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Brain. 2015 Jun;138(Pt 6):1484-91. doi: 10.1093/brain/awv054. Epub 2015 Mar 25.

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

Author information

1
1 Department of Medicine, Yong Loo Lin School of Medicine, National University of Singapore, Singapore.
2
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.
3
2 Aix-Marseille Université, CNRS, CRN2M-UMR 7286, Marseille, France.
4
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.

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.

KEYWORDS:

autoantibody; chronic inflammatory demyelinating polyneuropathy; contactin 1; myelin; nodes of Ranvier

PMID:
25808373
PMCID:
PMC4614146
DOI:
10.1093/brain/awv054
[Indexed for MEDLINE]
Free PMC Article

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