抗神经束蛋白 155 免疫球蛋白 G4 抗体相关慢性炎症性脱髓鞘性多发性神经病的电子显微镜异常和治疗效果。
ELectron microscopic abnormality and therapeutic efficacy in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin155 immunoglobulin G4 antibody.
机构信息
Department of Neurology, Kindai University Faculty of Medicine, 377-2 Ohno-Higashi, Osaka-Sayama, Osaka, 589-8511, Japan.
Department of Neurology, National Hospital Organization Minami-Kyoto Hospital, Kyoto, Japan.
出版信息
Muscle Nerve. 2018 Mar;57(3):498-502. doi: 10.1002/mus.25757. Epub 2017 Aug 23.
INTRODUCTION
Neurofascin155 (NF155) is a target antigen for autoantibodies in a subset of chronic inflammatory demyelinating polyneuropathy (CIDP).
METHODS
We report the cases of 4 patients with anti-NF155 immunoglobulin G4 (IgG4) antibody-positive CIDP who underwent sural nerve biopsies.
RESULTS
All patients were relatively young at onset. Three patients experienced tremors, and 2 patients had severe ataxia. Although the response to intravenous immunoglobulin was poor in all patients, plasma exchange and corticosteroids were at least partially effective. Immunoadsorption plasmapheresis was performed in 1 patient but was ineffective. Electron microscopic examination of sural nerve biopsies revealed loss of paranodal transverse bands in all patients.
DISCUSSION
Anti-NF155 IgG4 antibody-positive CIDP shows distinctive clinicopathological features, indicating that the IgG4 antibody is directly associated with the pathogenic mechanisms of anti-NF155 IgG4 antibody-positive CIDP. Muscle Nerve 57: 498-502, 2018.
简介
神经束蛋白 155（NF155）是一组慢性炎症性脱髓鞘性多发性神经病（CIDP）患者体内自身抗体的靶抗原。
方法
我们报告了 4 例抗 NF155 免疫球蛋白 G4（IgG4）抗体阳性 CIDP 患者接受腓肠神经活检的病例。
结果
所有患者的发病年龄均相对较轻。3 例患者出现震颤，2 例患者出现严重共济失调。尽管所有患者对静脉注射免疫球蛋白的反应均不佳，但血浆置换和皮质类固醇至少部分有效。1 例患者进行了免疫吸附血浆置换，但无效。腓肠神经活检的电子显微镜检查显示所有患者的连接蛋白横带丢失。
讨论
抗 NF155 IgG4 抗体阳性 CIDP 表现出独特的临床病理特征，表明 IgG4 抗体与抗 NF155 IgG4 抗体阳性 CIDP 的致病机制直接相关。《肌肉神经》57:498-502, 2018.
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