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Early diagnosis of Stargardt disease with multifocal electroretinogram in children

International Ophthalmology volume 34, pages 613–621 (2014)Cite this article

Abstract

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.

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Abbreviations

EDTs:

Electrodiagnostic tests

EOG:

Electrooculogram

ERG:

Electroretinogram

FVEPs:

Flash visual evoked potentials

FAF:

Fundus autofluorescence

FFA:

Fundus fluorescein angiography

ISCEV:

International Society of Clinical Electrophysiology of Vision

mfERG:

Multifocal electroretinogram

PERGs:

Pattern electroretinograms

PRVEP:

Pattern-reversal visual evoked potentials

RPE:

Retinal pigment epithelium

SNR:

Signal-to-noise ratio

STGD:

Stargardt disease

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Conflict of interest

No authors have any financial/proprietary interests to disclose.

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Affiliations

  1. Department of Paediatric Ophthalmology, Alder Hey Children’s NHS Foundation Trust, Eaton Road, Liverpool, L12 2AP, UK

    Anna Praidou, Richard Hagan, William Newman & Arvind Chandna

Authors
  1. Anna Praidou
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  2. Richard Hagan
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  3. William Newman
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  4. Arvind Chandna
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Correspondence to Anna Praidou.

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Praidou, A., Hagan, R., Newman, W. et al. Early diagnosis of Stargardt disease with multifocal electroretinogram in children. Int Ophthalmol 34, 613–621 (2014). https://doi.org/10.1007/s10792-013-9812-9

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  • DOI: https://doi.org/10.1007/s10792-013-9812-9

Keywords

  • Multifocal electroretinogram
  • Macular dystrophy
  • Stargardt disease
  • Pediatric