Contactin 1 IgG4 associates to chronic inflammatory demyelinating polyneuropathy with sensory ataxia
Contactin 1 IgG4 associates to chronic inflammatory demyelinating polyneuropathy with sensory ataxia
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DOI：
10.1093/brain/awv054
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发表时间：
2015-06-01
期刊：
影响因子：
14.5
通讯作者：
Yuki, Nobuhiro
中科院分区：
文献类型：
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作者：
Miura, Yumako;Devaux, Jerome J.;Yuki, Nobuhiro
一个西班牙小组最近报道，4名携带抗接触蛋白1 IgG 4自身抗体的慢性炎性脱髓鞘性多发性神经病患者表现出攻击性症状发作和对静脉注射免疫球蛋白的反应不良。我们的目的是描述日本慢性炎性脱髓鞘性多发性神经病患者的临床和血清学特征显示抗接触蛋白1抗体。533例慢性炎性脱髓鞘性多发性神经病患者中有13例（2.4%）有抗接触蛋白1 IgG 4，而疾病或正常对照组患者均无（P = 0.02）。13例患者中有3例（23%）表现为亚急性症状发作，但所有患者均表现为感觉性共济失调。6/10例（60%）抗接触素1抗体阳性患者对静脉注射免疫球蛋白的反应较差，而8/11例（73%）抗体阳性患者对皮质类固醇的反应良好。抗接触蛋白1 IgG 4抗体是指导治疗选择的可能生物标志物。
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.