Department of Neurology, Tokushima University School of Medicine, Tokushima, Japan.
Sobell Department of Movement Neuroscience, UCL Institute of Neurology, London, UK.
J Neurol Neurosurg Psychiatry. 2018 May;89(5):488-492. doi: 10.1136/jnnp-2017-316250. Epub 2017 Oct 31.
Dystonia is a disorder of motor programmes controlling semiautomatic movements or postures, with clinical features such as , which suggests sensorimotor mismatch as the basis. Dystonia was originally classified as a basal ganglia disease. It is now regarded as a ' 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 synaptic 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.
肌张力障碍是一种运动程序障碍，控制半自动运动或姿势，具有临床特征，如 ，这表明感觉运动不匹配是其基础。肌张力障碍最初被归类为基底节疾病。现在它被认为是一种“包括小脑在内的障碍”，但确切的发病机制尚不清楚。罕见的尸检研究发现纹状体和小脑都有病理学改变，患者的体感皮层也存在功能失调。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突触连接。我们根据小脑与基底节之间新的功能相互作用来回顾临床证据，并提出一个假设，即肌张力障碍是一种基底节障碍，可以由小脑异常传入输入引起。
