Neuroimmunology Reports最新文献

英文中文

Oral cladribine for management of multiple sclerosis–Clinical experience from Hong Kong 口服克拉德滨治疗多发性硬化症——香港临床经验

Neuroimmunology Reports

Pub Date : 2025-01-01 DOI: 10.1016/j.nerep.2024.100238

Chun-Ho Choi , Jason Ka-Yeung Fong , Siu-Hung Li , Kary Ka-Wai Chan , Alexander Yuk-Lun Lau

Background

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.

Methods

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.

Results

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.

Conclusions

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.

背景：口服克拉德滨是一种选择性免疫重建疗法，为多发性硬化症（MS）的疾病改善治疗（DMT）提供了一种短期选择。多发性硬化症（MS）是一种慢性神经退行性疾病，以炎症和脱髓鞘为特征，影响全球280万人。尽管在2期和3期试验中证明了其安全性和有效性，但亚洲人群口服氯德里滨治疗的数据仍然有限。本病例系列旨在通过介绍在香港接受治疗的亚洲多发性硬化症患者，为疾病的临床谱提供真实的经验。方法：本回顾性病例系列包括2019年至2021年在香港诊断为复发-缓解型多发性硬化症并接受口服克拉德里滨治疗的9例患者。这些患者在公立和私立医院接受治疗，随访以评估口服克拉德里滨治疗的效果。从患者病历中提取相关数据。结果总体上是积极的，9例患者中有6例在治疗开始2-4年后显示稳定或改善的扩展残疾状态量表（EDSS）评分，7例在治疗开始2-5年内无复发。仅有2例患者出现轻度复发。后续MRI扫描未发现任何患者出现新的病变。1例短暂性淋巴细胞减少被注意到，这并不需要停止治疗。结论我们对这些患者的临床经验与临床试验报告的安全性和有效性结果一致。这些观察结果支持短期治疗方案在减轻患者总体治疗负担和提高治疗依从性方面的有效性。为了证实和建立这些发现，并在更大的队列中探索长期结果，有必要进一步研究和持续监测患者。

{"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}

引用次数: 0

Erratum to “Persistent spinal cord enhancement in longitudinal extensive transverse myelitis associated with α-1-antitrypisn deficiency: A case report” [Neuroimmunology Reports Volume 2, 2022, 100090] 对“α-1-抗胰蛋白酶缺乏相关的纵向广泛横贯脊髓炎的持续脊髓强化：一例报告”的勘误[神经免疫学报告第2卷，2022,100090]

Neuroimmunology Reports

Pub Date : 2025-01-01 DOI: 10.1016/j.nerep.2024.100243

Osman Ozel , Svetlana P. Eckert , Dejan Jakimovski , Nicholas Silvestri , David Hojnacki , Bianca Weinstock-Guttman

引用次数: 0

Contactin-associated protein-like 2 (CASPR2) antibody generation in acute herpes simplex encephalitis: Case report and review of the literature 急性单纯疱疹脑炎中接触相关蛋白样2 （CASPR2）抗体的产生：病例报告及文献复习

Neuroimmunology Reports

Pub Date : 2025-01-01 DOI: 10.1016/j.nerep.2025.100254

Dong Hyun Kim , Christoph Friedli

Introduction

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.

Methods

This is a case presentation and literature review of HSE with CASPR2 antibody positivity.

Results

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.

Conclusion

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.

简介：单纯疱疹病毒性脑炎（HSE）是散发性病毒性脑炎最常见的病因，可导致自身抗体的产生。HSE主要与n-甲基- d -天冬氨酸受体（NMDAR）抗体相关，但也有与接触蛋白相关蛋白样2 （CASPR2）相关的病例。本文旨在强调CASPR2抗体生成在HSE中的相关性。方法对CASPR2抗体阳性的HSE进行病例报告和文献复习。结果患者男性62岁，于2023年9月首次出现发热、头痛、意识不清等症状。随后他出现了需要插管的癫痫持续状态。脑部MRI显示广泛的双侧中颞叶异常伴出血成分。脑脊液（CSF）分析单纯疱疹病毒1型DNA阳性，CSF和血清CASPR2抗体也阳性。阿昔洛韦静脉注射3周，短疗程大剂量地塞米松静脉注射。尽管接受了治疗，病人的病情仍在恶化，最终死于该病的并发症。在这种情况下，很难确定患者是否有CASPR2相关疾病的症状，或者是否是HSE引发的附带现象。结论CASPR2阳性的HSE病例报告较多。虽然我们的病人死于这种疾病，但其他病例在阿昔洛韦、皮质类固醇和静脉注射免疫球蛋白后表现出临床反应。由于缺乏可靠的临床试验数据，没有具体的治疗指南，因此需要对这一主题进行更多的研究。

{"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}

引用次数: 0

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 1例MOGAD患者在接受利妥昔单抗治疗后出现持续严重的免疫球蛋白缺乏：一份关于利妥昔单抗如何揭示原发性免疫缺陷的病例报告

Neuroimmunology Reports

Pub Date : 2025-01-01 DOI: 10.1016/j.nerep.2025.100258

A. Ali , L. Bernasconi , N. Cantoni , A.C. Lecourt , A.K. Pröbstel , A. Conen , O. Findling

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.

我们提出了一个年轻的女性患者的临床方案，谁提出了进行性感觉运动四肢瘫痪和疲劳。经调查诊断为抗mog相关疾病（MOGAD）。在高剂量静脉注射类固醇治疗后，开始了以6个月为周期的利妥昔单抗免疫抑制治疗。有了这个，神经系统的状况稳定下来，没有任何进一步的复发。但患者开始出现复发性呼吸道感染，甚至在停止使用利妥昔单抗治疗两年之后。调查显示，潜在的严重IgA缺乏症和IgG缺乏症。

引用次数: 0

High-activity multiple sclerosis in feminizing hormone therapy for gender reassignment 高活性多发性硬化症女性化激素治疗的性别重新分配

Neuroimmunology Reports

Pub Date : 2025-01-01 DOI: 10.1016/j.nerep.2024.100234

Irene Expósito Ruiz, Cintia Tuñas Gesto, Alfredo Puy Núñez, Eva Costa Arpín

引用次数: 0

MS developing after NMDAR encephalitis: A Portuguese case report and literature review NMDAR脑炎后发生多发性硬化：葡萄牙病例报告及文献复习

Neuroimmunology Reports

Pub Date : 2025-01-01 DOI: 10.1016/j.nerep.2025.100256

Ana Rita Castro , Daniela Oliveira , Bárbara Teixeira , João Macedo Cunha , Ivânia Alves , Luis Ruano

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).

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.

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.

抗n -甲基- d -天冬氨酸受体（NMDAR）脑炎是一种自身免疫性疾病，其特征是IgG自身抗体靶向NMDAR的GluN1亚基。我们报告一例20岁男性诊断为NMDAR脑炎，两年后出现多发性硬化症（MS）的症状和MRI病变。患者最初表现为抑郁症状，并发展为严重的神经精神症状，包括紧张症、幻觉和吞咽困难。在脑脊液和血清中发现抗nmdar抗体阳性，广泛的调查，包括成像和脑脊液分析，排除了其他诊断。经甲基强的松龙和静脉注射免疫球蛋白治疗后，患者完全恢复，无症状，直到两年后出现视神经炎和新的脱髓鞘MRI病变，与MS一致。患者水通道蛋白-4和髓鞘少突胶质细胞糖蛋白抗体检测阴性，符合2017年MS的麦当劳标准。该病例增加了NMDAR脑炎与MS重叠的稀疏文献。提示这些疾病的自身免疫过程之间存在潜在联系需要进一步的研究来阐明潜在的机制和优化此类病例的管理策略。

{"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}

引用次数: 0

Sudden onset orthostatic dysarthria as a presenting symptom of bulbar onset myasthenia gravis: a video case report 突发性直立性构音障碍是球源性重症肌无力的主要症状：一个视频病例报告

Neuroimmunology Reports

Pub Date : 2025-01-01 DOI: 10.1016/j.nerep.2024.100235

Dr. Jānis Dāvis Osipovs , Dr. Eva Šankova , Prof. Guntis Karelis , Dr. Ziedīte Želve , Dr. Elīna Polunosika , Mg. sc. sal. Līga Savicka

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.

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.

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.

重症肌无力（MG）是一种罕见的自身免疫性神经肌肉疾病，可呈现各种早期临床症状和体征。对于神经肌肉疾病经验有限的临床医生来说，诊断伴有球症状的MG在临床上具有挑战性。这个病例报告提出了一个突然发作的直立性构音障碍的老年患者，在诊断的高度怀疑导致短时间内直到确诊。本临床病例为球源性MG患者，在症状出现后第4天开始对症治疗。在住院第11天接受了MG的血清学确认。本病例报告包含一个评估直立性构音障碍的短视频。直立性构音障碍是球源性MG的罕见表现。关于评价直立性构音障碍的信息很少。考虑到球源性MG可能危及生命，我们想分享我们快速诊断和成功治疗的积极经验。

{"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}

引用次数: 0

Longitudinally extensive transverse myelitis: Etiologies and common diagnostic pitfalls 纵向广泛横贯脊髓炎：病因和常见的诊断缺陷

Neuroimmunology Reports

Pub Date : 2025-01-01 DOI: 10.1016/j.nerep.2025.100255

Samar Antoine Abbas , Jad Costa , Joelle Saba , Christian Matta , Halim Abboud

Objectives

report the different etiologies of Longitudinally Extensive Transverse Myelitis (LETM), along with their key characteristics.

Methods

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).

Results

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.

Conclusions

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.

目的报道纵向广泛横断面脊髓炎（LETM）的不同病因及其主要特征。方法在黎巴嫩贝鲁特的法国主宫医院进行回顾性研究。我们收集了2021年1月至2024年9月期间入院的LETM患者的人口统计学、临床、放射学和生化数据。排除了压缩性脊髓病或数据缺失的患者。在整个样本中描述了LETM特征，以及炎症性脊髓炎和脊髓硬膜动静脉瘘（SDAVF）患者。结果入选32例，排除12例。LETM最常见的原因是SDAVF(25%)，其次是MOG抗体相关疾病、多发性硬化症、特发性横脊髓炎、神经结节病和感染性脊髓炎（各占10%）。视谱神经脊髓炎（NMOSD） 1例。其他病因包括mog阴性的急性播散性脑脊髓炎、副肿瘤脊髓炎、脊髓肿瘤和亚急性脊髓合并变性（各占5%）。炎性脊髓炎共13例（65%）。SDAVF患者以男性为主（80%），年龄相对于炎性脊髓炎患者。不同的MRI表现：所有炎性lem均累及颈髓和/或胸髓，69%为部分性，造影后增强。血管性脊髓病在80%的病例中累及胸腰椎区。髓周血流空是常见的（80%），但不一致。结论神经内科医师对LETM的治疗应超越NMOSD。在诊断性腰椎穿刺或经验性类固醇治疗之前，是否存在胸腰椎LETM伴或不伴髓周血流空洞需要磁共振血管造影。

{"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}

引用次数: 0

Comparison of visual outcomes in patients with aquaporin 4 immunoglobulin g-positive, myelin oligodendrocyte glycoprotein immunoglobulin g-positive, and double seronegative optic neuritis following severe optic neuritis 重度视神经炎后水通道蛋白4免疫球蛋白g阳性、髓鞘少突胶质细胞糖蛋白免疫球蛋白g阳性和双血清阴性视神经炎患者视力结局的比较

Neuroimmunology Reports

Pub Date : 2025-01-01 DOI: 10.1016/j.nerep.2025.100260

Rawipreeya Laosirirat, Metha Apiwattanakul, Saharat Aungsumart

Background

Data regarding idiopathic autoimmune-mediated optic neuritis is limited to visual acuity at specific time points post-treatment, lacking longitudinal comparisons. This study compared visual outcomes in patients with severe visual impairment due to optic neuritis who were aquaporin-4 (AQP4) immunoglobulin G (IgG)-positive, myelin oligodendrocyte glycoprotein (MOG)-IgG-positive, or double seronegative.

Methods

This retrospective longitudinal study was conducted at the Neurological Institute of Thailand, examining visual outcomes among three patient groups presenting with severe visual impairment—defined as best corrected visual acuity (BCVA) of 20/200 or worse—between June 2020 and May 2023. Visual outcomes were assessed with the “time to good visual recovery”—defined as ≥66.77 % improvement in BCVA from post-attack to baseline—and “complete visual recovery”—defined as BCVA returning to baseline.

Results

This study included 45 affected eyes of 30 patients, grouped as AQP4-IgG-positive (n = 10), MOG-IgG-positive (n = 5), and double seronegative (n = 30). Median BCVA at onset was 1.7 (logMAR). Using MOG-IgG as a comparator, the hazard ratios for complete visual recovery in the AQP4-IgG-positive and double seronegative groups were 0.158 (p = 0.135) and 0.421 (p = 0.288), respectively. For good visual recovery, the AQP4-IgG-positive and double seronegative subtypes had hazard ratios of 0.187 (p = 0.013) and 0.189 (p = 0.005), respectively, compared with the MOG-IgG-positive subtype. Furthermore, all MOG-IgG-positive cases achieved good visual recovery, in contrast to fewer than 50 % of the AQP4-IgG-positive and double seronegative subtypes.

Conclusion

The MOG-IgG-positive subtype exhibited the best visual prognosis and the shortest recovery time compared to the AQP4-IgG-positive and double seronegative subtypes.

背景：关于特发性自身免疫介导的视神经炎的数据仅限于治疗后特定时间点的视力，缺乏纵向比较。本研究比较了水通道蛋白-4 （AQP4）免疫球蛋白G （IgG）阳性、髓鞘少突胶质细胞糖蛋白(MOG)-IgG阳性或双血清阴性的视神经炎所致严重视力损害患者的视力结果。方法：这项回顾性纵向研究是在泰国神经学研究所进行的，研究了2020年6月至2023年5月期间，三组出现严重视力障碍的患者（定义为最佳矫正视力（BCVA）为20/200或更差）的视力结果。视力结果以“良好视力恢复时间”（定义为BCVA从发作后到基线改善≥66.77%）和“完全视力恢复”（定义为BCVA恢复到基线）进行评估。结果本研究纳入30例患者45只眼，分为aqp4 - igg阳性（n = 10）、mog - igg阳性（n = 5）和双血清阴性（n = 30）。发病时中位BCVA为1.7 （logMAR）。以MOG-IgG为对照，aqp4 - igg阳性组和双血清阴性组视力完全恢复的危险比分别为0.158 （p = 0.135）和0.421 （p = 0.288）。对于良好的视力恢复，aqp4 - igg阳性和双血清阴性亚型与mog - igg阳性亚型相比，风险比分别为0.187 （p = 0.013）和0.189 （p = 0.005）。此外，所有mog - igg阳性病例的视力恢复良好，而aqp4 - igg阳性和双血清阴性亚型的视力恢复不到50%。结论mog - igg阳性亚型与aqp4 - igg阳性和双血清阴性亚型相比，视力预后最好，恢复时间最短。

{"title":"Comparison of visual outcomes in patients with aquaporin 4 immunoglobulin g-positive, myelin oligodendrocyte glycoprotein immunoglobulin g-positive, and double seronegative optic neuritis following severe optic neuritis","authors":"Rawipreeya Laosirirat, Metha Apiwattanakul, Saharat Aungsumart","doi":"10.1016/j.nerep.2025.100260","DOIUrl":"10.1016/j.nerep.2025.100260","url":null,"abstract":"<div><h3>Background</h3><div>Data regarding idiopathic autoimmune-mediated optic neuritis is limited to visual acuity at specific time points post-treatment, lacking longitudinal comparisons. This study compared visual outcomes in patients with severe visual impairment due to optic neuritis who were aquaporin-4 (AQP4) immunoglobulin G (IgG)-positive, myelin oligodendrocyte glycoprotein (MOG)-IgG-positive, or double seronegative.</div></div><div><h3>Methods</h3><div>This retrospective longitudinal study was conducted at the Neurological Institute of Thailand, examining visual outcomes among three patient groups presenting with severe visual impairment—defined as best corrected visual acuity (BCVA) of 20/200 or worse—between June 2020 and May 2023. Visual outcomes were assessed with the “time to good visual recovery”—defined as ≥66.77 % improvement in BCVA from post-attack to baseline—and “complete visual recovery”—defined as BCVA returning to baseline.</div></div><div><h3>Results</h3><div>This study included 45 affected eyes of 30 patients, grouped as AQP4-IgG-positive (<em>n</em> = 10), MOG-IgG-positive (<em>n</em> = 5), and double seronegative (<em>n</em> = 30). Median BCVA at onset was 1.7 (logMAR). Using MOG-IgG as a comparator, the hazard ratios for complete visual recovery in the AQP4-IgG-positive and double seronegative groups were 0.158 (<em>p</em> = 0.135) and 0.421 (<em>p</em> = 0.288), respectively. For good visual recovery, the AQP4-IgG-positive and double seronegative subtypes had hazard ratios of 0.187 (<em>p</em> = 0.013) and 0.189 (<em>p</em> = 0.005), respectively, compared with the MOG-IgG-positive subtype. Furthermore, all MOG-IgG-positive cases achieved good visual recovery, in contrast to fewer than 50 % of the AQP4-IgG-positive and double seronegative subtypes.</div></div><div><h3>Conclusion</h3><div>The MOG-IgG-positive subtype exhibited the best visual prognosis and the shortest recovery time compared to the AQP4-IgG-positive and double seronegative subtypes.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"7 ","pages":"Article 100260"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144239683","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}

引用次数: 0

Erratum to “Transverse myelitis 48 hours after the administration of an mRNA COVID 19 vaccine” [Neuroimmunology Reports Volume 1, December 2021, 100019] “注射mRNA COVID - 19疫苗48小时后横贯脊髓炎”的勘误[神经免疫学报告第1卷，2021年12月，100019]

Neuroimmunology Reports

Pub Date : 2025-01-01 DOI: 10.1016/j.nerep.2024.100240

Patrick McLean, Lori Trefts

引用次数: 0

首页上一页

下一页尾页

类型

全部化学•材料生命科学医学物理工程技术环境•农林材料科学地球科学法学管理学化学环境科学与生态学计算机科学教育学经济学农林科学人文科学生物学数学物理与天体物理心理学综合性期刊其他工业工程理学历史学农学文学信息工程

数据库

全部 ACS Publications Elsevier ieeexplore Springer The Royal Society of Chemistry Wiley

期刊

Neuroimmunology Reports

全部 Acc. Chem. Res. ACS Applied Bio Materials ACS Appl. Electron. Mater. ACS Appl. Energy Mater. ACS Appl. Mater. Interfaces ACS Appl. Nano Mater. ACS Appl. Polym. Mater. ACS BIOMATER-SCI ENG ACS Catal. ACS Cent. Sci. ACS Chem. Biol. ACS Chemical Health & Safety ACS Chem. Neurosci. ACS Comb. Sci. ACS Earth Space Chem. ACS Energy Lett. ACS Infect. Dis. ACS Macro Lett. ACS Mater. Lett. ACS Med. Chem. Lett. ACS Nano ACS Omega ACS Photonics ACS Sens. ACS Sustainable Chem. Eng. ACS Synth. Biol. Anal. Chem. BIOCHEMISTRY-US Bioconjugate Chem. BIOMACROMOLECULES Chem. Res. Toxicol. Chem. Rev. Chem. Mater. CRYST GROWTH DES ENERG FUEL Environ. Sci. Technol. Environ. Sci. Technol. Lett. Eur. J. Inorg. Chem. IND ENG CHEM RES Inorg. Chem. J. Agric. Food. Chem. J. Chem. Eng. Data J. Chem. Educ. J. Chem. Inf. Model. J. Chem. Theory Comput. J. Med. Chem. J. Nat. Prod. J PROTEOME RES J. Am. Chem. Soc. LANGMUIR MACROMOLECULES Mol. Pharmaceutics Nano Lett. Org. Lett. ORG PROCESS RES DEV ORGANOMETALLICS J. Org. Chem. J. Phys. Chem. J. Phys. Chem. A J. Phys. Chem. B J. Phys. Chem. C J. Phys. Chem. Lett. Analyst Anal. Methods Biomater. Sci. Catal. Sci. Technol. Chem. Commun. Chem. Soc. Rev. CHEM EDUC RES PRACT CRYSTENGCOMM Dalton Trans. Energy Environ. Sci. ENVIRON SCI-NANO ENVIRON SCI-PROC IMP ENVIRON SCI-WAT RES Faraday Discuss. Food Funct. Green Chem. Inorg. Chem. Front. Integr. Biol. J. Anal. At. Spectrom. J. Mater. Chem. A J. Mater. Chem. B J. Mater. Chem. C Lab Chip Mater. Chem. Front. Mater. Horiz. MEDCHEMCOMM Metallomics Mol. Biosyst. Mol. Syst. Des. Eng. Nanoscale Nanoscale Horiz. Nat. Prod. Rep. New J. Chem. Org. Biomol. Chem. Org. Chem. Front. PHOTOCH PHOTOBIO SCI PCCP Polym. Chem.

﹀