J Korean Ophthalmol Soc.  1999 Mar;40(3):864-868.

A Case of Kearns-Sayre Syudrome

• Yang HC
• Choi WC

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.

Keyword

Kearms-Sayer syndrome; Mitochondrial disorder; Ragged red fiber

MeSH Terms

Biopsy
Diagnosis
Heart Block
Microscopy, Electron
Mitochondria
Mitochondrial Diseases
Mitochondrial Myopathies
Ophthalmoplegia, Chronic Progressive External
Pigmentation
Retinaldehyde
Retinaldehyde