Feng Mei, Zhou Zhen, Kang Qingyun, Wang Miao, Tang Jingwen, Wu Liwen
Department of Neurology, The Affiliated Children's Hospital of Xiangya School of Medicine, Central South University (Hunan children's hospital), Changsha, China.
Front Neurol. 2024 May 3;15:1388970. doi: 10.3389/fneur.2024.1388970. eCollection 2024.
Anti-IgLON5 antibody-related encephalitis is a rare autoimmune disorder of the central nervous system, predominantly occurring in middle-aged elderly individuals, with paediatric cases being exceptionally rare. This study aims to enhance the understanding of paediatric anti-IgLON5 antibody-related encephalitis by summarising its clinical and therapeutic characteristics.
A retrospective analysis was conducted on two paediatric patients diagnosed with anti-IgLON5 antibody-related encephalitis at Hunan Children's Hospital from August 2022 to November 2023. This involved reviewing their medical records and follow-up data, in addition to a literature review.
The study involved two patients, one male and one female, aged between 2.5 and 9.6 years, both presenting with an acute/subacute course of illness. Clinically, both exhibited movement disorders (including dystonia, involuntary movements, and ataxia), cognitive impairments, sleep disturbances, and psychiatric symptoms. Patient 1 experienced epileptic seizures, while Patient 2 exhibited brainstem symptoms and abnormal eye movements. Neither patient showed autonomic dysfunction. Patient 1 had normal cerebrospinal fluid (CSF) and Brain MRI findings, whereas Patient 2 showed moderate leukocytosis and mild protein elevation in the CSF, and Brain MRI revealed symmetrical lesions in the basal ganglia and cerebellum. Oligoclonal bands in the CSF were positive in both cases. Both patients tested negative for HLA-DQB05:01 and HLA-DRB10:01. They received both first-line and second-line immunotherapies, with Patient 2 showing a poor response to treatment.
Paediatric cases of anti-IgLON5 antibody-related encephalitis similarly present sleep disturbances as a core symptom, alongside various forms of movement disorders. Immunotherapy is partially effective. Compared to adult patients, these paediatric cases tend to exhibit more pronounced psychiatric symptoms, a more rapid onset, and more evident inflammatory changes in the CSF. The condition appears to have a limited association with HLA-DQB05:01 and HLA-DRB10:01 polymorphisms.
抗IgLON5抗体相关脑炎是一种罕见的中枢神经系统自身免疫性疾病，主要发生在中老年个体中，儿科病例极为罕见。本研究旨在通过总结小儿抗IgLON5抗体相关脑炎的临床和治疗特征，加深对其的认识。
对2022年8月至2023年11月在湖南省儿童医院确诊为抗IgLON5抗体相关脑炎的两名儿科患者进行回顾性分析。这包括查阅他们的病历和随访数据，以及进行文献回顾。
该研究涉及两名患者，一名男性和一名女性，年龄在2.5至9.6岁之间，均表现为急性/亚急性病程。临床上，两人均出现运动障碍（包括肌张力障碍、不自主运动和共济失调）、认知障碍、睡眠障碍和精神症状。患者1出现癫痫发作，而患者2表现出脑干症状和眼球运动异常。两名患者均未出现自主神经功能障碍。患者1的脑脊液（CSF）和脑部MRI检查结果正常，而患者2的脑脊液显示中度白细胞增多和轻度蛋白升高，脑部MRI显示基底节和小脑有对称性病变。两例患者脑脊液中的寡克隆带均为阳性。两名患者的HLA-DQB05:01和HLA-DRB10:01检测均为阴性。他们接受了一线和二线免疫治疗，患者2对治疗反应不佳。
小儿抗IgLON5抗体相关脑炎同样以睡眠障碍为核心症状，同时伴有各种形式的运动障碍。免疫治疗部分有效。与成年患者相比，这些儿科病例往往表现出更明显的精神症状、起病更迅速，脑脊液中的炎症变化更明显。该疾病似乎与HLA-DQB05:01和HLA-DRB10:01多态性的关联有限。
