Introduction/aims: Immunoglobulin M neuropathy associated with anti-myelin-associated glycoprotein antibody (IgM/anti-MAG) neuropathy typically presents with chronic, distal-dominant symmetrical sensory or sensorimotor deficits. Ultrasonographic studies of IgM/anti-MAG neuropathy are limited, and were all performed on Western populations. We aimed to characterize the nerve ultrasonographic features of IgM/anti-MAG neuropathy in the Japanese population and evaluate whether they differ from the findings of the common subtypes of chronic inflammatory demyelinating polyneuropathy (CIDP).
Methods: In this cross-sectional study, we retrospectively reviewed medical records and extracted the cross-sectional areas (CSAs) of C5-C7 cervical nerve roots and median and ulnar nerves of 6 IgM/anti-MAG neuropathy patients, 10 typical CIDP (t-CIDP) patients, 5 multifocal CIDP (m-CIDP) patients, and 17 healthy controls (HCs).
Results: Cervical nerve root CSAs were significantly larger at every examined site on both sides in IgM/anti-MAG neuropathy than in m-CIDP and HCs but were comparable to those in t-CIDP. Peripheral nerve enlargements were greatest at common entrapment sites (ie, wrist and elbow) in IgM/anti-MAG neuropathy, a pattern shared with t-CIDP but not with m-CIDP. The degree of nerve enlargement at entrapment sites compared to non-entrapment sites was significantly higher in IgM/anti-MAG neuropathy than in t-CIDP.
Discussion: Our study delineated the ultrasonographic features of IgM/anti-MAG neuropathy in the Japanese population and observed similar characteristics to those of t-CIDP, with subtle differences. Further studies comparing results from various populations are required to optimize the use of nerve ultrasound worldwide.
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http://dx.doi.org/10.1002/mus.27542 | DOI Listing |
Neurol 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.
View Article and Find Full Text PDFJ Peripher Nerv Syst
June 2023
Department of Neurology, Maastricht University Medical Center+, Maastricht, The Netherlands.
Background: International consensus on IgM ± anti-MAG ± PNP (IgM PNP) is lacking. Despite increasing interest in clinical trials, validated disease-specific measures are needed to adequately capture limitations and changes over time. The IMAGiNe (IgM ± anti-myelin associated glycoprotein [MAG] peripheral neuropathy) study surges as an international collaboration to create a standardized registry of patients with IgM ± anti-MAG PNP.
View Article and Find Full Text PDFNeurol Neuroimmunol Neuroinflamm
May 2023
From the Department of Neurology and Clinical Neuroscience (R.S., F.S., Y.S., Y.T., Michiaki Koga, T.K.), Yamaguchi University Graduate School of Medicine, Ube; Department of Neurology (Motoi Kuwahara, S.K.), Kindai University Faculty of Medicine, Osaka; Center for Gene Research (Y.M., K.W.), Yamaguchi University, Ube, Japan; and Japan Community Health Care Organization (S.K.).
Background And Objectives: Deposition of myelin-associated glycoprotein (MAG) immunoglobulin M (IgM) antibodies in the sural nerve is a key feature in anti-MAG neuropathy. Whether the blood-nerve barrier (BNB) is disrupted in anti-MAG neuropathy remains elusive.We aimed to evaluate the effect of sera from anti-MAG neuropathy at the molecular level using our in vitro human BNB model and observe the change of BNB endothelial cells in the sural nerve of anti-MAG neuropathy.
View Article and Find Full Text PDFMuscle Nerve
June 2022
Department of Neurology, Kansai Electric Power Hospital, Osaka, Japan.
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