Neurofascin-155 IgG4 in chronic inflammatory demyelinating polyneuropathy
Neurology. 2016-02-03; 86(9): 800-807
Lire sur PubMed
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.