Neurofascin-155 IgG4 in chronic inflammatory demyelinating polyneuropathy

authors

• Devaux Jérôme J.
• Miura Yumako
• Fukami Yuki
• Inoue Takayuki
• Manso Constance
• Belghazi Maya
• Sekiguchi Kenji
• Kokubun Norito
• Ichikawa Hiroo
• Wong Anna Hiu Yi
• Yuki Nobuhiro

keywords

• Autoantibodies
• Biomarkers
• Cell Adhesion Molecules
• Child
• Female
• Humans
• Immunoglobulin G
• Incidence
• Japan
• Male
• Middle Aged
• Nerve Growth Factors
• Polyradiculoneuropathy
• Chronic Inflammatory Demyelinating
• Risk Assessment
• Young Adult
• Adolescent
• Adult
• Aged
• 80 and over
• Age Distribution

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.ăMETHODS: 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.ăRESULTS: 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.ăCONCLUSION: 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.

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