Neuroimmunology ReportsPub Date : 2024-12-01Epub Date: 2024-07-15DOI: 10.1016/j.nerep.2024.100222
Rufaidah Rushdi , Nevin Shalaby
{"title":"Pediatric Multiple Sclerosis: Unveiling the Trajectory of a Toddler-Onset Case","authors":"Rufaidah Rushdi , Nevin Shalaby","doi":"10.1016/j.nerep.2024.100222","DOIUrl":"10.1016/j.nerep.2024.100222","url":null,"abstract":"<div><p>Pediatric multiple sclerosis (MS) is predominantly a relapsing-remitting demyelinating disorder. Toddler-onset MS (12–36 months) is rarely studied despite the likelihood of early-life adversity influencing its incidence. In this case, we present a 9-year-6-month-old girl who first exhibited symptoms at 30 months. Her disease course reflects the challenging mission of reaching a definitive MS diagnosis. Noteworthy observations include a complex perinatal history, initially normal diagnostic workup, and complete recovery after each relapse. It is crucial to recognize the hidden dynamics of MS, namely smoldering MS and the related progression independent of relapse activity, to commence a high-efficacy early treatment modality and establish a comprehensive multidisciplinary management strategy.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"6 ","pages":"Article 100222"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000238/pdfft?md5=c53ddea9b608eb80571a67a5888cbe36&pid=1-s2.0-S2667257X24000238-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141710339","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}
Neuroimmunology ReportsPub Date : 2024-12-01Epub Date: 2024-06-21DOI: 10.1016/j.nerep.2024.100219
Cesar D. Bautista-Sanchez , Luis E. Martínez-Bravo , Diego A. Hidalgo-Díaz
{"title":"Pancytopenia in neuromyelitis optica spectrum disorder induced by azathioprine-allopurinol interaction","authors":"Cesar D. Bautista-Sanchez , Luis E. Martínez-Bravo , Diego A. Hidalgo-Díaz","doi":"10.1016/j.nerep.2024.100219","DOIUrl":"https://doi.org/10.1016/j.nerep.2024.100219","url":null,"abstract":"<div><h3>Background</h3><p>Azathioprine (AZA) remains a widely used treatment for neuromyelitis optica spectrum disorder (NMOSD). It heightens the risk of myelosuppression, a risk further intensified with the addition of allopurinol.</p></div><div><h3>Case presentation</h3><p>To the best of our knowledge, there have been no reported cases of this complication in NMOSD.</p></div><div><h3>Case report</h3><p>We present a 63-year-old female with NMOSD on AZA, who experienced severe myelosuppression upon initiating allopurinol. Following the discontinuation of these drugs, we prescribed granulocyte stimulants, recombinant erythropoietin, and supportive care, which resulted in hematologic improvement.</p></div><div><h3>Conclusions</h3><p>This case highlights a potential myelosuppression risk when combining AZA and allopurinol in NMOSD patients.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"6 ","pages":"Article 100219"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000202/pdfft?md5=61c35b54f2390ba44144e23ca8c6129b&pid=1-s2.0-S2667257X24000202-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141481654","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}
Neuroimmunology ReportsPub Date : 2024-12-01Epub Date: 2024-06-11DOI: 10.1016/j.nerep.2024.100216
Feng Xiang, Xu Zhang, Rongfei Wang, Xiangqing Wang
{"title":"Case report: Neuromyelitis optica spectrum disorder in a patient presented with orbital apex syndrome","authors":"Feng Xiang, Xu Zhang, Rongfei Wang, Xiangqing Wang","doi":"10.1016/j.nerep.2024.100216","DOIUrl":"https://doi.org/10.1016/j.nerep.2024.100216","url":null,"abstract":"<div><h3>Background</h3><p>Orbital apex syndrome as the presentation of neuromyelitis optica spectrum disorder has not been reported in literature.</p></div><div><h3>Case presentation</h3><p>We reported a case in which the patient initially presented with ptosis, diplopia, ophthalmoplegia and visual impairment of the right eye. Clinical examination revealed no light perception in the right eye with the presence of relative afferent pupillary defect and involvement of cranial nerves II, III, V, and VI. Magnetic resonance imaging of the cranial and orbital regions revealed thickening in the intraorbital segment of the right optic nerve with T2-weighted hyperintensity, and Gadolinium-enhancement of the right optic nerve and its myelin sheath. Longitudinally extensive transverse myelitis subsequently developed, with detection of serum anti-aquaporin-4 antibody. Intravenous methylprednisolone was initiated, leading to significant clinical improvement.</p></div><div><h3>Conclusion</h3><p>This case report highlights the diverse manifestations of neuromyelitis optica spectrum disorder, including the orbital apex syndrome.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"6 ","pages":"Article 100216"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000172/pdfft?md5=7d2932e7bbd9739ff7dbc452293d28ce&pid=1-s2.0-S2667257X24000172-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141481655","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}
Neuroimmunology ReportsPub Date : 2024-12-01Epub Date: 2024-11-26DOI: 10.1016/j.nerep.2024.100233
Mario B Prado , Karen Joy Adiao
{"title":"Autoimmune demyelinating central nervous system disease in young male with persistently positive fluorescent treponemal antibody absorption test: A case report","authors":"Mario B Prado , Karen Joy Adiao","doi":"10.1016/j.nerep.2024.100233","DOIUrl":"10.1016/j.nerep.2024.100233","url":null,"abstract":"<div><div>For NMOSD and MOGAD, onset and relapses are often preceded by non-specific infections, but up until now no known microorganism has been identified to be strongly associated with these conditions. Syphilis as inciting event for NMOSD or MOGAD has only been reported once (Wilcox et al., 2008). This case report examines whether syphilis triggers autoimmune CNS condition or autoimmune CNS disease may cause false positive confirmatory treponemal test. The patient is a 30-year-old who came in with 8-month history of sudden onset spasticity and ataxia of bilateral lower extremities and blindness, confirmed to be transverse myelitis via imaging and optic neuritis by optic coherence tomography respectively. Anti-AQ4 and anti-MOG were negative, however, the patient repeatedly tested positive in FTA-ABS. We suspect that through molecular mimicry and bystander activation, there is a possibility that syphilis may have incited the onset and relapse of his autoimmune CNS demyelinating condition.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"6 ","pages":"Article 100233"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142744787","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}
Neuroimmunology ReportsPub Date : 2024-12-01Epub Date: 2024-09-08DOI: 10.1016/j.nerep.2024.100226
Krithika Arrabothu , Srinivas Govindan , Wilson Rodriguez , Christopher Tapia , Robert L. White III
{"title":"A case of autoimmune glial fibrillary acidic protein astrocytopathy with concurrent renal cell carcinoma","authors":"Krithika Arrabothu , Srinivas Govindan , Wilson Rodriguez , Christopher Tapia , Robert L. White III","doi":"10.1016/j.nerep.2024.100226","DOIUrl":"10.1016/j.nerep.2024.100226","url":null,"abstract":"<div><p>Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a novel central nervous system disorder characterized by subacute neuropsychiatric symptom development and rapid remission with corticosteroid therapy. In this paper, we describe a case of GFAP encephalitis found to have renal cell carcinoma. The patient was a 71-year-old man with a history of transient ischemic attacks, severe diabetic retinopathy and monoclonal gammopathy of undetermined significance, who presented with subacute cognitive decline. Anti-GFAP antibodies were detected in the cerebrospinal fluid. Computer Tomography (CT) of chest, abdomen and pelvis identified a renal mass that was resected. Subsequent pathology confirmed clear cell renal cell carcinoma. The patient was treated with corticosteroids with cognitive recovery near to baseline. In conclusion, we encourage the early consideration of autoimmune GFAP astrocytopathy for patients with clinical manifestation of subacute neuropsychiatric symptoms after other common conditions have been ruled out, as it usually has a great response to immunosuppressive therapy. In addition, this case highlights the importance of full body scan to identify possible underlying malignancy as a possible precipitating factor.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"6 ","pages":"Article 100226"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000275/pdfft?md5=2065be24e880ef5864893cd0798d0453&pid=1-s2.0-S2667257X24000275-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142241536","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}
Neuroimmunology ReportsPub Date : 2024-12-01Epub Date: 2024-09-14DOI: 10.1016/j.nerep.2024.100227
S Gandelman , S Parauda , C Dohle
{"title":"Very late-onset neuromyelitis optica presenting with simultaneous bilateral optic neuritis and myelitis: A case report","authors":"S Gandelman , S Parauda , C Dohle","doi":"10.1016/j.nerep.2024.100227","DOIUrl":"10.1016/j.nerep.2024.100227","url":null,"abstract":"<div><h3>Background</h3><p>New-onset Neuromyelitis Optica Spectrum Disorder (NMOSD) has rarely been reported in adults over age 70. We describe a severe presentation in a 77-year-old female.</p></div><div><h3>Case</h3><p>The patient presented with left arm numbness, followed by left leg numbness and bilateral optic neuritis. Neuroimaging showed a C2-T1 lesion with C4-C7 holocord enhancement, and bilateral optic nerve enhancement extending to the optic chiasm. Serum testing detected anti-aquaporin-4 antibodies. She was left with dyschromatopsia, myelopathy, and ambulatory dysfunction. She had a history notable for rheumatoid arthritis, treated with rituximab, which had been discontinued six years prior to presentation.</p></div><div><h3>Discussion</h3><p>Very-late-onset NMOSD encompasses a unique cohort of patients with significant immunogenicity despite immunosenescence. Limited data suggest diagnostic delays, severe morbidity, and decreased utilization of disease-modifying therapies.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"6 ","pages":"Article 100227"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000287/pdfft?md5=911d5b73fdd8fbd05a4f3ee1c3b475a6&pid=1-s2.0-S2667257X24000287-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142241537","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}
Neuroimmunology ReportsPub Date : 2024-01-01Epub Date: 2024-05-08DOI: 10.1016/j.nerep.2024.100214
Swansu Batra, Ruchika Tandon, Saurabh Nigam
{"title":"Progressive multifocal leukoencephalopathy successfully treated with intravenous immunoglobulin","authors":"Swansu Batra, Ruchika Tandon, 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}
Neuroimmunology ReportsPub Date : 2024-01-01Epub Date: 2024-04-20DOI: 10.1016/j.nerep.2024.100210
Mohammad Amin Sadri , Sarina Loghmani , Zahra Nikoo , Farinaz Tabibian , Vahid Shaygannejad
{"title":"Susac syndrome with complete triad and concurrent kidney failure treated with plasmapheresis and rituximab: A case report","authors":"Mohammad Amin Sadri , Sarina Loghmani , Zahra Nikoo , Farinaz Tabibian , 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}
Neuroimmunology ReportsPub Date : 2024-01-01Epub Date: 2024-03-12DOI: 10.1016/j.nerep.2024.100205
Fazila Aseem, Irena Dujmovic Basuroski
{"title":"Neuromyelitis optica spectrum disorder in a pediatric patient with ARCN1-related syndrome: A coincidental co-morbidity or disease association?","authors":"Fazila Aseem, 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>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, > 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}
Neuroimmunology ReportsPub Date : 2024-01-01Epub Date: 2024-01-07DOI: 10.1016/j.nerep.2024.100200
Osman Fehmi Beyazal , Ferhat Yıldız , Koray Apaydın , Zülfiye Yıldız , Gültekin Saday
{"title":"On-pump coronary artery bypass graft in a patient with multiple sclerosis: A case report","authors":"Osman Fehmi Beyazal , Ferhat Yıldız , Koray Apaydın , Zülfiye Yıldız , 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}