Early diagnosis of Stargardt disease with multifocal electroretinogram in children

Publication Type: Journal Article
Publication: International ophthalmology, Volume 34 (2013)

To present two pediatric cases where

multifocal electroretinogram (mfERG) was able to

establish an earlier diagnosis compared to full field

electroretinogram (ERG) Case 1: an 11-year-old boy

with reduced visual acuity, pale discs, macular

pigmentation with white dots bilaterally. Case 2: a

12-year-old girl with reduced vision in her right eye,

slight pallor of the right optic disc, intense pigmentation

at both maculae and scattered punctate lesions

throughout the peripheral fundi. Both had been

investigated with electrodiagnostic tests according to

the International Society of Clinical Electrophysiology

for Vision protocol. Full-field ERGs for both

children showed normal responses. Case 1: mfERG

revealed a severe reduction in function in the inner

20. Case 2: mfERG showed attenuated responses in

each eye. Clinical examination and mfERG were

consistent with Stargardt disease. mfERG is applicable

to children and is a sensitive tool for early


diagnosis of retinal dystrophies.