Korean Journal of Pediatrics 2007;50(7):694-697.
Published online July 15, 2007.
A familial case report of paroxysmal kinesigenic dyskinesia in three brothers
Oh Dae Kwon1, Sung Jin Hwang2, Jun Hwa Lee2, Ji Eun Kim1, Kyung Jib Kim1, Eul Ju Seo3
1Department of Neurology, School of Medicine, Catholic University of Daegu, Daegu, Korea
2Department of Pediatrics, College of Medicine, Sungkyunkwan University, Masan Samsung Hospital, Masan, Korea
3Department of Laboratory Medicine, University of Ulsan College of medicine and Asan Medical Center, Seoul, Korea
한 가족 3형제에게서 발견 된 발작성 운동이상증 1례
권오대1, 황성진2, 이준화2, 김지언1, 김경집1, 서을주3
1대구가톨릭대학교 의과대학 신경과학교실
2성균관대학교 의과대학 마산삼성병원 소아과학교실
3울산대학교 의과대학 서울아산병원 진단검사의학과학교실
Correspondence: 
Jun Hwa Lee, Email: ljh3643@hanmail.net
Abstract
Paroxysmal kinesigenic dyskinesia (PKD), previously referred to as movement-provoked seizures, is a rare neurological condition that is characterized by short duration dystonic or choreoathetotic movements precipitated by sudden movement, a change in position or hyperventilation. It can be difficult to distinguish this syndrome from seizures. We reported on three brothers in one family all of whom developed abnormal involuntary dystonic or choreoathetotic movement with a tingling or stiffness sensory aura. Evaluations of the patients included general physical examinations, endoclinologic, metabolic studies, chromosomal analysis, video electroencephalograms and brain MRI imaging. All of these studies were normal except for an arachnoid cyst found in one patient. All symptoms showed excellent response to oxcarbamazepine (Trileptal ) or carbamazepine. Use of the video electroencephalogram can help differentiate familial PKD from seizures.
Key Words: Familial, Kinesigenic, Dyskinesia, Oxcarbamazepine (Trileptal )


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