HLA Association With AQP4-IgG-Positive Neuromyelitis Optica Spectrum Disorder in the Korean Population

韩国人群中人类白细胞抗原与水通道蛋白4抗体阳性的视神经脊髓炎谱系疾病的关联

Association of human leukocyte antigen (HLA) with anti-aquaporin-4 immunoglobulin G-positive neuromyelitis optica spectrum disorder (AQP4-IgG NMOSD) has been reported. However, this association in the Korean population has not been previously investigated. We aimed to evaluate whether specific HLA subtypes were associated with Korean patients with AQP4-IgG NMOSD and whether the HLA genotype is associated with specific clinical features. In a Korean cohort of patients withAQP4-IgG NMOSD, the HLA-DRB1*12:02-DQB1*03:01 haplotype was associated with disease severity at onset. HLA-DRB1*03:01, broadly reported as a significant susceptibility allele across diverse ethnic groups, showed a significant risk association in Korean patients with AQP4-IgG NMOSD.

已有研究报道人类白细胞抗原（HLA）与抗水通道蛋白 - 4 免疫球蛋白 G 阳性的视神经脊髓炎谱系疾病（AQP4 - IgG NMOSD）存在关联。然而，此前尚未对韩国人群中的这种关联进行过研究。我们旨在评估特定的 HLA 亚型是否与韩国 AQP4 - IgG NMOSD 患者相关，以及 HLA 基因型是否与特定的临床特征相关。在韩国 AQP4 - IgG NMOSD 患者队列中，HLA - DRB1*12:02 - DQB1*03:01 单倍型与疾病发病时的严重程度相关。HLA - DRB1*03:01 被广泛报道为不同种族群体中的重要易感等位基因，在韩国 AQP4 - IgG NMOSD 患者中显示出显著的风险关联。

REF: Hyun JW, Kim S, Moon J, et al. HLA Association With AQP4-IgG-Positive Neuromyelitis Optica Spectrum Disorder in the Korean Population. Neurol Neuroimmunol Neuroinflamm. 2025;12(3):e200366. doi:10.1212/NXI.0000000000200366 PMID: 40020215

Evolution of Chronic Lesion Tissue in Relapsing-Remitting Patients With Multiple Sclerosis: An Association With Disease Progression

复发缓解型多发性硬化患者慢性病灶组织的演变：与疾病进展的关联

In this study, we examine the long-term changes in chronic lesion tissue (CLT) among patients with relapsing-remitting MS (RRMS), focusing on its impact on clinical and radiologic disease progression indicators. This study demonstrates that, over a period of up to 5 years, patient-specific enlargement of CLT, when present, progresses at a constant rate and significantly influences brain atrophy and disease progression. In addition, the study underscores CLT as a promising biomarker for RRMS progression and suggests the feasibility of smaller, targeted clinical trials to evaluate treatments aimed at reducing chronic lesion expansion.

在这项研究中，我们考察了复发缓解型多发性硬化症（RRMS）患者慢性病灶组织（CLT）的长期变化，重点关注其对临床和影像学疾病进展指标的影响。这项研究表明，在长达5年的时间里，若存在患者特异性的CLT扩大情况，其会以恒定速率进展，并显著影响脑萎缩和疾病进展。此外，该研究强调了CLT是RRMS进展的一个有前景的生物标志物，并表明开展规模更小、更有针对性的临床试验来评估旨在减少慢性病灶扩大的治疗方法是可行的。

REF: Klistorner S, Barnett M, Parratt JDE, et al. Evolution of Chronic Lesion Tissue in Relapsing-Remitting Patients With Multiple Sclerosis: An Association With Disease Progression. Neurol Neuroimmunol Neuroinflamm. 2025;12(3):e200377. doi:10.1212/NXI.0000000000200377 PMID: 40020214

Abnormal Brain MRI in Anti-NMDA Receptor Encephalitis: Clinical and Prognostic Implications

抗 N - 甲基 - D - 天冬氨酸受体脑炎患者的脑部磁共振成像异常：临床及预后意义

Abnormal brain MRI is associated with poor outcomes in anti-N-methyl-d-aspartate receptor encephalitis (NMDARE). We aimed to characterize the lesions on brain MRI in NMDARE and to assess the clinical and prognostic associations. Brain MRI lesions in NMDARE include limbic hyperintensities and 3 patterns of extralimbic lesions, which are associated with nontypical NMDARE symptoms. Moreover, MS-like and extensive lesions, but not fluffy nor hippocampal lesions, are associated with overlapping demyelinating syndromes and poor clinical outcomes at 2 years. These findings can have practical implications on the monitoring of patients with NMDARE.

抗N - 甲基 - D - 天冬氨酸受体脑炎（NMDARE）患者脑部磁共振成像（MRI）异常与不良预后相关。我们旨在描述NMDARE患者脑部MRI病变特征，并评估其与临床情况及预后的关联。NMDARE患者脑部MRI病变包括边缘系统高信号以及3种类型的边缘系统外病变，这些病变与非典型NMDARE症状相关。此外，多发性硬化（MS）样病变和广泛病变（而非絮状病变或海马病变）与重叠脱髓鞘综合征相关，且与2年时不良临床结局相关。这些发现对NMDARE患者的监测具有实际意义。

REF: Khatib L, Pique J, Ciano-Petersen NL, et al. Abnormal Brain MRI in Anti-NMDA Receptor Encephalitis: Clinical and Prognostic Implications. Neurol Neuroimmunol Neuroinflamm. 2025;12(3):e200378. doi:10.1212/NXI.0000000000200378 PMID: 39999393

Single-Cell Transcriptomics Identifies a Prominent Role for the MIF-CD74 Axis in Myasthenia Gravis Thymus

单细胞转录组学揭示巨噬细胞迁移抑制因子（MIF）-分化簇74（CD74）轴在重症肌无力胸腺中的重要作用

Myasthenia gravis (MG) is an autoimmune disease most frequently caused by autoantibodies (auto-Abs) against the acetylcholine receptor (AChR) located at the neuromuscular junction. Thymic follicular hyperplasia is present in most of the patients with early-onset AChR-Ab+ MG (EOMG), but its cellular and molecular drivers and development remain poorly understood. Our data not only illustrate and define hyperplastic thymic niches in MG as favorable environments for pathogenic B-cell proliferation, maturation, and persistence but also suggest that the MIF-CD74 axis should be investigated for potential novel therapeutic targeting in EOMG.

重症肌无力（MG）是一种自身免疫性疾病，最常见的病因是针对位于神经肌肉接头处的乙酰胆碱受体（AChR）的自身抗体（auto - Abs）。大多数早发型AChR抗体阳性MG（EOMG）患者存在胸腺滤泡增生，但其细胞和分子驱动因素及发展过程仍不甚明了。我们的数据不仅阐明并界定了MG中增生性胸腺微环境是致病性B细胞增殖、成熟和持续存在的有利环境，还提示应研究巨噬细胞移动抑制因子（MIF） - 分化簇74（CD74）轴，以寻找EOMG潜在的新型治疗靶点。

REF: Terroba-Navajas P, Lu IN, Quast I, et al. Single-Cell Transcriptomics Identifies a Prominent Role for the MIF-CD74 Axis in Myasthenia Gravis Thymus. Neurol Neuroimmunol Neuroinflamm. 2025;12(3):e200384. doi:10.1212/NXI.0000000000200384 PMID: 40117520

Diagnostic Utility of Kappa Free Light Chain Index in Adults With Inaugural Optic Neuritis

κ游离轻链指数在初发视神经炎成人患者中的诊断价值

A simple, quick, and reproducible procedure for distinguishing multiple sclerosis (MS), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), and neuromyelitis optica spectrum disorder (NMOSD) at inaugural optic neuritis (ION) could be highly valuable in guiding early management. The K-FLC index is an accessible biomarker to guide early diagnosis in patients with ION. The probability of MOGAD in patients with ION and a K-FLC index ≥4.9 is low even in case of normal brain/spinal cord MRI.

一种在初发视神经炎（ION）时区分多发性硬化（MS）、髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD）和视神经脊髓炎谱系疾病（NMOSD）的简单、快速且可重复的方法，对指导早期治疗可能极具价值。κ-游离轻链（K-FLC）指数是一种可用于指导ION患者早期诊断的易得生物标志物。即使脑/脊髓磁共振成像（MRI）结果正常，ION患者K-FLC指数≥4.9时，患MOGAD的可能性也较低。

REF: Demortiere S, Stolowy N, Perriguey M, et al. Diagnostic Utility of Kappa Free Light Chain Index in Adults With Inaugural Optic Neuritis. Neurol Neuroimmunol Neuroinflamm. 2025;12(3):e200386. doi:10.1212/NXI.0000000000200386 PMID: 40085804

SARS-CoV-2 Vaccines and Multiple Sclerosis: An Update

严重急性呼吸综合征冠状病毒 2 型（SARS-CoV - 2）疫苗与多发性硬化症：最新进展

The highly contagious zoonosis coronavirus disease 2019 (COVID-19), caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), was declared a pandemic by the World Health Organization on March 11, 2020, and has led to a global health crisis with nearly 777 million confirmed infections and over 7 million deaths worldwide by November 10, 2024.1-3 Over time, various variants emerged, with Omicron and its sublines dominating the world over the past 3 years.4 In addition, there is increasing evidence regarding the immune response of SARS-CoV-2 vaccines, especially for people with multiple sclerosis (MS) receiving disease-modifying therapies. Hence, with this review, we aim to provide an updated overview and recommendations for clinical practice regarding MS and SARS-CoV-2 vaccines, including efficacy and safety, SARS-CoV-2 variants, vaccine hesitancy, and the immune response under treatment with respective disease-modifying therapies.

由严重急性呼吸综合征冠状病毒 2（SARS-CoV-2）引发的高传染性人畜共患疾病 2019 冠状病毒病（COVID-19），于 2020 年 3 月 11 日被世界卫生组织宣布为大流行病，并引发了一场全球健康危机。截至 2024 年 11 月 10 日，全球确诊感染病例近 7.77 亿例，死亡人数超过 700 万。1 - 3 随着时间的推移，出现了各种变异株，在过去 3 年里，奥密克戎及其亚分支在全球占据主导地位。4 此外，越来越多的证据表明了 SARS-CoV-2 疫苗的免疫反应情况，特别是对于正在接受疾病修正治疗的多发性硬化症（MS）患者。因此，通过这篇综述，我们旨在就 MS 和 SARS-CoV-2 疫苗提供最新的概述并为临床实践提出建议，内容包括疫苗的有效性和安全性、SARS-CoV-2 变异株、疫苗犹豫现象以及在接受相应疾病修正治疗时的免疫反应。

REF: Monschein T, Zrzavy T, Rommer PS, et al. SARS-CoV-2 Vaccines and Multiple Sclerosis: An Update. Neurol Neuroimmunol Neuroinflamm. 2025;12(3):e200393. doi:10.1212/NXI.0000000000200393 PMID: 40279527