Isolated focal dystonia phenotypes are associated with distinct patterns of altered microstructure.
Isolated focal dystonia phenotypes are associated with distinct patterns of altered microstructure.
复制标题
DOI：
10.1016/j.nicl.2018.06.004
复制
发表时间：
2018
期刊：
影响因子：
--
通讯作者：
Nagae LM
中科院分区：
文献类型：
--
作者：
Berman BD;Honce JM;Shelton E;Sillau SH;Nagae LM
孤立性成人发作的局灶性肌张力障碍被认为是一种网络障碍，运动基底神经节和小脑回路的紊乱起着病理生理学作用，但为什么特定的身体区域受到影响仍不清楚。我们的目的是使用扩散张量成像来确定局灶性肌张力障碍的两种最常见的表型是否与影响运动网络的微结构变化有关。招募了15名眼睑痉挛患者，20名颈部肌张力障碍患者和30名年龄和性别匹配的健康对照。分数各向异性和平均扩散率的地图进行了分析，使用基于体素的方法和自动区域感兴趣的技术，以评估深灰质核。扩散措施和肌张力障碍的严重程度之间的相关性进行了测试，并进行事后判别分析。基于体素的分析显示，与对照组相比，颈部肌张力障碍患者右侧小脑的各向异性分数显著降低，左侧尾状核的平均扩散率增加，颈部肌张力障碍患者右侧小脑的各向异性分数相对于眼睑痉挛患者降低。除了相对于对照组和眼睑痉挛患者，颈肌张力障碍患者双侧尾状核的各向异性分数降低外，感兴趣区域分析显示，与对照组和颈肌张力障碍患者相比，眼睑痉挛患者右侧苍白球内侧和左侧红核的各向异性分数显著降低。眼睑痉挛患者红核的弥散度测量与疾病严重程度相关。在三组判别分析中，参与者仅以中等可靠性（67-75%）被正确分类，但在两组判别分析中，患者可以以高可靠性（83-100%）彼此区分。不同的局灶性肌张力障碍表型与基底神经节和小脑回路组成区域内不同的微结构改变模式相关。眼睑痉挛时苍白球和红核的显微结构改变。颈部肌张力障碍时尾状核和小脑的微结构改变。眼睑痉挛的疾病严重程度与红核扩散率相关。扩散性差异可以区分眼睑痉挛和颈部肌张力障碍。基底神经节和小脑网络破坏可能在肌张力障碍表型上不同。
Isolated adult-onset focal dystonia is considered a network disorder with disturbances to the motor basal ganglia and cerebellar circuits playing a pathophysiological role, but why specific body regions become affected remains unknown. We aimed to use diffusion tensor imaging to determine if the two most common phenotypes of focal dystonia are associated with distinguishing microstructural changes affecting the motor network. Fifteen blepharospasm patients, 20 cervical dystonia patients, and 30 age- and sex-matched healthy controls were recruited. Maps of fractional anisotropy and mean diffusivity were analyzed using a voxel-based approach and an automated region-of-interest technique to evaluate deep gray matter nuclei. Correlations between diffusion measures and dystonia severity were tested, and post hoc discriminant analyses were conducted. Voxel-based analyses revealed significantly reduced fractional anisotropy in the right cerebellum and increased mean diffusivity in the left caudate of cervical dystonia patients compared to controls, as well as lower fractional anisotropy in the right cerebellum in cervical dystonia patients relative to blepharospasm patients. In addition to reduced fractional anisotropy in the bilateral caudate nucleus of cervical dystonia patients relative to controls and blepharospasm patients, region-of-interest analyses revealed significantly reduced fractional anisotropy in the right globus pallidus internus and left red nucleus of blepharospasm patients compared to both controls and cervical dystonia patients. Diffusivity measures in the red nucleus of blepharospasm patients correlated with disease severity. In a three-group discriminant analysis, participants were correctly classified with only modest reliability (67–75%), but in a two-group discriminant analysis, patients could be distinguished from each other with high reliability (83–100%). Different focal dystonia phenotypes are associated with distinct patterns of altered microstructure within constituent regions of basal ganglia and cerebellar circuits. Globus pallidus and red nucleus microstructure is altered in blepharospasm. Caudate and cerebellar microstructure is altered in cervical dystonia. Disease severity in blepharospasm correlates with diffusivity in the red nucleus. Diffusivity differences can differentiate blepharospasm from cervical dystonia. Basal ganglia and cerebellar network disruptions may differ in dystonia phenotypes.
登录
查看更多内容
影响因子：
8.6
作者：
Jinnah, H. A.;Berardelli, Alfredo;Comella, Cynthia;DeFazio, Giovanni;DeLong, Mahlon R.;Factor, Stewart;Galpern, Wendy R.;Hallett, Mark;Ludlow, Christy L.;Perlmutter, Joel S.;Rosen, Ami R.
通讯作者：
Rosen, Ami R.
肌张力障碍是一种网络障碍：小脑的作用是什么？
影响因子：
3.3
作者：
Prudente, C. N.;Hess, E. J.;Jinnah, H. A.
通讯作者：
Jinnah, H. A.
DOI：
10.1136/jnnp-2017-316250
发表时间：
2018-05
期刊：
Journal of neurology, neurosurgery, and psychiatry
影响因子：
--
作者：
Kaji R;Bhatia K;Graybiel AM
通讯作者：
Graybiel AM
影响因子：
1.9
作者：
KLEMM, WR;BRATTON, GR;DZIEZYC, J
通讯作者：
DZIEZYC, J
影响因子：
8.6
作者：
Bonilha, Leonardo;de Vries, Paulien M.;Hinson, Vanessa K.
通讯作者：
Hinson, Vanessa K.