Clinical and Experimental Neuroimmunology最新文献

{"title":"Therapeutic progress and future prospects for immune-mediated neuropathy","authors":"Kenichi Kaida","doi":"10.1111/cen3.12792","DOIUrl":"https://doi.org/10.1111/cen3.12792","url":null,"abstract":"","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140914593","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":"Therapeutic progress and future prospects of chronic inflammatory demyelinating polyradiculoneuropathy","authors":"Tomoko Okamoto","doi":"10.1111/cen3.12788","DOIUrl":"10.1111/cen3.12788","url":null,"abstract":"<p>Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare and heterogeneous but treatable immune-mediated neuropathy. The pathogenesis of CIDP is complex interplay of diverse immune mechanisms involving cellular and humoral pathways. The European Academy of Neurology/Peripheral Nerve Society guidelines were reissued in 2021, and the classification and diagnostic criteria were changed. Treatments include immunoglobulin, steroid, and plasmapheresis are effective, including remission induction and maintenance therapy. Maintenance treatments are often required for years, and treatment regimens require careful and regular adjustments to avoid undertreatment or overtreatment. In this review, the new guidelines, treatment recommendations based on guidelines and expert opinion, and future treatments including anti CD20 monoclonal antibody, FcRn blocker, and Cs1 inhibitor are discussed.</p>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140667578","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":"Eculizumab administration for myasthenia gravis also stabilizes thrombogenicity of catastrophic antiphospholipid syndrome","authors":"Sunao Takahashi,&nbsp;Nobuo Sanjo,&nbsp;Ryuji Koike,&nbsp;Takanori Yokota","doi":"10.1111/cen3.12790","DOIUrl":"10.1111/cen3.12790","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>The co-occurrence of myasthenia gravis and primary antiphospholipid syndrome (APS) is rare. Notably, both the diseases share common complement-mediated mechanisms.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>A 36-year-old woman, who was previously diagnosed with myasthenia gravis and APS, developed multiple embolisms, involving the brain, kidney and spleen, with severe anemia and platelet reduction. She was diagnosed as catastrophic APS, and intensive immunotherapies, including plasma exchange, high-dose corticosteroid and rituximab, were introduced. After these therapies, symptoms of both APS and myasthenia gravis worsened, consistent with elevation of immunoglobulin G anti-beta-2-glycoprotein-I antibody and anti-acetylcholine receptor antibody. We started eculizumab, which resulted in stabilizing the disease activity of both diseases without notable adverse events.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Eculizumab can be effective for controlling multiple complement-mediated pathophysiology.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140673988","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":"Radiotherapy and stereotactic radiotherapy for multifocal intracranial and intramedullary Rosai–Dorfman disease","authors":"Salvatore D'Oria,&nbsp;Martina Rossitto,&nbsp;David Giraldi,&nbsp;Vincenzo Fanelli,&nbsp;Ilaria Bonaparte,&nbsp;Maria Paola Ciliberti,&nbsp;Alba Fiorentino","doi":"10.1111/cen3.12785","DOIUrl":"10.1111/cen3.12785","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>We present a case of a 51-year-old man affected by Rosai–Dorfman disease with multiple disseminated intraparenchymal and a single spinal cord localization, presenting with dysphasia and paraparesis.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>The patient elected to receive medical steroidal therapy and two radiotherapy cycles. Steroids allowed initial regression of some lesions, while radiotherapy constituted an optimal maintenance treatment. At 6-year follow up, the patient did not develop any new neurological damage in respect to baseline.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Adjuvant therapy with radiotherapy and steroidal therapy is a valid option in multicentric Rosai–Dorfman disease patients not eligible for surgery.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140714396","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":"Expert teleconsultation involving patients and their primary neurologists for the management of multiple sclerosis in regions without specialists","authors":"Y. Miyazaki, S. Ura, Kazuhiro Horiuchi, Takeshi Matsuoka, Hideki Houzen, K. Tsuzaka, Yuichi Makino, Manami Koshida, G. Oyama, Chika Sato, R. Naganuma, I. Amino, S. Akimoto, M. Niino, N. Minami, E. Takahashi, Susumu Ota, Nobutaka Hattori, I. Yabe, S. Kikuchi","doi":"10.1111/cen3.12787","DOIUrl":"https://doi.org/10.1111/cen3.12787","url":null,"abstract":"This study aimed to describe the usefulness of our teleconsultation system for managing multiple sclerosis (MS) in regions without specialists.A cross‐sectional questionnaire survey involving 11 MS patients and their primary neurologists was carried out between May and December 2023. Real‐time video conferences were conducted between an MS specialist at a hub hospital and patients with their primary neurologists at one of the four regional core hospitals in Hokkaido, Japan. Patients and their primary neurologists completed questionnaires to evaluate the usefulness of the teleconsultation system.The patients and their primary neurologists were generally satisfied with the teleconsultations and expressed willingness to continue using the system. In particular, alleviating the burden of visiting distant MS‐specialized clinics was highly appreciated by patients. In addition, patients gave high scores for questions regarding increased satisfaction with the primary neurologists' care and the treatments they offered. The primary neurologists thought the system enhanced their knowledge of MS management. However, they did not think that the system could ease their burden for managing MS patients because of challenges in time allocation and scheduling for teleconsultation sessions.The present study suggested that our expert teleconsultation system for MS reduces the burden on patients of visiting distant MS‐specialized clinics, and enhances knowledge of MS management for the primary neurologists. It also promotes trust between patients and their primary neurologists, and taken together, these aspects could collectively lead to independent and sustainable MS management in regions without MS specialists.","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-04-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140718640","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":"Immune-mediated spastic ataxia masquerading as clinically probable multisystem atrophy in an elderly woman","authors":"Rithvik Ramesh,&nbsp;Anuhya Chadalawada,&nbsp;Pedapati Radhakrishna,&nbsp;Lakshmi Narasimhan Ranganathan,&nbsp;Philo Hazeena,&nbsp;Sundar Shanmugam,&nbsp;Deepa Avadhani","doi":"10.1111/cen3.12786","DOIUrl":"10.1111/cen3.12786","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Autoimmune neurological syndromes pose diagnostic challenges due to their resemblance to neurodegenerative conditions. Autoimmune spastic ataxia is a rare phenomenon. This case presents a 56-y-old woman with subacute-onset spastic ataxia, highlighting the complexities in diagnosis and the role of autoimmunity in such cases.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>A woman in her fifties developed progressive spastic ataxia over a year and presented to our outpatient department for evaluation. The patient exhibited clinical signs including saccadic intrusions, gaze-evoked nystagmus, mixed dysarthria, spasticity, exaggerated reflexes, and cerebellar dysfunction. Brain magnetic resonance imaging (MRI) displayed the “hot cross bun sign” and cerebral and cerebellar atrophy. Initial tests yielded minimal abnormalities, but a positive antinuclear antibody (ANA) emerged. The patient initially declined immunotherapy. Upon symptom progression, a repeat cerebrospinal fluid (CSF) analysis showed inflammatory changes and a whole-body positron emission tomography (PET) scan indicated reduced uptake in the cerebellum and brainstem. Subsequent paraneoplastic antibody testing revealed an unspecified neuronal antibody targeting capillaries and arterioles. Treatment with steroids, plasmapheresis, and azathioprine led to sustained improvement, reducing spasticity, and enabling her to walk short distances.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>This case emphasizes the diagnostic complexity of autoimmune neurological syndromes, particularly spastic ataxia. Autoimmune etiology should be considered even when neurodegenerative conditions seem likely. The presence of neuronal antibodies, inflammatory CSF, and response to immunotherapy underscores the role of autoimmunity in this case. Additionally, the “hot cross bun sign” may not always signify neurodegeneration, but can indicate immune-mediated neural damage. Recognizing autoimmune involvement early offers therapeutic possibilities and highlights the need for a comprehensive diagnostic approach in such cases.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140382558","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":"Eculizumab use throughout pregnancy in two patients with aquaporin-4-positive neuromyelitis optica spectrum disorder","authors":"Takeshi Fujimoto,&nbsp;Yasuhiro Maeda","doi":"10.1111/cen3.12784","DOIUrl":"10.1111/cen3.12784","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Patients with neuromyelitis optica spectrum disorder (NMOSD) are at an increased risk of pregnancy complications. Lack of NMOSD treatment during pregnancy is a risk factor for relapse. Here, we report two cases of pregnant women with anti-aquaporin-4 antibody-positive (AQP4+) NMOSD treated with eculizumab during pregnancy.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>Patient 1 was diagnosed with AQP4+ NMOSD 2 mo after giving birth to her first child. She was treated with tacrolimus and prednisolone, before switching to prednisolone monotherapy. Following concerns of teratogenicity associated with immunosuppressive therapy and oral steroid use, she began eculizumab treatment prior to a second pregnancy. When she became pregnant, eculizumab treatment was briefly paused while safety data were reviewed with her neurologist. A total of three doses were missed.</p>\u0000 \u0000 <p>Patient 2 was diagnosed with AQP4+ NMOSD and began prednisolone treatment. Following a relapse, tacrolimus was added to her treatment regimen. Prior to pregnancy, she began eculizumab treatment alongside prednisolone and tacrolimus and maintained this regimen throughout her pregnancy.</p>\u0000 \u0000 <p>No relapses or meningococcal infections occurred after eculizumab initiation in either patient, and both gave birth without complications to healthy babies. Patient 2 continues to receive eculizumab while breastfeeding.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>We present two cases of pregnant women with AQP4+ NMOSD treated with eculizumab. Both women gave birth to healthy babies, have had no relapses since initiating eculizumab, and continued their treatment after birth. These cases are further evidence of the successful use of eculizumab during pregnancy.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140253858","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":"Paraproteinemia-associated neuropathy with or without anti-myelin-associated glycoprotein antibody","authors":"Masanori Nakajima","doi":"10.1111/cen3.12783","DOIUrl":"10.1111/cen3.12783","url":null,"abstract":"<p>Paraproteinemia-associated neuropathy (PAN) develops with paraproteinemia and is mainly caused by the monoclonal proliferation of mature B cells, especially monoclonal gammopathy of undetermined significance (MGUS). PAN tends to increase with age, and in a super-aging society, its frequency is expected to increase. Among paraproteinemias, the immunoglobulin (Ig)G type is most common, but the frequency of PAN is reported to be higher for the IgM type. IgG/IgA-type PAN includes MGUS, plasma cell myeloma, and polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes syndrome. IgM-type PAN includes anti-myelin-associated glycoprotein antibody-associated neuropathy, chronic ataxic neuropathy with IgM antibody that recognizes disialosyl ganglioside and IgM-type PAN without anti-nerve antibodies. Light chain-type PAN includes immunoglobulin-related amyloidosis. PAN has the characteristics of a blood disease, as well as an immune-mediated disease, and is treated by immunotherapy. As an example, anti-myelin-associated glycoprotein antibody-associated neuropathy is treated with rituximab, plasmapheresis and intravenous immunoglobulin therapy, but their effectiveness has not been established. As novel treatments, lenalidomide, Bruton tyrosine kinase inhibitors, B-cell lymphoma 2 inhibitors and mimetic human natural killer-1 epitope polymers have been investigated. By analyzing the human natural killer-1-related sugar chain structure as an antigen, the selective removal of pathological antibodies might be a new therapeutic target.</p>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-02-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140428452","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":"Vasculitic neuropathy: Therapeutic progress and prospects","authors":"Michiaki Koga","doi":"10.1111/cen3.12781","DOIUrl":"10.1111/cen3.12781","url":null,"abstract":"<p>Vasculitic neuropathy has a broad range of etiologies, and roughly comprises nonsystemic vasculitic neuropathy and neuropathies accompanied by antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Recent morphological analyses of sural nerve specimens from patients with vasculitic neuropathy showed some distinct pathogeneses within vasculitic neuropathy. Several randomized, controlled studies and updated practice guidelines cover immunotherapies for AAV, whereas there is a paucity of evidence for vasculitic neuropathy, including neuropathies associated with AAV. Corticosteroids have been the mainstay of immunotherapy for vasculitic neuropathy, and cyclophosphamide is indispensable for refractory cases. Recent AAV guidelines are shifting their recommendations toward minimizing the harm caused by corticosteroids and cyclophosphamide with a reduced-dose corticosteroid regimen and the recent advent of various optional drugs, especially molecular-targeted agents. Clinicians expect the efficacy of reduced-dose corticosteroid regimens and molecular-targeted agents in treating vasculitic neuropathy to be verified, and clarification of the mechanism of vasculitic neuropathy might lead to the development of the best treatment based on the background pathogenesis of individual cases.</p>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-02-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140446816","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":"Dropped head syndrome in anti-MuSK antibody-positive myasthenia gravis with possible concurrent axial myopathy","authors":"So Okubo,&nbsp;Mitsuhiro Kainaga,&nbsp;Shin-ichi Tokushige,&nbsp;Ayumi Uchibori,&nbsp;Chizuko Oishi,&nbsp;Teruyuki Hirano,&nbsp;Yaeko Ichikawa","doi":"10.1111/cen3.12782","DOIUrl":"10.1111/cen3.12782","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Dropped head syndrome (DHS) is a group of disorders that result in anterior neck flexion. Myasthenia gravis (MG) is a common cause of DHS. Previous reports have suggested that concurrent myopathy involving the paraspinal musculature may underlie DHS in patients with anti-acetylcholine receptor antibody-positive or thymoma-associated MG.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>A 64-year-old woman presented with a 3-month history of head drop and lordosis. Neurological examination revealed bilateral ptosis and weakness of the posterior neck extensors. Electrophysiology suggested neuromuscular junction involvement. Serum anti-MuSK antibodies were positive, and generalized anti-MuSK antibody-positive myasthenia gravis (MuSK-MG) was diagnosed. Needle electromyography (nEMG) of the splenius capitus and paraspinal muscles revealed acute and chronic myogenic changes. Imaging suggested paraspinal muscle atrophy. nEMG and magnetic resonance imaging (MRI) of both extremities were normal, and autoimmune myopathy-related antibody testing was negative.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Thymoma-negative MuSK-MG, demonstrating treatment-refractory DHS, may present with concurrent axial myopathy.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139959391","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}