中国现代神经疾病杂志 ›› 2023, Vol. 23 ›› Issue (2): 124-130. doi: 10.3969/j.issn.1672-6731.2023.02.009

• 癫痫及发作性疾病 • 上一篇    下一篇

2 发作性运动诱发性运动障碍神经回路研究进展

李资益1, 方侃1, 黄啸君2, 曹立1   

  1. 1 200233 上海交通大学医学院附属第六人民医院神经内科;
    2 200025 上海交通大学医学院附属瑞金医院神经内科
  • 收稿日期:2023-02-16 出版日期:2023-02-25 发布日期:2023-03-01
  • 通讯作者: 曹立,Email:caoli2000@yeah.net
  • 基金资助:
    国家自然科学基金资助项目(项目编号:81870889);“促进市级医院临床技能与临床创新三年行动计划”研究型医师创新转化能力培训项目(项目编号:SHDC2022CRD037)

Advances on neural circuits of paroxysmal kinesigenic dyskinesia

LI Zi-yi1, FANG Kan1, HUANG Xiao-jun2, CAO Li1   

  1. 1 Department of Neurology, Shanghai Sixth People's Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai 200233, China;
    2 Department of Neurology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai 200025, China
  • Received:2023-02-16 Online:2023-02-25 Published:2023-03-01
  • Supported by:
    This study was supported by the National Natural Science Foundation of China (No. 81870889), and "Three-year Action Plan for Promoting Clinical Skills and Clinical Innovation in Municipal Hospitals" Innovation and Transformation Ability Training Program for Research Physicians (No. SHDC2022CRD037).

摘要: 发作性运动诱发性运动障碍(PKD)是一类由突然动作诱发的以发作性不自主运动为特点的神经系统疾病。PKD患者基底节、丘脑和皮质等脑区之间存在结构或功能连接异常,基底节对丘脑的抑制功能减弱或丘脑自身功能障碍均可引起下游皮质的过度激活,产生不自主动作。小脑功能异常也在PKD的发病机制中发挥重要作用,小脑皮质异常可减弱其对小脑深部核团的正常抑制,同样导致丘脑-皮质通路的过度激活。本文综述PKD基底神经节-丘脑-皮质回路和小脑-丘脑-皮质回路的神经回路机制及研究进展,以提高临床医师对运动障碍性疾病发病机制的认识。

关键词: 运动障碍, 神经传导, 综述

Abstract: Paroxysmal kinesigenic dyskinesia (PKD) is a kind of nervous system disease characterized by paroxysmal involuntary movements induced by sudden movements. Neuroimaging studies have found that there are structural changes or abnormal functional connectivity in the basal ganglia, thalamus, cortex and other brain regions in PKD patients. The weakened inhibitory and regulatory function of the basal ganglia to the thalamus or the dysfunction of the thalamus itself may cause over-activation of the cortical region, resulting in involuntary movements. Recent studies have found that abnormal cerebellar function plays an important role in the pathogenesis of PKD, and abnormal cerebellar cortex can affect its normal inhibitory effect on deep cerebellar nuclei, also leading to excessive activation of thalamic-cortical pathway. This paper reviewed the mechanism and research progress of PKD in recent years, mainly focusing on the basal ganglia-thalamic-cortical circuit and the cerebellar-thalamic-cortical circuit, so as to improve clinicians' understanding of the pathogenesis of movement disorders.

Key words: Motor disorders, Neural conduction, Review