Demyelinating polyneuropathiy associated with IgM paraproteinemia and high titers of anti-MAG IgM antibodies (MAG-PN) is considered different from chronic inflammatory demyelinating polyneuropathy, particularly because of the poorer response to treatment of MAG-PN patients. Therefore, anti-MAG anitbodies may have relevant prognostic value. Available anti-MAG antibody assays require central nervous system myelin proteins from autopsied human brains. This study investigated the feasibility of detecting anti-MAG antibody by immunofluorescence and flow cytometry using a panel of human neuroblastoma cell lines as targets. We report here on the evaluation of the LA-N-1 cell line as an appropriate substrate for the detection of anti-MAG antibody by indirect immunoflourescence.
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http://dx.doi.org/10.1016/j.jneuroim.2004.12.005 | DOI Listing |
Brain Nerve
January 2025
Internal Medicine, Morioka Health Cooperative Association, Kawakubo Hospital.
Anti-myelin-associated glycoprotein (Anti-MAG) neuropathy and autoimmune nodopathies with antibodies targeting nodal or paranodal proteins have recently been reclassified as distinct conditions, separate from chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). This distinction is based on the clinical homogeneity observed in antibody-positive cases, their unique response to treatment compared to CIDP, and evidence indicating the pathogenic role of these autoantibodies. The significance of identifying conditions outside the CIDP category lies in the elucidation of their distinct pathological mechanisms and providing appropriate immunotherapy accordingly.
View Article and Find Full Text PDFJ Neurol Neurosurg Psychiatry
December 2024
Aston Medical School, Aston University, Birmingham, UK
Antimyelin-associated glycoprotein (MAG) neuropathy is a rare autoimmune demyelinating peripheral neuropathy caused by IgM autoantibodies targeting MAG. The typical presentation is that of a slowly progressive, distal, length-dependent, predominantly sensory, sometimes ataxic neuropathy, frequently accompanied by upper limb tremor. Distal motor weakness may subsequently occur.
View Article and Find Full Text PDFPeripheral neuropathy (PN) is a significant cause of morbidity associated with Waldenström macroglobulinemia (WM). The phase 3 ASPEN study compared the efficacy and safety of zanubrutinib with ibrutinib in patients with WM. This ad hoc analysis examined treatment outcomes with zanubrutinib or ibrutinib on PN symptoms associated with WM in patients enrolled in ASPEN.
View Article and Find Full Text PDFNeurol Neuroimmunol Neuroinflamm
January 2025
From the Department of Neurology and Neurosurgery (J.P.M.M., A.F.J.E.V., N.C.N., W.L.v.d.P.), UMC Utrecht Brain Center; Center for Translational Immunology (K.B., K.D.); Department of Hematology (M.C.M.), University Medical Center Utrecht, Utrecht University, The Netherlands.
Background And Objectives: Polyneuropathy associated with an immunoglobulin M (IgM) monoclonal gammopathy is characterized by slowly progressive, predominantly distal sensorimotor deficits, sensory ataxia, and electrophysiologic features of demyelination. IgM antibodies against myelin-associated glycoprotein (MAG) are present in serum from most patients. Nerve damage most likely results from the concerted action of binding of anti-MAG antibodies to nerves, followed by complement activation.
View Article and Find Full Text PDFEur J Neurol
January 2025
argenx, Ghent, Belgium.
Background And Purpose: Complement factor C2 is a potential therapeutic target in immune-mediated neuropathies. However, literature suggests that classical complement pathway activation may proceed to C3 in the absence of C2, a so-called "C2 bypass." Here, we evaluated a C2 bypass mechanism during complement activation by pathogenic human IgM from patients with immune-mediated neuropathies.
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