Anti-neurofascin 155 antibody-related neuropathy
Anti-neurofascin 155 antibody-related neuropathy
复制标题
抗神经成束蛋白 155 抗体相关神经病
DOI：
10.1111/cen3.12444
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发表时间：
2018
影响因子：
--
通讯作者：
Yamasaki Ryo
中科院分区：
文献类型：
--
作者：
Ogata Hidenori;Yamasaki Ryo
慢性炎性脱髓鞘性多神经病变（CIDP）是一种影响周围神经的顽固性炎性疾病。CIDP的病因仍有待确定，但它被认为是一种异质性疾病的混合物，呈现出多种临床和电生理表现。近年来，针对旁腺细胞粘附分子的自身抗体，如神经束蛋白155 （NF155）、接触蛋白1和接触蛋白相关蛋白1，已在CIDP患者亚群中检测到。与抗NF155抗体阴性的CIDP患者相比，抗NF155抗体阳性CIDP的临床特征包括发病年龄更年轻，远端肌无力、感觉性失调和震颤的频率更高，脑脊液蛋白水平更高，神经传导研究中更明显的脱髓鞘，磁共振神经造影显示神经根肥大。免疫球蛋白G4亚类的显著升高也是本病的一个特征。抗NF155抗体阳性的CIDP患者的腓肠神经活检标本显示，在没有炎症细胞浸润或洋葱球形成的偏执症患者中，轴神经胶质脱离。虽然静脉注射免疫球蛋白对抗NF155抗体阳性的CIDP患者没有预期的那么有效，但其他免疫疗法，如皮质类固醇、血浆置换和B细胞消耗疗法似乎是有效的。早期诊断和治疗是预防继发性轴突变性的重要手段。本文综述了抗NF155抗体相关神经病变的最新进展。
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an intractable inflammatory disease affecting peripheral nerves. The etiology of CIDP remains to be established, but it is regarded as a mixture of heterogeneous conditions presenting a variety of clinical and electrophysiological manifestations. In recent years, autoantibodies against paranodal cell adhesion molecules, such as neurofascin 155 (NF155), contactin 1 and contactin‐associated protein 1, have been detected in subsets of CIDP patients. The clinical characteristics of anti‐NF155 antibody‐positive CIDP have been delineated, and include a younger onset age, higher frequency of distal muscle weakness, sensory ataxia and tremor, higher cerebrospinal fluid protein levels, more conspicuous demyelination on nerve conduction studies, and nerve root hypertrophy on magnetic resonance neurography, when compared with anti‐NF155 antibody‐negative CIDP patients. Predominant elevation of immunoglobulin G4 subclass is also a characteristic of this disease. Sural nerve biopsy specimens of anti‐NF155 antibody‐positive CIDP patients show axo‐glial detachment in paranodes without inflammatory cell infiltrates or onion bulb formation. While intravenous immunoglobulin is not as effective as expected against anti‐NF155 antibody‐positive CIDP patients, other immunotherapies, such as corticosteroids, plasmapheresis and B‐cell depletion therapy, seem to be effective. Early diagnosis and treatment is important for preventing secondary axonal degeneration. The present review summarizes the emerging details of anti‐NF155 antibody‐related neuropathy.