Parallel fluctuation of anti-neurofascin 155 antibody levels with clinico-electrophysiological findings in patients with chronic inflammatory demyelinating polyradiculoneuropathy
Parallel fluctuation of anti-neurofascin 155 antibody levels with clinico-electrophysiological findings in patients with chronic inflammatory demyelinating polyradiculoneuropathy
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DOI：
10.1016/j.jns.2017.11.035
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发表时间：
2018-01-15
影响因子：
4.4
通讯作者：
Kira, Jun-ichi
中科院分区：
文献类型：
--
作者：
Fujita, Atsushi;Ogata, Hidenori;Kira, Jun-ichi
背景资料：慢性炎性脱髓鞘性多发性神经根神经病（CIDP）与抗神经成束蛋白155（NF 155）抗体的长期临床过程和密切相关的生物标志物仍有待阐明。方法：我们回顾性研究了3名日本男性抗NF 155抗体阳性CIDP患者的纵向临床过程。结果：3例患者均表现为慢性进行性感觉运动障碍，发病年龄分别为16、26和34岁，随访时间分别为58、31和38个月。所有患者均有姿势性震颤和全身深腱反射降低。脑脊液蛋白峰值水平> 400 mg/dl，神经传导研究（NCS）显示严重的脱髓鞘模式。包括静脉注射免疫球蛋白、血浆置换、皮质类固醇和其他免疫抑制剂在内的联合免疫疗法改善了临床严重程度和NCS异常，握力改善> 10 kg，F波潜伏期改善至少20%。然而，他们的症状在免疫治疗逐渐减少后加重。结论：抗NF 155抗体阳性CIDP患者的临床病程提示，抗NF 155抗体水平和NCS表现可作为CIDP患者疾病活动性的标志。
Background: The long-term clinical course and closely related biomarkers in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with anti-neurofascin 155 (NF155) antibodies remain to be elucidated.Methods: We retrospectively studied the longitudinal clinical courses of three Japanese male anti-NF155 antibody -positive CIDP patients. Anti-NF155 antibody levels were measured by flow cytometry using HEK293 cell lines stably expressing human NF155.Results: All three patients presented with chronic progressive sensorimotor disturbance, with ages at onset of 16, 26, and 34 years old, and they were followed for 58, 31, and 38 months, respectively, from the onset. All patients had postural tremor and generalized decreased deep tendon reflexes. Peak cerebrospinal fluid protein levels were > 400 mg/dl, and nerve conduction studies (NCS) showed severe demyelination patterns. Combined immunotherapies including intravenous immunoglobulin, plasma exchange, corticosteroids, and other immunosuppressants ameliorated clinical severity and NCS abnormalities, with improvements of > 10 kg in grip strength and at least 20% in F-wave latencies. However, their symptoms exacerbated after the immunotherapies were tapered. Anti-NF155 antibody levels varied in parallel with the clinical and electrophysiological changes, or preceded them.Conclusion: The patients' clinical courses suggest that anti-NF155 antibody levels and NCS findings could be disease activity markers in anti-NF155 antibody-positive CIDP.