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Multifocal motor neuropathy with high titers of anti-MAG antibodies

Steck, Andreas
Published in Journal of the peripheral nervous system. 2014, vol. 19, no. 2, p. 180-2
Abstract Multifocal motor neuropathy (MMN) and anti-myelin-associated glycoprotein (anti-MAG)-associated neuropathy are clinically and electrophysiologically distinct entities. We describe a patient with characteristic features of both neuropathies, raising the possibility of an overlap syndrome. A 49-year-old patient reported a history of slowly progressive predominantly distal tetraparesis, with mild sensory deficits. Nerve conduction studies demonstrated persistent motor conduction blocks outside compression sites, typical of MMN. Laboratory findings revealed persistently high titers of anti-MAG immunoglobulin Mλ (IgMλ) paraprotein in the context of a monoclonal gammapathy of unknown significance. Skin biopsy of distal lower limb revealed IgM positive terminal nerve perineurium deposits. This case suggests that the distinction between subtypes of chronic inflammatory neuropathies may not be as clear as initially thought, and that the pattern of pathogenicity of anti-MAG antibodies may vary.
Keywords Autoantibodies/bloodFemaleHumansImmunoglobulin M/metabolismIntermediate Filaments/metabolismMiddle AgedMyelin Sheath/metabolism/pathologyMyelin-Associated Glycoprotein/immunologyNeural ConductionParaproteinemias/blood/complications/immunologyPolyneuropathies/blood/complications/immunology
PMID: 24863375
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Research group Scléroses en plaques, neuroimmunologie, encéphalite autoimmune expérimentale (843)
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BRIDEL, Claire et al. Multifocal motor neuropathy with high titers of anti-MAG antibodies. In: Journal of the peripheral nervous system, 2014, vol. 19, n° 2, p. 180-2. doi: 10.1111/jns5.12069 https://archive-ouverte.unige.ch/unige:89941

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Deposited on : 2016-12-07

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