Contactin 1 IgG4 associates to chronic inflammatory demyelinating polyneuropathy with sensory ataxia

• Published in: Brain (London, England : 1878) Vol. 138; no. 6; pp. 1484 - 1491
• Main Authors: Miura, Yumako, Devaux, Jérôme J., Fukami, Yuki, Manso, Constance, Belghazi, Maya, Wong, Anna Hiu Yi, Yuki, Nobuhiro
• Format: Journal Article
• Language: English
• Published: England Oxford University Press 01.06.2015
• Series: Editor's Choice
• Subjects: Adrenal Cortex Hormones - therapeutic use; Adult; Aged; Aged, 80 and over; Ataxia - blood; Ataxia - complications; Ataxia - drug therapy; Ataxia - immunology; Autoantibodies - blood; Autoantibodies - immunology; Biomarkers, Pharmacological - blood; Case-Control Studies; Cells, Cultured; Contactin 1 - immunology; Contactin 1 - metabolism; Epitopes - immunology; Female; Ganglia, Spinal - metabolism; Humans; Immunoglobulin G - blood; Immunoglobulin G - immunology; Immunoglobulins, Intravenous - therapeutic use; Life Sciences; Male; Middle Aged; Neurons and Cognition; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating - blood; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating - complications; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating - drug therapy; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating - immunology; Retrospective Studies; myelin; chronic inflammatory demyelinating polyneuropathy; nodes of Ranvier; autoantibody; contactin 1
• ISSN: 0006-8950; 1460-2156; 1460-2156
• DOI: 10.1093/brain/awv054

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.