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Is distal motor and/or sensory demyelination a distinctive feature of anti-MAG neuropathy?

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Abstract

To report the frequency of the different patterns of sensory and motor electrophysiological demyelination distribution in patients with anti-MAG neuropathy in comparison with patients with IgM neuropathy without MAG reactivity (IgM-NP). Thirty-five anti-MAG patients at early disease stage (20.1 months) were compared to 23 patients with IgM-NP; 21 CIDP patients and 13 patients with CMT1a neuropathy were used as gold standard neuropathies with multifocal and homogeneous demyelination, respectively. In all groups, standard motor and sensory electrophysiological parameters, terminal latency index and modified F ratio were investigated. Motor electrophysiological demyelination was divided in four profiles: distal, homogeneous, proximal, and proximo-distal. Distal sensory and sensorimotor demyelination were evaluated. Anti-MAG neuropathy is a demyelinating neuropathy in 91 % of cases. In the upper limbs, reduced TLI is more frequent in anti-MAG neuropathy, compared to IgM-NP. But, predominant distal demyelination of the median nerve is encountered in only 43 % of anti-MAG neuropathy and is also common in IgM-NP (35 %). Homogeneous demyelination was the second most frequent pattern (31 %). Concordance of electrophysiological profiles across motor nerves trunks is low and median nerve is the main site of distal motor conduction slowing. Reduced sensory conduction velocities occurs in 14 % of patients without evidence of predominant distal slowing. Simultaneous sensory and motor distal slowing was more common in the median nerve of anti-MAG neuropathy than IgM-NP. Electrophysiological distal motor demyelination and sensory demyelination are not a distinctive feature of anti-MAG reactivity. In anti-MAG neuropathy it is mainly found in the median nerve suggesting a frequent nerve compression at wrist.

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Authors and Affiliations

  1. Service de Physiologie Clinique-Explorations Fonctionnelles, AP-HP, Hôpital Lariboisière, 2, rue Ambroise Paré, 75010, Paris, France

    Pierre Lozeron, Marion Brisset & Nathalie Kubis

  2. Institut de Cancérologie Gustave Roussy, Villejuif, France

    Vincent Ribrag

  3. Department of Neurology, French National Reference Centre for Familial Amyloid Polyneuropathies and Other Rare Diseases, Hôpital de Bicêtre, Le Kremlin Bicêtre, France

    David Adams & Marie Theaudin

  4. Department of Immuno-Hematology, AP-HP, Hôpital Saint-Louis, Paris, France

    Marguerite Vignon, Marine Baron, Marion Malphettes & Bertrand Arnulf

  5. INSERM UMR965, Université Paris Diderot, Sorbonne Paris Cité, Paris, France

    Pierre Lozeron & Nathalie Kubis

  6. INSERM U788, Univ Paris 11, Orsay, France

    David Adams

Authors
  1. Pierre Lozeron
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  2. Vincent Ribrag
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  3. David Adams
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  4. Marion Brisset
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Correspondence to Pierre Lozeron.

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The other authors declare that they have no conflict of interest.

Funding

Dr. Lozeron received a speaker honorarium from Pfizer, Astellas and Sanofi and received travel grants from CSL Behring.

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Lozeron, P., Ribrag, V., Adams, D. et al. Is distal motor and/or sensory demyelination a distinctive feature of anti-MAG neuropathy?. J Neurol 263, 1761–1770 (2016). https://doi.org/10.1007/s00415-016-8187-z

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  • DOI: https://doi.org/10.1007/s00415-016-8187-z

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