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Unilateral negative electroretinogram presenting as photophobia

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Abstract

Purpose

We aimed to describe four cases with an acquired unilateral negative electroretinogram (ERG) and severe unilateral photophobia and assess the underlying pathology.

Methods

We performed a retrospective chart view of the four cases by visiting two independent hospitals.

Results

Over the last 10 years, a 65-year-old man, 71-year-old woman, 68-year-old man, and 73-year-old woman presented to the hospitals with unilateral photophobia. Symptom onset was relatively obvious in all the patients. Comprehensive examinations, including visual acuity and visual field assessment, optical coherence tomography, and fluorescein angiography, showed minimal change in the eye with photophobia. However, only in the affected eye, the mixed rod–cone response in full-field ERG showed a markedly electronegative pattern, namely the amplitude of a-wave was preserved and larger than that of b-wave, and the rod and cone responses were very low. In fact, the cone responses were almost absent in all four patients. ERG findings indicate dysfunction of both rod and cone visual pathways, and the preserved a-wave in the mixed rod–cone ERG suggests that the disturbance of the rod visual pathway exists in post-photoreceptors. Moreover, although multifocal ERG showed a very low amplitude in the entire area, the preservation of the responses was detected to some extent only in the center. These symptoms and examination findings remained unchanged for more than 4 years.

Conclusions

Four patients with acquired unilateral negative ERG associated with severe photophobia showed similar clinical findings. To our knowledge, no known disorders can explain these conditions.

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Funding

Japan Society for the promotion of science (26861445) provided financial support in the form of JSPS funding. The sponsor had no role in the design or conduct of this research.

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Correspondence to Ken Ogino.

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Conflict of interest

All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials discussed in this manuscript.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.

Statement of human rights and statement on the welfare of animals

This study was adhered to the tenets of the Declaration of Helsinki. The institutional Review Board at Kyoto University did not require written informed consent from each patient in this case report.

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Cite this article

Kido, A., Ogino, K., Miyake, Y. et al. Unilateral negative electroretinogram presenting as photophobia. Doc Ophthalmol 133, 71–79 (2016). https://doi.org/10.1007/s10633-016-9544-z

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  • DOI: https://doi.org/10.1007/s10633-016-9544-z

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