Kearns-Sayre syndrome: a case report and review.

Abstract:

:In 1958, Kearns and Sayre described a multisystem entity, now known as Kearns-Sayre syndrome (KSS). The syndrome is defined as exhibiting a triad of thus far unexplained degenerative conditions: progressive external ophthalmoplegia, retinal pigmentary degeneration, and heart block. Commonly accompanying findings include cerebellar dysfunction and CSF protein levels above 100 mg/dl. Symptoms usually appear in early childhood, but the onset has been seen occasionally in young adults. KSS is a mitochondrial disorder that occurs rarely; the actual incidence is unknown. Ocular findings consist of bilateral ptosis, chronic progressive external ophthalmoplegia, and pigmentary retinopathy. Corneal clouding and optic neuritis are infrequent. We herein report a classic case of Kearns Sayre syndrome and discuss the findings.

journal_name

Eur J Ophthalmol

authors

Gross-Jendroska M,Schatz H,McDonald HR,Johnson RN

subject

Has Abstract

pub_date

1992-01-01 00:00:00

pages

15-20

issue

1

eissn

1120-6721

issn

1724-6016

journal_volume

2

pub_type

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