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抗γ-氨基丁酸B受体脑炎的研究进展及1例副肿瘤性相关脑炎病例报告与治疗分析

Research progress of anti-γ-aminobutyric acid B receptor encephalitis and a case report of paraneoplastic associated encephalitis and treatment analysis.

作者信息

Lv Yi-Kun, Zhang Hai-Qing, Zhang Jun

机构信息

Department of Neurology Affiliated Hospital of Zunyi Medical University Zunyi China.

出版信息

Ibrain. 2022 Feb 10;8(1):15-22. doi: 10.1002/ibra.12017. eCollection 2022 Spring.

Abstract

Encephalitis is one of the common diseases in neurology. Early diagnosis and appropriate treatments are essential. Autoimmune encephalitis (AE) generally refers to a type of encephalitis mediated by autoimmune mechanisms. It is gradually considered to be an important cause of reversible encephalitis caused by noninfectious factors. It can occur in children, adolescents, and adults, and is clinically characterized by multifocal or diffuse brain damage such as personality changes, seizures, and cognitive impairment, with an overall good effect of immunotherapy. According to the clinical features of the patients, blood and cerebrospinal fluid tests, neuroelectrophysiology, cranial imaging, treatment and prognosis, AEs can be broadly divided into specific antigen (antibody)-related AEs and nonspecific antigen (or antibody) -related AEs. With the development of AEs research, more and more anti-neuron antibodies have been found, which provides an important reference for the diagnosis and treatment of AEs. Understanding the knowledge about AEs is important to discover new diseases and deepen the understanding of the immunopathological mechanisms of existing central nervous system diseases. Anti-γ-aminobutyric acid B (GABA-B) receptor encephalitis is a type of AE, but this disease is rare in AE, often develop to the clinical manifestations of marginal encephalitis, accompanied by obvious seizures or status epilepticus, Some patients had tumors, mainly small-cell carcinoma, prompt diagnosis, early immunotherapy and, if necessary, tumor treatment resulted in complete or partial neurological improvement in most patients.

摘要

脑炎是神经内科常见疾病之一。早期诊断和恰当治疗至关重要。自身免疫性脑炎(AE)通常指一类由自身免疫机制介导的脑炎。它逐渐被认为是由非感染因素导致的可逆性脑炎的重要病因。可发生于儿童、青少年及成人,临床特征为多灶性或弥漫性脑损害,如人格改变、癫痫发作及认知障碍,免疫治疗总体效果良好。根据患者的临床特征、血液及脑脊液检查、神经电生理学、头颅影像学、治疗及预后情况,AE可大致分为特异性抗原(抗体)相关AE和非特异性抗原(或抗体)相关AE。随着AE研究的发展,越来越多的抗神经元抗体被发现,这为AE的诊断和治疗提供了重要参考。了解有关AE的知识对于发现新疾病以及加深对现有中枢神经系统疾病免疫病理机制的认识很重要。抗γ-氨基丁酸B(GABA-B)受体脑炎是AE的一种类型,但该疾病在AE中较为罕见,常发展为边缘性脑炎的临床表现,伴有明显的癫痫发作或癫痫持续状态,部分患者有肿瘤,主要为小细胞癌,及时诊断、早期免疫治疗,必要时进行肿瘤治疗,多数患者可实现完全或部分神经功能改善。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c98b/10528969/02e890bfab5e/IBRA-8-15-g002.jpg

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