Structural white matter abnormalities in patients with idiopathic dystonia
Structural white matter abnormalities in patients with idiopathic dystonia
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DOI：
10.1002/mds.21295
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发表时间：
2007-06-15
影响因子：
8.6
通讯作者：
Hinson, Vanessa K.
中科院分区：
文献类型：
--
作者：
Bonilha, Leonardo;de Vries, Paulien M.;Hinson, Vanessa K.
我们研究了白质结构异常，以扩散张量成像（DTI）测量的轴突一致性和完整性破坏的形式，是否构成特发性肌张力障碍（ID）的潜在病理机制，独立于基因型状态。我们研究了7例ID患者：所有患者均以颈肌张力障碍为主要症状（1例患者同时患有痉挛性发声障碍，2例患者同时患有全身性肌张力障碍，均为dyt1阴性）。我们将患者的DTI MR图像与10个对照组进行比较，评估平均扩散率（MD）和分数各向异性（FA）的差异。ID与丘脑和邻近白质以及额叶中回下白质FA值升高有关。ID还与双侧白质和壳核相邻白质、左尾状核和皮层下半球区域（包括中央后回）MD增加有关。ID患者的FA和MD异常表明异常的轴突一致性和完整性有助于肌张力障碍的病理生理。这些发现表明，ID不仅是一种功能障碍，而且与大脑结构变化有关。连通性受损和信息流中断可能导致肌张力障碍患者运动规划和调节功能受损。(c) 2007年运动障碍协会。
We investigated whether structural white matter abnormalities, in the form of disruption of axonal coherence and integrity as measured with diffusion tensor imaging (DTI), constitute an underlying pathological mechanism of idiopathic dystonia (ID), independent of genotype status. We studied seven subjects with ID: all had cervical dystonia as their main symptom (one patient also had spasmodic dysphonia and two patients had concurrent generalized dystonia, both DYT1-negative). We compared DTI MR images of patients with 10 controls, evaluating differences in mean diffusivity (MD) and fractional anisotropy (FA). ID was associated with increased FA values in the thalamus and adjacent white matter, and in the white matter underlying the middle frontal gyrus. ID was also associated with increase in MD in adjacent white matter to the pallidum and putamen bilaterally, left caudate, and in subcortical hemispheric regions, including the postcentral gyrus. Abnormal FA and MD in patients with ID indicate that abnormal axonal coherence and integrity contribute to the pathophysiology of dystonia. These findings suggest that ID is not only a functional disorder, but also associated with structural brain changes. Impaired connectivity and disrupted flow of information may contribute to the impairment of motor planning and regulation in dystonia. (c) 2007 Movement Disorder Society.