Department of Neurology and Center of Clinical Neuroscience, Charles University in Prague, First Faculty of Medicine and General University Hospital, Prague, Czech Republic.
Department of Radiology, Charles University in Prague, First Faculty of Medicine and General University Hospital, Prague, Czech Republic.
Mov Disord. 2020 Jun;35(6):994-1001. doi: 10.1002/mds.28018. Epub 2020 Mar 17.
MRI is a sensitive method for the assessment of brain abnormalities in Wilson disease, that is, T hyperintensities, T hypointensities, and atrophy, but a validated scoring system for the classification of radiological severity is lacking. The objective of this study was to develop and validate a brain MRI visual rating scale for Wilson disease.
The proposed Wilson disease brain MRI severity scale consists of acute toxicity and chronic damage subscores from predefined structures. The former, calculated by summing scores of T hyperintensities (excluding cavitation), is likely to be partially reversible with treatment. The latter, representing the sum of scores of T hypointensities and brain atrophy, reflects pathology that is not readily reversible. Validation was performed on MRI scans acquired using 1.5T system from 39 Wilson disease patients examined at baseline and after 24 months on anticopper treatment. Intraclass correlation coefficients of 5 ratings from 3 raters were calculated. Temporal evolution of the MRI severity score and its association with clinical severity, assessed using the Unified Wilson Disease Rating Scale part III, was calculated.
Intrarater and interrater agreement were good (r > 0.93; P < 0.001; and r > 0.74; P < 0.001, respectively). In neurologic Wilson disease patients, the total MRI severity score improved over 2 years (P = 0.032), mainly because of reduced acute toxicity (P = 0.0015), whereas the chronic damage score deteriorated (P = 0.035). Unified Wilson Disease Rating Scale part III score was positively associated with chronic damage and total score at baseline (P = 0.005 and P = 0.003, respectively) and in month 24 (P < 0.001 and P = 0.001, respectively).
The Wilson disease brain MRI severity scale is a simple, reliable, and valid instrument that allows semiquantitative assessment of radiological Wilson disease severity. © 2020 International Parkinson and Movement Disorder Society.
磁共振成像（MRI）是评估威尔逊病脑异常的一种敏感方法，即 T1 高信号、T1 低信号和萎缩，但缺乏用于分类放射学严重程度的经过验证的评分系统。本研究的目的是开发和验证一种用于威尔逊病的脑 MRI 视觉评分量表。
提出的威尔逊病脑 MRI 严重程度评分由来自预定义结构的急性毒性和慢性损伤子评分组成。前者通过对 T1 高信号（不包括空洞）的评分相加来计算，可能在治疗后部分可逆。后者代表 T1 低信号和脑萎缩评分的总和，反映了不易逆转的病理学。对使用 1.5T 系统获得的 39 例基线和 24 个月铜螯合治疗后进行 MRI 扫描进行验证。由 3 名评分者中的 5 名评分的组内相关系数进行计算。计算 MRI 严重程度评分的时间演变及其与使用统一威尔逊病评分量表第三部分评估的临床严重程度的相关性。
内部和外部评分者之间的一致性良好（r>0.93；P<0.001；和 r>0.74；P<0.001）。在神经威尔逊病患者中，总 MRI 严重程度评分在 2 年内有所改善（P=0.032），主要是由于急性毒性降低（P=0.0015），而慢性损伤评分恶化（P=0.035）。统一威尔逊病评分量表第三部分评分与基线时的慢性损伤和总评分呈正相关（P=0.005 和 P=0.003），与 24 个月时也呈正相关（P<0.001 和 P=0.001）。
威尔逊病脑 MRI 严重程度评分是一种简单、可靠和有效的工具，可用于半定量评估放射学威尔逊病严重程度。
