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KMID : 1130320070500070694
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Korean Journal of Pediatrics
2007 Volume.50 No. 7 p.694 ~ p.697
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A familial case report of paroxysmal kinesigenic dyskinesia in three brothers
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Kwon Oh-Dae
Seo Eul-Ju
Kim Kyung-Jib
Hwang Sung-Jin
Lee Jun-Wha
Kim Ji-Eun
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Abstract
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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(R)) or carbamazepine. Use of the video electroencephalogram can help differentiate familial PKD from seizures.
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KEYWORD
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Familial, Kinesigenic, Dyskinesia, Oxcarbamazepine(Trileptal)
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