Distal acquired demyelinating symmetric neuropathy
Distal acquired demyelinating symmetric neuropathy
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DOI：
10.1212/wnl.54.3.615
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发表时间：
2000-02-08
期刊：
影响因子：
9.9
通讯作者：
Barohn, RJ
中科院分区：
文献类型：
--
作者：
Katz, JS;Saperstein, DS;Barohn, RJ
目的：描述一种具有远端感觉或感觉运动特征的获得性、对称、脱髓鞘神经病变变异。背景：典型的慢性炎症性脱髓鞘性多根神经病变（CIDP）患者有明显的近端和远端虚弱。然而，慢性脱髓鞘神经病变可能表现为不同的表型。一种主要根据虚弱的模式来区分这些疾病的方法可能对临床医生有用。方法：回顾性分析53例获得性对称性脱髓鞘多神经病变患者，首先根据病变类型，其次根据单克隆蛋白（m蛋白）的存在和类型进行分类。作者区分了远端感觉或感觉运动受累的患者，被称为远端获得性脱髓鞘对称（DADS）神经病变，与近端和远端虚弱的患者，被称为CIDP。结果：22%的CIDP患者存在m蛋白。没有明显区分有或没有m蛋白的CIDP患者的特征，几乎所有这些患者都对免疫调节治疗有反应。相比之下，近三分之二的DADS神经病变患者患有免疫球蛋白M (IgM) kappa单克隆γ病变，这种特异性组合预示着对免疫调节治疗的反应较差。67%的患者存在抗髓鞘相关糖蛋白（anti-MAG）抗体。结论：通过表型区分获得性脱髓鞘神经病变通常可以预测IgM kappa m蛋白、抗mag抗体的存在以及对免疫调节治疗的反应。
Objective: To characterize an acquired, symmetric, demyelinating neuropathic variant with distal sensory or sensorimotor features. Background: Classic chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients have prominent proximal and distal weakness.' However, chronic demyelinating neuropathies may present with different phenotypes. An approach that distinguishes these: disorders primarily according to the pattern of weakness may be useful to the clinician. Methods: A total of 53 patients with acquired symmetric demyelinating polyneuropathies were classified primarily according to the pattern of the neuropathy and secondarily according to the presence and type of monoclonal protein (M-protein) in this retrospective review. The authors distinguished between patients with distal sensory or sensorimotor involvement, designated as distal acquired demyelinating symmetric (DADS) neuropathy, from those with proximal and distal weakness, who were designated las CIDP, Results: M-proteins were present in 22% of patients with CIDP. There were no features that distinguished clearly between CIDP patients with or without an M-protein, and nearly all of these patients responded to immunomodulating therapy. In contrast, nearly two-thirds of the patients with DADS neuropathy had immunoglobulin M (IgM) kappa, monoclonal gammopathies, and this specific combination predicted a poor response to immunomodulating therapy. Antimyelin-associated glycoprotein (anti-MAG) antibodies were present in 67% of these patients, Conclusion: Distinguishing acquired demyelinating neuropathies by phenotype can often predict the presence of IgM kappa M-proteins, anti-MAG antibodies, and responses to immunomodulating therapy.