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【案例報告】原發性陣發性動作型運動不良症:三例案例報告
Paroxysmal Kinesigenic Dyskinesia: A Report of Three Cases
陣發性動作型運動不良症、抗癲癇藥、carbamazepine、paroxysmal kinesigenic dyskinesia, anticonvulsants
吳佩霖Pei-Lin Wu*1 、陳俊憲 Chun-Hsien Chen2
1臺南市立醫院 藥劑科 、2臺南市立醫院 神經內科
原發性陣發性動作型運動不良症(paroxysmal kinesigenic dyskinesia;PKD)是一種發 作時間短暫但頻繁的運動障礙疾病,典型的表現為病人會出現舞蹈症、肌肉張力不全等症 狀,且常會被突然改變的動作(例如:由坐著要站起來、由走路變成小跑步)、情緒所引發。 此疾病並不常見,好發於 6-16 歲的青少年,由於臨床表現與癲癇相似,過去常被混淆,或 病患及其家長也不曉得應求助於哪科而延誤診治。治療方面,雖然病程可能會隨著年齡增 長而逐漸消失,但若影響到了病患生活品質仍需積極治療,大多數病患對抗癲癇藥物反應 良好,尤其是 carbamazepine,通常在低劑量(100mg/day)下動作障礙即可獲得控制。我 們收集了三個陣發性動作型運動不良症的病例,本文將就病因、各病患的表現、藥物治療 作一討論。

Paroxysmal kinesigenic dyskinesia (PKD) is characterized by brief, frequent episodes of abnormal movements that are precipitated by sudden involuntary movement of the body, arms, and legs. The clinical presentation is not limited to chorea or athetosis, variations may present with chorea, athetosis, dystonia, or a combination of these hyperkinetic movements. PKD is an uncommon disorder that usually has its onset between 6 and 16 years. Because there was not a clear distinction between PKD and epilepsy, the patients had no idea whom to consult for their condition. PKD may remit spontaneously with age, the patients usually respond well to anticonvulsants, especially carbamazepine. We report three cases of PKD, and will discuss the pathophysiology, clinical characteristics, treatment of these patients.
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