In childhood, multiple sclerosis (MS) with a primary progressive disease course is very rare.1,2 Therefore, we read the recent article of Abdel-Mannan et al.3 presenting six pediatric patients fulfilling McDonald 2017 criteria for primary progressive MS (PPMS) with great interest. All patients presented with a progressive myelopathy, including progressive balance, and/or lower limb function impairment for at least 1 year. During follow-up, they had an ongoing progressive disease course, without sustained improvement and with a faster decline of Expanded Disability Status Scale (EDSS) compared to pediatric relapsing-remitting MS patients. In the four patients treated with intravenous methylprednisolone (ivMP), this treatment was unsuccessful. Patients continued to progress despite immunomodulatory treatment (azathioprine or hematopoietic stem cell transplantation). Only one patient was started on first-line disease-modifying therapy (DMT) for MS (interferon-beta-1a) but did not tolerate this. Although rare, Abdel-Mannan et al.3 plead for recognition of PPMS in childhood in order to start registered PPMS treatment in these patients in hope to prevent further deterioration.

中文翻译：

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患有原发性进行性样疾病的小儿 MS 患者可能仍具有相关的炎症活动，并可能受益于常规 MS 治疗

在儿童时期，具有原发性进展性疾病病程的多发性硬化症 (MS) 非常罕见。 1,2 因此，我们阅读了 Abdel-Mannan 等人最近发表的文章 3，其中介绍了 6 名符合 McDonald 2017 原发性进展性 MS (PPMS) 标准的儿科患者) 很有兴趣。所有患者均出现进行性脊髓病，包括进行性平衡和/或下肢功能受损至少 1 年。在随访期间，与儿科复发缓解型 MS 患者相比，他们的病程持续进行，没有持续改善，并且扩展残疾状态量表 (EDSS) 下降更快。在接受静脉注射甲基强的松龙 (ivMP) 的四名患者中，这种治疗没有成功。尽管进行了免疫调节治疗（硫唑嘌呤或造血干细胞移植），患者仍继续进展。只有一名患者开始接受 MS（干扰素-β-1a）的一线疾病缓解治疗 (DMT)，但无法耐受。尽管罕见，但 Abdel-Mannan 等人 3 恳求在儿童时期承认 PPMS，以便在这些患者中开始注册 PPMS 治疗，以期防止进一步恶化。