Objective

To elucidate the clinical characteristics of Japanese pediatric patients with acquired demyelinating diseases (ADS), positive for myelin oligodendrocyte glycoprotein antibody (MOG-IgG), we conducted a nation-wide survey.

Methods

Information about pediatric patients under 18 years old with ADS was solicited with surveys sent to 323 facilities. In an initial survey, we asked whether the center had any patients with ADS, and the MOG-IgG serostatus of the patients. In a follow-up survey, we requested more precise information on patients with ADS.

Results

Initial survey: 263 replies providing information on 175 patients were received. MOG-IgG were examined in 78 patients and 54 of those (69%) were positive for MOG-IgG. Follow-up survey: The characteristic involvement was optic neuritis, with visual disturbance and optic pain as characteristic symptoms. The relapse rate was 44% in patients positive for MOG-IgG, which was higher than that in seronegative patients (38%). For acute phase treatments, corticosteroid (CS), plasma exchange, and intravenous immunoglobulin (IVIG) were useful. To prevent relapse, CS, intermittent IVIG, immunosuppressants, and monoclonal antibodies were useful, but the efficacies of disease modifying drugs were uncertain. Sequelae such as visual disturbance, cognitive impairment, motor dysfunction, and epilepsy were observed in 11% of patients with MOG-IgG.

Conclusions

MOG antibody-associated diseases were found to be common among pediatric ADS patients. Since a variety of sequelae were observed in these patients, it is important to identify the appropriate treatment to ensure the best outcome. The presence of the MOG autoantibody should be taken into consideration as part of the diagnostic criteria for pediatric ADS.

中文翻译：

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日本儿童 MOG 抗体相关疾病的全国调查

客观的

为了阐明日本获得性脱髓鞘疾病 (ADS)、髓鞘少突胶质细胞糖蛋白抗体 (MOG-IgG) 阳性儿科患者的临床特征，我们进行了一项全国性调查。

方法

向 323 家机构发送调查问卷，收集了有关 18 岁以下患有 ADS 的儿科患者的信息。在初步调查中，我们询问该中心是否有任何 ADS 患者，以及患者的 MOG-IgG 血清状况。在后续调查中，我们要求获得有关 ADS 患者的更准确信息。

结果

初步调查：收到 263 份回复，提供 175 名患者的信息。在 78 名患者中检查了 MOG-IgG，其中 54 名 (69%) 为 MOG-IgG 阳性。随访调查：特征性受累为视神经炎，以视力障碍和视神经痛为特征性症状。MOG-IgG阳性患者的复发率为44%，高于血清阴性患者（38%）。对于急性期治疗，皮质类固醇 (CS)、血浆置换和静脉注射免疫球蛋白 (IVIG) 是有用的。为了防止复发，CS、间歇性IVIG、免疫抑制剂和单克隆抗体是有用的，但改善疾病的药物的疗效尚不确定。11% 的 MOG-IgG 患者出现视觉障碍、认知障碍、运动功能障碍和癫痫等后遗症。

结论

发现 MOG 抗体相关疾病在儿科 ADS 患者中很常见。由于在这些患者中观察到了各种后遗症，因此确定适当的治疗以确保最佳结果非常重要。MOG 自身抗体的存在应作为儿科 ADS 诊断标准的一部分予以考虑。