抗接触蛋白1抗体相关慢性炎性脱髓鞘性多发性神经根神经病一例
Anti-contactin-1 IgG4 antibody associated chronic inflammatory demyelinating polyradiculoneuropathy: a case report
摘要抗接触蛋白1 IgG4抗体相关慢性炎性脱髓鞘性多发性神经根神经病(CIDP)的临床表现独特,患者发病年龄偏高,进展迅速,肢体无力明显,伴显著的感觉性共济失调,对静脉输注丙种球蛋白反应差,神经电生理检查表现为传导速度减慢,早期复合肌肉动作电位波幅下降明显但不伴有波形离散。在此报道1例抗接触蛋白1 IgG4抗体阳性CIDP患者,并复习相关文献,以提高临床医师对这一少见疾病的认识。
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abstractsAnti-contactin-1(CNTN1) IgG4 antibody is a reliable biomarker of a specific subset of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients with distinct clinical features, including advanced age, aggressive symptom, predominantly motor involvement, sensory ataxia, and poor response to intravenous immunoglobulin. Nerve conduction study showed decrease of nerve conduction velocity, reduction of compound motor active potential amplitude without temporal dispersion at early stage of the disease. The article reported an anti-CNTN1 IgG4 antibody positive case, reviewed the current relevant literature, and then summarized the clinical characteristics of anti-CNTN1 IgG4 antibody associated CIDP.
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