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

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Vallat, Jean-Michel | Deschamps, Nathalie | Richard, Laurence | Magy, Laurent | Devaux, Jérôme J. | Mathis, Stéphane

Edité par CCSD ; Elsevier -

International audience. 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.

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