Diffusion tensor imaging in Joubert syndrome
Diffusion tensor imaging in Joubert syndrome
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DOI：
10.3174/ajnr.a0703
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发表时间：
2007-11-01
影响因子：
3.5
通讯作者：
Huisman, T. A. G. M.
中科院分区：
文献类型：
--
作者：
Poretti, A.;Boltshauser, E.;Huisman, T. A. G. M.
背景和目的：Joubert综合征（JS）的神经病理学表现和初步影像学研究表明，个别患者缺乏锥体束和上级小脑脚交叉。我们假设，功能结构的神经影像学检查结果没有不同的遗传形式的JS.MATERIALS和METHODS：MR成像进行了3T MR成像单元。多平面T2和TI加权成像后进行扩散张量成像（DTI）。各向同性扩散加权图像，表观扩散系数地图，和颜色编码的分数各向异性地图，包括tractography，随后calculated.RESULTS：在所有6例研究中，DTI显示，上级小脑脚的纤维没有交叉在中脑和皮质脊髓束未能交叉在延髓尾侧。患者代表各种遗传形式的JS。结论：在JS，锥体束和上级小脑脚的纤维不交叉，无论潜在的突变。
BACKGROUND AND PURPOSE: Neuropathologic findings and preliminary imaging studies demonstrated the absence of pyramidal tract and superior cerebellar peduncular decussation in individual patients with Joubert syndrome (JS). We hypothesized that functional-structural neuroimaging findings do not differ between the genetic forms of JS.MATERIALS AND METHODS: MR imaging was performed with a 3T MR imaging-unit. Multiplanar T2-and TI-weighted imaging was followed by diffusion tensor imaging (DTI). Isotropic diffusion-weighted images, apparent diffusion coefficient maps, and color-coded fractional anisotropy maps, including tractography, were subsequently calculated.RESULTS: In all 6 patients studied, DTI showed that the fibers of the superior cerebellar peduncles did not decussate in the mesencephalon and the corticospinal tract failed to cross in the caudal medulla. The patients represented various genetic forms of JS.CONCLUSION: In JS, the fibers of the pyramidal tract and the superior cerebellar peduncles do not cross, irrespective of the underlying mutation.