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Clinical phenotype of patients with neuropathy associated with monoclonal gammopathy: a comparative study and a review of the literature

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Abstract

The objective of this study was to investigate if the clinical and electrophysiological phenotype of patients with polyneuropathy associated with IgM monoclonal gammopathy (IgM-PNP) is related to the presence of antibodies against gangliosides or myelin-associated glycoprotein (MAG). We compared clinical and nerve conduction study (NCS) characteristics of 11 IgM-PNP patients with antibodies against asialo-GM1 or gangliosides (GM1, GD1a, GD1b, GM2 or GQ1b) to 11 consecutive IgM-PNP patients with anti-MAG neuropathy and to 9 IgM-PNP patients without antibodies against either MAG or gangliosides. Patients with anti-ganglioside antibodies could not be differentiated from those with anti-MAG antibodies based on clinical characteristics. However, within the group of anti-ganglioside antibody positive patients, antibodies against GD1b and GQ1b were associated with a purely sensory neuropathy (p = 0.002), while asymmetric weakness with symmetric sensory loss was associated with anti-asialo-GM1 antibodies. In conclusion, polyneuropathy associated with IgM monoclonal gammopathy and anti-ganglioside antibodies clinically resembles anti-MAG neuropathy. Pure sensory neuropathy and marked asymmetry may suggest the presence of anti-ganglioside rather than anti-MAG antibodies.

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Acknowledgments

W.L. van der Pol received travel grants from Baxter International Inc. and research support from the Prinses Beatrix Spierfonds and the Stichting Spieren voor Spieren. B.C. Jacobs received research support from the Netherlands Organization for Health Research and Development, Erasmus MC, Prinses Beatrix Fonds, GBS-CIDP Foundation International and travel support from Baxter Biopharmaceutics

Conflicts of interest

N.C. Notermans reports no conflicts of interest. A.C.J. Stork reports no conflicts of interest. H. Franssen reports no conflicts of interest.

Ethical standards

Approval for this study was obtained from the ethical standards committee of the UMC Utrecht. All patients gave their informed consent prior to their inclusion in this study.

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Authors and Affiliations

  1. Department of Neurology and Neurosurgery, Brain Center Rudolf Magnus, University Medical Center Utrecht, Heidelberglaan 100, PO Box 85500 3508 GA, 3584 CX, Utrecht, The Netherlands

    Abraham C. J. Stork, W-Ludo van der Pol, Hessel Franssen & Nicolette C. Notermans

  2. Departments of Neurology and Immunology, Erasmus Medical Center, ‘s Ggravendijkwal 230, 3015 CE, Rotterdam, The Netherlands

    Bart C. Jacobs

Authors
  1. Abraham C. J. Stork
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  2. W-Ludo van der Pol
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  3. Hessel Franssen
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  4. Bart C. Jacobs
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  5. Nicolette C. Notermans
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Correspondence to Abraham C. J. Stork.

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Stork, A.C.J., van der Pol, WL., Franssen, H. et al. Clinical phenotype of patients with neuropathy associated with monoclonal gammopathy: a comparative study and a review of the literature. J Neurol 261, 1398–1404 (2014). https://doi.org/10.1007/s00415-014-7354-3

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  • DOI: https://doi.org/10.1007/s00415-014-7354-3

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