Roy Arun Grace, Vinayan Kollencheri Puthenveetil, Kannoth Sudheeran
Departments of Pediatric Neurology and Neurology, Amrita Institute of Medical Sciences, Amrita Vishwa Vidyapeetham University, Kochi, Kerala, India.
Neurol India. 2020 Nov-Dec;68(6):1374-1377. doi: 10.4103/0028-3886.304087.
Anti-N-methyl-d-aspartate receptor (Anti-NMDAR) Encephalitis classically presents with polysymptomatic presentation of behavioral or cognitive changes, seizures, and focal deficits. Large series in adults and children have described the above features. Monosymptomatic presentation of Anti NMDAR encephalitis is rare and in literature single case reports in adults and children are available. Here we report a series of 6 children presenting with seizure alone and thus expanding the clinical spectrum of Anti NMDAR encephalitis.
This is a a retrospective case series of 6 cases of anti NMDAR encephalitis treated in our institute, which is a tertiary referral center between 2010 and 2014. All the patients with NMDA encephalitis were initially included. The baseline demographics, clinical presentations, investigations (CSF, MRI and EEG), course in the hospital, details of treatment, short and long term outcomes were documented from the available medical records. Children presenting with monosymptomatic seizure clusters were only included in the final analysis.
Twenty eight children were diagnosed with ant NMDA R encephalitis during the study period. 22 children had classical polysymptomatic presentations and were not included in this analysis. The remaining 6 children (5 girls and one boy), who presented with only acute seizure clusters were included in the study. All children presented with acute cluster of focal seizures. Four out of six had focal status epilepticus while 2 out of six had recurrent focal seizures. Commonest semiology was clonic seizure in 4/6 and one child had dystonic seizure and one had tonic seizure. All patients were started on steroids and antiepileptic drugs. No other immunomodulators or immunosuppressants were used. On discharge all patients where seizure free and with no focal deficits.
This is the first series of Anti NMDAR encephalitis presenting as new onset seizure clusters in children. Unlike the existing literature, these children did not develop any other symptoms. We propose that focal encephalitis could be the reason for this monosymptomatic presentation.
抗N-甲基-D-天冬氨酸受体（Anti-NMDAR）脑炎的典型表现为行为或认知改变、癫痫发作和局灶性神经功能缺损等多种症状。针对成人和儿童的大量研究系列均描述过上述特征。抗NMDAR脑炎的单症状表现较为罕见，文献中仅有成人和儿童的单病例报告。在此，我们报告6例仅以癫痫发作为表现的儿童病例，从而拓展了抗NMDAR脑炎的临床谱。
这是一项回顾性病例系列研究，纳入了2010年至2014年期间在我院（一家三级转诊中心）接受治疗的6例抗NMDAR脑炎病例。所有疑似NMDA脑炎的患者均最初纳入。从现有病历中记录了患者的基线人口统计学资料、临床表现、检查（脑脊液、磁共振成像和脑电图）、住院病程、治疗细节以及短期和长期预后。最终分析仅纳入表现为单症状癫痫发作群的儿童。
研究期间，28例儿童被诊断为抗NMDA R脑炎。22例儿童有典型的多症状表现，未纳入本分析。其余6例儿童（5名女孩和1名男孩）仅表现为急性癫痫发作群，被纳入研究。所有儿童均表现为急性局灶性癫痫发作群。6例中有4例出现局灶性癫痫持续状态，6例中有2例出现复发性局灶性癫痫发作。最常见的发作形式是4/6的患者为阵挛性发作，1例儿童为张力障碍性发作，1例为强直性发作。所有患者均开始使用类固醇和抗癫痫药物。未使用其他免疫调节剂或免疫抑制剂。出院时所有患者均无癫痫发作且无局灶性神经功能缺损。
这是首例以新发癫痫发作群表现的儿童抗NMDAR脑炎系列病例。与现有文献不同，这些儿童未出现任何其他症状。我们认为局灶性脑炎可能是这种单症状表现的原因。
