Japanese Journal of Ophthalmology

, Volume 63, Issue 2, pp 172–180 | Cite as

Three cases of acute-onset bilateral photophobia

  • Shinji UenoEmail author
  • Daiki Inooka
  • Monika Meinert
  • Yasuki Ito
  • Kazushige Tsunoda
  • Kaoru Fujinami
  • Takeshi Iwata
  • Hisao Ohde
  • Hiroko Terasaki
Clinical Investigation



To report the findings in 3 cases of bilateral negative electroretinograms (ERGs) with acute onset of photophobia.

Study design

Retrospective case series.


The medical charts of the 3 patients were reviewed.


A 43-year-old woman, a 68-year-old woman, and a 41-year-old woman were referred to Nagoya University Hospital. Their main symptom was bilateral acute photophobia. None of the patients had any systemic diseases or specific medical history. The decimal best-corrected visual acuity (> 0.8) and Humphrey visual fields (mean deviation > -3 dB) were relatively well preserved in all 3 patients. The optical coherence tomography (OCT) and fundus autofluorescence findings were essentially normal. Fluorescein angiography showed mild leakage in 1 patient but no abnormality in the other 2 patients. However, the ERGs of the 3 patients had the features of abnormal ERGs found in patients with incomplete congenital stationary night blindness (CSNB). Exome analyses found no pathogenic variants related to known CSNB-related genes. The symptoms and ERGs of the 3 patients have not progressed or recovered after a relatively long follow-up period.


The ERG characteristics of 3 patients with bilateral photophobia were similar to those of incomplete CSNB, suggesting post-phototransductional abnormalities. The symptoms and genetic analyses indicated the possibility of an acquired condition rather than a hereditary retinal disease.


Acquired retinal disease Incomplete-type congenital stationary night blindness Negative-type ERG Post-phototransduction abnormality 



We thank Professor Emeritus Duco Hamasaki of the Bascom Palmer Eye Institute for the discussions and editing of the final version of the manuscript. We also thank Professor Yozo Miyake of Aichi Medical University and Professor Mineo Kondo of Mie University for his precious advice regarding our study.

This study was supported in part by a Grant-in-Aid for Scientific Research C (no. 16K11320 to S.U.) from the Ministry of Education, Culture, Sports, Science and Technology of Japan.

Conflicts of interest

S. Ueno, Lecture fees (Alcon, Chuo Sangio, Nidek, Santen); D. Inooka, None; M. Meinert, None; Y. Ito, None; K. Tsunoda, None; K, Fujinami, Grant (Astellas), Honoraria (Acucela, Astellas, Kubota), Lecture fee (Santen); T. Iwata, None; H. Ohde, None; H. Terasaki; Grant (Alcon, Allergan Japan, HOYA, Kowa, Nidek, Novartis, Otsuka, Pfizer, Santen, Senju, Wakamoto), Honoraria (Daiichi Sankyo, Rohto), Lecture fees (AbbVie, Alcon, Bayer, Carl Zeiss Meditec, Chuo Sangio, Graybug Vision, Kowa, Mitsubishi Tanabe, Nidek, Nihon Tenganyaku, Novartis, Otsuka, Pfizer, Sanofi, Santen, Senju, Takeda, Wakamoto), Equipment (Carl Zeiss Meditec), Patent (Nidek, Nagoya University graduate School of Medicine, and the author have the patent applied for image analyzing device for optical coherence tomography).


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Copyright information

© Japanese Ophthalmological Society 2019

Authors and Affiliations

  • Shinji Ueno
    • 1
    Email author
  • Daiki Inooka
    • 1
  • Monika Meinert
    • 1
    • 2
  • Yasuki Ito
    • 1
  • Kazushige Tsunoda
    • 3
  • Kaoru Fujinami
    • 3
    • 4
    • 5
  • Takeshi Iwata
    • 3
  • Hisao Ohde
    • 4
  • Hiroko Terasaki
    • 1
  1. 1.Department of OphthalmologyNagoya University Graduate School of MedicineNagoyaJapan
  2. 2.Department of OphthalmologyLund UniversityLundSweden
  3. 3.National Institute of Sensory Organs, National Hospital Organization Tokyo Medical CenterTokyoJapan
  4. 4.Department of OphthalmologyKeio University School of MedicineTokyoJapan
  5. 5.Department of GeneticsUCL Institute of OphthalmologyLondonUK

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