Chen Shimeng, Kessi Miriam, He Fang, Yin Fei, Peng Jing, Yang Lifen
Department of Pediatrics, Xiangya Hospital, Central South University, Changsha, People's Republic of China.
Hunan Intellectual and Developmental Disabilities Research Center, Pediatrics, Changsha, People's Republic of China.
Immunotargets Ther. 2024 Dec 17;13:805-811. doi: 10.2147/ITT.S498345. eCollection 2024.
Anti-glutamate kainate receptor subunit 2 (anti-GluK2) antibodies mediated encephalitis is very rare in both children and adults. This study aimed to describe the second report of the anti-GluK2 encephalitis worldwide, the first youngest patient worldwide, and the first case ever in Asia. Besides, this study provides a summary of the clinical manifestations of all previous reported cases.
The patient was attended at the Department of Pediatrics, Xiangya Hospital, Central South University. Anti-GluK2 antibodies were tested in the serum and cerebrospinal fluid (CSF) by the indirect immunofluorescence on cell-based assays. The clinical information of the patient was collected. In addition, a literature search was carried out in the PubMed.
Our patient was a male who presented with lethargy, recurrent dizziness and vomiting, headache and cerebellar ataxia at the age of 13 years. Prodromal illnesses included Herpes Zoster infection and Mycoplasma pneumonia. The anti-GluK2 antibodies and elevated IL-6 levels were detected in serum while high oligoclonal bands levels were found in the CSF. The intravenous methylprednisolone, immunoglobulin, antibiotics and other symptomatic treatments helped the patient to recover full. We could only find one previous report in the literature (from Barcelona). The literature review plus our report unveiled eight patients with pure anti-GluK2 antibodies related encephalitis. The median age of onset was 28.50 years and majority were males (75.00%). Most of the cases (87.50%) presented with acute cerebellitis symptoms and signs. Preceding or concurrent infection was observed in two patients, while paraneoplastic tumors were observed in two patients. Patients had non-parenchymal brain lesions; the commonest anomalies were those localized in the cerebellum (62.50%).
Our report provides more evidence that anti-GluK2 antibodies may be pathogenic for the autoimmune encephalitis (cerebellitis). Immunotherapy can be used to treat it with good outcome.
抗谷氨酸盐红藻氨酸受体亚基2（抗GluK2）抗体介导的脑炎在儿童和成人中都非常罕见。本研究旨在描述全球第二例抗GluK2脑炎报告、全球首例最年轻患者以及亚洲首例病例。此外，本研究还总结了所有先前报道病例的临床表现。
该患者在中南大学湘雅医院儿科就诊。通过基于细胞的间接免疫荧光法检测血清和脑脊液（CSF）中的抗GluK2抗体。收集患者的临床信息。此外，还在PubMed上进行了文献检索。
我们的患者为男性，13岁时出现嗜睡、反复头晕和呕吐、头痛及小脑共济失调。前驱疾病包括带状疱疹感染和支原体肺炎。血清中检测到抗GluK2抗体和升高的白细胞介素-6水平，而脑脊液中发现高寡克隆带水平。静脉注射甲基强的松龙、免疫球蛋白、抗生素及其他对症治疗帮助患者完全康复。我们在文献中仅找到一篇先前的报告（来自巴塞罗那）。文献综述加上我们的报告共揭示了8例与纯抗GluK2抗体相关的脑炎患者。发病年龄中位数为28.50岁，大多数为男性（75.00%）。大多数病例（87.50%）表现为急性小脑炎症状和体征。两名患者观察到前驱或并发感染，两名患者观察到副肿瘤性肿瘤。患者有非实质性脑病变；最常见的异常位于小脑（62.50%）。
我们的报告提供了更多证据表明抗GluK2抗体可能是自身免疫性脑炎（小脑炎）的致病因素。免疫疗法可用于治疗，效果良好。
