Razek Ahmed Abdel Khalek Abdel, Taman Saher Ebrahiem, El Regal Mohamed Ezz, Megahed Ahmed, Elzeny Sherine, El Tantawi Noha
From the Department of Diagnostic Radiology.
Gastroenterology and Hepatology Unit of Mansoura Children Hospital.
J Comput Assist Tomogr. 2020 May/Jun;44(3):393-398. doi: 10.1097/RCT.0000000000001008.
This study aimed to evaluate the role of diffusion tensor imaging of microstructural changes in gray and white matter in Crigler-Najjar syndrome type I.
A prospective study was conducted on 10 patients with Crigler-Najjar syndrome type I and 10 age- and sex-matched children who underwent diffusion tensor imaging of the brain. Mean diffusivity (MD) and fractional anisotropy (FA) of gray and white matter were measured.
There was a significantly higher MD of the gray matter regions including the globus pallidus, thalamus, caudate head, substantia nigra, and dentate nucleus in patients versus controls (P = 0.007, 0.001, 0.014, 0.003, and 0.002), respectively. The areas under the curve (AUC) of MD of the globus pallidus and thalamus used to differentiate patients from controls were 0.93 and 0.925, respectively. There was a significant difference in MD of the frontal white matter and posterior limb of the internal capsule in patients versus controls (P = 0.001 and 0.02), respectively. The AUCs of MD of these regions used to differentiate patients from controls were 0.82 and 0.8. There was a significant difference in FA of the frontal white matter and posterior limb of the internal capsule in patients versus controls (P = 0.006 and 0.006), respectively. The AUCs of FA of these regions were 0.83 and 0.85, respectively. The MD of the globus pallidus correlated with serum bilirubin (r = 0.87 and P = 0.001).
Diffusion tensor imaging can detect microstructural changes of deep gray matter and some regions of white matter in Crigler-Najjar syndrome type I.
本研究旨在评估弥散张量成像在I型克里格勒 - 纳贾尔综合征中对灰质和白质微观结构变化的作用。
对10例I型克里格勒 - 纳贾尔综合征患者以及10例年龄和性别匹配的儿童进行了前瞻性研究，这些儿童均接受了脑部弥散张量成像。测量了灰质和白质的平均扩散率（MD）和各向异性分数（FA）。
与对照组相比，患者苍白球、丘脑、尾状核头部、黑质和齿状核等灰质区域的MD显著更高（P分别为0.007、0.001、0.014、0.003和0.002）。用于区分患者与对照组的苍白球和丘脑MD的曲线下面积（AUC）分别为0.93和0.925。与对照组相比，患者额叶白质和内囊后肢的MD存在显著差异（P分别为0.001和0.02）。这些区域用于区分患者与对照组的MD的AUC分别为0.82和0.8。与对照组相比，患者额叶白质和内囊后肢的FA存在显著差异（P分别为0.006和0.006）。这些区域FA的AUC分别为0.83和0.85。苍白球的MD与血清胆红素相关（r = 0.87，P = 0.001）。
弥散张量成像可检测I型克里格勒 - 纳贾尔综合征中深部灰质和部分白质区域的微观结构变化。
