Contactin 1 IgG4 associates to chronic inflammatory demyelinating polyneuropathy with sensory ataxia
Brain. 2015-03-25; 138(6): 1484-1491
DOI: 10.1093/brain/awv054
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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.