Neuroimmunology Reports最新文献

{"title":"Severe presentation of myelin oligodendrocyte glycoprotein antibody-associated disease: A case report","authors":"Dominique Comeau ,&nbsp;Olivia Cull ,&nbsp;Yanis Saheb ,&nbsp;Remi Leblanc ,&nbsp;Ludivine Chamard-Witkowski","doi":"10.1016/j.nerep.2024.100217","DOIUrl":"https://doi.org/10.1016/j.nerep.2024.100217","url":null,"abstract":"<div><h3>Background</h3><p>Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an inflammatory demyelinating disease of the central nervous system. Relapse may be moderate to severe with an Expanded Disability Status Scale (EDSS) above 4.0 in half of patients, albeit most experience good to excellent motor recovery.</p></div><div><h3>Results</h3><p>Herein, we present an atypically severe case of MOGAD with an unusual clinical course. Patient initially presented with diplopia, lower limb motor deficit and hypoesthesia which rapidly deteriorated into quadriplegia. Corticosteroid regimen did not initially translate to significant clinical improvement (EDSS=9.0). At the 1 year follow up, patient had regained some mobility although with marked sequela (EDSS=6.5).</p></div><div><h3>Conclusion</h3><p>Although MOGAD is generally thought to be benign, it could also present with severe episodes.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"6 ","pages":"Article 100217"},"PeriodicalIF":0.0,"publicationDate":"2024-06-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000184/pdfft?md5=06ef7b6b09aee0cdcbb316f6cf60bb56&pid=1-s2.0-S2667257X24000184-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141332976","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":"Susac syndrome with complete triad and concurrent kidney failure treated with plasmapheresis and rituximab: A case report","authors":"Mohammad Amin Sadri ,&nbsp;Sarina Loghmani ,&nbsp;Zahra Nikoo ,&nbsp;Farinaz Tabibian ,&nbsp;Vahid Shaygannejad","doi":"10.1016/j.nerep.2024.100210","DOIUrl":"10.1016/j.nerep.2024.100210","url":null,"abstract":"<div><h3>Introduction</h3><p>Susac syndrome (SuS) is a rare autoimmune disease characterized by the clinical triad of brain dysfunction, branch retinal artery occlusion, and hearing loss. Many cases have been reported with irreversible sequelae due to misdiagnosis and late treatment of the disease.</p></div><div><h3>Case</h3><p>In this article, we describe the clinical course and diagnosis of SuS in a young woman with a history of type 1 diabetes mellitus and kidney transplantation who presented with the complete triad. The complex medical history required a tailored treatment approach, including plasmapheresis and rituximab, which significantly improved her condition.</p></div><div><h3>Conclusion</h3><p>This report highlights the diagnostic complexity of SuS and the value of personalized treatment strategies, contributing to the understanding of this rare disorder, and briefly reviews the current knowledge of the disease and discusses the probable pathophysiological relationship between SuS and kidney failure.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"5 ","pages":"Article 100210"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000111/pdfft?md5=13d1bb55b1b04759af609450efe4a57e&pid=1-s2.0-S2667257X24000111-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140757340","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":"Progressive multifocal leukoencephalopathy successfully treated with intravenous immunoglobulin","authors":"Swansu Batra,&nbsp;Ruchika Tandon,&nbsp;Saurabh Nigam","doi":"10.1016/j.nerep.2024.100214","DOIUrl":"https://doi.org/10.1016/j.nerep.2024.100214","url":null,"abstract":"<div><p>A 52-year-old lady developed apathy, reduced verbal output, decreased visuospatial orientation, cognitive decline, visual impairment and left hemiparesis for 4 months. On MRI head, she displayed T2/FLAIR asymmetric white matter hyperintensities in parietal and occipital lobes and cerebellar peduncle and her CSF PCR was positive for John Cunningham (JC) virus and HIV ELISA and other bacterial, mycobacterial and viral markers were negative, confirming Progressive multifocal leukoencephalopathy (PML). Thereafter, we administered monthly intravenous immunoglobulin (IVIg) and patient's condition and neuroimaging has been improving for past 8 months. Hence, IVIg improving the condition of a potentially fatal disease patient, offers some hope.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"5 ","pages":"Article 100214"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000159/pdfft?md5=bf17e38c5cb88fa4347a2b32e7aff696&pid=1-s2.0-S2667257X24000159-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140948779","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":"On-pump coronary artery bypass graft in a patient with multiple sclerosis: A case report","authors":"Osman Fehmi Beyazal ,&nbsp;Ferhat Yıldız ,&nbsp;Koray Apaydın ,&nbsp;Zülfiye Yıldız ,&nbsp;Gültekin Saday","doi":"10.1016/j.nerep.2024.100200","DOIUrl":"https://doi.org/10.1016/j.nerep.2024.100200","url":null,"abstract":"<div><h3>Background</h3><p>Multiple sclerosis (MS) is a chronic autoimmune disease of the central nervous system characterized by inflammation, demyelination, gliosis, and neuronal loss. The risk of relapse may increase in the perioperative period in surgical interventions, especially in cardiac surgeries performed using cardiopulmonary bypass (CPB) and the use of CPB with MS patients is controversial.</p></div><div><h3>Case presentation</h3><p>In this case report, the management of a patient with MS to reduce the risk of relapse in the perioperative period is described and a case of coronary artery bypass graft (CABG) performed with CPB without any problems is presented.</p></div><div><h3>Conclusions</h3><p>CABG with CPB can be performed successfully in MS patients. Adjusting the operation time and neurological medications, closely monitoring body temperature and electrolytes in the intraoperative and postoperative period, and ensuring hemodynamic stability can reduce the risk of relapse and infection.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"5 ","pages":"Article 100200"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000019/pdfft?md5=2da13190a9278f51d62d62d485db16d4&pid=1-s2.0-S2667257X24000019-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139434533","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":"Acute onset of autoimmune hepatitis after initiation of B cell depletion therapy in multiple sclerosis","authors":"Mara Bahri ,&nbsp;Ankur Sheel ,&nbsp;Austin Bolker ,&nbsp;Wei Chen ,&nbsp;Khalid Mumtaz ,&nbsp;Cole A. Harrington","doi":"10.1016/j.nerep.2024.100206","DOIUrl":"https://doi.org/10.1016/j.nerep.2024.100206","url":null,"abstract":"<div><h3>Background</h3><p>Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease that may coexist in the multiple sclerosis population and remain undiagnosed. AIH has previously been reported following treatment with interferon beta, glatiramer acetate, natalizumab and high dose corticosteroids in people with multiple sclerosis (MS).</p></div><div><h3>Case presentation</h3><p>We present a rare case of autoimmune hepatitis onset after B cell depletion with ocrelizumab in a person with multiple sclerosis (MS).</p></div><div><h3>Case report</h3><p>Two weeks after the second dose of ocrelizumab, patient presented with jaundice and acute liver injury. Liver biopsy revealed pathological features of autoimmune hepatitis with negative workup for viral etiologies. A six month steroid taper and azathioprine resulted in normalization of liver function tests and clinical and radiological stability of multiple sclerosis and AIH at two years of follow-up.</p></div><div><h3>Conclusions</h3><p>Whether B cell depletion with ocrelizumab directly resulted in emergence of AIH is unclear. Regulatory B cells have been proposed to play a protective role in AIH pathogenesis and B cell depletion may provoke the emergence of AIH through loss of regulatory B cells.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"5 ","pages":"Article 100206"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X2400007X/pdfft?md5=f7059dfe0f44995fa5b316e79d181db1&pid=1-s2.0-S2667257X2400007X-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140187102","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":"Two cases of nasopharyngeal negative COVID-19 infections in patients with demyelinating diseases on anti-CD20 treatments","authors":"Alexandra Balshi,&nbsp;Jacob A Sloane","doi":"10.1016/j.nerep.2023.100197","DOIUrl":"10.1016/j.nerep.2023.100197","url":null,"abstract":"<div><p>We report two cases of nasopharyngeal negative COVID-19 infections in patients with demyelinating diseases on anti-CD20 treatments. Despite negative nasopharyngeal polymerase chain reaction (PCR) tests, both patients exhibited radiological features consistent with SARS-CoV-2 infection, with one confirmed in bronchoalveolar lavage fluid. Immunocompromised patients may have limited immune responses, possibly contributing to persistent COVID-19 infections despite vaccination history. These findings underscore the need for increased vigilance and consideration of lower respiratory tract infections in immunocompromised patients on anti-CD20 therapies.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"5 ","pages":"Article 100197"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X23000359/pdfft?md5=fcae974d72a548531ac7e57592fa75a4&pid=1-s2.0-S2667257X23000359-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138988574","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":"Tongue dystonia as CIS and presenting symptom of multiple sclerosis","authors":"Farid Shamlou ,&nbsp;Narges Ebrahimi ,&nbsp;Ahmad Chitsaz","doi":"10.1016/j.nerep.2023.100191","DOIUrl":"https://doi.org/10.1016/j.nerep.2023.100191","url":null,"abstract":"<div><p>Multiple sclerosis, as a chronic neurodegenerative disease, causes various complications for patients. The presentation of the disease with a neurological dysfunction called clinically isolated syndrome(CIS) is commonly vision problems and weakness in the body or extremities. Dystonia as a movement disorder is a rare presentation in MS. Here we report a case presenting lingual dystonia as the first manifestation of multiple sclerosis.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"5 ","pages":"Article 100191"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X23000293/pdfft?md5=fb754b8facfe3bde6b083ed80e64c17b&pid=1-s2.0-S2667257X23000293-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139109167","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":"Neuromyelitis optica spectrum disorder in a pediatric patient with ARCN1-related syndrome: A coincidental co-morbidity or disease association?","authors":"Fazila Aseem,&nbsp;Irena Dujmovic Basuroski","doi":"10.1016/j.nerep.2024.100205","DOIUrl":"https://doi.org/10.1016/j.nerep.2024.100205","url":null,"abstract":"<div><h3>Background</h3><p>Neuromyelitis Optica Spectrum Disorder (NMOSD) is a rare central nervous system autoimmune disease commonly associated with aquaporin-4 antibody (AQP4 Ab). ARCN1-related syndrome is a rare genetic syndrome caused by mutations in archain 1 (ARCN1) gene that encodes the coatomer subunit delta protein in the coat protein complex-I involved in intracellular protein transport. Impaired intracellular trafficking might predispose to autoimmunity. We report a boy with AQP4 Ab positive NMOSD and ARCN1<em>-</em>related syndrome.</p></div><div><h3>Case report</h3><p>A 7-year-old boy with ARCN1<em>-</em>related syndrome (c.508C&gt;T; p.Arg170*; pathogenic variant) presented with severe right sided optic neuritis, fever, intractable hiccups and vomiting. The patient was diagnosed with NMOSD based on clinical presentation (optic neuritis, area postrema syndrome) and positive AQP4 Ab (serum titer, &gt; 1:100,000). After a 5- day intravenous course of high dose methylprednisolone (30 mg/kg/day), his vision did not improve but other symptoms resolved. Maintenance treatment with intravenous immunoglobulin (IVIG, 0.4 g/kg/monthly) was started, but the patient continued to have recurrent left optic neuritis attacks despite escalating maintenance treatment regimen to oral prednisone in addition to IVIG. At the age of 8, the patient was started on a combination therapy with Rituximab and IVIG and has been NMOSD relapse free since then.</p></div><div><h3>Conclusion</h3><p>To date, this is the only reported case of NMOSD in a patient with ARCN1 mutation. Our case might further support an increased risk of autoimmunity in carriers of ARCN1 mutation.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"5 ","pages":"Article 100205"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000068/pdfft?md5=b9e19f4965e2b670e5e7bd9842b693a4&pid=1-s2.0-S2667257X24000068-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140139063","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":"Neurologic autoimmunity in the immunodeficient: A case of neuromyelitis optica in an individual with common variable immunodeficiency","authors":"Evan J. Luxenberg ,&nbsp;Monica Solorio ,&nbsp;Kathleen Munger ,&nbsp;Andrew Ayars ,&nbsp;Yujie Wang","doi":"10.1016/j.nerep.2024.100208","DOIUrl":"https://doi.org/10.1016/j.nerep.2024.100208","url":null,"abstract":"<div><h3>Background</h3><p>Neuromyelitis optica (NMO) is a relapsing CNS autoimmune disorder most commonly characterized by longitudinally extensive transverse myelitis (LETM) and/or optic neuritis (ON). Common variable immunodeficiency (CVID) is the most common clinically significant primary immunodeficiency, characterized by immune dysregulation and decreased immunoglobulin production. Autoimmunity is common in CVID, however neurologic autoimmunity is exceedingly rare and co-occurrence with NMO has not been previously described. We present the case of a man in his 60′s with CVID who developed LETM and was subsequently diagnosed with Aquaporin (AQP) 4 Immunoglobulin G (IgG) seropositive NMO.</p></div><div><h3>Methods</h3><p>Chart review of a patient treated at the University of Washington.</p></div><div><h3>Results</h3><p>Our patient had been treated with intravenous immunoglobulin therapy for CVID for 5 years when he presented with subacute onset of ascending paraparesis, sensory loss, and urinary retention. MRI identified LETM from C3 to T9. AQP4 IgG was markedly elevated at a titer of &gt;1:100,000. He responded well to immunotherapy.</p></div><div><h3>Conclusion</h3><p>Autoimmunity in CVID is thought to be from loss of regulatory T cell function as well as increased peripheral plasmablasts. We highlight the importance of considering autoimmune CNS diseases in primary immunodeficiencies as well as review possible pathophysiology of neurologic autoimmunity in CVID.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"5 ","pages":"Article 100208"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000093/pdfft?md5=fe80dd97279820ef37010933feaee642&pid=1-s2.0-S2667257X24000093-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140644928","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":"A rare presentation of a young adult with Lambert-Eaton myasthenic syndrome and respiratory distress: Case report","authors":"Daan P.C. van Doorn ,&nbsp;Sandra M.H. Huijs ,&nbsp;Sjoerd A.M.E.G. Timmermans ,&nbsp;Hannelore T.L. Pollet ,&nbsp;Marcel J.H. Aries ,&nbsp;Corinne Horlings ,&nbsp;Pieter van Paassen","doi":"10.1016/j.nerep.2024.100201","DOIUrl":"https://doi.org/10.1016/j.nerep.2024.100201","url":null,"abstract":"<div><h3>Background</h3><p>Lambert-Eaton myasthenic syndrome (LEMS) is a rare condition characterized by proximal muscle weakness, autonomic symptoms, reduced tendon reflexes, the presence of pathogenic autoantibodies to P/Q-type voltage-gated calcium channels, and repetitive nerve stimulation abnormalities. Non-specific symptoms and coexisting organ-specific autoantibodies can be misleading.</p></div><div><h3>Case report</h3><p>A 24-year-old male patient with a history of autoimmune thyroid disease presented with proximal muscle weakness and weight loss. Electromyography and muscle biopsy showed signs of myopathy. The presence of anti-OJ antibodies and ground glass opacities suggested anti-synthetase syndrome. Plasmapheresis, corticosteroids, mycophenolate mofetil, and tacrolimus were started. His clinical condition improved, but muscle strength did not fully recover. Three years later, his muscle strength declined and he developed diplopia, areflexia, autonomic dysfunction, and respiratory failure. In this critically ill and frail patient, multitargeted therapy with plasmapheresis, corticosteroids, and mycophenolate mofetil was started in combination with eculizumab. Anti-voltage gated calcium channel seropositivity then proved LEMS, whereafter amifampridine, pyridostigmine, and rituximab were added. His condition improved. No malignancy or genetic cause was found.</p></div><div><h3>Conclusion</h3><p>We report a case of non-tumour LEMS in a young patient with autoimmune comorbidities (Graves’ hyperthyroidism and anti-synthetase syndrome), respiratory symptoms, and initial signs of myopathy, leading to a substantial diagnostic delay. This critically ill patient responded well to multitargeted immunotherapy in combination with amifampridine and pyridostigmine. This case illustrates the diagnostic challenges in this rare presentation.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"5 ","pages":"Article 100201"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000020/pdfft?md5=5bbc8b2f49ce9b27cd4cada54acce06b&pid=1-s2.0-S2667257X24000020-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139674625","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}