Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired disorder of peripheral nerves and nerve roots. Its cause is unknown, but recently antibodies to nodal and paranodal proteins have been discovered in a small subset of CIDP patients. These contactin and neurofascin-related immune-mediated neuropathies are thought to be variants of CIDP and often respond suboptimally to standard therapy. Here, we report a patient with both anti-contactin 1 and anti-neurofascin 140 antibodies whose presentation resembled phenotypes of both CIDP variants.
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| http://dx.doi.org/10.1016/j.ensci.2018.11.016 | DOI Listing |
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Favorable long-term outcomes of autoimmune nodopathy with mycophenolate mofetil.
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Department of Translational Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea.
Autoimmune nodopathy (AN) is a rare immune-mediated neuropathy characterized by autoantibodies against nodal or paranodal proteins. Patients with AN generally respond poorly to immunoglobulin therapy, and as a newly defined condition, there are currently no established treatment guidelines. Although rituximab shows potential as a therapeutic option, its high cost, limited availability, and the need for infusion monitoring hinder its use as a first-line treatment in many countries.
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View Article and Find Full Text PDF[The significance of autoantibodies against nodal and paranodal proteins in autoimmune nodopathies].
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Department of Neurology, Kyushu University Hospital.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is recognized as a syndrome caused by multiple pathologies. Since the 2010s, it has been clarified that autoantibodies against membranous proteins localized in the nodes of Ranvier and paranodes are positive in subsets of CIDP patients, leading to proposing a new disease concept called autoimmune nodopathies, which is independent of CIDP, in the revised international CIDP guidelines. This article reviews the significance of these autoantibodies, especially anti-neurofascin 155 and anti-contactin 1 antibodies, which have been the most prevalent and achieved a higher degree of consensus.
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