Pub Date : 2024-10-18DOI: 10.1016/j.jneuroim.2024.578467
Seongmi Kim , Suin Lee , Yeon Hak Chung , Hyunjin Ju , Yeon-Lim Suh , Ju-Hong Min
Primary angiitis of the central nervous system (PACNS) is a rare inflammatory disease that affects both small- and medium-sized vessels of the CNS, while myelin oligodendrocyte glycoprotein (MOG) antibody–associated disease (MOGAD) is a novel antibody-mediated inflammatory demyelinating disorder that causes damage to the myelin in CNS. We report a case diagnosed as MOGAD due to a history of recurrent myelitis, brain lesions, and positive anti-MOG, but the brain biopsy showed vasculitis without demyelination.
{"title":"Myelin oligodendrocyte glycoprotein antibody–associated disease with histopathologic features of primary CNS angiitis without demyelination: Case report and literature review","authors":"Seongmi Kim , Suin Lee , Yeon Hak Chung , Hyunjin Ju , Yeon-Lim Suh , Ju-Hong Min","doi":"10.1016/j.jneuroim.2024.578467","DOIUrl":"10.1016/j.jneuroim.2024.578467","url":null,"abstract":"<div><div>Primary angiitis of the central nervous system (PACNS) is a rare inflammatory disease that affects both small- and medium-sized vessels of the CNS, while myelin oligodendrocyte glycoprotein (MOG) antibody–associated disease (MOGAD) is a novel antibody-mediated inflammatory demyelinating disorder that causes damage to the myelin in CNS. We report a case diagnosed as MOGAD due to a history of recurrent myelitis, brain lesions, and positive anti-MOG, but the brain biopsy showed vasculitis without demyelination.</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"396 ","pages":"Article 578467"},"PeriodicalIF":2.9,"publicationDate":"2024-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142502426","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-10-15DOI: 10.1016/j.jneuroim.2024.578466
Ali Emre Köse , Tayfun Turan , Eser Kilic
High Mobility Group Box Protein-1 (HMGB1), which has proinflammatory properties, is known to be involved in psychiatric disorders as far as we know, there are only one clinical studies investigating the role of HMGB1 in major depressive disorder (MDD). In this study, we aimed to investigate the role of HMGB1 in the etiopathogenesis of MDD and whether HMGB1 can be used as a biomarker in MDD by measuring the serum HMGB1 levels of depressed patients in the episode and remission periods. This study included 30 patients diagnosed with MDD in episode, 30 patients in remission and 30 healthy controls. Each group comprised 20 female and 10 male participants. In this study, serum HMGB1 levels were found to be lower in the patient group in the episode compared to the patient group in the remission period and the healthy control group. There was no significant difference between the patient group in remission and the healthy control group in terms of serum HMGB1 levels. The fact that serum HMGB1 levels were lower in the patient group in the episode compared to the patient group in the remission period and the control group may be related to the neuroprotective effects of HMGB1. HMGB1 may be used as a biomarker for MDD.
{"title":"May high mobility group box protein-1 be a biomarker for major depressive disorder?","authors":"Ali Emre Köse , Tayfun Turan , Eser Kilic","doi":"10.1016/j.jneuroim.2024.578466","DOIUrl":"10.1016/j.jneuroim.2024.578466","url":null,"abstract":"<div><div>High Mobility Group Box Protein-1 (HMGB1), which has proinflammatory properties, is known to be involved in psychiatric disorders as far as we know, there are only one clinical studies investigating the role of HMGB1 in major depressive disorder (MDD). In this study, we aimed to investigate the role of HMGB1 in the etiopathogenesis of MDD and whether HMGB1 can be used as a biomarker in MDD by measuring the serum HMGB1 levels of depressed patients in the episode and remission periods. This study included 30 patients diagnosed with MDD in episode, 30 patients in remission and 30 healthy controls. Each group comprised 20 female and 10 male participants. In this study, serum HMGB1 levels were found to be lower in the patient group in the episode compared to the patient group in the remission period and the healthy control group. There was no significant difference between the patient group in remission and the healthy control group in terms of serum HMGB1 levels. The fact that serum HMGB1 levels were lower in the patient group in the episode compared to the patient group in the remission period and the control group may be related to the neuroprotective effects of HMGB1. HMGB1 may be used as a biomarker for MDD.</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"396 ","pages":"Article 578466"},"PeriodicalIF":2.9,"publicationDate":"2024-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142468198","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-10-09DOI: 10.1016/j.jneuroim.2024.578464
Yingkai Li , Pei Chen , Xin Huang , Hao Huang , Qian Ma , Zhongqiang Lin , Li Qiu , Changyi Ou , Weibin Liu
In our study, we investigated the impact of tacrolimus (TAC) on CD4+ T cell subsets in 41 AChR-MG patients over 12 weeks. Twenty-seven patients were classified as the response group (RG) (improved myasthenia gravis composite scores ≥3), while 14 were non-response. We found that TAC treatment significantly reduced Th17 and pathogenic Th17 cells, along with IL-17 levels in RG, while Th1 and Tfh cells slightly decreased without affecting Th2 or Treg subsets. This indicates that TAC's clinical benefits may be due to its inhibitory effect on the Th17 response, enhancing our insight into its immunomodulatory mechanisms in MG management.
{"title":"Pathogenic Th17 cells are a potential therapeutic target for tacrolimus in AChR-myasthenia gravis patients","authors":"Yingkai Li , Pei Chen , Xin Huang , Hao Huang , Qian Ma , Zhongqiang Lin , Li Qiu , Changyi Ou , Weibin Liu","doi":"10.1016/j.jneuroim.2024.578464","DOIUrl":"10.1016/j.jneuroim.2024.578464","url":null,"abstract":"<div><div>In our study, we investigated the impact of tacrolimus (TAC) on CD4+ T cell subsets in 41 AChR-MG patients over 12 weeks. Twenty-seven patients were classified as the response group (RG) (improved myasthenia gravis composite scores ≥3), while 14 were non-response. We found that TAC treatment significantly reduced Th17 and pathogenic Th17 cells, along with IL-17 levels in RG, while Th1 and Tfh cells slightly decreased without affecting Th2 or Treg subsets. This indicates that TAC's clinical benefits may be due to its inhibitory effect on the Th17 response, enhancing our insight into its immunomodulatory mechanisms in MG management.</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"396 ","pages":"Article 578464"},"PeriodicalIF":2.9,"publicationDate":"2024-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142406495","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-10-04DOI: 10.1016/j.jneuroim.2024.578463
Yan Lin , Xiajun Zhou , Jun Wu, Yufang Mei, Liping Ni, Huiying Qiu, Yan Zhou, Ying Chen, Wenbin Wan
Objective
This study aims to evaluate the effectiveness of double-filtration plasmapheresis (DFPP) in reducing immunoglobulins and culprit antibodies in neuroimmune disorders.
Methods
A retrospective analysis was conducted on 51 patients with neuroimmune diseases treated with DFPP, immunotherapy, and symptomatic treatment. Immunoglobulin and antibody levels were measured pre- and post-treatment, along with neurological function assessments using scales like the modified Rankin Scale (mRS), Expanded Disability Status Scale (EDSS), Clinical Assessment Scale for Autoimmune Encephalitis (CASE), and Myasthenia Gravis-specific scales.
Results
The cohort included patients with neuromyelitis optica spectrum disorder (NMOSD), autoimmune encephalitis (AIE), myasthenia gravis (MG), anti-myelin oligodendrocyte glycoprotein associated disease (MOGAD), and paraneoplastic neurological syndromes (PNS). DFPP significantly reduced immunoglobulin levels (IgG, IgA, IgM) by ∼70 %. Most patients showed decreased antibody titers and significant neurological improvement. The median mRS score improved from 2 (IQR 2–3) to 1 (IQR 1–2) post-treatment, with further improvement at 90 days. Notable improvements were observed across various scales specific to NMOSD, MOGAD, AIE, and MG. Minor adverse events were reported, with no serious adverse events.
Conclusions
DFPP is effective in reducing immunoglobulin and antibody levels, leading to improved neurological function in neuroimmune disorders. Further large-scale studies are warranted to confirm these findings.
{"title":"Effectiveness of double-filtration plasmapheresis in reducing immunoglobulin and culprit antibody levels in neuroimmune disorders: A single-center retrospective analysis from China","authors":"Yan Lin , Xiajun Zhou , Jun Wu, Yufang Mei, Liping Ni, Huiying Qiu, Yan Zhou, Ying Chen, Wenbin Wan","doi":"10.1016/j.jneuroim.2024.578463","DOIUrl":"10.1016/j.jneuroim.2024.578463","url":null,"abstract":"<div><h3>Objective</h3><div>This study aims to evaluate the effectiveness of double-filtration plasmapheresis (DFPP) in reducing immunoglobulins and culprit antibodies in neuroimmune disorders.</div></div><div><h3>Methods</h3><div>A retrospective analysis was conducted on 51 patients with neuroimmune diseases treated with DFPP, immunotherapy, and symptomatic treatment. Immunoglobulin and antibody levels were measured pre- and post-treatment, along with neurological function assessments using scales like the modified Rankin Scale (mRS), Expanded Disability Status Scale (EDSS), Clinical Assessment Scale for Autoimmune Encephalitis (CASE), and Myasthenia Gravis-specific scales.</div></div><div><h3>Results</h3><div>The cohort included patients with neuromyelitis optica spectrum disorder (NMOSD), autoimmune encephalitis (AIE), myasthenia gravis (MG), anti-myelin oligodendrocyte glycoprotein associated disease (MOGAD), and paraneoplastic neurological syndromes (PNS). DFPP significantly reduced immunoglobulin levels (IgG, IgA, IgM) by ∼70 %. Most patients showed decreased antibody titers and significant neurological improvement. The median mRS score improved from 2 (IQR 2–3) to 1 (IQR 1–2) post-treatment, with further improvement at 90 days. Notable improvements were observed across various scales specific to NMOSD, MOGAD, AIE, and MG. Minor adverse events were reported, with no serious adverse events.</div></div><div><h3>Conclusions</h3><div>DFPP is effective in reducing immunoglobulin and antibody levels, leading to improved neurological function in neuroimmune disorders. Further large-scale studies are warranted to confirm these findings.</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"396 ","pages":"Article 578463"},"PeriodicalIF":2.9,"publicationDate":"2024-10-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142422735","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-09-30DOI: 10.1016/j.jneuroim.2024.578462
Kelly N. Brice , Paige N. Braden-Kuhle , Shelby K. Miller , Allison Regan , Vivienne Lacy , Michael J. Chumley , Gary W. Boehm
Although adequate sleep is imperative for proper physiological function, over one-third of US adults obtain insufficient sleep. The current research investigated the impact of chronic sleep restriction (CSR) on inflammatory markers and hippocampal BDNF mRNA, following an immune insult in both male and female mice. Patterns of cytokine expression were different when the study was done in males vs. females, indicating potential sex differences in the inflammatory response following CSR. Further, CSR led to suppressed hippocampal BDNF expression in males, an effect not observed in females. These data suggest a complex interaction between chronic sleep loss, inflammation, and sex that warrants further exploration.
{"title":"Chronic sleep loss alters the inflammatory response and BDNF expression in C57BL/6J mice","authors":"Kelly N. Brice , Paige N. Braden-Kuhle , Shelby K. Miller , Allison Regan , Vivienne Lacy , Michael J. Chumley , Gary W. Boehm","doi":"10.1016/j.jneuroim.2024.578462","DOIUrl":"10.1016/j.jneuroim.2024.578462","url":null,"abstract":"<div><div>Although adequate sleep is imperative for proper physiological function, over one-third of US adults obtain insufficient sleep. The current research investigated the impact of chronic sleep restriction (CSR) on inflammatory markers and hippocampal BDNF mRNA, following an immune insult in both male and female mice. Patterns of cytokine expression were different when the study was done in males vs. females, indicating potential sex differences in the inflammatory response following CSR. Further, CSR led to suppressed hippocampal BDNF expression in males, an effect not observed in females. These data suggest a complex interaction between chronic sleep loss, inflammation, and sex that warrants further exploration.</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"396 ","pages":"Article 578462"},"PeriodicalIF":2.9,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142391297","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-09-18DOI: 10.1016/j.jneuroim.2024.578460
Ying Liu , Chun-Lin Yang , Xue-Lu Zhao , Yuan-Jing Zhao , Tong Du , Cong-Cong Wang , Xue-Min Li , Yu-Dong Liu , Rui-Sheng Duan , Bing Yang , Xiao-Li Li
Background
Autoimmune nodopathy (AN) is a very rare new disease entity, especially when combined with membranous nephropathy (MN).
Methods
Antibodies against nodal-paranodal cell adhesion molecules in the serum were detected using cell-based assays. Antibody subtypes against contactin-1 (CNTN1) were confirmed. Cases of anti-CNTN1 antibody-positive AN with and without MN were retrieved through a literature search to compare clinical and electrophysiological characteristics.
Results
A 65-year-old male patient with MN developed limb numbness and weakness, along with walking instability. Serum CNTN1 antibodies were positive, primarily those of the IgG4 subtype. Electromyography showed prominent demyelination patterns in both the proximal and distal segments of the nerves compared to the middle nerve trunk. Magnetic resonance imaging revealed enlargement of the bilateral brachial and lumbosacral plexuses and local hyperintensity of the right C5-C6 nerve roots. Thirty-five cases with anti-CNTN1 antibody-positive AN with MN and 51 cases with anti-CNTN1 antibody-positive AN without MN were compared. Furthermore, the proportion of patients with MN combined with AN presenting with acute or subacute onset was higher than that observed in the MN without AN group. Nevertheless, no substantial differences were noted between the two groups concerning the clinical and electrophysiological characteristics, which were mainly elderly men, manifested as sensory ataxia, IgG4 antibody subtype, electrophysiological demyelination, and a certain effect on immunotherapy.
Conclusion
In cases of electrophysiological manifestation of demyelinating peripheral neuropathy, especially in distal and poximal segments of nerves, AN should be considered, and further screening for renal function should be performed. Concomitant MN does not aggravate or alleviate peripheral nerve symptoms.
背景自身免疫性结节病（AN）是一种非常罕见的新疾病，尤其是在合并膜性肾病（MN）的情况下。方法使用细胞检测法检测血清中针对结节-副结节细胞粘附分子的抗体。证实了针对接触素-1（CNTN1）的抗体亚型。通过文献检索找到了抗 CNTN1 抗体阳性 AN（伴有和不伴有 MN）病例，以比较临床和电生理特征。血清 CNTN1 抗体呈阳性，主要是 IgG4 亚型。肌电图显示，与中神经干相比，近端和远端神经节段均有突出的脱髓鞘模式。磁共振成像显示双侧肱神经丛和腰骶神经丛增大，右侧C5-C6神经根局部高密度。研究人员比较了 35 例抗 CNTN1 抗体阳性 AN 伴 MN 患者和 51 例抗 CNTN1 抗体阳性 AN 无 MN 患者。此外，MN 合并 AN 患者中急性或亚急性发病的比例高于无 AN 的 MN 组。尽管如此，两组患者在临床和电生理特点方面并无本质区别，均以老年男性为主，表现为感觉性共济失调、IgG4 抗体亚型、电生理脱髓鞘以及对免疫治疗有一定影响。并发 MN 不会加重或减轻周围神经症状。
{"title":"Characteristics of anti-contactin1 antibody positive autoimmune nodopathies combined with membranous nephropathy","authors":"Ying Liu , Chun-Lin Yang , Xue-Lu Zhao , Yuan-Jing Zhao , Tong Du , Cong-Cong Wang , Xue-Min Li , Yu-Dong Liu , Rui-Sheng Duan , Bing Yang , Xiao-Li Li","doi":"10.1016/j.jneuroim.2024.578460","DOIUrl":"10.1016/j.jneuroim.2024.578460","url":null,"abstract":"<div><h3>Background</h3><div>Autoimmune nodopathy (AN) is a very rare new disease entity, especially when combined with membranous nephropathy (MN).</div></div><div><h3>Methods</h3><div>Antibodies against nodal-paranodal cell adhesion molecules in the serum were detected using cell-based assays. Antibody subtypes against contactin-1 (CNTN1) were confirmed. Cases of anti-CNTN1 antibody-positive AN with and without MN were retrieved through a literature search to compare clinical and electrophysiological characteristics.</div></div><div><h3>Results</h3><div>A 65-year-old male patient with MN developed limb numbness and weakness, along with walking instability. Serum CNTN1 antibodies were positive, primarily those of the IgG4 subtype. Electromyography showed prominent demyelination patterns in both the proximal and distal segments of the nerves compared to the middle nerve trunk. Magnetic resonance imaging revealed enlargement of the bilateral brachial and lumbosacral plexuses and local hyperintensity of the right C5-C6 nerve roots. Thirty-five cases with anti-CNTN1 antibody-positive AN with MN and 51 cases with anti-CNTN1 antibody-positive AN without MN were compared. Furthermore, the proportion of patients with MN combined with AN presenting with acute or subacute onset was higher than that observed in the MN without AN group. Nevertheless, no substantial differences were noted between the two groups concerning the clinical and electrophysiological characteristics, which were mainly elderly men, manifested as sensory ataxia, IgG4 antibody subtype, electrophysiological demyelination, and a certain effect on immunotherapy.</div></div><div><h3>Conclusion</h3><div>In cases of electrophysiological manifestation of demyelinating peripheral neuropathy, especially in distal and poximal segments of nerves, AN should be considered, and further screening for renal function should be performed. Concomitant MN does not aggravate or alleviate peripheral nerve symptoms.</div></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"396 ","pages":"Article 578460"},"PeriodicalIF":2.9,"publicationDate":"2024-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142310887","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-09-11DOI: 10.1016/j.jneuroim.2024.578459
Trajano Aguiar Pires Gonçalves , Juliana Naback Toniolo , Matheus Compart Hemerly , Maria Clara Zanon Zotin , Anna Letícia de Moraes Alves , Katharina Vieira Messias , Vanessa Daccach Marques
Intravascular large B-cell lymphoma (IVLBCL) is a rare hematological malignancy where its development in the intravascular environment is the main characteristic. Despite its ability to affect multiple organic systems, there is a tropism for the central nervous system, which may be related to several clinical syndromes, making this condition a great mimic and consequently a diagnostic challenge. Rapidly progressive dementia may be one of the presenting phenotypes of IVLBCL. This case report aims to highlight the main red flags, such as sustained elevation of lactate dehydrogenase, organomegaly and specific lesions with vasculitis-like bleeding, all that can be used as clinical clues to direct the differential diagnosis. In addition, it reinforces the role of early brain biopsy in this context, since IVLBCL is a treatable disease.
血管内大 B 细胞淋巴瘤（IVLBCL）是一种罕见的血液恶性肿瘤，其主要特征是在血管内环境中发展。尽管它能影响多个器官系统，但它对中枢神经系统有趋向性，这可能与多种临床综合征有关，因此这种疾病的模仿性很强，也是诊断上的一个难题。快速进展性痴呆可能是IVLBCL的表现型之一。本病例报告旨在强调主要的警示信号，如乳酸脱氢酶持续升高、器官肿大和伴有血管炎样出血的特殊病变，所有这些都可以作为临床线索来指导鉴别诊断。此外，报告还强调了早期脑活检在这种情况下的作用，因为IVLBCL是一种可以治疗的疾病。
{"title":"Intravascular lymphoma: A diagnostic challenge for a treatable cause of rapidly progressive dementia","authors":"Trajano Aguiar Pires Gonçalves , Juliana Naback Toniolo , Matheus Compart Hemerly , Maria Clara Zanon Zotin , Anna Letícia de Moraes Alves , Katharina Vieira Messias , Vanessa Daccach Marques","doi":"10.1016/j.jneuroim.2024.578459","DOIUrl":"10.1016/j.jneuroim.2024.578459","url":null,"abstract":"<div><p>Intravascular large B-cell lymphoma (IVLBCL) is a rare hematological malignancy where its development in the intravascular environment is the main characteristic. Despite its ability to affect multiple organic systems, there is a tropism for the central nervous system, which may be related to several clinical syndromes, making this condition a great mimic and consequently a diagnostic challenge. Rapidly progressive dementia may be one of the presenting phenotypes of IVLBCL. This case report aims to highlight the main red flags, such as sustained elevation of lactate dehydrogenase, organomegaly and specific lesions with vasculitis-like bleeding, all that can be used as clinical clues to direct the differential diagnosis. In addition, it reinforces the role of early brain biopsy in this context, since IVLBCL is a treatable disease.</p></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"396 ","pages":"Article 578459"},"PeriodicalIF":2.9,"publicationDate":"2024-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142244230","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-09-11DOI: 10.1016/j.jneuroim.2024.578458
Rong Lai , Zichao Wu , Haiyan Wang , Li Feng , Xunsha Sun , Cunzhou Shen , Huiyu Feng , Hongyan Zhou
Rituximab is recommended as the preferred second-line immunotherapy for autoimmune encephalitis (AE). However, Ofatumumab (OFA), a novel fully human anti-CD20 antibody, has been reported infrequently in patients with AE. Among the various forms of AE, anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis is the most common and severe. This study presents three cases of severe anti-NMDAR encephalitis treated with OFA following the failure of first-line immunotherapy. The results indicated that the patients experienced no significant adverse reactions after receiving OFA, and their clinical symptoms improved markedly within one week of treatment. One month post-treatment with OFA, scores on the Glasgow Coma Scale (GCS) and the Barthel Index of Activities of Daily Living (Barthel-ADL) increased, while scores on the modified Rankin Scale (mRS), Clinical Assessment Scale in Autoimmune Encephalitis (CASE), and Paroxysmal Sympathetic Hyperactivity Assessment Measure (PSH-AM) decreased. During the three-month and six-month follow-up periods, patients exhibited further symptomatic improvement, suggesting that OFA is a safe and effective treatment option for anti-NMDAR encephalitis. These findings propose a novel therapeutic strategy for severe refractory anti-NMDAR encephalitis.
{"title":"Ofatumumab treatment for severe refractory anti-NMDAR encephalitis: A case series","authors":"Rong Lai , Zichao Wu , Haiyan Wang , Li Feng , Xunsha Sun , Cunzhou Shen , Huiyu Feng , Hongyan Zhou","doi":"10.1016/j.jneuroim.2024.578458","DOIUrl":"10.1016/j.jneuroim.2024.578458","url":null,"abstract":"<div><p>Rituximab is recommended as the preferred second-line immunotherapy for autoimmune encephalitis (AE). However, Ofatumumab (OFA), a novel fully human anti-CD20 antibody, has been reported infrequently in patients with AE. Among the various forms of AE, anti-<em>N</em>-methyl-<span>d</span>-aspartate receptor (anti-NMDAR) encephalitis is the most common and severe. This study presents three cases of severe anti-NMDAR encephalitis treated with OFA following the failure of first-line immunotherapy. The results indicated that the patients experienced no significant adverse reactions after receiving OFA, and their clinical symptoms improved markedly within one week of treatment. One month post-treatment with OFA, scores on the Glasgow Coma Scale (GCS) and the Barthel Index of Activities of Daily Living (Barthel-ADL) increased, while scores on the modified Rankin Scale (mRS), Clinical Assessment Scale in Autoimmune Encephalitis (CASE), and Paroxysmal Sympathetic Hyperactivity Assessment Measure (PSH-AM) decreased. During the three-month and six-month follow-up periods, patients exhibited further symptomatic improvement, suggesting that OFA is a safe and effective treatment option for anti-NMDAR encephalitis. These findings propose a novel therapeutic strategy for severe refractory anti-NMDAR encephalitis.</p></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"396 ","pages":"Article 578458"},"PeriodicalIF":2.9,"publicationDate":"2024-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142228496","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Agrin is essential for neuromuscular junction (NMJ) formation and maintenance. The C-terminal agrin fragment (CAF), generated by neurotrypsin-mediated cleavage of agrin, has been gaining attention as a potential biomarker for sarcopenia. We investigated serum CAF levels in myasthenia gravis (MG), a NMJ disorder. Compared to healthy controls, serum CAF levels were significantly elevated in acetylcholine receptor antibody-positive MG (AChR-MG) patients, but not in muscle-specific kinase antibody-positive MG patients. In AChR-MG, baseline and post-treatment CAF levels inversely correlated with post-treatment MG activities of daily living scores, suggesting that elevated CAF levels may reflect protective mechanisms against AChR-MG pathogenesis, such as improved NMJ regeneration.
{"title":"Elevated serum levels of C-terminal agrin fragment in acetylcholine receptor antibody-positive myasthenia gravis","authors":"Manato Yasuda , Akiyuki Uzawa , Yosuke Onishi , Hideo Handa , Hiroyuki Akamine , Etsuko Ogaya , Yukiko Ozawa , Hiroki Masuda , Masahiro Mori , Satoshi Kuwabara","doi":"10.1016/j.jneuroim.2024.578455","DOIUrl":"10.1016/j.jneuroim.2024.578455","url":null,"abstract":"<div><p>Agrin is essential for neuromuscular junction (NMJ) formation and maintenance. The C-terminal agrin fragment (CAF), generated by neurotrypsin-mediated cleavage of agrin, has been gaining attention as a potential biomarker for sarcopenia. We investigated serum CAF levels in myasthenia gravis (MG), a NMJ disorder. Compared to healthy controls, serum CAF levels were significantly elevated in acetylcholine receptor antibody-positive MG (AChR-MG) patients, but not in muscle-specific kinase antibody-positive MG patients. In AChR-MG, baseline and post-treatment CAF levels inversely correlated with post-treatment MG activities of daily living scores, suggesting that elevated CAF levels may reflect protective mechanisms against AChR-MG pathogenesis, such as improved NMJ regeneration.</p></div>","PeriodicalId":16671,"journal":{"name":"Journal of neuroimmunology","volume":"396 ","pages":"Article 578455"},"PeriodicalIF":2.9,"publicationDate":"2024-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S0165572824001747/pdfft?md5=fba0b36fc84af680557ddf7bacb73955&pid=1-s2.0-S0165572824001747-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142228607","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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