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首页> 外文期刊>AJR: American Journal of Roentgenology : Including Diagnostic Radiology, Radiation Oncology, Nuclear Medicine, Ultrasonography and Related Basic Sciences >Correlation of neurodevelopmental features and MRI findings in infantile Krabbe's disease.
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Correlation of neurodevelopmental features and MRI findings in infantile Krabbe's disease.

机译：婴儿Krabbe病的神经发育特征与MRI表现的相关性。

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OBJECTIVE: The purpose of our study was to compare MRI findings with neurobehavioral development in infants with Krabbe's disease. MATERIALS AND METHODS: Nine infants with Krabbe's disease underwent a total of 19 MR studies during the first year of life as well as tests of mental development, gross motor skills, and fine motor skills (score range: 0-100) within 1 month of imaging. MR scans were scored using the Loes severity scale based on signal abnormality and atrophy, ranging from 0 (best) to 32. We performed three comparisons (Student's t test): each test versus total brain Loes score, fine motor and gross motor tests versus Loes score for the pyramidal tract, and fine motor and gross motor tests versus Loes score for the internal capsule. RESULTS: Mean test results were 65+/-31 for mental development, 48+/-39 for gross motor score, 57+/-35 for fine motor score, and mean total brain score was 7.79+/-6.20. Correlations for total Loes score were -0.78 (p=0.003) for mental development, -0.74 (p=0.003) for gross motor function, and -0.80 (p<0.001) for fine motor function. Correlations for pyramidal system Loes scores were -0.73 (p=0.003) for fine motor function and -0.58 (p=0.028) for gross motor function. Correlation between Loes scores for internal capsule and fine motor function was -0.38 (p>0.05) and between Loes scores for internal capsule and gross motor function was -0.35 (p>0.05). CONCLUSION: The very good correlation between testing results and Loes scores for the entire brain and moderately good correlation between test results and scores for specific brain regions indicate the Loes scoring system likely provides a reasonable means for assessing prognosis and therapeutic response for infants with Krabbe's disease.
机译：目的：我们的研究目的是比较克拉博病患儿的MRI表现与神经行为发展。材料和方法：9例患有Krabbe病的婴儿在出生后的第一年共接受了19项MR研究，并在1个月内进行了智力发育，粗略运动技能和精细运动技能（得分范围：0-100）的测试。成像。根据信号异常和萎缩程度，使用Loes严重性量表对MR扫描进行评分，范围从0（最佳）到32。我们进行了三项比较（Student's t检验）：每项测试与总脑Loes得分，精细运动和总体运动测试与锥体束的Loes得分，精细运动和大运动测试对内囊的Loes得分。结果：智力发育平均测试结果为65 +/- 31，整体运动评分为48 +/- 39，精细运动评分为57 +/- 35，平均总脑得分为7.79 +/- 6.20。对于精神发育而言，总Loes得分的相关性为-0.78（p = 0.003），对于总体运动功能而言，其相关性为-0.74（p = 0.003），对于精细运动功能而言，其相关性为-0.80（p <0.001）。锥体系统的Loes分数对于精细运动功能的相关性为-0.73（p = 0.003），对于总体运动功能的相关性为-0.58（p = 0.028）。内囊的Loes得分与精细运动功能的相关性为-0.38（p> 0.05），内囊的Loes得分与总体运动功能的相关性为-0.35（p> 0.05）。结论：整个大脑的测试结果与Loes得分之间的良好相关性，以及特定大脑区域的测试结果与得分之间的中度良好的相关性表明，Loes评分系统可能为评估Krabbe病婴儿的预后和治疗反应提供合理的手段。

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