Introduction. Polyneuropathies associated with IgM monoclonal gammopathy were recently recognized. Antibodies can react with glycoproteins such as myelin asso ciated glycoprotein (MAG), or gangliosides containing one ...Introduction. Polyneuropathies associated with IgM monoclonal gammopathy were recently recognized. Antibodies can react with glycoproteins such as myelin asso ciated glycoprotein (MAG), or gangliosides containing one sialosyl epitope such as GM1 or several sialosyl epitopes (polysialyted gangliosides) including GD2, G D3, GT1b, GT1a, GQ1b. Methods. We report on three patients presenting oculomotor dysfunction, chronic sensitive ataxic polyneuropathy, high sedimentation rate, IgM monoclonal paraprotein of unknown signification and antidisialosyl IgM antib odies and for two of them cold agglutinins. Such features have been previously d escribed under the acronym “CANOMAD”(chronic ataxic neuropathy with ophthalmop legia, M protein, agglutination and disialosyl antibodies). Results. One of the patients presents extra membranous glomerulopathy and severe motor disability as sociated with this syndrome. The pathophysiology of the glomerulopathy seems to be linked with the polyneuropathy. Patients were treated either by intravenous i mmunoglobulin, corticosteroids or cyclophosphamid. Response to treatment differs in the three cases and there is currently no consensus. Conclusion. Our study d emonstrates that spectrum of polyneuropathy associated with monoclonal polyneuro pathy may be larger than originally described.展开更多
文摘Introduction. Polyneuropathies associated with IgM monoclonal gammopathy were recently recognized. Antibodies can react with glycoproteins such as myelin asso ciated glycoprotein (MAG), or gangliosides containing one sialosyl epitope such as GM1 or several sialosyl epitopes (polysialyted gangliosides) including GD2, G D3, GT1b, GT1a, GQ1b. Methods. We report on three patients presenting oculomotor dysfunction, chronic sensitive ataxic polyneuropathy, high sedimentation rate, IgM monoclonal paraprotein of unknown signification and antidisialosyl IgM antib odies and for two of them cold agglutinins. Such features have been previously d escribed under the acronym “CANOMAD”(chronic ataxic neuropathy with ophthalmop legia, M protein, agglutination and disialosyl antibodies). Results. One of the patients presents extra membranous glomerulopathy and severe motor disability as sociated with this syndrome. The pathophysiology of the glomerulopathy seems to be linked with the polyneuropathy. Patients were treated either by intravenous i mmunoglobulin, corticosteroids or cyclophosphamid. Response to treatment differs in the three cases and there is currently no consensus. Conclusion. Our study d emonstrates that spectrum of polyneuropathy associated with monoclonal polyneuro pathy may be larger than originally described.
