AI Article Synopsis
- * In a study of 4 patients with anti-NF155 IgG4 positive CIDP, symptoms included tremors and severe ataxia, with varied treatment responses; intravenous immunoglobulin was ineffective but plasma exchange and corticosteroids provided some relief.
- * Analysis of sural nerve biopsies showed damage to specific nerve structures, and findings suggest that anti-NF155 IgG4 antibodies play a significant role in the disease's pathology.
Article Abstract
Introduction: Neurofascin155 (NF155) is a target antigen for autoantibodies in a subset of chronic inflammatory demyelinating polyneuropathy (CIDP).
Methods: We report the cases of 4 patients with anti-NF155 immunoglobulin G4 (IgG4) antibody-positive CIDP who underwent sural nerve biopsies.
Results: All patients were relatively young at onset. Three patients experienced tremors, and 2 patients had severe ataxia. Although the response to intravenous immunoglobulin was poor in all patients, plasma exchange and corticosteroids were at least partially effective. Immunoadsorption plasmapheresis was performed in 1 patient but was ineffective. Electron microscopic examination of sural nerve biopsies revealed loss of paranodal transverse bands in all patients.
Discussion: Anti-NF155 IgG4 antibody-positive CIDP shows distinctive clinicopathological features, indicating that the IgG4 antibody is directly associated with the pathogenic mechanisms of anti-NF155 IgG4 antibody-positive CIDP. Muscle Nerve 57: 498-502, 2018.
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| http://dx.doi.org/10.1002/mus.25757 | DOI Listing |
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