Journal of neuroimmunology最新文献

{"title":"Toward an immunological classification of autism spectrum disorder: A PRISMA-ScR-compliant scoping review.","authors":"Adil Abdul-Rehman Siddiq Al-Salihy","doi":"10.1016/j.jneuroim.2026.578962","DOIUrl":"https://doi.org/10.1016/j.jneuroim.2026.578962","url":null,"abstract":"<p><p>Autism Spectrum Disorder (ASD) is a heterogeneous neurodevelopmental condition increasingly linked to disturbances in immune signaling and neuroimmune cross-talk. This PRISMA-ScR-guided scoping review synthesizes contemporary evidence to propose a structured immunological classification of ASD comprising six immune-related subtypes: immune overactivation, immune deficiency, autoimmunity-linked ASD, gut-immune axis dysregulation, post-infectious or immune-triggered onset patterns, and maternal immune activation. Each subtype is defined by characteristic neuroimmune features - including cytokine imbalances, aberrant microglial activation, altered microbiome-immune communication, and prenatal immune priming - reflecting distinct biological pathways through which immune dysfunction may influence neurodevelopment. Based on 42 mapped sources identified through a search strategy that primarily emphasized literature published between 2020 and 2025, while incorporating selected foundational earlier studies through citation chaining when necessary for conceptual and mechanistic context, and spanning human clinical and epidemiological studies, animal models, and integrative neuroimmune reviews, this synthesis identifies candidate biomarkers and immune signatures relevant to each subtype, including systemic and CNS-localized inflammation, autoantibodies, disrupted gut-immune-brain pathways, and maternal cytokine profiles. The framework also clarifies ongoing debates by distinguishing immune-mediated vulnerability and timing-dependent unmasking of susceptibility from assumptions of direct causation regarding environmental or infectious exposures. Conceptualizing ASD along immune-related subtypes provides a foundation for precision-based diagnostic and therapeutic approaches, highlighting opportunities for targeted immunomodulation, microbiome-informed interventions, and biomarker-driven stratification, thereby advancing translational efforts at the interface of immunology, neuroscience, and developmental psychopathology.</p>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"417 ","pages":"578962"},"PeriodicalIF":2.5,"publicationDate":"2026-05-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147856657","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Immuno-deficient features of thymoma-associated myasthenia gravis patients with hypogammaglobulinemia: A condition comparable to Good's syndrome","authors":"Saki Nakashima ,&nbsp;Kaori Sakuishi ,&nbsp;Manato Hara ,&nbsp;Reiko Kawasaki ,&nbsp;Toshiyuki Kakumoto ,&nbsp;Hiroyuki Ishiura ,&nbsp;Tatsushi Toda","doi":"10.1016/j.jneuroim.2026.578885","DOIUrl":"10.1016/j.jneuroim.2026.578885","url":null,"abstract":"<div><div>Good's syndrome (GS) is a rare immunodeficiency disorder associated with thymoma, characterized by hypogammaglobulinemia and recurrent infections; however, its clinical significance in thymoma-associated myasthenia gravis (TAMG) remains unclear. We retrospectively reviewed 30 patients with TAMG admitted to our center between January 2010 and March 2022. We defined GS-like immunodeficiency as serum IgG below the institutional cutoff of 861 mg/dL and a history of two or more infections requiring antimicrobial treatment; 11 patients (36.7%) met this definition. Compared with the remaining patients, the GS-like group had higher incidences of malignancy (45.5% vs. 5.3%, <em>p</em> = 0.016) and autoimmune diseases other than MG (36.4% vs. 5.3%, <em>p</em> = 0.047), lower peripheral lymphocyte counts (median 1100/μL vs. 2200/μL, <em>p</em> = 0.0051), and more frequent airflow obstruction defined by one second to forced vital capacity ratio of less than 70% (60.0% vs. 5.3%, <em>p</em> = 0.0026). Five deaths occurred in the GS-like group, and none in the other; median survival from the first antimicrobial-treated infection was 5.0 years. These findings imply that TAMG patients with GS-like immunodeficiency have a worse prognosis, underscoring the need for close monitoring and timely adjustments of MG management. (189 words).</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"414 ","pages":"Article 578885"},"PeriodicalIF":2.5,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146165826","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Possible risk of rheumatoid arthritis treated with methotrexate to the central nervous system relapse of diffuse large B-cell lymphoma","authors":"Rie Tabata ,&nbsp;Hiroko Uesugi ,&nbsp;Chiharu Tabata","doi":"10.1016/j.jneuroim.2026.578877","DOIUrl":"10.1016/j.jneuroim.2026.578877","url":null,"abstract":"<div><h3>Purpose</h3><div>Relapse in the CNS is uncommon but often a fatal event in the patients with diffuse large B-cell lymphoma. An important management strategy consists of identifying patients in the high-risk group for CNS relapse and choosing those who will benefit from CNS prophylaxis. To evaluate rheumatoid arthritis as an additional risk factor for CNS relapse, we retrospectively examined the patients with DLBCL.</div></div><div><h3>Methods</h3><div>We examined 85 patients with diffuse large B-cell lymphoma, admitted to the hospital between June 2016 and May 2021 and followed up for at least three years, and investigate the background of nine patients with CNS relapse. CNS relapse was diagnosed by imaging, clinical, and histological or cytological findings. Also, we evaluated patients with a history of rheumatoid arthritis in these 85 patients. Statistical analysis was performed using Fisher's exact test (Statcel-the Useful Addin Forms on Excel-4th ed.)</div></div><div><h3>Results</h3><div>In the present study, we showed that nine of 85 patients (10.6%) developed CNS relapse. Three of nine cases with CNS relapse had none of the previously reported risks, but received low-dose methotrexate for rheumatoid arthritis. We observed a high proportion of CNS relapse among patients with diffuse large B-cell lymphoma and concomitant rheumatoid arthritis The association between CNS relapse and concomitant rheumatoid arthritis in diffuse large B-cell lymphoma was statistically significant. (CNS relapse / history of RA: +/+ 3, +/− 6, −/+ 5, −/− 71; <em>p</em> = 0.036), using Fisher's exact test.</div></div><div><h3>Conclusions</h3><div>Here we demonstrated a possible risk of rheumatoid arthritis for CNS relapse in diffuse large B-cell lymphoma. High-dose intravenous methotrexate has been increasingly used as a CNS prophylaxis instead of intrathecal methotrexate injection alone in patients with high-risk diffuse large B-cell lymphoma. However, our findings suggest that MTX re-administration in patients with rheumatoid arthritis with diffuse large B-cell lymphoma and high CNS relapse risk warrants particular caution.</div><div>Clinicians should be aware of the possible risks in these patients, and careful consideration is needed.</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"414 ","pages":"Article 578877"},"PeriodicalIF":2.5,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146125374","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Docosahexaenoic acid supplementation aggravates myasthenia gravis through immune dysregulation","authors":"Linqi Liu ,&nbsp;Dan Lu ,&nbsp;Wenjun Que ,&nbsp;Rui Fan ,&nbsp;Wei Zheng ,&nbsp;Yaoqi Gan ,&nbsp;Fei Xiao","doi":"10.1016/j.jneuroim.2026.578889","DOIUrl":"10.1016/j.jneuroim.2026.578889","url":null,"abstract":"<div><div>Myasthenia gravis (MG) is an autoimmune disorder characterized by the disruption of immune cell homeostasis and inflammatory processes. However, the impact of metabolic abnormalities on immune regulation in MG has not been well defined. The objective of this study was to identify serum metabolites causally linked to MG and to explore their role in the onset and progression of the disease. This will provide a theoretical foundation for targeted clinical interventions and therapeutic strategies. To establish the causal relationship between serum metabolites and MG, we employed Mendelian randomization. Furthermore, we conducted dietary interventions with docosahexaenoic acid (DHA) to observe its effects on the disease progression and immune cell subpopulations in experimental autoimmune myasthenia gravis (EAMG) rats. We also performed metabolomic and transcriptomic analyses of regulatory T cells (Treg) during MG progression. Our findings suggest that dysregulated lipid metabolism, particularly elevated DHA levels, is a significant risk factor for MG, influencing various markers associated with Treg cells in both MG patients and in EAMG models. The addition of 1% DHA to the diet exacerbated the severity of EAMG, enhanced B cell immune responses, and promoted antibody production. However, it also led to an increase in the proportion of Treg cells. Further in vitro experiments confirmed that DHA accumulation in Treg cells enhances their proliferation but impairs their inhibitory function, partially through the PI3K-Akt signaling pathway. These results imply that modulating lipid metabolism, especially through the PI3K-Akt pathway in Treg cells, could be critical in controlling MG.</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"414 ","pages":"Article 578889"},"PeriodicalIF":2.5,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146172462","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Longitudinal MuSK antibody levels may correlate with disease severity in MuSK myasthenia gravis","authors":"Manato Yasuda ,&nbsp;Akiyuki Uzawa ,&nbsp;Etsuko Ogaya ,&nbsp;Hideo Handa ,&nbsp;Kentaro Kurumada ,&nbsp;Kyosuke Takasaka ,&nbsp;Hiroyuki Akamine ,&nbsp;Yukiko Ozawa ,&nbsp;Satoshi Kuwabara","doi":"10.1016/j.jneuroim.2026.578876","DOIUrl":"10.1016/j.jneuroim.2026.578876","url":null,"abstract":"<div><h3>Background</h3><div>In muscle-specific kinase antibody-positive myasthenia gravis (MuSK-MG), the clinical utility of MuSK antibody levels as a biomarker for disease severity is debated, with conflicting reports on its correlation with clinical status. This study aimed to clarify this association by applying a rigorous statistical model to a longitudinal dataset followed from an immunotherapy-naïve state.</div></div><div><h3>Methods</h3><div>We conducted a retrospective longitudinal study on 6 patients with MuSK-MG tracked from their treatment-naïve baseline. The primary analysis used 103 data points where MuSK antibody levels and MG activities of daily living (MG-ADL) scores were available. A secondary analysis used the subset of 81 data points where total immunoglobulin G (IgG) levels were also concurrently measured. Generalized linear mixed-effects models (GLMM) were used to assess the intrapatient correlation, adjusting for interpatient variability by including a random intercept for each subject.</div></div><div><h3>Results</h3><div>First, the GLMM analysis of 103 data points revealed a strong positive intrapatient correlation between MuSK antibody levels and MG-ADL scores (<em>P</em> &lt; 0.001). Second, in the subset analysis (<em>n</em> = 81), MuSK antibody levels remained positively and specifically correlated with MG-ADL scores (<em>P</em> = 0.002), whereas total IgG levels showed no independent correlation (<em>P</em> = 0.61) when included in the same model.</div></div><div><h3>Conclusions</h3><div>MuSK antibody level, unlike total IgG, is a specific and valuable biomarker for monitoring intrapatient disease activity and therapeutic response. These findings strongly support the utility of serial MuSK antibody monitoring within individual patients.</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"414 ","pages":"Article 578876"},"PeriodicalIF":2.5,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146137488","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effect of pharmacological intervention and pathogenesis discussion about rats with tic disorders","authors":"Xiaofang Liu ,&nbsp;Xiaohong Jiang ,&nbsp;Zijia Chen ,&nbsp;Xiaona Xue ,&nbsp;Sumei Wang ,&nbsp;Lvping Lin","doi":"10.1016/j.jneuroim.2026.578878","DOIUrl":"10.1016/j.jneuroim.2026.578878","url":null,"abstract":"<div><div>To investigate the pathogenesis of Tic disorders (TD), particularly their relationship with central nervous system inflammation, we conducted an animal experiment. A TD model was established for rats, and their behavior and immune cytokines and receptor in the striatum were compared between a diseased group and healthy control group of rats. After receiving effective drug interventions (the traditional Chinese medicine Jian-Pi-Zhi-Dong decoction (JPZDD) and the Western medicine Tiapride), we observed changes in behavior and immune cytokine and receptor expression in the striatum and analyzed the association between central nervous system inflammation and TD. The results are as follows: (1) Successful modeling: Compared to normal rats, TD diseased rats exhibited increased spontaneous activity, stereotypical exercise, and elevated expression of inflammatory cytokines and receptor in the striatum. (2) Effective pharmacological intervention: Tiapride and JPZDD reduced spontaneous activity, stereotypical exercise, and the expression of inflammatory cytokines and receptor in rats with TD. (3) The number of spontaneous activities and stereotypical exercise scores was positively correlated with central nervous system inflammation. The expression of Toll-like receptor 4 (TLR4), interleukin-6 (IL-6), interleukin-8 (IL-8), and tumor necrosis factor-alpha (TNF-α) in the striatum of the diseased group rats were significantly greater than those in the striatum of the normal group rats. Effective pharmacological intervention reduced the expression of inflammatory cytokine and receptor in the striatum, bringing them to expression similar to those in normal rats. Based on these results, we conclude that TD is associated with central nervous system inflammation and that the severity of TD is positively correlated with the severity of central nervous system inflammation. We hypothesize that the pathogenesis of TD may involve elevated TLR4, which triggers overactivation of microglia in the brain resulting in the release of excessive IL-6, IL-8, and TNF-α. This process damages neurons and leads to tic symptoms in patients.</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"414 ","pages":"Article 578878"},"PeriodicalIF":2.5,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146172391","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Acanthamoeba rhombencephalomyelitis: Novel white matter tract-based neuroinvasive pattern in a retrospective observational study from India.","authors":"Remya Prakash, Aravind Reghukumar, K Rammohan, V L Abin, Abhishek Vijayan, M S Sharmad, Neetha Thayil Ramankutty, Jaichand Johnson, Swapna R Nath, Saritha Narayanankutty, Deepthi Somarajan, Megha Sayikumar, Athira Vijayan Remadevi, Alby Maria Mathews, Fida Ali Kalathil, Ananthu Nair, K R Aravind Sreeraj","doi":"10.1016/j.jneuroim.2026.578956","DOIUrl":"https://doi.org/10.1016/j.jneuroim.2026.578956","url":null,"abstract":"<p><strong>Objective: </strong>To describe a distinct rhombencephalomyelitis pattern in patients with central nervous system infection due to free-living amoebae, differing from the classical granulomatous amoebic encephalitis phenotype.</p><p><strong>Methods: </strong>This retrospective observational study included twelve consecutive patients with brainstem and/or spinal cord involvement associated with central nervous system infection due to Acanthamoeba species at a tertiary care centre in Kerala, India. Diagnosis was confirmed by wet mount examination and amoeba species-specific 18S rRNA PCR of cerebrospinal fluid. Comprehensive evaluation excluded bacterial, mycobacterial, fungal, and autoimmune etiologies.</p><p><strong>Results: </strong>Twelve patients (seven men; median age 50 years; IQR: 33-60 years) presented with subacute cranial neuropathies (58.3%) and/or gait disturbance (91.7%). Seven (58.3%) were immunocompetent; only two had traditional risk factors (ritualistic nasal ablution). MR scan demonstrated involvement of the spinal cord (66.7%), pons (58.3%), middle cerebellar peduncle (50%), and medulla (41.7%). This distinctive neuroimaging pattern reflected white matter tract-based spread along cerebellar peduncles and/or corticospinal tracts. CSF analysis revealed lymphocytic pleocytosis (median: 12 cells/mm³, IQR: 5-47). with hypoglycorrhachia (CSF-to-blood glucose ratio 0.45 IQR: 0.4-0.5). Acanthamoeba spp. were confirmed by CSF PCR in ten (83.3%) and presumptively by wet mount examination in two (16.7%). Nine patients (75.0%) survived, of whom eight (66.7%) achieved favourable functional outcome (mRS 1-2).</p><p><strong>Conclusion: </strong>Acanthamoeba spp. causes distinct white matter tract-based non-granulomatous rhombencephalomyelitis. Neurologists should screen for free-living amoebae CNS infection even without traditional risk factors, as this pattern may mimic demyelinating disorders (NMOSD, MOGAD, ADEM, or MS). When identified early, Acanthamoeba rhombencephalomyelitis carries a favourable prognosis.</p>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"417 ","pages":"578956"},"PeriodicalIF":2.5,"publicationDate":"2026-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147839279","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Brain tumor classification using hybrid spinal-EfficientNet using MRI images.","authors":"Ponlatha Sambandham, Someswari Perla, Ramachandro Majji, Parul Datta","doi":"10.1016/j.jneuroim.2026.578948","DOIUrl":"https://doi.org/10.1016/j.jneuroim.2026.578948","url":null,"abstract":"<p><p>Traditional approaches to brain tumor classification frequently encounter issues such as limited efficiency, class imbalance, and high computational time, which can hinder timely clinical decision-making. To address these challenges, a new hybrid framework named Spinal-EfficientNet is introduced, aiming to enhance both classification accuracy and processing speed. The proposed pipeline starts with Magnetic Resonance Imaging (MRI) brain scans obtained from a curated dataset, followed by a preprocessing stage where noise and artifacts are reduced and image quality is improved using wavelet-domain filtering techniques. Tumor segmentation is then performed using SegNet, followed by image augmentation techniques including random erasing, rotation, and shearing to strengthen model generalization. Next, significant features are extracted, encompassing texture descriptors like Angular Second Moment (ASM), contrast, sum entropy, maximal correlation coefficient, Pyramid Histogram of Oriented Gradients (PHOG), Complete Local Binary Pattern (CLBP) and statistical measures such as mean, variance, kurtosis, skewness. In the final stage, tumor classification is performed using Spinal-EfficientNet, a hybrid architecture that combines EfficientNet with SpinalNet via customized layer modifications, allowing more reliable and accurate identification of brain tumors. Experimental evaluation demonstrates that the proposed model achieves strong performance, with specificity of 92.5%, sensitivity of 92.9%, accuracy of 92.5%, and an F1-score of 91.6% under k-fold cross-validation on the BRATS 2018 dataset. The Spinal-EfficientNet framework demonstrates notable gains in accuracy when compared with existing methods. In performance comparisons, it achieves improvements of 6.05% over Convolutional Neural Network and Support Vector Machine (CNN-SVM), 4.76% over Visual Geometry Group Stacked Classifier Network (VGG-SCNet), 3.46% over Ultra-Light Brain Tumor Detection (UL-BTD), 2.92% over Adaptive Fuzzy Deep Neural Network (AFDNN), 2.70% over ResNet50 with the Enhanced Watershed Segmentation (ResNet50-EWS), 2.59% over EfficientNet-B0, and 1.41% over SpinalNet. These consistent enhancements across a range of architectures indicate its reliability and effectiveness as a strong approach for handling complex classification problems.</p>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"417 ","pages":"578948"},"PeriodicalIF":2.5,"publicationDate":"2026-04-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147839251","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Bilateral optic neuritis preceding a Baló concentric sclerosis lesion: A case report and literature review","authors":"David F. Alfonso-Cedeño ,&nbsp;Lorena Medina-Lozano ,&nbsp;Paula V. Gaete ,&nbsp;Patricia Quintero-Cusguen","doi":"10.1016/j.jneuroim.2026.578874","DOIUrl":"10.1016/j.jneuroim.2026.578874","url":null,"abstract":"<div><div>Baló's concentric sclerosis (BCS) is a rare inflammatory demyelinating disorder characterised by a pathologic appearance of concentric layers of demyelinated and partially myelinated fibres, with a distinctive “onion bulb” pattern on magnetic resonance imaging (MRI). Due to its rarity and overlapping features with multiple sclerosis and neuromyelitis optica spectrum disorder (NMOSD), diagnosis is challenging. We report the case of a 60-year-old male with hypertension who presented with acute bilateral painless vision loss, absent pupillary light reflex, and marked photophobia. Initial brain and orbital MRI were normal, but cervical spine MRI revealed a demyelinating lesion from C5 to C6. Cerebrospinal fluid (CSF) showed type 1 oligoclonal bands and negative anti-aquaporin 4 and anti-myelin oligodendrocyte glycoprotein antibodies. The patient was initially diagnosed with seronegative NMOSD and treated with intravenous methylprednisolone without improvement. One month later, he developed spastic dysarthria and dysphagia; brain MRI showed a right pre-Rolandic lesion with concentric rings associated with the previously reported demyelinated lesion in the cervical spinal cord and other demyelinated lesions in the brainstem. CSF analysis during relapse revealed hyperproteinorrhachia and mild hypoglycorrhachia without infectious or autoimmune markers. Plasmapheresis led to the resolution of bulbar symptoms. Given clinical relapse and lesion progression, rituximab therapy was initiated, achieving clinical stability. This case illustrates the diagnostic complexity of BCS, the importance of integrating imaging and immunological findings, and the potential role of B-cell–depleting therapy in preventing new relapses.</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"413 ","pages":"Article 578874"},"PeriodicalIF":2.5,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146079053","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Efgartigimod for steroid-resistant autoimmune glial fibrillary acidic protein astrocytopathy: a case report and literature review","authors":"Peng Lei ,&nbsp;Yun Lu ,&nbsp;Jie Xia ,&nbsp;Tao Wang ,&nbsp;Ling Zhong","doi":"10.1016/j.jneuroim.2026.578866","DOIUrl":"10.1016/j.jneuroim.2026.578866","url":null,"abstract":"<div><h3>Background</h3><div>Neonatal Fc receptor (FcRn) inhibitors rapidly and specifically clear serum immunoglobulin G (IgG) levels and, therefore, are increasingly used for the treatment of neurological autoimmune diseases, such as myasthenia gravis. However, whether FcRn inhibitors could alleviate steroid-unresponsive glial fibrillary acidic protein astrocytopathy (GFAP-A) has not been reported.</div></div><div><h3>Case presentation</h3><div>We report a case of a 68-year-old male patient who presented with gait instability and numbness in the left hand. Cranial magnetic resonance imaging (MRI) revealed multiple demyelinating lesions. The initial clinical diagnosis was demyelinating encephalopathy. After 1 month of intravenous methylprednisolone pulse therapy, followed by sequential oral prednisone, the patient's gait instability did not improve. Upon re-examination at our hospital, cranial MRI revealed no significant changes in the lesions. Testing for central nervous system demyelinating antibodies revealed serum anti-GFAP IgG antibody (titer 1:100), cerebrospinal fluid anti-GFAP IgG antibody (titer 1:1), and negative result for oligoclonal bands in blood and the cerebrospinal fluid. The final diagnosis was GFAP-A. Treatment with the FcRn inhibitor efgartigimod significantly improved clinical symptoms and brain lesions.</div></div><div><h3>Conclusions</h3><div>This rare case indicates that efgartigimod is a promising treatment option for steroid-unresponsive GFAP-A.</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"413 ","pages":"Article 578866"},"PeriodicalIF":2.5,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146079050","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}