Summary
A case of ataxic neuropathy associated with IgMκ M proteinemia is reported. Double filtration plasmapheresis effectively treated the neuropathy. The IgMκ antibody had anti-Pr2 cold agglutinin activity. We demonstrated reactivities of the IgMκ 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 para proteinemic 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.
Similar content being viewed by others
References
Ando S, Chang N, Yu R (1978) High performance thin-layer chromatography and densitometric determination of brain ganglioside compositions of several species. Anal Biochem 89:437–450
Ariga T, Sekine M, Yu R, Miyatake T (1982) Disialogangliosides in bovine adrenal medulla. J Biol Chem 257:2230–2235
Fong J, Ledeen R, Kundu S, Brostoff S (1976) Gangliosides of peripheral nerve myelin. J Neurochem 26:157–162
Freddo L, Yu R, Latov N, Donofrio P, Hays A, Greenberg H, Albers J, Allessi A, Keren D (1986) Gangliosides GM1 and GD1b are antigens for IgM M-protein in a patient with motor neuron disease. Neurology 36:454–458
Gillard B, Jones M, Marcus D (1987) Glycosphingolipids of human umbilical vein endothelial cells and smooth muscle cells. Arch Biochem Biophys 256:435–445
Ilyas A, Quarles R, Dalakas M, Fishman P, Brady R (1985) Monoclonal IgM in a patient with paraproteinemic neuropathy binds to gangliosides containing disialosyl groups. Ann Neurol 18:655–659
Lenhard V, Seelig H, Geisen H, Roelcke D (1978) Identification of I/i, Pr1–3 and Gd antigens in the human kidney: possible relevance to hyperacute graft rejection induced by cold agglutinins. Clin Exp Immunol 33:276–282
Li Y, Mansson J, Vanier M, Svennerholm L (1973) Structure of the major glucosamine-containing ganglioside of human tissues. J Biol Chem 248:2634–2636
Miyatani N, Baba H, Sato S, Nakamura K, Yuasa T, Miyatake T (1987) Antibody to sialosyllactosaminylparagloboside in a patient with IgM paraproteinemia and polyradiculoneuropathy. J Neuroimmunol 14:189–196
Miyatani N, Kohriyama T, Maeda Y, Yu R (1990) Sulfated glucuronyl paragloboside in rat brain microvessels. J Neurochem 55:577–582
Quarles R (1990) Human monoclonal antibodies associated with neuropathy. Methods Enzymol 179:291–299
Quarles R, Ilyas A, Willison H (1986) Antibodies to glycolipids in demyelinating diseases of the human peripheral nervous system. Chem Phys Lipids 42:235–248
Roelcke D (1973) Serological studies on the Pr1/Pr2 antigens using dog erythrocytes. Vox Sang 24:354–361
Sato S, Baba H, Inuzuka T, Miyatake T (1986) Anti-myelin-associated glycoprotein antibody in sera from patients with demyelinating diseases. Acta Neurol Scand 74:115–120
Uemura K, Roelcke D, Nagai Y, Feizi T (1984) The reactivities of human erythrocyte autoantibodies anti-Pr2, anti-Gd, F1 and Sa with gangliosides in a chromatogram binding assay. Biochem J 219:865–874
Yu R, Ariga T, Kohriyama T, Kusunoki S, Maeda Y, Miyatani N (1990) Autoimmune mechanisms in peripheral neuropathies. Ann Neurol 27[Suppl]:S30-S35
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Arai, M., Yoshino, H., Kusano, Y. et al. Ataxic polyneuropathy and anti-Pr2 IgMκ M proteinemia. J Neurol 239, 147–151 (1992). https://doi.org/10.1007/BF00833915
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00833915