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Neurology. 2016 Mar 1;86(9):800-7. doi: 10.1212/WNL.0000000000002418. Epub 2016 Feb 3.

Neurofascin-155 IgG4 in chronic inflammatory demyelinating polyneuropathy.

Author information

1
From Aix-Marseille Université (J.J.D., C.M., M.B.), CNRS, CRN2M-UMR 7286, Marseille, France; Departments of Medicine (Y.M., Y.F., T.I., A.H.Y.W., N.Y.) and Physiology (N.Y.), Yong Loo Lin School of Medicine, National University of Singapore; Brain and Mind Centre (N.Y.), University of Sydney, Australia; Division of Neurology (K.S.), Kobe University Graduate School of Medicine; Department of Neurology (N.K.), Dokkyo Medical University, Tochigi; and Department of Neurology (H.I.), Brain Nerve Center, Showa University Fujigaoka Hospital, Tokyo, Japan.
2
From Aix-Marseille Université (J.J.D., C.M., M.B.), CNRS, CRN2M-UMR 7286, Marseille, France; Departments of Medicine (Y.M., Y.F., T.I., A.H.Y.W., N.Y.) and Physiology (N.Y.), Yong Loo Lin School of Medicine, National University of Singapore; Brain and Mind Centre (N.Y.), University of Sydney, Australia; Division of Neurology (K.S.), Kobe University Graduate School of Medicine; Department of Neurology (N.K.), Dokkyo Medical University, Tochigi; and Department of Neurology (H.I.), Brain Nerve Center, Showa University Fujigaoka Hospital, Tokyo, Japan. gbs.yuki.cidp@gmail.com.

Abstract

OBJECTIVE:

We report the clinical and serologic features of Japanese patients with chronic inflammatory demyelinating polyneuropathy (CIDP) displaying anti-neurofascin-155 (NF155) immunoglobulin G4 (IgG4) antibodies.

METHODS:

In sera from 533 patients with CIDP, anti-NF155 IgG4 antibodies were detected by ELISA. Binding of IgG antibodies to central and peripheral nerves was tested.

RESULTS:

Anti-NF155 IgG4 antibodies were identified in 38 patients (7%) with CIDP, but not in disease controls or normal participants. These patients were younger at onset as compared to 100 anti-NF155-negative patients with CIDP. Twenty-eight patients (74%) presented with sensory ataxia, 16 (42%) showed tremor, 5 (13%) presented with cerebellar ataxia associated with nystagmus, 3 (8%) had demyelinating lesions in the CNS, and 20 of 25 (80%) had poor response to IV immunoglobulin. The clinical features of the antibody-positive patients were statistically more frequent as compared to negative patients with CIDP (n = 100). Anti-NF155 IgG antibodies targeted similarly central and peripheral paranodes.

CONCLUSION:

Anti-NF155 IgG4 antibodies were associated with a subgroup of patients with CIDP showing a younger age at onset, ataxia, tremor, CNS demyelination, and a poor response to IV immunoglobulin. The autoantibodies may serve as a biomarker to improve patients' diagnosis and guide treatments.

PMID:
26843559
PMCID:
PMC4793783
[Available on 2017-03-01]
DOI:
10.1212/WNL.0000000000002418
[Indexed for MEDLINE]
Free PMC Article

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