Chronic Demyelinating Polyneuropathies

Jeffrey A. Allen, MD Peripheral Nerve and Motor Neuron Disorders p. 1310-1331 October 2017, Vol.23, No.5 doi: 10.1212/CON.0000000000000517
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Purpose of Review: This article reviews the chronic demyelinating neuropathies, with a focus on the diagnosis and treatment of immune-mediated neuropathies and the features that can help differentiate immune-mediated neuropathies from other chronic demyelinating peripheral nerve conditions.

Recent Findings: Advances in clinical phenotyping and outcomes assessment have enabled neurologists to improve disease recognition, treatment, and disease monitoring. Our understanding of the immunopathogenesis of demyelinating neuropathies is evolving. Identification of new antibodies and recognition that node of Ranvier dysfunction may be an early pathogenic feature may herald further diagnostic and treatment advancements.

Summary: The chronic demyelinating polyneuropathies are heterogeneous. The clinical and diagnostic features are sometimes overlapping, and the specific disorders are variable in pathogenesis, treatment, and prognosis. This heterogeneity underscores the importance of achieving diagnostic accuracy and implementing disease-specific treatment approaches.

Address correspondence to Dr Jeffrey Allen, Department of Neurology, 12-150 PWB, MMC 295, 516 Delaware St SE, Minneapolis, MN 55455, [email protected].

Relationship Disclosure: Dr Allen serves on the global medical advisory board of the GBC/CIDP Foundation International, on the organizing committee of the Inflammatory Neuropathy Consortium, and as a consultant for CSL Behring and Grifols. Dr Allen receives research/grant support from CSL Behring and has received personal compensation for speaking engagements from Grifols.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Allen discusses the unlabeled/investigational use of alemtuzumab, azathioprine, bevacizumab, chlorambucil, cyclophosphamide, cyclosporine, fludarabine, interferon beta-1a, lenalidomide, melphalan, methotrexate, mycophenolate mofetil, rituximab, tacrolimus, and thalidomide for the treatment of chronic demyelinating polyneuropathies.

Copyright © 2017 by the American Academy of Neurology.