Foveal cone dysfunction syndrome.
Our objective was to describe and expand the clinical spectrum of a rarely detected, previously reported photoreceptor disorder restricted to the foveal cones. Three patients with bilaterally decreased acuity and hemeralopia were examined to exclude a structural, vascular, inflammatory, or degenerative process. Each patient underwent a full neuroophthalmic examination, including full-field and focal cone electroretinogram (ERG). All three patients had normal-appearing fundi, mild dyschromatopsia, central or paracentral visual field depressions, normal full-field photopic and scotopic ERGs, and markedly reduced focal, foveal cone ERG responses. One patient had a ring scotoma and an asymptomatic family member with abnormal full-field and focal cone ERG. The syndrome of acquired foveal cone dysfunction presents as a bilateral, painless, progressive central visual loss with minimal or absent fundus changes. It eludes diagnosis until focal, foveal cone ERG is performed.
ten Hove
M W
MW
Bascom Palmer Eye Institute, University of Miami School of Medicine, Florida, USA.
Siatkowski
R M
RM
Smith
J L
JL
D000368:Aged; D004596:Electroretinography; D005260:Female; D005584:Fovea Centralis / Q000473:pathology*; D005654:Fundus Oculi; D006801:Humans; D008297:Male; D008875:Middle Aged; D017949:Retinal Cone Photoreceptor Cells / Q000473:pathology* / Q000503:physiopathology; D012164:Retinal Diseases / Q000175:diagnosis* / Q000503:physiopathology; D013577:Syndrome; D014786:Vision Disorders / Q000175:diagnosis / Q000503:physiopathology; D014792:Visual Acuity; D014794:Visual Fields