Department of Pediatric Neurology, The Affiliated Hospital of Qingdao University, Qingdao, China.
Department of Anesthesiology, The Affiliated Hospital of Qingdao University, Qingdao, China.
Int J Dev Neurosci. 2024 Nov;84(7):791-796. doi: 10.1002/jdn.10364. Epub 2024 Jul 14.
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.
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.
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.
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.
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。
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。
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。
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。
