title: Contactin 1 IgG4 associates to chronic inflammatory demyelinating polyneuropathy with sensory ataxia.
abstract: A Spanish group recently reported that four patients with chronic inflammatory demyelinating polyneuropathy carrying IgG4 autoantibodies against contactin 1 showed aggressive symptom onset and poor response to intravenous immunoglobulin. We aimed to describe the clinical and serological features of Japanese chronic inflammatory demyelinating polyneuropathy patients displaying the anti-contactin 1 antibodies. Thirteen of 533 (2.4%) patients with chronic inflammatory demyelinating polyneuropathy had anti-contactin 1 IgG4 whereas neither patients from disease or normal control subjects did (P = 0.02). Three of 13 (23%) patients showed subacute symptom onset, but all of the patients presented with sensory ataxia. Six of 10 (60%) anti-contactin 1 antibody-positive patients had poor response to intravenous immunoglobulin, whereas 8 of 11 (73%) antibody-positive patients had good response to corticosteroids. Anti-contactin 1 IgG4 antibodies are a possible biomarker to guide treatment option.
authors: 1 Department of Medicine, Yong Loo Lin School of Medicine, National University of Singapore, Singapore.
authors: Yumako
authors: Y
authors: Miura
authors: 2 Aix-Marseille Université, CNRS, CRN2M-UMR 7286, Marseille, France 1 Department of Medicine, Yong Loo Lin School of Medicine, National University of Singapore, Singapore.
authors: Jérôme J
authors: JJ
authors: Devaux
authors: 1 Department of Medicine, Yong Loo Lin School of Medicine, National University of Singapore, Singapore.
authors: Yuki
authors: Y
authors: Fukami
authors: 2 Aix-Marseille Université, CNRS, CRN2M-UMR 7286, Marseille, France.
authors: Constance
authors: C
authors: Manso
authors: 2 Aix-Marseille Université, CNRS, CRN2M-UMR 7286, Marseille, France.
authors: Maya
authors: M
authors: Belghazi
authors: 1 Department of Medicine, Yong Loo Lin School of Medicine, National University of Singapore, Singapore.
authors: Anna Hiu Yi
authors: AH
authors: Wong
authors: 1 Department of Medicine, Yong Loo Lin School of Medicine, National University of Singapore, Singapore 3 Department of Physiology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore mdcyuki@nus.edu.sg.
authors: Nobuhiro
authors: N
authors: Yuki
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001259:Ataxia / Q000097:blood / Q000150:complications / Q000188:drug therapy / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood / Q000276:immunology*; D054316:Biomarkers, Pharmacological / Q000097:blood*; D016022:Case-Control Studies; D002478:Cells, Cultured; D058967:Contactin 1 / Q000276:immunology* / Q000378:metabolism; D000939:Epitopes / Q000276:immunology; D005260:Female; D005727:Ganglia, Spinal / Q000378:metabolism*; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology*; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000097:blood / Q000150:complications / Q000188:drug therapy / Q000276:immunology*; D012189:Retrospective Studies
chemical_list: D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D054316:Biomarkers, Pharmacological; D058967:Contactin 1; D000939:Epitopes; D007074:Immunoglobulin G; D016756:Immunoglobulins, Intravenous
keywords: autoantibody; chronic inflammatory demyelinating polyneuropathy; contactin 1; myelin; nodes of Ranvier