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抗 IgLON5 脑炎以癫痫发作为首发症状的罕见儿科病例。

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

机构信息

Department of Pediatric Neurology, The Affiliated Hospital of Qingdao University, Qingdao, China.

Department of Anesthesiology, The Affiliated Hospital of Qingdao University, Qingdao, China.

出版信息

Int J Dev Neurosci. 2024 Nov;84(7):791-796. doi: 10.1002/jdn.10364. Epub 2024 Jul 14.

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.

摘要

背景

抗 IgLON5 脑炎是一种罕见的神经和异质性疾病,主要发生在成年人中。与抗 IgLON5 病相关的癫痫发作很少有报道。

方法

使用免疫荧光双重染色的细胞基础检测法(CBA)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。通过大鼠脑组织和肾组织的组织基础检测法(TBA)进一步确认血清中的抗体。

结果

我们报告了一例儿科病例,表现为癫痫发作、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。静脉注射免疫球蛋白和大剂量静脉注射甲基强的松龙治疗后,她的病情迅速改善。

结论

我们描述了第二例儿科抗 IgLON5 脑炎病例,也是首例以癫痫发作为首发表现的病例。抗 IgLON5 脑炎可能表现较轻。对于新发癫痫发作伴有认知障碍和睡眠障碍的患者,即使是儿童,也应尽早检测抗 IgLON5 抗体。

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