Clinical and Experimental Neuroimmunology最新文献

{"title":"Clinical and Radiological Advances in Autoimmune GFAP Astrocytopathy: Analysis of 387 Patients in Japan","authors":"Akio Kimura","doi":"10.1111/cen3.70007","DOIUrl":"https://doi.org/10.1111/cen3.70007","url":null,"abstract":"<div>\u0000 \u0000 <p>Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) is an autoimmune inflammatory central nervous system disease. Recent neuropathological findings indicate that GFAP-specific cluster of differentiation (CD)8⁺ T cells are likely the effectors of GFAP-A. Of 387 individuals in Japan identified as having GFAP-A, most presented with headache and/or fever followed by neurological symptoms including consciousness disturbance, urinary dysfunction, hyperreflexia, movement disorders, and papilledema. Sixteen (5.9%) of the 270 GFAP-A patients tested had coexisting antibodies. Cerebrospinal fluid (CSF) examination revealed lymphocytic pleocytosis and increased protein levels. Moreover, transiently increased CSF adenosine deaminase, decreased glucose, and positive oligoclonal band results were sometimes observed. Brain magnetic resonance imaging (MRI) occasionally showed T2-hyperintensity lesions. Linear perivascular radial gadolinium-enhancement patterns were observed and may be an imaging hallmark of GFAP-A. Spinal cord MRI sometimes exhibited T2-hyperintensity spinal cord lesions, most of which were longitudinally extensive. Most patients were treated with immunotherapies, including intravenous methylprednisolone pulse therapy with or without intravenous immunoglobulin therapy and/or plasma exchange; this was followed by oral corticosteroid therapy, which was gradually tapered. Some refractory patients received second-line immunotherapies including rituximab or cyclophosphamide. In 203 patients with follow-up ≥ 6 months, the median modified Rankin scale score at last follow-up was 1 (range: 0–6); however, 44 patients (21.7%) had scores of 3 or greater, and six patients died. The most common neurological finding at last follow-up was cognitive dysfunction, followed by urinary dysfunction; the recurrence rate was 10.5%. CSF GFAP-immunoglobulin G should be examined in patients who present with these characteristic clinical and radiological features.</p>\u0000 <p>\u0000 <b>Trial Registration:</b> Autoimmune GFAP astrocytopathy registry (UMIN: 000054387).</p>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"16 2","pages":"174-187"},"PeriodicalIF":0.0,"publicationDate":"2025-04-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143919947","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":"Unveiling the Mysteries of Rare Neuroimmunological Disorders","authors":"Yoshihisa Yamano","doi":"10.1111/cen3.70009","DOIUrl":"https://doi.org/10.1111/cen3.70009","url":null,"abstract":"","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"16 2","pages":"148"},"PeriodicalIF":0.0,"publicationDate":"2025-04-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143919928","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":"Autoimmune cerebellar ataxia: An overlooked, but treatable, neuroimmune condition","authors":"Hiroaki Yaguchi,&nbsp;Akihiko Kudo,&nbsp;Ichiro Yabe","doi":"10.1111/cen3.70004","DOIUrl":"https://doi.org/10.1111/cen3.70004","url":null,"abstract":"<p>Autoimmune cerebellar ataxia (ACA) is a condition in which the cerebellum is the primary location of inflammation due to autoimmune encephalitis caused by neuroimmune conditions. Although ACA is rare, it remains an important differential diagnosis, distinct from other neurodegenerative conditions, such as multiple system atrophy. An accurate diagnosis requires the integration of clinical history, blood tests, cerebrospinal fluid analysis, magnetic resonance imaging and malignancy screening. Over 30 neural antibodies associated with ACA have been reported as diagnostic biomarkers. The clinical profile of frequently reported antibodies, such as anti-Yo and anti-GAD, are well-defined, although that of rarer antibodies remain unclear. Cell-based assays are the standard method for detecting most neural antibodies, but testing for a wide range of antibodies is costly. Immunoblots assays and tissue-based assays are useful for screening. Further investigations into clinical profiles and advancements in screening methods are required to identify neural antibodies. ACA should not be overlooked, due to its treatable nature. Acute phase treatments, such as intravenous methylprednisolone and immunoglobulin, plasma exchange, and rituximab, are effective. Maintenance therapy using steroids and/or immunosuppressants is used to prevent relapse and progression. However, maintenance therapy requires individualized decisions due to limited clinical evidence. Additionally, treatment responses might vary depending on the type of neural antibody. In the future, the development of biomarkers and improved autoantibody testing methods is important to develop novel therapies and optimal immunotherapy for patients with ACA.</p>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"16 2","pages":"161-173"},"PeriodicalIF":0.0,"publicationDate":"2025-03-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143919543","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":"Human T-cell leukemia virus type 1-associated myelopathy/tropical spastic paraparesis and multiple sclerosis: Viral strategies and their implications","authors":"Makoto Nakashima,&nbsp;Yoshihisa Yamano","doi":"10.1111/cen3.70001","DOIUrl":"https://doi.org/10.1111/cen3.70001","url":null,"abstract":"<p>Human T-cell leukemia virus type 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a chronic inflammatory disease of the central nervous system (CNS), particularly affecting the spinal cord, and is caused by HTLV-1 infection. The biased geographic distribution of HTLV-1-infected individuals facilitated the early identification of a causal relationship between HTLV-1 infection and related diseases soon after the discovery of the virus. In contrast, such a relationship would have been challenging to establish without this geographic clustering. Similarly, Epstein–Barr virus (EBV), which infects ~95% of the global population, has recently been implicated in the pathogenesis of multiple sclerosis (MS), another chronic inflammatory CNS disease. Advances in seroepidemiology, immunology, genomics, and biomarker research in large MS cohorts have shed light on EBV's critical role in disease development. These insights provide a framework for understanding the molecular mechanisms underlying both MS and HAM/TSP, which remain incompletely elucidated. In this review, we compare HTLV-1 with EBV, focusing on their shared strategies for establishing long-term latency in the host and their roles in CNS inflammation. By analyzing the relationship between HAM/TSP and MS through virological and molecular biological perspectives, we propose that both conditions may represent chronic inflammatory diseases of the CNS triggered by viral infections. This hypothesis offers a novel perspective on the pathogenesis of these diseases. Finally, we discuss current challenges and future directions in the treatment and prevention of HAM/TSP and MS.</p>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"16 2","pages":"149-160"},"PeriodicalIF":0.0,"publicationDate":"2025-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143919604","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":"A thank you note to our reviewers","authors":"","doi":"10.1111/cen3.12832","DOIUrl":"https://doi.org/10.1111/cen3.12832","url":null,"abstract":"","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"16 1","pages":"84"},"PeriodicalIF":0.0,"publicationDate":"2025-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143455668","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":"Muscle magnetic resonance imaging findings in patients with idiopathic inflammatory myopathies","authors":"Tadanori Hamano,&nbsp;Tomoko Kamisawa,&nbsp;Sayaka Sanada,&nbsp;Kouji Hayashi","doi":"10.1111/cen3.12831","DOIUrl":"https://doi.org/10.1111/cen3.12831","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Idiopathic inflammatory myopathies (IIMs) are disorders that cause chronic muscle inflammation and weakness due to an autoimmune pathogenesis. Dermatomyositis (DM) is a typical IIM disorder, along with others including antisynthetic syndrome (ASS), immune-mediated necrotizing myopathy (IMNM), overlap myositis, inclusion body myositis (IBM), and anti-mitochondrial M2 antibody-positive myositis (AMA-myositis). Noninvasive muscle magnetic resonance imaging (MRI) is useful for determining the distribution, nature, and extent of lesions in affected muscles. T₁-weighted MRI is useful for observing morphological changes, including muscle atrophy. Short tau inversion recovery images or T₂-weighted images are useful for detecting muscle inflammation and edema and are suitable for selecting optimal biopsy sites. Muscle MRI is also useful for follow-up studies.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>On muscle MRI, patients with DM show a symmetric pattern with prominent fasciitis. The MRI findings in ASS are similar to those in DM. In IMNM, MRI findings are asymmetric, present a rapid and severe course, and fasciitis is less prominent. In IBM, atrophy is more severe at presentation than in other IIMs, and fasciitis is absent. In AMA-myositis, fasciitis is severe and atrophy is mild.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Muscle MRI can help differentiate between IIMs along with using other laboratory findings, including myositis-specific antibodies.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"16 1","pages":"72-83"},"PeriodicalIF":0.0,"publicationDate":"2025-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/cen3.12831","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143456106","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":"Interferons in myositis: Pathogenesis and therapy","authors":"Nozomu Tawara","doi":"10.1111/cen3.12827","DOIUrl":"https://doi.org/10.1111/cen3.12827","url":null,"abstract":"<p>Idiopathic inflammatory myopathies (IIMs), or myositis, are a heterogeneous group of autoimmune diseases affecting skeletal muscle and other organs. Recent research has revealed the important role of interferons (IFNs) in the pathogenesis of IIMs. This review summarizes the three types of IFNs and their functions in the immune system, focusing on their association with different IIM subtypes. Dermatomyositis (DM) is strongly associated with Type I IFNs. In contrast, inclusion body myositis (IBM), polymyositis with mitochondrial pathology (PM-mito), and anti-synthetase syndrome (ASyS) are predominantly associated with Type II IFN. This review explores the molecular mechanisms underlying these associations and their impact on muscle function. In addition, the potential of the IFN signaling pathway as a therapeutic target for IIMs will be discussed. Several clinical trials are currently underway or planned that target the IFN pathway using JAK inhibitors and monoclonal antibodies against Type I IFNs. JAK inhibitors have shown promise in treating DM, particularly in refractory cases. However, more research is needed to fully understand their efficacy and safety profiles in IIMs. The review concludes by highlighting the importance of ongoing research in this area and the potential for new targeted therapies in treating IIMs.</p>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"16 1","pages":"64-71"},"PeriodicalIF":0.0,"publicationDate":"2025-01-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143455840","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":"Unmasking the muscle mysteries: Cutting-edge insights into inflammatory myopathies","authors":"Satoshi Yamashita","doi":"10.1111/cen3.12826","DOIUrl":"https://doi.org/10.1111/cen3.12826","url":null,"abstract":"","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"16 1","pages":"42-43"},"PeriodicalIF":0.0,"publicationDate":"2025-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143455743","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":"Update on pathology of inflammatory myopathy","authors":"Akinori Uruha,&nbsp;Satoko Uruha","doi":"10.1111/cen3.12824","DOIUrl":"https://doi.org/10.1111/cen3.12824","url":null,"abstract":"<p>Inflammatory myopathy is classified into primary autoimmune myositis and secondary myositis due to various factors, such as drugs and autoimmune connective tissue diseases. Autoimmune myositis mainly consists of dermatomyositis, antisynthetase syndrome-associated myositis, immune-mediated necrotizing myopathy, and inclusion body myositis. This review aims to provide insights into muscle pathology for clinical practice and an understanding of pathophysiology in inflammatory myopathy by summarizing current knowledge about the pathology of each subform of autoimmune myositis and some secondary myositis. Dermatomyositis is associated with type I interferon upregulation. Expression of myxovirus resistance protein A (a type I interferon-induced protein) in myofibers is utilized as a sensitive diagnostic marker. Antisynthetase syndrome-associated myositis is morphologically characterized by perifascicular necrosis. A recent study suggests the presence of a characteristic immunological micromilieu suitable for plasma cells in the skeletal muscle tissue. Immune-mediated necrotizing myopathy features an active necrotic and regenerating process. In inclusion body myositis, inflammatory cellular infiltration and rimmed vacuoles reflecting autophagy disruption are observed. The lymphocytes invading myofibers are composed of a highly differentiated T-cell population, which is considered a potential therapeutic target.</p>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"16 1","pages":"55-63"},"PeriodicalIF":0.0,"publicationDate":"2025-01-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143455935","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":"Myositis-specific/associated autoantibodies as diagnostic keys and disease drivers","authors":"Satoshi Yamashita","doi":"10.1111/cen3.12819","DOIUrl":"https://doi.org/10.1111/cen3.12819","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Myositis-specific autoantibodies (MSAs) and myositis-associated autoantibodies (MAAs) have emerged as crucial biomarkers in idiopathic inflammatory myopathies (IIMs).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This review synthesizes recent research on MSAs and MAAs in various IIM subtypes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Specific autoantibodies correlate with distinct clinical manifestations and pathological features. For example, anti-MDA5 antibodies are linked to rapidly progressive interstitial lung disease, while anti-TIF1-γ antibodies are associated with increased malignancy risk in adult dermatomyositis. Animal models have demonstrated the pathogenic potential of certain antibodies, such as anti-TIF1-γ, anti-SRP, and anti-HMGCR, in inducing experimental myositis.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Understanding the roles of MSAs and MAAs is crucial for elucidating disease mechanisms, developing targeted therapies, and improving patient outcomes. Further research is needed to fully characterize their functional implications and explore their potential as biomarkers for disease activity, prognosis, and treatment response.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"16 1","pages":"44-54"},"PeriodicalIF":0.0,"publicationDate":"2024-11-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143455722","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}