Clinical and Experimental Neuroimmunology最新文献

{"title":"Longitudinal imaging for monitoring disease activity in late‐onset Rasmussen's encephalitis during multimodal rehabilitation and immune therapy","authors":"Lucas C. Adam, Lana Gilly, Joerg Mueller, Joerg Wissel, Anatol Kivi","doi":"10.1111/cen3.12805","DOIUrl":"https://doi.org/10.1111/cen3.12805","url":null,"abstract":"Rasmussen's encephalitis (RE) is a rare autoimmune encephalopathy typically manifesting in early childhood, causing unilateral autoimmune inflammation of the cerebral cortex, leading to progressive neurological deficits, notably focal epileptic seizures. The late‐onset variant of RE in adults progresses slower and presents atypical features. Despite extensive research, the etiology remains elusive, hindering accurate diagnosis and treatment options.We present a biopsy‐confirmed late‐onset variant of RE case in a 71‐year‐old man with a disease course of 12 years. After the initiation of intravenous immunoglobulin therapy and immunosuppressive treatment, disease stabilization was achieved, as evidenced by clinical assessments and imaging. Initially, the affected hemisphere swelled hyperacutely, followed by years of atrophic encephalopathy stabilizing into a residual state, with emerging focal disease signs in the contralateral hemisphere. Multimodal rehabilitation and immune therapy attenuated brain atrophy and reduced signal enhancement.Late‐onset variant of RE rehabilitation remains underdeveloped, focusing on symptom management and functional recovery post‐surgery. Longitudinal imaging is crucial for monitoring immune therapy response in clinical practice.","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141828015","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Response to: Eculizumab use throughout pregnancy in two patients with aquaporin‐4‐positive neuromyelitis optica spectrum disorder","authors":"I. Kister, Alla Wilson","doi":"10.1111/cen3.12807","DOIUrl":"https://doi.org/10.1111/cen3.12807","url":null,"abstract":"","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141661504","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":"Th17 pathway‐related immune signatures in the pathobiology of myasthenia gravis: Integrating the roles of regulatory/effector cytokines and transcription factors","authors":"Nibu Varghese, M. Nagappa, N. Sreenivas, Saikat Dey, T. Mullapudi, Anagha Pettututhazhe Kuniyil, S. Shivaram, D. Seshagiri, B. V. Nair, M. Debnath","doi":"10.1111/cen3.12804","DOIUrl":"https://doi.org/10.1111/cen3.12804","url":null,"abstract":"Myasthenia gravis (MG) is an autoimmune disorder mediated by antibodies against the acetylcholine receptor (AChR), muscle specific kinase (MuSK), and other antigens in the neuromuscular junction. Antibody production is influenced by T lymphocytes. The T helper 17 (Th17) subset, an inflammatory lineage of Th cells, is associated with several autoimmune diseases. Functional interactions between T lymphocytes and pathogenic antibody responses including aberrant Th17 cell responses have been reported in MG. However, the precise mechanism(s) underlying the activation and/or pathogenic transformation of Th17 cells are not clearly known. The current study aimed at simultaneously exploring the roles of the inducers, master regulator transcription factors, and effectors of Th17 cells in patients with MG.In this cross‐sectional study, quantification of gene expression of IL6, IL17A, STAT3, and RORC in the peripheral mononuclear cells by quantitative polymerase chain reaction (qPCR) as well as estimation of plasma levels of IL‐1β, IL‐6, and IL‐17A cytokines by multiplex suspension assay were carried out in 59 patients with MG and 61 healthy controls.Gene expression levels of IL17A were significantly upregulated in patients as compared to healthy controls. The plasma levels of IL‐1β, IL‐6, and IL‐17A were significantly elevated in patients compared to healthy controls. IL17A as well as RORC gene expressions correlated with the clinical features. IL17A gene expression levels were higher in AChR‐MG patients.The present study supports the crucial role of the Th17 pathway in the pathobiology of MG, including its potential influence on disease severity.","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141667298","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":"Successful treatment with plasmapheresis of severe Bickerstaff brainstem encephalitis with high cerebrospinal fluid CXCL‐10 levels after COVID‐19 infection: A case report","authors":"Naoki Iijima, Kenzo Sakurai, Riyoko Ko, K. Isahaya, Y. Yamano","doi":"10.1111/cen3.12806","DOIUrl":"https://doi.org/10.1111/cen3.12806","url":null,"abstract":"Bickerstaff brainstem encephalitis (BBE) is an autoimmune disease affecting the brainstem, typically caused by a prior infection. However, BBE after coronavirus disease 2019 (COVID‐19) infection is rare. Here, we present a severe case of BBE after COVID‐19 infection, highlighted by increased levels of CXCL‐10.A 28‐year‐old woman presented with symptoms of cold and fever lasting 5 days, accompanied by numbness, weakness and unsteadiness in the distal parts of her limbs before being admitted. Upon admission, her condition was classified with a Glasgow Coma Scale score of E1V1M4, absence of bilateral ocular cephalic reflexes, eyes fixed in the midline position and pathological reflex in lower limbs. COVID‐19 antigen tests were positive, and cerebrospinal fluid CXCL‐10 levels were elevated. Positive serum anti‐GQ1b antibodies, along with other clinical findings, confirmed the diagnosis of BBE. Initial treatment with high‐dose intravenous immunoglobulin was ineffective, leading to mechanical ventilation on day 2 from admission. Additional steroid pulse therapy and plasmapheresis were initiated on day 7. Communication abilities were restored by day 19, and the patient was extubated on day 21. Continuous alleviation of symptoms was observed, with no sequelae at discharge on day 42.BBE related to COVID‐19 with high CXCL‐10 levels can become severe. However, early intensive immunotherapy, including plasmapheresis, might result in favorable prognosis.","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141668079","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":"Intensity of subarachnoid space inflammation corresponds to the evolution of vessel wall remodeling during the acute and chronic phases of bacterial meningitis","authors":"Vivig Shantha Kumar, Vignarth Shantha Kumar, Ruthvik Thaghalli Sunil Kumar","doi":"10.1111/cen3.12794","DOIUrl":"https://doi.org/10.1111/cen3.12794","url":null,"abstract":"Cerebrovascular alterations in acute bacterial meningitis significantly contribute to adverse patient outcomes, with reported complication rates ranging from 10% to 29%. Focal alterations in arterial lumens, leading to vasoconstriction, are common in cerebral ischemic and inflammatory conditions, such as bacterial meningitis, presenting neurological complications, such as seizures, brain swelling, hydrocephalus, hearing loss and ischemic or hemorrhagic brain damage. The observed arterial narrowing during meningitis is attributed to diverse factors, including direct encroachment by inflammatory exudate, vascular wall edema, vasospasm, and vasculitis due to cellular infiltration and vessel remodeling. Early‐stage constriction might result from a watery exudate's encroachment, whereas persistent inflammation leads to thicker exudates, attracting inflammatory cells and inducing arteriopathic growth factor synthesis. This process promotes structural modifications in the vessel wall, progressing from subintimal infiltration to organic intimal thickening, culminating in vasculitis and the risk of cerebral ischemia. Accordingly, this review seeks to more clearly delineate the intricate relationship between subarachnoid space inflammation and acute and chronic vessel wall remodeling during bacterial meningitis. Conceivably, understanding this pathological process holds promise in unveiling potential treatment avenues to improve patient outcomes, and reduced morbidity and mortality associated with cerebrovascular complications during bacterial meningitis.","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141670127","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The clinical, diagnostic and treatment spectrum of seropositive and seronegative autoimmune encephalitis: Single‐center cohort study of 51 cases and review of the literature","authors":"Ahmed Elrefaey, Ahmed Mohamedelkhair, Lara Fahmy, Mohammad Affan, Lonni R. Schultz, M. Cerghet, A. Memon","doi":"10.1111/cen3.12802","DOIUrl":"https://doi.org/10.1111/cen3.12802","url":null,"abstract":"Autoimmune encephalitis (AE) comprises a spectrum of inflammatory neurological syndromes characterized by immune responses to neuronal autoantigens, leading to diverse clinical manifestations, particularly behavioral and cognitive decline.This single‐center retrospective study included 51 patients diagnosed with AE from 2013 to 2019 in a southeast Michigan tertiary care hospital. Patients were then divided into two groups, seropositive AE (AE+) and seronegative AE (AE−), based on antibody detection in the serum, cerebrospinal fluid or both when available. The study compares AE+ and AE− subtypes across clinical, diagnostic, and therapeutic parameters.A total of 34 patients were classified as AE+, and 17 as AE−. Demographic analysis showed no significant differences in age, sex or race between the two groups. Clinical presentations varied widely, encompassing psychiatric symptoms, movement disorders, seizures and confusion; 24% patients had a prior malignancy. Laboratory assessments found diverse autoantibodies in AE+ patients' serum. Radiological and electrophysiological assessments showed no significant differences between the groups. AE− patients had higher rates of confusion compared with AE+ patients (59% vs. 18%, P = 0.004).This study focuses on the complexities associated with diagnosing AE, emphasizing the challenges posed by the heterogeneity of symptoms and often negative antibody test results. Rapid identification of AE, regardless of seropositivity or seronegativity, emerges as a critical factor for clinicians, facilitating the prompt initiation of immunotherapy and/or tumor removal if needed. These insights contribute to a better understanding of the landscape of this condition, offering clinicians the tools to refine their diagnostic and treatment strategies. Ultimately, the study aimed to enhance the management of AE, empowering healthcare professionals to make accurate and timely interventions for patients.","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141367315","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":"q‐Space Myelin Map: A new myelin‐specific imaging technique for treatment monitoring of multiple sclerosis","authors":"Satoshi Kitagawa, Kenji Kufukihara, Haruhiko Motegi, Koji Sekiguchi, Yayoi Sato, Jin Nakahara","doi":"10.1111/cen3.12796","DOIUrl":"https://doi.org/10.1111/cen3.12796","url":null,"abstract":"In multiple sclerosis (MS) patients, hyperintense signals on T2‐weighted images by magnetic resonance imaging are signs of demyelination; however, T2 signals lack specificity and fail to detect remyelination. For more precise monitoring of MS, a new magnetic resonance imaging technique, q‐space Myelin Map (qMM), which specifically identifies myelin, has been developed. This study aimed to explore clinical factors associated with remyelination for different disease‐modifying drugs, and to examine the utility and feasibility of qMM in clinical practice.Data from sequential patients with relapsing–remitting MS initiating disease‐modifying drugs at our center were collected. After treatment initiation, qMM was carried out at 6‐month intervals and the resulting images analyzed for evidence of remyelination.A total of 48 patients with relapsing–remitting MS were included: 22 with dimethyl fumarate, 14 with fingolimod, four with glatiramer acetate and eight with natalizumab. qMM showed qMM‐remyelination in 22 patients (45.8%). In natalizumab patients, baseline ages were 33.6 ± 6.9 years (n = 5) and 47.3 ± 5.8 years (n = 3) in patients with or without qMM remyelination, respectively. In dimethyl fumarate patients, the proportion of women was 100% (n = 10) and 50% (n = 12) in patients with or without qMM myelination, respectively.This exploratory study suggested the potential clinical utility of qMM for visualizing remyelination in MS patients and fine‐tuning their pharmacotherapy. Two potential clinical factors promoting qMM‐remyelination were identified: female sex with dimethyl fumarate and younger baseline age with natalizumab; a larger prospective study is warranted to confirm these results.","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-06-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141273543","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":"Exploring digital biomarkers using smartphones for fatigue assessment in patients with multiple sclerosis","authors":"Kenzo Sakurai, Naoki Takao, Yoko Nakano, Takeshi Imai, K. Isahaya, Y. Yamano","doi":"10.1111/cen3.12793","DOIUrl":"https://doi.org/10.1111/cen3.12793","url":null,"abstract":"The treatment goals for multiple sclerosis (MS) are shifting from relapse inhibition to the improvement of long‐term prognosis and quality of life (QoL). Fatigue reduces QoL in patients with MS. The methods for assessing and treating fatigue, particularly those that are easily applicable in clinical settings remain unestablished. This study aimed to explore and identify digital biomarkers related to fatigue using smartphones.The patients with MS attending the Department of Neurology at St. Marianna University School of Medicine (Kanagawa, Japan) were surveyed via mail and responses were obtained online. The survey items included patient background such as age and sex, Fatigue Assessment Scale (FAS), which is one of the patient‐reported outcomes (PROs) and serves as a questionnaire method for assessing fatigue, as well as self‐assessments of depression, sleep, and pain. Additionally, the number of steps recorded on smartphones was collected as personal health records (PHRs) along with the time spent using smartphones.Overall, 27 (18 female) participants responded (response rate: 46.6%). The mean age was 41.0 ± 14.6 y and the majority (12 participants) had a disease duration of <5 y. According to self‐assessments, moderate or higher symptoms of depression, sleep disturbances, and pain were observed in 13 participants, 10 participants, and 6 participants, respectively. The daily number of steps ranged from 805 to 15 263 (median of 4514 steps). The number of steps was negatively correlated with FAS (r = −0.47, P = .02), and in cases with any physical disability, the number of steps was negatively correlated with FAS (r = −0.69, n = 0.01).The number of steps automatically measured by smartphones could be a digital biomarker reflecting fatigue in MS patients.","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141103879","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":"Case report of ADEM in an adult patient with chikungunya","authors":"João Alfredo M. M. Barros,&nbsp;Arthur Felipe Barbosa Vasconcelos,&nbsp;Francisco Anderson de Sá Carvalho,&nbsp;Gilmar Leite Pessoa Filho,&nbsp;Ana Luísa Castelo Branco Gomes,&nbsp;Raíssa N. L. F. Leite,&nbsp;João Felipe Bezerra,&nbsp;Juliana Magalhães Leite,&nbsp;Rafael de Souza Andrade,&nbsp;Bianca Etelvina Santos de Oliveira,&nbsp;Alex T. Meira","doi":"10.1111/cen3.12795","DOIUrl":"10.1111/cen3.12795","url":null,"abstract":"<p>Acute Disseminated Encephalomyelitis (ADEM) is a demyelinating immune-mediated disease characterized by bilateral and confluent lesions in white matter (WM), with an acute onset. This condition may arise due to a myriad of etiological factors, encompassing mainly vaccines and viral infections. This case report describes a 39-y-old patient who presented with a sudden onset of fever, confusion, and reduced level of consciousness, associated with paraparesis in the lower limbs and urinary retention, 2 d before admission to the neurological emergency department. The work-up included analysis of the cerebrospinal fluid (CSF), which showed 1.6 cells/mm³ and elevated proteins (91 g/dL); in addition to magnetic resonance imaging (MRI) of the brain and the spinal cord, in which hyperintense ovoid lesions with asymmetrical and bilateral distribution in the WM and basal ganglia were observed in the T2 and FLAIR. Later, chikungunya virus was detected in a molecular viral panel in the CSF. The patient exhibited an improvement radiologically, and in his condition following pulse with methylprednisolone and intravenous immunoglobulin therapy, and 40 mg of prednisone was prescribed for management during outpatient follow-up. This study highlights arbovirus infections as a possible cause of acute neurological conditions, involving both the brain and the spinal cord. Furthermore, the findings observed in the report were compared with those described in the literature, including other arboviruses. In conclusion, it was observed that the majority of patients responded to treatment with corticosteroids or immunoglobulins, with some neurological deficits eventually persisting. Therefore, more studies are needed to better investigate therapeutic options.</p>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141124057","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 nodopathy","authors":"Masahiro Iijima","doi":"10.1111/cen3.12791","DOIUrl":"https://doi.org/10.1111/cen3.12791","url":null,"abstract":"<p>Autoimmune nodopathy (AN) is characterized by the presence of autoantibodies targeting molecules essential for saltatory conduction in myelinated nerves. Clinical manifestations of AN show similarities with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), including progressive and symmetrical sensorimotor deficits with electrophysiological demyelinating features in nerve conduction studies. Although no common autoantibodies have yet been identified in CIDP, AN is characterized by autoantibodies that primarily target specific molecular complex structures represented by the Ranvier node and paranode. Furthermore, these autoantibodies, such as neurofascin-155 (NF155), contactin-1 (CNTN1), contactin-related protein 1 (Caspr1), and the CNTN1/Caspr1 complex, are illustrative examples of such autoantibodies. They can disrupt the septal-like junction of paranodes without triggering cellular immune responses. AN manifests uniquely with symptoms such as ataxia, tremors, and markedly high cerebrospinal fluid (CSF) protein levels, often accompanied by nerve root and cranial nerve hypertrophy. Notably, this condition is resistant to immunotherapies typically effective against CIDP, including intravenous immunoglobulin therapy (IVIg). Current evidence suggests that B-cell depletion therapies, such as rituximab, could benefit AN treatment. Since it has been suggested that existing treatments for CIDP may be effective in cases of autoantibody positivity of subclasses other than IgG4, CIDP that is resistant to conventional therapy requires novel therapeutic strategies that take into account the possibility of IgG4 autoantibodies.</p>","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":"140914610","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}