陣發性動作型運動不良症

Paroxysmal Kinesigenic Dyskinesia

蔡洪瑩(Hung-Ying Tsai) ； 林應然(Yung-Zen Lin) ； 邱婷芳(Ting-Fang Chiu) ； 李坤美(Kun-Mei Lee) ； 吳幸霖(Hsin-Lin Wu) ； 黃耀霆(Yao-Ting Huang) ； 楊勝君(Sheng-Chun Yang) ； 孫中淑(Chung-Shu Sun) ； 吳文豪(Wen-Hao Wu) ； 吳康文(Kang-Wen Wu) ； 黃富源(Fu-Yuan Hua)

《北市醫學雜誌》 2卷3期 (2005/03) Pp. 300-304

https://doi.org/10.6200/TCMJ.2005.2.3.10

摘要

原發性陣發性動作型運動不良症(Paroxysmal Kinesigenic Dyskinesias)是一個好發在6至16歲之間的孩童及青少年運動性疾病，在兒科門診並不常見，和其他陣發性動作障礙疾病最不同的就是「Kinesigenic」的部分，意即「由突然而來的動作所引發」的意思，所以典型的症狀就是在患者突然的動作改變（例如：從坐著或蹲著突然要站起來）或行徑中速度變化（由慢而快、由走變跑）產生不自主的肌張力不全或舞蹈症而使原本要執行的動作中斷，持續十多秒至一分鐘，最多不超過5分鐘，在門診以臨床表現即可診斷，並不困難。此症對抗癲癇藥物治療反應很好。本文所述4個病例均有延遲診斷的情形，一方面可能因為患者家屬不知求助於什麼醫師，一方面可能是臨床醫師均沒有這方面的經驗，所以特以此文與大家分享，期望在未來可以使這群可能被延誤診斷的病患能獲得更早的診斷及更好的照顧。

關鍵字

陣發性動作型運動不良症 ； 舞蹈症 ； 徐動症 ； 肌張力不全 ； 癲通

並列摘要

Primary paroxysmal kinesigenic dyskineisa (PKD) is an uncommon childhood paroxysmal movement disorder that usually occurs between the ages of 6 and 16 years. The word ”kinesigenic” means sudden attacks of dyskinesia, that is the major difference between primary PKD and other paroxysmal movement disorders. The typical symptoms include patients' sudden movement changes (e.g., standing up from a sitting or squat position abruptly) or sudden changes during a movement from slow to fast or walking to running. As a result, the patients experience dystonia, chorea, athetosis, or ballism and stop the movements that they were doing. Often these sudden movement changes last from several seconds to one minute, with 5 minutes at the most. The diagnosis of primary PKD can easily be made based on the patients' medical history of their symptoms on an outpatient basis. The patients with this disorder usually respond well to antiepileptic agents. The four cases presented in this article all experienced a delay in diagnosis. The delay may be, in part, due to the parents' not knowing whom to turn to for their children's conditions; it may be also due to physicians' lack of experiences with diagnosing primary PKD. The purpose of this article is to share our experiences with these four cases so that these patients will be diagnosed and treated early.

並列關鍵字

paroxysmal kinesigenic dyskineisa ； chorea ； athetosis ； dystonia ； Tegretol