Widening of myelin lamellae in polyneuropathy with immunoglobulin-M monoclonal gammopathy, without activity against myelin-associated glycoprotein, responsive to treatment.

We report the case of a patient with a very severe predominantly demyelinating sensorimotor polyneuropathy (with axonal loss) that had developed over several months, along with an immunoglobulin-M monoclonal gammopathy without anti-myelin associated glycoprotein antibodies (or other antibodies against myelin). Widening of myelin lamellae were frequently observed by electron microscopic examination of a nerve biopsy: immunoglobulin-M targeting an unknown myelin antigen appears to be responsible for the nerve lesions similar to those observed in anti-myelin associated glycoprotein polyneuropathy. Usually, if in anti-myelin associated glycoprotein neuropathy the response to immunotherapies is not optimal, in this case the combination of plasma exchanges and rituximab was effective, suggesting an autoimmune origin. Copyright © 2022. Published by Elsevier B.V.

Citation

Jean-Michel Vallat, Nathalie Deschamps, Laurence Richard, Laurent Magy, Jérôme Devaux, Stéphane Mathis. Widening of myelin lamellae in polyneuropathy with immunoglobulin-M monoclonal gammopathy, without activity against myelin-associated glycoprotein, responsive to treatment. Neuromuscular disorders : NMD. 2022 Aug;32(8):678-681

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PMID: 35618575

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