抗腺苷酸激酶5脑炎:这种罕见疾病的临床特征、诊断和治疗

IF 4.7 Q2 IMMUNOLOGY
Er-Chuang Li , Qi-Lun Lai , Meng-Ting Cai , Gao-Li Fang , Chun-Hong Shen , Mei-Ping Ding , Yin-Xi Zhang
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引用次数: 0

摘要

在过去的几十年里,抗体阳性自身免疫性脑炎(AE)的范围和理解已经扩大。2007年,一种罕见的AE亚型——抗腺苷酸激酶5 (AK5)脑炎首次被报道。此病常见于老年男性,以边缘脑炎为核心表型(以亚急性顺行性遗忘为特征,有时伴有精神症状,很少伴有癫痫发作)。脑磁共振成像典型表现为初始颞叶T2/液体衰减反转恢复高强度,随后萎缩。尚未发现伴发肿瘤。针对细胞内抗原的AK5抗体是非副肿瘤t细胞自身免疫反应的生物标志物,可以通过基于组织和基于细胞的检测在血清和脑脊液中检测到。细胞毒性t细胞介导的神经元损伤和丢失在抗ak5脑炎的免疫发病机制中起关键作用。患者大多对免疫治疗反应差,因此长期预后差。在此,我们回顾了文献并提供了这一鲜为人知的实体的最新知识,重点是临床特征,临床旁发现,诊断过程和治疗方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Anti-adenylate kinase 5 encephalitis: Clinical characteristics, diagnosis, and management of this rare entity

Anti-adenylate kinase 5 encephalitis: Clinical characteristics, diagnosis, and management of this rare entity

Anti-adenylate kinase 5 encephalitis: Clinical characteristics, diagnosis, and management of this rare entity

Anti-adenylate kinase 5 encephalitis: Clinical characteristics, diagnosis, and management of this rare entity

The spectrum and understanding of antibody-positive autoimmune encephalitis (AE) have expanded over the past few decades. In 2007, a rare subtype of AE known as anti-adenylate kinase 5 (AK5) encephalitis, was first reported. This disease is more common in elderly males, with limbic encephalitis as the core phenotype (characterized by subacute anterograde amnesia, sometimes with psychiatric symptoms, and rarely with seizures). Brain magnetic resonance imaging typically demonstrated initial temporal lobe T2/fluid-attenuated inversion recovery hyperintensities, and subsequent atrophy. No concomitant tumors have been found yet. AK5 antibody, targeting the intracellular antigen, is a biomarker for a non-paraneoplastic T-cell autoimmunity response, and can be detected in serum and cerebrospinal fluid using tissue-based and cell-based assays. Cytotoxic T-cell-mediating neuronal injury and loss play a pivotal role in the immunopathogenesis of anti-AK5 encephalitis. Patients mostly show poor response to immunotherapy and thus a poor prognosis in the long run. Herein, we review the literature and provide updated knowledge of this less-known entity, focusing on clinical characteristics, paraclinical findings, diagnosis process, and therapeutic approaches.

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来源期刊
Journal of Translational Autoimmunity
Journal of Translational Autoimmunity Medicine-Immunology and Allergy
CiteScore
7.80
自引率
2.60%
发文量
33
审稿时长
55 days
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