Acute Unilateral Cone Dysfunction Syndrome

Abstract

Abstract: : Purpose: To report three Japanese patients who presented with an acute–onset, unilateral cone dysfunction. Methods: Three patients underwent routine ophthalmological examinations in addition to fundus photography, fluorescein angiography, Goldmann visual field testing, full–field electroretinograms (ERGs), multifocal ERGs, and rod–cone perimetry. One of these patients has been reported previously (Nomura et al. Ophthalmology, 2000). Results: The fundus examination and fluorescein angiograms were essentially normal at the onset for all patients. The corrected visual acuity in the affected eyes was 0.03, 0.06, and 0.16. A relative central scotoma was detected in all patients. Full–field ERG cone responses were essentially absent, but rod responses were within the normal range in all patients. Psychophysical rod–cone perimetry demonstrated that the cone system was severely impaired at nearly all locations tested whereas the rod sensitivity was normal. Multifocal ERGs were non–detectable in all affected eyes. The fellow eyes were completely normal in all cases. Conclusions: These findings suggest that there is a group of patients who have an acute–onset, severe unilateral cone dysfunction leaving the rod function intact.

Keywords: electroretinography: clinical • retina