Wright Sukhvir, Hacohen Yael, Jacobson Leslie, Agrawal Shakti, Gupta Rajat, Philip Sunny, Smith Martin, Lim Ming, Wassmer Evangeline, Vincent Angela
Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK Department of Paediatric Neurology, Birmingham Children's Hospital, Birmingham, UK.
Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK.
Arch Dis Child. 2015 Jun;100(6):521-6. doi: 10.1136/archdischild-2014-306795. Epub 2015 Jan 30.
N-methyl-D-aspartate receptor antibody (NMDAR-Ab) encephalitis is a well-recognised clinico-immunological syndrome that presents with neuropsychiatric symptoms cognitive decline, movement disorder and seizures. This study reports the clinical features, management and neurological outcomes of paediatric NMDAR-Ab-mediated neurological disease in the UK.
A prospective surveillance study. Children with NMDAR-Ab-mediated neurological diseases were voluntarily reported to the British Neurological Surveillance Unit (BPNSU) from November 2010 to December 2011. Initial and follow-up questionnaires were sent out to physicians.
Thirty-one children fulfilled the criteria for the study. Eight presented during the study period giving an incidence of 0.85 per million children per year (95% CI 0.64 to 1.06); 23 cases were historical. Behavioural change and neuropsychiatric features were present in 90% of patients, and seizures and movement disorders both in 67%. Typical NMDAR-Ab encephalitis was reported in 24 children and partial phenotype without encephalopathy in seven, including predominantly psychiatric (four) and movement disorder (three). All patients received steroids, 22 (71%) received intravenous immunoglobulin, 9 (29%) received plasma exchange,and 10 (32%) received second-line immunotherapy. Of the 23 patients who were diagnosed early, 18 (78%) made a full recovery compared with only 1 of 8 (13%) of the late diagnosed patients (p=0.002, Fisher's exact test). Seven patients relapsed, with four needing additional second-line immunotherapy.
Paediatric NMDAR-Ab-mediated neurological disease appears to be similar to adult NMDAR-Ab encephalitis, but some presented with a partial phenotype. Early treatment was associated with a quick and often full recovery.
N-甲基-D-天冬氨酸受体抗体（NMDAR-Ab）脑炎是一种公认的临床免疫综合征，表现为神经精神症状、认知功能下降、运动障碍和癫痫发作。本研究报告了英国儿童NMDAR-Ab介导的神经系统疾病的临床特征、治疗及神经学转归。
一项前瞻性监测研究。2010年11月至2011年12月期间，NMDAR-Ab介导的神经系统疾病患儿自愿向英国神经监测单位（BNSU）报告。向医生发放初始问卷和随访问卷。
31名儿童符合研究标准。8名在研究期间发病，年发病率为每百万儿童0.85例（95%CI 0.64至1.06）；23例为既往病例。90%的患者出现行为改变和神经精神症状，67%的患者出现癫痫发作和运动障碍。24名儿童报告为典型的NMDAR-Ab脑炎，7名儿童表现为无脑病的部分表型，包括以精神症状为主（4例）和运动障碍为主（3例）。所有患者均接受了类固醇治疗，22例（71%）接受了静脉注射免疫球蛋白，9例（29%）接受了血浆置换，10例（32%）接受了二线免疫治疗。在23例早期诊断的患者中，18例（78%）完全康复，而在8例晚期诊断的患者中只有1例（13%）完全康复（p=0.002，Fisher精确检验）。7例患者复发，4例需要额外的二线免疫治疗。
儿童NMDAR-Ab介导的神经系统疾病似乎与成人NMDAR-Ab脑炎相似，但有些表现为部分表型。早期治疗与快速且常为完全康复相关。
