Korean Journal of Pediatrics 2009;52(4):471-475.
Published online April 15, 2009.
Visually evoked potential in children with mitochondrial respiratory chain defects
Ji Eun Song1, He Min Kim1, Sang-Chul Lee2, Yoon-Gghil Park3, Suk-Ho Byeon4, Young-Mock Lee1, Joon-Soo Lee1, Heung-Dong 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
소아 사립체 질환에서 시각 유발전위검사의 유용성
송지은1, 김혜민1, 이상철2, 박윤길3, 변석호4, 이영목1, 이준수1, 김흥동1
1연세대학교 의과대학 소아과학교실
2관동대학교 의과대학 재활의학교실
3연세대학교 의과대학 재활의학교실
4연세대학교 의과대학 안과학교실
: 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.
: 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.
: 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.
: Visually evoked potential study could be used as an effective screening tool for mitochondrial disorders to detect ophthalmologic and neurological abnormalities.
Key Words: Mitochondria, Child, Eye, Evoked Potentials, Visual

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