Korean J Pediatr.  2007 Jul;50(7):694-697. 10.3345/kjp.2007.50.7.694.

A familial case report of paroxysmal kinesigenic dyskinesia in three brothers

Affiliations
  • 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. ljh3643@hanmail.net
  • 3Department of Laboratory Medicine, University of Ulsan College of medicine and Asan Medical Center, Seoul, Korea.

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(R)) or carbamazepine. Use of the video electroencephalogram can help differentiate familial PKD from seizures.

Keyword

Familial; Kinesigenic; Dyskinesia; Oxcarbamazepine (Trileptal(R)

MeSH Terms

Arachnoid
Brain
Carbamazepine
Dyskinesias*
Electroencephalography
Epilepsy
Humans
Hyperventilation
Magnetic Resonance Imaging
Physical Examination
Seizures
Siblings*
Carbamazepine
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