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Autoantibody Encephalitis: Presentation, Diagnosis, and Management.自身免疫性脑炎:临床表现、诊断与治疗
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2
Glutamate receptor antibodies in neurological diseases: anti-AMPA-GluR3 antibodies, anti-NMDA-NR1 antibodies, anti-NMDA-NR2A/B antibodies, anti-mGluR1 antibodies or anti-mGluR5 antibodies are present in subpopulations of patients with either: epilepsy, encephalitis, cerebellar ataxia, systemic lupus erythematosus (SLE) and neuropsychiatric SLE, Sjogren's syndrome, schizophrenia, mania or stroke. These autoimmune anti-glutamate receptor antibodies can bind neurons in few brain regions, activate glutamate receptors, decrease glutamate receptor's expression, impair glutamate-induced signaling and function, activate blood brain barrier endothelial cells, kill neurons, damage the brain, induce behavioral/psychiatric/cognitive abnormalities and ataxia in animal models, and can be removed or silenced in some patients by immunotherapy.神经疾病中的谷氨酸受体抗体:抗AMPA - GluR3抗体、抗NMDA - NR1抗体、抗NMDA - NR2A/B抗体、抗mGluR1抗体或抗mGluR5抗体存在于以下疾病患者的亚组中:癫痫、脑炎、小脑共济失调、系统性红斑狼疮(SLE)和神经精神性SLE、干燥综合征、精神分裂症、躁狂症或中风。这些自身免疫性抗谷氨酸受体抗体可在少数脑区与神经元结合,激活谷氨酸受体,降低谷氨酸受体的表达,损害谷氨酸诱导的信号传导和功能,激活血脑屏障内皮细胞,杀死神经元,损伤大脑,在动物模型中诱发行为/精神/认知异常和共济失调,并且在一些患者中可通过免疫疗法去除或使其失活。
J Neural Transm (Vienna). 2014 Aug;121(8):1029-75. doi: 10.1007/s00702-014-1193-3. Epub 2014 Aug 1.
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The Diagnosis and Treatment of Autoimmune Encephalitis.自身免疫性脑炎的诊断与治疗
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Glutamate Receptor Antibodies in Autoimmune Central Nervous System Disease: Basic Mechanisms, Clinical Features, and Antibody Detection.自身免疫性中枢神经系统疾病中的谷氨酸受体抗体:基本机制、临床特征及抗体检测
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Cureus. 2024 Sep 9;16(9):e69038. doi: 10.7759/cureus.69038. eCollection 2024 Sep.
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VH2+ Antigen-Experienced B Cells in the Cerebrospinal Fluid Are Expanded and Enriched in Pediatric Anti-NMDA Receptor Encephalitis.脑脊液中 VH2+ 抗原经历的 B 细胞在儿科抗 NMDA 受体脑炎中扩增和富集。
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Biomarkers in autoimmune diseases of the central nervous system.中枢神经系统自身免疫性疾病的生物标志物。
Front Immunol. 2023 Apr 5;14:1111719. doi: 10.3389/fimmu.2023.1111719. eCollection 2023.

本文引用的文献

1
Human Leukocyte Antigen Association Study Reveals DRB1*04:02 Effects Additional to DRB1*07:01 in Anti-LGI1 Encephalitis.人类白细胞抗原关联研究揭示抗 LGI1 脑炎中 DRB1*04:02 除 DRB1*07:01 之外的作用
Neurol Neuroimmunol Neuroinflamm. 2022 Feb 3;9(2). doi: 10.1212/NXI.0000000000001140. Print 2022 Mar.
2
IgG4-Mediated Neurologic Autoimmunities: Understanding the Pathogenicity of IgG4, Ineffectiveness of IVIg, and Long-Lasting Benefits of Anti-B Cell Therapies.IgG4 相关的神经免疫性疾病:认识 IgG4 的致病性、IVIg 的无效性和抗 B 细胞治疗的持久获益。
Neurol Neuroimmunol Neuroinflamm. 2021 Nov 29;9(1). doi: 10.1212/NXI.0000000000001116. Print 2022 Jan.
3
LGI1 antibody encephalitis: acute treatment comparisons and outcome.LGI1 抗体脑炎:急性期治疗比较和结局。
J Neurol Neurosurg Psychiatry. 2022 Mar;93(3):309-315. doi: 10.1136/jnnp-2021-327302. Epub 2021 Nov 25.
4
Limitations of a Commercial Assay as Diagnostic Test of Autoimmune Encephalitis.作为自身免疫性脑炎诊断检测方法的商业检测的局限性
Front Immunol. 2021 Jun 29;12:691536. doi: 10.3389/fimmu.2021.691536. eCollection 2021.
5
Encephalitis with Autoantibodies against the Glutamate Kainate Receptors GluK2.伴有抗谷氨酸红藻氨酸受体GluK2自身抗体的脑炎
Ann Neurol. 2021 Jul;90(1):101-117. doi: 10.1002/ana.26098. Epub 2021 May 17.
6
Clinical and Prognostic Value of Immunogenetic Characteristics in Anti-LGI1 Encephalitis.抗 LGI1 脑炎的免疫遗传特征的临床和预后价值。
Neurol Neuroimmunol Neuroinflamm. 2021 Mar 5;8(3). doi: 10.1212/NXI.0000000000000974. Print 2021 May.
7
Efficacy and safety of bortezomib in rituximab-resistant anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis as well as the clinical characteristics: An observational study.硼替佐米治疗利妥昔单抗耐药性抗 N-甲基-D-天冬氨酸受体(抗 NMDAR)脑炎的疗效和安全性以及临床特征:一项观察性研究。
J Neuroimmunol. 2021 May 15;354:577527. doi: 10.1016/j.jneuroim.2021.577527. Epub 2021 Feb 22.
8
Relationship Between Serum NMDA Receptor Antibodies and Response to Antipsychotic Treatment in First-Episode Psychosis.血清 NMDA 受体抗体与首发精神病患者抗精神病治疗反应的关系。
Biol Psychiatry. 2021 Jul 1;90(1):9-15. doi: 10.1016/j.biopsych.2020.11.014. Epub 2020 Nov 24.
9
Influence of methodological and patient factors on serum NMDAR IgG antibody detection in psychotic disorders: a meta-analysis of cross-sectional and case-control studies.方法学和患者因素对精神病性障碍患者血清 NMDAR IgG 抗体检测的影响:横断面和病例对照研究的荟萃分析。
Lancet Psychiatry. 2021 Feb;8(2):109-120. doi: 10.1016/S2215-0366(20)30432-6. Epub 2020 Dec 21.
10
Seizure-related 6 homolog like 2 autoimmunity: Neurologic syndrome and antibody effects.与癫痫相关的 6 同源物样 2 自身免疫:神经综合征和抗体作用。
Neurol Neuroimmunol Neuroinflamm. 2020 Nov 3;8(1). doi: 10.1212/NXI.0000000000000916. Print 2021 Jan.

自身免疫性脑炎:临床表现、诊断与治疗

Autoantibody Encephalitis: Presentation, Diagnosis, and Management.

作者信息

Lancaster Eric

机构信息

Department of Neurology, The University of Pennsylvania, Philadelphia, PA, USA.

出版信息

J Clin Neurol. 2022 Jul;18(4):373-390. doi: 10.3988/jcn.2022.18.4.373.

DOI:10.3988/jcn.2022.18.4.373
PMID:35796263
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9262450/
Abstract

Autoantibody encephalitis causes distinct clinical syndromes involving alterations in mentation, abnormal movements, seizures, psychiatric symptoms, sleep disruption, spasms, and neuromyotonia. The diagnoses can be confirmed by specific antibody tests, although some antibodies may be better detected in spinal fluid and others in serum. Each disorder conveys a risk of certain tumors which may inform diagnosis and be important for treatment. Autoantibodies to receptors and other neuronal membrane proteins are generally thought to be pathogenic and result in loss of function of the targets, so understanding the pharmacology of the receptors may inform our understanding of the syndromes. Patients may be profoundly ill but the syndromes usually respond to immune therapy, although there are differences in the types of immune therapy that are thought to be most effective for the various disorders.

摘要

自身抗体性脑炎可引发独特的临床综合征,包括精神状态改变、异常运动、癫痫发作、精神症状、睡眠障碍、痉挛和神经性肌强直。尽管有些抗体在脑脊液中检测效果更佳,而另一些在血清中检测效果更佳,但通过特定抗体检测可确诊。每种疾病都伴有某些肿瘤的风险,这可能有助于诊断且对治疗很重要。针对受体和其他神经元膜蛋白的自身抗体通常被认为具有致病性,并导致靶标功能丧失,因此了解受体的药理学特性可能有助于我们理解这些综合征。患者可能病情严重,但这些综合征通常对免疫治疗有反应,尽管对于各种疾病,人们认为最有效的免疫治疗类型存在差异。