PDF(Pubmed)
Abstract：
UNASSIGNED: Cranial magnetic resonance imaging (MRI) could be a crucial tool for the assessment for neurological symptoms in patients with Wilson\'s disease (WD). Diffusion-weighted imaging (DWI) hyperintensity reflects the acute brain injuries, which mainly occur in specific brain regions. Therefore, this study aimed to develop a weighted cranial DWI scale for patients with WD, with special focus on specific brain regions.
UNASSIGNED: In total, 123 patients with WD were enrolled, 118 of whom underwent 1.5 T-MRI on admission. The imaging score was calculated as described previously and depended on the following sequences: one point was acquired when abnormal intensity occurred in the T1, T2, and fluid-attenuation inversion recovery sequences, and two points were acquired when DWI hyperintensity were found. Consensus weighting was conducted based on the symptoms and response to treatment.
UNASSIGNED: Intra-rater agreement were good (r = 0.855 [0.798-0.897], p < 0.0001). DWI hyperintensity in the putamen was a high-risk factor for deterioration during de-copper therapy (OR = 8.656, p < 0.05). The high-risk factors for readmission for intravenous de-copper therapies were DWI hyperintensity in the midbrain (OR = 3.818, p < 0.05) and the corpus callosum (OR = 2.654, p < 0.05). Both scoring systems had positive correlation with UWDRS scale (original semi-quantitative scoring system, r = 0.35, p < 0.001; consensus semi-quantitative scoring system, r = 0.351, p < 0.001.). Compared to the original scoring system, the consensus scoring system had higher correlations with the occurrence of deterioration (OR = 1.052, 95%CI [1.003, 1.0103], p < 0.05) and readmission for intravenous de-copper therapy (OR = 1.043, 95%CI [1.001, 1.086], p < 0.05).
UNASSIGNED: The predictive performance of the consensus semi-quantitative scoring system for cranial MRI was improved to guide medication, healthcare management, and prognosis prediction in patients with WD. For every point increase in the neuroimaging score, the risk of exacerbations during treatment increased by 5.2%, and the risk of readmission to the hospital within 6 months increased by 4.3%.
UNASSIGNED: In total, 123 patients with WD were enrolled, 118 of whom underwent 1.5 T-MRI on admission. The imaging score was calculated as described previously and depended on the following sequences: one point was acquired when abnormal intensity occurred in the T1, T2, and fluid-attenuation inversion recovery sequences, and two points were acquired when DWI hyperintensity were found. Consensus weighting was conducted based on the symptoms and response to treatment.
UNASSIGNED: Intra-rater agreement were good (r = 0.855 [0.798-0.897], p < 0.0001). DWI hyperintensity in the putamen was a high-risk factor for deterioration during de-copper therapy (OR = 8.656, p < 0.05). The high-risk factors for readmission for intravenous de-copper therapies were DWI hyperintensity in the midbrain (OR = 3.818, p < 0.05) and the corpus callosum (OR = 2.654, p < 0.05). Both scoring systems had positive correlation with UWDRS scale (original semi-quantitative scoring system, r = 0.35, p < 0.001; consensus semi-quantitative scoring system, r = 0.351, p < 0.001.). Compared to the original scoring system, the consensus scoring system had higher correlations with the occurrence of deterioration (OR = 1.052, 95%CI [1.003, 1.0103], p < 0.05) and readmission for intravenous de-copper therapy (OR = 1.043, 95%CI [1.001, 1.086], p < 0.05).
UNASSIGNED: The predictive performance of the consensus semi-quantitative scoring system for cranial MRI was improved to guide medication, healthcare management, and prognosis prediction in patients with WD. For every point increase in the neuroimaging score, the risk of exacerbations during treatment increased by 5.2%, and the risk of readmission to the hospital within 6 months increased by 4.3%.
摘要：
■颅骨磁共振成像（MRI）可能是评估Wilson病（WD）患者神经系统症状的重要工具。弥散加权成像（DWI）高强度反映了急性脑损伤，主要发生在特定的大脑区域。因此,这项研究旨在开发WD患者的加权头颅DWI量表，特别关注特定的大脑区域。
■总共，123例WD患者入组，其中118人入院时接受了1.5T-MRI检查。如前所述计算成像分数，并取决于以下序列：当T1，T2和流体衰减反转恢复序列中出现异常强度时，采集一个点，当发现DWI高强度时，获得了两个点。基于症状和对治疗的反应进行共识加权。
■内部评分协议良好（r=0.855[0.798-0.897]，p<0.0001)。在去铜治疗期间，壳核的DWI高强度是恶化的高风险因素（OR=8.656，p<0.05）。静脉脱铜治疗再入院的高危因素是中脑DWI高信号(OR=3.818,p<0.05)和call体(OR=2.654,p<0.05)。两种评分系统均与UWDRS量表(原始半定量评分系统，r=0.35，p<0.001；共识半定量评分系统，r=0.351,p<0.001。).与原始评分系统相比，共识评分系统与恶化的发生有更高的相关性(OR=1.052,95CI[1.003,1.0103]，p<0.05）和静脉脱铜治疗的再入院（OR=1.043,95CI[1.001,1.086]，p<0.05)。
■改善了头颅MRI共识半定量评分系统的预测性能，以指导用药，医疗保健管理，并对WD患者的预后进行预测。神经影像评分每增加一分,治疗期间恶化的风险增加了5.2%，6个月内再次入院的风险增加了4.3%。
■总共，123例WD患者入组，其中118人入院时接受了1.5T-MRI检查。如前所述计算成像分数，并取决于以下序列：当T1，T2和流体衰减反转恢复序列中出现异常强度时，采集一个点，当发现DWI高强度时，获得了两个点。基于症状和对治疗的反应进行共识加权。
■内部评分协议良好（r=0.855[0.798-0.897]，p<0.0001)。在去铜治疗期间，壳核的DWI高强度是恶化的高风险因素（OR=8.656，p<0.05）。静脉脱铜治疗再入院的高危因素是中脑DWI高信号(OR=3.818,p<0.05)和call体(OR=2.654,p<0.05)。两种评分系统均与UWDRS量表(原始半定量评分系统，r=0.35，p<0.001；共识半定量评分系统，r=0.351,p<0.001。).与原始评分系统相比，共识评分系统与恶化的发生有更高的相关性(OR=1.052,95CI[1.003,1.0103]，p<0.05）和静脉脱铜治疗的再入院（OR=1.043,95CI[1.001,1.086]，p<0.05)。
■改善了头颅MRI共识半定量评分系统的预测性能，以指导用药，医疗保健管理，并对WD患者的预后进行预测。神经影像评分每增加一分,治疗期间恶化的风险增加了5.2%，6个月内再次入院的风险增加了4.3%。
