Electrophysiologic alterations in patients with optic nerve hypoplasia

Márta Janáky, Andrea Deák, Zsuzsanna Pelle, G. Benedek

Research output: Contribution to journalArticle

10 Citations (Scopus)

Abstract

The clinical and electrophysiologic data (electroretinograms and visual evoked potentials) were studied in 45 patients with optic nerve hypoplasia. The patients were divided into three fairly distinct groups on the basis of their electrophysiologic alterations. Group 1 consisted of 13 patients with almost extinguished visual evoked potentials and with mild electroretinographic alterations. These were the cases that are traditionally recognized as optic nerve hypoplasia. The serious visual impairment in these cases was accompanied by various developmental ophthalmologic and nonophthalmologic abnormalities. Group 2 included 26 patients without any significant visual evoked potential or electroretinographic alterations, but with overt funduscopic signs of optic nerve hypoplasia. These patients were consistently suffering from strabismus and/or amblyopia. The visual functions based on visual evoked potential and electroretinographic recordings could be fairly normal apart from a pathologic ophthalmoscopic picture characteristic of optic nerve hypoplasia. Group 3 included six patients with abnormal albeit well-recordable visual evoked potentials and subnormal or negative-type electroretinograms that suggested an accompanying retinal disease. This finding seems to prove that a subset of patients with optic nerve hypoplasia with nystagmus may have a primary retinal abnormality. Our study provides further evidence that optic nerve hypoplasia is not a uniform disease entity.

Original languageEnglish
Pages (from-to)247-257
Number of pages11
JournalDocumenta Ophthalmologica
Volume86
Issue number3
DOIs
Publication statusPublished - Mar 1994

Fingerprint

Optic Nerve
Visual Evoked Potentials
Retinal Diseases
Amblyopia
Strabismus
Vision Disorders

Keywords

  • Electroretinogram
  • Optic nerve hypoplasia
  • Visual evoked response

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Electrophysiologic alterations in patients with optic nerve hypoplasia. / Janáky, Márta; Deák, Andrea; Pelle, Zsuzsanna; Benedek, G.

In: Documenta Ophthalmologica, Vol. 86, No. 3, 03.1994, p. 247-257.

Research output: Contribution to journalArticle

Janáky, Márta ; Deák, Andrea ; Pelle, Zsuzsanna ; Benedek, G. / Electrophysiologic alterations in patients with optic nerve hypoplasia. In: Documenta Ophthalmologica. 1994 ; Vol. 86, No. 3. pp. 247-257.
@article{f4ceabc186d142a682e41c1063980f4c,
title = "Electrophysiologic alterations in patients with optic nerve hypoplasia",
abstract = "The clinical and electrophysiologic data (electroretinograms and visual evoked potentials) were studied in 45 patients with optic nerve hypoplasia. The patients were divided into three fairly distinct groups on the basis of their electrophysiologic alterations. Group 1 consisted of 13 patients with almost extinguished visual evoked potentials and with mild electroretinographic alterations. These were the cases that are traditionally recognized as optic nerve hypoplasia. The serious visual impairment in these cases was accompanied by various developmental ophthalmologic and nonophthalmologic abnormalities. Group 2 included 26 patients without any significant visual evoked potential or electroretinographic alterations, but with overt funduscopic signs of optic nerve hypoplasia. These patients were consistently suffering from strabismus and/or amblyopia. The visual functions based on visual evoked potential and electroretinographic recordings could be fairly normal apart from a pathologic ophthalmoscopic picture characteristic of optic nerve hypoplasia. Group 3 included six patients with abnormal albeit well-recordable visual evoked potentials and subnormal or negative-type electroretinograms that suggested an accompanying retinal disease. This finding seems to prove that a subset of patients with optic nerve hypoplasia with nystagmus may have a primary retinal abnormality. Our study provides further evidence that optic nerve hypoplasia is not a uniform disease entity.",
keywords = "Electroretinogram, Optic nerve hypoplasia, Visual evoked response",
author = "M{\'a}rta Jan{\'a}ky and Andrea De{\'a}k and Zsuzsanna Pelle and G. Benedek",
year = "1994",
month = "3",
doi = "10.1007/BF01203548",
language = "English",
volume = "86",
pages = "247--257",
journal = "Documenta Ophthalmologica",
issn = "0012-4486",
publisher = "Springer Netherlands",
number = "3",

}

TY - JOUR

T1 - Electrophysiologic alterations in patients with optic nerve hypoplasia

AU - Janáky, Márta

AU - Deák, Andrea

AU - Pelle, Zsuzsanna

AU - Benedek, G.

PY - 1994/3

Y1 - 1994/3

N2 - The clinical and electrophysiologic data (electroretinograms and visual evoked potentials) were studied in 45 patients with optic nerve hypoplasia. The patients were divided into three fairly distinct groups on the basis of their electrophysiologic alterations. Group 1 consisted of 13 patients with almost extinguished visual evoked potentials and with mild electroretinographic alterations. These were the cases that are traditionally recognized as optic nerve hypoplasia. The serious visual impairment in these cases was accompanied by various developmental ophthalmologic and nonophthalmologic abnormalities. Group 2 included 26 patients without any significant visual evoked potential or electroretinographic alterations, but with overt funduscopic signs of optic nerve hypoplasia. These patients were consistently suffering from strabismus and/or amblyopia. The visual functions based on visual evoked potential and electroretinographic recordings could be fairly normal apart from a pathologic ophthalmoscopic picture characteristic of optic nerve hypoplasia. Group 3 included six patients with abnormal albeit well-recordable visual evoked potentials and subnormal or negative-type electroretinograms that suggested an accompanying retinal disease. This finding seems to prove that a subset of patients with optic nerve hypoplasia with nystagmus may have a primary retinal abnormality. Our study provides further evidence that optic nerve hypoplasia is not a uniform disease entity.

AB - The clinical and electrophysiologic data (electroretinograms and visual evoked potentials) were studied in 45 patients with optic nerve hypoplasia. The patients were divided into three fairly distinct groups on the basis of their electrophysiologic alterations. Group 1 consisted of 13 patients with almost extinguished visual evoked potentials and with mild electroretinographic alterations. These were the cases that are traditionally recognized as optic nerve hypoplasia. The serious visual impairment in these cases was accompanied by various developmental ophthalmologic and nonophthalmologic abnormalities. Group 2 included 26 patients without any significant visual evoked potential or electroretinographic alterations, but with overt funduscopic signs of optic nerve hypoplasia. These patients were consistently suffering from strabismus and/or amblyopia. The visual functions based on visual evoked potential and electroretinographic recordings could be fairly normal apart from a pathologic ophthalmoscopic picture characteristic of optic nerve hypoplasia. Group 3 included six patients with abnormal albeit well-recordable visual evoked potentials and subnormal or negative-type electroretinograms that suggested an accompanying retinal disease. This finding seems to prove that a subset of patients with optic nerve hypoplasia with nystagmus may have a primary retinal abnormality. Our study provides further evidence that optic nerve hypoplasia is not a uniform disease entity.

KW - Electroretinogram

KW - Optic nerve hypoplasia

KW - Visual evoked response

UR - http://www.scopus.com/inward/record.url?scp=0028142250&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0028142250&partnerID=8YFLogxK

U2 - 10.1007/BF01203548

DO - 10.1007/BF01203548

M3 - Article

VL - 86

SP - 247

EP - 257

JO - Documenta Ophthalmologica

JF - Documenta Ophthalmologica

SN - 0012-4486

IS - 3

ER -