抗NF155 IgG4抗体阳性慢性炎性脱髓鞘性多发性神经根神经病一例并文献复习

The features of an anti-NF155 IgG4 antibodies positive chronic inflammatory demyelinating polyradiculoneuropathy patient and a literature review

目的总结抗NF155IgG4抗体阳性慢性炎性脱髓鞘性多发性神经根神经病(chronic inflammatory demyelinating polyradiculoneuropathy,CIDP)的临床特点。方法收集作者医院收治的1例具有特殊表型的CIDP患者的临床资料,经流式细胞技术及免疫组化方法检测其血清抗NF155IgG4抗体,并结合文献进行复习。结果该患者抗NF155抗体阳性,且为IgG4亚型。临床具有发病年龄早、远端对称性脱髓鞘性周围神经病、感觉性共济失调及震颤等表现,影像学可见脊神经根对称增粗水肿,神经病理学可见有髓纤维中度减少伴髓鞘脱失。结论抗NF155IgG4抗体阳性的CIDP患者具有较为特异的临床特征,确诊需进行血清NF155IgG4抗体检测。该病对丙种球蛋白治疗效果差,提高对本病的认识和鉴别能力对治疗具有指导意义。

Objective To investigate the clinical features of anti-NF155 antibodies positive chronic inflammatory demyelinating polyradiculoneuropathy（CIDP）.Methods We analyzed a CIDP patient with specific clinical features,detected the serum anti-NF155 IgG4 antibodies by flow cytometry（FCM）and immunohistochemistry and reviewed the literature.Results This patient has anti-NF155 antibodies of IgG4 subtype.He has a feature of younger onset,distal acquired demyelinating symmetric neuropathy with sensory ataxia and tremor.Symmetric hypertrophy of nerve roots can be seen on MRI.Myelinated fibers are moderately decreased and demyelinated.Conclusions The anti-NF155IgG4 antibodies positive patient has specific features and is confirmed by screening the serum anti-NF155 IgG4 antibodies.Due to its poor response to IV immunoglobulin（IvIg）,a better understanding and identification of this disease is necessary to optimal treatment.