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.
| Original language | English |
|---|---|
| Pages (from-to) | 498-502 |
| Number of pages | 5 |
| Journal | Muscle and Nerve |
| Volume | 57 |
| Issue number | 3 |
| DOIs | |
| Publication status | Published - Mar 2018 |
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All Science Journal Classification (ASJC) codes
- Physiology
- Clinical Neurology
- Cellular and Molecular Neuroscience
- Physiology (medical)
Cite this
ELectron microscopic abnormality and therapeutic efficacy in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin155 immunoglobulin G4 antibody. / Kuwahara, Motoi; Suzuki, Hidekazu; Oka, Nobuyuki; Ogata, Hidenori; Yanagimoto, Satoshi; Sadakane, Shuji; Fukumoto, Yuta; Yamana, Masaki; Yuhara, Yoshiko; Yoshikawa, Keisuke; Morikawa, Miyuki; Kawai, Shigeru; Okazaki, Masahiro; Tsujimoto, Toru; Kira, Jun Ichi; Kusunoki, Susumu.
In: Muscle and Nerve, Vol. 57, No. 3, 03.2018, p. 498-502.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - ELectron microscopic abnormality and therapeutic efficacy in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin155 immunoglobulin G4 antibody
AU - Kuwahara, Motoi
AU - Suzuki, Hidekazu
AU - Oka, Nobuyuki
AU - Ogata, Hidenori
AU - Yanagimoto, Satoshi
AU - Sadakane, Shuji
AU - Fukumoto, Yuta
AU - Yamana, Masaki
AU - Yuhara, Yoshiko
AU - Yoshikawa, Keisuke
AU - Morikawa, Miyuki
AU - Kawai, Shigeru
AU - Okazaki, Masahiro
AU - Tsujimoto, Toru
AU - Kira, Jun Ichi
AU - Kusunoki, Susumu
PY - 2018/3
Y1 - 2018/3
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85028435982&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85028435982&partnerID=8YFLogxK
U2 - 10.1002/mus.25757
DO - 10.1002/mus.25757
M3 - Article
C2 - 28796305
AN - SCOPUS:85028435982
VL - 57
SP - 498
EP - 502
JO - Muscle and Nerve
JF - Muscle and Nerve
SN - 0148-639X
IS - 3
ER -