Electroretinograms in idiopathic infantile nystagmus, optic nerve hypoplasia and albinism

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Multifocal Electroretinograms
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Multifocal Electroretinograms

Published on: December 4, 2011

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Alma Kurent1, Jelka Brecelj1, Branka Stirn-Kranjc1

  • 1Eye Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia.

European Journal of Ophthalmology
|December 14, 2018
PubMed

Insights

Electroretinograms in infantile nystagmus syndrome, optic nerve hypoplasia, and albinism generally show normal waveforms with increased amplitudes compared to controls. Reduced electroretinograms were observed but lacked a specific pattern distinct from early-onset retinal dystrophies.

Area of Science:

  • Ophthalmology
  • Clinical Electrophysiology
  • Pediatric Neurology

Background:

  • Infantile nystagmus syndrome (INS) encompasses conditions like idiopathic infantile nystagmus, optic nerve hypoplasia, and albinism.
  • Electroretinograms (ERGs) are crucial for assessing retinal function in pediatric visual impairments.

Purpose of the Study:

  • To investigate and compare electroretinogram (ERG) findings in children diagnosed with infantile nystagmus syndrome (INS).
  • Specifically, to analyze ERGs in idiopathic infantile nystagmus, optic nerve hypoplasia, and albinism.
  • To compare these ERG results against age-matched healthy controls.

Main Methods:

  • A cohort of 71 children was studied, including 30 with idiopathic infantile nystagmus, 18 with optic nerve hypoplasia, and 18 with albinism.
  • Three distinct ERG protocols were employed, tailored to the child's age and utilizing either skin or HK electrodes.
  • ERG results were systematically compared with those obtained from age-matched control subjects.

Main Results:

  • Electroretinogram waveforms were found to be comparable across all studied groups (idiopathic infantile nystagmus, optic nerve hypoplasia, albinism) and controls.
  • Increased ERG amplitudes (scotopic, photopic, 30-Hz) were frequently observed in all three conditions.
  • Reduced ERG amplitudes were noted in some cases but did not follow a consistent pattern indicative of specific retinal dystrophies.

Conclusions:

  • Electroretinogram waveforms in infantile nystagmus syndrome, optic nerve hypoplasia, and albinism are typically normal.
  • Most patients exhibit increased ERG amplitudes, suggesting altered retinal function rather than absence of response.
  • The observed reduced ERGs do not present a distinct pattern characteristic of early-onset retinal dystrophies.
Abstract

Keywords:
Idiopathic infantile nystagmusalbinismchildrenelectroretinogramnystagmusoptic nerve hypoplasia

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