Li Zi-Yi, Tian Wo-Tu, Huang Xiao-Jun, Cao Li
Department of Neurology, Shanghai Sixth People's Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Department of Neurology and Institute of Neurology, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Mov Disord. 2023 Apr;38(4):537-544. doi: 10.1002/mds.29326. Epub 2023 Jan 31.
Paroxysmal kinesigenic dyskinesia (PKD) is a movement disorder characterized by recurrent and transient episodes of involuntary movements, including dystonia, chorea, ballism, or a combination of these, which are typically triggered by sudden voluntary movement. Disturbance of the basal ganglia-thalamo-cortical circuit has long been considered the cause of involuntary movements. Impairment of the gating function of the basal ganglia can cause an aberrant output toward the thalamus, which in turn leads to excessive activation of the cerebral cortex. Structural and functional abnormalities in the basal ganglia, thalamus, and cortex and abnormal connections between these brain regions have been found in patients with PKD. Recent studies have highlighted the role of the cerebellum in PKD. Insufficient suppression from the cerebellar cortex to the deep cerebellar nuclei could lead to overexcitation of the thalamocortical pathway. Therefore, this literature review aims to provide a comprehensive overview of the current research progress to explore the neural circuits and pathogenesis of PKD and promote further understanding and outlook on the pathophysiological mechanism of movement disorders. © 2023 International Parkinson and Movement Disorder Society.
发作性运动诱发性运动障碍(PKD)是一种运动障碍,其特征为反复出现的短暂性不自主运动发作,包括肌张力障碍、舞蹈症、投掷症或这些症状的组合,通常由突然的自主运动触发。长期以来,基底神经节-丘脑-皮质回路紊乱一直被认为是不自主运动的原因。基底神经节门控功能受损可导致向丘脑的异常输出,进而导致大脑皮层过度激活。在PKD患者中发现了基底神经节、丘脑和皮层的结构和功能异常以及这些脑区之间的异常连接。最近的研究强调了小脑在PKD中的作用。小脑皮层对小脑深部核团的抑制不足可能导致丘脑皮质通路过度兴奋。因此,本文献综述旨在全面概述当前的研究进展,以探索PKD的神经回路和发病机制,并促进对运动障碍病理生理机制的进一步理解和展望。© 2023国际帕金森病和运动障碍协会。