Diffusion tensor imaging detects abnormalities in the corticospinal tracts of neonates with infantile Krabbe disease.
Diffusion tensor imaging detects abnormalities in the corticospinal tracts of neonates with infantile Krabbe disease.
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DOI：
10.3174/ajnr.a1476
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发表时间：
2009-05
期刊：
影响因子：
--
通讯作者：
Styner M
中科院分区：
文献类型：
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作者：
Escolar ML;Poe MD;Smith JK;Gilmore JH;Kurtzberg J;Lin W;Styner M
通过全州范围内的新生儿筛查项目，无法确定被诊断患有克拉伯病的婴儿是否会在婴儿、青少年或成人时期患上这种疾病。对于这种致命的神经退行性疾病，唯一可用的治疗方法是不相关的脐带移植，但这种治疗只有在临床症状出现之前才有效。因此，需要一种疾病进展的标志。本研究的目的是评估弥散张量成像（DTI）与纤维追踪在识别无症状新生儿克拉布病主要运动束早期变化中的应用。6名因家族史而确诊患有克拉布氏病的婴儿在出生后4周内接受了脑部核磁共振检查。对皮质脊髓束进行了六方向DTI和定量束造影。假设检验，每个半球一个，用来检验患有克拉布氏病的婴儿的FA比率是否与45个年龄和性别匹配的对照组有显著差异。Krabbe患者左束和右束平均FA比分别为0.89和0.87 （p= 0.002和p<0.001）。在调整胎龄、出生胎龄、出生体重、性别和种族后，6例Krabbe患者的FA值显著低于对照组（p<0.001）。DTI与定量神经束造影检测到无症状的新生儿早发克拉布病的皮质脊髓束的显著差异。一旦标准化和验证，该工具有可能被用作通过全州新生儿筛查项目诊断的婴儿疾病进展的标志。
It is not possible to determine if babies diagnosed with Krabbe disease through statewide newborn screening programs will develop disease as infants, juveniles or adults. The only available treatment for this fatal neurodegenerative condition is unrelated umbilical cord transplantation but this treatment is only effective before clinical symptoms appear. Therefore, a marker of disease progression is needed. The purpose of this study was to evaluate the use of Diffusion Tensor Imaging (DTI) with fiber tracking in identifying early changes in major motor tracts of asymptomatic neonates with infantile Krabbe disease. Six babies with infantile Krabbe disease identified because of family history had a brain MRI within the first 4 weeks of life. Six direction DTI and quantitative tractography of the corticospinal tracts were performed. Hypothesis tests, one for each hemisphere, were used to test whether the FA ratio of the babies with infantile Krabbe disease was significantly different from 45 age and gender matched controls. The average FA ratio for the Krabbe patients was 0.89 and 0.87 for left and right tracts, respectively (p= 0.002 and p<0.001). After adjusting for gestational age, gestational age at birth, birth weight, gender and race, the 6 Krabbe patients had significantly lower FA values than the controls (p<0.001). DTI with quantitative tractography detected significant differences in the corticospinal tracts of asymptomatic newborns that had the early onset form of Krabbe Disease. Once standardized and validated, this tool has the potential to be used as a marker of disease progression in babies diagnosed through statewide newborn screening programs.