A rare pediatric patient of anti-IgLON5 encephalitis with epileptic seizures as the first symptom

IF 1.7 4区医学 Q3 DEVELOPMENTAL BIOLOGY

International Journal of Developmental Neuroscience Pub Date : 2024-07-14 DOI:10.1002/jdn.10364

Jiao Xue, Zhenfeng Song, Hongshan Zhao, Zhi Yi, Fei Li, Chengqing Yang, Kaixuan Liu, Ying Zhang

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引用次数: 0

Abstract

Background

Anti-IgLON5 encephalitis was a rare neurological and heterogeneous disorder, which was mainly found in adults. Epileptic seizures related to anti-IgLON5 disease were rarely reported.

Methods

Neural antibodies associated with autoimmune encephalitis in serum and cerebrospinal fluid (CSF) were tested using cell-based assays (CBA) with immunofluorescence double staining. The antibodies in serum were further confirmed by tissue-based assay (TBA) with rat brain and kidney tissue.

Results

We reported a pediatric case presented with epileptic seizures, cognitive impairments, and sleep disorders. Autoantibody screening showed anti-IgLON5 antibody IgG (1:100+) and anti-NMDAR antibody IgG (1:10+) in the serum. She was diagnosed as anti-IgLON5 encephalitis. Her conditions improved rapidly by treated with intravenous immunoglobulin and high dose intravenous methylprednisolone.

Conclusion

We described the second pediatric case with anti-IgLON5 encephalitis, who was also the first presented with epileptic seizures as the initial presentation. Anti-IgLON5 encephalitis might have mild manifestations. For patients with new onset seizures associated with cognitive impairments and sleep disturbances, anti-IgLON5 antibody should be tested as early, even in children.

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一名罕见的以癫痫发作为首发症状的抗 IgLON5脑炎儿童患者。

背景：抗IgLON5脑炎是一种罕见的神经系统异质性疾病，主要见于成人。与抗IgLON5疾病相关的癫痫发作鲜有报道：方法：使用细胞检测法（CBA）和免疫荧光双重染色法检测血清和脑脊液（CSF）中与自身免疫性脑炎相关的神经抗体。结果：我们报告了一个小儿病例，该病例伴有癫痫发作、认知障碍和睡眠障碍。自身抗体筛查显示血清中存在抗 IgLON5 抗体 IgG（1:100+）和抗 NMDAR 抗体 IgG（1:10+）。她被诊断为抗IgLON5脑炎。经静脉注射免疫球蛋白和大剂量甲基强的松龙治疗后，她的病情迅速好转：我们描述了第二例抗IgLON5脑炎患儿，她也是第一例以癫痫发作为首发症状的患儿。抗 IgLON5 脑炎可能表现轻微。对于伴有认知障碍和睡眠障碍的新发癫痫患者，即使是儿童，也应尽早检测抗 IgLON5 抗体。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

International Journal of Developmental Neuroscience 医学-发育生物学

CiteScore

3.30

自引率

5.60%

发文量

审稿时长

6-12 weeks

期刊介绍： International Journal of Developmental Neuroscience publishes original research articles and critical review papers on all fundamental and clinical aspects of nervous system development, renewal and regeneration, as well as on the effects of genetic and environmental perturbations of brain development and homeostasis leading to neurodevelopmental disorders and neurological conditions. Studies describing the involvement of stem cells in nervous system maintenance and disease (including brain tumours), stem cell-based approaches for the investigation of neurodegenerative diseases, roles of neuroinflammation in development and disease, and neuroevolution are also encouraged. Investigations using molecular, cellular, physiological, genetic and epigenetic approaches in model systems ranging from simple invertebrates to human iPSC-based 2D and 3D models are encouraged, as are studies using experimental models that provide behavioural or evolutionary insights. The journal also publishes Special Issues dealing with topics at the cutting edge of research edited by Guest Editors appointed by the Editor in Chief. A major aim of the journal is to facilitate the transfer of fundamental studies of nervous system development, maintenance, and disease to clinical applications. The journal thus intends to disseminate valuable information for both biologists and physicians. International Journal of Developmental Neuroscience is owned and supported by The International Society for Developmental Neuroscience (ISDN), an organization of scientists interested in advancing developmental neuroscience research in the broadest sense.