Neurofascin-155 IgG4 in chronic inflammatory demyelinating polyneuropathy

Jérôme J. Devaux, Yumako Miura, Yuki Fukami, Takayuki Inoue, Constance Manso, Maya Belghazi, Kenji Sekiguchi, Norito Kokubun, Hiroo Ichikawa, Anna Hiu Yi Wong, Nobuhiro Yuki
Neurology. 2016-02-03; 86(9): 800-807
DOI: 10.1212/wnl.0000000000002418

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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
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
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.

Auteurs Bordeaux Neurocampus