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Motor neuropathies and serum IgM binding to NS6S heparin disaccharide or GM1 ganglioside
  1. Alan Pestronk,
  2. Miguel Chuquilin,
  3. Rati Choksi
  1. Department of Neurology, Washington University in St Louis School of Medicine, St Louis, Missouri, USA
  1. Correspondence to Dr A Pestronk, Department of Neurology, Washington University in St Louis School of Medicine, 660 South Euclid Avenue, Box 8111, St Louis, MO 63110, USA; pestronka{at}neuro.wustl.edu

Abstract

Background Serum IgM binding to GM1 ganglioside (GM1) is often associated with chronic acquired motor neuropathies. This study compared the frequency and clinical associations of serum IgM binding to a different antigen, a disulphated heparin disaccharide (NS6S), with results of IgM binding to GM1.

Methods Serums and clinical features were retrospectively compared from 75 patients with motor neuropathies and 134 controls with amyotrophic lateral sclerosis (ALS), chronic immune demyelinating polyneuropathy (CIDP) and sensory neuropathies. Clinical correlations of positive IgM anti-GM1 testing found in 27 of 2113 unselected serums were also reviewed. Serum testing for IgM binding to NS6S and GM1 used covalent antigen linkage to ELISA plates.

Results High titre IgM binding to NS6S and GM1 each occurred in 43%, and to one of the two in 64%, of motor neuropathy patients. Motor neuropathy syndromes were present in 25 of 27 patients with high titre serum IgM binding to GM1 in the unselected serums. IgM anti-GM1 or NS6S antibody related motor neuropathy syndromes usually have asymmetric, predominantly distal, upper extremity weakness.

Conclusions IgM binding to NS6S disaccharide is associated with motor neuropathy syndromes and occurs with similar frequency to IgM binding to GM1. Testing for IgM binding to NS6S in addition to GM1 increases the frequency of finding IgM autoantibodies in motor neuropathies from 43% to 64%. High titres of serum IgM binding to GM1, tested with covalent ELISA methodology, have 93% specificity for motor neuropathy syndromes. High titres of serum IgM binding to NS6S have specificity for immune motor neuropathies compared with ALS and CIDP.

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Footnotes

  • Linked articles 205310.

  • Funding This study was supported by the Washington University Neuromuscular Research Fund.

  • Competing interests AP serves on the scientific advisory board of the Myositis Association. He receives revenue related to patents and speaker honoraria from Athena. He owns stock in Johnson & Johnson. He is a director of the Washington University Neuromuscular Clinical Laboratory which performs antibody testing and muscle and nerve pathology analysis. The Washington University Neurology Department bills for these procedures. AP receives research support from NIH, the Muscular Dystrophy Association, Genzyme, Insmed, Knopp, Prosensa, ISIS, Cytokinetics and Sanofi.

  • Ethics approval This study was conducted with the approval of the Washington University HPRO.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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