Clinical and Experimental Neuroimmunology最新文献

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Myelin oligodendrocyte glycoprotein antibody-associated disorders: An overview 髓鞘少突胶质细胞糖蛋白抗体相关疾病:概述
Clinical and Experimental Neuroimmunology Pub Date : 2023-12-19 DOI: 10.1111/cen3.12771
Tatsuro Misu, Yuki Matsumoto, Kimihiko Kaneko, Toshiyuki Takahashi, Yoshiki Takai, Hirohiko Ono, Chihiro Namatame, Shuhei Nishiyama, Juichi Fujimori, Hiroshi Kuroda, Ichiro Nakashima, Kazuo Fujihara, Masashi Aoki
{"title":"Myelin oligodendrocyte glycoprotein antibody-associated disorders: An overview","authors":"Tatsuro Misu,&nbsp;Yuki Matsumoto,&nbsp;Kimihiko Kaneko,&nbsp;Toshiyuki Takahashi,&nbsp;Yoshiki Takai,&nbsp;Hirohiko Ono,&nbsp;Chihiro Namatame,&nbsp;Shuhei Nishiyama,&nbsp;Juichi Fujimori,&nbsp;Hiroshi Kuroda,&nbsp;Ichiro Nakashima,&nbsp;Kazuo Fujihara,&nbsp;Masashi Aoki","doi":"10.1111/cen3.12771","DOIUrl":"10.1111/cen3.12771","url":null,"abstract":"<p>In recent years, there is growing evidence of associations between antibodies against myelin oligodendrocyte glycoprotein (MOG) and several phenotypes of acute inflammatory demyelinating diseases such as acute disseminated encephalomyelitis (ADEM), optic neuritis, brainstem, and cerebral cortical encephalitis, called MOG antibody associated disorders (MOGAD). Monophasic course is known in about half of cases especially in pediatric onset ADEM and optic neuritis, mainly in cases with transient positivity of MOG antibody. Pathological features of MOGAD are considered as acute demyelinating lesions with CD4 dominant cell infiltrations, the deposition of humoral immunity, perivascular inflammation and perivenous demyelination, which is distinct from multiple sclerosis. Now the diagnosis of MOGAD is based on the international panel criteria of MOGAD launched in 2023, which the diagnostic frameworks are three parts, including MOGAD-specific clinical features, MOG antibody positivity, and the exclusion of other diseases. The prognosis of MOGAD patients is considered relatively mild, but the problem is refractory relapsing cases. For its prevention, there are no approved drugs, but oral tapering corticosteroids, immunosuppressants such as azathioprine and mycophenolic mofetil, rituximab, and the maintenance intravenous immunoglobulin are recommended, and now there are a few clinical trials of promising biological drugs already approved in other neurological disorders.</p>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"15 1","pages":"6-15"},"PeriodicalIF":0.0,"publicationDate":"2023-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/cen3.12771","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138959762","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}
引用次数: 0
Holmes tremor in progressive multifocal leukoencephalopathy: A video case report 进行性多灶性白质脑病中的霍姆斯震颤:视频病例报告
Clinical and Experimental Neuroimmunology Pub Date : 2023-11-27 DOI: 10.1111/cen3.12775
Takako Matsushima, Ryotaro Ikeguchi, Mutsumi Iijima, Ayato Shimomura, Shuntaro Takahashi, Kazuo Nakamichi, Yuko Shimizu, Kazuo Kitagawa
{"title":"Holmes tremor in progressive multifocal leukoencephalopathy: A video case report","authors":"Takako Matsushima,&nbsp;Ryotaro Ikeguchi,&nbsp;Mutsumi Iijima,&nbsp;Ayato Shimomura,&nbsp;Shuntaro Takahashi,&nbsp;Kazuo Nakamichi,&nbsp;Yuko Shimizu,&nbsp;Kazuo Kitagawa","doi":"10.1111/cen3.12775","DOIUrl":"10.1111/cen3.12775","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease of the central nervous system caused by the John Cunningham virus. Various brain regions are affected by PML. Therefore, patients with PML show various neurological symptoms. However, tremors are rare neurological symptoms of PML.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>A 49-year-old man developed intermittent slow tremor in the left hand, bilateral dysesthesia and gait disturbance. Brain magnetic resonance imaging showed hyperintense lesions in the right parietofrontal lobe, right thalamus, left middle cerebellar peduncle, left dentate nucleus, pons and medulla oblongata on fluid-attenuated inversion recovery images. The patient was positive for HIV antibodies. In addition, HIV-1 RNA was increased. Quantitative real-time polymerase chain reaction identified John Cunningham virus DNA in the cerebrospinal fluid; HIV-associated PML was diagnosed. Surface electromyography showed 3-Hz grouped discharges in the left flexor carpi ulnaris and extensor carpi radialis, which were consistent with Holmes tremor (HT). Although we administered antiretroviral therapy and mirtazapine, the neurological and radiological findings progressively worsened, and the patient died on day 90. Including the present case, there have been 10 reported cases of PML with HT.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Although tremors are rarely observed in PML, HT might be a common tremor phenotype in patients with PML. If the neurologist observes HT in patients with multiple brain lesions, PML should be considered.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"15 2","pages":"101-104"},"PeriodicalIF":0.0,"publicationDate":"2023-11-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139230789","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}
引用次数: 0
Performance evaluation of the EUROIMMUN anti-acetylcholine receptor enzyme-linked immunoassay EUROIMMUN 抗乙酰胆碱受体酶联免疫测定的性能评估
Clinical and Experimental Neuroimmunology Pub Date : 2023-11-20 DOI: 10.1111/cen3.12774
Naoki Kawaguchi, Asami Imafuku, Satoshi Fujii, Ji-Hoon Sohn
{"title":"Performance evaluation of the EUROIMMUN anti-acetylcholine receptor enzyme-linked immunoassay","authors":"Naoki Kawaguchi,&nbsp;Asami Imafuku,&nbsp;Satoshi Fujii,&nbsp;Ji-Hoon Sohn","doi":"10.1111/cen3.12774","DOIUrl":"10.1111/cen3.12774","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>Anti-acetylcholine receptor antibodies (AChR Ab) are one of the pathogenic autoantibodies in myasthenia gravis (MG), and they are specifically mentioned in the Japanese clinical guidelines for MG. In the serological diagnosis and measurement of autoantibodies as an adjunct marker for MG, the radioimmunoassay (RIA) method has been conventionally used as the gold standard. However, there have been several worldwide concerns regarding the use of radioisotopes. In this study, we evaluated the performance of the EUROIMMUN AChR Ab enzyme-linked immunosorbent assay (ELISA) in comparison with RIA.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>ELISA reproducibility and dilution linearity were analyzed using AChR Ab-positive samples. Sera from 50 patients with suspected MG and 50 healthy donors were used to assess the correlation and qualitative agreement between the two methods.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The ELISA showed good interassay precision and dilution linearity. The correlation coefficient, sensitivity, specificity and overall agreement of the ELISA in comparison with the conventional RIA amounted to 0.87, 98%, 92% and 95% (kappa = 0.895), respectively. Analysis of the discordant results suggested that the ELISA might be more capable of detecting low AChR Ab reactivity in patients with suspected MG.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>The AchR Ab ELISA provides a reliable tool for the quantification of AchR Ab, supporting the diagnosis of MG. As a non-isotopic assay with good precision, shorter handling time and high correlation with the conventional RIA, the AChR ELISA could be a useful alternative in the laboratory routine.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"15 2","pages":"109-115"},"PeriodicalIF":0.0,"publicationDate":"2023-11-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139257810","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}
引用次数: 0
Acute-onset distal dominant neuropathy after severe acute respiratory syndrome coronavirus 2 vaccination in a male patient with recent dengue infection: A case report 一名近期感染登革热的男性患者接种严重急性呼吸系统综合征冠状病毒 2 疫苗后突发远端支配性神经病:病例报告
Clinical and Experimental Neuroimmunology Pub Date : 2023-11-08 DOI: 10.1111/cen3.12773
Mario B. Prado Jr, Karen Joy B. Adiao
{"title":"Acute-onset distal dominant neuropathy after severe acute respiratory syndrome coronavirus 2 vaccination in a male patient with recent dengue infection: A case report","authors":"Mario B. Prado Jr,&nbsp;Karen Joy B. Adiao","doi":"10.1111/cen3.12773","DOIUrl":"10.1111/cen3.12773","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Guillain–Barré Syndrome (GBS) is an autoimmune degenerative disease commonly presenting with acute progressive sensorimotor paralysis, sometimes associated with dysautonomia, facial diplegia and severe respiratory distress. Gastroenteritis due to <i>Campylobacter jejuni</i> and respiratory infection secondary to Epstein–Barr virus usually precede GBS; however, vaccination or recent dengue infection as temporal causes are rarely reported.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>Here, we present a 20-year-old man, who complained of a 5-day history of a progressively worsening tingling sensation isolated in the hands and feet, and unilateral Bell's palsy on the left side of his face, which occurred 2 weeks after his severe acute respiratory syndrome coronavirus 2 vaccination and 6 weeks from his recent hospitalization from Dengue fever. Except for the left complete unilateral facial paralysis, weakness in the intrinsic hands and feet muscles, and sensory ataxia, the rest of his physical examination was unremarkable. On work-up, the findings of albuminocytological dissociation and distal sensorimotor demyelinating polyneuropathy in the nerve conduction study supported the diagnosis of GBS.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>The enhanced immune response from a recent dengue infection and severe acute respiratory syndrome coronavirus 2 vaccination might increase the risk of GBS. The predominantly distal GBS phenotype has rarely been reported in the literature, adding to the peculiarity of this case.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"15 2","pages":"105-108"},"PeriodicalIF":0.0,"publicationDate":"2023-11-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135392756","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}
引用次数: 0
Expanding clinical spectrum from Hashimoto's encephalopathy to anti-NAE antibody-associated disorders (NAEAD) 临床范围从桥本脑病扩展到抗NAE抗体相关疾病(NAEAD)
Clinical and Experimental Neuroimmunology Pub Date : 2023-11-02 DOI: 10.1111/cen3.12772
Akiko Matsunaga, Masamichi Ikawa, Makoto Yoneda
{"title":"Expanding clinical spectrum from Hashimoto's encephalopathy to anti-NAE antibody-associated disorders (NAEAD)","authors":"Akiko Matsunaga,&nbsp;Masamichi Ikawa,&nbsp;Makoto Yoneda","doi":"10.1111/cen3.12772","DOIUrl":"10.1111/cen3.12772","url":null,"abstract":"<p>Using proteomic analysis, we identified anti-NAE antibodies (autoantibodies against <span>N</span>H<sub>2</sub>-terminal of <span>a</span>lpha-<span>e</span>nolase) as a diagnostic marker for Hashimoto's encephalopathy (HE). As cases of HE with serum anti-NAE antibodies accumulated, the clinical spectrum of HE with anti-NAE antibodies became expanded. In addition, it is now known that antibodies can be detected in other diseases that differ from conventional HE. We recently reported the detection of anti-NAE antibodies in patients with a clinical diagnosis of multiple system atrophy or corticobasal syndrome. These findings suggest that anti-NAE antibodies indicate an immune mechanism in the pathogenesis of neurodegenerative diseases. We propose a novel disease concept for anti-NAE antibody-associated disorders (NAEAD). The clinical spectrum of NAEAD is not limited to HE but is a broad spectrum that partially shares several autoimmune neurological diseases, including autoimmune acute encephalopathy, autoimmune cerebellar ataxia, and autoimmune psychosis, and even extends to immune-associated neurodegenerative diseases.</p>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"15 1","pages":"24-31"},"PeriodicalIF":0.0,"publicationDate":"2023-11-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/cen3.12772","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135974396","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}
引用次数: 0
What might the COVID-19 pandemic in regard to people with neuromyelitis optica spectrum disorder teach us regarding the future? COVID-19 大流行对神经脊髓炎视网膜频谱障碍患者的未来有何启示?
Clinical and Experimental Neuroimmunology Pub Date : 2023-10-08 DOI: 10.1111/cen3.12769
Cavid Baba, Ipek Yavas, Ulvi Samadzade, Asiye Tuba Ozdogar, Serkan Ozakbas
{"title":"What might the COVID-19 pandemic in regard to people with neuromyelitis optica spectrum disorder teach us regarding the future?","authors":"Cavid Baba,&nbsp;Ipek Yavas,&nbsp;Ulvi Samadzade,&nbsp;Asiye Tuba Ozdogar,&nbsp;Serkan Ozakbas","doi":"10.1111/cen3.12769","DOIUrl":"10.1111/cen3.12769","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>The study aimed to examine the demographic, clinical and therapeutic characteristics of people with neuromyelitis optica spectrum disorders (NMOSD, pwNMOSD) during the coronavirus disease 2019 (COVID-19) pandemic.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This was an observational study of pwNMOSD at a tertiary care clinic. Data on COVID-19 infection were collected between 11 March 2020 and 30 April 2022. Data on COVID-19 symptoms, severity and death rate were analyzed.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We observed 16 confirmed COVID-19 cases and three suspected cases. Three (15.8%) patients had severe infections, whereas 16 (84.2%) had mild conditions. Only one person was admitted to the hospital due to pneumonia. COVID-19 re-infection was reported by three patients. No pwNMOSD died as a result of COVID-19 disease. Cough was the most frequently reported symptom. The incidence of COVID-19 infection in our cohort was 21.1%. Immunosuppressants were taken by 57.9% of pwNMOSD with COVID-19, and by 84.5% of pwNMOSD without COVID-19.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Due to the small sample size, there was no predominant difference in infection between infected and uninfected patients with or without immunosuppressant drugs. However, given that immunosuppressants are a risk for infection, patient decision-making in their selection is important.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"15 1","pages":"40-44"},"PeriodicalIF":0.0,"publicationDate":"2023-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135197758","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}
引用次数: 0
Anti-aquaporin-4 immunoglobulin G/anti-myelin oligodendrocyte glycoprotein immunoglobulin G double-positive paraneoplastic neurological syndrome in a patient with triple-negative breast cancer 一名三阴性乳腺癌患者的抗水杨酸-4 免疫球蛋白 G/抗髓鞘少突胶质细胞糖蛋白免疫球蛋白 G 双阳性副肿瘤性神经综合征
Clinical and Experimental Neuroimmunology Pub Date : 2023-09-18 DOI: 10.1111/cen3.12767
Amna Siddiqui, Dylan Ross, Ronak H. Jani, Vikram C. Prabhu, Shelly Lo, Derek A. Wainwright, Stasia Rouse, Tamer Refaat, Yirong Zhu, Jigisha P. Thakkar
{"title":"Anti-aquaporin-4 immunoglobulin G/anti-myelin oligodendrocyte glycoprotein immunoglobulin G double-positive paraneoplastic neurological syndrome in a patient with triple-negative breast cancer","authors":"Amna Siddiqui,&nbsp;Dylan Ross,&nbsp;Ronak H. Jani,&nbsp;Vikram C. Prabhu,&nbsp;Shelly Lo,&nbsp;Derek A. Wainwright,&nbsp;Stasia Rouse,&nbsp;Tamer Refaat,&nbsp;Yirong Zhu,&nbsp;Jigisha P. Thakkar","doi":"10.1111/cen3.12767","DOIUrl":"10.1111/cen3.12767","url":null,"abstract":"<p>We report a rare case of paraneoplastic neurological syndrome with dual seropositivity of anti-aquaporin-4 and myelin oligodendrocyte glycoprotein antibodies in a 40 year-old woman with metastatic triple-negative breast cancer. She received multiple lines of anti-neoplastic treatment, including immunotherapy with pembrolizumab, as well as cytotoxic chemotherapy. Paraneoplastic meningoencephalomyelitis developed 2 years after diagnosis of breast cancer and 1 year after discontinuation of immunotherapy with pembrolizumab. She first developed longitudinally extending transverse myelitis followed by left optic neuritis and meningoencephalitis with new enhancing lesions in the brain and spinal leptomeninges. Cerebrospinal fluid analysis during both episodes showed normal glucose and protein, and elevated white blood cell count. Cytology was negative for malignancy. Cerebrospinal fluid was positive for neuromyelitis optica immunoglobulin G antibody anti-aquaporin-4, and autoimmune myelopathy panel was positive for myelin oligodendrocyte glycoprotein antibody. The patient had significant clinical and radiographic improvement after completion of five cycles of plasmapheresis followed by intravenous immunoglobulin. She did not have recurrence of paraneoplastic syndrome with maintenance rituximab every 6 months and daily low-dose prednisone. She succumbed to progressive systemic metastatic disease 4.5 years after her breast cancer diagnosis. This case shows that these antibodies can occur concurrently and cause clinical features, such as both neuromyelitis optica spectrum disorder and myelin oligodendrocyte glycoprotein antibody disease, in a patient with a singular type of cancer. We highlight the importance of testing for paraneoplastic etiology in cancer patients with radiographic menigoencephalomyelitis or meningitis with atypical symptoms of meningeal carcinomatosis and/or cerebrospinal fluid profile negative for leptomeningeal carcinomatosis.</p>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"15 1","pages":"55-60"},"PeriodicalIF":0.0,"publicationDate":"2023-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135202381","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}
引用次数: 0
Efficacy and safety of zilucoplan in Japanese patients with generalized myasthenia gravis: A subgroup analysis of the phase III randomized RAISE study 齐鲁霉素对日本全身性肌无力患者的疗效和安全性:III 期随机 RAISE 研究的分组分析
Clinical and Experimental Neuroimmunology Pub Date : 2023-09-15 DOI: 10.1111/cen3.12766
Kimiaki Utsugisawa, Kazushi Deguchi, Shingo Konno, Masayuki Masuda, Naoya Minami, Hiroyuki Murai, Shigeaki Suzuki, Yasushi Suzuki, Akira Tsujino, Akiyuki Uzawa, Babak Boroojerdi, Guillemette de la Borderie, Melissa Brock, Petra W. Duda, Mark Vanderkelen, James F. Howard Jr
{"title":"Efficacy and safety of zilucoplan in Japanese patients with generalized myasthenia gravis: A subgroup analysis of the phase III randomized RAISE study","authors":"Kimiaki Utsugisawa,&nbsp;Kazushi Deguchi,&nbsp;Shingo Konno,&nbsp;Masayuki Masuda,&nbsp;Naoya Minami,&nbsp;Hiroyuki Murai,&nbsp;Shigeaki Suzuki,&nbsp;Yasushi Suzuki,&nbsp;Akira Tsujino,&nbsp;Akiyuki Uzawa,&nbsp;Babak Boroojerdi,&nbsp;Guillemette de la Borderie,&nbsp;Melissa Brock,&nbsp;Petra W. Duda,&nbsp;Mark Vanderkelen,&nbsp;James F. Howard Jr","doi":"10.1111/cen3.12766","DOIUrl":"10.1111/cen3.12766","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>RAISE (NCT04115293) was a randomized, multicenter, double-blind, placebo-controlled phase III study of zilucoplan, a macrocyclic peptide and complement component 5 inhibitor with a dual mechanism of action, in patients with acetylcholine receptor autoantibody-positive generalized myasthenia gravis (MG). RAISE showed clinically meaningful and statistically significant improvements in MG-specific outcomes in the overall population. Here, we assess efficacy and safety of zilucoplan in patients with generalized myasthenia gravis in the Japanese patients enrolled in RAISE.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Adults with acetylcholine receptor autoantibody-positive generalized MG (MGFA disease class II–IV) were randomized 1:1 to daily self-administered subcutaneous zilucoplan 0.3 mg/kg or placebo injections for 12 weeks. The primary efficacy endpoint was change from baseline to week 12 in the MG Activities of Daily Living score. Safety was assessed by the incidence of treatment-emergent adverse events. Efficacy and safety outcomes of a Japanese subgroup were prespecified.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Overall, 16 Japanese patients were randomized to zilucoplan 0.3 mg/kg (<i>n</i> = 7) or placebo (<i>n</i> = 9). There was a clinically meaningful improvement in the MG Activities of Daily Living score at week 12 for zilucoplan versus placebo in the Japanese population, with least squares mean difference of −4.26 (95% confidence interval −7.40, −1.12), which was comparable with the overall population. The incidence of treatment-emergent adverse events was similar in both treatment arms, with 57.1% and 55.6% of patients in the zilucoplan and placebo groups, respectively, experiencing treatment-emergent adverse events.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>In a subgroup of Japanese patients, zilucoplan showed clinically meaningful improvement in MG-specific outcomes with a favorable safety profile, consistent with the overall RAISE population.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"15 1","pages":"45-54"},"PeriodicalIF":0.0,"publicationDate":"2023-09-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/cen3.12766","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135436315","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}
引用次数: 0
Disorders related to antineuronal antibodies: Autoimmune epilepsy 与抗神经元抗体相关的疾病:自身免疫性癫痫
Clinical and Experimental Neuroimmunology Pub Date : 2023-08-30 DOI: 10.1111/cen3.12765
Shusuke Koto, Norio Chihara, Atsushi Hara, Riki Matsumoto
{"title":"Disorders related to antineuronal antibodies: Autoimmune epilepsy","authors":"Shusuke Koto,&nbsp;Norio Chihara,&nbsp;Atsushi Hara,&nbsp;Riki Matsumoto","doi":"10.1111/cen3.12765","DOIUrl":"10.1111/cen3.12765","url":null,"abstract":"<p>Autoimmune epilepsy is characterized as a subtype of autoimmune encephalitis, where epileptic seizures serve as the primary or predominant manifestation of the disease. Among patients who are refractory to antiepileptic drug therapy, a part of them experience improved seizure control with immunotherapy. Some of these individuals have been found to possess autoantibodies that target the neuronal surface, intracellular, or extracellular antigens. In 2017, the International League Against Epilepsy (ILAE) proposed a new classification of epilepsy syndromes that, for the first time, recognized “immune” as one of the etiologies of epilepsy. Since early and prompt diagnosis and treatment of autoimmune epilepsy may improve the prognosis, it is crucial to actively consider the utilization of reported diagnostic features and treatment with immunotherapy in the management of patients with refractory epilepsy. We herein provide a review of the literature concerning the clinical features, laboratory findings, pathophysiology, and treatment options associated with this disease.</p>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"15 1","pages":"32-39"},"PeriodicalIF":0.0,"publicationDate":"2023-08-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48897849","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}
引用次数: 0
Twenty-nail dystrophy and alopecia areata in an adult male with thymoma-associated myasthenia gravis: A case report 一例患有胸腺瘤相关重症肌无力的成年男性二十甲营养不良和斑秃:一例报告
Clinical and Experimental Neuroimmunology Pub Date : 2023-06-20 DOI: 10.1111/cen3.12761
Mario B. Prado Jr, Maria Franchesca Quinio, Karen Joy B. Adiao
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