[Ocular changes in MELAS syndrome].

Abstract:

:The present paper reports on the clinical findings of a 34-year-old male patient with MELAS syndrome. MELAS syndrome (mitochondrial encephalomyopathy, lactic acidosis and strokelike episodes) belongs to a group of syndromes called mitochondrial encephalomyopathies that are characterized by changes of the mitochondrial respiratory chain and the histological finding of "ragged red fibers" in muscle biopsy. In our case the diagnosis was confirmed by multiple neurologic tests including muscle biopsy and biochemical analysis of the respiratory chain. The ocular findings included reversible, homonymous hemianopic visual field loss documented six years earlier, atypical retinitis pigmentosa with marked attenuation of the scotopic ERG, myopia and nuclear cataract of the right eye. An extracapsular cataract extraction with implantation of a posterior chamber lens was performed on the rigt eye, the course was unremarkable and vision improved. In dealing with patients presenting with ocular or neurologic signs indicating mitochondrial encephalopathy, the ophthalmologist should consider MELAS syndrome or any other of the mitochondrial encephalomyopathy syndromes as a possible etiology and take the necessary steps for further medical and neurologic evaluation of the patient.

journal_name

Klin Monbl Augenheilkd

authors

Küchle M,Brenner PM,Engelhardt A,Naumann GO

doi

10.1055/s-2008-1046280

subject

Has Abstract

pub_date

1990-09-01 00:00:00

pages

258-64

issue

3

eissn

0023-2165

issn

1439-3999

journal_volume

197

pub_type

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