Department of Neurology, Washington University in St. Louis School of Medicine, St. Louis, Missouri.
Department of Neurology, Washington University in St. Louis School of Medicine, St. Louis, Missouri.
Pediatr Neurol. 2023 Sep;146:21-25. doi: 10.1016/j.pediatrneurol.2023.05.013. Epub 2023 May 25.
The central vein sign (CVS) on brain magnetic resonance imaging (MRI) is a promising diagnostic marker for distinguishing adult multiple sclerosis (MS) from other demyelinating conditions, but its prevalence is not well-established in pediatric-onset multiple sclerosis (POMS) versus myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). MOGAD can mimic MS radiologically. This study seeks to determine the utility of CVS, together with other radiological findings, in distinguishing POMS from MOGAD in children.
Children with POMS or MOGAD were identified in a pediatric demyelinating database. Two reviewers, blinded to diagnosis, fused fluid-attenuated inversion recovery sequences and susceptibility-weighted imaging from clinical imaging to identify CVS. Agreement in CVS number was reported using intraclass correlation coefficients (ICC). We performed topographic analyses as well as characterization of the clinical information and lesions on brain, spinal cord, and orbital MRI when available.
Twenty children, 10 with POMS and 10 with MOGAD, were assessed. The median lesion percentage of CVS was higher in POMS versus MOGAD for both raters (rater 1: 80% vs 9.8%; rater 2: 22.7% vs 7.5%). Inter-rater reliability for identifying total white matter lesions was strong (ICC 0.94 [95% confidence interval [CI] 0.84, 0.97]); however, it was poor for detecting CVS lesions (ICC -0.17 [95% CI: -0.37, 0.58]).
The CVS can be a useful diagnostic tool for differentiating POMS from MOGAD. However, advanced clinical imaging tools that can better detect CVS are needed to increase inter-rater reliability before clinical application.
脑磁共振成像（MRI）上的中央静脉征（CVS）是鉴别成人多发性硬化（MS）与其他脱髓鞘疾病的有前途的诊断标志物，但在儿童发病的多发性硬化（POMS）与髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD）中其患病率尚未得到充分证实。MOGAD 在影像学上可模拟 MS。本研究旨在确定 CVS 与其他影像学发现相结合，在儿童中区分 POMS 与 MOGAD 的作用。
在儿科脱髓鞘数据库中确定 POMS 或 MOGAD 患儿。两名审阅者在不了解诊断的情况下，融合临床影像中的液体衰减反转恢复序列和磁敏感加权成像，以识别 CVS。使用组内相关系数（ICC）报告 CVS 数量的一致性。我们进行了拓扑分析，以及对脑、脊髓和眼眶 MRI 上的临床信息和病变进行了特征描述。
评估了 20 名儿童，其中 10 名患有 POMS，10 名患有 MOGAD。两名评估者均发现 POMS 患者 CVS 的病变百分比中位数高于 MOGAD（评估者 1：80% vs 9.8%；评估者 2：22.7% vs 7.5%）。两名评估者识别总白质病变的一致性很强（ICC 0.94 [95%置信区间（CI）0.84，0.97]）；然而，检测 CVS 病变的一致性很差（ICC -0.17 [95% CI：-0.37，0.58]）。
CVS 可作为区分 POMS 与 MOGAD 的有用诊断工具。然而，在临床应用之前，需要更先进的能够更好地检测 CVS 的临床影像学工具来提高评估者间的可靠性。
