儿童阵发性运动诱发的运动障碍二例报告及文献复习

To report 2 cases of childrenwith paroxysmal kinesigenic dyskinesia and review of literature

目的报告2例阵发性运动诱发的运动障碍(paroxysmal kinesigenic dyskinesia,PKD)并复习相关文献。方法通过复习阵发性运动诱发的运动障碍的相关文献,结合报告的2例该病,总结阵发性运动诱发的运动障碍的临床特点、发病机制及目前分子生物学的研究进展。结果临床上阵发性运动诱发的运动障碍多由运动触发,发作持续时间一般<1min,发作过程中无意识障碍或疼痛,神经系统检查正常,无其他器质性疾病,拉莫三嗪、苯妥英或卡马西平等抗癫痫药物治疗有效等特点。PKD的临床表现与离子通道源性神经系统疾病存在许多相似之处,离子通道学说被认为是PKD最可能的一种发病机制。结论 PKD由运动触发,病史是明确诊断的唯一依据。临床可应用抗癫痫药物治疗,预后一般良好。

Objective： To report 2 patients with paroxysmal kinesigenic dyskinesia（PKD） and the review of the literature.Methods：Review of paroxysmal kinesigenic dyskinesia in the literature,combined with reports of 2 cases of the disease,to summarize the clinical features,pathogenesis and progress in molecular biology of PKD.Results： Clinically,paroxysmal kinesigenic dyskinesia triggered by the involuntary movement more,generally short duration of attack,generally1min,no pain or unconscious barriers during attack,neurological examination was normal,no other organic diseases,lamotrigine,phenytoin or carbamazepine and other antiepileptic drug treatment is effective and so on.The clinical manifestations of PKD are similarities with the nervous system disorders with endogenous ion channels,ion channel theory is considered the most likely kind of PKD pathogenesis.Conclusion PKD triggered by the involuntary movement.Therefore,diagnosis is based solely on history.Antiepileptic drug can be applied to clinical therapeutics,the prognosis is generally better.