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自身免疫性脑炎——一种多方面的病理学表现

Autoimmune Encephalitis-A Multifaceted Pathology.

作者信息

Neațu Monica, Jugurt Ana, Covaliu Anca, Davidescu Eugenia Irene, Popescu Bogdan Ovidiu

机构信息

Department of Clinical Neurosciences, "Carol Davila" University of Medicine and Pharmacy, 050474 Bucharest, Romania.

Department of Neurology, Colentina Clinical Hospital, 020125 Bucharest, Romania.

出版信息

Biomedicines. 2023 Aug 2;11(8):2176. doi: 10.3390/biomedicines11082176.

Abstract

Autoimmune encephalitis is a complex and multifaceted pathology that involves immune-mediated inflammation of the brain. It is characterized by the body's immune system attacking the brain tissue, leading to a cascade of inflammatory processes. What makes autoimmune encephalitis vast is the wide range of causes, mechanisms, clinical presentations, and diagnostic challenges associated with the condition. The clinical presentations of autoimmune encephalitis are broad and can mimic other neurological disorders, making it a challenging differential diagnosis. This diverse clinical presentation can overlap with other conditions, making it crucial for healthcare professionals to maintain a high level of suspicion for autoimmune encephalitis when evaluating patients. The diagnostic challenges associated with autoimmune encephalitis further contribute to its vastness. Due to the variable nature of the condition, there is no definitive diagnostic test that can confirm autoimmune encephalitis in all cases. In this context, personalized patient management is crucial for achieving favorable outcomes. Each patient's treatment plan should be tailored to their specific clinical presentation, underlying cause, and immune response. Our objective is to raise awareness about the frequent yet underdiagnosed nature of autoimmune encephalitis by sharing five cases we encountered, along with a brief literature review.

摘要

自身免疫性脑炎是一种复杂且多方面的病理状况,涉及免疫介导的脑部炎症。其特征是人体免疫系统攻击脑组织,引发一系列炎症过程。自身免疫性脑炎情况复杂的原因在于与该病症相关的病因、机制、临床表现及诊断挑战范围广泛。自身免疫性脑炎的临床表现多种多样,可模仿其他神经系统疾病,这使其成为具有挑战性的鉴别诊断难题。这种多样的临床表现可能与其他病症重叠,因此医疗专业人员在评估患者时对自身免疫性脑炎保持高度怀疑至关重要。与自身免疫性脑炎相关的诊断挑战进一步加剧了其复杂性。由于该病症的多变性,没有一种确定性的诊断测试能在所有病例中确诊自身免疫性脑炎。在此背景下,个性化的患者管理对于取得良好治疗效果至关重要。每个患者的治疗方案应根据其特定的临床表现、潜在病因和免疫反应进行定制。我们的目的是通过分享我们遇到的五例病例以及简要的文献综述,提高对自身免疫性脑炎常见但诊断不足这一情况的认识。

相似文献

2
[Autoimmune encephalitis. A review].[自身免疫性脑炎。综述]
Rev Med Chil. 2018 Mar;146(3):351-361. doi: 10.4067/s0034-98872018000300351.
7
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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Long-Term Memory Dysfunction in Limbic Encephalitis.边缘叶性脑炎的长期记忆功能障碍
Front Neurol. 2019 Apr 26;10:330. doi: 10.3389/fneur.2019.00330. eCollection 2019.
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Acute encephalitis in immunocompetent adults.免疫功能正常成年人的急性脑炎。
Lancet. 2019 Feb 16;393(10172):702-716. doi: 10.1016/S0140-6736(18)32526-1. Epub 2019 Feb 14.
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Sleep disturbances are common in patients with autoimmune encephalitis.睡眠障碍在自身免疫性脑炎患者中很常见。
J Neurol. 2019 Apr;266(4):1007-1015. doi: 10.1007/s00415-019-09230-2. Epub 2019 Feb 11.
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Molecular mimicry and autoimmunity.分子模拟与自身免疫。
J Autoimmun. 2018 Dec;95:100-123. doi: 10.1016/j.jaut.2018.10.012. Epub 2018 Oct 26.

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