Almuqbil Mohammed, Aljomah Lama, Almahmoud Nora, Altuwaijri Waleed, Alrumayyan Ahmad, Alrifai Muhammad T
College of Medicine, King Saud Bin Abdulaziz University for Health Sciences (KSAU-HS), Riyadh, Saudi Arabia.
King Abdullah International Medical Research Center (KAIMRC), Ministry of National Guard, Riyadh, Saudi Arabia.
Neurol Ther. 2025 Jun 4. doi: 10.1007/s40120-025-00768-0.
Although acquired demyelinating syndromes (ADS) are rare in children, the incidence and prevalence of ADS vary internationally. As data on pediatric ADS in Saudi Arabia is limited, the aim of this study was to describe the clinical spectrum of pediatric ADS, its clinical characteristics, and management options at a tertiary hospital in Saudi Arabia.
A retrospective observational study was conducted at King Abdulaziz Medical City (KAMC) and King Abdullah Specialized Children Hospital (KASCH) in Riyadh, Saudi Arabia between January 2016 and December 2022. All patients with ADS fulfilling criteria of each subtype (multiple sclerosis (MS), clinically isolated syndrome (CIS), acute disseminated encephalomyelitis (ADEM), and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD)) were included in this study.
Forty-five pediatric patients with ADS were analyzed, with the majority diagnosed with MS. The median age of onset was higher in the MS group compared to monophasic CIS and MOGAD, with statistically significant differences in age at onset between the MS group and both the CIS and MOGAD groups (p = 0.0002). Significant differences were also observed in the type of initial central nervous system (CNS) attack, with optic neuritis being more common in MS and transverse myelitis in CIS (p < 0.0001). Laboratory results revealed a higher incidence of cerebrospinal fluid (CSF) oligoclonal bands in patients with MS, which was statistically significant (p = 0.04), and MOG antibodies were found in all patients with MOGAD. Intravenous pulse steroids were administered in most patients, while disease-modifying drugs (DMTs) were employed most frequently in patients with MS. The Expanded Disability Status Scale scores indicated little disability in most patients with MS and CIS, with more disability noted in a subgroup of ADEM. Overall, the study underscores the clinical heterogeneity of pediatric ADS and points out the statistically significant difference in age at onset, presenting features, and laboratory findings among ADS subtypes.
This study provides a thorough overview of pediatric ADS, including important distinctions between MS, ADEM, CIS, and MOGAD. Marked differences in age at onset, presentation, and imaging among these subtypes are informative for maximizing diagnosis and treatment. The key findings are the subsequent development of MS from CIS and MOGAD, varying patterns of first attack, and imaging characteristics like callososeptal interface lesions in MS and posterior fossa hyperintensities in MOGAD. All these indicate the need for individualized diagnostic and therapeutic approaches for improved outcomes.
尽管获得性脱髓鞘综合征（ADS）在儿童中较为罕见，但ADS的发病率和患病率在国际上有所不同。由于沙特阿拉伯关于儿童ADS的数据有限，本研究的目的是描述沙特阿拉伯一家三级医院中儿童ADS的临床谱、其临床特征及治疗选择。
2016年1月至2022年12月期间，在沙特阿拉伯利雅得的阿卜杜勒阿齐兹国王医疗城（KAMC）和阿卜杜拉国王专科医院（KASCH）进行了一项回顾性观察研究。所有符合各亚型标准（多发性硬化症（MS）、临床孤立综合征（CIS）、急性播散性脑脊髓炎（ADEM）和髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD））的ADS患者均纳入本研究。
对45例儿童ADS患者进行了分析，其中大多数被诊断为MS。与单相CIS和MOGAD相比，MS组的发病年龄中位数更高，MS组与CIS组和MOGAD组之间的发病年龄存在统计学显著差异（p = 0.0002）。在初始中枢神经系统（CNS）发作类型上也观察到显著差异，视神经炎在MS中更常见，横贯性脊髓炎在CIS中更常见（p < 0.0001）。实验室结果显示，MS患者脑脊液（CSF）寡克隆带的发生率更高，具有统计学显著性（p = 0.04），并且在所有MOGAD患者中均发现了MOG抗体。大多数患者接受了静脉脉冲类固醇治疗，而疾病修饰药物（DMTs）在MS患者中使用最为频繁。扩展残疾状态量表评分表明，大多数MS和CIS患者几乎没有残疾，而ADEM亚组中残疾情况更为明显。总体而言，该研究强调了儿童ADS的临床异质性，并指出了ADS各亚型在发病年龄、表现特征和实验室检查结果方面的统计学显著差异。
本研究全面概述了儿童ADS，包括MS、ADEM、CIS和MOGAD之间的重要区别。这些亚型在发病年龄、表现和影像学方面的显著差异有助于优化诊断和治疗。关键发现包括CIS和MOGAD后续发展为MS、首发发作的不同模式以及MS中的胼胝体间隔界面病变和MOGAD中的后颅窝高信号等影像学特征。所有这些都表明需要采用个体化的诊断和治疗方法以改善预后。
