Clinical and Experimental Neuroimmunology最新文献

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Response to: Unveiling Oversights in Hypothermia's Neuroprotective Mechanisms 回应：揭示低温的神经保护机制的疏忽

Q4 Immunology and Microbiology

Clinical and Experimental Neuroimmunology

Pub Date : 2025-11-14 DOI: 10.1111/cen3.70022

Tomohiro Matsui

引用次数: 0

Response to: Not All Patients Start From the Same Line: Rethinking Outcome Interpretation in Myasthenia Gravis 回应：并非所有患者都从同一条线开始：重症肌无力的重新思考结果解释

Q4 Immunology and Microbiology

Clinical and Experimental Neuroimmunology

Pub Date : 2025-11-11 DOI: 10.1111/cen3.70038

Akiyuki Uzawa, Ema Rodrigues, Hiroyuki Murai

引用次数: 0

Reply to “Reply to Seroconversion After SARS-CoV-2 Vaccination in B-Cell-Depleted Patients: The Missing Factors” 回复《对b细胞缺失患者接种SARS-CoV-2后血清转化的回复：缺失因素》

Q4 Immunology and Microbiology

Clinical and Experimental Neuroimmunology

Pub Date : 2025-11-05 DOI: 10.1111/cen3.70036

Christian Messina

引用次数: 0

An Adult Case of Severe, Refractory, and Progressive Leukodystrophy-Like Diffuse Encephalitis With MOG-IgG Treated With Rituximab 利妥昔单抗治疗MOG-IgG治疗严重、难治性进展性白质营养不良样弥漫性脑炎1例

Q4 Immunology and Microbiology

Clinical and Experimental Neuroimmunology

Pub Date : 2025-11-03 DOI: 10.1111/cen3.70035

Yuki Matsumoto, Hirohiko Ono, Tatsuro Misu, Yoshiki Takai, Shuhei Nishiyama, Toshiyuki Takahashi, Kazuhiro Kurosawa, Masashi Aoki

Background

Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is typically considered responsive to immunotherapy with a good prognosis. However, some patients develop severe symptoms and the underlying pathologic and immunologic mechanisms remain unclear. We report a severe, progressive, and fatal case of MOGAD treated with rituximab (RTX).

Case Presentation

A 43-year-old woman was admitted to the hospital for bilateral vision loss followed by a convulsive seizure and comatose state. Her serum and cerebrospinal fluid (CSF) tested positive for MOG-IgG using our in-house living cell–based assay, with higher antibody titers in her CSF than in her serum. She was diagnosed with MOGAD and treated with intravenous methylprednisolone, plasma exchange therapy, and immunoglobulin therapy. However, she was refractory to these treatments, leading to progressive leukodystrophy-like white matter lesions. A brain biopsy at this time revealed widespread demyelination as well as massive infiltration of CD4-positive cells and macrophages. We then treated her with intravenous cyclophosphamide therapy. Still, her MRI showed progressive exacerbation, and we detected a marked increase in B-cell lineage cells such as CD19⁺ CD27⁻ IgD⁻ CXCR5⁻ (double-negative B cells) and plasma cells in peripheral blood and CSF. As a result, we tried RTX, which resulted in MRI lesion reduction in part initially after a progressively worsened course. The comatose state persisted, and she eventually died of aspiration pneumonia 1 year later.

Conclusions

We report a severe refractory case of MOGAD that responded only to RTX. Early RTX treatment should be considered for patients with severe MOGAD.

髓鞘少突胶质细胞糖蛋白（MOG）抗体相关疾病（MOGAD）通常被认为对免疫治疗有反应，预后良好。然而，一些患者出现严重症状，潜在的病理和免疫机制尚不清楚。我们报告了一例严重的、进行性的、致命的MOGAD病例，采用利妥昔单抗（RTX）治疗。一名43岁女性因双侧视力丧失而入院，随后惊厥发作和昏迷状态。她的血清和脑脊液（CSF）检测MOG-IgG阳性，使用我们内部的活细胞检测，脑脊液中的抗体滴度高于血清。她被诊断为MOGAD，并接受静脉注射甲基强的松龙、血浆交换治疗和免疫球蛋白治疗。然而，她对这些治疗难治，导致进行性脑白质样病变。此时的脑组织活检显示广泛的脱髓鞘以及大量cd4阳性细胞和巨噬细胞浸润。然后我们给她静脉注射环磷酰胺治疗。尽管如此，她的MRI显示进行性恶化，我们检测到外周血和脑脊液中B细胞系细胞如CD19+ CD27 - IgD - CXCR5 -（双阴性B细胞）和浆细胞明显增加。因此，我们尝试了RTX，结果在病程逐渐恶化后，MRI病变部分减少。昏迷状态持续，最终于1年后死于吸入性肺炎。结论：我们报告了一例仅对RTX有反应的严重难治性MOGAD病例。对于严重MOGAD患者应考虑早期RTX治疗。

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引用次数: 0

Heterogeneity of Ocular Myasthenia Gravis: Potential Subgroups 眼重症肌无力的异质性：潜在亚群

Q4 Immunology and Microbiology

Clinical and Experimental Neuroimmunology

Pub Date : 2025-10-26 DOI: 10.1111/cen3.70034

Tomoko Narita, Akiko Nagaishi, Shunya Nakane, Hidenori Matsuo

Objectives

The clinical features of ocular myasthenia gravis (OMG) appeared to be diverse in terms of age at onset and autoantibody titers, suggesting the possibility of the existence of multiple subgroups. To characterize each subgroup, we investigated the clinical characteristics of Japanese patients with OMG.

Methods

We performed a retrospective cross-sectional survey of OMG patients from eight hospitals in Japan. Clinical information was obtained, including age at onset, autoantibodies including anti-acetylcholine receptor (AChR) titers, complications, and clinical course.

Results

In total, 135 patients with OMG (67 men, 68 women) were included. There appeared to be three peaks in onset age; one was below 5 years old, and others at 45–49 and 60–64 years old. Moreover, 20.7% developed OMG after 65 years old. Anti-AChR antibodies were detected in 77.6%, among which 31.3% had rather low titers (0.2–1.9 nmol/L), while 12 patients had very high titers (50–1720 nmol/L). There were thymic abnormalities and/or autoimmune complications, as seen in patients with generalized MG.

Conclusions

The clinical features of OMG appeared to be heterogeneous, with several potential subgroups including childhood onset, a subset with very high antibody titers, and an elderly onset group.

目的眼重症肌无力（OMG）的临床特征在发病年龄和自身抗体效价方面表现出多样性，提示其可能存在多个亚群。为了确定每个亚组的特征，我们调查了日本OMG患者的临床特征。方法对日本8家医院的OMG患者进行回顾性横断面调查。获得临床信息，包括发病年龄、自身抗体包括抗乙酰胆碱受体（AChR）滴度、并发症和临床病程。结果共纳入135例OMG患者（男性67例，女性68例）。发病年龄有三个高峰；其中一名年龄在5岁以下，另外两名年龄在45-49岁和60-64岁之间。此外，20.7%的人在65岁以后发展为OMG。77.6%的患者检测到抗achr抗体，其中31.3%的患者滴度较低（0.2 ~ 1.9 nmol/L）， 12例患者滴度很高（50 ~ 1720 nmol/L）。有胸腺异常和/或自身免疫性并发症，见于全身性MG患者。结论：OMG的临床特征似乎是异质性的，有几个潜在的亚组，包括儿童期发病、抗体滴度很高的亚组和老年发病组。

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引用次数: 0

Recurrent Asystole Without Generalized Dysautonomia in Anti-NMDA Receptor Encephalitis: A Case Report 抗nmda受体脑炎复发性无广泛性自主神经异常的心脏骤停1例

Q4 Immunology and Microbiology

Clinical and Experimental Neuroimmunology

Pub Date : 2025-10-17 DOI: 10.1111/cen3.70033

Tetsuya Oyama, Hirotaka Nakanishi

Background

Life-threatening arrhythmias in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis are typically regarded as part of a generalized autonomic storm. However, severe bradyarrhythmia occurring as the primary life-threatening feature in the absence of a generalized autonomic storm is rare.

Case Presentation

A 27-year-old woman presented with a 2-week history of neuropsychiatric symptoms. She was diagnosed with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, supported by a positive cerebrospinal fluid (CSF) antibody test and an ovarian teratoma. Her hospital course was complicated by recurrent asystolic cardiac arrests, which occurred as the most critical feature of her autonomic instability and required temporary pacing. The patient recovered fully after immunotherapy and tumor resection.

Conclusions

This report identifies a critical diagnostic pitfall and highlights the necessity of continuous cardiac monitoring during the acute phase, even in patients who appear autonomically stable.

背景：抗n -甲基- d -天冬氨酸受体（NMDAR）脑炎导致的危及生命的心律失常通常被认为是全身性自主神经风暴的一部分。然而，在没有全身性自主神经风暴的情况下，严重的慢性心律失常作为主要的危及生命的特征是罕见的。一名27岁女性，有2周的神经精神症状史。她被诊断为抗n -甲基- d -天冬氨酸受体（NMDAR）脑炎，脑脊液（CSF）抗体检测呈阳性，卵巢畸胎瘤。她的住院过程因复发性心脏停搏而变得复杂，这是她自主神经不稳定的最关键特征，需要临时起搏。患者经免疫治疗和肿瘤切除后完全康复。本报告指出了一个关键的诊断缺陷，并强调了在急性期进行持续心脏监测的必要性，即使在自主功能稳定的患者中也是如此。

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引用次数: 0

The Japan Neuromyelitis Optica Spectrum Disorders/Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease Registry: Background Analysis of the Initial 116 Cases 日本视神经脊髓炎频谱障碍/髓鞘少突胶质细胞糖蛋白抗体相关疾病登记：116例初始病例的背景分析

Q4 Immunology and Microbiology

Clinical and Experimental Neuroimmunology

Pub Date : 2025-10-10 DOI: 10.1111/cen3.70032

Yusei Miyazaki, Hideyuki Takeuchi, Takao Kiriyama, Hikoaki Fukaura, Juichi Fujimori, Katsuhisa Masaki, Sonoko Misawa, Katsuichi Miyamoto, Nobuaki Yoshikura, Shoko Ukita, Kengo Nagashima, Ichiro Nakashima

Objectives

To describe the baseline clinical characteristics of patients with neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) enrolled in the Japan NMOSD/MOGAD Registry (UMIN000051988) and to assess their representativeness of both conditions in the Japanese population.

Methods

This prospective multicenter observational registry study was conducted at 10 neurology referral centers in Japan. Patients with NMOSD or MOGAD were enrolled based on established diagnostic criteria. Clinical data, treatment history, and biological samples were collected annually. The present analysis includes patients registered between December 20, 2023 and July 31, 2024. Descriptive statistics were used to summarize the clinical data.

Results

A total of 116 patients (NMOSD, n = 110; MOGAD, n = 6) were analyzed. Among NMOSD patients, the mean age at onset was 50.0 years, and 89.1% were female. Most were anti-aquaporin 4 antibody seropositive (93.6%). The median Expanded Disability Status Scale (EDSS) was 3.5%, and 67.3% had a relapsing course. Biologic agents were used in 63.6% of NMOSD patients. Among MOGAD patients, the mean age at onset was 27.3 years, and all were anti-myelin oligodendrocyte glycoprotein antibody seropositive. Visual symptoms predominated, and the median EDSS was 2.25.

Conclusions

The clinical characteristics of NMOSD patients in the registry are consistent with prior epidemiological data, supporting the representativeness of the cohort. The high rate of biologic agent use reflects current treatment practices in Japan. In contrast, the representativeness of MOGAD cases could not be evaluated due to their limited number, highlighting the need to further accumulate cases.

目的描述日本NMOSD/MOGAD登记处（UMIN000051988）登记的视神经脊髓炎谱系障碍（NMOSD）和髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD）患者的基线临床特征，并评估其在日本人群中这两种疾病的代表性。方法本前瞻性多中心观察登记研究在日本的10个神经病学转诊中心进行。NMOSD或MOGAD患者根据既定的诊断标准入组。每年收集临床资料、治疗史和生物样本。本分析包括2023年12月20日至2024年7月31日登记的患者。采用描述性统计方法对临床资料进行汇总。结果共分析116例患者，其中NMOSD 110例，MOGAD 6例。NMOSD患者平均发病年龄为50.0岁，女性占89.1%。抗水通道蛋白4抗体血清学阳性占93.6%。扩展残疾状态量表（EDSS）中位数为3.5%，67.3%有复发过程。63.6%的NMOSD患者使用生物制剂。MOGAD患者的平均发病年龄为27.3岁，抗髓鞘少突胶质细胞糖蛋白抗体血清均呈阳性。以视觉症状为主，EDSS中位数为2.25。结论登记的NMOSD患者的临床特征与之前的流行病学数据一致，支持该队列的代表性。生物制剂的高使用率反映了日本目前的治疗做法。相比之下，MOGAD病例的代表性由于数量有限而无法评估，这突出了进一步积累病例的必要性。

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引用次数: 0

Reply to “Seroconversion After SARS-CoV-2 Vaccination in B-Cell-Depleted Patients: The Missing Factors” 回复“b细胞缺失患者接种SARS-CoV-2后血清转化：缺失因素”

Q4 Immunology and Microbiology

Clinical and Experimental Neuroimmunology

Pub Date : 2025-10-06 DOI: 10.1111/cen3.70031

Hideyuki Takeuchi

引用次数: 0

NMOSD: Adaptive Immunity Revisited NMOSD：重新审视适应性免疫

Q4 Immunology and Microbiology

Clinical and Experimental Neuroimmunology

Pub Date : 2025-10-03 DOI: 10.1111/cen3.70030

Ritsu Akatani, Norio Chihara

Accumulating evidence underscores the pivotal role of adaptive immunity in the pathogenesis of AQP4-IgG-positive neuromyelitis optica spectrum disorder (NMOSD). Autoreactive B cells produce pathogenic antibodies against aquaporin-4 (AQP4), and their dysregulation is evident in both peripheral blood and cerebrospinal fluid. These abnormalities include an increased frequency of plasmablasts and the expansion of atypical B-cell subsets enriched for autoreactivity. Their activation and survival are supported by inflammatory cytokines such as interleukin-6 (IL-6), a key target of current immunotherapies. T-cell subsets, including follicular helper T (Tfh) cells and Th17 cells, play essential roles by promoting B-cell differentiation and amplifying inflammation. The interplay between B and T cells, together with cytokine dysregulation, constitutes a central immunopathogenic axis in NMOSD. Notably, some disease-modifying therapies for multiple sclerosis have been shown to worsen NMOSD, suggesting fundamental differences in the underlying immune dynamics between the two diseases. This contrast highlights the need for disease-specific therapeutic approaches and a deeper understanding of adaptive immune processes unique to NMOSD. Here, we revisit the immunopathogenesis of AQP4-IgG-positive NMOSD with a focus on adaptive immune mechanisms. We discuss current evidence on B- and T-cell abnormalities, intrathecal immune responses, and how these insights have informed the development of targeted therapies.

越来越多的证据强调适应性免疫在aqp4 - igg阳性视神经脊髓炎谱系障碍（NMOSD）发病机制中的关键作用。自身反应性B细胞产生针对水通道蛋白-4 （AQP4）的致病性抗体，其失调在外周血和脑脊液中都很明显。这些异常包括质母细胞频率的增加和非典型b细胞亚群的扩增，这些细胞亚群富集于自身反应性。它们的激活和存活是由炎症细胞因子如白细胞介素-6 （IL-6）支持的，白细胞介素-6是当前免疫疗法的关键靶点。T细胞亚群，包括滤泡辅助性T （Tfh）细胞和Th17细胞，通过促进b细胞分化和放大炎症发挥重要作用。B细胞和T细胞之间的相互作用，以及细胞因子的失调，构成了NMOSD的中心免疫致病轴。值得注意的是，一些用于多发性硬化症的疾病修饰疗法已被证明会加重NMOSD，这表明这两种疾病之间潜在的免疫动力学存在根本差异。这种对比突出了对疾病特异性治疗方法的需求，以及对NMOSD特有的适应性免疫过程的更深入了解。在这里，我们重新审视aqp4 - igg阳性NMOSD的免疫发病机制，重点关注适应性免疫机制。我们讨论了目前关于B细胞和t细胞异常、鞘内免疫反应的证据，以及这些见解如何告知靶向治疗的发展。

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引用次数: 0

Seroconversion After SARS-CoV-2 Vaccination in B-Cell-Depleted Patients: The Missing Factors b细胞缺失患者接种SARS-CoV-2疫苗后血清转化：缺失因素

Q4 Immunology and Microbiology

Clinical and Experimental Neuroimmunology

Pub Date : 2025-10-01 DOI: 10.1111/cen3.70028

Christian Messina

引用次数: 0

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