All issues > Volume 52(4); 2009

Original Article

Korean J Pediatr. 2009;52(4):471-475. Published online April 15, 2009.

Visually evoked potential in children with mitochondrial respiratory chain defects

Ji Eun JE Song¹, He Min HM Kim¹, Sang-Chul SC Lee², Yoon-Gghil YG Park³, Suk-Ho SH Byeon⁴, Young-Mock YM Lee¹, Joon-Soo JS Lee¹, Heung-Dong HD Kim¹

¹Department of Pediatrics, Severance Children`s Hospital
²Department of Rehabilitation Medicine, Collegy of Medicine, Kwandong University
³Departments of Rehabilitation Medicine, Yonsei University College of Medicine
⁴Departments of Ophthalmology, Yonsei University College of Medicine

Abstract

Purpose
: Mitochondrial disorders are a clinical entity characterized by diverse symptoms and signs of involvement of various systems. Furthermore, the disorders are known to show ophthalmologic manifestations as well as neurological findings. Visually evoked potential is a sensitive measure to check the integrity of the visual pathway. In this study, we have investigated the value of visually evoked potential in mitochondrial disorders with respiratory chain defects.
Methods
: Nineteen patients diagnosed with mitochondrial respiratory chain complex I defect as confirmed by spectrophotometric enzyme assay in muscle samples were enrolled for this study. The patients underwent a visually evoked potential study. We classified the results into four groups and compared these with clinical ophthalmologic findings.
Results
: Among the 19 patients, 14 showed abnormal visually evoked potential findings. Seven patients showed abnormal clinical ophthalmologic findings. All patients with abnormal ophthalmologic findings showed abnormal visually evoked potential findings. Among the 12 patients with normal ophthalmologic findings, seven showed abnormal results in visually evoked potential.
Conclusion
: Visually evoked potential study could be used as an effective screening tool for mitochondrial disorders to detect ophthalmologic and neurological abnormalities.

Keywords :Mitochondria, Child, Eye, Evoked Potentials, Visual