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.
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.
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.
(1)Expert Review of Neurotherapeutics, Advisory Panel, 15 years (2) Journal of the Neurological Sciences, Associate Editor, 13 years (3) Journal of the Peripheral Nervous Systems, Editorial Board, 8 years (4) Journal of Neurology, Neurosurgery and Psychiatry, Editorial Board, 2 year (5) Journal of Alzheimer?s Disease, Editorial Board, 1 year
(1) Singapore National Medical Research Council, IRG 10nov086 (2) Singapore National Medical Research Council, CSA/047/2012 to NY
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