期刊
BRAIN
卷 138, 期 -, 页码 1484-1491出版社
OXFORD UNIV PRESS
DOI: 10.1093/brain/awv054
关键词
autoantibody; chronic inflammatory demyelinating polyneuropathy; contactin 1; nodes of Ranvier; myelin
资金
- Singapore National Medical Research Council [IRG 10nov086, CSA/047/2012]
- Agence Nationale pour la Recherche (ACAMIN)
- ERA-Net for Research on Rare Diseases
- CSL Behring's grant in immunology
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.
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