A Case of Kearns-Sayre Syudrome.

Yang, Hee Chang; Choi, Woong Chul

Case Report

Journal of the Korean Ophthalmological Society 1999;40(3):864-868.
Published online March 1, 1999.

A Case of Kearns-Sayre Syudrome.

Hee Chang Yang, Woong Chul Choi

Department of Ophthalmology, St. Marys Hospital, College of Medicine, The Catholic University.

Kearns-Sayre 증후군 1예

양희창(Hee Chang Yang),최웅철(Woong Chul Choi)

Abstract

Kearns-Sayer syndrome, a rare mitochondrial disorder, is composed of chronic progressive external ophthalmoplegia, atypical retinal pigmentation and complete heart block, and also causes numerous neurologic or endocrinologic symptoms. On muscle biopsy, a "ragged red fiber" was seen with Gomori trichrome stain, On electron microscopy, aggregations of abnormal mitochondria were demonstrated, confirming the diagnosis of mitochondrial myopathy. We report a case of Kearns-Sayer syndrome we have experienced.

Key Words: Kearms-Sayer syndrome;Mitochondrial disorder;Ragged red fiber