SOD Neurologia 1, Dipartimento Neuromuscolo-Scheletrico e degli Organi di Senso, Azienda Ospedaliero Universitaria Careggi, Florence, Italy.
Department of Neuroscience, Psychology, Drug Research and Child Health and Centro di Ricerca, University of Florence, Florence, Italy.
J Neurol Sci. 2021 Apr 15;423:117335. doi: 10.1016/j.jns.2021.117335. Epub 2021 Feb 17.
Polyneuropathies associated with monoclonal gammopathy of undetermined significance (MGUS) encompass a group of phenotypically and immunologically heterogeneous neuropathies. While the best characterized is that associated with anti-myelin glycoprotein (MAG) antibodies, there are phenotypical and immunological neuropathy variants that still lack a clear classification. We analyzed a significant number of patients, in order to better evaluate the distribution of neuropathy phenotypes and to look for some common characteristics.
Clinical, neurophysiological, and laboratory data from 87 consecutive MGUS patients with peripheral neuropathy were analyzed and compared among patient groups with different MGUS classes and autoantibody reactivity.
Anti-MAG neuropathy cases account for the most homogeneous group with regard to clinical and neurophysiological findings. Patients with anti-gangliosides or sulfatide (GS) antibodies, despite a marked phenotype heterogeneity, still share several common features, including a younger age at diagnosis, a more severe disease, and a prompt and sustained response to both immunoglobulin and rituximab therapies, mostly requiring chronic administration of immune treatment.
Although heterogeneous, MGUS-associated, anti-GS antibody positive neuropathies have important similar features possibly resulting from a similar biological background.
与意义未明的单克隆丙种球蛋白血症（MGUS）相关的多发性神经病包括一组表型和免疫上异质性的神经病。虽然最具特征性的是与抗髓鞘糖蛋白（MAG）抗体相关的神经病，但仍存在表型和免疫性神经病变体，这些变体缺乏明确的分类。我们分析了大量患者，以便更好地评估神经病表型的分布，并寻找一些共同特征。
分析了 87 例连续的 MGUS 伴周围神经病患者的临床、神经生理学和实验室数据，并比较了不同 MGUS 类别和自身抗体反应患者组之间的差异。
抗-MAG 神经病病例在临床和神经生理学发现方面是最具同质性的一组。尽管抗神经节苷脂或硫酸软骨素（GS）抗体的患者表现出明显的表型异质性，但仍有一些共同特征，包括诊断时年龄较小、疾病更严重、以及对免疫球蛋白和利妥昔单抗治疗均有迅速和持续的反应，大多数需要长期免疫治疗。
尽管 MGUS 相关的抗-GS 抗体阳性多发性神经病表现多样，但具有重要的相似特征，可能是由于类似的生物学背景所致。
