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Multifactorial glial responses and their contributions to Alzheimer's disease continuum 多因子神经胶质反应及其对阿尔茨海默病连续性的贡献
Q4 Immunology and Microbiology Pub Date : 2023-02-09 DOI: 10.1111/cen3.12745
Masanori Hijioka, Tatsuya Manabe, Takashi Saito

Alzheimer's disease (AD) is the most common neurocognitive disorder. Various factors are intricately intertwined before clinical symptoms appear, although both amyloid-β peptide deposition and neurofibrillary tangle formation (i.e. pathological hallmarks of the AD brain) are established. Among such factors, glial responses have been increasingly recognized as important roles in the progression of these pathologies and viewed as one component of the AD continuum. However, the detailed molecular and cellular mechanisms of glial function underlying AD pathogenesis remain to be elucidated. Recent studies showed that peripheral immunity, gut microbiota or environmental factors influence brain pathophysiologies through communication with glial cells in the brain. This disease complexity makes understanding AD etiology difficult and hinders the development of effective therapeutic strategies to tackle this disease. Conversely, aged patients often suffer from multiple – not a single – diseases as multimorbidity, and AD pathogenesis might be related to pathologies caused by other diseases. Hence, investigating AD as a systemic disease has become critical for identifying therapeutic interventions. This review aimed to summarize current knowledge on AD research and share perspectives for understanding glial functions regarding AD pathophysiology.

阿尔茨海默病(AD)是最常见的神经认知障碍。在临床症状出现之前,各种因素错综复杂地交织在一起,尽管淀粉样蛋白β肽沉积和神经原纤维缠结形成(即阿尔茨海默病大脑的病理标志)都是确定的。在这些因素中,神经胶质反应越来越被认为是这些病理进展中的重要角色,并被视为AD连续体的一个组成部分。然而,阿尔茨海默病发病机制中胶质细胞功能的详细分子和细胞机制仍有待阐明。最近的研究表明,外周免疫、肠道微生物群或环境因素通过与脑胶质细胞的交流影响脑病理生理。这种疾病的复杂性使得了解阿尔茨海默病的病因变得困难,并阻碍了有效治疗策略的发展,以应对这种疾病。相反,老年患者往往患有多种疾病,而不是单一疾病,AD的发病机制可能与其他疾病引起的病理有关。因此,将AD作为一种全身性疾病进行研究对于确定治疗干预措施至关重要。本文综述了阿尔茨海默病的研究现状,并就阿尔茨海默病的病理生理方面的神经胶质功能的认识进行了综述。
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引用次数: 2
Identification of neuromuscular medullary thymic epithelial cells in thymoma with myasthenia gravis 胸腺瘤伴重症肌无力患者胸腺神经肌肉髓质上皮细胞的鉴定
Q4 Immunology and Microbiology Pub Date : 2023-01-30 DOI: 10.1111/cen3.12743
Tatsusada Okuno, Yoshiaki Yasumizu, Hideki Mochizuki
Autoimmune diseases lead to antibodies mistakenly recognizing and attacking host cells as foreign invaders. One such disease is myasthenia gravis (MG), where the antibodies target neuromuscular-associated proteins, including the acetylcholine receptor. MG commonly occurs in patients with thymoma; however, the reasons for this remain unclear. Recently, a bioinformatic approach was undertaken to examine the relationship between these two diseases.
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引用次数: 0
Transverse myelitis following bivalent COVID-19 booster vaccine and quadrivalent seasonal influenza vaccine 二价新冠肺炎加强疫苗和四价季节性流感疫苗后的横贯性脊髓炎
Q4 Immunology and Microbiology Pub Date : 2023-01-24 DOI: 10.1111/cen3.12742
Teresa L. Xiao, Alexandre Zaharia, Anas S. Al-Smadi, Caleb J. Murphy

We present a case of acute partial transverse myelitis (TM) that developed 6 d following covaccination with a bivalent COVID-19 booster vaccine and quadrivalent influenza vaccine. Although initial imaging of the thoracic spine was nonspecific, repeat magnetic resonance imaging (MRI) showed a T2 hyperintense lesion with contrast enhancement, consistent with TM. The risk of rare but catastrophic neurologic complications following the bivalent COVID-19 booster vaccine and/or covaccination with COVID-19 and influenza vaccines is the subject of ongoing investigation in the medical community.

一名78岁女性,既往有骨质疏松、二尖瓣反流和高脂血症病史,于2022年9月出现左下肢无力和麻木。在就诊前24小时,患者处于神经系统基线,正在活动,突然出现头晕和左大腿感觉异常,随后出现全身左腿无力。她去了急诊室,无法独立行走。她注意到左上腹部有疼痛的带状感觉,但否认鞍状麻醉或大便失禁。在左下肢体检中,髋关节和膝关节屈曲和伸展的力量为4/5,多个皮节对轻度触摸的感觉普遍下降,巴宾斯基征呈阳性。对右下肢的检查并不显著。实验室检查包括严重急性呼吸系统综合征冠状病毒2型聚合酶链式反应、C反应蛋白、肌酸激酶、促甲状腺激素、维生素B12、血管紧张素转换酶、抗核抗体、抗HMG-CoA还原酶抗体、抗SSA和SSB抗体以及抗双链DNA抗体均在正常范围内或阴性。没有造影剂的脑磁共振成像(MRI)并不显著。经对比的初步胸部MRI显示,在T5-6时出现T2高信号的非特异性、非增强性病变(见图1A、B)。医院第4天的重复胸部成像再次是非特异性的,鉴别诊断仍然很广泛。起初,患者的力量和功能每天都有适度的改善,但从医院第7天到第9天,她的功能改善停滞不前,出现了尿潴留和便秘。在住院期间,尿潴留逐渐改善,在住院第7天至第13天,记录的排尿后残余量(PVR)逐渐降低。第8天在医院进行腰椎穿刺。脑脊液研究在正常范围内或阴性,包括细胞计数(收集的第四管中为零核细胞)、革兰氏染色和培养、多病原体脑膜炎组(包括水痘-带状疱疹病毒和单纯疱疹病毒1和2)以及寡克隆带。在医院第9天,将血清样本送往外部实验室,用荧光激活细胞分选(FACS)测定法检测抗MOG和抗NMO/AQP4抗体。两次检测结果均为阴性。由于症状的进展和缺乏替代诊断,在医院第10天重复进行了胸部MRI对比检查。这显示出更明显的T2高信号,现在从T3延伸到T7,同时在T5–T6出现病理对比增强,与横贯性脊髓炎一致(TM;见图1D–F)。值得注意的是,对第三医院影像学的回顾性审查显示,轴向影像学有微弱、不明确的增强(见图1C)。她否认最近发生过任何传染事件、生病的接触者或咳嗽或流涕等常见传染症状。她没有已知的自身免疫性疾病家族史。然而,她在症状出现前6天接种了二价Pfizer-BioNTech新冠肺炎加强疫苗和Fluzone四价流感疫苗。患者被诊断为横贯性脊髓炎,并完成了5天的高剂量静脉注射甲基强的松龙,之后出院完成门诊物理治疗。出院后2个月,她被接收:2022年11月22日修订:2022年12月21日接受:2023年1月5日
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引用次数: 0
The Japanese clinical guidelines 2022 for myasthenia gravis and Lambert–Eaton myasthenic syndrome 2022年日本重症肌无力和Lambert–Eaton肌无力综合征临床指南
Q4 Immunology and Microbiology Pub Date : 2023-01-09 DOI: 10.1111/cen3.12739
Hiroyuki Murai, Kimiaki Utsugisawa, Masakatsu Motomura, Tomihiro Imai, Akiyuki Uzawa, Shigeaki Suzuki

The revised Japanese clinical guidelines for myasthenia gravis (MG) and Lambert–Eaton myasthenic syndrome (LEMS) were published in 2022. The notable points in these guidelines (GLs) are as follows: (i) these are the first Japanese GLs to include a description of LEMS; (ii) diagnostic criteria of MG are revised to lessen the incidence of false negative patients; (iii) MG is divided into six clinical subtypes; (iv) a high-dose oral steroid regimen with escalation and de-escalation schedule is not recommended by the GLs; (v) the GLs promote the early fast-acting treatment strategy initially proposed in the previous GLs; (vi) refractory MG is defined; (vii) the use of molecular targeted drugs is included; (viii) diagnostic criteria of LEMS are proposed; and (ix) treatment algorithms for both MG and LEMS are presented. These new GLs are expected to improve the patients' quality of life and will serve to bridge the present era with the molecular targeted treatment eras.

修订后的日本重症肌无力(MG)和Lambert-Eaton肌无力综合征(LEMS)临床指南于2022年出版。这些指南(GLs)中值得注意的要点如下:(i)这是第一个包含LEMS描述的日本GLs;(ii)修改MG的诊断标准,以减少假阴性患者的发生率;(iii) MG临床分为6个亚型;(iv) GLs不推荐高剂量口服类固醇治疗方案的升级和降级计划;(v) gl促进了之前gl中最初提出的早期快速治疗策略;(vi)定义难熔MG;(七)包括分子靶向药物的使用;(viii)提出LEMS的诊断标准;(ix)给出了MG和LEMS的处理算法。这些新的GLs有望改善患者的生活质量,并将为当前时代和分子靶向治疗时代架起一座桥梁。
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引用次数: 6
All differential diagnoses of cerebellar ataxia should be ruled out before SARS-CoV-2 is blamed as the cause 在将SARS-CoV-2归咎于病因之前,应排除所有小脑共济失调的鉴别诊断
Q4 Immunology and Microbiology Pub Date : 2022-12-08 DOI: 10.1111/cen3.12737
Josef Finsterer
We read
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引用次数: 0
Epidemiological study of myasthenia gravis in Japan 日本重症肌无力的流行病学研究
Q4 Immunology and Microbiology Pub Date : 2022-11-23 DOI: 10.1111/cen3.12736
Hiroaki Yoshikawa

Myasthenia gravis (MG) is an autoimmune-mediated neurological disorder. The relationship between MG and thymic abnormalities is well recognized, and thymectomy is one of the therapies for anti-acetylcholine receptor antibody-positive MG. The major pathogenic factor is anti-acetylcholine receptor antibody followed by anti-muscle-specific kinase antibody, and commercial kits are available to detect these antibodies. Several decades ago, the prognosis of MG was not favorable; therefore, the Ministry of Health and Welfare (predecessor of the Ministry of Health, Labor and Welfare) organized a Taskforce for Intractable Diseases, which included MG, in 1972. The Taskforce carried out consecutive epidemiological studies for MG in 1973, 1987, 2006 and 2018. The four studies found: (i) increasing prevalence; (ii) increasing late- and elderly-onset; (iii) decreasing female dominancy; (iv) decreasing infantile-onset (onset age of 0–4 years); and (v) decreasing frequencies of crisis. The latest epidemiological study in Japan and studies from other countries suggest an increasing number of patients with anti-acetylcholine receptor antibody-positive MG without thymoma in the elderly. It is important to find out the causes of this phenomenon, which will improve the prevention of MG.

重症肌无力(MG)是一种自身免疫介导的神经系统疾病。MG与胸腺异常之间的关系已被公认,胸腺切除术是抗乙酰胆碱受体抗体阳性MG的治疗方法之一。主要致病因素是抗乙酰胆碱接收器抗体,其次是抗肌肉特异性激酶抗体,商业试剂盒可用于检测这些抗体。几十年前,MG的预后并不好;因此,厚生劳动省(厚生劳动部的前身)于1972年组织了一个包括MG在内的顽固性疾病特别工作组。工作组于1973年、1987年、2006年和2018年对MG进行了连续的流行病学研究。四项研究发现:(一)患病率上升;(ii)晚发和老年发病率增加;(iii)女性主导地位下降;(iv)婴儿发病率下降(发病年龄0–4岁 年);以及(v)减少危机发生的频率。日本的最新流行病学研究和其他国家的研究表明,在老年人中,越来越多的抗乙酰胆碱受体抗体阳性MG患者没有胸腺瘤。找出这一现象的原因对提高MG的预防水平具有重要意义。
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引用次数: 1
Issue Information 问题信息
Q4 Immunology and Microbiology Pub Date : 2022-11-01 DOI: 10.1111/odi.14414
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引用次数: 0
Autoantibodies against contactin-associated protein 1 and complexes of paranode-specific proteins in chronic inflammatory demyelinating polyradiculoneuropathy 慢性炎症性脱髓鞘多发性神经根病变中针对接触蛋白相关蛋白1和副节点特异性蛋白复合物的自身抗体
Q4 Immunology and Microbiology Pub Date : 2022-10-26 DOI: 10.1111/cen3.12735
Michiaki Koga, Toshihiko Maeda, Fumitaka Shimizu, Takashi Kanda

Objective

To investigate the frequency of serum autoantibodies targeting contactin-associated protein 1 (Caspr1) and its complexes with other paranode antigens, contactin-1 (CNTN1) and neurofascin 155 (NF155), in Japanese patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

Methods

Sera from 26 CIDP patients, 35 patients with Guillain–Barré syndrome, and 31 healthy individuals participated. Paranodal immunoglobulin G antibodies were quantified using enzyme-linked immunosorbent assays with commercially available recombinant proteins as antigens.

Results

Anti-Caspr1 antibodies were present in one participant (case 1, 3.8%) of CIDP, and negative in all Guillain–Barré syndrome patients and healthy participant. Case 1 was a man who developed subacute distal limb-predominant muscle weakness and sensory ataxia with postural hand tremor at 69 years-of-age, and therapeutic benefit of intravenous immunoglobulin and oral steroids was inadequate. The detected anti-Caspr1 antibodies predominantly belonged to the immunoglobulin G4 subclass, and the addition of CNTN1 to Caspr1 as an antigen increased antibody reactivity, although the increase was just 20–30% at most. The presence of autoantibodies against paranode protein complexes, including Caspr1/CNTN1, Caspr1/NF155 and Caspr1/CNTN1/NF155, was confirmed in several CIDP patients, although they also had anti-Caspr1 or anti-NF155 antibodies; thus, in our cohort, there were no patients with autoantibodies that specifically recognized paranode protein complexes.

Conclusions

This is the first confirmed Japanese case of anti-Caspr1 antibody-positive CIDP with a clinical signature similar to that of patients of Western origin. Our preliminary study did not identify the presence of specific antibodies against the paranode protein complexes, and the primary target antigen is likely Caspr1.

研究日本慢性炎症性脱髓鞘性多神经根神经病(CIDP)患者血清中针对接触蛋白相关蛋白1(Caspr1)及其与其他副极抗原接触蛋白1(CNTN1)和神经筋膜素155(NF155)复合物的自身抗体的频率。
{"title":"Autoantibodies against contactin-associated protein 1 and complexes of paranode-specific proteins in chronic inflammatory demyelinating polyradiculoneuropathy","authors":"Michiaki Koga,&nbsp;Toshihiko Maeda,&nbsp;Fumitaka Shimizu,&nbsp;Takashi Kanda","doi":"10.1111/cen3.12735","DOIUrl":"10.1111/cen3.12735","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>To investigate the frequency of serum autoantibodies targeting contactin-associated protein 1 (Caspr1) and its complexes with other paranode antigens, contactin-1 (CNTN1) and neurofascin 155 (NF155), in Japanese patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Sera from 26 CIDP patients, 35 patients with Guillain–Barré syndrome, and 31 healthy individuals participated. Paranodal immunoglobulin G antibodies were quantified using enzyme-linked immunosorbent assays with commercially available recombinant proteins as antigens.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Anti-Caspr1 antibodies were present in one participant (case 1, 3.8%) of CIDP, and negative in all Guillain–Barré syndrome patients and healthy participant. Case 1 was a man who developed subacute distal limb-predominant muscle weakness and sensory ataxia with postural hand tremor at 69 years-of-age, and therapeutic benefit of intravenous immunoglobulin and oral steroids was inadequate. The detected anti-Caspr1 antibodies predominantly belonged to the immunoglobulin G4 subclass, and the addition of CNTN1 to Caspr1 as an antigen increased antibody reactivity, although the increase was just 20–30% at most. The presence of autoantibodies against paranode protein complexes, including Caspr1/CNTN1, Caspr1/NF155 and Caspr1/CNTN1/NF155, was confirmed in several CIDP patients, although they also had anti-Caspr1 or anti-NF155 antibodies; thus, in our cohort, there were no patients with autoantibodies that specifically recognized paranode protein complexes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>This is the first confirmed Japanese case of anti-Caspr1 antibody-positive CIDP with a clinical signature similar to that of patients of Western origin. Our preliminary study did not identify the presence of specific antibodies against the paranode protein complexes, and the primary target antigen is likely Caspr1.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"14 2","pages":"116-121"},"PeriodicalIF":0.0,"publicationDate":"2022-10-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45050723","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A case of natalizumab-associated progressive multifocal leukoencephalopathy followed by immune reconstitution inflammatory syndrome with difficulty in the timing of immunotherapy 1例natalizumab相关的进行性多灶性白质脑病,随后出现免疫重建炎症综合征,免疫治疗时机困难
Q4 Immunology and Microbiology Pub Date : 2022-10-18 DOI: 10.1111/cen3.12734
Takamichi Sugimoto, Shuichiro Neshige, Shiro Aoki, Kazuhide Ochi, Ruoyi Ishikawa, Megumi Nonaka, Masahiro Nakamori, Tomohisa Nezu, Kazuo Nakamichi, Yu Yamazaki, Hirofumi Maruyama

Background

Details regarding the clinical course of natalizumab-associated progressive multifocal leukoencephalopathy (NAT-PML) have not been reported in Japanese patients. We experienced a Japanese NAT-PML case and report it for the purpose of clarifying the challenge it posed in medical treatment.

Case Presentation

Herein, we describe a 58-y-old multiple sclerosis patient who had NAT-PML with immune reconstitution inflammatory syndrome (IRIS). Before NAT-PML developed, the patient's Expanded Disability Status Scale (EDSS) score was 5.5. She received natalizumab (300 mg) every 3–7 w, and her EDSS score had not changed for 9.1 y. The John Cunningham virus (JCV) index was 0.43 before NAT-PML developed. She developed a gait disturbance when NAT-PML emerged. Natalizumab was administered a total of 108 times. The patient was diagnosed with probable NAT-PML on the basis of punctate lesions on T2-weighted imaging and a JCV-PCR result of 29 copies/ml from cerebrospinal fluid (CSF) testing. Intravenous methylprednisolone (IVMP) was initiated when the contrast-enhanced lesion was first detected, but the NAT-PML lesion was rather enlarged despite the temporary disappearance of the contrast-enhanced lesion. An obvious increase in the IgG index and a slight increase in the CSF cell count were recognized at the time the immunological response was activated. Three cycles of a 3-d course of IVMP were administered every 2 w for IRIS, and the patient's worst EDSS score was 9.5.

Conclusion

Treatment sequencing should be executed before the onset of NAT-PML. Changes in CSF cell count and IgG index may be useful for treatment decision; further research is needed.

关于natalizumab相关的进行性多灶性白质脑病(NAT - PML)的临床病程细节尚未在日本患者中报道。我们经历了一个日本的NAT - PML病例,并报告了它,目的是澄清它在医学治疗中所带来的挑战。
{"title":"A case of natalizumab-associated progressive multifocal leukoencephalopathy followed by immune reconstitution inflammatory syndrome with difficulty in the timing of immunotherapy","authors":"Takamichi Sugimoto,&nbsp;Shuichiro Neshige,&nbsp;Shiro Aoki,&nbsp;Kazuhide Ochi,&nbsp;Ruoyi Ishikawa,&nbsp;Megumi Nonaka,&nbsp;Masahiro Nakamori,&nbsp;Tomohisa Nezu,&nbsp;Kazuo Nakamichi,&nbsp;Yu Yamazaki,&nbsp;Hirofumi Maruyama","doi":"10.1111/cen3.12734","DOIUrl":"10.1111/cen3.12734","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Details regarding the clinical course of natalizumab-associated progressive multifocal leukoencephalopathy (NAT-PML) have not been reported in Japanese patients. We experienced a Japanese NAT-PML case and report it for the purpose of clarifying the challenge it posed in medical treatment.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>Herein, we describe a 58-y-old multiple sclerosis patient who had NAT-PML with immune reconstitution inflammatory syndrome (IRIS). Before NAT-PML developed, the patient's Expanded Disability Status Scale (EDSS) score was 5.5. She received natalizumab (300 mg) every 3–7 w, and her EDSS score had not changed for 9.1 y. The John Cunningham virus (JCV) index was 0.43 before NAT-PML developed. She developed a gait disturbance when NAT-PML emerged. Natalizumab was administered a total of 108 times. The patient was diagnosed with probable NAT-PML on the basis of punctate lesions on T2-weighted imaging and a JCV-PCR result of 29 copies/ml from cerebrospinal fluid (CSF) testing. Intravenous methylprednisolone (IVMP) was initiated when the contrast-enhanced lesion was first detected, but the NAT-PML lesion was rather enlarged despite the temporary disappearance of the contrast-enhanced lesion. An obvious increase in the IgG index and a slight increase in the CSF cell count were recognized at the time the immunological response was activated. Three cycles of a 3-d course of IVMP were administered every 2 w for IRIS, and the patient's worst EDSS score was 9.5.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Treatment sequencing should be executed before the onset of NAT-PML. Changes in CSF cell count and IgG index may be useful for treatment decision; further research is needed.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"14 2","pages":"110-115"},"PeriodicalIF":0.0,"publicationDate":"2022-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48754605","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Level of CSF CXCL10 is highly elevated and decreased after steroid therapy in patients with autoimmune glial fibrillary acidic protein astrocytopathy 自身免疫性胶质纤维酸性蛋白星形细胞病患者的CSF CXCL10水平在类固醇治疗后高度升高和降低
Q4 Immunology and Microbiology Pub Date : 2022-10-10 DOI: 10.1111/cen3.12732
Takayuki Kikuchi, Naoki Takao, Tomoo Sato, Kenji Isahaya, Sakae Hino, Mayumi Kaburagi, Keiji Tachikawa, Riyoko Ko, Soichiro Shibata, Kei Kaburagi, Naoki Iijima, Heisuke Mizukami, Kenzo Sakurai, Junji Yamauchi, Akio Kimura, Takayoshi Shimohata, Yoshihisa Yamano

Objectives

To examine the chemokine profile in the cerebrospinal fluid (CSF) of patients with glial fibrillary acidic protein astrocytopathy (GFAP-A), central nervous system immune-related adverse event (CNS-irAE), neurosarcoidosis (NS), neuromyelitis optica spectrum disorders (NMOSD), multiple sclerosis (MS), and human T-cell leukemia virus-1 (HTLV-1)-associated myelopathy (HAM).

Methods

The study included 38 patients presenting to St. Marianna University Hospital between May 2013 and November 2021 with GFAP-A, CNS-irAE, NMOSD, MS, NS, HAM and noninflammatory neurological diseases (NIND). We recorded the age, sex, duration of disease, brain/spinal lesions on magnetic resonance imaging (MRI), blood data, and measured chemokines (CXCL9, −10, −13, CCL3, −4, −17, −20, −22) in CSF. In patients with GFAP-A, clinical symptoms, and CSF CXCL10 levels were compared before and after steroid treatment.

Results

Patients with GFAP-A had higher CSF levels of CXCL10, CXCL13, and CCL22 (10736.1 [8786.7–149079.0] pg/ml (p < .05), 378.4 [239.9–412.2] pg/ml (p < .01) and 159.9 [130.5–413.9] pg/ml (p < .01), respectively). The CSF levels of CXCL10 improved from 10736.1 [8786.7–149079.0] pg/ml to 1879.0 [783.9–4360.0] pg/ml in patients with GFAP-A by steroid therapy.

Conclusion

CSF CXCL10 levels were particularly high in GFAP-A, and changes in levels after treatment correlated with clinical improvements, suggesting CXCL10 involvement in GFAP-A pathogenesis.

检测胶质纤维酸性蛋白星形细胞病(GFAP‐A)、中枢神经系统免疫相关不良事件(CNS‐irAE)、神经肉瘤(NS)、视神经脊髓炎(NMOSD)、多发性硬化症(MS)和人类T细胞白血病病毒-1(HTLV‐1)相关脊髓病(HAM)患者脑脊液(CSF)中的趋化因子谱。
{"title":"Level of CSF CXCL10 is highly elevated and decreased after steroid therapy in patients with autoimmune glial fibrillary acidic protein astrocytopathy","authors":"Takayuki Kikuchi,&nbsp;Naoki Takao,&nbsp;Tomoo Sato,&nbsp;Kenji Isahaya,&nbsp;Sakae Hino,&nbsp;Mayumi Kaburagi,&nbsp;Keiji Tachikawa,&nbsp;Riyoko Ko,&nbsp;Soichiro Shibata,&nbsp;Kei Kaburagi,&nbsp;Naoki Iijima,&nbsp;Heisuke Mizukami,&nbsp;Kenzo Sakurai,&nbsp;Junji Yamauchi,&nbsp;Akio Kimura,&nbsp;Takayoshi Shimohata,&nbsp;Yoshihisa Yamano","doi":"10.1111/cen3.12732","DOIUrl":"10.1111/cen3.12732","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>To examine the chemokine profile in the cerebrospinal fluid (CSF) of patients with glial fibrillary acidic protein astrocytopathy (GFAP-A), central nervous system immune-related adverse event (CNS-irAE), neurosarcoidosis (NS), neuromyelitis optica spectrum disorders (NMOSD), multiple sclerosis (MS), and human T-cell leukemia virus-1 (HTLV-1)-associated myelopathy (HAM).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>The study included 38 patients presenting to St. Marianna University Hospital between May 2013 and November 2021 with GFAP-A, CNS-irAE, NMOSD, MS, NS, HAM and noninflammatory neurological diseases (NIND). We recorded the age, sex, duration of disease, brain/spinal lesions on magnetic resonance imaging (MRI), blood data, and measured chemokines (CXCL9, −10, −13, CCL3, −4, −17, −20, −22) in CSF. In patients with GFAP-A, clinical symptoms, and CSF CXCL10 levels were compared before and after steroid treatment.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Patients with GFAP-A had higher CSF levels of CXCL10, CXCL13, and CCL22 (10736.1 [8786.7–149079.0] pg/ml (<i>p</i> &lt; .05), 378.4 [239.9–412.2] pg/ml (<i>p</i> &lt; .01) and 159.9 [130.5–413.9] pg/ml (<i>p</i> &lt; .01), respectively). The CSF levels of CXCL10 improved from 10736.1 [8786.7–149079.0] pg/ml to 1879.0 [783.9–4360.0] pg/ml in patients with GFAP-A by steroid therapy.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>CSF CXCL10 levels were particularly high in GFAP-A, and changes in levels after treatment correlated with clinical improvements, suggesting CXCL10 involvement in GFAP-A pathogenesis.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"14 1","pages":"61-68"},"PeriodicalIF":0.0,"publicationDate":"2022-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48892579","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
期刊
Clinical and Experimental Neuroimmunology
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