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A case of ataxic neuropathy associated with IgM kappa M proteinemia is reported. Double filtration plasmapheresis effectively treated the neuropathy. The IgM kappa antibody had anti-Pr2 cold agglutinin activity. We demonstrated reactivities of the IgM kappa antibody to sialosyl paragloboside, sialosyl lactosaminyl paragloboside, GT1b, GD1a, GD1b, GM3 and GD3 on high-performance thin-layer chromatography immunostaining and enzyme-linked immunosorbent assay, which is previously unreported antigenic specificity of the M proteins in cases of paraproteinemic neuropathy. IgM M protein with anti-Pr2 cold agglutinin activity may play a pathogenetic role in peripheral nerve demyelination, because the target antigens of the M protein are present in myelin and possibly in endothelial cells of the peripheral nervous system.
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