{"title":"Spinocerebellar ataxia masquerading as multiple sclerosis, a case report","authors":"Darla Wheeler , Mariam Bezih , Nicholas Lannen","doi":"10.1016/j.jneuroim.2024.578385","DOIUrl":"10.1016/j.jneuroim.2024.578385","url":null,"abstract":"","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"393 ","pages":"Article 578385"},"PeriodicalIF":3.3,"publicationDate":"2024-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141266206","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Neurotropic murine coronavirus mediated demyelination: Factors dampening pathogenesis","authors":"Mihyun Hwang , Cornelia C. Bergmann","doi":"10.1016/j.jneuroim.2024.578382","DOIUrl":"10.1016/j.jneuroim.2024.578382","url":null,"abstract":"<div><p>Virus infections and autoimmune responses are implicated as primary triggers of demyelinating diseases. Specifically, the association of Epstein-Barr virus (EBV) infection with development of multiple sclerosis (MS) has re-ignited an interest in virus induced autoimmune responses to CNS antigens. Nevertheless, demyelination may also be caused by immune mediated bystander pathology in an attempt to control direct infection in the CNS. Tissue damage as a result of anti-viral responses or low level viral persistence may lead to immune activation manifesting in demyelinating lesions, axonal damage and clinical symptoms. This review focuses on the neurotropic mouse coronavirus induced demyelination model to highlight how immune responses activated during the acute phase pave the way to dampen pathology and promote repair. We specifically discuss the role of immune dampening factors programmed cell death ligand 1 (PD-L1) and interleukin (IL)-10, as well as microglia and triggering receptor expressed on myeloid cells 2 (Trem2), in limiting demyelination independent of viral persistence.</p></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"393 ","pages":"Article 578382"},"PeriodicalIF":3.3,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141233757","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Infection, vaccination and narcolepsy type 1: Evidence and potential molecular mechanisms","authors":"","doi":"10.1016/j.jneuroim.2024.578383","DOIUrl":"10.1016/j.jneuroim.2024.578383","url":null,"abstract":"<div><p>NT1 is a rare, chronic and disabling neurological disease causing excessive daytime sleepiness and cataplexy. NT1 is characterized pathologically by an almost complete loss of neurons producing the hypocretin (HCRT)/orexin neuropeptides in the lateral hypothalamus<em>.</em> While the exact etiology of NT1 is still unknown, numerous studies have provided compelling evidence supporting its autoimmune origin. The prevailing hypothetical view on the pathogenesis of NT1 involves an immune-mediated loss of HCRT neurons that can be triggered by Pandemrix® vaccination and/or by infection in genetically susceptible patients, specifically carriers of the <em>HLA-DQB1*06:02</em> MHC class II allele. The molecular mechanisms by which infection/vaccination can induce autoimmunity in the case of NT1 remain to be elucidated. In this review, evidence regarding the involvement of vaccination and infection and the potential mechanisms by which it could be linked to the pathogenesis of NT1 will be discussed in light of the existing findings in other autoimmune diseases.</p></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"393 ","pages":"Article 578383"},"PeriodicalIF":2.9,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141235316","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Antoine Pegat , Jean-Baptiste Chanson , Pierre Lozeron , Bastien Joubert , Alexandre Bani-Sadr , Isabelle Quadrio , Léo Vidoni , Philippe Latour
{"title":"Identification of rare variants in the FBXO38 gene of patients with chronic inflammatory demyelinating polyradiculoneuropathy","authors":"Antoine Pegat , Jean-Baptiste Chanson , Pierre Lozeron , Bastien Joubert , Alexandre Bani-Sadr , Isabelle Quadrio , Léo Vidoni , Philippe Latour","doi":"10.1016/j.jneuroim.2024.578381","DOIUrl":"10.1016/j.jneuroim.2024.578381","url":null,"abstract":"<div><p>Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare immune-mediated neuropathy for which there is no clearly identified risk factor. The present study identified rare variants in the <em>FBXO38</em> gene in three familial cases of CIDP with response to corticosteroids in three generations with incomplete penetrance, and in an unrelated fourth case with diffuse nerve hypertrophy. <em>FBXO38</em> may be involved in the regulation of the immunity mediated by CD8 T cells, which have an important role in CIDP pathophysiology, through PD1 degradation. Considering these findings, <em>FBXO38</em> should be investigated as a potential genetic factor in larger cohorts of patients with CIDP.</p></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"392 ","pages":"Article 578381"},"PeriodicalIF":3.3,"publicationDate":"2024-05-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S0165572824000997/pdfft?md5=fdb2a02d919d779d5d38d4f2132c9d2d&pid=1-s2.0-S0165572824000997-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141186618","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Neuroinflammation, neurodegeneration and alteration of spatial memory in BALB/c mice through ampicillin-induced gut dysbiosis; NOS2 and NFL involvement in a microbiota-gut-brain axis model","authors":"Nassima Behairi , Arezki Samer , Lynda Sahraoui , Djehane Houria Mataam , Ryad Trari , Billel Flissi , Houda Belguendouz , Zine-Charaf Amir , Chafia Touil-Boukoffa","doi":"10.1016/j.jneuroim.2024.578374","DOIUrl":"https://doi.org/10.1016/j.jneuroim.2024.578374","url":null,"abstract":"<div><p>We aimed to investigate ampicillin (AMP) mechanisms in microbiota-gut-brain axis. We evaluated its effect on two gut and brain regions and behavioral performances. We administred AMP (1 g/l) to BALB/c mice for 21 days. Then, we analyzed body weigth change, stool consistency scoring, gut length, intestinal microbiota composition, nitric oxide synthase 2 (NOS2) expression and tissue integrity. We subsequently evaluated NOS2, GFAP, CD68 and NFL cerebral expression and spatial memory.Interestingly, our data showed gut microbiota disruption, NOS2 upregulation and tissue damage, associated to cerebral NOS2, GFAP, CD68 and NFL over-expression and behavioral alteration. Antiobiotic therapy should be prescribed with great caution.</p></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"392 ","pages":"Article 578374"},"PeriodicalIF":3.3,"publicationDate":"2024-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141095860","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Attenuated adiponectin, omentin, increased interleukin-6 and tumor necrosis factor-alpha levels with altered cognition and depression in non-Hodgkin lymphoma patients: A case-control study","authors":"Meghavi Kathpalia , Pinki Mishra , Afsha Majid , Mohd. Ashif Khan , Anurag Sharma , Dinesh Bhurani , Nidhi","doi":"10.1016/j.jneuroim.2024.578372","DOIUrl":"https://doi.org/10.1016/j.jneuroim.2024.578372","url":null,"abstract":"<div><h3>Background and purpose</h3><p>Immune dysfunction is one of the risk factors which plays an important role in the development of non-Hodgkin lymphoma (NHL), and inflammation may be involved in its etiology. Minimal data is available on the effect of cytokine levels on neurobehavioral function in lymphoma before the initiation of chemotherapy. Therefore, we aimed to explore the risk of NHL by assessment of cytokine and adipokine levels and their correlation with neurobehavioral changes.</p></div><div><h3>Methods</h3><p>This case-control study enrolled 62 subjects (age-sex matched: 31 cases and 31 controls). Neurobehavioral assessment was done using Montreal Cognitive Assessment questionnaire (MoCA) and Patient Health Questionnaire (PHQ-9). EORTC Core Quality of Life questionnaire (EORTC QLQ-C30) was used to assess quality of life. Questionnaire assessment and sample collection were done after the patient enrolment and before first cycle of chemotherapy.</p></div><div><h3>Results</h3><p>Mean age of NHL patients and healthy controls was 51.9 ± 11.8 and 50 ± 10.9 years, respectively. NHL patients showed significantly higher levels of IL-6 (0.77 ± 0.11) and TNF- α (1.47 ± 1.31) than controls (0.55 ± 0.4 and 0.66 ± 0.89, respectively) with <em>p</em>-value<0.005. Also, NHL patients showed significantly lower levels of adiponectin (0.31 ± 0.24) and omentin (0.46 ± 0.1) than controls (0.42 ± 0.13 and 0.53 ± 0.11, respectively) with <em>p</em>-value<0.005. Lower MoCA and EORTC QLQ C-30 scores and higher PHQ-9 scores were observed in NHL patients in comparison to healthy control.</p></div><div><h3>Conclusion</h3><p>Our results showed that adiponectin, omentin IL-6 and TNF-α may be used as pre-diagnostic markers of NHL risk. Neurobehavioral changes observed in NHL patients may alter the quality of life.</p></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"392 ","pages":"Article 578372"},"PeriodicalIF":3.3,"publicationDate":"2024-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141084279","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Targeted DeSUMOylation as a therapeutic strategy for multiple sclerosis","authors":"S. Sriram, Kwang Woon Kim, Åsa Ljunggren-Rose","doi":"10.1016/j.jneuroim.2024.578371","DOIUrl":"10.1016/j.jneuroim.2024.578371","url":null,"abstract":"<div><p>SUMO (small ubiquitin like modifier) conjugated proteins have emerged as an important post translational modifier of cellular function. SUMOylation modulates several cellular processes involved in transcriptional regulation of genes, protein-protein interactions and DNA damage and repair. Since abnormalities in SUMOylation has been observed in neoplastic and neurodegenerative disorders, the SUMO pathway has become an attractive site for targeting of new therapies to regulate SUMOylation and reduce disease burden. Conjugation of SUMO to their respective substrates is orchestrated by an enzymatic cascade involving three main enzymes, E1, activation enzyme, E2, conjugating enzyme and E3, a protein ligase. Each of these enzymes are therefore potential “druggable” sites for future therapeutics.</p><p>SUMOylation is a well-known mechanism by which the innate immune response is regulated in response to viral infections and in the adaptive immune response to tumor immunity. We have shown that small molecules which inhibit the SUMO activation pathway are also capable of inhibiting autoimmune response. TAK981 which forms adducts with SUMO and anacardic acid which inhibits the E1 enzyme of the SUMO pathway were effective in preventing the development of experimental allergic encephalitis (EAE), a mouse model of multiple sclerosis. Anacardic acid and TAK981 inhibited activation of TH17 cells and reduced clinical and pathological injury in IL-17 mediated myelin oligodendrocyte glycoprotein (MOG) induced EAE. Ginkgolic acid, another known inhibitor of SUMO pathway, was also shown to be effective in reducing the severity of inflammatory arthropathies which is also IL-17 mediated. In addition, the increase in the transcription of myelin genes with TAK981 and anacardic acid improved remyelination in experimental models of demyelination.</p><p>In the present review paper, we examine the mechanism of action of inhibitors of the SUMO pathway on regulating the immune response and the possibility of the use of these agents as therapeutics for MS.</p></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"392 ","pages":"Article 578371"},"PeriodicalIF":3.3,"publicationDate":"2024-05-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S0165572824000894/pdfft?md5=788a31406eea598453c3c2eb1e270d96&pid=1-s2.0-S0165572824000894-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140961804","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Baló concentric sclerosis: Literature review and report of two cases","authors":"Angelo Fonseca , Ernestina Santos , Ricardo Taipa","doi":"10.1016/j.jneuroim.2024.578370","DOIUrl":"10.1016/j.jneuroim.2024.578370","url":null,"abstract":"<div><h3>Background</h3><p>Baló's concentric sclerosis (BCS) is a rare variant of multiple sclerosis characterized by unique pathological features of alternating demyelination and preserved myelin.</p></div><div><h3>Objectives</h3><p>To describe two cases of BCS, radiological and pathological findings and its clinical course.</p></div><div><h3>Results</h3><p>We report two distinct cases of BCS that presented with unique MRI findings suggestive of BCS, but with different clinical courses and responses to treatment. The first case demonstrated substantial recovery following corticosteroid therapy, while the second case, initially suspected to be a malignant tumour, showed improvement after surgical intervention and immunoglobulin therapy.</p></div><div><h3>Conclusion</h3><p>These cases highlight the variability in presentation and course of BCS, underscoring the challenges in diagnosis and the importance of considering BCS in the differential diagnosis of demyelinating and tumefactive lesions. The cases also emphasize the potential for favourable outcomes with appropriate management, challenging the traditional view of BCS as uniformly severe.</p></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"392 ","pages":"Article 578370"},"PeriodicalIF":3.3,"publicationDate":"2024-05-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141057626","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Morten Zachariassen , Martin Munthe Thomsen , Thore Hillig , Pelle Trier-Petersen , Andreas Vestergaard Jensen , Lennart Jan Friis-Hansen , Christian Thomas Brandt
{"title":"Tenascin-C in patients with central nervous system infections","authors":"Morten Zachariassen , Martin Munthe Thomsen , Thore Hillig , Pelle Trier-Petersen , Andreas Vestergaard Jensen , Lennart Jan Friis-Hansen , Christian Thomas Brandt","doi":"10.1016/j.jneuroim.2024.578373","DOIUrl":"10.1016/j.jneuroim.2024.578373","url":null,"abstract":"<div><h3>Background</h3><p>The extracellular matrix protein tenascin-C has been discovered to be an important regulator of the response to tissue injury and repair in cerebrovascular diseases. This study investigated if tenascin-C is released in response to infections in the central nervous system (CNS).</p></div><div><h3>Methods</h3><p>Tenascin-C concentration in the cerebrospinal fluid (CSF) was measured in patients, (>18 years) with and without CNS infections, admitted to a department of infectious diseases in Denmark. CSF tenascin-C was measured on the Meso-scale platform.</p></div><div><h3>Results</h3><p>174 patients were included of which 140 were diagnosed with a CNS infection and 34 where this was ruled out (control group). Median CSF tenascin-C levels were significantly higher among patients with bacterial meningitis (147 pg/mL), viral meningitis (33 mg/mL), viral encephalitis (39 pg/mL) and Lyme neuroborreliosis (45 pg/mL) when compared to controls (21 pg/mL). Correlations between tenascin-C and CSF markers of inflammation and age were only moderate.</p></div><div><h3>Conclusion</h3><p>Levels of CSF tenascin-C are higher among patients with bacterial and viral neuroinfections, already on admission, but exhibit only a modest correlation with baseline indices of neuroinflammation. CSF tenascin-C is highest among patients with bacterial meningitis compared to the other CNS infections. Patients with unfavorable outcomes presented with higher median CSF tenascin-C than their counterparts.</p></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"392 ","pages":"Article 578373"},"PeriodicalIF":3.3,"publicationDate":"2024-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S0165572824000912/pdfft?md5=b21f4363609aaa672966b768773bed8e&pid=1-s2.0-S0165572824000912-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141028713","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}