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.
Muscle Nerve. 2018 Mar;57(3):498-502. doi: 10.1002/mus.25757. Epub 2017 Aug 23.
Neurofascin155 (NF155) is a target antigen for autoantibodies in a subset of chronic inflammatory demyelinating polyneuropathy (CIDP).
We report the cases of 4 patients with anti-NF155 immunoglobulin G4 (IgG4) antibody-positive CIDP who underwent sural nerve biopsies.
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.
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.
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。
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。
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。
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.
