Journal
MUSCLE & NERVE
Volume 57, Issue 3, Pages 498-502Publisher
WILEY
DOI: 10.1002/mus.25757
Keywords
antibody; chronic inflammatory demyelinating polyneuropathy; electron microscopy; IgG4; immunotherapy; neurofascin155
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Funding
- Ministry of Education, Culture, Sports, Science, and Technology of Japan [15H04845]
- Japan Agency for Medical Research and Development [16ek0109056h0003, 16ek0109115h0002]
- Ministry of Health, Labour, and Welfare of Japan
- Grants-in-Aid for Scientific Research [16H07051, 15H04845] Funding Source: KAKEN
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IntroductionNeurofascin155 (NF155) is a target antigen for autoantibodies in a subset of chronic inflammatory demyelinating polyneuropathy (CIDP). MethodsWe report the cases of 4 patients with anti-NF155 immunoglobulin G4 (IgG4) antibody-positive CIDP who underwent sural nerve biopsies. ResultsAll 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. DiscussionAnti-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 Nerve57: 498-502, 2018
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