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病例报告:小儿抗γ-氨基丁酸B受体脑炎,预后良好。

Case report: Pediatric anti-gamma aminobutyric acid-B receptor encephalitis with benign prognosis.

作者信息

Wang Yeping, Ren Xiaoyan, Shen Yu, Hua Yi, Xu Lu, Zhang Weiran, Sheng Guoxia, Jiang Peifang, Yuan Zhefeng, Liu Liu, Gao Feng

机构信息

Department of Neurology, Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China.

Department of Pediatric, Jinhua Maternity and Child Health Care Hospital, Jinhua, China.

出版信息

Front Pediatr. 2023 Mar 3;11:1104001. doi: 10.3389/fped.2023.1104001. eCollection 2023.

Abstract

OBJECTIVE

To explore the clinical characteristics of pediatric anti-gamma-aminobutyric acid-B receptor (GABAR) encephalitis to enhance the understanding and improve the diagnostic and therapeutic strategies for this disease.

METHODS

We report a rare case of a female pediatric patient with anti-GABAR encephalitis who was treated at the Children's Hospital of Zhejiang University School of Medicine. Literature search was performed to explore the clinical characteristics of pediatric anti-GABAR encephalitis.

RESULTS

The patient exhibited recurrent epileptic seizure, status epilepticus, and psychiatric symptoms at the age of 11 years and 10 months. Anti-GABAR antibodies were positive in cerebrospinal fluid and serum. Brain magnetic resonance imaging (MRI) exhibited abnormal signals in the left hippocampus. Symptoms and abnormality of brain MRI were improved after administration of immunosuppressants, anti-seizure and antipsychotic drugs. Two of pediatric anti-GABAR encephalitis with clinical data were identified through literature search. Analysis of these three cases suggested that the pediatric patients primarily experienced limbic encephalitis, with no tumor incidence. A favorable immunotherapy response was demonstrated with a superior prognosis in all the cases.

CONCLUSIONS

We reported a pediatric anti-GABAR encephalitis case with early age of onset. Promt autoimmune antibody testing and tumor screening, as well as immunomodulatory treatment immediately after a definitive diagnosis are warranted to improve prognosis.

摘要

目的

探讨小儿抗γ-氨基丁酸B受体(GABAR)脑炎的临床特征,以提高对该病的认识并改进其诊断和治疗策略。

方法

我们报告了1例在浙江大学医学院附属儿童医院接受治疗的患抗GABAR脑炎的罕见小儿女性患者。进行文献检索以探究小儿抗GABAR脑炎的临床特征。

结果

该患者在11岁10个月时出现反复癫痫发作、癫痫持续状态及精神症状。脑脊液和血清中抗GABAR抗体呈阳性。脑磁共振成像(MRI)显示左侧海马区有异常信号。给予免疫抑制剂、抗癫痫药和抗精神病药物后,症状及脑MRI异常得到改善。通过文献检索确定了另外2例有临床资料的小儿抗GABAR脑炎病例。对这3例病例的分析表明,小儿患者主要表现为边缘叶脑炎,无肿瘤发生。所有病例均显示出良好的免疫治疗反应及较好的预后。

结论

我们报告了1例发病年龄较早的小儿抗GABAR脑炎病例。应及时进行自身免疫抗体检测和肿瘤筛查,并在明确诊断后立即进行免疫调节治疗,以改善预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6b5d/10020610/36eb3f2a8ec9/fped-11-1104001-g001.jpg

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