Clinical and Experimental Neuroimmunology最新文献

{"title":"Successful Treatment of Refractory Myelin Oligodendrocyte Glycoprotein Antibody–Associated Disease With Tocilizumab: A Case Report and Literature Review","authors":"Takahiko Katsuyama,&nbsp;Ryohei Horiguchi,&nbsp;Yuki Matsunaga,&nbsp;Akihiro Nishimachi,&nbsp;Keisuke Morihara,&nbsp;Hideyuki Takeuchi","doi":"10.1111/cen3.70040","DOIUrl":"https://doi.org/10.1111/cen3.70040","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Approximately half of patients with myelin oligodendrocyte glycoprotein (MOG) antibody–associated disease (MOGAD) experience relapses, and optimal relapse-prevention strategies remain under discussion. The interleukin-6 (IL-6) signaling pathway plays a pivotal role in MOGAD pathogenesis, and its inhibition has been proposed as a promising therapeutic approach.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>We describe an 18-year-old male with refractory MOGAD characterized by multiple MRI-detected lesions showing an acute disseminated encephalomyelitis (ADEM)-like pattern, whose condition dramatically improved with tocilizumab. His symptoms included fever, headache, nausea, paresthesia, moria, somnolence, and seizure. Despite multiple courses of intravenous methylprednisolone, plasma exchange, and intravenous immunoglobulin, his condition remained uncontrolled. Weekly subcutaneous administration of tocilizumab, a monoclonal antibody against the IL-6 receptor, resulted in marked clinical and radiological improvement and sustained disease stabilization.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>This case highlights the potential of IL-6 receptor inhibition with tocilizumab as an effective treatment strategy for refractory MOGAD.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"17 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-12-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145695350","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}

{"title":"Assessing the Benefits of “Circle”: A Patient Support Program for Neuromyelitis Optica Spectrum Disorder for Patients Receiving Satralizumab Treatment","authors":"Kazuo Fujihara,&nbsp;Chiaki Sugita,&nbsp;Katsuhisa Yamashita,&nbsp;Yuta Kamei","doi":"10.1111/cen3.70037","DOIUrl":"https://doi.org/10.1111/cen3.70037","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>Although satralizumab, an interleukin-6 receptor inhibitor, effectively prevents relapses in neuromyelitis optica spectrum disorder (NMOSD), infections are reported to be the most common adverse reactions. To support Japanese patients receiving satralizumab, “Circle”—a 1-year patient support program—was developed. “Circle” provides educational materials, including information on NMOSD, satralizumab treatment, and potential infections occurring during treatment, thereby promoting the early detection and prevention of infection. We assessed the impact of the “Circle” program through a questionnaire-based patient survey.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Patients receiving satralizumab treatment who had completed 1 year in the program were invited to participate via mailed postcards between May 2023 and August 2024.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Of the 657 patients who had completed 1 year post-enrollment and received the questionnaire, 236 (35.9%) responded. Among the responders, 95.3% continued satralizumab treatment. Overall, 68.6% of responders felt more positive about their NMOSD treatment, and 88.6% reported increased infection awareness. Among 209 responders who reported increased infection awareness, 76.6% became more conscious of infection control measures (e.g., handwashing and gargling), 54.1% started using a pulse oximeter for the early detection of respiratory tract infections, 50.2% started consulting their physician or nurse about physical changes, and 28.7% started daily monitoring of their body temperature.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>The “Circle” program enhanced patient knowledge and improved infection control behaviors, potentially ensuring adherence to satralizumab treatment in patients with NMOSD. This initiative may play a crucial role in future treatment support.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"17 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/cen3.70037","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145751241","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Unveiling Oversights in Hypothermia's Neuroprotective Mechanisms","authors":"Mona Hassan Ali Al Farhan,&nbsp;Javed Iqbal,&nbsp;Manal Chihab Al Deri","doi":"10.1111/cen3.70039","DOIUrl":"https://doi.org/10.1111/cen3.70039","url":null,"abstract":"","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"17 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-11-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145751100","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}

{"title":"Response to: Unveiling Oversights in Hypothermia's Neuroprotective Mechanisms","authors":"Tomohiro Matsui","doi":"10.1111/cen3.70022","DOIUrl":"https://doi.org/10.1111/cen3.70022","url":null,"abstract":"","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"17 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-11-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145751195","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}

{"title":"Response to: Not All Patients Start From the Same Line: Rethinking Outcome Interpretation in Myasthenia Gravis","authors":"Akiyuki Uzawa,&nbsp;Ema Rodrigues,&nbsp;Hiroyuki Murai","doi":"10.1111/cen3.70038","DOIUrl":"https://doi.org/10.1111/cen3.70038","url":null,"abstract":"","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"17 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-11-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145750996","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}

{"title":"Reply to “Reply to Seroconversion After SARS-CoV-2 Vaccination in B-Cell-Depleted Patients: The Missing Factors”","authors":"Christian Messina","doi":"10.1111/cen3.70036","DOIUrl":"https://doi.org/10.1111/cen3.70036","url":null,"abstract":"","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"17 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145750642","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}

{"title":"An Adult Case of Severe, Refractory, and Progressive Leukodystrophy-Like Diffuse Encephalitis With MOG-IgG Treated With Rituximab","authors":"Yuki Matsumoto,&nbsp;Hirohiko Ono,&nbsp;Tatsuro Misu,&nbsp;Yoshiki Takai,&nbsp;Shuhei Nishiyama,&nbsp;Toshiyuki Takahashi,&nbsp;Kazuhiro Kurosawa,&nbsp;Masashi Aoki","doi":"10.1111/cen3.70035","DOIUrl":"https://doi.org/10.1111/cen3.70035","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>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).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>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.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>We report a severe refractory case of MOGAD that responded only to RTX. Early RTX treatment should be considered for patients with severe MOGAD.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"17 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-11-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145750445","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}

{"title":"Heterogeneity of Ocular Myasthenia Gravis: Potential Subgroups","authors":"Tomoko Narita,&nbsp;Akiko Nagaishi,&nbsp;Shunya Nakane,&nbsp;Hidenori Matsuo","doi":"10.1111/cen3.70034","DOIUrl":"https://doi.org/10.1111/cen3.70034","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>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.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>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.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>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.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>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.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"17 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-10-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145751340","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}

{"title":"Recurrent Asystole Without Generalized Dysautonomia in Anti-NMDA Receptor Encephalitis: A Case Report","authors":"Tetsuya Oyama,&nbsp;Hirotaka Nakanishi","doi":"10.1111/cen3.70033","DOIUrl":"https://doi.org/10.1111/cen3.70033","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>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.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>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.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>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.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"17 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145751366","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}

{"title":"The Japan Neuromyelitis Optica Spectrum Disorders/Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease Registry: Background Analysis of the Initial 116 Cases","authors":"Yusei Miyazaki,&nbsp;Hideyuki Takeuchi,&nbsp;Takao Kiriyama,&nbsp;Hikoaki Fukaura,&nbsp;Juichi Fujimori,&nbsp;Katsuhisa Masaki,&nbsp;Sonoko Misawa,&nbsp;Katsuichi Miyamoto,&nbsp;Nobuaki Yoshikura,&nbsp;Shoko Ukita,&nbsp;Kengo Nagashima,&nbsp;Ichiro Nakashima","doi":"10.1111/cen3.70032","DOIUrl":"https://doi.org/10.1111/cen3.70032","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>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.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>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.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>A total of 116 patients (NMOSD, <i>n</i> = 110; MOGAD, <i>n</i> = 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.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>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.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"17 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145750923","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}