Pediatric Autoimmune encephalitis: Experience from a Tertiary Care Hospital in Bangladesh

K. Fatema, Md Mizanur Rahman
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Abstract

Background: Autoimmune encephalitis (AIE) are distinct group of encephalitis where production of autoimmune antibody causes neuroinflammation. The core clinical features are encephalopathy, psychiatric disorder, movement disorder and seizure. The investigation and treatment modalities are different from that of infectious encephalitis. There are limited studies in pediatric population in particularly in developing country like Bangladesh. Thus this study has been done to describe patients with AIE from a tertiary care hospital. Method: This is a retrospective study done in children of 1-16 year from January 2018 to December 2019. AIE was diagnosed on the basis of clinical, electrographic and neuroimaging features and was confirmed with detection of autoantibody in CSF. Treatment was given according to the published literature with immunotherapy mainly. Results: Total 15 children were studied, 14 patients were antiNMDAR encephalitis and 1 was antiMOG antibody syndrome. Mean age was 5.98 and 4.5 year respectively. Seizure was the most common clinical feature, mostly focal in nature. Other manifestations were movement disorder, psychiatric disorder, loss of consciousness etc. Most of the patients had abnormal EE, focal epileptic discharge being the commonest. Eight out of 15 had abnormal MRI of brain. Cortical  hyperintensity was important feature located mostly in temporal region. In the case of antiMOG antibody syndrome there was demyelinating lesion in multiple areas. Cornerstone of the treatment was mostly combination immunotherapy with IV methylprednisolone and IV immunoglobulin followed by oral steroid. Majority of the patients showed improvement however 3 patients had complete recovery. Complications observed were epilepsy, speech disorder, cognitive disorder, behavioural disorder, ataxia and visual impairment. Conclusion:  Timely diagnosis and prompt treatment of AIE is very important as proper treatment can cause significant improvement.  
小儿自身免疫性脑炎:来自孟加拉国三级保健医院的经验
背景:自身免疫性脑炎(AIE)是一种独特的脑炎,其自身免疫抗体的产生引起神经炎症。核心临床特征为脑病、精神障碍、运动障碍和癫痫发作。该病的调查和治疗方式与传染性脑炎不同。对儿童人口的研究有限,特别是在孟加拉国这样的发展中国家。因此,本研究对三级医院的AIE患者进行了描述。方法:对2018年1月至2019年12月1-16岁儿童进行回顾性研究。AIE的诊断依据临床、电图及神经影像学特征,经脑脊液自身抗体检测证实。参照已发表文献给予治疗,以免疫治疗为主。结果:共15例患儿,抗inmdar脑炎14例,抗mog抗体综合征1例。平均年龄分别为5.98岁和4.5岁。癫痫是最常见的临床特征,主要是局灶性的。其他表现为运动障碍、精神障碍、意识丧失等。大多数患者有异常的情绪表达,局灶性癫痫放电是最常见的。15人中有8人的脑部MRI异常。皮层高信号是主要发生在颞区的重要特征。抗mog抗体综合征患者多部位出现脱髓鞘病变。治疗的基础主要是免疫治疗联合静脉注射甲基强的松龙和静脉注射免疫球蛋白,然后口服类固醇。多数患者症状有所改善,3例患者完全康复。观察到的并发症有癫痫、语言障碍、认知障碍、行为障碍、共济失调和视力障碍。结论:及时诊断和治疗AIE非常重要,治疗得当可使病情明显好转。
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