Department of Neurology, University Hospital Würzburg, Josef-Schneider-Str. 11, 97080, Würzburg, Germany
Department of Neurology, University Hospital Würzburg, Josef-Schneider-Str. 11, 97080, Würzburg, Germany.
J Neurol Neurosurg Psychiatry. 2018 Nov;89(11):1145-1151. doi: 10.1136/jnnp-2018-318170. Epub 2018 Jun 26.
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.
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.
IgM autoantibodies against neurofascin-155 were detected by ELISA in five patients, four with inflammatory demyelinating polyradiculoneuropathy (CIDP) and one with Guillain-Barré 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.
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.
最近，针对不同神经节段蛋白的 IgG 自身抗体已被检测到，这对炎性神经病的治疗有重要的推动作用。相比之下，针对神经节段蛋白的 IgM 自身抗体知之甚少。
在本研究中，我们筛选了 140 例炎性神经病患者，检测了针对神经束蛋白-155、神经束蛋白-186 或接触蛋白-1 的 IgM 自身抗体。
通过 ELISA 在五名患者中检测到针对神经束蛋白-155 的 IgM 自身抗体，其中四名患有炎性脱髓鞘性多发性神经根神经病（CIDP），一名患有格林-巴利综合征（GBS），通过 ELISA 预吸收实验和 Western blot 得到证实。滴度范围为 1:100 至 1:400。在本队列中未检测到 IgM 抗神经束蛋白-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无力和感觉减退。值得注意的是，三名 CIDP 患者存在震颤，且 GBS 患者在疾病的急性期后出现震颤。神经传导研究显示远端运动潜伏期和 F 波潜伏期延长。神经活检显示三名患者存在继发性轴索损伤的迹象，一名患者存在脱髓鞘特征。纤维 teased 制备未显示神经节段损伤。
总之，针对炎性神经病患者，可能值得检测 IgM 神经束蛋白-155 自身抗体。未来的实验需要确定其致病性作用。
