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Paroxysmal kinesigenic dyskinesia (PKD) is a rare movement disorder of the nervous system, and little is known about its pathogenesis. Currently, the diagnosis of PKD is primarily based on clinical manifestations, with little objective evidence. Neuroimaging has been used to explore the pathological changes in cerebral structure and function associated with PKD. The current review highlights recent advances in neuroimaging to provide a better understanding of the neural mechanisms and early diagnosis of this disorder. Several studies uti