发作性运动诱发性运动障碍神经回路研究进展

Advances on neural circuits of paroxysmal kinesigenic dyskinesia

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。

Paroxysmal kinesigenic dyskinesia(PKD) is a kind of nervous system diseasecharacterized by paroxysmal involuntary movements induced by sudden movements. Neuroimaging studieshave 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 functionof the basal ganglia to the thalamus or the dysfunction of the thalamus itself may cause over-activation ofthe cortical region, resulting in involuntary movements. Recent studies have found that abnormal cerebellarfunction plays an important role in the pathogenesis of PKD, and abnormal cerebellar cortex can affect itsnormal inhibitory effect on deep cerebellar nuclei, also leading to excessive activation of thalamic-corticalpathway. This paper reviewed the mechanism and research progress of PKD in recent years, mainlyfocusing on the basal ganglia-thalamic-cortical circuit and the cerebellar-thalamic-cortical circuit, so as toimprove clinicians’ understanding of the pathogenesis of movement disorders.