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Testing for myelin oligodendrocyte glycoprotein antibodies: Who, what, where, when, why, and how.
Mult Scler
January 2025
Department of Neurology, Mayo Clinic, Rochester, MN, USA.
Testing for myelin oligodendrocyte glycoprotein immunoglobulin G antibodies (MOG-IgG) is essential to the diagnosis of MOG antibody-associated disease (MOGAD). Due to its central role in the evaluation of suspected inflammatory demyelinating disease, the last 5 years has been marked by an abundance of research into MOG-IgG testing ranging from appropriate patient selection, to assay performance, to utility of serum titers as well as cerebrospinal fluid (CSF) testing. In this review, we synthesize current knowledge pertaining to the "who, what, where, when, why, and how" of MOG-IgG testing, with the aim of facilitating accurate MOGAD diagnosis in clinical practice.
View Article and Find Full Text PDFAdverse drug events (ADEs) risk signal mining related to eculizumab based on the FARES database.
Front Pharmacol
January 2025
The First Department of Specialty Medicine, Inner Mongolia Corps Hospital of The Chinese People's Armed Police Force, Hohhot, China.
Introduction: Eculizumab is a C5 complement inhibitor approved by the FDA for the targeted treatment of four rare diseases, paroxysmal nocturnal hemoglobinuria (PNH), atypical hemolytic uremic syndrome (aHUS), generalized myasthenia gravis (gMG), and aquaporin-4 immunoglobulin G-positive optic neuromyelitis optica spectrum disorders (AQP4-IgG+NMOSD). The current study was conducted to assess real-world adverse events (AEs) associated with eculizumab through data mining of the FDA Adverse Event Reporting System (FAERS).
Methods: Disproportionality analyses, including Reporting Ratio Ratio (ROR), Proportional Reporting Ratio (PRR), Bayesian Confidence Propagation Neural Network (BCPNN), and Multi-Item Gamma Poisson Shrinker (MGPS) algorithms were used to quantify the signals of eculizumab-associated AEs.
Acute eculizumab treatment in a pediatric patient with AQP4-IgG+ NMOSD.
Mult Scler
January 2025
NYU Langone Medical Center, New York, NY, USA.
Background: Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disorder that occurs in children and adults.
Case: We report a case of a 10-year-old female with AQP4+ NMOSD who presented with paraparesis from longitudinally extensive transverse myelitis (LETM) from C2 to the conus medullaris. The patient showed gradual improvement in strength and sensation with solumedrol and plasma exchange therapy.
Eculizumab treatment during pregnancy in a patient with AQP4-IgG-seropositive NMOSD: A case report.
Mult Scler
January 2025
Department of Translational Biomedicine and Neurosciences, University of Bari Aldo Moro, Bari, Italy.
Eculizumab proved a strong anti-inflammatory effect in neuromyelitis optica spectrum disorders (NMOSD), rare autoimmune diseases affecting the central nervous system in which aquaporin 4-immunoglobulin G (AQP4-IgG) is the main pathogenic antibody. Pregnancy in NMOSD patients is considered at high-risk for neurological and gynecological outcomes, requiring a careful consideration about treatment maintenance. In this case report, we describe a successful pregnancy, resulting in the birth of a healthy child, in a young woman with AQP4-IgG-seropositive NMOSD who was maintained on eculizumab during all pregnancy.
View Article and Find Full Text PDFTDP43 augments astrocyte inflammatory activity through mtDNA-cGAS-STING axis in NMOSD.
J Neuroinflammation
January 2025
Department of Neurology, Center for Medical Research on Innovation and Translation, Institute of Clinical Medicine, Guangzhou First People's Hospital, School of Medicine, South China University of Technology, Guangzhou, China.
Abnormality in transactivating response region DNA binding protein 43 (TDP43) is well-recognized as the pathological hallmark of neurodegenerative diseases. However, the role of TDP43 in neuromyelitis optica spectrum disorder (NMOSD) remains unknown. Here, our observations demonstrate an upregulation of TDP43 in both in vitro and in vivo models of NMOSD, as well as in biological samples from NMOSD patients.
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