In:
Muscle & Nerve, Wiley, Vol. 57, No. 3 ( 2018-03), p. 498-502
Abstract:
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
Type of Medium:
Online Resource
ISSN:
0148-639X
,
1097-4598
Language:
English
Publisher:
Wiley
Publication Date:
2018
detail.hit.zdb_id:
1476641-3
SSG:
12