多发性硬化症与法布雷病患者脑白质病变的神经影像学鉴别。
Neuroradiological differentiation of white matter lesions in patients with multiple sclerosis and Fabry disease.
机构信息
Department of Neurology, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria.
Department of Biomedical Imaging and Image-Guided Therapy, Medical University of Vienna, Vienna, Austria.
出版信息
Orphanet J Rare Dis. 2022 Feb 5;17(1):37. doi: 10.1186/s13023-022-02187-y.
OBJECTIVE
White matter lesions (WML) in multiple sclerosis (MS) differ from vascular WML caused by Fabry disease (FD). However, in atypical cases the discrimination can be difficult and may vary between individual raters. The aim of this study was to evaluate interrater reliability of WML differentiation between MS and FD patients.
MATERIALS AND METHODS
Brain MRI scans of 21 patients with genetically confirmed FD were compared to 21 matched patients with MS. Pseudonymized axial FLAIR sequences were assessed by 6 blinded raters and attributed to either the MS or the FD group to investigate interrater reliability. Additionally, localization of WML was compared between the two groups.
RESULTS
The median age of patients was 46 years (IQR 35-58). Interrater reliability was moderate with a Fleiss' Kappa of 0.45 (95%CI 0.3-0.59). Overall, 85% of all ratings in the MS group and 75% in the FD group were correct. However, only 38% of patients with MS and 33% of patients with FD were correctly identified by all 6 raters. WML involving the corpus callosum (p < 0.001) as well as juxtacortical (p < 0.001) and infratentorial lesions (p = 0.03) were more frequently observed in MS patients.
CONCLUSION
Interrater reliability regarding visual differentiation of WML in MS from vascular WML in FD on standard axial FLAIR images alone is only moderate, despite the distinctive features of lesions in each group.
目的
多发性硬化症（MS）中的脑白质病变（WML）与法布里病（FD）引起的血管性 WML 不同。然而，在不典型病例中，这种鉴别可能具有难度，并且在不同的评估者之间可能存在差异。本研究旨在评估评估者之间区分 MS 和 FD 患者 WML 的可靠性。
材料与方法
将 21 例经基因证实的 FD 患者的脑部 MRI 扫描与 21 例匹配的 MS 患者进行比较。对 6 名盲法评估者的匿名轴向 FLAIR 序列进行评估，并将其归因于 MS 或 FD 组，以调查评估者之间的可靠性。此外，比较两组之间 WML 的定位。
结果
患者的中位年龄为 46 岁（IQR 35-58）。Fleiss' Kappa 为 0.45（95%CI 0.3-0.59），提示评估者之间的可靠性为中度。在 MS 组中，85%的评分和 FD 组中 75%的评分是正确的。然而，只有 38%的 MS 患者和 33%的 FD 患者被所有 6 名评估者正确识别。胼胝体受累（p<0.001）、皮质下（p<0.001）和幕下病变（p=0.03）在 MS 患者中更常见。
结论
仅凭标准轴向 FLAIR 图像对 MS 中的 WML 与 FD 中的血管性 WML 进行视觉区分的评估者之间的可靠性仅为中度，尽管两组病变的特征明显不同。
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