Pathogenesis of dystonia: is it of cerebellar or basal ganglia origin?

Pathogenesis of dystonia: is it of cerebellar or basal ganglia origin?
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DOI:
10.1136/jnnp-2017-316250
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发表时间:
2018-05
期刊:
Journal of neurology, neurosurgery, and psychiatry
影响因子:
--
通讯作者:
Graybiel AM
Graybiel AM
中科院分区:
其他
文献类型:
--
作者:
Kaji R;Bhatia K;Graybiel AM

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肌张力障碍是一种控制半自动运动或姿势的运动程序紊乱,具有感觉特技等临床特征,提示感觉运动不匹配是基础。肌张力障碍最初被归类为基底节疾病。它现在被认为是一种包括小脑在内的‘网络’障碍,但确切的发病机制尚不清楚。罕见的尸检研究在纹状体和小脑都发现了病理,据报道,患者的躯体感觉皮质出现了功能紊乱。最近的动物研究表明,小脑和纹状体之间存在生理上紧密的双突触联系。根据小脑和基底节之间的这种新的功能相互作用,我们回顾了临床证据,并提出了一个假说,即肌张力障碍是一种基底节疾病,可由小脑的异常传入输入引起。
Dystonia is a disorder of motor programmes controlling semiautomatic movements or postures, with clinical features such as sensory trick, which suggests sensorimotor mismatch as the basis. Dystonia was originally classified as a basal ganglia disease. It is now regarded as a ‘network’ disorder including the cerebellum, but the exact pathogenesis being unknown. Rare autopsy studies have found pathology both in the striatum and the cerebellum, and functional disorganisation was reported in the somatosensory cortex in patients. Recent animal studies showed physiologically tight disynaptic connections between the cerebellum and the striatum. We review clinical evidence in light of this new functional interaction between the cerebellum and basal ganglia, and put forward a hypothesis that dystonia is a basal ganglia disorder that can be induced by aberrant afferent inputs from the cerebellum.
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