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Electroretinography in congenital nystagmus patients with a normal fundus examination

  • Clinical Investigation
  • Published:
Japanese Journal of Ophthalmology Aims and scope Submit manuscript

Abstract

Purpose

To identify the ophthalmic causes of congenital nystagmus with normal eye examination by electroretinography (ERG).

Study design

Retrospective observational study.

Methods

We reviewed the medical records of patients younger than 6 months of age who presented between June 2008 and November 2011 with nystagmus and no other neurological signs following an otherwise normal eye examination. A complete ophthalmic examination and ERG (Nicolet Bravo system; Nicolet Biomedial & RETIscan; Roland Instruments), fundus photography, and Ishihara color test were performed to identify any ophthalmic causes of congenital nystagmus.

Results

Thirty-three patients met the criteria. Rod dysfunction was diagnosed in 4 patients (12.1%), cone dysfunction in 2 patients (6.1%), and cone-rod dysfunction in 1 patient (3.0%). The results of ERG were negative in 2 patients (6.1%). Idiopathic infantile nystagmus was diagnosed in the remaining 24 patients (72.7%) based on their normal ERG examination.

Conclusions

In Korean congenital nystagmus patients with a normal fundus examination, achromatopsia and Leber’s congenital amaurosis are uncommon causes. ERG is needed to make a definite diagnosis and provide prognostic information in congenital idiopathic nystagmus patients with a normal fundus examination.

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Author information

Authors and Affiliations

  1. Department of Ophthalmology, Dongtan Sacred Heart Hospital, Hallym University College of Medicine, Hwaseong, Republic of Korea

    Soolienah Rhiu

  2. Department of Ophthalmology, Soonchunhyang University College of Medicine, Bucheonsi, Gyeonggido, Republic of Korea

    Jee Ho Chang & Tae Kwan Park

  3. Department of Ophthalmology, Kangwon National University Hospital, Kangwon National University School of Medicine, Chuncheon, Republic of Korea

    Yong Woo Lee

  4. Kong Eye Clinic, Seoul, Republic of Korea

    Jong Bok Lee

  5. Department of Radiology, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea

    Inyoung Youn

  6. Department of Ophthalmology, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, 29 Saemunan-ro, Jongno-gu, Seoul, 03181, Republic of Korea

    So Young Han

Authors
  1. Soolienah Rhiu
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  2. Jee Ho Chang
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  3. Tae Kwan Park
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  4. Yong Woo Lee
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  5. Jong Bok Lee
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  6. Inyoung Youn
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  7. So Young Han
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Corresponding author

Correspondence to So Young Han.

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Conflicts of interest

S. Rhiu, None; J. H. Chang, None; T. K. Park, None; Y. W. Lee, None; J. B. Lee, None; I. Youn, None; S. Y. Han, None.

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Corresponding Author: So Young Han

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Rhiu, S., Chang, J.H., Park, T.K. et al. Electroretinography in congenital nystagmus patients with a normal fundus examination. Jpn J Ophthalmol 68, 243–249 (2024). https://doi.org/10.1007/s10384-024-01058-2

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  • DOI: https://doi.org/10.1007/s10384-024-01058-2

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