{"title":"Exposure to satralizumab in AQP4-positive NMOSD during pregnancy: A case report and review of the literature","authors":"Ricardo Alonso","doi":"10.1016/j.nerep.2025.100248","DOIUrl":"10.1016/j.nerep.2025.100248","url":null,"abstract":"<div><div>Neuromyelitis optica spectrum disorder (NMOSD) is a severe autoimmune disease of the central nervous system. It mainly affects young women and can lead to serious episodes involving the spinal cord and optic nerves. Treatments, may pose risks to the fetus, with limited safety data available. Satralizumab, is effective in reducing relapse rates but lacks sufficient safety data for use during pregnancy. The case of a 32-year-old woman with NMOSD who was treated with satralizumab during her first trimester is presented. This case provides preliminary evidence on satralizumab use in pregnancy, suggesting it might be a viable option with careful management.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"7 ","pages":"Article 100248"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143171844","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}
Adam L. Morehead , Eoin P. Flanagan , Andrew J. Solomon
{"title":"Tumefactive and longitudinally extensive myelitis: A rare presentation of multiple sclerosis?","authors":"Adam L. Morehead , Eoin P. Flanagan , Andrew J. Solomon","doi":"10.1016/j.nerep.2025.100257","DOIUrl":"10.1016/j.nerep.2025.100257","url":null,"abstract":"<div><div>Cerebral tumefactive demyelinating lesions are well described in multiple sclerosis (MS). Yet few cases of MS-associated tumefactive spinal cord demyelination have been described. We report a patient with a clinical course typical of relapsing-remitting MS who presented with a relapse associated with a tumefactive and longitudinally extensive cervical spinal cord lesion. Despite extensive evaluation, and with two years of longitudinal follow up, no better explanation than MS could be identified. This case highlights the diagnostic and prognostic challenges associated with presumed tumefactive spinal cord demyelination in MS, and the unmet need for further cases to guide therapeutic decision-making.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"7 ","pages":"Article 100257"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143838539","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}
Chun-Ho Choi , Jason Ka-Yeung Fong , Siu-Hung Li , Kary Ka-Wai Chan , Alexander Yuk-Lun Lau
{"title":"Oral cladribine for management of multiple sclerosis–Clinical experience from Hong Kong","authors":"Chun-Ho Choi , Jason Ka-Yeung Fong , Siu-Hung Li , Kary Ka-Wai Chan , Alexander Yuk-Lun Lau","doi":"10.1016/j.nerep.2024.100238","DOIUrl":"10.1016/j.nerep.2024.100238","url":null,"abstract":"<div><h3>Background</h3><div>Oral cladribine, a selective immune reconstitution therapy, offers a short-course option for disease-modifying therapy (DMT) in multiple sclerosis (MS), a chronic neurodegenerative disease characterized by inflammation and demyelination affecting 2.8 million people worldwide. Despite its proven safety and efficacy in phase 2 and 3 trials, data for oral cladribine treatment in Asian populations remain limited. This case series aims to contribute real-world experience across the clinical spectrum of disease by presenting Asian MS patients treated in Hong Kong.</div></div><div><h3>Methods</h3><div>This retrospective case series comprises 9 patients diagnosed with relapsing-remitting MS and received oral cladribine treatment in Hong Kong between 2019 and 2021. These patients, treated in both public and private hospitals, were followed up to assess the outcomes of oral cladribine therapy. Relevant data were extracted from patient's medical records.</div></div><div><h3>Results</h3><div>Outcomes were generally positive, with 6 out of 9 patients showing stable or improved Expanded Disability Status Scale (EDSS) scores 2–4 years after treatment initiation and 7 remaining relapse-free within 2–5 years of treatment initiation. Only 2 patients experienced mild relapses. Follow-up MRI scans revealed no new lesion development in any patient. One case of transient lymphopenia was noted, which did not require discontinuation of treatment.</div></div><div><h3>Conclusions</h3><div>Our clinical experience with these patients aligned with the safety and efficacy outcomes reported in clinical trials. These observations support the efficacy of a short-course regimen in reducing overall treatment burden for patients and potentially improving adherence to therapy. To substantiate and build upon these findings and explore long-term outcomes in larger cohorts, further research and continued monitoring of patients is necessary.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"7 ","pages":"Article 100238"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143171846","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":"Contactin-associated protein-like 2 (CASPR2) antibody generation in acute herpes simplex encephalitis: Case report and review of the literature","authors":"Dong Hyun Kim , Christoph Friedli","doi":"10.1016/j.nerep.2025.100254","DOIUrl":"10.1016/j.nerep.2025.100254","url":null,"abstract":"<div><h3>Introduction</h3><div>Herpes simplex encephalitis (HSE) is the most common cause of sporadic viral encephalitis, and can lead to auto-antibody generation. HSE has mainly been reported in association with N-methyl-D-aspartate receptor (NMDAR) antibodies, but there have also been cases associated with contactin-associated protein-like 2 (CASPR2). This article aims to highlight the relevance of CASPR2 antibody generation in HSE.</div></div><div><h3>Methods</h3><div>This is a case presentation and literature review of HSE with CASPR2 antibody positivity.</div></div><div><h3>Results</h3><div>A 62-year-old male initially presented with fever, headache and confusion in September 2023. He then developed status epilepticus requiring intubation. MRI of the brain showed extensive bilateral mesiotemporal lobe abnormalities with an haemorrhagic component. Cerebrospinal fluid (CSF) analysis was positive for herpes simplex virus 1 DNA, and CSF and serum were also positive for CASPR2 antibodies. He was treated with intravenous aciclovir for 3 weeks and short course of high dose intravenous dexamethasone. The patient continued to deteriorate despite treatment and died from the complications of the disease. In this case, it was difficult to ascertain if the patient had symptoms of CASPR2 associated disease or if it was an epiphenomenon triggered by HSE.</div></div><div><h3>Conclusion</h3><div>A number of case reports have described HSE with CASPR2 positivity. Whereas our patient succumbed to the disease, other cases showed clinical response with aciclovir, corticosteroids and intravenous immunoglobulin. More research on this topic is warranted as there are no specific treatment guidelines due to the lack of robust clinical trial data.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"7 ","pages":"Article 100254"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143680775","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}
A. Ali , L. Bernasconi , N. Cantoni , A.C. Lecourt , A.K. Pröbstel , A. Conen , O. Findling
{"title":"Persistent severe immunoglobulin deficiency after treatment with rituximab in a patient with MOGAD: A case report on how rituximab may, or may not, unmask primary immunodeficiencies","authors":"A. Ali , L. Bernasconi , N. Cantoni , A.C. Lecourt , A.K. Pröbstel , A. Conen , O. Findling","doi":"10.1016/j.nerep.2025.100258","DOIUrl":"10.1016/j.nerep.2025.100258","url":null,"abstract":"<div><div>We present the clinical scenario of a young female patient, who presented with a progressive sensory motor tetraparesis and fatigue. Upon investigation anti-MOG-associated disease (MOGAD) was diagnosed. After high dose intravenous steroid therapy, an immunosuppressive treatment with rituximab, in six-monthly cycles, was initiated. With this, the neurological condition stabilized without any further relapses. But the patient started to suffer from recurrent respiratory tract infections, even after the treatment with rituximab was paused for two years. Investigations revealed an underlying strikingly severe IgA deficiency alongside an IgG Deficiency.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"7 ","pages":"Article 100258"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144169606","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}
Ana Rita Castro , Daniela Oliveira , Bárbara Teixeira , João Macedo Cunha , Ivânia Alves , Luis Ruano
{"title":"MS developing after NMDAR encephalitis: A Portuguese case report and literature review","authors":"Ana Rita Castro , Daniela Oliveira , Bárbara Teixeira , João Macedo Cunha , Ivânia Alves , Luis Ruano","doi":"10.1016/j.nerep.2025.100256","DOIUrl":"10.1016/j.nerep.2025.100256","url":null,"abstract":"<div><div>Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder characterized by IgG autoantibodies targeting the GluN1 subunit of the NMDAR. We present a case of a 20-year-old male diagnosed with NMDAR encephalitis, who two years later developed symptoms and MRI lesions suggestive of multiple sclerosis (MS).</div><div>The patient initially exhibited depressive symptoms progressing to severe neuropsychiatric manifestations including catatonia, hallucinations, and dysphagia. Positive anti-NMDAR antibodies in the CSF and serum were found, and extensive investigations, including imaging and CSF analysis, ruled out alternative diagnoses. After treatment with methylprednisolone and intravenous immunoglobulins the patient recovered completely and remained asymptomatic until presenting two years later with optic neuritis and new demyelinating MRI lesions consistent with MS. The patient had negative tests for aquaporin-4 and myelin oligodendrocyte glycoprotein antibodies, and met 2017 McDonald criteria for MS.</div><div>This case adds to the sparse literature on overlap between NMDAR encephalitis and MS, suggesting a potential link between the autoimmune processes in these disorders. Further research is warranted to elucidate the underlying mechanisms and optimize management strategies for such cases.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"7 ","pages":"Article 100256"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143863354","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}
Dr. Jānis Dāvis Osipovs , Dr. Eva Šankova , Prof. Guntis Karelis , Dr. Ziedīte Želve , Dr. Elīna Polunosika , Mg. sc. sal. Līga Savicka
{"title":"Sudden onset orthostatic dysarthria as a presenting symptom of bulbar onset myasthenia gravis: a video case report","authors":"Dr. Jānis Dāvis Osipovs , Dr. Eva Šankova , Prof. Guntis Karelis , Dr. Ziedīte Želve , Dr. Elīna Polunosika , Mg. sc. sal. Līga Savicka","doi":"10.1016/j.nerep.2024.100235","DOIUrl":"10.1016/j.nerep.2024.100235","url":null,"abstract":"<div><div>Myasthenia gravis (MG) is a rare autoimmune neuromuscular disorder that can present with various early clinical symptoms and signs. The diagnosis of MG with bulbar symptom onset can be clinically challenging for clinicians with limited experience with neuromuscular disorders.</div><div>This case report presents an elderly patient with an abrupt onset of orthostatic dysarthria, in whom a high level of diagnostic suspicion leads to a short period until confirmation of diagnosis. This clinical case shows symptomatic treatment initiation on the 4th day after the onset of symptoms in a patient with bulbar onset MG. Serologic confirmation of MG was received on the 11th hospitalisation day. This case report contains a short video of the evaluation of orthostatic dysarthria.</div><div>Orthostatic dysarthria is a rare presentation symptom for bulbar onset MG. Information on the evaluation of orthostatic dysarthria is sparse. Considering that bulbar onset MG can be a life-threatening condition, we would like to share our positive experience of rapid diagnosis and successful treatment.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"7 ","pages":"Article 100235"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143104688","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}
Samar Antoine Abbas , Jad Costa , Joelle Saba , Christian Matta , Halim Abboud
{"title":"Longitudinally extensive transverse myelitis: Etiologies and common diagnostic pitfalls","authors":"Samar Antoine Abbas , Jad Costa , Joelle Saba , Christian Matta , Halim Abboud","doi":"10.1016/j.nerep.2025.100255","DOIUrl":"10.1016/j.nerep.2025.100255","url":null,"abstract":"<div><h3>Objectives</h3><div>report the different etiologies of Longitudinally Extensive Transverse Myelitis (LETM), along with their key characteristics.</div></div><div><h3>Methods</h3><div>A retrospective study was conducted at Hotel-Dieu de France University Hospital, Beirut, Lebanon. We collected demographic, clinical, radiologic and biochemical data of patients admitted for LETM between January 2021 and September 2024. Patients with compressive myelopathy or missing data were excluded. LETM characteristics were described in the whole sample, as well as in patients with inflammatory myelitis and those with spinal dural arteriovenous fistula (SDAVF).</div></div><div><h3>Results</h3><div>32 patients were selected, of whom 12 patients were excluded. The most common cause of LETM was SDAVF (25%), followed by MOG antibody-associated disease, multiple sclerosis, idiopathic transverse myelitis, neurosarcoidosis and infectious myelitis (10% each). There was only one case of Neuromyelitis Optica Spectrum Disorder (NMOSD). Other causes included MOG-negative acute disseminated encephalomyelitis, paraneoplastic myelitis, spinal cord neoplasm and subacute combined degeneration of spinal cord (5% each). In total, there were 13 cases of inflammatory myelitis (65%). Patients with SDAVF were predominantly males (80%) and relatively older than those with inflammatory myelitis. Different MRI patterns were described: all inflammatory LETM involved cervical and/or thoracic cord, 69% were partial and enhanced on postcontrast sequences. Vascular myelopathy affected thoracolumbar region in 80% of cases. Perimedullary flow voids were common (80%) but inconsistent.</div></div><div><h3>Conclusions</h3><div>Neurologist should think beyond NMOSD in case of LETM. The presence of thoracolumbar LETM with or without perimedullary flow voids requires MR angiography, before diagnostic lumbar puncture or empirical steroid therapy.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"7 ","pages":"Article 100255"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143738719","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}