Clinical and Experimental Neuroimmunology最新文献

{"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}

{"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}

{"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>H₂-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}

{"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}

{"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}

{"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}

{"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}

{"title":"Twenty-nail dystrophy and alopecia areata in an adult male with thymoma-associated myasthenia gravis: A case report","authors":"Mario B. Prado Jr,&nbsp;Maria Franchesca Quinio,&nbsp;Karen Joy B. Adiao","doi":"10.1111/cen3.12761","DOIUrl":"10.1111/cen3.12761","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>To date, there is no journal that reports the coexistence of thymoma-associated myasthenia gravis, alopecia areata, and twenty-nail dystrophy. This paper hypothesizes that the presence of thymoma increases the risk of these conditions.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case presentation</h3>\u0000 \u0000 <p>The patient was a 37-year-old Filipino who came in for a year of history of fluctuating, but progressive, left-sided ptosis and diplopia; difficulty swallowing; dysarthria; and aspiration and symmetric proximal muscle weakness confirmed to be myasthenia gravis by abnormal repetitive nerve stimulation and the presence of thymoma on histology. Before his thymectomy, he also developed gradually worsening thinning and longitudinal ridging of all fingernails and toenails; and patches of bald areas on the scalp; diagnosed to be twenty-nail dystrophy and alopecia areata, respectively, by a board-certified dermatologist.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Thymoma is the possible risk factor and link for the co-occurrence of the three conditions in the present patient.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"14 3","pages":"149-152"},"PeriodicalIF":0.0,"publicationDate":"2023-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46129032","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":"Neuroinflammation and neuroimmunology in Alzheimer's disease","authors":"Koji Yamanaka","doi":"10.1111/cen3.12750","DOIUrl":"10.1111/cen3.12750","url":null,"abstract":"Neuroinflammation, mediated by activated glial cells and infiltrated lymphocytes, leads to the subsequent production of pro-inflammatory cytokines and related molecules. It is associated with the pathomechanisms of various neurodegenerative diseases, including Alzheimer's disease (AD). Microglia, innate immune cells in the central nervous system, are the principal component of neuroinflammation in health and disease. In particular, the phenotypic heterogeneity of microglia has been extensively examined through single-cell RNA sequencing technology, providing a clue to further understanding of neuroinflammation in AD and related neurological diseases. In addition, the role of brain lymphocytes has gained attention in the disease setting. In the past, the central nervous system is known to be an immuneprivileged tissue in which adaptive immunity and inflammation are highly restricted and controlled. However, an increasing number of reports show the detrimental and protective role of T lymphocytes in AD rodent models. In this issue of Clinical and Experimental Neuroimmunology, we invited three review articles by leading researchers in the fields of AD and neuroimmunology. Saito et al. reviewed the role of neuroinflammation in rodent models for AD. The authors have contributed significantly to the development of novel mouse models of AD, such as App knock-in mice. In this review, they focused on the role of the glial cell network in the AD continuum, the pathological sequence of amyloid β, tau and neurodegeneration in AD, and discussed the contribution of disease-associated microglia/homeostatic microglia in disease. Furthermore, they discussed the brain–periphery interaction in AD pathogenesis, which is a hot topic in AD research. Finally, multimorbidity in AD pathogenesis was discussed based on the clinical evidence. Chihara et al. reviewed the role of T lymphocytes in AD pathogenesis. Prominent T-lymphocyte infiltration, with its contribution to disease development and progression, is well known in multiple sclerosis. Increased permeability of the blood–brain barrier and infiltration of T lymphocytes have been documented in various studies using the human AD brain. In this regard, further investigation of the immune mechanisms resulting from T-cell infiltration into the central nervous system during disease initiation and exacerbation is required. They also provided an extensive review on the contribution of each subset of T lymphocytes in various AD models. Although the effects of each T lymphocyte on AD pathology are variable among AD mouse models, we need to carefully investigate the role of T lymphocytes in AD pathogenesis. Maekawa and Yamanaka reviewed the role of sex steroid hormone in AD, based on human and experimental evidence. As the incidence and prevalence of AD are dominant in women, the putative roles of sex hormones in AD pathology have been investigated. Although the role of estrogens was extensively investigated in the studies of ","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"14 2","pages":"78-79"},"PeriodicalIF":0.0,"publicationDate":"2023-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48658686","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":"Two cases of unilateral cortical fluid-attenuated inversion recovery-hyperintense lesions in anti-myelin oligodendrocyte glycoprotein-associated encephalitis with seizures (FLAMES)","authors":"Kunihiko Ishizawa,&nbsp;Osamu Iwasaki,&nbsp;Hironori Oka,&nbsp;Takashi Sugawara,&nbsp;Masakuni Amari,&nbsp;Takeshi Kawarabayashi,&nbsp;Koichi Okamoto,&nbsp;Kimihiko Kaneko,&nbsp;Toshiyuki Takahashi,&nbsp;Yoshio Ikeda,&nbsp;Masamitsu Takatama,&nbsp;Mikio Shoji","doi":"10.1111/cen3.12753","DOIUrl":"10.1111/cen3.12753","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 a rare inflammatory disease of the central nervous system. Unilateral cortical fluid-attenuated inversion recovery-hyperintense lesions in anti-MOG-associated encephalitis with seizures (FLAMES) has recently been proposed as a subcategory of MOGAD. FLAMES is characterized by fluid-attenuated inversion recovery (FLAIR) imaging showing hyperintense cortical lesions in MOG-associated encephalitis with seizures.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>We herein report two cases of unilateral cortical FLAMES. The first case was a 29-year-old woman who developed headaches, fever, convulsions and right hemiparesis. Brain FLAIR magnetic resonance imaging (MRI) showed hyperintense cortical lesions on the left side. The second case was a 37-year-old women who developed headaches and fever. Brain FLAIR MRI showed hyperintense cortical lesions on the left side. Both cases were positive for anti-MOG antibodies in cerebrospinal fluid and serum, and were diagnosed with unilateral cortical FLAMES in MOGAD. Both patients were treated with intravenous methylprednisolone followed by oral corticosteroids, which improved MRI findings and clinical symptoms.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Both patients were diagnosed with MOGAD due to characteristic unilateral cortical encephalitis on brain FLAIR MRI. Unilateral cortical FLAMES is an important clue for the clinical diagnosis of MOGAD.</p>\u0000 </section>\u0000 </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":"14 3","pages":"133-137"},"PeriodicalIF":0.0,"publicationDate":"2023-05-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44532334","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}