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Korean Pediatric Society, Korean Journal of Pediatrics, 7(50), p. 694, 2007

DOI: 10.3345/kjp.2007.50.7.694

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A familial case report of paroxysmal kinesigenic dyskinesia in three brothers

Journal article published in 2007 by Oh Dae Kwon ORCID, Sung Jin Hwang, Jun Hwa Lee, Ji Eun Kim, Kyung Jib Kim, Eul Ju Seo
This paper is made freely available by the publisher.
This paper is made freely available by the publisher.

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Data provided by SHERPA/RoMEO

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.