Background

Myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) may rarely be associated with peripheral nervous system involvement. We aimed to test MOG-Abs in patients with undetermined peripheral neuropathy (PN).

Methods

Consecutive patients with available sural nerve biopsy and paired serum sample were retrospectively identified (January, 1st 2016-November, 1st 2021) and tested for MOG-Abs with live cell-based assay (CBA). Patients with antibody titre ≥1:160 (secondary H + L antibody) and selective MOG-IgG presence (IgG-Fc predominance) were considered MOG-IgG positive. All positive samples were analysed with immunohistochemistry and CBAs for antibodies against Neurofascin-155 and Contactin-1. Clinical and neuropathological data were collected through clinical reports.

Results

Among 163 patients, 5 (3%) resulted positive for predominantly IgG MOG-Abs (median titer 1:320, range 1:160–1:5120), none showed other concomitant antibodies. Median age was 74 years-old (range 55–81), median disease duration was 60 months (range 1–167), 60% of patients were female. Of these, 4/5 cases had clinical features suggestive of acute (n = 1) or chronic (n = 3) inflammatory demyelinating neuropathy, 2/5 fulfilled the criteria of combined central and peripheral demyelination (CCPD) whilst 3/5 had isolated PNS involvement. Neuropathological findings showed mixed axonal-demyelinating features in 2/5, predominant demyelination in 3/5 cases. Other neuropathological hallmarks included paranodal demyelination (n = 3), myelin outfoldings (n = 4), slight inflammatory infiltrates (n = 3), onion bulbs (n = 3), and clusters of regeneration (n = 4).

Discussion

MOG-IgG can be detected in patients with isolated PN or CCPD. Clinical and neuropathological features are suggestive for demyelination and slight inflammation. Further studies should include larger cohorts of patients to elucidate the utility of MOG-Abs testing in PN.

中文翻译：

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周围神经病变和 MOG-IgG：一项临床和神经病理学回顾性研究

背景

髓鞘少突胶质细胞糖蛋白抗体 (MOG-Abs) 可能很少与周围神经系统受累相关。我们的目的是在未确定的周围神经病变 (PN) 患者中测试 MOG-Abs。

方法

回顾性地确定了具有可用腓肠神经活检和配对血清样本的连续患者（2016 年 1 月 1 日至 2021 年 11 月 1 日），并使用基于活细胞的测定法 (CBA) 测试了 MOG-Abs。抗体滴度≥1:160（二级H  +  L抗体）和选择性 MOG-IgG 存在（IgG-Fc 优势）的患者被认为是 MOG-IgG 阳性。所有阳性样品均用免疫组织化学和 CBA 分析了针对 Neurafascin-155 和 Contactin-1 的抗体。通过临床报告收集临床和神经病理学数据。

结果

在 163 名患者中，5 名 (3%) 的 IgG MOG-Abs 呈阳性（中位滴度 1:320，范围 1:160–1:5120），没有其他伴随抗体。中位年龄为 74 岁（范围 55-81），中位病程为 60 个月（范围 1-167），60% 的患者为女性。其中，4/5 例具有提示急性 ( n  = 1) 或慢性 ( n  = 3) 炎症性脱髓鞘性神经病的临床特征，2/5 符合中枢和外周联合脱髓鞘 (CCPD) 的标准，而 3/5 已孤立PNS 参与。神经病理学结果显示 2/5 的混合轴突脱髓鞘特征，3/5 的病例以脱髓鞘为主。其他神经病理学标志包括旁路脱髓鞘（n  = 3）、髓鞘外翻（n = 4）、轻微炎症浸润（n  = 3）、洋葱鳞茎（n  = 3）和再生簇（n  = 4）。

讨论

MOG-IgG 可在孤立性 PN 或 CCPD 患者中检测到。临床和神经病理学特征提示脱髓鞘和轻微炎症。进一步的研究应包括更大的患者队列，以阐明 MOG-Abs 检测在 PN 中的效用。