graus等人的患者来选择受试者。的可能抗 NMDAR 抗体脑炎的标准，在印度东部的一家三级护理中心的神经内科住院。材料和方法：在 2018 年 9 月至 2020 年 2 月期间，通过确定 25 名在 CSF 和/或血清中具有抗 NMDAR 抗体的患者进行了一项前瞻性纵向研究。统计分析：卡方检验用于比较变量。结果：在筛选的 98 名患者中，25 名受试者（14 名女性：11 名男性）抗 NMDAR 自身抗体呈阳性，平均年龄为 17 岁。13名受试者属于儿科年龄组。最常见的表现特征是记忆/学习缺陷 (88%)，其次是行为异常 (84%) 和癫痫发作 (68%)。11 名 (44%) 患者需要升级至二线治疗利妥昔单抗。7 名 (28%) 和 12 名 (48%) 患者分别接受了完全 (mRS 0-1) 和部分恢复 (mRS 2-3)，而 4 (16%) 名患者被禁用 (mRS 4-5)。死亡率为8%。儿科人群在残疾方面有更好的结果（p = 0.043）。结论：抗 NMDAR-Ab 脑炎是全球抗体阳性自身免疫性脑炎的最常见原因。有重要的临床标志物和研究概况具有预后意义。
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