Review
Immunology
Andreas J. Steck
Summary: Acquired chronic demyelinating neuropathies encompass a growing number of disease entities with characteristic, often overlapping clinical presentations, mediated by distinct immune mechanisms and responding to different therapies. Recently, a novel antigen-specific immunotherapy targeting anti-MAG antibodies with a carbohydrate-based ligand mimicking the natural HNK-1 glycoepitope has been described, showing promising results in treating this condition.
JOURNAL OF NEUROIMMUNOLOGY
(2021)
Review
Immunology
Meihui Xu, Chi Ma, Ming Dong, Chunjie Guo, Simin Yang, Yue Liu, Xu Wang
Summary: This study aims to characterize the clinical symptoms, MRI findings, and prognosis of adult cerebral cortical encephalitis (CCE) with anti-MOG antibody. Two adult cases of CCE with anti-MOG antibody were presented and a systematic review of the literature was conducted. The majority of patients had a favorable prognosis after treatment, highlighting the importance of early detection and immunotherapy in improving outcomes.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Clinical Neurology
Giorgia Teresa Maniscalco, Lia Allegorico, Gennaro Alfieri, Massimo Napolitano, Angelo Ranieri, Rosaria Renna, Giovanna Servillo, Marianna Pezzella, Elisa Capone, Lidia Altomare, Michele Spiniello, Sergio Ferrari, Valentino Manzo, Sara Mariotto
Summary: Anti-myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD) are new emerging diseases with heterogeneous course, treatment, response, and prognosis. Early diagnosis and timely treatment are fundamental to improving clinical outcome for this extremely variable disease.
NEUROLOGICAL SCIENCES
(2021)
Review
Clinical Neurology
Norman Latov
Summary: The diagnosis of Myelin-Associated Glycoprotein (MAG) neuropathy is primarily based on elevated IgM anti-MAG antibody titers, often associated with IgM monoclonal gammopathy and a slowly progressive, distal demyelinating phenotype. Recent advancements have shown that higher antibody levels are more indicative of typical MAG phenotype or treatment response, and testing for cross-reactivity with HNK1 can enhance assay specificity. It is also important to consider the diagnosis in patients with demyelinating neuropathy, even without monoclonal gammopathy or typical phenotype, and to monitor antibody levels for response to B-cell depleting therapy.
CURRENT OPINION IN NEUROLOGY
(2021)
Article
Clinical Neurology
Karima Amaador, Luuk Wieske, Marleen J. A. Koel-Simmelink, A. Kamp, Ilse Jongerius, Koen de Heer, Charlotte E. Teunissen, Monique C. Minnema, Nicolette C. Notermans, Filip Eftimov, Marie Jose Kersten, Josephine M. Vos
Summary: In this prospective cohort study of treatment-naive patients with anti-MAG PN, serum NfL, CNTN1, C3b/c and C4b/c levels did not show potential as biomarkers, indicating that further research with larger cohorts and longitudinal follow-up may be needed.
JOURNAL OF NEUROLOGY
(2022)
Review
Immunology
Shujiang Zhang, Yuan Yang, Wenyu Liu, Zuoxiao Li, Jinmei Li, Dong Zhou
Summary: Anti-N-methyl-d-aspartate receptor encephalitis (NMDARe) is commonly accompanied by demyelinating disorders, including multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). The clinical characteristics of patients with different overlapping syndromes were compared. Patients with overlapping syndromes showed atypical symptoms and a lower ratio of ovarian teratoma compared to classical NMDARe. Patients with MOGAD overlap were younger, while patients with MS and AQP4-Ab-positive NMOSD overlap tended to be older. Majority of female patients overlapped with MS or AQP4-Ab-positive NMOSD, but not in the case of MOGAD. The interval between NMDARe and demyelinating diseases was longest in patients with NMDARe overlapped with MS. Further investigation is needed for the long-term prognosis of overlapping syndromes.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Clinical Neurology
Pascal Haenggi, Butrint Aliu, Kea Martin, Ruben Herrendorff, Andreas Johann Steck
Summary: The retrospective analysis confirmed the hypothesis that a relative reduction in serum anti-MAG IgM antibodies is associated with a clinical response to immunotherapies; a sustained reduction of at least 50% compared with pretreatment titers or levels could be a valuable indicator for therapeutic response.
NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION
(2022)
Article
Clinical Neurology
Liang Wang, Hongmei Tan, Wenjuan Huang, Xuechun Chang, Jingzi ZhangBao, Lei Zhou, Chuanzhen Lu, Min Wang, Jiahong Lu, Chongbo Zhao, Chao Quan
Summary: This study evaluated the clinical characteristics and prognosis of late onset neuromyelitis optica spectrum disorder (LO-NMOSD) and compared them with early onset NMOSD (EO-NMOSD) and NMOSD with different antibody serostatuses. The study found that patients with LO-NMOSD, especially those with AQP4 antibodies, had a worse prognosis. Age at disease onset and antibody serostatus were predictors of blindness and motor dysfunction.
EUROPEAN JOURNAL OF NEUROLOGY
(2022)
Article
Clinical Neurology
D. Doig, C. McNamara, L. Mewasingh, S. Beri, B. Jones, C. Kachramanoglou, W. Jan
Summary: This study described two children presenting with generalized seizures due to cortical encephalitis, both of whom were found to have anti-MOG antibodies. MRI scans showed bilateral cortical swelling and abnormal signal intensities.
JOURNAL OF NEUROLOGY
(2021)
Article
Clinical Neurology
Jie Xu, Lingjuan Liu, Jie Xiong, Lu Zhang, Peng Huang, Li Tang, Yangyang Xiao, Xingfang Li, Jian Li, Yingying Luo, Huiling Li, Dingan Mao, Liqun Liu
Summary: Clinical differences between pediatric and adult patients with MOG-EM were identified in this study. Pediatric patients had a higher prevalence of bilateral optic neuritis and fever, while adult patients more commonly presented with NMOSD and unilateral optic nerve involvement. Visual impairment was the predominant symptom in both age groups, with fever being more common among pediatric patients.
FRONTIERS IN NEUROLOGY
(2021)
Article
Pediatrics
Xiaoang Sun, Meiyan Liu, Xiaona Luo, Fang Yuan, Chunmei Wang, Simei Wang, Quanmei Xu, Yuanfeng Zhang, Yucai Chen
Summary: Background research on MOG-Ab-associated disease (MOGAD) among Chinese children is limited. This study explored and analyzed the clinical characteristics and prognoses of Chinese children with acquired demyelinating syndromes (ADSs) who tested positive or negative for MOG-Ab. The findings showed differences in clinical features and imaging between MOG-Ab-positive and MOG-Ab-negative children with ADSs. Additional diagnoses and personalized treatment plans should be developed to improve the prognoses of children with MOGAD.
Article
Clinical Neurology
Pietro E. Doneddu, Marta Ruiz, Elisa Bianchi, Giuseppe Liberatore, Fiore Manganelli, Dario Cocito, Giuseppe Cosentino, Luana Benedetti, Girola A. Marfia, Massimiliano Filosto, Chiara Briani, Claudia Giannotta, Eduardo Nobile-Orazio
Summary: A diagnostic score was developed to distinguish anti-MAG neuropathy from CIDP and was applied to patients with atypical anti-MAG neuropathy. The score allowed an accurate discrimination between the two groups and may help in selecting proper treatment for patients with anti-MAG antibodies.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Article
Immunology
Yunfei Bai, Wei Li, Chuanzhu Yan, Ying Hou, Qinzhou Wang
Summary: A Chinese male patient with anti-NF155 nodopathy undergoing rituximab treatment showed the presence of anti-rituximab antibodies (ARAs), which had a negative impact on the efficacy of rituximab. This is the first reported case of ARAs in patients with anti-NF155 antibodies, highlighting the need for early testing of these antibodies.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Pediatrics
Yuko Ichimiya, Pin Fee Chong, Yuri Sonoda, Vlad Tocan, Mitsuru Watanabe, Hiroyuki Torisu, Ryutaro Kira, Toshiyuki Takahashi, Jun-Ichi Kira, Noriko Isobe, Yasunari Sakai, Shouichi Ohga
Summary: This study investigated the clinical features of children with ADS and found that MOG-Ab has a significant impact on the clinical presentation of children with ADS, with MOGAD patients more likely to experience headache and/or somatosensory symptoms.
EUROPEAN JOURNAL OF PEDIATRICS
(2023)
Article
Immunology
Keita Takahashi, Hideyuki Takeuchi, Ryoko Fukai, Haruko Nakamura, Keisuke Morihara, Yuichi Higashiyama, Toshiyuki Takahashi, Hiroshi Doi, Fumiaki Tanaka
Summary: In these cases, patients developed relapses and seroconversion of anti-MOG antibodies after switching to DMF treatment, suggesting that DMF may exacerbate the humoral immune imbalance in MOGAD.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Biochemistry & Molecular Biology
Ninu Poulose, Raghavan Raju
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
(2015)
Article
Biochemistry & Molecular Biology
Ahmar Ayub, Ninu Poulose, Raghavan Raju
MOLECULAR MEDICINE
(2015)
Article
Biochemistry & Molecular Biology
Sumin Lu, Alex Aguilar, Kumar Subramani, Ninu Poulose, Ahmar Ayub, Raghavan Raju
Article
Neurosciences
P. Benson Ham, Raghavan Raju
PROGRESS IN NEUROBIOLOGY
(2017)
Article
Multidisciplinary Sciences
Kumar Subramani, Sumin Lu, Marie Warren, Xiaogang Chu, Haroldo A. Toque, R. William Caldwell, Michael P. Diamond, Raghavan Raju
SCIENTIFIC REPORTS
(2017)
Article
Urology & Nephrology
Nino Kvirkvelia, Malgorzata McMenamin, Marie Warren, Ravirajsinh N. Jadeja, Sai Karthik Kodeboyina, Ashok Sharma, Wenbo Zhi, Paul M. O'Connor, Raghavan Raju, Rudolf Lucas, Michael P. Madaio
KIDNEY INTERNATIONAL
(2018)
Article
Biochemistry & Molecular Biology
Bixi Jian, Shaolong Yang, Irshad H. Chaudry, Raghavan Raju
MOLECULAR MEDICINE
(2014)
Article
Multidisciplinary Sciences
Marlene E. Starr, Allison M. Steele, Mizuki Saito, Bill J. Hacker, B. Mark Evers, Hiroshi Saito
Article
Immunology
Kumar Subramani, Srikrishnan P. Raju, Xiaogang Chu, Marie Warren, Chirayu D. Pandya, Nasrul Hoda, Sadanand Fulzele, Raghavan Raju
INTERNATIONAL IMMUNOPHARMACOLOGY
(2018)
Article
Biochemistry & Molecular Biology
Kumar Subramani, Xiaogang Chu, Marie Warren, Mariah Lee, Sumin Lu, Nagendra Singh, Raghavan Raju
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
(2019)
Article
Cell Biology
Xiaogang Chu, Jin Wen, Raghavan Pillai Raju
Article
Neurosciences
Raghavan Pillai Raju, Lun Cai, Alpna Tyagi, Subbiah Pugazhenthi
Summary: In this study, using RNA-seq analysis, we found significant changes in gene expression related to pathways including oxidation-reduction, oxidative phosphorylation, innate immune response, ribosomal protein synthesis, and ubiquitin proteosome system in the brains of 5XFAD mice. The downregulation of genes related to oxidation-reduction and upregulation of immune response genes were the most striking features observed. Gene interaction analysis revealed at least three distinct interaction clusters, with the predominant one relating to cellular energetics.
MOLECULAR NEUROBIOLOGY
(2023)
Article
Biochemistry & Molecular Biology
Marie Warren, Kumar Subramani, Richard Schwartz, Raghavan Raju
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
(2017)
Review
Geriatrics & Gerontology
Ninu Poulose, Raghavan Raju
Article
Geriatrics & Gerontology
Chandrasekharam N. Nagineni, Raghavan Raju, Krishnasai K. Nagineni, Vijay K. Kommineni, Aswini Cherukuri, R. Krishnan Kutty, John J. Hooks, Barbara Detrick