Neurofascin-155 IgM autoantibodies in patients with inflammatory neuropathies
Neurofascin-155 IgM autoantibodies in patients with inflammatory neuropathies
复制标题
DOI：
10.1136/jnnp-2018-318170
复制
发表时间：
2018-11-01
影响因子：
11
通讯作者：
Sommer, Claudia
中科院分区：
文献类型：
--
作者：
Doppler, Kathrin;Stengel, Helena;Sommer, Claudia
目的近年来，针对不同旁结蛋白的IgG自身抗体被检测到，这使得炎症性神经病的治疗取得了重要进展。方法对140例炎性神经病患者进行了抗神经成束蛋白155、186和contactin-1的IgM自身抗体筛查。结果ELISA法检测5例患者存在抗神经成束蛋白155、186和contactin-1的IgM自身抗体。其中4例为炎性脱髓鞘性多神经根神经病（CIDP），1例为格林-巴利综合征（GBS），并经ELISA预吸收实验和Western blot证实。滴度范围为1：100至1：400。我们在该队列中未检测到IgM抗神经成束蛋白-186或抗接触蛋白-1抗体。所有患者均表现为远端加重的四肢轻瘫和感觉减退。值得注意的是，震颤存在于三名CIDP患者中，并发生在疾病急性期后的GBS患者中。神经传导检查显示远端运动延迟和F波延迟延长。神经活检显示，在三名患者的继发性轴突损伤的迹象，脱髓鞘的功能，在一名患者。梳理纤维制剂没有表现出paranodaldamage.Conclusion总之，IgM neurofascin-155自身抗体可能是值得测试的炎症性神经病变患者。它们的致病作用需要在未来的实验中确定。
Objectives Recently, IgG autoantibodies against different paranodal proteins have been detected and this has led to important advances in the management of inflammatory neuropathies. In contrast, not much is known on IgM autoantibodies against paranodal proteins.Methods In the present study, we screened a large cohort of patients (n=140) with inflammatory neuropathies for IgM autoantibodies against neurofascin-155, neurofascin-186 or contactin-1.Results IgM autoantibodies against neurofascin-155 were detected by ELISA in five patients, four with inflammatory demyelinating polyradiculoneuropathy (CIDP) and one with Guillain-Barre syndrome (GBS), and were confirmed by ELISA-based preabsorption experiments and Western blot. Titres ranged from 1:100 to 1:400. We did not detect IgM anti-neurofascin-186 or anti-contactin-1 antibodies in this cohort. All patients presented with distally accentuated tetraparesis and hypesthesia. Remarkably, tremor was present in three of the patients with CIDP and occurred in the patients with GBS after the acute phase of disease. Nerve conduction studies revealed prolonged distal motor latencies and F wave latencies. Nerve biopsies showed signs of secondary axonal damage in three of the patients, demyelinating features in one patient. Teased fibre preparations did not demonstrate paranodal damage.Conclusion In summary, IgM neurofascin-155 autoantibodies may be worth testing in patients with inflammatory neuropathies. Their pathogenic role needs to be determined in future experiments.