Acute unilateral inner retinal dysfunction with photophobia: importance of electrodiagnosis
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CLINICAL INVESTIGATION
Acute unilateral inner retinal dysfunction with photophobia: importance of electrodiagnosis Toshiaki Hirakata1,2 · Kaoru Fujinami2,3,4 · Wataru Saito5,6 · Atsuhiro Kanda5 · Akito Hirakata7 · Susumu Ishida5 · Akira Murakami1 · Kazushige Tsunoda2 · Yozo Miyake8 Received: 21 May 2020 / Accepted: 17 August 2020 © Japanese Ophthalmological Society 2020
Abstract Purpose To establish with negative electroretinogram (ERG) the clinical entity of eight patients with unilateral severe photophobia, essentially normal fundus, good visual acuity, and severe cone and rod dysfunction. Study design Multicenter retrospective observation case series. Methods Comprehensive ophthalmologic examinations were performed, including best-corrected visual acuity (BCVA), full-field ERGs and multifocal ERGs (mfERGs), fundus photographs, and OCT. Systemic and genetic examinations were performed. Results The mean (± SD) age at the onset was 60.0 ± 8.4 years, and the six patients noticed severe photophobia in the affected eye in spite of almost normal fundus appearance and good BCVA. The dark-adapted bright flash ERGs in the affected eye had relatively well-preserved a-waves and depressed b-waves, i.e., a negative ERG. Cone ERGs and both b- and d-waves of the photopic long-duration ERGs were almost undetectable. Rod ERGs were severely reduced; however, only two patients complained of night blindness. In five patients, the mfERGs were extinguished in the periphery but preserved in the central retina, resulting in good BCVA. Electrophysiological findings indicated a severe diffuse dysfunction of the inner retina affecting bipolar cells of both ON- and OFF-pathways, and in five patients there was a reduction in the thickness of the inner nuclear layer. In seven patients the retinal arteries were attenuated. Anti-retinal antibodies were detected in the serum of two patients. No genetic causes were found. Conclusions The common features in the eight patients with unilateral negative ERGs suggest a new disease entity of unilateral acute inner retinal layer dysfunction. In most patients, the only subjective complain was photophobia. Keywords Unilateral bipolar cell dysfunction · Negative ERG · Photophobia · Normal fundus · Normal visual acuity
Introduction Corresponding Author: Toshiaki Hirakata Electronic supplementary material The online version of this article (https://doi.org/10.1007/s10384-020-00780-x) contains supplementary material, which is available to authorized users. * Toshiaki Hirakata t‑[email protected] 1
Department of Ophthalmology, Juntendo University Faculty of Medicine, Hongo 3‑1‑3, Bunkyo‑ku, Tokyo 113‑8431, Japan
Laboratory of Visual Physiology, Division of Vision Research, National Institute of Sensory Organs, National Hospital Organization Tokyo Medical Center, Tokyo, Japan
2
3
UCL Institute of Ophthalmology, London, UK
Negative electroretinograms (ERGs) are characterized by the smaller amplitude of b-wave than a-wave in bright flash mixed rod-cone ERG using full-field stimul
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